Primary lateral sclerosis [chapter]

John H. J. Wokke, Pieter A. van Doorn, Jessica E. Hoogendijk, Marianne de Visser
Neuromuscular Disease: A Case-Based Approach  
Keywords Name of disease and synonyms Name of excluded diseases Diagnostic criteria / definition Differential diagnosis Frequency Clinical description Management including treatment Etiology Diagnosis Unresolved questions References Abstract Primary lateral sclerosis (PLS) is an idiopathic non-familial neurodegenerative disorder of upper motor neurons, presenting as a slowly progressive pyramidal tract syndrome. Disease onset is usually between 40 and 60 years with spasticity in the legs. Onset
more » ... in the legs. Onset in the pseudobulbar region with speech and swallowing disturbance is also possible. Ultimately, a tetrapyramidal syndrome develops, sometimes with marked pseudobulbar features (forced laughing and crying). Life expectancy is normal. Pringle criteria for PLS consist of adult onset of symptoms, a negative family history, normal findings of blood, urine and CSF tests, no abnormalities on EMG, and on brain and spinal cord MRI. PLS is considered to be a benign variant of Amyotrophic lateral sclerosis (ALS). The etiology is not known. The annual incidence and the prevalence of PLS are estimated to 1 in 1.000.000 and 10-20 in 1.000.000, respectively. Treatment of PLS is symptomatic and can involve prescription of antispasticity medication and rehabilitation.
doi:10.1017/cbo9780511735905.005 fatcat:rt4tta3kavck5kexjlk6eqx4mq