Rare Diseases: Neuromuscular 3

What are the domains of STG for patients with PPS?

Energy conservation and fatigue management Lifestyle mod: loos weight Use of AD and orthotics Postural correction and modified strength and endurance

What is the best type of stretching to avoid contractures?

Low load and sustained stretching to protect from contractures resting or night splints as well.

Are the following upper motor neuron, lower motor neuron or bulbar impairments of ALS? Muscle weakness Hyporeflexia Hypotonicity Atrophy Muscle cramps Faciculations

Lower motor neuron

CMT affects mainly the UMN or lower motor neurons?

Lower motor neuron

What pathology has a hallmark of hammertoe deformity in great toe with pes cavus or high arch?

MArie's TOOTH

What are the main incidence of brain tumors by types?

MEningionma- benigh, 33% of all tumors Blioblastoma multiform: malignant and in 17% of primary tumors Astrocytoma is 15% Pituitary is 8%

What are normal characteristics of impairments for Huntington's disease?

Muscle weakness and fatigue Hyperreflexia/Hypertonicity Difficulty swallowing and communication Atrophy Poor balance and trunk control and falls Impaired mood and cognition UMN signs!

What are the main goals for patients with brain tumor?

Must: Maximize function Provide task oriented movement strategies Emphasize various movement options to promote mobility

What is the hallmark sign of charcot marie tooth?

"Inverted coke bottle" shape of legs

What are the deformities with gait and the foot with charcot marie?

"Steppage gait" - dragging toes, high likelihood of stumbles and falls Hallmark = hammertoe deformity in great toes with pes cavus; high arches Intrinsic foot musculature atrophy alters rear-mid-forefoot mechanics and WB phases of gait Dry or shiny and flaky skin, hair loss in distal LE's, cool to touch Can progress to affect hands and wrists in later stages: May see cool hands and deformities similar to late stage RA

Describe the characteristics gait of CMT

"steppage gait" - dragging toes, high likelihood of stumbles and falls

What type of HA indicate a brain tumor HA?

HA: interrupts sleep, worse on waking, but improves throughout the day. Elicited by postural change, coughing and exercise More severe or of a different type than usual Associated with N&V, papilledema and focal neurological signs

What are the general signs and symptoms of a brain tumor?

HA: interrupts sleep, worse on waking, but improves throughout the day. Elicited by postural change, coughing and exercise More severe or of a different type than usual Associated with N&V, papilledema and focal neurological signs Seizures in 10-20% Altered mental status- initial symptoms are subtle, associated with frontal lobe and increased ICP Papilledema- swelling of optic nerve Vomiting and vertigo with posterior fossa types

What is the main difference between PD and HD?

HD is hyperkinetic movement disorder PD is hypokinetic movement disorder

What is the nature of HD impairments?

HD is hyperkinetic movement disorder Too much movement in limbs and trunk Unwanted and involuntary that can be rapid and jerky, or non-jerky Can be slow and writhing, rhythmical or continuous or unpredictable Hallmark sign is choreiform movements

What does the literature say about exercise with ALS?

What is too much or too little, as you don't want to overexerte them Fine balance between inadequate exercise and excessive exercise Moderately intense exercise may slow progression of disease, especially with copper gene mutation High intensity exercise can hasten disease and lead to death Evidence is limited though Exercise inconclusive of overall benefit. ***USE CLINICAL JUDGEMENT***

When does cerebellar stroke have best recovery?

When deep cerebellar nuclei are not involved

What is the difference between imbalance vs. leg incoordination during ataxic gait?

Imbalance has much greater impact on gait than leg incoordination Critical element of walking: stability during dynamic mobility while maintaing forward propulsion Patients with minimal balance deficits but (+) leg incoordination present with less gait abnormalities

What are the classic presentation of ataxic gait?

Imbalanced: leads to falls backward or ipsilesional Shortened steps Slow, irregular timing Unequal step lengths Wide BOS Veering along path with trunk and limbs Difficulty with turning and stopping, especially with speed.

Describe the impairments, activity limitations and participation restrictions for the middle phase of ALS

Impairments: Increased number of symptoms and severity AL: Minimal to moderate limitations PR: Begin to develop

What is the framework for middle rehab stages of HD in relation to ICF model?

Impairments: Increased severity and symptoms AL: Mild to mod limitations PR: More pronounced Prevent secondary complications and try and maintain best quality of life. Change POC as needed

Describe the impairments, activity limitations and participation restrictions for the late phase of ALS

Impairments: Numerous and severe AL: Becomes dependent in all aspects of care PR: Dependent

What is the framework for late rehab stages of HD in relation to ICF model?

Impairments: Numerous and severe AL: Dependent in all aspects of mobility and self-care PR: Total dependence LOOK AT PAillative care

What is the framework for early rehab stages of HD in relation to ICF model?

Impairments: Variety of abnormal S&S AL: Minor limitations PR: Variable Don't seek to make large gains or improvements since it's progressive

Describe the impairments, activity limitations and participation restrictions for the early phase of ALS

Impairments: Variety of abnormal signs and symptoms AL: Minor limitations present PR: none

When is MRE using PNF recommended for patients with ALS?

Implemented earlier in disease progression Muscle grades of 4/5-5/5, hold up well to MRE. Focus on stronger muscles Generally no deletrius effects

Diagnose: Decreased muscle strength, muscle endurance, atrophy Fatigue, pain, NM weakness Cold intolerance

Post-polio

The following is part of rehab framework for what condition? No overexertion No intense exercise Compensation of function within capabilities and use of AD and social support Energy conservation

Post-polio syndrome

Where is cerebellar brain tumor most common?

Posterior fossa

Name the cerebellar stroke based off impairments: Vertigo, unsteadiness, walking ataxia, nystagmus

Posterior inferior cerebellar artery

What are the most common neuromuscular disorder outcome measures? Great for Charcot Marie, since it doesn't have own outcome measure

10 m walk test 4-stair climb Time to rise from floor TUG 30 sec chair stand test 2 or 6 MWT GMFM

What the typical NMD outcome measures commonly used?

10 m walk, 6 MWT 4-stair climb Time to rise from floor TUG, 30 sec STS Brook/Vignos scale GMFM All common for neuromuscular disorder

When is aerobic endurance recommended for patients with ALS?

10-15 minutes, due to their decreased fatigue

What % of polio patients have PPS 15 years later?

15-80%

What is the prognosis for NON-progressive cerebellar ataxia?

1st time ischemic cerebellar stroke: Recovery is EXCELLENT 83% have minimal to no residual deficits Deep nuclei incur damage = poorer outcome

What are the risk factors of ALS?

20% is hereditary cases by defect with gene encoding copper-zinc super oxide dismutase Neurotoxic exposure and diet Age Family history Gender and Western pacific desent: Men more than women

Describe spinocerebellar ataxia

30 different types Onset is mid-later life Genetic counseling needed before testing No pharm intervention

Describe the epidemiology and etiology of ALS

30,000 individuals live with this High incidence in Japan and Guam and western pacific Onset is typically mid to late 50's Men 7x greater than women Sporadic ALS > familial ALS 70-80% is limb onset 20-30% is bulbar onset

Describe the ALS assessment questionnaire

40 questions related to Quality of life 5 areas of health: mobility, aDL, eating, communication, emotional functioning 0-4 scale: worst to best health status

What are the 5 areas of health assessed on ALS assessment questionnaire

5 areas of health: mobility, ADL, eating, communication, emotional functioning 0-4 scale, based on worst to best health status

What are common examination findings in regards to sleep for patients with PPS?

50% prevelance Periodic limb movement

Describe the incidence and etiology of brain tumor

>60000 new adult cases and 4000 pediatric cases each each Increases in indicidence may be due to being better at diagnosising the probelm

What are the 3 domains of the FIM?

ADL function Mobility activities Speech and cognition Used for patients with brain tumors

The following are strengthening factors for what population? Concentric focus Moderate resistance Grade >3+/5 only strengthen these muscles Monitory for overwork weakness

ALS

The key differences for what population during the examination include the main 2 things: Evaluate patient subjective or observed functional problems related to respiration, caregiver tasks, rate of disease progression and enviornmental blocks Activity logs of pain, fatigue and effort

ALS

What is the degeneration and loss of motor neurons in SC, brainstem and brain?

ALS

What is the most common motor neuron disease?

ALS (amyotrophic lateral sclerosis)

What is the main outcome measure used for ALS?

ALS functional rating scale: Activity limitations ALS assessment questionnaire: for participation

Patients with progressive NM disorders love what type of exercise?

AQUATICS They love being able to move muscles more and carry less weight

Describe the prevalence of major cerebellar pathologies

Acquired: stroke <5% happen in cerebellum Degenerative non-hereditary: MS most common and cerebellar signs present in 10-50% of cases hereditary: Friedrichs ataxia SCA #6 most common

What are some acquired disorders of cerebellum?

Acquired: stroke, tumor, trauma, infection, toxicity, endocrine, MS

What are the 3 types of cerebellar pathology?

Acquired: stroke, tumor, trauma, infection, toxicity, endocrine, MS Degenerative non-hereditary: Multiple systems atrophy, idiopathic late-onset cerebellar ataxia, MS is both Hereditary: SCAtaxia or episodic ataxia, autosomal dominant disorders, Autosomal recessive dissorders. Friedrichs ataxia

The following describes what outcome measure?

Activity level 8 items, quantifies performance and not categorized Reliable and valid for SCA's

What is Poliovirus?

Acute anterior poliomyelitis virus or heine-medin disease Affect motor neurons of anterior horn cells of CNS Parital recovery can occurs, as motor unit is denervated, adjacent motor neurons and reintervating the large motor units

The following are Red Flags for what condition?

