A 31-year-old woman had a progressive history of gait and limb incoordination and head tremor.

Our patient had a 4-year history of progressive ataxia beginning in her early 20s. Family history was remarkable, suggesting a dominant pattern of inheritance (with age of onset in the 20s). Her most prominent features were diplopia, sensory loss, and tremor. Frequent headaches complicated her clinical course. Neurologic examination demonstrated prominent dysarthria, left esotropia, and marked head and hand tremors. Muscle stretch reflexes were hypoactive. Gait was wide based and ataxic.

Our patient appeared to have a hereditary cerebellar ataxia. Investigation of these patients requires appropriate use of clinical algorithms based on mode of inheritance and MRI patterns of atrophy (Fig. 71.1).