Ataxia
If a Pt has focal ataxia for hours to days what would be a suspected causes?
1. *Cerebellar Infarction or Hemorrhage* 2. SAH 3. Cerebellar Abscess --> + mass lesion on MRI/CT
Pt has focal ataxia for months to years what are some causes?
1. *Congenital Lesion --> Chiari or Dandy-Walker* malformations
If a pt has experienced symmetrical ataxia for months to years what would you suspect?
1. *Inherited ataxia* 2. MSA-cerebellar 3. Paraneoplastic 4. Autoimmune --> Anti-GAD 5. Hypothyroidism
What are some causes of a pt with symmetrical ataxia for anywhere from several hours to days?
1. *Intoxication* --> alcohol, Lithium, or barbituates. Need +history and tox screen 2. *Acute Viral Cerebellitis* --> CSF supportive of acute viral infection 3. Postinfectious Syndrome --> usually viral
Pt Presents with motor/sensory loss and cranial nerve palsies that are asymmetric and might have impaired level of consciousness. What might you suspect?
1. Acute Stroke or Bleed 2. Get a CT --> look for bleed or infarct
Once you diagnose a pt with Friedreich Ataxia, what do you need to watch out for? What are other common characteristics?
1. Ambulation lost by 9-15 years after onset 2. Foot Deformity, Diabetes , and cardiac hypertrophy
Progressive Ataxias can be?
1. Autosomal Dominant and Recessive 2. Mitocondrial 3. X-linked
What are different types of ataxias?
1. Cerebellar 2. Sensory 3. Misc. Ataxia --> Vestibular ataxia, Thalamic ataxia, Frontal Dysequilibrium
Features of Episodic Ataxias?
1. Channelopathies, enzyme defects (autosomal, X-linked)
Non-progressive ataxia characteristics and example?
1. Congenital, Autosomal, X-linked --> cerebellar malformations 2. Dandy-Walker
Sudden episodes of ataxia provoked by movement --> being startled or emotion (Seconds to Minutes)?
1. EA 1 2. Problem with Voltage Gated Potassium Gene 3. Treat with Azetazolamide
Sudden Episodic bouts of ataxia with nystagmus, vertigo, and vertigo provoked by stress, exercise, fatigue, coffee/alcohol (hours to days)?
1. EA 2 2. Calcium Channel Gene
In cerebellar ataxia what is the problem?
1. Faulty proprioceptive integration
If you a have patient under the age of 30 who presents with progressive limb and gait ataxia what might you suspect? What is the causes?
1. Friedreich Ataxia --> Progressive disorder that is Autosomal *Recessive* 2. GAA triplet repeat expansion of X25gene --> reduced *Frataxin* Protein
What are some causes of acute ataxic gate in an alcoholic pt? How would you treat this?
1. Intoxication 2. Thiamine Deficiency --> Wernicke's Encephalopathy = confusion, eye movement abnormality
What are some causes of a pt with symmetrical ataxia for days to weeks?
1. Intoxication --> mercury, solvents, gasoline, glue 2. Cytotoxic chemo drugs --> cytarabine 3. *Alcoholic-Nutritional* --> Vit B1 and B12 deficiency 4. EBV, CMV, Lyme
If you have a pediatric pt who you might suspect has an Ataxic disorder what are you looking for? What is the workup? Big thing that is on your differential?
1. May appear wobbly when sitting 2. Commonly due to *Intoxication* --> a) Ask about Meds at home. b) think infection (*viral*) or post infectious inflammation 3. Admit --> Utox --> Drug Screen --> CT Head --> LP if *ALL NEGATIVE* 4. Medulloblastoma
1. Your pt has trancul ataxia, where might lesion be?
1. Midline Cerebellar lesion --> truncal ataxia
What would you see in a pt who as is an alcoholic and has developed chronic ataxic gait>
1. Midline cerebellar atrophy 2. neuropathy in feet 3. Dorsal column damage from B12 deficiency
Pt presents with focal ataxia for days to weeks what are some causes?
1. Neoplastic -> cerebellar *glioma* or metastatic tumor 2. Demyelinating --> *Multiple Sclerosis*
What are some of signs associated with ataxia in Geriatric Population?
