Nerve ultrasound as a diagnostic tool for sensory neuronopathy in spinocerebellar ataxia syndrome. Issue 4 (April 2019)

• Record Type: Journal Article
• Authors: Leadbetter, Ruth; Weatherall, Mark; Pelosi, Luciana
• Is Part Of: Clinical neurophysiology. Volume 130:Issue 4(2019:Apr.)
• Page Start: 568
• Page End: 572
• Publication Date: 2019-04
• Subjects: Cerebellar ataxia neuropathy vestibular areflexia syndrome -- Nerve ultrasound -- Sensory neuronopathy -- Spinocerebellar ataxia type 2; AUC area under the curve -- AUC ROC area under the curve of the receiver operating characteristic curve -- CANVAS cerebellar ataxia neuropathy vestibular areflexia syndrome -- CSA cross-sectional area -- ROC receiver operating characteristic -- SCA2 spinocerebellar ataxia type 2; Neurophysiology -- Periodicals; Electroencephalography -- Periodicals; Electromyography -- Periodicals; Neurology -- Periodicals; 612.8
• DOI: 10.1016/j.clinph.2018.12.010
• Languages: English
• ISSNs: 1388-2457

Abstract

Highlights: Upper limb ultrasound is highly accurate in detecting sensory neuronopathy in cerebellar ataxia neuropathy vestibular areflexia syndrome (CANVAS). Diagnostic criteria for CANVAS also diagnosed sensory neuronopathy in spinocerebellar ataxia type 2. Upper limb ultrasound may have a role in diagnosing sensory neuronopathy in spinocerebellar ataxias. Abstract: Objective: The objective was to assess if nerve ultrasound has a role in diagnosing sensory neuronopathy in spinocerebellar ataxia syndrome (SCA) by examining if proposed diagnostic cut-off criteria of ultrasound in sensory neuronopathy caused by cerebellar ataxia neuropathy vestibular areflexia syndrome (CANVAS) were also discriminatory for SCA-related sensory neuronopathy. Methods: Optimal diagnostic cut-off criteria for nerve size measured by diagnostic ultrasound were developed in 14 patients with CANVAS and 42 healthy controls using six peripheral nerve sites; and logistic regression and receiver operating characteristic (ROC) curves. These proposed cut-off values were tested in seven patients with spinocerebellar ataxia type 2 (SCA2) patients with sensory neuronopathy. Results: Ultrasound of upper limb nerves was highly accurate in differentiating between CANVAS and healthy controls with areas under the ROC curves between 0.97 and 0.99. Optimal cut-off measurements from the CANVAS patients also accurately diagnosed sensory neuronopathy in all patients with SCA2. Conclusions: Upper limb ultrasound is a sensitive tool for detecting sensory neuronopathy in established cases of CANVAS and SCA2. Significance: Ultrasound could aid the diagnosis of sensory neuronopathy in spinocerebellar ataxias.