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 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.
Mazzucco
S
S
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. sara.mazzucco@univr.it
Ferrari
S
S
Mezzina
C
C
Tomelleri
G
G
Bertolasi
L
L
Rizzuto
N
N
D000893:Anti-Inflammatory Agents / Q000627:therapeutic use; D001259:Ataxia / Q000209:etiology; D004576:Electromyography; D005334:Fever / Q000150:complications*; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use; D007155:Immunologic Factors / Q000627:therapeutic use; D008297:Male; D008875:Middle Aged; D020277:Polyradiculoneuropathy, Chronic Inflammatory Demyelinating / Q000473:pathology / Q000503:physiopathology* / Q000517:prevention & control; D011241:Prednisone / Q000627:therapeutic use; D012008:Recurrence; D013497:Sural Nerve / Q000473:pathology
D000893:Anti-Inflammatory Agents; D016756:Immunoglobulins, Intravenous; D007155:Immunologic Factors; D011241:Prednisone