Evaluation of the Peripheral Nerve Ultrasound as a Diagnostic Tool in CANVAS Neuropathies

Neurodegenerative Diseases Clinical Trial

— CANVAS  

Official title:

Evaluation of the Peripheral Nerve Ultrasound as a Diagnostic Tool in CANVAS (Cerebellar Ataxia With Neuropathy and Vestibular Areflexia Syndrome) Neuronopathies

Clinical Trial Details — Status: Completed

Administrative data

• NCT number: NCT05095870
• Other study ID #: LOCAL/2021/II-02
• Secondary ID: IRB 21.09.30
• Status: Completed
• Start date: October 25, 2021
• Est. completion date: March 30, 2022

Study information

• Verified date: May 2022
• Source: Centre Hospitalier Universitaire de Nimes
• Contact: n/a
• Is FDA regulated: No
• Study type: Observational

Clinical Trial Summary

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.

Recruitment information / eligibility

• Status: Completed
• Enrollment: 35
• Est. completion date: March 30, 2022
• Est. primary completion date: March 30, 2022
• Accepts healthy volunteers: No
• Gender: All
• Age group: 18 Years and older

Eligibility

CANVAS Patients :

Inclusion Criteria:

• Patients followed at the University Hospital of Nîmes between 2018-2021

• Age > 18 years

• Diagnosis of CANVAS with genetic evidence (RFC1+)

• Diagnosis of neuronopathy

Exclusion Criteria:

• Age <18 years

• Chronic polyradiculoneuritis or other demyelinating neuropathy

Control group, inclusion criteria :

Patient with axonal sensory-motor or pure sensory neuropathy confirmed by electro neuromyography, and concordant with the clinic.

Related Conditions & MeSH terms

• Ataxia
• Cerebellar Ataxia
• Neurodegenerative Diseases
• Neuropathy

Intervention

Other:
• No intervention
Pure observational study

Locations

Country	Name	City	State
France	CHU de Nîmes	Nîmes

Sponsors (1)

Lead Sponsor	Collaborator
Centre Hospitalier Universitaire de Nimes

Country where clinical trial is conducted

France, 

Outcome

Type	Measure	Description	Time frame	Safety issue
Primary	median ultrasound	Comparison of axial cross-sectional area to Bilateral exploration of median nerve ultrasound	Baseline
Primary	ulnar nerve at wrist ultrasound	Comparison of axial cross-sectional area to Bilateral exploration of ulnar nerve at wrist, ultrasound	Baseline
Primary	mid-arm ultrasound	Comparison of axial cross-sectional area to Bilateral exploration of ulnar nerve, mid-arm, ultrasound	Baseline
Primary	elbow ultrasound	Comparison of axial cross-sectional area to Bilateral exploration of ulnar nerve, elbow, ultrasound	baseline
Primary	ultrasound	Comparison of axial cross-sectional area to Bilateral exploration of ulnar nerve, mid-forearm, ultrasound	Day of the evaluation
Primary	radial nerve ultrasound	Comparison of axial cross-sectional area to Bilateral exploration of radial nerve ultrasound	Baseline
Primary	superficial radial branch nerve ultrasound	Comparison of axial cross-sectional area to Bilateral exploration of superficial radial branch nerve ultrasound	Baseline
Primary	C5, C6 ultrasound	Comparison of axial cross-sectional area to Bilateral exploration of C5, C6 roots ultrasound	Baseline
Primary	sural nerve ultrasound	Comparison of axial cross-sectional area to Bilateral exploration of sural nerve ultrasound	Baseline
Primary	superficial fibular nerve ultrasound	Comparison of axial cross-sectional area to Bilateral exploration of superficial fibular nerve ultrasound	Baseline
Primary	popliteus ultrasound	Comparison of axial cross-sectional area to Bilateral exploration of popliteus tibial nerve at ankle ultrasound	Baseline
Primary	tibial ultrasound	Comparison of axial cross-sectional area to Bilateral exploration of tibial nerveat ankle ultrasound	Baseline
Primary	fibular ultrasound	Comparison of axial cross-sectional area to Bilateral exploration of fibular nerve at the neck ultrasound	Baseline