Primary lateral sclerosis
- 9 January 2007
- journal article
- review article
- Published by Wiley in Muscle & Nerve
- Vol. 35 (3), 291-302
- https://doi.org/10.1002/mus.20728
Abstract
The spectrum of motor neuron diseases ranges from disorders that clinically are limited to lower motor neurons to those that exclusively affect upper motor neurons. Primary lateral sclerosis (PLS) is the designation for the syndrome of progressive upper motor neuron dysfunction when no other etiology is identified. Distinction between PLS and the more common amyotrophic lateral sclerosis (ALS) relies primarily on recognition of their symptoms and signs, as well as on ancillary, although non‐specific, laboratory data. In this review, we survey the history of PLS from the initial descriptions to the present. We discuss the role of laboratory, electrodiagnostic, and imaging studies in excluding other diagnoses; the findings from major case series of PLS patients; and proposed diagnostic criteria. Consistent differences are evident in patients classified as PLS compared to those with ALS, indicating that, despite its limitations, this clinical designation retains important utility. Muscle Nerve, 2007Keywords
This publication has 65 references indexed in Scilit:
- Primary lateral sclerosis; a debated entityActa Neurologica Scandinavica, 2009
- Novel mutation in the ALS2 gene in juvenile amyotrophic lateral sclerosisAnnals of Neurology, 2005
- Primary Lateral SclerosisJournal of Clinical Neuromuscular Disease, 2005
- Motor Neuron Firing Dysfunction in Spastic Patients With Primary Lateral SclerosisJournal of Neurophysiology, 2005
- Diffusion tensor imaging in the diagnosis of primary lateral sclerosisJournal of Magnetic Resonance Imaging, 2003
- Creatine kinase activity in amyotrophic lateral sclerosis patientsNeurological Sciences, 2003
- An ALS2 gene mutation causes hereditary spastic paraplegia in a Pakistani kindredAnnals of Neurology, 2002
- Primary lateral sclerosis: clinical, neurophysiological, and magnetic resonance findingsJournal of Neurology, Neurosurgery & Psychiatry, 2001
- A case of primary lateral sclerosis taking a prolonged clinical course with dementia and having an unusual dendritic ballooningNeuropathology, 1999
- Primary lateral sclerosisNeuropathology, 1997