S. MazzuccoS. FerrariC. MezzinaG. TomelleriL. BertolasiN. Rizzuto Hyperpyrexia-triggered relapses in an unusual case of ataxic chronic inflammatory demyelinating polyradiculoneuropathy

The ataxic form of chronic inflammatory demyelinating polyradiculoneuropathy (ataxic-CIDP) has been recently described as a subtype of chronic ataxic neu- ropathy, distinguished by steroid responsiveness and rela- tive preservation of myelinated fibres at sural nerve biop- sy. We report on a case of progressive, predominantly sen- sory, steroid-responsive neuropathy with clinical, labora- tory, electrophysiological and pathological features of this uncommon form of CIDP. Moreover, the present case dis- plays peculiar hyperpyrexia-triggered relapses leading to transitory severe tetraparesis, bilateral facial drooping, dysphonia, dysphagia and dyspnoea, which leave clini- cians with some unresolved questions.