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Ataxia is a symptom of cerebellar dysfunction. Slowly progressive ataxia, dysarthria in an adult with a positive family history suggests an inherited cerebellar ataxia. We present an adult with gradually progressive ataxia and slow saccades. There was history of similar illness in his son. Genetic testing for spinocerebellar ataxia 2 was positive. We discuss the various inherited ataxias, causes of acute, progressive ataxia syndromes, episodic ataxias and ataxia associated with other neurological signs like peripheral neuropathy, pyramidal features, movement disorders and cognitive decline.

A 24-year-old female presented with wasting and weakness of both hands and fasciculations over the chin since 12 years, followed by imbalance while walking and speech changes since 10 years. Her 12-year-old sister also had a similar clinical presentation. There were fasciculations over the chin, tongue, hands, back, thighs with wasting and weakness in tongue, and C7, C8, T1 segments in both upper limbs along with bipyramidal signs. There was limb and gait ataxia. Magnetic resonance imaging brain showed pancerebellar atrophy, and electromyography was suggestive of anterior horn cell involvement in bulbar, cervical, thoracic, and lumbar segments. Next-generation sequencing identified a novel likely pathogenic deletion mutation: chr6:152527389_152527399del, c.22711_22721del, and p.Ala7571ArgfsTer4 in exon 125 of synaptic nuclear envelope protein 1 ( SYNE1 ) gene. This mutation leads to frameshift and premature termination of the protein 'Nesprin 1'. Amyotrophic lateral sclerosis-like presentation followed by cerebellar ataxia have been described with SYNE1 ataxia. This unique phenotype and novel deletion mutation of SYNE1 gene is the first case reported from India.

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Background Vertigo as a symptom of cerebrovascular disease is relatively uncommon. All types of cerebrovascular diseases namely ischemia, infarction, hemorrhage can produce vertigo. Since, cerebrovascular disease is an emergency prompt recognition and treatment is necessary to prevent neurologic deficit and death. Among cerebrovascular diseases vertebrobasilar territory strokes commonly present with vertigo. Since, the term vertigo is used nonspecifically by patients this may lead to delay in diagnosis of these strokes. This article reviews the epidemiology of vertigo in cerebrovascular diseases and the various stroke syndromes associated with vertigo. Summary Cerebrovascular diseases in the vertebrobasilar territory have vertigo, imbalance, dizziness in addition to other symptoms and signs. Posterior inferior cerebellar artery, anterior inferior cerebellar artery, superior cerebellar artery and basilar artery territory strokes can present with true vertigo. A high index of suspicion of stroke in patients with vertigo and risk factors for stroke is essential. Other vascular causes of vertigo are small cerebellar hemorrhage, vestibular cortex stroke, rotational vertebral artery syndrome, transverse/sigmoid sinus thrombosis and vestibular paroxysmia. Conclusion Cerebrovascular disorders are estimated to account for 3 to 4% of patients with vertigo or dizziness. Early detection and treatment is necessary to prevent disability and death in these cases of vascular vertigo. How to cite this article Chakor RT, Eklare N. Vertigo in Cerebrovascular diseases. Int J Otorhinolaryngol Clin 2012;4(1):46-53.