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Paroxysmal dyskinesias are a heterogeneous group of disorders characterized by intermittent attacks of hyperkinetic involuntary movements without loss of consciousness. The paroxysmal movements may affect the arm or leg on one or both sides of the body, or all extremities. All kinds of paroxysmal dyskinesias may be symptomatic in origin. Twenty-two percent of one series of patients with paroxysmal movement disorders had underlying causative diseases. History and neurologic examination are the cornerstones on the way to the diagnosis of paroxysmal dyskinesias. The paroxysmal nature and predominantly short duration of the attacks, the aura symptoms and the response to even low doses of anticonvulsant drugs suggest that paroxysmal dyskinesias closely resemble epilepsy. Sandifer syndrome is a rare movement disorder in toddlers. During or immediately after feeding, affected children exhibit severe dystonic movements or postures of the head and neck. They often vomit and are malnourished.
The term ataxia is derived from ancient Greek and literally means absence of order. In modern clinical neurology, ataxia is used to denote disturbances of coordinated muscle activity. Ataxia is caused by disorders of the cerebellum and its afferent or efferent connections. Spinal afferent pathways are often involved in ataxia disorders. Diseases of the peripheral nervous system, such as chronic idiopathic demyelinating polyneuropathy, may also cause ataxia. However, ataxia is rarely the prominent symptom in these disorders.
The afferent and efferent connections of the cerebellar cortex are topographically organized resulting in functional specialization of different parts of the cerebellum. Dysfunction of the lower vermis (vestibulocerebellum) leads to truncal ataxia. Spinocerebellar lesions (upper vermis and anterior parts of hemispheres) are characterized by unsteadiness of gait and stance which are more evident after eye closure (positive Rombergism). The most prominent symptom of neocerebellar damage (cerebellar hemispheres) is ataxia of intended limb movements. Ataxic limb movements are irregular and jerky and tend to overshoot the target (past-pointing). They are often accompanied by rhythmic side-to-side movements as the target is approached (action or intention tremor). Dysarthria characterized by slow and segmented speech with variable intonation and disturbances of ocular movements (broken-up smooth pursuit, saccadic hypermetria, gaze-evoked nystagmus) almost invariably accompany ataxia of gait and limb movements (Diener & Dichgans, 1992; Thach et al., 1992).
Knowledge of the topographical organization of the cerebellum is helpful for the localisation of focal cerebellar disease.
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