Floeter Mary Kay, Mills Reversa
Electromyography Section, National Institutes of Neurological Disorders and Stroke, 10 Center Drive, Bethesda, Maryland 20892, USA.
Amyotroph Lateral Scler. 2009 Oct-Dec;10(5-6):339-46. doi: 10.3109/17482960903171136.
To determine whether rates and patterns of progression differ among primary lateral sclerosis (PLS) patients.
Fifty patients fulfilling clinical criteria for PLS were classified on initial presentation into three subtypes: ascending, multifocal, and sporadic paraparesis (PLS-A, PLS-M or PLS-SP). Patients were surveyed annually. Measures of movement speed, clinical rating scales, and transcranial magnetic stimulation were re-assessed at 1-5 year intervals for spread to additional body regions and progression of severity within affected regions.
Forty-seven patients continued to fulfill criteria for PLS over a mean follow-up of 6.6 years, with a mean disease duration > 14 years. PLS-A patients had more predictable progression to additional body regions. Severity progressed faster in newly affected regions followed by stabilization in PLS-A or PLS-M subtypes.
Clinical progression in PLS does not occur steadily, but has periods of faster decline upon spreading to a newly affected region. Classification of PLS patients by subtype is more relevant to predicting the spread of disease, but not progression of severity.
确定原发性侧索硬化症（PLS）患者的病情进展速率和模式是否存在差异。
50例符合PLS临床标准的患者在初次就诊时被分为三种亚型：上行型、多灶型和散发性轻截瘫型（PLS-A、PLS-M或PLS-SP）。每年对患者进行随访。每隔1至5年重新评估运动速度、临床评定量表和经颅磁刺激，以观察病情是否扩散至身体其他部位以及受累部位严重程度的进展情况。
47例患者在平均6.6年的随访期内仍符合PLS标准，平均病程>14年。PLS-A型患者病情进展至身体其他部位的情况更具可预测性。在新受累区域，病情严重程度进展更快，随后在PLS-A或PLS-M亚型中趋于稳定。
PLS的临床进展并非稳步发生，而是在扩散至新受累区域时会有病情快速下降的时期。根据亚型对PLS患者进行分类对于预测疾病扩散更具相关性，但对病情严重程度的进展则不然。
