We herein report the case of an 81-year-old woman with midbrain infarction causing pupil-sparing oculomotor nerve palsy with ipsilateral cerebellar ataxia. The lesion was located at the rostral end of the decussation of the superior cerebellar peduncle touching the dorsal side, further caudal and dorsal to causal lesions of Claude's syndrome, which presented as oculomotor palsy and contralatera...

OBJECTIVE Spinocerebellar ataxias are neurodegenerative disorders involving the cerebellum and its connections. There are more than 30 distinct subtypes, 16 of which are associated with an identified gene. The aim of the current study was to evaluate a large group of patients from 104 Brazilian families with spinocerebellar ataxias. METHODS We studied 150 patients from 104 families with spino...

how to cite this article: shervin badv r, niksirat a. downward vertical gaze palsy as a prominent manifestation of episodic ataxia type 2: a case report. iran j child neurol. 2013 autumn; 7(4):58- 60.   objective episodic ataxia type 2 (ea2) is an inherited autosomal dominant disorder characterized by intermittent ataxia, nausea, vomiting, dysarthria, or nystagmus. we report a case of ea2, whic...

Dear Editor, The initial presentation of cerebellar ataxia remains a diagnostic challenge due to its multiple etiologies, one of which is paraneoplastic cerebellar degeneration (PCD), a rare neurological disease. Although paraneoplastic antibody testing may be helpful, seronegative PCD may account for up to 50% of PCD cases.1 The causes of ataxia in the Asian population differ from that of the ...

Two sisters are reported who both developed partial cranial diabetes insipidus in their 4th decade, followed by progressive cerebellar ataxia. This appears to be the first report of cerebellar ataxia and diabetes insipidus occurring together as a genetic entity.

BACKGROUND Dentatorubral-pallidoluysian atrophy (DRPLA) is a rare neurodegenerative disorder mainly diagnosed in Japan. Its prevalence is low in other countries. Three phenotypes are described: choreoathetoid movements, cerebellar ataxia, and progressive myoclonic epilepsy. OBJECTIVE To evaluate the frequency of DRPLA in European patients with sporadic or autosomal dominant cerebellar ataxia....

Cortical cerebellar atrophy (CCA) and multiple system atrophy with predominant cerebellar ataxia (MSA-C) are the two major forms of adult-onset sporadic ataxia. Contrary to MSA-C, there are neither diagnostic criteria nor neuroimaging features pathognomonic for CCA. Therefore, it is assumed that the category of CCA in the Japanese national registry include heterogeneous cerebellar ataxic disord...

Three patients presented with cerebellar ataxia among 350 cases of systemic lupus erythematosus (SLE) seen over the last 14 years. Cerebellar signs were unilateral in one and bilateral in the other two patients. Other neurological findings were present in all three patients. One initially presented with only cerebellar ataxia; other features of SLE appeared a few years later. Lupus anticoagulan...

Abstract Background and purpose Immune mediated cerebellar ataxias account for a substantial proportion of all progressive ataxias. A diagnostic serological test is not always available. This particularly problematic in Primary Autoimmune Cerebellar Ataxia, hence the necessity criteria recently devised published by an International Task Force. We present our experience use commercially availabl...

Consumption of alcohol excessively causes dependence and leads to psychological discomfort. Motivation drink is influenced by a variety neurobiological environmental variables. The proclivity an individual imbibe thought reflect balance between alcohol’s positive reinforcing (i.e., rewarding) effects, such as euphoria anxiety reduction anxiolysis), the drug...