A Diagnostic Approach to Spastic ataxia Syndromes

PEDROSO, JOSÉ LUIZ; VALE, THIAGO CARDOSO; FRANÇA JUNIOR, MARCONDES C.; KAUFFMAN, MARCELO A.; TEIVE, HELIO; BARSOTTINI, ORLANDO GRAZIANI POVOAS; MUNHOZ, RENATO PUPPI

CEREBELLUM

SPRINGER

Año: 2022 vol. 21 p. 1073 - 1084

1473-4222

Spastic ataxia is characterized by the combination of cerebellar ataxia with spasticity and other pyramidal features. It is the hallmark of some hereditary ataxias, but it can also occur in some spastic paraplegias and acquired conditions. It often presents with heterogenous clinical features with other neurologic and non-neurological symptoms, resulting in complex phenotypes. In this review, the differential diagnosis of spastic ataxias are discussed and classified in accordance with inheritance. Establishing an organized classification method based on mode inheritance is fundamental for the approach to patients with these syndromes. For each differential, the clinical features, neuroimaging and genetic aspects are reviewed. A diagnostic approach for spastic ataxias is then proposed.