Acute onset of bulbar muscle dysfunction: Affects of swallowing and breathing Present with : Dysphagia, hypoventilation, sleep apnea and CP insufficiency

What are the exam red flags for patients with PPS?

Acute onset of bulbar muscle dysfunction: Affects of swallowing and breathing Present with : Dysphagia, hypoventilation, sleep apnea and CP insufficiency

What does evidence suggests for treatment approach in regards to cerebellar ataxia aerobic exercise and resistance training?

Advocated for those who won't return to baseline Aerobic endurance trainging and submax resistance trianing can reduce fatigability-which may reduce falls Caution with Friedrich's ataxia and cardiac comorbidity

What causes CMT (charcot marie)

Affects patients due to genetic variations. All present as hereditary, motor and sensory neuropathy Can be autosomal dominant or recessive Demyelinating or axonal degeneration affects afferent and efferent nerve conduction velocity and motor unit action potential

ALS usually affects what tracts and upper or motor neurons?

Affects: UMN in cortex Corticospinal tract and motor output affected Brainstem nuclei for CN 5, 7, 9, 10 and 12 are effected. Usually anterior horn cells are lost

What impacts the persons stability of state of health for PPS?

Aging Comorbidities Overuse

What are the main theories with postpolio?

Aging Physical activity and weightgain over time have caused metabolic failure to thrive of axons... due to previous polio ocndition Neurons that are previously affected are vulnerable

What is a role that plays a major part with onset of PPS, since poliovirus isn't reactivated in patients who had polio?

Aging!

Where are the most common locations of primary tumors?

All accross cortex in frontal, parietal and occipital = 17% 16% in cerbellum 11% in brianstem

The following describes exercise for patients in what population? Light to moderate resistive exercise is NOT contraindicated in progressive neuro disorders Light to moderate aerobic exercise can be beneficial for persons with neuro disorders Patients, MUST MUST MUST "listen to their levels of exertion and time needed for recovery after bouts of exercise Physical activity should be encouraged within the limits

All progressive neuromuscular diseases. Exercise doesn't HURT muscles. It doesn't make progression more rapid.

Name the cerebellar stroke based off impairments: Dysmetria, vestibular signs, facial sensory loss

Anterior

Describe the relationship of aging with PPS?

Anterior horn cells start to become lost in 5th decade By age 60, loss of neurons may be 50% or more Limited motor neuron pool with patients with polio now exists Overuse and fatigue of already weakend neurons Neurons previously affect are at risk for premature aging and failure

What are the 3 regions of the cerebellum?

Anterior lobe Posterior lobe Flocculonodular lobe

What is a treatment for cardiopulmonary for patients with post-polio syndrome?

Aquatic therapy, endurance train Cycle ergometer

Describe the ICARES scale:

Assess cerebellar ataxia International cooperative ataxia rating scale: Activity level 4 categories: postural control, limb movements, speech, occulomotor Used for progressive and non-progressive ataxia

What are the central mechanisms related to fatiguability of patients with PPS?

Assessed using TMS, with observed motor evoked potentials in the brain Weaker motor evoked potentials in those polio survivors Suggests changes in motor cortex ability to command that motor output = fatiguability?

What are the main motor impairments present with cerebellar ataxia?

Ataxia or drunken gait... Dysmetria Dyssynergia Dysdiadochokinesia: impaired rapid alternating movement between antagonist and agonist Rebount affect: inability to stop movement following isometric contraction action tremor: ilicited during movement Hypotonia Imbalance and gait, occulomotor and speech and motor learning

Your patient presents with the following diagnosis: Overly high step with excessive hip and knee flexion in swing. Forceful and uncontrolled lowering of leg in stance. Shortened steps, slow, wide BOS and unequal step length with walking They are imbalanced and fall backwards often

Ataxic gait

Describe Huntington's disease

Autosomal Dominant inherited movement disorder. "Hereditary cortical Neurodegeneration" Disease of the basal ganglia structures

Describe what the treatment approach should be for the following impairment for someone with cerebellar ataxia: Altered timing of stepping

Avoid dual task Conscious attention to gait

What are typical STG for patients with PPS?

Balance with functional activities Pain reduction Functional endurance and respiratory function Transfer ability

What is the classification system for tumors? What is it based on?

Based on microscopic characteristics PRimary vs. secondary tumor (metastatic)

Describe the clinical diagnosis for Charcot Marie Tooth

Based on: Onset and characterization of symptoms Family history and pedigree Genetic testing to confirm CMT allele EMG findings to confirm demyelination or axonal degeneration through decreased NCV, MUAP or increased F wate latency Nerve biopsies

For patients with ALS, that exercise unsupervised, what do you warn them about?

Be cautious about the following: Morning fatigue, muscle pain beyond 48 hours Patient feels weaker within 30 min of exercise Excessive soreness, muscle cramping and SOB.

Why is CMT a zebra?

Because when you see it you will know!!! Foot is super high arch, with a thor like hammer toe and flakey skin hands have super bony deformities that looks like RA

What are typical activity limitations for a patient with cerebellar ataxia?

Bed mobility Transfers Gait: Pertubations, inclines, obstacle negotiation, wide vs. narrow BOS Stair climbing ADL for limb use: help from OT

Describe the clinical presentation and treatment of stage 6 of ALS

Bedridden and completely dependent on ADL Treatment: Continue family education, positioning, turning schedule, use of lift devices to change position and transfer Impairment specific: dysphage, soft diet, adaptive feeding, tube feeding Salivation- surgery and suction Dysarthria-speech amplication and palatal life Respiratory-clera airway, trach, vent Requires many health care members

Describe the DC planning and hospice considerations for patients with Brain tumor?

Begins as early as possible Palliative or hospice if medical treatment is no longer beneficial Therapy may still may be indicated Community resources and support Provide comfort to patient as they go through end of life.

What are some things with questionable research for patients with cerebellar ataxia?

Biofeedback and EMG: with limited carryover for auditory feedback for stepping speed Can improve feeding and postural sway TMS: or deep brain stimulation: reduction of motor cortex excitability. Does improve hand function and clinical measures Weighting: slows movement but no carryover Reducing upper limb tremor or ataxia: manipulation of visual information can improve movement from memory Lycra compression: insufficient evidence Promote independence of mobility: only with adequte trunk support and alternative control

What does the evidence say for biofeedack and transcranial magnetic stimulation or DBS for patients with cerebellar ataxia?

Biofeedback and EMG: with limited carryover for auditory feedback for stepping speed Can improve feeding and postural sway TMS: or deep brain stimulation: reduction of motor cortex excitability. Does improve hand function and clinical measures. Research is all in on this! ***Focus more on gait and balance training though***

The following are S&S of what pathology? HA: interrupts sleep, worse on waking, but improves throughout the day. Elicited by postural change, coughing and exercise More severe or of a different type than usual Associated with N&V, papilledema and focal neurological signs Seizures in 10-20% Altered mental status- initial symptoms are subtle, associated with frontal lobe and increased ICP Papilledema- swelling of optic nerve Vomiting and vertigo with posterior fossa types

Brain tumor

The following are red flags for what? HA, Seizures Focal weakness Ataxia, Aphasia Mental and behavioral limitations Signs of increased ICP

Brain tumor

What cranial nerves are usually affected with ALS?

Brainstem nuclei for CN 5, 7, 9, 10 and 12 are effected. Usually anterior horn cells are lost

Describe episodic ataxia

Brief bouts of ataxia usually due to excitement, stress or exercise Lasts minutes to hours Responds to medication

Are the following upper motor neuron, lower motor neuron or bulbar impairments of ALS? Bulbar muscle weakness Dysarthria Dysphagia Sialorrhea: increased salivation Pseudobulbar affect: affects emotions

Bulbar

What are the impairments associated with bulbar associated ALS?

Bulbar muscle weakness Dysarthria Dysphagia Sialorrhea: increased salivation Pseudobulbar affect of emotions

What type of ALS is characterized by loss of swallowing and breathing?

Bulbar onset: 20-30%

What are the typical home or outpatient interventions for a patient with a brain tumor?

Caregiver training in home Continued recovery and compensatory methods Exercise principles for cancer related fatigue: Aerobic and resistance training.

PES CAVUS with high arch with some flaky skin in LE with steppage gait is characteristics of what?

CMT

What pathology has the following gait? "steppage gait" - dragging toes, high likelihood of stumbles and falls

CMT

What population has the following rehab management? Major aim is to maintain independence and QOL as disease progresses Frequent skin checks on feet to avoid ulceration consult OT Fall prevention/balance program. BALANCE INTERVENTIONS IS KEY!!! AD for mobility matching level of progression Home assessment/evaluation and modifications Family and caregiver education/safety planning!!! this is ALSO KEY!! for safety and optimal function

CMT

The following describes the epidemiology and etiology of what disease? Affects patients due to genetic variations. All present as hereditary, motor and sensory neuropathy Can be autosomal dominant or recessive Demyelinating or axonal degeneration affects afferent and efferent nerve conduction velocity and motor unit action potential

CMT Charcot marie tooth

What pathology often has signs and symptoms with chronic inflammatory demyelinating polyneurothaty. But there chief complaints don't involved numbness or tingling?

CMT (Marie tooth)

What pathology do they often NOT complain of paradoxically numbness or tingling, pain or weakness in distal LE?

CMT (tooth)

What causes "coke bottle shape" LE with CMT?

Calf and ankle DF musculature atrophy is hallmark sign of CMT

What are the secondary effects of chemo following a brain surgery?

Can be very taxing and fatiguing.

