Hyperpyrexia-triggered relapses in an unusual case of ataxic chronic inflammatory demyelinating polyradiculoneuropathy.

Abstract

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.

• Authors: S Mazzucco, S Ferrari, C Mezzina, G Tomelleri, L Bertolasi, N Rizzuto
• Journal: Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology (Neurol Sci) Vol. 27 Issue 3 Pg. 176-9 (Jul 2006) ISSN: 1590-1874 [Print] Italy
• PMID: 16897630 (Publication Type: Case Reports, Journal Article)

Chemical References

• Anti-Inflammatory Agents
• Immunoglobulins, Intravenous
• Immunologic Factors
• Prednisone

Topics

• Anti-Inflammatory Agents (therapeutic use)
• Ataxia (etiology)
• Electromyography
• Fever (complications)
• Humans
• Immunoglobulins, Intravenous (therapeutic use)
• Immunologic Factors (therapeutic use)
• Male
• Middle Aged
• Polyradiculoneuropathy, Chronic Inflammatory Demyelinating (pathology, physiopathology, prevention & control)
• Prednisone (therapeutic use)
• Recurrence
• Sural Nerve (pathology)