Dados do Trabalho

Título

STEROID-RESPONSIVE PONTINE PERIVASCULAR ENHANCEMENT DUE TO CHRONIC LYMPHOCYTIC INFLAMMATION (CLIPPERS) IN A YOUNG PATIENT AS A DIFFERENTIAL DIAGNOSIS WITH INFLAMMATORY CENTRAL NERVOUS SYSTEM DISEASES: A UNCOMMUN REPORT CASE.

RESUMO

Case: M.L.N.P, female, 22 y, admitted to a neurology ER reporting a holocranial headache associated with 3 weeks of intermittent rotational vertigo, diplopia and strabismus. On physical exam observed sixth cranial nerve ophthalmoplegia leading to right eye convergent strabismus and horizontal nystagmus, rapid phase to the left side, ataxia in left hemibody with dysmetria and ataxic gait. Hypothesis of CNS inflammatory disease was evented and brain MRI with positive signal anomaly in the right nucleocapsular region, extending to midbrain, pons, middle cerebellar peduncles and left cerebellar hemisphere, with punctate enhancement was identified, suggesting CLIPPERS diagnosis. Other labs and supplementary tests were normal results. Performed intravenous therapy with methylprednisolone 1g/d for 5 days and improvement mainly strabismus was identified. Prednisone 20mg daily was prescribed, in accordance with literature findings just until Rituximab as a Corticosteroid-Sparing Therapy (CST) starts. Outpatient care 30 days later, demonstrated no neurological deficits, with remission of previous symptoms and new MRI identified no signs perivascular enhancement. CST was introduced 4 months later, without adverse events or new episodes of inflammatory outbreaks at last 6 month. Discussion: CLIPPERS has become a crucial differential diagnosis of CSN immune-mediated diseases. Its pathophysiology, characterized by autoreactive lymphocytes-mediated perivascular inflammation, remains unclear. In Brazil, only few cases of female patients with this syndrome has been reported since it classically affects men, with a wide range of age distribution. This clinical case is relevant to Brazilian neurological may the use CST, so far, has been scarely described. Conclusion: Diagnosis of CLIPPERS was established using clinical and complementary criteria, described by Pittock et al, and treatment is successfully corelated by symptoms remission. This case might contributes to the knowledge that CLIPPERS is a differential diagnosis of immune-mediated CNS diseases, understanding follow-up and therapy used with positive results. Cases estimeteds in the world is uncertain and a literature review founded about 40 cases, predominant in older men, where reports on female patients is uncommon.

Palavras Chave

CLIPPERS; INFLAMATORY DISEASE; CENTRAL NERVOUS SYSTEM.