Continuum Ataxia Jeopardy

Anatomy

100

This cerebellar peduncle transmits fibers of the pontocerebellar tract

Middle cerebellar peduncle

200

These are the layers of the cerebellar cortex in order from outermost to innermost

Molecular layer (outer), Purkinje layer (middle), Granule layer (inner)

300

This afferent tract arises from the dentate nucleus, passes through the red nucleus, and ends in the contralateral ventrolateral nucleus of the thalamus

Dentatothalamic tract

400

These are the deep nuclei of the cerebellum

Dentate nuclei, Interposed nuclei (globose and emboliform), Fastigial nuclei

500

These are the primary functions of the floculonodular lobe of the cerebellum

Maintaining balance, regulating eye movements, integrating vestibular information

Name That Finding

100

This is the inability to perform rapid alternating movements

Dysdiadochokinesia

200

This is the inability to modulate distance of movements

Dysmetria

300

This results from damage to proprioceptive fibers and can cause high steppage gait and imbalance with positive Romberg sign

Sensory ataxia

400

These are a type of saccadic intrusion characterized by saccadic movement away from a point of fixation followed by a corrective saccade back to the point of fixation

Square wave jerks

500

This is a low frequency (4.5 hz) resting, postural, and action tremor caused by midbrain lesions

Rubral tremor or "Holmes tremor"

Genetic Ataxias

100

Pattern of inheritance for most spinocerebellar ataxias and episodic ataxias

Autosomal dominant

200

Most common inherited ataxia (and the responsible gene)

Friedreich's ataxia, FXN gene on chromosome 9

300

This gene is associated with SCA6 as well as episodic ataxia type 2

CACNA1A (also associated with familial hemiplegic migraine and epilepsy)

400

Fragile-X Associated Tremor/Ataxia Syndrome (FXTAS) is caused by what number range of repeats in the FMR1 gene

55-200 CGG repeats

500

This autosomal recessive ataxia is caused by an AAGGG repeat expansion in the RFC1 gene

Cerebellar ataxia, neuropathy, vestibular areflexia syndrome (CANVAS)

Acquired Ataxias

100

This is the most common cause of toxic cerebellar degeneration

Chronic alcohol use

200

This seizure medication is associated with cerebellar degeneration with long-term use

Phenytoin

300

These environmental toxins are most commonly associated with cerebellar degeneration

Heavy metals (mercury, lead, manganese) and solvents (toluene, benzene)

400

These vitamin deficiencies are most commonly associated with cerebellar ataxia

Vitamin B12, vitamin E, vitamin B1

500

These paraneoplastic antibodies are commonly associated with paraneoplastic cerebellar degeneration (name 3)

Anti-Yo, anti-Hu, anti-Ri, anti-Tr, anti-Ma2, anti-CV2

Ataxia Treatment

100

This is the treatment for paraneoplastic cerebellar degeneration

Anti-neoplastic treatment and immunomodulatory therapy (steroids, IVIG, PLEX)

200

This medication is used in patients with episodic ataxia type 2 (also used in patients with MS)

4-aminopyridine

300

This medication is used in the treatment of both episodic ataxia type 1 and episodic ataxia type 2

Acetazolamide

400

This is the treatment for cerebellar ataxia associated with the presence of anti-tTg (tissue transglutaminase), anti-EMA (endomysial), and anti-DGP (deamidated gliadin peptide) antibodies

Gluten free diet

500

This is an FDA-approved drug used for the treatment of Friedreich's ataxia (name and mechanism)

Omaveloxelone, Nrf2 activator, protects cells from oxidative damage