What is the major goals and aims of Huntingtons disease?

Can't alter disease trajectory and will get worse: Therefore --> Maintain independence and QOL as disease progressions. Utilize other professions. SPEECH is HUGE for feeding/swallowing/talking Fall prevention/balance program Family and caregiver education and safety planning Home assessment and evaluation

What are the brain structures most commonly affected with Huntington's?

Caudate nucleus atrophy and enlarged lateral ventricles

The following are systems review considerations for what population? Fatigability: this pathology leads to increased effort of movement Monitor 2 or 6 min walk test, sustained aerobic workload, monitor vitals and RPE MSK endurance: max reps recorded of various muscle groups before reduced force or ROM observed

Cerebellar ataxia

The following are key body structure function considerations for a patient with what pathology? Muscle tone is usually hypotonic voluntary movement coordination is important to test: finger taping, supination/pronation, handwriting, toe tapping Static and dynamic balance with pertubations or narrow BOS Oculomotor performance: smooth pursuit, saccades, gaze evoked nystagmus

Cerebellar ataxia or pathology

Compensation mechanism of partially denervated motor units in muscles and rehab is a peripheral mechanism for loss of strength with PPS. Describe this

Changes the structural muscle fiber and changes from type II to type I Altered phenotypic expression of light and heavy myosin chains may affect changes in speed of contraction of type I fibers This structure change may explain challenge for the muscles to adapt to mechanical constraints

The following is the rehab summary for what condition: Multidisciplinary Utilize aquatics when safe and no contraindications Incorporate non-WB and partial WB cardiorespiratory fitness Allow for longer rest breaks in between bouts of work/exercise. Light-mod strength training is NOT contraindicated and there is no evidence that it shows disease progression Patient and family caregiver education is imperative Consider who factors and females may complain about AFO wearing

Charcot Marie

The following is the medical management for what disease? NSAID's or corticosteroids: consider the timing treatments of fatigue from ADL's Patients may be on antidepressants as well. On NSAIDS or corticosteroids to target the inflammatory degeneration process.. Multidisciplinary Symptomatic mangement and use of lyrica for neuropathic pain

Charcot Marie Tooth disease

The following is the pathophysiology of what pathology? Genetic mutation causes abnormalities in mostly peripheral nerve function Over time, demylination and axonal degeneration leads to neuromuscular impairment as seen in other disorders like MS Leads to gross motor weakness and sensory dysfunction Progression is natural in time Preferentially selective to distal extremities Advances in distal to proximal fashion

Charcot Marie Tooth disease

The following are gait and foot abnormalities with what pathology? "Steppage gait" - dragging toes, high likelihood of stumbles and falls Hallmark = hammertoe deformity in great toes with pes cavus; high arches Intrinsic foot musculature atrophy alters rear-mid-forefoot mechanics and WB phases of gait Dry or shiny and flaky skin, hair loss in distal LE's, cool to touch Can progress to affect hands and wrists in later stages: May see cool hands and deformities similar to late stage RA

Charcot Marie's tooth

The following are characteristics of what pathology? Intrinsic foot musculature atrophy alters rear-mid-forefoot mechanics and WB phases of gait Dry or shiny and flaky skin, hair loss in distal LE's, cool to touch

Charcot marie tooth

The following are rehab management and care considerations for what population? Will often need increased support from caregivers as disease progresses and increasingly impact function Often requires AFO's, GRAFO's and AD for ambulation. High risk for LE sprains SPC and walking sticks preferred initially Females don't ususally want AFO's for cosmetic reason

Charcot marie tooth

The following are the main impairments with what pathology? Muscle Weakness and fatigue Hyporeflexia Selective hypotonicity Atrophy Poor balance and trunk control Falls LMN deficits

Charcot marie tooth

What is one of the most common disorders of the rare disorders?

Charcot marie tooth disease

What is the most commoly inherited neuropathy?

Charcot marie tooth disease

The following is the clinical manifestation of what pathology? Disease presentation: S&S can mimic chronic inflammatory demyelinating or alcoholic polyneuropathy Paradoxically often DO NOT c/o numbness or tingling, pain or weakness in distal WL Calf and ankle DF musculature atrophy (hallmark = "inverted coke bottle" shape) Stumbles, falls, clumsy gait are very common Falls tend to be underreported

Charcot marie tooth's

The following are rehab interventions for what population? Stretching to avoid contractures in hands, may need bracing or splints in later stages. Low load and sustained stretching to protect from contractures Resting or night splints, serial casting of ankles Continual evaluations for DME and assistive devices May need protective socks similar to diabetic neuropathy

Charcot marie tooth. Protect them feet as well...

The following are exam considerations for what pathology? Pt Hx and systems review Tests and measures for fall questionnaires, balance outcomes, and functional ambulation GMFM and LEFS Disease-specific measures and patient reported impacts

Charcot marie tooths

The following describes the severity of what disease Variable in severity and age of onset of symptoms ranges from early childhood to early adulthood. Extremely variable in disease presentation

Charcot mariet tooth CMT

The following describes the prevalance and information for what pathology? rare neuromuscular disorder Most commonly inherited neuropathy: One of most commonly inherited human disease and 10x more common than ALS and HD combined! 125-150,000 in US Often undiagnosed or misdagnosed due to its overlapping symptomology

Charcot-Marie Tooth disease

What is typical medical treatment for patients with brain tumors?

Chemo, radiation and sterotatic radiosurgery Chemo: used with radiation, surgery or alone Methotrexate used for CNS Radiation: For malignant tumors. 6 weeks, 1-5 minutes a day Adverse effects of healthy tissue and burns Sterotatic radiosurgery: Gamma knife: deeply embedded small tumors, robotic arm to focus beams of radiation to target tumor. Minimal damage to healthy cells

What is hallmark sign of Huntington's?

Choreiform movements

What characterizes Huntington's disease?

Choreiform movements Later stage profound dementia associated with cortical destruction Atrophy of Caudate Nucleus- enlarged lateral ventricles seen on imaging

The following describes what charactertic? Hyperkinetic movements in limbs Rapid-jerky-involuntar-writhing and uncontrollable In late stages: present as mixed neurodegenerative movement disorder with elements of hypokinetics due to obliteration of basal ganglia structure

Choreiform movements in regards to Huntington's

For our interventions how should we adapt for fatigue, low blood count or GI complaints for patients with brain tumor?

Clinical decision making should be used: will my patient's health and safety be compromised? Flexibility of providing intervention: timing and patient presentation Considerations of cognitive effects: similar to patients with stroke or TBI

WHat is the medical management of Huntingtons'

Clonazepam to depress CNS and control chorea Haldol or tetrabenzine. Consider timing of treatments due to lethargy and somnolence. Pt may be on anti-psychotics, anti-anxiety or antidepressants Multidiscipinary Symptom management: Palliative care in later stages

What are the PT examination and considerations of ALS?

Cognition/psychosocial function Pain Joint integrity/rom and muscle length Muscle performance and motor function CN integrity and tone and reflexes Sensation/integumentary Postural alignment, control/balance, gait functional status, enviornmental barriers and fatigue Respiratory failure

What are the goals for PPS?

Collaborative effort between family you and patient Focus on symptom management Achievement depends on commitment and compliance LTG: Self management of HEP and lifestyle changes STG: symptoms mangement QOL considered

For patients with cerebellar ataxia, are the following compensatory or recovery strategies: Slow down movement, visual cues, widen gait, minimize distraction. Using AD only if can be managed safely

Compensation

What are peripheral mechanisms of PPS leading to loss of strength?

Compensation mechanism of partially denervated motor units in muscles and rehab Denervation can induce metabolic changes in muscles Enlarged motor units due to recovery process of initial virus create instability and may lose axons with intense overuse

What is the suggested treatment focus for patients with gait ataxia, based on the location of lesion who had a vestibular problem?

Compensatory head fixing or train VOR Strength/balance exercises

When strengthening muscles, what do you focus on for patients with ALS?

Concentric focus Moderate resistance Grade >3+/5 only strengthen these muscles Monitory for overwork weakness

Describe what the treatment approach should be for the following impairment for someone with cerebellar ataxia: Reduced automaticity of walking

Conscious attention to what they are doing.. Gait training, have them pay attention a lot High intensity!!!!!!!!!

What are the PT considerations for examination of Huntington's?

Consider early hyperkinetic stages and later stages may display hypokinetic features Pt Hx Disease specific measures: Patient reported impacts UHDRS- unified HD rating scale

What side effects should we consider for a patient with a brain tumor?

Consider fatigue from chemo or radiation Fatigue due to central fatigue from CNS Low blood count Gastrointestinal complaints

If a patient has a brain tumor and a poor prognosis, what should we focus on?

Consider focus on family training, DME, palliative care/Hospice needs

Why you need continuous reassessment for patients with a brain tumor?

Continuous reassessment due to side-effects of treatment include cerebral edema, hydrocephalus, tumor regrowth and infection

What is critical element of walking?

Critical element of walking: stability during dynamic mobility while maintaing forward propulsion

What are some superior cerebellar artery stroke impairments?

Dysmetria of ipsilateral arm. Unsteady walking, dysarthria and nystagmus Common coordination and dysmetria are always IPSILATERAL!!!

Damage to normal muscle fibers with repetitive eccentric work is a negative outcome for ALS exercise prescirption. Describe why this occurs

Damage to normal muscle fiber with repetitive eccentric work: Normal muscle in a patient with ALS may not have the repair capabilities

What happens with normal aging?

Decreased muscle strength and endurance Joint pain and limitations Multifactorial health issues-leading to functional loss

What are the primary symptoms of post-polio?

