Neuropathy and Ataxia Flashcards
What is the function of the semicircular canals?
Perception of rotational and angular movements of the head.
What are the otolith organs (utricle and saccule) concerned with?
Linear acceleration of the head.
What neuropathies commonly show vestibular involvement?
Demyelinating neuropathies like Guillain-Barre Syndrome and CIDP.
Also seen in a majority of those with CMT.
What diseases have reported auditory neuropathy with peripheral neuropathy?
CMT, CIDP, Diabetes mellitus type 1, Friedreich ataxia, Refsum’s.
What are cerebellar ataxia syndromes associated with PN?
SCA, Fragile X, Ataxia w/ Vit E deficiency, CMT.
What cerebellar ataxia syndromes are associated with sensory neuronopathy?
Celiac, Sarcoidosis, Sjogren’s, Crohn’s, GAD ab syndrome, Paraneoplastic, Amiodarone.
What cerebellar + vestibular syndromes are associated with polyneuropathy?
Friedrich’s = DRG, vestibular, cochlear, cerebellar; CANVAS (RFC1) = DRG, vestibular, cerebellar;
SCA 3.
What does an abnormal VVOR indicate?
Combined bilateral vestibular hypofunction AND cerebellar oculomotor dysfunction.
How is an abnormal VVOR demonstrated?
By turning a patient’s head slowly from side to side while the patient’s gaze is directed at a fixed target, while observing broken-up eye movements (as opposed to smooth).
What are the 2 key MRI findings in FXTAS?
White matter lesions in middle cerebellar peduncles and splenium of corpus callosum (suggestive but not diagnostic of Fragile X).
What are the clinical MRI brain findings in CANVAS?
Anterior and dorsal vermis atrophy.
What symptom can precede other symptoms in CANVAS by years to decades?
Chronic cough.
