Mary Kay Floeter1, Reversa Mills. 1. Electromyography Section, National Institutes of Neurological Disorders and Stroke, 10 Center Drive, Bethesda, Maryland 20892, USA. floeterm@ninds.nih.gov
Abstract
OBJECTIVE: To determine whether rates and patterns of progression differ among primary lateral sclerosis (PLS) patients. METHODS: Fifty patients fulfilling clinical criteria for PLS were classified on initial presentation into three subtypes: ascending, multifocal, and sporadic paraparesis (PLS-A, PLS-M or PLS-SP). Patients were surveyed annually. Measures of movement speed, clinical rating scales, and transcranial magnetic stimulation were re-assessed at 1-5 year intervals for spread to additional body regions and progression of severity within affected regions. RESULTS: Forty-seven patients continued to fulfill criteria for PLS over a mean follow-up of 6.6 years, with a mean disease duration > 14 years. PLS-A patients had more predictable progression to additional body regions. Severity progressed faster in newly affected regions followed by stabilization in PLS-A or PLS-M subtypes. CONCLUSION: Clinical progression in PLS does not occur steadily, but has periods of faster decline upon spreading to a newly affected region. Classification of PLS patients by subtype is more relevant to predicting the spread of disease, but not progression of severity.
OBJECTIVE: To determine whether rates and patterns of progression differ among primary lateral sclerosis (PLS) patients. METHODS: Fifty patients fulfilling clinical criteria for PLS were classified on initial presentation into three subtypes: ascending, multifocal, and sporadic paraparesis (PLS-A, PLS-M or PLS-SP). Patients were surveyed annually. Measures of movement speed, clinical rating scales, and transcranial magnetic stimulation were re-assessed at 1-5 year intervals for spread to additional body regions and progression of severity within affected regions. RESULTS: Forty-seven patients continued to fulfill criteria for PLS over a mean follow-up of 6.6 years, with a mean disease duration > 14 years. PLS-A patients had more predictable progression to additional body regions. Severity progressed faster in newly affected regions followed by stabilization in PLS-A or PLS-M subtypes. CONCLUSION: Clinical progression in PLS does not occur steadily, but has periods of faster decline upon spreading to a newly affected region. Classification of PLSpatients by subtype is more relevant to predicting the spread of disease, but not progression of severity.
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