Decreased muscle strength and endurance in both previously affected and unaffected muscles Pain and fatigue

What is the main MS problem with PPS?

Decreased muscle strength, endurance and fatigue

When is supervised gentle exercise recommended for patients with ALS?

Decreased pain and stiffness Improved psychosocial

What are some anterior cerebellar artery stroke impairments?

Dysmetria, vestibular signs, facial sensory loss

What are the late effects of polio syndrome from chronic impairments?

Degenerative arthirtis from overuse Bursitis and tendonitis

WHat are some degenerative non-hereditary cerebellum disorders?

Degenerative non-hereditary: Multiple systems atrophy, idiopathic late-onset cerebellar ataxia, MS is both

What is the main phyisological problems with CMT? marie tooth

Demyelinating or axonal degeneration affects afferent and efferent nerve conduction velocity and motor unit action potential

What is the clinical manifestations of CMT?

Disease presentation: S&S can mimic chronic inflammatory demyelinating or alcoholic polyneuropathy Paradoxically often DO NOT c/o numbness or tingling, pain or weakness in distal WL Calf and ankle DF musculature atrophy (hallmark = "inverted coke bottle" shape) Stumbles, falls, clumsy gait are very common Falls tend to be underreported

What are the disease specific outcomes measures for HD?

Disease specific measures: Patient reported impacts UHDRS- unified HD rating scale Also use balance scales and fall reporting since this is very common

For people with PPS, they may have trouble with Walking Climbing stairs and dressing PR with meeting demands of work or home. For what reasons?

Due to primary weakness from overuse, or secondary disuse

What helps detect seizure activity?

EEG

When you consider a treatment approach for CMT you should consider what?

Early in the day to prevent ADL fatigue Multidisciplinary approach. they may be on antidepressants too control disease

Describe the Framework for rehab for CMT stages

Early: Variety of abnormal S&S, with minor limitations present with AL and variable PR. Middle: Increased number of S&S and severity: moderate to minimal limitations with ADL's, PR are more pronounced Late": Numerous and severe impairments, AL become dependent in all aspects of mobility and self-care. Total dependence in PR.

What is a treatment for fatigue for patients with post-polio syndrome?

Energy conservation PAcing, rests, naps Lifestyle changes Lightweight orthotics, power mobilty

What does the research say about exercise and brain tumor?

Evidence supports aerobic and resistive program related to: Reversing effects of muscle wasting from prolonged steroidal use Cancer related fatigue syndrome

Exercise intolerance is a negative outcome for ALS exercise prescirption. Describe why this occurs

Exercise intolerance: Mitochondrial dysfunction, abnormal muscle metabolism, impaired activation and central activation failure

True or False: For patients with PROGRESSIVE neurodenerative disorders, light to moderate resistive exercises is NOT contraindicated

False: They CAN!! But be gentle and allow patient to listen to body and give patient extra time between bouts like CMT and ALS

What is key factor for exercise with progressive NM diseases?

Importantly, patients must "listen" to their levels of exertion and time needed for recovery THEY FATIGUE VERY QUICKLY

What are the main portions of systems review of examination of cerebellar pathology?

Fatigability: cerebellar damage leads to increased effort of movement Monitory 2 or 6 min walk test, sustained aerobic workload, monitor vitals and RPE MSK endurance: max reps recorded of various muscle groups before reduced force or ROM observed

What is the clinical trio that diagnosis PPS?

Fatigue Pain Deterioration of motor deficits

What zone of cerebellum controls eye movement and balance with regards to health of VOR?

Flocculonodular lobes

Due to weak DF, what could be seen with CMT?

Foot slap, but they compensate with steppage gait pattern

Describe the recovery-restorative approach framework for rehabilition

For non-progressive cerebellar ataxia Anticipated improvement based on examination

What is the PT role for a patient in the hospital for a brain tumor?

Functional Eval, family support Schedule home eval and education Mobility and positioning, mobility training and safety Family training, AD and f/u

What is the main outcome measure PT's use for brain tumors?

Functional independence measures: ADL function, mobility activities and speech and cognition Analysis of 3 domains to guide POC

What are the main outcome measurements for brain tumor?

Functional independence measures: ADL function, mobility activities and speech and cognition Physician evaluative tool: Karnofsky Performance sclae index: 0-100, level of independence. Health related Quality of life: physical, emotional, spiritual and intellectual functioning State-trait anxiety inventor Self-rating depression scale

What is the common gait analysis for patients with PPS?

Gait analysis --> Overuse and substitution of vastus lateralis, biceps femoris, glutes max and soleus not functioning

What does the research say about interventions for cerebellar ataxia?

Gaps in evidence. Most effective intervention approahc has NOT yet been established Non randomized, non controlled, smalle sample size literature only avaliable. Not randomized control trails

Describe the pathophyiology and natural history of Charcot marie tooth

Genetic mutation causes abnormalities in mostly peripheral nerve function Over time, demylination and axonal degeneration leads to neuromuscular impairment as seen in other disorders like MS Leads to gross motor weakness and sensory dysfunction Progression is natural in time Preferentially selective to distal extremities Advances in distal to proximal fashing

What is important when testing static and dynamic balance for a patient with cerebellar ataxia?

Give pertubations and do in sitting and standing

What is the part of POC for ALS?

Goal development that is collaborative with patient for psychosocial components Progressive diagnosis, so don't cure or delay progression Broad goals: Maximize independence and positive quality of life Address psychosocial and caregiver issues

What is the next steps after a brain tumor is found on imaging?

Goal of surgery to maximize tumor resection Biopsy to make diagnosis with ultimate goal for complete resection Ciopsies- open, needle or sterotactic Resections occur with craniotomy as patients are sometimes awake. Scary terry.

What are the interventions based on for patients with brain tumor?

Goals from patient

Describe what the treatment approach should be for the following impairment for someone with cerebellar ataxia: Reduce adaptability in environment

Graded exposure, sensory cues Conscious attention to what they are doing

What are Red flags for referal because you suspect a primary brain tumor or a gliomas?

HA, Seizures Focal weakness Ataxia, Aphasia Mental and behavioral limitations Signs of increased ICP

Describe the clinical presentation and treatment of stage 4 of ALS

Hanging arm syndrome, shoulder pain, edmatous hand , W/C dependent, severe LE weakness, can perform ADL with fatigue Treatment: Heat/massage for spasm, edema prevention, AA and PROM of limbs Isometric contractions to tolerance, orthotic UE support- sling/trays and power mobility

What are the key differences for examining someone with PPS?

Health hx: growth and developmental history and family history More complex systems review includes: sleep, temperature intolerance, fatigue, pain and ageing process Tests and measures with emphasis on MSK system Ask about systems beyond the main ones... sleep? temperature?

What is the main self-questionnaire for a patient with brain tumor?

Health related quality of life to address physical, emotion and intellectual functioning and spiritual

What is the ALS functional rating scale used for?

Helps measure how patient functions with activity limitations. 12 questions about UE function, putting clothes on, walking and turning in bed and respiration. Will guide treatment planning and make goal important to patient. Also can refer to OT and pulmonary

What causes brain tumors?

Hereditary in theory Casual relationship with: toxins, chemical, organic solvents and rubber Farming, manufacturing Ionizing radiation or electromagnetic fields

What are some hereditary cerebellum disorders?

Hereditary: SCAtaxia or episodic ataxia, autosomal dominant disorders, Autosomal recessive dissorders. Friedrichs ataxia

Who is most affected by ALS?

High incidence in western pacific Men > Women 7x

CMT is associated with what ankle problems and gait considerations?

High risk for LE sprains. Usually require AFO's and need SPC or walking sticks for gait to not fall

Describe the clinical manifestation of ALS

Highly variable Depends on extent of motor neuron loss, degrees and combination of UMN and LMN, pattern of onset, and progression, body regions affected stage of disease Usually symptoms are focal and asymmetrical at onset

How is the medical diagnosis of a brain tumor completed?

History of present illness Neurologic exam Tumor imaging

The following are impairments related to what pathology? Muscle weakness and fatigue Hyperreflexia/Hypertonicity Difficulty swallowing and communication Atrophy Poor balance and trunk control and falls Impaired mood and cognition UMN signs!

Huntington's

The following care considerations are important for what pathology? Can't alter disease trajectory and will get worse: Therefore --> Maintain independence and QOL as disease progressions. Utilize other professions. SPEECH is HUGE for feeding/swallowing/talking since UMN disease Fall prevention/balance program Family and caregiver education and safety planning Home assessment and evaluation

Huntington's

The following happens with what pathology? Too much movement in limbs and trunk Unwanted and involuntary that can be rapid and jerky, or non-jerky Can be slow and writhing, rhythmical or continuous or unpredictable

Huntington's

The following is medical management for what pathology? Clonazepam to depress CNS and control chorea Haldol or tetrabenzine. Consider timing of treatments due to lethargy and somnolence. Pt may be on anti-psychotics, anti-anxiety or antidepressants Multidiscipinary Symptom management: Palliative care in later stages

Huntington's

The following is the pathophys of what disorder? Over time, gene mutation leads to neuro impairment Cortical destruction and atrophy occurs in caudate nucleus Late stage profound dementia May have mood/psychiatric involvement Life expectancy: 15-25 years beyond symptoms Progressive degeneration with enlarged lateral ventricles

Huntington's

The following describes what pathology? Autosomal Dominant inherited movement disorder. "Hereditary cortical Neurodegeneration" Disease of the basal ganglia structures

Huntington's Disease

The following are characteristics of what pathology? Choreiform movements Later stage profound dementia associated with cortical destruction Atrophy of Caudate Nucleus- enlarged lateral ventricles seen on imaging

Huntington's disease

The following is the epidemiology and etiology of what pathology? Typically between 45-50 y.o 5/100000: Affects males slightly more than females >40 "CAG" repeats on HTT allele of Chromosome #4. Genetic mutation = abnormal folding of protiens in neurological structures of brain and basal ganglia

Huntington's disease

The following are care considerations for what pathology? Rhythm and cadence via auditory stimulus (metronome) had positive effects on gait disturbances Smaller amplitude motor control emphasized like Tia Chi Relaxation techniques may be helpful in reducing hallmark sign of this pathology PNF techniques and rhythmic stabilization for trunk control Timed therapy after meds to maximize session

Huntingtons' disease

What is the main part of examination and evaluation for a patient with brain tumor?

