Probable chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids syndrome: management with corticosteroids and intravenous immunoglobulin-a case report

Abstract

BACKGROUND: Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids is a rare, subacute inflammatory disorder of the central nervous system with an unknown etiology. It is characterized by distinct clinical (diplopia, ataxia, dysarthria, and altered facial sensation), radiological (punctiform lesions detected on magnetic resonance imaging), and histopathological (predominantly perivascular lymphocytic infiltration, mainly affecting the pons and cerebellum) features. The condition typically demonstrates a favorable response to corticosteroid therapy. CASE PRESENTATION: We report the case of a 54-year-old Caucasian European male who presented with clinical and radiological findings consistent with chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids. The patient attended the emergency department at his referral hospital following a 1-month history of progressive neurological symptoms, including diplopia, blurred vision, facial paresis, and gait disturbance attributed to lower limb weakness. Given the clinical presentation and magnetic resonance imaging findings, treatment with oral prednisolone (70 mg/day) was initiated. At 2 weeks post-treatment initiation, the patient exhibited significant clinical improvement, with complete resolution of neurological symptoms. A follow-up brain magnetic resonance imaging scan, performed at the referral center 1 month after starting corticosteroid therapy, demonstrated a reduction in both the size and number of hyperintense lesions in the brainstem on T2-weighted sequences, along with resolution of contrast enhancement. These radiological findings indicated a favorable therapeutic response to chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids treatment. At the 1-month follow-up, the patient remained asymptomatic, leading to a gradual tapering of corticosteroid therapy in light of both clinical and radiological improvement. CONCLUSION: Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids is a rare neurological disorder that typically presents with ataxic gait, diplopia, and dysarthria. Diagnosis is based on a combination of clinical features, neuroimaging, and histopathology, although brain biopsy is not always feasible. The cornerstone of treatment is immunosuppressive therapy, primarily with corticosteroids, often supplemented with other immunosuppressive agents to prevent relapse.