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[Cerebellar ataxia with neuropathy and vestibular areflexia syndrome (CANVAS): a case report].
Rinsho Shinkeigaku. 2019 Jan 30; 59(1):27-32.RS

Abstract

Cerebellar ataxia with neuropathy and vestibular areflexia syndrome (CANVAS) is a rare form of multisystem ataxia defined by a triad of cerebellar impairment, bilateral vestibular hypofunction, and somatosensory deficit. Here we present a patient with CANVAS. A 76-year-old woman whose parents were cousins had noted slowly worsening gait imbalance since age 67. Peripheral sensory impairment was evident since age 73. When examined at 74, she had a frequent cough. The neurologic examinations showed scanning speech, downbeat nystagmus, pursuit eye movements with saccadic features, truncal ataxia, and mild dysmetria of the extremities. The Romberg test was positive. Light touch, pinprick, and vibration sensation were absent in the distal lower limbs, where allodynia could be demonstrated. Ankle jerk reflex was diminished. Muscle strength was normal. Nerve conduction studies disclosed absence of sensory nerve action potentials in all limbs, while motor conduction was normal except for decreased amplitude of left median and bilateral ulnar nerve compound motor action potentials. MRI of the brain demonstrated cerebellar atrophy. The eye tracking test for the smooth pursuit and visually enhanced vestibulo-ocular reflex test demonstrated functional impairments. Both the bithermal caloric test and the video head impulse testing showed sever hypofunction of the bilateral semicircular canal. In sum, somatosensory deficit and otoneurologic examinations indicated bilateral vestibulopathy which, together with the patient's and cerebellar impairment, confirmed the diagnosis of CANVAS.

Authors+Show Affiliations

Department of Neurology, National Hospital Organization Minamikyushu National Hospital.Department of Otolaryngology, Gifu University Graduate School of Medicine.Department of Neurology, National Hospital Organization Minamikyushu National Hospital.

Pub Type(s)

Case Reports
Journal Article

Language

jpn

PubMed ID

30606994

Citation

Maruta, Kyoko, et al. "[Cerebellar Ataxia With Neuropathy and Vestibular Areflexia Syndrome (CANVAS): a Case Report]." Rinsho Shinkeigaku = Clinical Neurology, vol. 59, no. 1, 2019, pp. 27-32.
Maruta K, Aoki M, Sonoda Y. [Cerebellar ataxia with neuropathy and vestibular areflexia syndrome (CANVAS): a case report]. Rinsho Shinkeigaku. 2019;59(1):27-32.
Maruta, K., Aoki, M., & Sonoda, Y. (2019). [Cerebellar ataxia with neuropathy and vestibular areflexia syndrome (CANVAS): a case report]. Rinsho Shinkeigaku = Clinical Neurology, 59(1), 27-32. https://doi.org/10.5692/clinicalneurol.cn-001209
Maruta K, Aoki M, Sonoda Y. [Cerebellar Ataxia With Neuropathy and Vestibular Areflexia Syndrome (CANVAS): a Case Report]. Rinsho Shinkeigaku. 2019 Jan 30;59(1):27-32. PubMed PMID: 30606994.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - [Cerebellar ataxia with neuropathy and vestibular areflexia syndrome (CANVAS): a case report]. AU - Maruta,Kyoko, AU - Aoki,Mitsuhiro, AU - Sonoda,Yoshito, Y1 - 2018/12/29/ PY - 2019/1/5/pubmed PY - 2019/2/16/medline PY - 2019/1/5/entrez KW - bilateral vestibulopathy KW - cerebellar ataxia KW - cerebellar ataxia with neuropathy and vestibular areflexia syndrome (CANVAS) KW - neuronopathy KW - sensory neuropathy SP - 27 EP - 32 JF - Rinsho shinkeigaku = Clinical neurology JO - Rinsho Shinkeigaku VL - 59 IS - 1 N2 - Cerebellar ataxia with neuropathy and vestibular areflexia syndrome (CANVAS) is a rare form of multisystem ataxia defined by a triad of cerebellar impairment, bilateral vestibular hypofunction, and somatosensory deficit. Here we present a patient with CANVAS. A 76-year-old woman whose parents were cousins had noted slowly worsening gait imbalance since age 67. Peripheral sensory impairment was evident since age 73. When examined at 74, she had a frequent cough. The neurologic examinations showed scanning speech, downbeat nystagmus, pursuit eye movements with saccadic features, truncal ataxia, and mild dysmetria of the extremities. The Romberg test was positive. Light touch, pinprick, and vibration sensation were absent in the distal lower limbs, where allodynia could be demonstrated. Ankle jerk reflex was diminished. Muscle strength was normal. Nerve conduction studies disclosed absence of sensory nerve action potentials in all limbs, while motor conduction was normal except for decreased amplitude of left median and bilateral ulnar nerve compound motor action potentials. MRI of the brain demonstrated cerebellar atrophy. The eye tracking test for the smooth pursuit and visually enhanced vestibulo-ocular reflex test demonstrated functional impairments. Both the bithermal caloric test and the video head impulse testing showed sever hypofunction of the bilateral semicircular canal. In sum, somatosensory deficit and otoneurologic examinations indicated bilateral vestibulopathy which, together with the patient's and cerebellar impairment, confirmed the diagnosis of CANVAS. SN - 1882-0654 UR - https://wwww.unboundmedicine.com/medline/citation/30606994/[Cerebellar_ataxia_with_neuropathy_and_vestibular_areflexia_syndrome__CANVAS_:_a_case_report]_ DB - PRIME DP - Unbound Medicine ER -