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Cerebellar ataxia, neuropathy, and vestibular areflexia syndrome: a slowly progressive disorder with stereotypical presentation.
J Neurol. 2016 Feb; 263(2):245-249.JN

Abstract

Cerebellar ataxia, neuropathy and vestibular areflexia syndrome (CANVAS) is a newly described condition with onset in adulthood, characterized by progressive balance impairment and sensory disturbances in the lower limbs, which can severely affect patients' quality of life. Its pathogenesis remains obscure and the diagnosis challenging. We described four patients complaining of slowly progressive gait unbalance and sensory disturbances at the feet followed, after a period ranging 2-6 years, by cerebellar dysfunction. All patients showed gait and limb ataxia, positive Romberg sign, cerebellar dysarthria, gaze-evoked nystagmus, absent deep tendon reflexes, and impaired vibratory sensation. Nerve conduction studies revealed axonal sensory neuropathy, brain magnetic resonance imaging showed cerebellar atrophy, and otoneurological investigation demonstrated bilateral vestibular areflexia with impaired vestibulo-ocular reflexes. The diagnosis of CANVAS should be suspected on clinical ground based on homogeneous course of symptoms and signs, and addressed by video-oculography eye movement recording.

Authors+Show Affiliations

Neuroalgology and Headache Unit, IRCCS Foundation, "Carlo Besta" Neurological Institute, Via Celoria, 11, 20133, Milan, Italy.Neuroalgology and Headache Unit, IRCCS Foundation, "Carlo Besta" Neurological Institute, Via Celoria, 11, 20133, Milan, Italy.Neuroalgology and Headache Unit, IRCCS Foundation, "Carlo Besta" Neurological Institute, Via Celoria, 11, 20133, Milan, Italy.Clinical Pathology and Genetics Unit, IRCCS Foundation, "Carlo Besta" Neurological Institute, Milan, Italy.Neuroalgology and Headache Unit, IRCCS Foundation, "Carlo Besta" Neurological Institute, Via Celoria, 11, 20133, Milan, Italy. glauria@istituto-besta.it.

Pub Type(s)

Case Reports
Journal Article

Language

eng

PubMed ID

26566912

Citation

Cazzato, Daniele, et al. "Cerebellar Ataxia, Neuropathy, and Vestibular Areflexia Syndrome: a Slowly Progressive Disorder With Stereotypical Presentation." Journal of Neurology, vol. 263, no. 2, 2016, pp. 245-249.
Cazzato D, Bella ED, Dacci P, et al. Cerebellar ataxia, neuropathy, and vestibular areflexia syndrome: a slowly progressive disorder with stereotypical presentation. J Neurol. 2016;263(2):245-249.
Cazzato, D., Bella, E. D., Dacci, P., Mariotti, C., & Lauria, G. (2016). Cerebellar ataxia, neuropathy, and vestibular areflexia syndrome: a slowly progressive disorder with stereotypical presentation. Journal of Neurology, 263(2), 245-249. https://doi.org/10.1007/s00415-015-7951-9
Cazzato D, et al. Cerebellar Ataxia, Neuropathy, and Vestibular Areflexia Syndrome: a Slowly Progressive Disorder With Stereotypical Presentation. J Neurol. 2016;263(2):245-249. PubMed PMID: 26566912.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Cerebellar ataxia, neuropathy, and vestibular areflexia syndrome: a slowly progressive disorder with stereotypical presentation. AU - Cazzato,Daniele, AU - Bella,Eleonora Dalla, AU - Dacci,Patrizia, AU - Mariotti,Caterina, AU - Lauria,Giuseppe, Y1 - 2015/11/14/ PY - 2015/08/27/received PY - 2015/10/22/accepted PY - 2015/10/20/revised PY - 2015/11/15/entrez PY - 2015/11/15/pubmed PY - 2016/12/15/medline KW - CANVAS KW - Cerebellar KW - Eye movements KW - Sensory neuropathy KW - Vestibular SP - 245 EP - 249 JF - Journal of neurology JO - J Neurol VL - 263 IS - 2 N2 - Cerebellar ataxia, neuropathy and vestibular areflexia syndrome (CANVAS) is a newly described condition with onset in adulthood, characterized by progressive balance impairment and sensory disturbances in the lower limbs, which can severely affect patients' quality of life. Its pathogenesis remains obscure and the diagnosis challenging. We described four patients complaining of slowly progressive gait unbalance and sensory disturbances at the feet followed, after a period ranging 2-6 years, by cerebellar dysfunction. All patients showed gait and limb ataxia, positive Romberg sign, cerebellar dysarthria, gaze-evoked nystagmus, absent deep tendon reflexes, and impaired vibratory sensation. Nerve conduction studies revealed axonal sensory neuropathy, brain magnetic resonance imaging showed cerebellar atrophy, and otoneurological investigation demonstrated bilateral vestibular areflexia with impaired vestibulo-ocular reflexes. The diagnosis of CANVAS should be suspected on clinical ground based on homogeneous course of symptoms and signs, and addressed by video-oculography eye movement recording. SN - 1432-1459 UR - https://wwww.unboundmedicine.com/medline/citation/26566912/Cerebellar_ataxia_neuropathy_and_vestibular_areflexia_syndrome:_a_slowly_progressive_disorder_with_stereotypical_presentation_ L2 - https://dx.doi.org/10.1007/s00415-015-7951-9 DB - PRIME DP - Unbound Medicine ER -