Cerebellar ataxia with neuropathy and bilateral areflexia syndrome (CANVAS) is a late onset neurodegenerative disorder with a slowly progressive ataxia. It's genetic causative etiology with an autosomal recessive inheritance has a recent discovery. It is clinically characterized by impaired visually enhanced vestibulo-ocular reflex, although patients commonly present with imbalance as a main concern, associated with sensory complaints. It has been demonstrated that sensory impairment in CANVAS patients is due to degeneration of dorsal root with abnormal sensory nerve conduction. Previously defined diagnostic criteria included cerebellar atrophy on brain MRI, neuronopathy on electrophysiological studies and negative genetic testing for other inherited ataxia syndromes like Friedriech ataxia and spinal cerebellar ataxia (SCA). Peripheral nerve ultrasound is a noninvasive technique, able to identify abnormal peripheral nerves with underlying injuries and specific sonographic characteristics. Pelosi et al established that patients with CANVAS have a smaller nerve cross sectional area (CSA) compared to healthy individuals and/ or axonal neuropathies. The main objective of this study was to obtaine a detailed description of peripheral nerves in consecutive patients with CANVAS syndrome followed in theneurology department of the Universitary Hospital of Nimes (France), using conventional electrophysiology and peripheral nerve ultrasound.
Study Type
OBSERVATIONAL
Enrollment
35
Pure observational study
CHU de Nîmes
Nîmes, France
median ultrasound
Comparison of axial cross-sectional area to Bilateral exploration of median nerve ultrasound
Time frame: Baseline
ulnar nerve at wrist ultrasound
Comparison of axial cross-sectional area to Bilateral exploration of ulnar nerve at wrist, ultrasound
Time frame: Baseline
mid-arm ultrasound
Comparison of axial cross-sectional area to Bilateral exploration of ulnar nerve, mid-arm, ultrasound
Time frame: Baseline
elbow ultrasound
Comparison of axial cross-sectional area to Bilateral exploration of ulnar nerve, elbow, ultrasound
Time frame: baseline
ultrasound
Comparison of axial cross-sectional area to Bilateral exploration of ulnar nerve, mid-forearm, ultrasound
Time frame: Day of the evaluation
radial nerve ultrasound
Comparison of axial cross-sectional area to Bilateral exploration of radial nerve ultrasound
Time frame: Baseline
superficial radial branch nerve ultrasound
Comparison of axial cross-sectional area to Bilateral exploration of superficial radial branch nerve ultrasound
Time frame: Baseline
C5, C6 ultrasound
Comparison of axial cross-sectional area to Bilateral exploration of C5, C6 roots ultrasound
Time frame: Baseline
sural nerve ultrasound
Comparison of axial cross-sectional area to Bilateral exploration of sural nerve ultrasound
Time frame: Baseline
superficial fibular nerve ultrasound
Comparison of axial cross-sectional area to Bilateral exploration of superficial fibular nerve ultrasound
Time frame: Baseline
popliteus ultrasound
Comparison of axial cross-sectional area to Bilateral exploration of popliteus tibial nerve at ankle ultrasound
Time frame: Baseline
tibial ultrasound
Comparison of axial cross-sectional area to Bilateral exploration of tibial nerveat ankle ultrasound
Time frame: Baseline
fibular ultrasound
Comparison of axial cross-sectional area to Bilateral exploration of fibular nerve at the neck ultrasound
Time frame: Baseline
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