Hx of occupational status, support, leisure and personal goals Neurological exam, multisystem assessment and functional assessment Evaluation includes clinical problem list, realistic and meaninguful goals Discharge planning Address patients as a whole!!!

Huntington's disease is a hyper or hypo kinectic movement?

Hyperkinetic Choreiform movements are hallmark of disease

What is choreiform movements found in Huntington's?

Hyperkinetic movements in limbs Rapid-jerky-involuntar-writhing and uncontrollable In late stages: present as mixed neurodegenerative movement disorder with elements of hypokinetics due to obliteration of basal ganglia structure

PPS is a disorder of exclusion. What do we need to rule out?

Hypothyrodism and other endocrine Respiratory and sleep apnea Cardiac symtoms RA, joint affections SMA, ALS, Cauda equina, MS, radiculopathy, lumbar stenosis

What are the diagnosis specific outcome measurements for cerebellar ataxia?

ICARES: international cooperative ataxia rating scale SARA: scale for the assessment and rating of ataxia

According to the research of ALS exercise prescription, what did it say about moderate intensity aerobic and resistance exercise?

Improved ALSFRS and Ashworth scores at 3 months, but not 6 months vs. usual care

According to the research of ALS exercise prescription, what did it say about moderate intensity resistance and stretching programs?

Improved ALSFRS, and short form 36 and voluntary maximum isometric contraction scores vs. non exercise group

According to the research of ALS exercise prescription, what did it say about treadmill walking to anaerobic threshold with ventilation for 1 year?

Improved functional independence, lowered rate of decline of respiratory and muscle endurance vs. control

Why is the rehab framework for PPS, hard for patients to grasp?

Initially after polio virus, it was all about no pain no gain and going balls to the wall to intense exercise and recovery. For PPS they need to chill and protect themselves from muscle overuse. Use AD and be a bro

Describe the framework for rehab for patients with PPS that is used in regards to initial viral infection protocol. Is this a good concept for PPS?

Intense exercise Stretching and strengthening regimen daily Use of ambitious compensations methods to function: muscle substitution, ligaments for stability and use of muscles at high levels of capacity Use of AD and orthotics NO! don't use this. do the opposite for PPS

What zone of cerebellum controls coordination of agonist-antagonist muscle pairs during variety of movement- walking and limb control? Dysdiadokinesia occurs as result of dysfunction

Intermediate zone

Describe the intermediate zone functions of the cerebellum

Intermediate zone: controls coordination of agonist-antagonist muscle pairs during variety of movement- walking and limb control

The following describe what scale? Activity level 4 categories: postural control, limb movements, speech, occulomotor Used for progressive and non-progressive ataxia

International cooperative ataxia rating scale ICARES

What is part of energy conservation for patients with PPS?

Is one trip sufficient? Can you make activity less effort full? Use AD? Break activity into parts? Can someone else help?

What is part of functional rehab of PPS?

Isokinetic and isometric endurance training PT for compensatory needs Energy conservation through behavioral changes

Why is locomotor training with BWS on treadmill not good for patients with gait and balance?

It doesn't address balance

What is the major rehab management for Charcot marie tooth?

It will get worse over time, so we must try our best to preserve function. Major aim is to maintain independence and QOL as disease progresses Frequent skin checks on feet to avoid ulceration consult OT Fall prevention/balance program AD for mobility matching level of progression Home assessment/evaluation and modifications Family and caregiver education/safety planning

What are the main non-motor impairments of cerebellar pathology?

Language processing working memory Learning associations between objects High order executive functions Loss of control over emotional behaviors

Describe the epidemiology of PPS?

Last epidemic of polio was in 1950... Before 600,000 children per year affected Causes the most motor deficits in the world! Prevelance of PPS is 15-80%

What zone of cerebellum controls complex, multijoint voluntary limb movement , especially with visual guidance; planning of complex movements and error detection?

Lateral zone

Describe the lateral zone functions of the cerebellum

Lateral zone: controls complex, multijoint voluntary limb movement , especially with visual guidance; planning of complex movements and error detection.

What is a steppage gait that is seen with CMT?

Lift knees and hike hips to compensate for DF weakness

Describe the exercise considerations for Nueromuscular disease

Light to mod resistive exercises is not contrainidcated in progressive NM disorders Light to mod aerobic exercise can be beneficial for persons with NM disease Importantly, patients must "listen" to their levels of exertion and time needed for recovery Physical activity should be encouraged

Describe the effects of exercise on patients with Charcot Marie tooth

Light to moderate resistive exercise is NOT contraindicated in progressive neuro disorders Light to moderate aerobic exercise can be beneficial for persons with neuro disorders Patients, MUST MUST MUST "listen to their levels of exertion and time needed for recovery after bouts of exercise Physical activity should be encouraged within the limits

What are some good specific goals for patients with ALS?

Maintence of mobility and independence and safe function for patient and caregiver Maintain max muscle strength and endurance within limits of ASL Prevention of secondary complications Management of energy conservation and respiratory function Determine adaptive equipment and mobility needs Eliminate or prevent pain

What are the main ways to introduce exercise for patients with ALS?

Make them formal but enjoyable! Activities include participation and social opportunity

What is the main population that has brain tumors?

Males > Females White > Black americans Children 0-15 y/o or adults in 5th-7th decades of life

The following is the appropriate exercise prescription for what population?

Manual resistance exercises using PNF implemented early in disease progress Supervised gentle exercises: decreased pain and stiffness and improved psychosocial Aerobic endurance of 10-15 min, decreased due to central fatigue or decreased motor units

What is best exercise prescription of ALS?

Manual resistance exercises using PNF implemented early in disease progress Supervised gentle exercises: decreased pain and stiffness and improved psychosocial Aerobic endurance of 10-15 min, decreased due to central fatigue or decreased motor units

Which pathology is based on the following clinical diagnosis? Based on: Onset and characterization of symptoms Family history and pedigree Genetic testing to confirm CMT allele EMG findings to confirm demyelination or axonal degeneration through decreased NerveConducationVelocity, MUAP or increased F wate latency Nerve biopsies

Marie charcot tooth

Describe the ALS functional rating scale: What is ICF domain? How is it scored?

Measures functional status for patients with ALS 0-4 scale: with 4 being normal function Reliable and valid for measuring decline in function related to muscle weakness. such as turning in bed Revised version includes respiratory function

What are the only areas to receive afferent information from the spinal cord. AKA spinal cerebellum

Medial and intermediate zones of cerebellum

Describe the medial zone functions of the cerebellum

Medial zone: afferent sensorimotor state of the limbs. Efferent output of posture, muscle tone, upright stance, locomotion and in gaze

Describe the functions of the three longitudinal zones

Medial zone: afferent sensorimotor state of the limbs. Efferent output of posture, muscle tone, upright stance, locomotion and in gaze Intermediate zone: controls coordination of agonist-antagonist muscle pairs during variety of movement- walking and limb control Lateral zone: controls complex, multijoint voluntary limb movement , especially with visual guidance; planning of complex movements and error detection.

What does evidence say about aerobic training for patients with PPS?

Methodology of studies is questionable.. Suggest 3 or more times per week protocol

Describe the clinical presentation and treatment of stage 1 of ALS

Midly weak, clumsiness, ambulatory and independent with ADL Treatment: Continue normal activity or increase if sedentary Gentle PRF strengthening, no overuse, stretching and psych referral if needed

What is the main overview and aspects involved with a primary brain tumor?

Mimic presentation of CNS disorders Improved survival rate due to advances of medical field Aggressive nature of disease and treatment create barriers to intervention Understanding natural history and complications of treatment

What are the typical acute or ICU interventions for a patient with a brain tumor?

Mobilization in and out of bed Monitoring ICP and vitals Ventriculostomy or tube inserted in the CSF to drain Communication with RN Mobility equipement

Describe the clinical presentation and treatment of stage 2 of ALS

Mod- selective weakness, ambulatory, with slight decrease in ADL independence- climbing stairs and buttoning cloths Treatment: Stretching, cautious strengthening for MMT >3+/5. Orthotic and AD

What is the appropriate exercise intensity to work with patients with ALS?

Moderately intense exercise may slow progression of disease, especially with copper gene mutation High intensity exercise can hasten disease and lead to death

With huntington's how does atrophy of caudate nucleus affect normal function

Moderates output of motor information. Therefore you get too much movement

What are the exercise prescription factors for patients with ALS in regards to endurance?

Monitor closely for fatigue Limit to <15 min Include rest Monitor fluids and oxygen, Supportive, adaptive, patient and family focused

Describe the pathology of a cerebellar brain tumor

More common in children: Most common in posterior fossa Recovery of function and ataxic movement: poorer prognosis in adults Damage of deep cerebellar nuclei may predict recover better than age

What is ALS?

Motor Neuron Disease Inherited or sporadic disease Clinical disorder of UMN, LMN Or both Most common is ALS, which is degeneration and loss of motor neurons in SC, brainstem and brain

What does the poliovirus usually affect?

