CANVAS Cerebellar ataxia, neuropathy, vestibular areflexia syndrome

1. C A N V A S
Cerebellar ataxia,
neuropathia,
vestibular areflexia syndrome
C A N V A S
Cerebellar ataxia,
neuropathia,
vestibular areflexia syndrome
Dušan Pavlović
Hearing and Balance Center

2. Vestibular function testing
DusanPavlovic.com March 30th
2015
positive VVOR test very characteristic
deficit in 3 compensatory eye movements:

SP /smooth pursuit/

OKR /optokinetic reflex/

VOR /vestibulo ocular refles/

3. Vestibular function testing
DusanPavlovic.com March 30th
2015

Peripheral neuropathy integral part of this
syndrome

MR finding (vermis atrophy)

4. Clinical presentation
DusanPavlovic.com

60yr mean age, equal sex distribution

11yr after onset

instability

dyasthesia

oscillopsia

dizziness

intrinsic falls
March 30th
2015

5. Cerebellar dysfunction
DusanPavlovic.com

gaze evoked Ny, downbeat Ny

saccadic smooth pursuit

gait ataxia

dysarthria

appendicular ataxia
March 30th
2015

6. Vestibular function testing
DusanPavlovic.com

Severely reduced response at caloric
testing and/or perverted Ny

Absent VOR with large overt saccades

Decreased DVA

VVOR reduced
March 30th
2015

7. Non-length dependent sensory
deficit
DusanPavlovic.com

absent ankle jerks

impaired vibration sensation

deficit of pin-prick sensation

sural nerve biopsy: severe axonal loss

loss of upper and lower limb SNAP
March 30th
2015

8. Autonomic dysfunction
DusanPavlovic.com

orthostatic hypotension

hypohidrosis

persistent cough

vagal neuronopathy ?
March 30th
2015

9. MRI findings,
Cerebellar atrophy:
DusanPavlovic.com

antherior and dorsal vermis

lobules VI, VIIa and VIIb

hemispheric crus I

no atrophy in the early disease process
March 30th
2015

10. Hystopathologic finding
DusanPavlovic.com

vestibular nerve, Scarpa's ganglion

facial nerve, geniculate ganglion

trigeminal nerve, trigeminal ganglion

atrophy dorsal root ganglia

Reduction of Purkinje cells

cochlear neuron and spiral gangion NOT
affected
March 30th
2015

11. Diferential diagnosis
DusanPavlovic.com

MSA multiple system atrophy

Creutzfeldt-Jakob disease

Machado-Joseph / SCA3

Wernicke encephalopathy

Late onset Friedreich ataxia
March 30th
2015

12. Conclusions
DusanPavlovic.com March 30th
2015
Etiology unknown
Late-onset recessive disorder
Easy to diagnose, positive VVOR
In patient with imbalance
Investigation for all three manifestations of CANVAS
In CABV patients – perform NCS