Differential diagnosis of cerebellar ataxias on the basis of the age at onset

Abstract

Ataxias are a complex and heterogeneous group of disorders characterized by the absence of order and coordination of voluntary movements and loss of equilibrium. The major clinical criteria for diagnosis include disease extension (focal vs non focal), etiology (hereditary vs symptomatic or idiopathic), progression rate, age at onset, and neurological and systemic symptoms that support the diagnosis of specifi c disorders. Age at onset is a very important clinical tool that along with inheritance are useful address the proper differential diagnosis. In this way three major categories of cerebellar ataxias may be recognized: (i) congenital ataxias in infancy and young children (<2 years), (ii) early-onset in childhood, adolescence and young adulthood (<25 years), and (iii) late-onset cerebellar ataxias.