Motor neurons in the anterior horn cells. Apoptosis of affected motorneurons

What are the responsibilities of the cerebellum for normal function?

Movement! Coordination of movement, Adaptation of movement Vestibular Balance Motor learning. Connects to prefrontal cortex and impacts cognition-memory, emotional regulation and language processing

What is the overall rehab summary for charcot marie tooth?

Multidisciplinary Utilize aquatics when safe and no contraindications Incorporate non-WB and partial WB cardiorespiratory fitness Allow for longer rest breaks in between bouts of work/exercise. Light-mod strength training is NOT contraindicated and there is no evidence that it shows disease progression Patient and family caregiver education is imperative Consider who factors and females may complain about AFO wearing

What are the main impairments related to CMT?

Muscle Weakness and fatigue Hyporeflexia Selective hypotonicity Atrophy Poor balance and trunk control Falls LMN deficits

What are the key body structure function differences for a patient with ataxia or cerebellum pathology?

Muscle tone is usually hypotonic voluntary movement coordination is important to test: finger taping, supination/pronation, handwriting, toe tapping Static and dynamic balance with pertubations or narrow BOS Oculomotor performance: smooth pursuit, saccades, gaze evoked nystagmus

What are the impariments related to lower motor neurons signs of ALS?

Muscle weakness Hyporeflexia Hypotonicity Atrophy Muscle cramps Faciculations

What is a treatment for pain for patients with post-polio syndrome?

NSAID Stretching Lifestyle changes that promote --> Pacing of activity Orthotic/AD/power mobility Heating modalities and cryotherapy

Describe the medical management for marie charcto tooth's

NSAID's or corticosteroids: consider the timing treatments of fatigue from ADL's Patients may be on antidepressants as well. On NSAIDS or corticosteroids to target the inflammatory degeneration process.. Multidisciplinary Symptomatic mangement and use of lyrica for neuropathic pain

What is post-polio syndrome?

Nervous condition that is sequelae from the poliovirus PPS occurs up to and beyond 15 years after initial viral infection Considered a chronic neurological impairment

What are the new symptoms usually associated with PPS muscle weakness?

New symptoms of: fasciculations, cramps, atrophy, elevation of muscle enzyme in blood

What is medical management of ALS?

No cure Palliative treatment Symptomatic mangement Medications such as riluzole help prolong life Cannabis helps patients with spasms and spasticity

What is the framework for rehab with PPS?

No overexertion No intense exercise Compensation of function within capabilities and use of AD and social support Energy conservation Protect joints and shift from recovery to health promotion.

What is the main thing to determine before developing POC for someone with cerebellar ataxia?

Non-progressive or Progressive!! Do we promote recovery/restoration or do we promote compensation or health promotion

What is a treatment for Pulmonary for patients with post-polio syndrome?

Noninvasive ventilator assistance Pulmonary therapy and breathing exercises N *Need referral

Are there CMT specific outcome measures?

Nope

What are the key differences when examining someone with ALS vs. other populations?

Note bulbar and respiratory impairments Environmental blocks to independence Caregiver demands and education of disease Rate of disease progression Activity log of : pain level, fatigue and effort of tasks

What are intention tremors? How may this effect our treatment session?

Occur in terminal portions of visually guided movement If reduced with eyes closed it's intention tremors. Reduced with decreased vision and isometric contractions, so plan accordingly

What is part of oculomotor performance for a patient with cerebellar ataxia?

Oculomotor performance: smooth pursuit, saccades, gaze evoked nystagmus ***Very important to assess***

What is part of the clinical diagnosis of HD?

Onset of symptoms Family history of HD? Genetic testing to confirm HTT allele or CAG repeats Neuroimaging to identify changes in basal ganglia and cortical atrophy

What are the typical Inpatient interventions for a patient with a brain tumor?

Optimize function to reduce burden of care and safely DC: use fim Personal goals and interest with community integration and rec therapy Combined recovery and compensatory approach Caregiver training/problem solving Motor learning and repetition!!!!!

What are common examination findings in regards to mobility/posture for patients with PPS?

Osteoarthritis of wrist and hand Gait analysis --> Overuse and substitution of vstus lateralis, biceps femoris, glutes max and soleus not functionning Decreased activity up to 16 hours sedentary

Describe the patholophysiology of Huntington's

Over time, gene mutation leads to neuro impairment Cortical destruction and atrophy occurs in caudate nucleus Late stage profound dementia May have mood/psychiatric involvement Life expectancy: 15-25 years beyond symptoms Progressive degeneration with enlarged lateral ventricles

What are considerations of perscription for PPS?

Overall inconclusive but... Apply to muscles with 3+/5 NONFATIGUING!!!!!!! supervised and testing every 3 months avg 12 wk program

Overwork damage is a negative outcome for ALS exercise prescirption. Describe why this occurs

Overwork damage: occurs in denervated muscles with <1/3 motor units Impairs dollateral sprouting

Although exercise sometimes works with ALS, what are the negative outcomes of exercise prescription?

Overwork damage: occurs in denervated muscles with <1/3 motor units Impairs dollateral sprouting Damage to normal muscle fiber with repetitive eccentric work: Normal muscle in a patient with ALS may not have the repair capabilities Exercise intolerance: Mitochondrial dysfunction, abnormal muscle metabolism, impaired activation and central activation failure ***Be attentive to their reports after program after instituting program With reports of morning fatigue or significant weakness

Cold intolerance is part of what pathology?

PPS

The following are functional rehab conditions for what pathology? Isokinetic and isometric endurance training PT for compensatory needs Energy conservation through behavioral changes

PPS

The following are secondary symptoms with what pathology? Sleep disorder, REspiratory disorder Dysphagia, dysarthria Fascicualations Joint deformities

PPS

The following are the primary symptoms of what disorder? Decreased muscle strength and endurance in both previously affected and unaffected muscles Pain and fatigue

PPS

The following is exercise prescription for what population? Overall inconclusive but... Apply to muscles with 3+/5 NONFATIGUING!!!!!!! supervised and testing every 3 months avg 12 wk program

PPS

This is the clinical trio of what disorder? Fatigue Pain Deterioration of motor deficits

PPS

The following are key differences when examining what population? Health hx: growth and developmental history and family history More complex systems review includes: sleep, temperature intolerance, fatigue, pain and ageing process Tests and measures with emphasis on MSK system

PPS ***Polio affected them as a kid, so what changed with them... GO MORE COMPLEX with respiration and potential issues surfacing

Drugs usually lack efficacy and effect on fatigue and strenght!!! for what condition?

PPS Therefore functional rehab is better

What does evidence suggests for treatment approach in regards to cerebellar ataxia gait and balance training?

Part task such as kneeling, sitting and quadruped Multifactoria training: gaze, static and dynamic activities. Address balance such as ankle mobility Treadmill training, visually guided stepping. Interventions MUST provide challenge and trail and error

What does evidence suggests for treatment approach in regards to cerebellar ataxia intensity and duration of intervention training?

Partial relearning is possible with select cerebellar population- 10 hrs/wk and 6 months

What is the main outcome measure Physicians' use for brain tumors?

Physician evaluative tool: Karnofsky Performance sclae index: 0-100, level of independence.

What causes the most motor deficits in the world?

Polio

The following are late concerns with what pathology? Degenerative arthirtis from overuse Bursitis and tendonitis

Polio virus

Denervation can induce metabolic changes in muscles for patients with PPS. Describe more about the peripheral mechanism

Possible decrease energy production in type I fibers- fatiguability

Quick or progressive loss of muscle strength/endurance of previously unaffected muscle in conjuction with cold intolerance and muscle fatigue, 15 years after another condition helps with diagnosis of what?

Post polio disease

The following are goals for what patient population? Collaborative effort between family you and patient Focus on symptom management Achievement depends on commitment and compliance LTG: Self management of HEP and lifestyle changes STG: symptoms mangement QOL considered

Post polio syndrome

The following are the causes of what pathology? Remaining genetic viral material could create immune response in CNS or PNS Imbalance between the dysfunction of degenerative and regenerative physiologic function of motor units Structural and functional impairments of muscle fibers

Postpolio

What is the presentation of muscle weakness with PPS?

Presentation is asymmetrical, proximal, distal or patchy, Noted more in repetitive and stabilization contractions Decreased ability to recovery rapidly after contraction Note noted with individual MMT as much

What are the things to note about muscle weakness with PPS?

Primarily affects muscles severely affected with initial infection Presentation is asymmetrical, proximal, distal or patchy, Noted more in repetitive and stabilization contractions Decreased ability to recovery rapidly after contraction New symptoms of: fasciculations, cramps, atrophy, elevation of muscle enzyme in blood

What are the classifications of primary vs. secondary brain tumors?

Primary: Gliomas, astrocytomas, glioblastoma multiforme exts Secondary: Metastatic and malignancies come from outside of CNS 25% with systemic cancer develop brain tumor 80% of cerebral tumor- frontal lobe 20% is posterior fossa 33% metastasizes to lungs

Describe the medical diagnosis process of PPS?

Prior episodes of paralytic polio --> Period of partial or complete neurologic recovery --> Extended interval of stability lasing >15 years --> Quick or progressive loss of muscle strength/endurance of previously unaffected muscle in conjuction with cold intolerance and muscle fatigue --> Symtoms progress for more than 1 year

What is the pathophysiology of ALS in connection to neuroantomical disease state of ALS?

