Cerebellar Ataxia

WHAT ARE THE AUTOSOMAL RECESSIVE ATAXIAS? 5

-Friedreich's ataxia -Ataxia telangiectasia -Ataxia with vitamin E deficiency -Ataxia with oculomotor apraxia (inability to perform particular purposive actions, as a result of brain damage.) -Spastic ataxia

WHAT ARE THE AUTOSOMAL DOMINANT ATAXIAs? 5

-Spinocerebellar ataxia -Dentatorubral-pallidoluysian atrophy -Episodic ataxia -Gerstmann-Sträussler syndrome -Dentatorubral-pallidoluysian atrophy

what anatomic division of the cerebellum? (Vermis, paleocerebellum), which underlies titubation, truncal ataxia, orthostatic tremor (high frequency), and gait imbalance

1. midline

The cerebellum has 3 anatomic divisions that account for the 3 types of dysfunction commonly seen:

1. midline 2. hemispheres 3. posterior

WHAT TYPE OF ATAXIA? injury or stroke to the brain (e.g., cerebellar stroke)

AQUIRED

WHAT ARE THE 3 TYPES OF WAYS TO GET ATAXIA ATAXIA?

AQUIRED HEREDITARY IDOPATHIC

____ tracts to get INTO the cerebellum

Afferent

WHAT TYPE OF ATAXIA? is a rare, neurodegenerative, autosomal recessive disease causing severe disability. Ataxia refers to poor coordination and telangiectasia to small dilated blood vessels, both of which are hallmarks of the disease Oculomotor apraxia, also known as Cogan oculomotor apraxia or saccadic initiation failure is the absence or defect of controlled, voluntary, and purposeful eye movement.

Ataxia telangiectasia

what type of cerebellar disorder? high velocity and low amplitude (fine tune)

Cortical cerebellar (anterior lobe) atrophy

what type of autosomal dominant ataxia? is an autosomal dominant spinocerebellar degeneration caused by an expansion of a CAG repeat encoding a polyglutamine tract in the atrophin-1 protein. Gerstmann-Sträussler-Scheinker syndrome is a very rare, usually familial, fatal neurodegenerative disease that affects patients from 20 to 60 years in age.

Dentatorubral-pallidoluysian atrophy

____-is a disorder of the motor component of speech articulation. Scanning speech- described as one-word-at-a-time quality, speech is slow, slurred, hesitant, prolonged syllables, inappropriate pauses.

Dysarthria-

____-is an impaired ability to perform rapid alternating movements. Example- like pronation to supination. The movements will be irregular, loss of range and rhythm especially if speed is increased

Dysdiadochokinesia

____-is an inability to judge the distance or range of movement.

Dysmetria (hypermetria/hypometria)

____-movement decomposition. Describes a movement performed in a sequence of component parts rather than a single, smooth activity. For example: when asked to touch the index finger to the nose, the patient might first flex the elbow, and then adjust the position of the wrist and fingers , further flex the elbow, and finally flex the shoulder

Dyssynergia

WHAT TYPE OF ATAXIA? is an autosomal recessive inherited disease that causes progressive damage to the nervous system. It manifests in initial symptoms of poor coordination such as gait disturbance; it can also lead to scoliosis, heart disease and diabetes, but does not affect cognitive function.

Friedreich's ataxia

____-involves ambulatory patterns that typically demonstrate a broad BOS. Upright stance stability is poor and the arms might be held away from the body to improve balance. Stepping patterns are irregular in direction and distance. Initiation of forward progression of a LE may start slowly, and then the extremity may unexpectedly be flung rapidly forward. Gait is unsteady, irregular, staggered

Gait disorder (ataxic)-

WHAT TYPE OF ATAXIA? inherited in the families (e.g., Friedreich's ataxia, spinocerebellar ataxia, etc.)

HEREDITARY

____-is decrease in muscle tone. It is believed to related to a disruption of afferent input from stretch receptors/ or lack of cerebellum input in fusimotor system.

Hypotonia

WHAT TYPE OF ATAXIA? unknown causes leading to damage to the cerebellum

IDOPATHIC

The primary function of the cerebellum is regulation of ____, ____, AND ___ ___.

