62yr woman with instability as main complain, lasting for 7 years, which deteriorated in last two years. She felt down for two times completely conscious. Six months ago she noticed double vision when looking to the right. She has slight headaches in occipital part and she is very forgetful. Paresthesias in extremities and clumsiness with hands. Her MRI is still normal.
gaze evoked Ny, from time to time down beat Ny
her speech is slight dysarthric
Romberg is positive: she falls to the right
cerebellar signs positive: dysmetria on the left
patellar reflexes exaggerated, Achilles reflexes absent
diminished sensitivity in the hands and foots
caloric test absent response at both sides
VHIT: absent vestibulo-ocular reflex at all canals
VNG: no smooth pursuit, saccades with prolonged latency to the right, slight overshoot dysmetria to the right and undershoot dysmetria to the left
For CANVAS patients very caracteristic sign is positive VVOR test: this test is positive due to lack of smooth pursuit and lack of vestibulo-ocular reflex and lack of optokinetic reflex.
At this video there’s obvious Horner sign on the right side.
Caracteristic hystory of slowly progressive instability with clinical signs of cerebellar dysfunction and bilateral vestibulopathy and peripheral neuropathy. More about CANVAS at: link