Progresive degeneration and loss of motor neurons in the SC, brainstem and motor cortex Affects: UMN in cortex Corticospinal tract and motor output affected Brainstem nuclei for CN 5, 7, 9, 10 and 12 are effected. Usually anterior horn cells are lost

Describe the health promotion-compensatory approach framework for rehabilition

Progressive ataxia Anticipate worsening of impairments and activity

The following is the pathophysiology of what disorder?

Progressive degeneration and loss of motor neurons in the SC, brainstem and motor cortex Affects: UMN in cortex Corticospinal tract and motor output affected Brainstem nuclei for CN 5, 7, 9, 10 and 12 are effected. Usually anterior horn cells are lost

CMT leads to gross motor weakness and sensory dysfunction. How does this progress?

Progressive over time and advances in distal to proximal fashion

What is the prognosis for progressive cerebellar ataxia?

Progressively worsening clinical signs and symptoms

Describe the important PT exam considerations for Charcot Marie Tooth disease

Pt Hx and systems review Tests and measures for fall questionnaires, balance outcomes, and functional ambulation GMFM and LEFS Disease-specific measures and patient reported impacts

WHo is in charge of a brain tumor patient POC in the hospital? What do they do?

RN Initiate care plan, education Dx, tx, coordinate teams, train family on tube feeds, skin care and review home care and meds

What is radiation therapy used for?

Radiation: For malignant tumors. 6 weeks, 1-5 minutes a day Adverse effects of healthy tissue and burns

The synthesis should focus on what aspects for care of ALS?

Rate of disease progression goals Distribution of weakness, spastcity, respiratory factors, fatiguability and bulbar involvement Phase of disease Pre-existing impairments or activity limitations

Describe the key differences that you may encounter for someone with a brain tumor?

Really consider ICF and emotional impacts of care Pain is prominent Progressive decline Continuous reassessment due to side-effects of treatment include cerebral edema, hydrocephalus, tumor regrowth and infection Foundational knoledge on nature, flutuating stuats and anticipating progressive decline of brain tumor

What predicts recovery function for a cerebellar brain tumor?

Recovery of function and ataxic movement: poorer prognosis in adults Damage of deep cerebellar nuclei may predict recover better than age

Who has a huge role to eval their leisure activities, skill builing and community resources for a patient with a brain tumor in the hospital?

Recreational therapist

What is a treatment for dysphagia for patients with post-polio syndrome?

Referral to dietician Breathing and swallowing techniques and monitor fatigue

What is a treatment for Psychosocial for patients with post-polio syndrome?

Referral to support polio group Counseling services/vocational counseling/interdisciplinary Behavioral modifications

What does evidence say about the pathophysiology of PPS?

Remaining genetic viral material could create immune response in CNS or PNS Imbalance between the dysfunction of degenerative and regenerative physiologic function of motor units Structural and functional impairments of muscle fibers

What is the clinical diagnosis process of ALS?

Requires presence of LMN, UMN or progression of disease within a region or to other regions Absence of electrophysical and pathological evidence of other disease Neuroimaging evidence of other disease processes

What is the suggested treatment focus for patients with gait ataxia, based on the location of lesion who had a CCA problelm

Retrain gait in all functional enviornment, avoid obstacles

What is the main focus for persons with huntingtons' rehab?

Rhythm and cadence control via metronom... SMaller amplitude movements PNF with focus on trunk control Look for End of dose or On:Off medications effects

Describe what works for the rehab management of Huntington's disease

Rhythm and cadence via auditory stimulus (metronome) had positive effects on gait disturbances Smaller amplitude motor control emphasized like Tia Chi Relaxation techniques may be helpful in reducing Choreiform movements PNF techniques and rhythmic stabilization for trunk control Timed therapy after meds to maximize session

Exam Review:

Room 301 at 8 am on Tuesday No SCI questions! Vestibular questions -> Think about Dix-Hallpike and results of this. Know findings and how to diagnose it What are brock-strings? Cupulothesis and Canalisthesis and what treatments you may use. Instead of Epley, it may say canal repositioning. Know different names, also with Libertarian and Semont Know different eye muscles and VOR Know sacule and utricle and what the purpose is and other anatomy. Know BPVV What is brant-daroff and what is hypofunction, what is presentation, when u suspect it what test would you use and treatments for hypofunction Review head-thrust results and head shaking and what it means Know clinical sub-groups of concussions and the clinical sub-group and acute symptoms LOTS OF QUESTIONS What would you expect with recovery, and VOMS RARE diseases: Pathologies of each, know clinical presentations HD, CMD, Brain tumor, PPS, ALS and cerebellar lesions. Know treatment approaches, understand short-terms vs. long-term approaches Know primary and secondary complications of HD, know how many people are diagnosed with _____ Describe presentations of diagnoses, know impairments, prognosis. What is evidence showing of CMD with research. Know requirements to diagnose post-polio. Know ataxia and describe it and outcome measures with ataxia and treatment goals that are most appropriate. Think about different outcome measures for each diagnoses. Don't forget about role of hospice when it comes to rare disease planning

Describe the SARA

Scale for the assessment and rating of ataxia: Activity level 8 items, quantifies performance and not categorized Reliaable and valid for SCA's

Describe type 1 CMT

Type 1 CMT's display peripherally demylinating

The following is characteristics of primary or secondary brain tumors? Metastatic and malignancies come from outside of CNS 25% with systemic cancer develop brain tumor 80% of cerebral tumor- frontal lobe 20% is posterior fossa 33% metastasizes to lungs

Secondary

Describe the clinical presentation and treatment of stage 5 of ALS

Severe LE weakness, Moderate to severe UE weakness, w/c dependent, dependent with ADL, skin breakdown. Treatment: Family training for transfers, positioning to reduce skin breakdown, turning schedule, home modifications for mobility Hospital bed with air mattress and possible home mechanical vent

Describe the clinical presentation and treatment of stage 3 of ALS

Severe weakness in ankles, wrists and hands Increase in respiratory effort Treatment: Continue stage 2 program- monitor fatigue closely , maintain physical independence through activity and AD Deep breathing exercises, chest stretching, postural drainage Potential for W/C prescription now

What is the clinical manifestation of Huntington's?

Severity may be variable at onset of symptoms Depends on extent of atrophy and number of deleterious CAG repeats and rate of progression. Chorieform movements: Hyperkinetic movements in limbs Rapid-jerky-involuntar-writhing and uncontrollable In late stages: present as mixed neurodegenerative movement disorder with elements of hypokinetics due to obliteration of basal ganglia structure

Is there evidence for strength gains for patients with PPS?

Shows retention of strength gains through performance No muscle damage If hypertrophy it is not shown, but motor learning may be occuring within muscle

True or false, there is muslce damage with strength training for someone with PPS? What actually happens with strengthening and PPS

Shows retention of strength gains through performance No muscle damage If hypertrophy it is not shown, but motor learning may be occuring within muscle

What are the secondary rare symptoms of PPS?

Sleep disorder, REspiratory disorder Dysphagia, dysarthria Fascicualations Joint deformities

What does evidence suggests for treatment approach in regards to cerebellar ataxia compensatory training?

Slow down movement Decomposition Visual cue, minimize distraction Widen gait Use of AD if managed safely

What are the typical impairments of Upper motor neuron disease?

Spasticity Hyperreflexia Clonus Pathological reflexes such as babinski

Describe what the treatment approach should be for the following impairment for someone with cerebellar ataxia: Reduced postural control, tone and tremor

Specific strength and balance compensation Enviornmental cues Aids

What type of ALS is most common?

Sporadic ALS > familial ALS 70-80% is limb onset 20-30% is bulbar onset

The following characteristics and treatment describe what stage of ALS? Midly weak, clumsiness, ambulatory and independent with ADL Treatment: Continue normal activity or increase if sedentary Gentle PRF strengthening, no overuse, stretching and psych referral if needed

Stage 1

The following characteristics and treatment describe what stage of ALS? Mod- selective weakness, ambulatory, with slight decrease in ADL independence- climbing stairs and buttoning cloths Treatment: Stretching, cautious strengthening for MMT >3+/5. Orthotic and AD

Stage 2

The following characteristics and treatment describe what stage of ALS? Severe weakness in ankles, wrists and hands Increase in respiratory effort Treatment: Continue stage 2 program- monitor fatigue closely , maintain physical independence through activity and AD Deep breathing exercises, chest stretching, postural drainage Potential for W/C prescription now

Stage 3

The following characteristics and treatment describe what stage of ALS? Hanging arm syndrome, shoulder pain, edmatous hand , W/C dependent, severe LE weakness, can perform ADL with fatigue Treatment: Heat/massage for spasm, edema prevention, AA and PROM of limbs Isometric contractions to tolerance, orthotic UE support- sling/trays and power mobility

Stage 4

The following characteristics and treatment describe what stage of ALS? Severe LE weakness, Moderate to severe UE weakness, w/c dependent, dependent with ADL, skin breakdown. Treatment: Family training for transfers, positioning to reduce skin breakdown, turning schedule, home modifications for mobility Hospital bed with air mattress and possible home mechanical vent

Stage 5

The following characteristics and treatment describe what stage of ALS? Bedridden and completely dependent on ADL Treatment: Continue family education, positioning, turning schedule, use of lift devices to change position and transfer Impairment specific: dysphage, soft diet, adaptive feeding, tube feeding Salivation- surgery and suction Dysarthria-speech amplication and palatal life Respiratory-clera airway, trach, vent Requires many health care members

Stage 6

What is static vs. dynamic types of imaging?

Static: CT and MRI Dynamic: PET, SPECT, MRS and fMRI

How does imaging occur for brain tumor?

