Sarcoid neuropathy

Summary In a case of subacute sensory and motor polyneuropathy associated with sarcoidosis, multiple epineurial and endoneurial granulomas were demonstrated in a sural nerve. Neighbouring nerve fibres were displaced by the granulomas and some were undergoing axonal degeneration. Ultrastructural and teased fibre studies showed axonal atrophy and degeneration with secondary demyelination. Histochemical studies indicated the presence of HLA-DR antigen on epithelioid cells in the granulomas. A non-specific inflammatory process in the nerve does not cause significant primary demyelination.     

Sarcoid peripheral neuropathy.

We studied 10 patients with sarcoidosis and peripheral neuropathy. Six had a subacute or chronic axonal sensorimotor neuropathy without cranial neuropathy, beginning months to years after established systemic sarcoidosis. One patient had severe enough diaphragmatic weakness to require mechanical ventilation. Four patients had atypical neuropathies: acute Guillain-Barré syndrome, mononeuritis multiplex, unilateral lumbosacral plexopathy, and a purely sensory neuropathy, all before systemic sarcoidosis became evident, and all except one had cranial nerve abnormalities. Autopsy in one patient with sensorimotor neuropathy showed only scattered foci of lymphocytes in spinal roots and ganglia with nerve fiber loss.     

Sarcoid polyneuropathy responsive to intravenous immunoglobulin

We describe a 38-year-old woman with a predominantly sensory axonal polyneuropathy in whom a nerve biopsy demonstrated sarcoid granulomas. The neuropathy did not respond to oral steroid therapy but there was a rapid and repeated response to intravenous immunoglobulin, which gradually diminished over subsequent treatments, but remained beneficial. The systemic sarcoidosis remained active.     

Sarcoid myopathy presenting with diaphragm weakness

Sarcoidosis involving muscle occurs frequently, but it is infrequently symptomatic. The clinical, electromyographic, and histologic features of sarcoidosis involving muscle in a 63-year-old woman presenting with diaphragm weakness are described. An electromyogram revealed widespread myotonia and an inflammatory myopathic process, suggestive of adult-onset acid maltase deficiency disease. Muscle biopsy showed noncaseating granulomas consistent with sarcoidosis. Clinical improvement followed the initiation of oral prednisone therapy. This case illustrates that muscular sarcoidosis may mimic adult-onset acid maltase deficiency in both its clinical and electromyographic features. © 1993 John Wiley & Sons, Inc.     

Myelopathy in Marfan''s syndrome

A patient with Marfan's syndrome and a myelopathy is reported, and the association of multiple spinal arachnoid cysts noted. It is proposed that the basic connective tissue defect in Marfan's syndrome may predispose to the formation of arachnoid diverticuli and that in this case spinal cord damage was the sequel.     

Sarcoid polyneuropathy: a histologically proved case

A 58-year-old patient had subacute polyneuropathy. Electrophysiological studies showed inexcitability of the distal portion of the peroneal and posterior tibial nerves and minimal reduction in amplitude of the muscle potential with minimal slowing in motor nerve conduction velocity. The sural nerve biopsy showed multiple sarcoid granulomas in the epineurial and perineurial spaces, periangiitis and panangiitis, and axonal degeneration. Remarkable clinical improvement followed corticosteroid treatment.     

Myelopathy induced by lactic acid

Summary This study defines the conditions required to provoke myelopathic changes by dripping lactic acid onto the surgically exposed spinal cord of adult male rats. A severe necrotizing myelopathy was observed after 24 h, principally in the posterior half of the cord at the level of lactic acid (pH 1.8) application. A profound early effect on small blood vessel walls, appearing nerotic after 30 min to 2 h, was identified. Nerve fiber alterations Iaxonal stasis, granular axoplasm, axonal calcification, and vesicular myelin), identical to those appearing early in the myelopathies of trauma and calcium toxicity, were apparent. However, the pathogenesis of these alterations in this model remains unclear, because of the vascular events and the presumed alterations of calcium metabolism by the acid. Further studies are required to elucidate the precise mechanisms of these important reactions of myelinated axons to the injuries provoked by acid, calcium, and trauma.Supported by NIH grant No. NS11066. This work was presented in part at the 60th Annual Meeting of the American Association of Neuropathologists, San Diego, CA; June 1984 (J Neuropathol Exp Neurol 43:324, 1984)     

铜缺乏性脊髓病临床分析

目的:探讨铜缺乏性脊髓病(CDM)的病因、临床表现及治疗方法。方法:回顾性分析7例铜缺乏性脊髓病患者的临床表现、辅助检查及治疗效果。结果:7例患者均有后索、侧索和周围神经损害,血清铜浓度降低;7例肌电图提示周围神经损害,体感诱发电位异常;2例MRI有胸髓改变。补铜治疗有效。结论:铜缺乏性脊髓病是以铜缺乏导致的后索、侧索及周围神经损害为主的疾病,早期诊断是治疗CDM的关键。