Laitman Benjamin M, Cook Karin, Fletcher Madhuri, Krieger Stephen C
Icahn School of Medicine at Mount Sinai, The Mount Sinai Hospital, New York, NY, USA.
MS Working Group, Harrison and Star, New York, NY, USA.
Mult Scler J Exp Transl Clin. 2018 Oct 14;4(4):2055217318806527. doi: 10.1177/2055217318806527. eCollection 2018 Oct-Dec.
Using the topographical model of multiple sclerosis (MS) to evaluate a longitudinal cohort we (1) test the recapitulation hypothesis, positing that patients' "disease topography" predicts the clinical pattern of disability accumulation; and (2) identify leading indicators of progression.
10 patients who transitioned from relapsing-remitting MS to secondary progressive MS (SPMS) were evaluated. Neurologic exams were analyzed from relapses, at time of SPMS diagnosis, and most recent visit. Functional systems (FS), location/laterality, and recovery were recorded. The pyramidal/motor system was the target FS assessing symptom laterality and severity at relapse and SPMS time-points. Each patient's clinical course was mapped using the topographical model software.
Cohort was 80% female, age 31.6 ± 8.6 years at diagnosis, followed average 23.8 ± 8.8 years, mean 3.1 relapses before SPMS. 83.3 ± 0.2% of relapse symptoms were present at transition to SPMS, increasing to 91.0 ± 0.2% at most recent visit. This demonstrates concordance between the topographical distribution of relapse symptoms and deficits from subsequent progression. In the topographical model, progression became apparent 7.75 years earlier than SPMS was diagnosed in practice.
We demonstrate the model's utility in depicting patients' disease topography as the loci of clinical progression. This could allow for earlier recognition of progressive disease by identifying leading indicators of progression.
运用多发性硬化症（MS）的地形学模型评估一个纵向队列，我们（1）检验重现假说，即假设患者的“疾病地形学”可预测残疾累积的临床模式；（2）识别疾病进展的主要指标。
对10例从复发缓解型MS转变为继发进展型MS（SPMS）的患者进行评估。分析了复发时、SPMS诊断时及最近一次就诊时的神经学检查结果。记录功能系统（FS）、部位/单侧性及恢复情况。锥体/运动系统是目标FS，用于评估复发和SPMS时间点的症状单侧性及严重程度。使用地形学模型软件绘制每位患者的临床病程。
队列中80%为女性，诊断时年龄为31.6±8.6岁，平均随访23.8±8.8年，SPMS前平均复发3.1次。83.3±0.2%的复发症状在转变为SPMS时出现，在最近一次就诊时增至91.0±0.2%。这表明复发症状的地形学分布与后续进展导致的缺陷之间具有一致性。在地形学模型中，进展在实际诊断出SPMS的7.75年前就已显现。
我们证明了该模型在将患者的疾病地形学描绘为临床进展位点方面的实用性。这可能通过识别进展的主要指标实现对进展性疾病的更早识别。
