Archives of neurology · 2009
A long-term prospective study of the natural course of sporadic adult-onset lower motor neuron syndromes.
СтатьяПоддержка исследования: вне правительства СШАНазвание и аннотация: Перевод пока не подготовлен
Обработка статьи · этапы, ошибки и управление
Загрузка состояния…
Ошибки и ограничения
История · было → стало
Состояние обновляется каждые 5 секунд, пока блок открыт. Процент показывается только там, где известен объём работы. Пауза останавливает продолжение этапа; текущий запрос к источнику может завершиться.
Van den Berg-Vos Renske M, Visser Jeldican, Kalmijn Sandra, Fischer Kathelijn, de Visser Marianne, de Jong Vianney, de Haan Rob J, Franssen Hessel, Wokke John H J, Van den Berg Leonard H
Аннотация
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.
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.
Предметные рубрики MeSH
AdultCohort StudiesDisability EvaluationDisease ProgressionExtremitiesFollow-Up StudiesHumansLongitudinal StudiesMotor Neuron DiseaseMotor NeuronsMuscle WeaknessMuscle, SkeletalMuscular Atrophy, SpinalNeurologic ExaminationPrognosisProspective StudiesRespiratory ParalysisSeverity of Illness IndexSpinal CordTime Factors