Ataxia is a lethal neurological disease characterized by incoordination, postural abnormalities, difficulties with gait, and problems with clarity of speech. The etiology of ataxia is divided equally between hereditary and sporadic forms. Regardless of cause, the cerebellar cortex is often a target in ataxia. Thus, how a disruption in cerebellar cortex might lead to ataxia is of considerable interest. A report in this issue of the JCI links ataxia to enhanced hyperexcitability of neurons in the deep cerebellar nuclei.
Harry T. Orr
Submitter: Dirk Ulbricht | ulbricht@macnews.de
Service de Neurologie, Hôpital de la Ville Esch-sur-Alzette, Luxembourg
Published February 18, 2004
Dr. Orr states that "...ataxia is a lethal neurological disease...“. This is wrong. Ataxia is a symptom of disease. The origin is primarily defined on clinical grounds with an anatomical hypothesis in mind, and the time course is used to define the probable cause. A difference can be made between cerebellar and spinal ataxia, the latter typically originating from lesions to the posterior column of the spinal cord, e.g. in case of Vitamin B12 deficiency. There are other symptoms called ataxia, but this is not nequivocal in the literature. It is also noteworthy that cerebellar ataxia arising from stroke, from MS, or from the residual damage of infantile neuroblastoma carries a usually benign prognosis. There is also a mixed ataxia when simply drinking a little bit too much, which is usually fairly well compatible with life. As the first sentence of the abstract, this leads the clinician to a somewhat suspicious attitude to the otherwise excellent editorial.