Mazzucco S, Ferrari S, Mezzina C, Tomelleri G, Bertolasi L, Rizzuto N
Department of Neurological Sciences and Vision, Section of Clinical Neurology, University of Verona, Policlinico "G.B. Rossi", Piazzale L.A. Scuro 10, I-37134, Verona, Italy.
Neurol Sci. 2006 Jul;27(3):176-9. doi: 10.1007/s10072-006-0664-1.
The ataxic form of chronic inflammatory demyelinating polyradiculoneuropathy (ataxic-CIDP) has been recently described as a subtype of chronic ataxic neuropathy, distinguished by steroid responsiveness and relative preservation of myelinated fibres at sural nerve biopsy. We report on a case of progressive, predominantly sensory, steroid-responsive neuropathy with clinical, laboratory, electrophysiological and pathological features of this uncommon form of CIDP. Moreover, the present case displays peculiar hyperpyrexia-triggered relapses leading to transitory severe tetraparesis, bilateral facial drooping, dysphonia, dysphagia and dyspnoea, which leave clinicians with some unresolved questions.
慢性炎症性脱髓鞘性多发性神经根神经病的共济失调型（ataxic-CIDP）最近被描述为慢性共济失调性神经病的一种亚型，其特征为对类固醇有反应，且在腓肠神经活检时髓鞘化纤维相对保留。我们报告一例进行性、以感觉为主、对类固醇有反应的神经病病例，具有这种罕见形式CIDP的临床、实验室、电生理和病理特征。此外，本例表现出特殊的高热触发复发，导致短暂性严重四肢轻瘫、双侧面部下垂、声音嘶哑、吞咽困难和呼吸困难，这给临床医生留下了一些未解决的问题。
