A Diagnostic Approach to Spastic ataxia Syndromes

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 o...

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Bibliographic Details
Published in:Cerebellum (London, England) England), 2022-12, Vol.21 (6), p.1073-1084
Main Authors: Pedroso, José Luiz, Vale, Thiago Cardoso, França Junior, Marcondes C., Kauffman, Marcelo A., Teive, Helio, Barsottini, Orlando Graziani Povoas, Munhoz, Renato Puppi
Format: Article
Language:English
Subjects:
Alzheimer's disease
Ataxia
Atrophy
Biomedical and Life Sciences
Biomedicine
Cerebellar ataxia
Cerebellum
Dementia
Differential diagnosis
Genotype & phenotype
Heredity
Intellectual disabilities
Medical diagnosis
Medical imaging
Mutation
Neurobiology
Neuroimaging
Neurology
Neurosciences
Paralysis
Patients
Phenotypes
Review
Spasticity
Spinal cord
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Summary: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.
ISSN:1473-4230
1473-4222
1473-4230
DOI:10.1007/s12311-021-01345-5