MOVEMENT POSTURE MUSCLE TONE

____-is a rhythmic, quick, oscillatory, back-and-back forth movement of the eyes. Linked to the cerebellum's influence on synergy and tone of the extraocular muscles.

Nystagmus

what type of ataxia? •Characterized by the progressive degeneration and subsequent loss of neurons •Accompanied by reactive gliosis, degeneration of fibers from the deteriorating neurons, and clinical symptoms reflecting the locations of the lost neurons

SPINOCEREBELLAR ATAXIA (SCA)

what type of ataxia? •Results from either genetically transmitted diseases with dominant inheritance or unknown causes with sporadic occurrence •Increasing unsteadiness of gait, incoordination of limb movements with impairment of skilled movements such as handwriting, and a distinctive dysarthria •Identification of the genes are responsible for the dominantly inherited cerebellar degenerations: SCA1, SCA2,.......... SCA30

SPINOCEREBELLAR ATAXIA (SCA)

what anatomic division of the cerebellum? (flocculonodular lobe, archicerebellum, which influences posture and gait as well as causing eye movement disorders (e.g. nystagmus, VOR disruption)

The posterior

____-refers to rhythmic oscillations of the head (side-to-side or forward and back movements, or rotatory components)

Titubation-

________-is an involuntary oscillatory movement resulting from alternate contractions of opposing muscle groups. Intention/kinetic tremor- occurs during voluntary motion of a limb and tends to increase as the limb nears it goal or increases speed Postural static tremor- back and fourth oscillatory movements of body parts while the patient maintains a standing posture.

Tremor (intention/kinetic)

what type of cerebellar disorder? low frequency and high amplitude (usually oculomotor)

Vestibulocerebellar lesion

WHAT TYPE OF ATAXIA? -Friedreich's ataxia -Ataxia telangiectasia -Ataxia with vitamin E deficiency -Ataxia with oculomotor apraxia (inability to perform particular purposive actions, as a result of brain damage.) -Spastic ataxia

WHAT ARE THE AUTOSOMAL RECESSIVE ATAXIAS?

_____-is generalized muscle weakness associated with cerebellar lesions

asthenia

____-is the loss of ability to associate muscles together for complex movements

asynergia

____-•Located just above the brainstem and toward the back of the brain •Involved in the coordination of voluntary motor movement, balance, equilibrium, and muscle tone.

cerebellum

____tracts to get out of the cerebellum

eFFERENT

what anatomic division of the cerebellum? (neocerebellum-right controlling the right side of the body and left controlling the left side) which contributes to limb ataxia (e.g. dysdiadochokinesia, dysmetria, and kinetic tremor), dysarthria and hypotonia

hemispheres

what type of cerebellar disorder? (neocerebellum)

hemispheres

what type of cerebellar disorder? -Damage to only one cerebellar hemisphere, dysfunction on the side of the body ipsilateral to the lesion -Limb ataxia (dysdiadochokinesia, dysmetria, and kinetic tremor), dysarthria, and hypotonia

hemispheres

____-overestimation

hypermetria

____-underestimation

hypometria

what type of cerebellar disorder? (vermis, paleocerebellum)

midline

what type of cerebellar disorder? -Titubation, truncal ataxia, orthostatic tremor, and gait imbalance

midline

what type of cerebellar disorder? (flocculonodular lobe, archicerebellum)

posterior

what type of cerebellar disorder? -Influences posture and gait -Eye movement disorders (nystagmus, vestibulo-ocular reflex disruption)

posterior

what type of cerebellar disorder? -Increased postural sway (high velocity and low amplitude) in the anterior-posterior dimension -Postural tremor -Increased intersegmental movements of the head, trunk, and legs.

•Cortical cerebellar (anterior lobe) atrophy

what type of cerebellar disorder? -Only slight postural instability, but indistinguishable from healthy subjects Cortical cerebellar (anterior lobe) atrophy- high velocity and low amplitude Vestibulocerebellar lesion = low frequency and high amplitude

•Neocerebellar (lateral) lesion

what type of cerebellar disorder? -Increased postural sway (low frequency and high amplitude) without a preferred direction -No increased intersegmental movements

•Vestibulocerebellar lesion