Static: CT and MRI Dynamic: PET, SPECT, MRS and fMRI Computer integration: Biopsy- stereotatcic EEG

Describe what the treatment approach should be for the following impairment for someone with cerebellar ataxia: Reduced learning from error

Stepwise prompts, high reps, conscious attention is important

What is the medical management for PPS?

Steroid medication Immunoglobulin Amantadine Drugs usually lack efficacy and effect on fatigue and strenght!!!

What is sterotactic radiosurgery?

Sterotatic radiosurgery: Gamma knife: deeply embedded small tumors, robotic arm to focus beams of radiation to target tumor. Minimal damage to healthy cells Used for smaller tumors

What is the suggested treatment focus for patients with gait ataxia, based on the location of lesion who had a superior cerebellar artery stroke?

Strength/balance, walking aides, cortically control walking, cue

WHat are the best interventions and rehab mangement for HD?

Stretching to avoid contractures Low load and sustained stretching or resting or night splints and serial casting Continual eval for DME or assistive devices Consider TENS for pain

Describe the rehab interventions for Charcot Marie tooth

Stretching to avoid contractures in hands, may need bracing or splints in later stages. Low load and sustained stretching to protect from contractures Resting or night splints, serial casting of ankles Continual evaluations for DME and assistive devices May need protective socks similar to diabetic neuropathy

What kind of ambulation occurs with CMT?

Stumbles, falls and glumsy gait are common with CMT

What does evidence say for exercise with PPS?

SubMAX and max contractions in low reps Aquatic therapy Correlations of improved strength with stair climing, walking and rising from chair

What kind of exercise do you do for someone with PPS?

SubMAX and max contractions in low reps LOW REPS IS KEY

What does evidence say about frequency of aerobic training for patients with PPS?

Suggest 3 or more times per week protocol

For patients with PPS, they have weaker motor evoked potentials. What does this suggest in regards to central fatiguability?

Suggests changes in motor cortex ability to command that motor output = fatiguability

Name the cerebellar stroke based off impairments: Dysmetria of ipsilateral arm. Unsteady walking, dysarthria and nystagmus Common coordination and dysmetria are always IPSILATERAL!!!

Superior cerebellar artery

Describe some swing phase and stance phase presentations of ataxic gait

Swing phase: overly high step: excessive hip and knee flexion Stance phase: forceful, uncontrolled lowering of leg to contact the floor

Describe what the treatment approach should be for the following impairment for someone with cerebellar ataxia: Poor coordination and grading of muscle power

Taking someone into standing to tall kneeling to reduce degrees of freedom External device Postural control Put in quadruped is great for this!!

Describe the clinical pathways of care for a patient with a brain tumor

Team includes patinet in middle --> MD, nurse, RN, SLP, OT, PT

What are common examination findings in regards to Temperature tolerance for patients with PPS?

Temperature tolerance: Abnormally cold extremities- sympathic nerve cell damage and heat loss Prevention of intolerance through proper outerwear.

List some common exam findings with mobility, temperature and sleep with PPS?

Temperature tolerance: Abnormally cold extremities- sympathic nerve cell damage and heat loss Prevention of intolerance through proper outerwear. Sleep: 50% prevelance Periodic limb movement Mobility: Osteoarthritis of wrist and hand Gait analysis --> Overuse and substitution of vstus lateralis, biceps femoris, glutes max and soleus not functionning Decreased activity up to 16 hours sedentary

What is a treatment for generalized weakness for patients with post-polio syndrome?

Therex- strengthening that is non-fatiguing Aerobic exercise Orthotics/AD Lifestle changes and weight loss

What pathology is the Demyelinating or axonal degeneration affects afferent and efferent nerve conduction velocity and motor unit action potential

Tooth marie charcot CMT

True or False: Patients with minimal balance deficits but (+) leg incoordination present with less gait abnormalities

True!

True or False: Brain has no sensory nerves

True!! Therefore you can have brain surgery when the patient is awake

True or False: ALS is variable, but progressive in nature

True: Death usually within 3-5 years with respiratory failure Better survival rate with limb-onset, and increased psychological well-being

Describe the 3 types of Charcot marie tooth disease

Type 1 CMT's display peripherally demylinating Type 2 CMTs: display peripherally axonal degeneration Type X CMT's: more rare and be central and peripheral

Describe type 2 CMT

Type 2 CMTs: display peripherally axonal degeneration

How does muscle fibers change with PPS, and what impairments may arise from this?

Type II fibers change to type I, which may affect gait and explosive muscles like the gastroc/soleus

Descrie type X CMT

Type X CMT's: more rare and be central and peripheral

What is the epidemiology and etiology of Huntington's disease?

Typically between 45-50 y.o 5/100000: Affects males slightly more than females >40 "CAG" repeats on HTT allele of Chromosome #4. Genetic mutation = abnormal folding of protiens in neurological structures of brain and basal ganglia

Overall should you exercise someone with ALS?

Use your clinical decision making: Risks don't necessarily outweigh benefits, so use collaborative effort to try and focus on goals

How do symptoms of ALS usually present with at first?

Usually symptoms are focal and asymmetrical at onset

CMT describe the severity

Variable in severity and age of onset of symptoms ranges from early childhood to early adulthood. Extremely variable in disease presentation

Describe the typical disease course and prognosis of ALS

Variable- average duration 27-43 months Death within 3-5 years, with respiratory failure <35-40 years have better 5 years survival rate Survival time greater with limb onset vs. bulbar Increased survival time with psychological well-being

The following is the typical prognosis for what pathology? Variable- average duration 27-43 months Death within 3-5 years, with respiratory failure <35-40 years have better 5 years survival rate Increased survival time with psychological well-being

Variable- average duration 27-43 months Death within 3-5 years, with respiratory failure <35-40 years have better 5 years survival rate Survival time greater with limb onset vs. bulbar Increased survival time with psychological well-being

What does evidence mainly support of exercise with PPS?

Various methods of nonfatiguing strengthening exercises

What causes CMT?

Vary on genetic allele on a specific chormosome

What are some postierior inferior cerebellar artery stroke impairments?

Vertigo, unsteadiness, walking ataxia, nystagmus

The flocculonodular lobe is important for what main function?

Vestibular

What is part of the rehab framework for a patient with a brain tumor?

Vital component is rehab framework for patients living with brain tumor Functional outcomes are similar to those post stroke and TBI Rehab plan should be flexible, allow for worsening impairments and sensitive to emotional impact Recovery, health promotion and palliative or end of life care all need to be considered.

Who do we consult for patients with brain tumor in regards to return to work, sport or leisure?

Vocational rehab Recreational therapy Neurophyschology

What are common AL with PPS? What are PR?

Walking Climbing stairs and dressing PR with meeting demands of work or home

What is the postoperative care and risks for a patient following brain surgery removal?

Watch for INtracranial hemorrhage and observe in ICU for 24 hours BP monitoring, DVT and PE monitor Use of compression stockings not anticoagulants Watch for hydrocephalus, infection, infarction and hemorrahge

What is the precaution with Friedrich's ataxia?

Watch for aerobic exercises and resistance training, as these patients usually have cardiac comorbidity

What is education for temperature intolerance for patients with PPS?

Wear proper outerwear

What are the main rehab management and care considerations for Charcot Marie tooth disease?

Will often need increased support from caregivers as disease progresses and increasingly impact function Often requires AFO's, GRAFO's and AD for ambulation. High risk for LE sprains SPC and walking sticks preferred initially Females don't ususally want AFO's for cosmetic reason

What is the best time of day for therapy for a patient with Charcot tooth marie disease?

Will respond to early in the day treatments

What are the most common MS impairments with PPS?

With both new and prior muscles affected by polio.. Decreased muscle strength, muscle endurance, atrophy Fatigue, pain, NM weakness Cold intolerance

What is the best way to see if your benefits outweight the risks when exercising someone with ALS?

With reports of morning fatigue or signifant weakness ***Be attentive to their reports after program after instituting program

With Charcot marie tooth disease, there is intrinsic foot musculature atrophy leading to poor mechanics during loading and WB. There is also dry or skiny and flaky skin on distal LE. Does it every progress to hand?

Yes can affect hands and wrists in later stages: May see cool hands and deformities similar to late stage RA

Are there hypokinetic features with HD?

Yes in late stages you may see some, but mainly hyperkinetic

Patient presents to your clinic with referral for vertigo. You take a thorough history to reveal subjectively a patient with history of cancer, recent issues with memory, vomiting, HA upon waking in the morning, and imbalance with mobility. Objective findings are poor abilities with the Rhomberg and Sharpened Rhomberg, decreased smooth pursuit with ocular testing, veering of his path during dynamic gait, negative dix hallpike, and inability to reproduce specific symptoms of dizziness with positional testing, + for clonus in Left ankle, 3 beats What might you suspect? What education may you provide? What is your next action as therapist?

You suspect a possible tumor of posterior fossa Educate about findings and that sometimes with inconclusive examination findings, a referral back to the physician may be warranted. Call the physician and report findings. Suggest patient may benefit from further medical evaluation

The following describes what pathology: Brief bouts of ataxia usually due to excitement, stress or exercise Lasts minutes to hours Responds to medication

episodic ataxia

What is a hallmark sign of CMT besides the coke-bottle shape?

hammertoe deformity in great toe with pes cavus or high arch

Describe Charcot-Marie-Tooth disease

rare neuromuscular disorder Most commonly inherited neuropathy: One of most commonly inherited human disease and 10x more common than ALS and HD combined! 125,000-150,000 in US Often undiagnosed or misdagnosed due to its overlapping symptomology

What is part of voluntary movement coordination examination for somone with cerebellar ataxia?

voluntary movement coordination is important to test: finger taping, supination/pronation, handwriting, toe tapping Heel to shin Finger to nose