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Arch Neurol. 2009 Jun;66(6):751-7. doi: 10.1001/archneurol.2009.91.

A long-term prospective study of the natural course of sporadic adult-onset lower motor neuron syndromes.

Author information

1
Department of Neurology, St Lucas Andreas Hospital, PO Box 9243, 1006 AE Amsterdam, the Netherlands. r.vandenberg-vos@slaz.nl

Abstract

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.

PMID:
19506135
DOI:
10.1001/archneurol.2009.91
[Indexed for MEDLINE]

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