Relapsing polychondritis and lymphocytic meningitis with varied neurological symptoms.

Relapsing polychondritis and lymphocytic meningitis with varied neurological symptoms.

Rev Bras Reumatol. 2016 Feb 5;

Authors: Ducci RD, Germiniani FM, Czecko LE, Paiva ES, Teive HA

Abstract
BACKGROUND: Relapsing polychondritis is a rare multisystem disease characterized by bilateral auricular and nasal chondritis and vestibular involvement, additionally affecting various other organs/systems. However, Central Nervous System involvement has only been reported in a few instances.
OBJECTIVE: To report the case of a patient with relapsing polychondritis and several neurological manifestations.
CASE REPORT: We report the case of a 69 year-old male with a 20-day history of ataxia, paraparesis, tinnitus, vertigo and confusion. Two months prior to his admission he started with bilateral auricular chondritis and arthritis of metacarpophalangeal joints and ankles. He had been previously seen at another hospital, where he had been treated for herpetic encephalitis, with improvement of confusion. On examination he had nystagmus, rigidity of upper limbs, paraparesis, absent reflexes, tactile hypoesthesia, dysmetric movements, gross postural and action tremor, bradykinesia and truncal ataxia. He also had swelling and a purplish erythema of both ear lobes and arthritis in the metacarpophalangeal joints of the right hand. Brain and cervical MRI disclosed a mild thickening of the dura. A lumbar puncture confirmed the presence of elevated leukocytes and laboratory exams disclosed augmented inflammatory activity. A diagnosis of relapsing polychondritis was made based on the association of chondritis, arthritis and vestibular ataxia with predominant neurological symptoms. Following a course of Prednisone 1mg/kg/d there was major improvement of chondritis, arthritis, ataxia and paraparesis, but the tremor remained unchanged.
CONCLUSION: Relapsing polychondritis is a challenging disease that can rarely manifest with varied neurological symptoms; recognition is extremely important, because high-dose corticosteroids should be used as soon as possible in suspected cases.

PMID: 26995500 [PubMed - as supplied by publisher]

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