Lumina: Indonesian Journal of Neurology
Vol. 1 No. 2 (2025): August : Lumina : Indonesian Journal of Neurology

CANVAS Syndrome: A Comprehensive Case Report on Rare Ataxia

Evlyne Erlyana Suryawijaya (1) Fellowship Program of Neuro-ophthalmology and Neuro-otology, Indonesian Collegium of Neurology, Cipto Mangunkusumo National General Hospital, Indonesia, 2) Department of Neurology, Faculty of Medicine, Pelita Harapan University & Siloam Hospital L)
Ni Nengah Rida Ariarini (Division of Neuro-ophthalmology and Neuro-otology, Faculty of Medicine, Universitas Indonesia / Cipto Mangunkusumo National General Hospital)
Freddy Sitorus (Division of Neuro-ophthalmology and Neuro-otology, Faculty of Medicine, Universitas Indonesia / Cipto Mangunkusumo National General Hospital)
Eva Dewati (Division of Neuro-ophthalmology and Neuro-otology, Faculty of Medicine, Universitas Indonesia / Cipto Mangunkusumo National General Hospital)
Dinda Diafiri (Division of Neuro-ophthalmology and Neuro-otology, Faculty of Medicine, Universitas Indonesia / Cipto Mangunkusumo National General Hospital)



Article Info

Publish Date
30 Aug 2025

Abstract

Introduction: Cerebellar ataxia with neuropathy and vestibular areflexia syndrome (CANVAS) is characterized by cerebellum, vestibular system, and sensory pathways impairments. It progresses slowly compared to other ataxias like Friedreich’s ataxia, and its genetic basis is complex and under investigation. Case Report: A 34-year-old male presented with worsening imbalance over the past year, vertigo, slurred speech, and hand tremors. Physical examination revealed bidirectional nystagmus, dysmetria, and cerebellar atrophy on MRI. Vestibular tests were abnormal, and neuropsychological assessments showed memory and executive function deficits. Sensory nerve conduction studies were normal. Discussion: The patient's symptoms of ataxia, vestibular areflexia, and sensory neuropathy are consistent with CANVAS. Cerebellar atrophy and Purkinje cell degeneration contribute to motor coordination deficits. Sensory neuropathy involves dorsal root ganglia degeneration. Despite supportive clinical features, genetic testing is necessary to confirm the diagnosis and exclude other genetic ataxias. Conclusion: CANVAS is a rare ataxia syndrome with autosomal recessive inheritance affecting the cerebellum, vestibular, and sensory systems. The patient's symptoms and MRI findings suggest CANVAS, but further genetic testing is required for definitive diagnosis.

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Journal Info

Abbrev

lumina

Publisher

Subject

Description

Focus and Scope Lumina: Indonesian Journal of Neurology is an open-access, peer-reviewed journal dedicated to advancing the field of neurological sciences, with a focus on both basic neuroscience and clinical neurology. The journal publishes original research, reviews, and intriguing case reports. ...