Mahan Mark A, Niederhauser Blake D, Amrami Kimberly K, Spinner Robert J
Barrow Neurological Institute, Phoenix, Arizona, USA.
Department of Radiology, Mayo Clinic, Rochester, Minnesota, USA.
World Neurosurg. 2014 Sep-Oct;82(3-4):492-9. doi: 10.1016/j.wneu.2013.09.005. Epub 2013 Sep 10.
Lipomatosis of nerve (LN) is a condition of massive peripheral nerve enlargement due to proliferation of fibrous and adipose tissue within the nerve, the natural history of which is currently unknown. We measured the pattern of growth in individuals with long-term radiologic follow-up.
Review of the searchable records for LN at our institution found 52 patients, confirmed by pathology or pathognomic appearance on MRI. Ten patients had serial MRI of the same anatomic region for more than 2 years of clinical follow-up. Volumetric analysis was performed using regions of interest on serially imaged segments of affected nerves. Adjustment for skeletal growth was performed for pediatric patients.
LN enlarged in 7 of 10 individuals, often both longitudinally along the nerve and in cross-sectional volume. Regarding cross-sectional volume, 2 of the 10 patients demonstrated volume growth more than doubling and 5 additional patients had a >20% increase in nerve volume; the remaining 3 patients were quiescent, where change in the nerve volume was within the error range of volumetric analysis. All cases with growth remained >20% after adjustment for skeletal growth. Five of 10 individuals had longitudinal extension, even with correction for skeletal growth. More significant growth was noted in younger patients (P=0.02). Growth rates more than 5% per year correlated with surgery, without statistical significance in this small population (P=0.14).
Serial MRI reveals progressive enlargement of LN. The rate of growth was more profound in youth, but also occurred in early adulthood.
神经脂肪增多症（LN）是一种由于神经内纤维和脂肪组织增生导致周围神经大量增粗的病症，其自然病程目前尚不清楚。我们对长期接受影像学随访的患者的生长模式进行了测量。
回顾我们机构中可检索到的LN记录，发现52例患者，经病理或MRI特征性表现确诊。10例患者对同一解剖区域进行了连续两年多的临床随访MRI检查。使用感兴趣区域对受累神经的连续成像节段进行体积分析。对儿科患者进行了骨骼生长调整。
10例患者中有7例LN增大，通常沿神经纵向和横截面积均增大。关于横截面积，10例患者中有2例体积增长超过一倍，另有5例患者神经体积增加>20%；其余3例患者病情静止，神经体积变化在体积分析误差范围内。所有生长病例在调整骨骼生长后仍>20%。10例患者中有5例有纵向延伸，即使校正了骨骼生长也是如此。较年轻患者生长更显著（P=0.02）。每年生长率超过5%与手术相关，在这个小样本中无统计学意义（P=0.14）。
连续MRI显示LN逐渐增大。生长速度在青少年中更明显，但在成年早期也会发生。
