Thomas P K, Claus D, Jaspert A, Workman J M, King R H, Larner A J, Anderson M, Emerson J A, Ferguson I T
Department of Clinical Neurosciences, Royal Free Hospital School of Medicine, London, UK.
Brain. 1996 Jun;119 ( Pt 3):765-74. doi: 10.1093/brain/119.3.765.
Observations are presented on nine selected patients with chronic upper limb demyelinating neuropathy to illustrate the range of manifestations that may be observed. In three, the involvement was purely motor, in five, mixed motor and sensory and, in one, virtually purely sensory; in seven the symptoms were unilateral and in two bilateral. The presence of reduced nerve conduction velocity and conduction block and the response to treatment in seven of the cases indicate that they represented examples of chronic inflammatory demyelinating polyneuropathy (CIDP) with focal involvement. This was confirmed by nerve biopsy in two cases. The presentation in one patient was accompanied by forearm swelling initially suspected of being a tumour but shown to be due to muscle hypertrophy. This was probably the consequence of recurrent muscle cramps and fasciculation and possibly neuromyotonia. The patient with predominant sensory involvement restricted to the upper limbs demonstrates that sensory CIDP can present focally. In one patient with monomelic motor and sensory involvement, nerve biopsy showed multifocal areas of hypertrophic demyelinating neuropathy distally in the ulnar nerve without inflammatory infiltration. This patient failed to respond to therapy. Response in the others was satisfactory, although one patient with a monomelic motor neuropathy showed a severe deterioration after being given corticosteroids; he subsequently improved with intravenous human immunoglobulin therapy.
本文报告了对9例慢性上肢脱髓鞘性神经病患者的观察结果，以说明可能观察到的一系列表现。其中3例仅累及运动神经，5例运动和感觉神经均受累，1例几乎仅累及感觉神经；7例症状为单侧，2例为双侧。7例患者存在神经传导速度减慢和传导阻滞以及对治疗的反应，表明它们代表了伴有局灶性受累的慢性炎症性脱髓鞘性多发性神经病（CIDP）的病例。2例经神经活检证实。1例患者最初伴有前臂肿胀，怀疑是肿瘤，但后来发现是肌肉肥大所致。这可能是反复出现肌肉痉挛和肌束震颤以及可能的神经性肌强直的结果。仅上肢主要累及感觉神经的患者表明，感觉性CIDP可呈局灶性表现。1例单肢运动和感觉神经受累的患者，神经活检显示尺神经远端有多处肥厚性脱髓鞘性神经病区域，无炎性浸润。该患者对治疗无反应。其他患者的反应令人满意，尽管1例单肢运动神经病患者在给予皮质类固醇后病情严重恶化；随后他通过静脉注射人免疫球蛋白治疗有所改善。
