In the diagnosis of disorders characterized by generalized cerebellar ataxia (affecting limbs, gait, and speech), the mode of onset, rate of development, and degree of permanence of the ataxia are of particular importance, as summarized in Table 5-3. Unilateral ataxia without accompanying signs is most often caused by infarction or tumor in the ipsilateral cerebellar hemisphere or by demyelinating disease affecting cerebellar connections to the brainstem. Each of the major causes is discussed in an appropriate chapter. In adults, toxic effects of medications, paraneoplastic, and demyelinating cases account for the largest proportion of cases of subacute onset, and hereditary forms are the usual cause of very slowly progressive and chronic ones, particularly if gait is predominantly affected. The last category of genetic ataxias constitutes a large and heterogeneous group for which the mutation has been established in many cases; they are described in Chap. 38.
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