Mahan Mark A, Howe B Matthew, Amrami Kimberly K, Spinner Robert J
Barrow Neurological Institute, Phoenix, AZ, USA.
Skeletal Radiol. 2014 Jul;43(7):963-8. doi: 10.1007/s00256-014-1871-0. Epub 2014 Apr 12.
Lipomatosis of nerve (LN) is a condition of massive peripheral nerve enlargement frequently associated with hypertrophy within the distribution of the nerve, and most commonly affecting the distal limbs. We sought to understand if LN of the lumbosacral plexus would be associated with the trophic effects of LN on surrounding tissue within the pelvis, which may be clinically occult, but present on MRI.
Fifty-one cases of LN, confirmed by pathology or pathognomonic appearance on MRI, were reviewed. Patients with LN of the sciatic nerve were investigated for radiological signs suggestive of overgrowth.
Five patients had involvement of the sciatic nerve, 4 of whom had MR imaging of the pelvis. Three patients had LN involving the lumbosacral plexus, and one patient had isolated involvement of the sciatic nerve. All patients with involvement of the lumbosacral plexus demonstrated previously unrecognized evidence of nerve territory overgrowth in the pelvis, including: LN, profound adipose proliferation, muscle atrophy and fatty infiltration, and bone hypertrophy and ankylosis. The patient with LN involving the intrapelvic sciatic nerve, but not the lumbosacral plexus did not demonstrate any radiological evidence of pelvic overgrowth.
LN is broader in anatomical reach than previously understood. Proximal plexal innervation may be involved, with a consequent effect on axial skeleton and intrapelvic structures.
神经脂肪过多症(LN)是一种周围神经大量增粗的疾病,常伴有神经分布区域内的肥大,最常见于远端肢体。我们试图了解腰骶丛的LN是否与LN对骨盆内周围组织的营养作用相关,这在临床上可能隐匿,但在磁共振成像(MRI)上可见。
回顾了51例经病理或MRI特征性表现确诊的LN病例。对坐骨神经LN患者进行了影像学检查,以寻找提示过度生长的征象。
5例患者坐骨神经受累,其中4例进行了骨盆MRI检查。3例患者腰骶丛受累,1例患者孤立性坐骨神经受累。所有腰骶丛受累患者均显示出骨盆内神经分布区域先前未被认识的过度生长证据,包括:LN、大量脂肪增生、肌肉萎缩和脂肪浸润,以及骨质肥大和关节强直。仅盆腔内坐骨神经受累而腰骶丛未受累的患者未显示出任何骨盆过度生长的影像学证据。
LN在解剖学上的累及范围比先前认为的更广。近端丛神经支配可能受累,从而对中轴骨骼和盆腔内结构产生影响。