Paroxysmal Ataxia

Paroxysmal ataxia with onset in infancy or early childhood is usually associated with mental retardation. [©i In children, a metabolic disorder should be expected, such as urea cycle deficiencies, aminoacidurias, and disorders of pyruvate and lactate metabolism. Episodic ataxia in adults might be caused by drug ingestion, multiple sclerosis, transient vertebrobasilar ischemic attack, foramen magnum compression, intermittent obstruction of the ventricular system, and dominantly inherited periodic ataxia.

Autosomal dominant forms of paroxysmal cerebellar ataxia are characterized by attacks of vestibulocerebellar ataxia presenting with ataxia of gait and stance, dysarthria, vertigo, and nystagmus. y In episodic ataxia type 1, attacks last for a few seconds or minutes, and patients present with interictal facial myokymia. In episodic ataxia type 2, attacks last for a few hours to several weeks. In most patients, there are mild neurological signs between attacks, such as gaze-evoked down-and-out nystagmus and mild gait ataxia. Rarely, paroxysmal cerebellar ataxia type 2 is associated with progressing and disabling permanent cerebellar deficits.y

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