Autosomal recessive spino-olivo-cerebellar degeneration without ataxia.
نویسندگان
چکیده
منابع مشابه
Spongy degeneration with cerebellar ataxia in Malinois puppies: a hereditary autosomal recessive disorder?
BACKGROUND There is a high incidence of hereditary degenerative diseases of the central nervous system in purebred dogs. HYPOTHESIS Cerebellar ataxia in Malinois puppies, caused by degenerative changes that predominate in cerebellar nuclei and the granular cell layer, is a hereditary disorder that is distinct from cerebellar cortical abiotrophies. ANIMALS Thirteen Malinois puppies with cere...
متن کاملUnique cerebellar-cerebral form of autosomal recessive ataxia.
We describe a unique condition affecting two siblings with a form of progressive spinocerebellar ataxia. After a period of very slowly progressive ataxia, the patients developed an extremely accelerated progression of the condition which consisted of cerebellar ataxia, seizure, progressive dementia and spastic tetraparesis. Age of onset was variable at 7 to 18 years. Brain magnetic resonance im...
متن کاملAtaxia and the olivo-cerebellar module.
Ataxia is a neurological symptom characterized by abnormal movement, due to cerebellar malfunction. Although the cause of the malfunction varies, from mutations in calcium or potassium channels to progressive degeneration of cerebellar tissue, the neurological symptoms of cerebellar-related ataxia are similar. In this short review we present a model that portrays the olivo-cerebellar system as ...
متن کاملAutosomal recessive cerebellar ataxias
Autosomal recessive cerebellar ataxias (ARCA) are a heterogeneous group of rare neurological disorders involving both central and peripheral nervous system, and in some case other systems and organs, and characterized by degeneration or abnormal development of cerebellum and spinal cord, autosomal recessive inheritance and, in most cases, early onset occurring before the age of 20 years. This g...
متن کاملVestibular findings in autosomal recessive ataxia.
OBJECTIVE This study aims to examine vestibular disorders in patients with recessive spinocerebellar ataxia. DESIGN A retrospective cross-sectional study was conducted. The patients underwent the following procedures: case history, ENT and vestibular evaluations. STUDY SAMPLE The tests were performed in 19 patients ranging from 6 to 63 years of age (mean age of 36.7). RESULTS Clinically, ...
متن کاملذخیره در منابع من
با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید
ژورنال
عنوان ژورنال: Journal of Neurology, Neurosurgery & Psychiatry
سال: 1983
ISSN: 0022-3050
DOI: 10.1136/jnnp.46.7.648