TY - JOUR
T1 - A long-term prospective study of the natural course of sporadic adult-onset lower motor neuron syndromes
AU - van den Berg-Vos, Renske M.
AU - Visser, Jeldican
AU - Kalmijn, Sandra
AU - Fischer, Kathelijn
AU - de Visser, Marianne
AU - de Jong, Vianney
AU - de Haan, Rob J.
AU - Franssen, Hessel
AU - Wokke, John H. J.
AU - van den Berg, Leonard H.
PY - 2009
Y1 - 2009
N2 - OBJECTIVE: To determine the natural course of sporadic adult-onset lower motor neuron syndrome in a long-term prospective study of patients with the syndrome. DESIGN: Inception cohort with a follow-up of 72 months. SETTING: Three university hospitals in the Netherlands (referral centers for neuromuscular diseases). PATIENTS: Thirty-two patients were classified as having the following phenotypes according to previously defined criteria: progressive muscular atrophy (PMA; 10 patients), segmental distal muscular atrophy (8 patients), and segmental proximal muscular atrophy (14 patients). A disease duration of at least 4 years was chosen to exclude most patients with amyotrophic lateral sclerosis (ALS). MAIN OUTCOME MEASURES: Muscle strength, functional impairment, and respiratory function were assessed at 0, 6, 12, 18, and approximately 72 months. RESULTS: The diagnosis had to be changed to ALS in 3 patients (classified at inclusion as PMA in 2 patients and segmental proximal muscular atrophy in 1) owing to the development of upper motor neuron signs in 2 patients and familial ALS in 1. The remaining 8 patients with PMA showed further deterioration, and the other 24 patients remained more or less stable during long-term follow-up. Respiratory insufficiency developed in 6 of the 11 patients with ALS or PMA, 5 of whom died. CONCLUSIONS: Patients with lower motor neuron syndromes and a disease duration of at least 4 years usually have a favorable prognosis if muscle involvement has a segmental distribution. In patients with a generalized phenotype, progression is relentlessly progressive and eventually leads to death due to respiratory insufficiency
AB - OBJECTIVE: To determine the natural course of sporadic adult-onset lower motor neuron syndrome in a long-term prospective study of patients with the syndrome. DESIGN: Inception cohort with a follow-up of 72 months. SETTING: Three university hospitals in the Netherlands (referral centers for neuromuscular diseases). PATIENTS: Thirty-two patients were classified as having the following phenotypes according to previously defined criteria: progressive muscular atrophy (PMA; 10 patients), segmental distal muscular atrophy (8 patients), and segmental proximal muscular atrophy (14 patients). A disease duration of at least 4 years was chosen to exclude most patients with amyotrophic lateral sclerosis (ALS). MAIN OUTCOME MEASURES: Muscle strength, functional impairment, and respiratory function were assessed at 0, 6, 12, 18, and approximately 72 months. RESULTS: The diagnosis had to be changed to ALS in 3 patients (classified at inclusion as PMA in 2 patients and segmental proximal muscular atrophy in 1) owing to the development of upper motor neuron signs in 2 patients and familial ALS in 1. The remaining 8 patients with PMA showed further deterioration, and the other 24 patients remained more or less stable during long-term follow-up. Respiratory insufficiency developed in 6 of the 11 patients with ALS or PMA, 5 of whom died. CONCLUSIONS: Patients with lower motor neuron syndromes and a disease duration of at least 4 years usually have a favorable prognosis if muscle involvement has a segmental distribution. In patients with a generalized phenotype, progression is relentlessly progressive and eventually leads to death due to respiratory insufficiency
U2 - https://doi.org/10.1001/archneurol.2009.91
DO - https://doi.org/10.1001/archneurol.2009.91
M3 - Article
C2 - 19506135
SN - 0003-9942
VL - 66
SP - 751
EP - 757
JO - Archives of neurology
JF - Archives of neurology
IS - 6
ER -