1. Normal Agre related changes (>65) --> decreased in gait speed, stride length, post. shift in center of gravity, & unable to perform tandem gait 2. *Multifactorial Gait Disorder* --> normal age related changes + neuropathy, mechanical arthritic changes, or sedative meds can result in falls
A geriatric pt with acute worsening of age related changes presents to your clinic. What should your course of action be? What are some possible therapies for geriatric pt's with ataxia?
1. Order CT 2. Look for subdural 3. *Physical Therapy* --> *gait retraining* or ambulatory aids 4. Reassess Medication List
Pt presents with ophthalmoplegia, spasticity, parkinsonism, facial myokymia, neuropathy, and cognitive decline?
1. SCA 3 = Machado Joseph Disease 2. Death occurs 15-20 years usually by aspiration PNA
What is the most common SCA? What is the causes?
1. SCA 3 = Machado Joseph Disease --> Autosomal dominant 2. CAG repeat --> ATXN3 gene = *Ataxin 3* protein
A benign, late onset SCA? SCA that presents with Vision Loss?
1. SCA 6 2. SCA 7
Pt comes in complaining of difficulty walking and problems with other voluntary movements. She notes that she has noticed that it worsens when her eyes are closed. What do you suspect? What is cause? What signs will you see?
1. Sensory Ataxia 2. Caused by Dorsal Column or Large Fiber Neuropathy 3. No association features of cerebellar ataxia 3. Will see Pseudo-ataxia and Romberg's
What would you expect to see in a pt with Dandy Walker in early infancy?
1. Slower motor development 2. progressive enlargement of skull
Gradual onset of cerebellar ataxia with associated features involving other systems?
1. Spinocerebellar Ataxias (SCA) --> Progressive disorder that is Autosomal *Dominant* 2. CAG Triplet Repeat 3. Usually *3rd-5th decade* of onset 4. SCA types 1-29 + DRPLA
How would you treat pt with thiamine deficiency leading to acute ataxic gate?
1. Start Thiamine 200 mg IV start before or with IV glucose
What are some features of Cerebellar ataxia?
Ataxia of Gait Dysdiadochokinesia Dysmetria Loss of check and *rebound* Intention tremor Eye Movements --> Nystagmus, Dysmetric, Saccades Scanning Dysarthria Hypotonia and slow Pendular Deep Tendon Relfex
If a pt presents with a mixture of features such as the following: rubral tremor, thalamic ataxia, frontal gait ataxia. Where might the lesion be?
Cerebellar inflow and outflow tracts
Triad of features: 1. Complete or partial *Agenesis of Vermis* 2. Cystic *dilation of 4th ventricle* 3. *Enlarged posterior fossa* w/ upward displacement of lateral sinus, tantrum and torcular herophili
Dandy Walker
Breakup and irregularity of movement when the limb is trying to carry out rapid movements
Dysdiadochokinesia
What is ataxia?
Lack of coordination resulting in breakdown of normal execution of voluntary movements
Pt has right sided limb ataxia. Where in the cerebellum might you suspect the lesion be?
Lateral Cerebellar Lesion --> Ipsilateral limb ataxia
Pt has right sided ataxia with sensory loss, where might the damage be?
Left Thalamic Infarct
Pt presents with ipsilateral dysdiadochokinesia, dysmetria, and intention tremor. Where would you suspect lesion?
Neocerebellum --> lateral hemisphere
Spoken words are broken up into separate syllables, often separated by a noticeable pause, and spoken with varying force
Scanning Dysarthria
Pt presents with gait ataxia or truncal ataxia. Where would you suspect lesion?
Spinocerebellum --> Vermis and paravermian region
Pt presents with ataxic gait, truncal ataxia, with vertigo, nausea, nystagmus & other eye movement abnormalities. Where would you suspect lesion?
Vestibulocerebellum --> Flocculonodular Lobe
What would you expect to see in a pt with Dandy walker in an older child? What treatment would you might need?
1. Raised ICP 2. Signs of Cerebellar Dysfunction 3. VP shunt for *hydrocephalus*
Unsteadiness with a wide base, body swaying, and inability to walk on tandem (heel to toe)
Gait Ataxia