Challenging differential diagnosis, Unusual or unexpected effect of treatment, Unexpected drug reaction , Rare disease, Educational Purpose (only if useful for a systematic review or synthesis)
María Rico, Javier Villafani, Alberto Tuñón, Valentín Mateos, Pedro Oliva-Nacarino
Department of Neurology, Hospital de Cabueñes, Gijón, Spain
Am J Case Rep 2016; 17:47-50
Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) is a recently described inflammatory disease of the central nervous system, distinguished by brainstem- and spinal cord-centered lesions with a characteristic contrast enhancement on MRI, a lymphocytic perivascular infiltrate on pathological exam, and a dramatic response to and dependence on steroids therapy. Since its initial description in 2010, different glucocorticoid-sparing agents, mostly immunosuppressant drugs, have been used to minimize the dosage, but these therapies also carry the risk of important secondary effects. We present the first reported case of CLIPPERS treated with interferon beta 1a as add-on therapy.
CASE REPORT: A previously healthy 31-year-old man presented with gait ataxia and dysarthria. MRI showed pons-centered hyperintense patchy lesions on T2-weighted images. Additional tests ruled out other possible diagnoses and symptoms reversed with intravenous methylprednisolone. Over the years the patient presented with several episodes of deterioration each year, which were partly reversed with glucocorticoid therapy, but leaving him with growing sequelae. Four years after the initial event, treatment with interferon-beta-1a was initiated, achieving reduced frequency of the relapses to 1 every 4 years, which were no longer associated to increasing disability. This allowed reducing glucocorticoids to 30 mg of Deflazacort every other day.
CONCLUSIONS: Interferon beta-1a could be an alternative to corticosteroid-combined therapy in CLIPPERS and its more benign profile of secondary effects compared to immunosuppressants could make it an attractive choice.
Keywords: Adjuvants, Immunologic - therapeutic use, Adult, Anti-Inflammatory Agents - administration & dosage, Encephalomyelitis - drug therapy, Humans, Interferon beta-1a - therapeutic use, Lymphatic Diseases - drug therapy, Magnetic Resonance Imaging, Male, Pons - pathology, Pregnenediones - administration & dosage