Ataxias

Ataxies are movement disorders. They are muscle group coordination problems deriving from a disturbed functioning in the peripheral nervous system, the spinal cord or the brain.

We distinguish between non-severe and severe forms of Ataxia. In some metabolic diseases, e.g. thyroid malfunction or tumor diseases, ataxias appear as a symptom, while other ataxias, such as Friedreichsche ataxia, are hereditary.

The Department of Neurology offers deep and thorough diagnostics of these different forms of ataxia.

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What forms of ataxia disorder are known?

To date, more than 30 different autosomal dominant hereditary Spinocerebellar ataxias (SCA) have become known. Of these, about half can be diagnosed in a molecular genetic examination.  In these patients – depending on the underlying form of the SCA – polyneuropathies, lesions of the motor pathways, a Parkinson syndrome, retinal diseases, cognitive disorders or muscle atrophies arise. Often several members of the family are affected.  In most patients with Friedreich ataxia (FA) – usually manifesting in their youth –, disturbed sensibility, and often spasticia of the leg and arm muscles appear along with coordination disorders deriving from cerebellar atrophy. The heart can also be involved and a Diabetes mellitus may be developing. Hereditary forms of ataxia that are even rarer can seem to be deceivingly similar to Friedreich ataxia.

Apart from these, there are many non-hereditary ataxia disorders.

They can seldom be associated with particular antibodies or tumors (paraneoplastic cerebellar degeneration, PCD). Diagnosis here is very important, since immunotherapeutics in certain circumstances can help these patients. Usually accompanying a Parkinson syndrome is the multisystem atrophy of the cerebellar type (MSA-C).

Not seldom given the limited body of knowledge on these diseases, we cannot establish definite diagnosis. If no proof of inheritable origin has been found, the diagnosis posed is sporadic adult ataxia of unclear origin (SAOA).