Averbuch-Heller L, Steiner I, Abramsky O
Department of Neurology, Hadassah University Hospital, Jerusalem, Israel.
Arch Neurol. 1992 Dec;49(12):1292-5. doi: 10.1001/archneur.1992.00530360094024.
Neurologic involvement in progressive systemic sclerosis is considered uncommon. We retrospectively examined the prevalence and nature of neurologic complications in 50 patients with progressive systemic sclerosis. In 20 (40%), neurologic abnormalities were detected, with a total of 28 neurologic manifestations. All levels of the central and peripheral nervous system were affected: muscle (22%), peripheral nerve (18%), spinal cord (8%), and brain (6%). Of note were the presence of myelopathy in four patients and inclusion-body myositis in two. In 10 patients (20%), no definable cause of the neurologic dysfunction could be identified, apart from progressive systemic sclerosis. Thus, neurologic presentations of progressive systemic sclerosis are much more common than previously reported and may be due to direct involvement of the nervous system by a primary pathologic process in a significant number of patients.
进行性系统性硬化症的神经系统受累被认为并不常见。我们回顾性研究了50例进行性系统性硬化症患者神经系统并发症的患病率和性质。20例（40%）患者检测到神经异常，共有28种神经表现。中枢神经系统和周围神经系统的各个层面均受累：肌肉（22%）、周围神经（18%）、脊髓（8%）和脑（6%）。值得注意的是，4例患者存在脊髓病，2例患者存在包涵体肌炎。10例患者（20%）除进行性系统性硬化症外，未发现明确的神经功能障碍病因。因此，进行性系统性硬化症的神经表现比先前报道的更为常见，在相当数量的患者中可能是由于原发性病理过程直接累及神经系统所致。
