Progression in primary lateral sclerosis: a prospective analysis.
Progression in primary lateral sclerosis: a prospective analysis.
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DOI：
10.3109/17482960903171136
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发表时间：
2009-10
期刊：
影响因子：
--
通讯作者：
Mills R
中科院分区：
文献类型：
--
作者：
Floeter MK;Mills R
确定原发性侧索硬化（PLS）患者的进展率和模式是否不同。符合PLS临床标准的50例患者在初始表现时分为3种亚型：上行性、多灶性和散发性下肢轻瘫（PLS-A、PLS-M或PLS-SP）。每年对患者进行调查。每隔1-5年重新评估运动速度、临床评定量表和经颅磁刺激的测量值，以确定是否扩散到其他身体区域和受影响区域内的严重程度进展。47例患者在平均6.6年的随访中继续符合PLS标准，平均病程> 14年。PLS-A患者有更可预测的进展到其他身体部位。在PLS-A或PLS-M亚型中，新受累区域的严重程度进展更快，随后稳定。PLS的临床进展不会稳定发生，但在扩散到新受影响的区域后会有更快的下降期。PLS患者的亚型分类与预测疾病的传播更相关，但与严重程度的进展无关。
To determine whether rates and patterns of progression differ among Primary Lateral Sclerosis (PLS) patients. 50 patients fulfilling clinical criteria for PLS were classified on initial presentation into 3 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 years 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.
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影响因子：
11.2
作者：
APPEL, V;STEWART, SS;APPEL, SH
通讯作者：
APPEL, SH
影响因子：
9.9
作者：
Brugman, F;Wokke, JHJ;Van den Berg, LH
通讯作者：
Van den Berg, LH
影响因子：
14.5
作者：
HAVERKAMP, LJ;APPEL, V;APPEL, SH
通讯作者：
APPEL, SH
影响因子：
3.4
作者：
Czaplinski, Adam;Yen, Albert A.;Appel, Stanley H.
通讯作者：
Appel, Stanley H.
影响因子：
9.9
作者：
Mitsumoto, H.;Ulug, A. M.;Shungu, D. C.
通讯作者：
Shungu, D. C.