SX05
Chronic Lymphocytic Inflammation with Pontine Perivascular Enhancement Responsive to Steroids: A Case Report with 5 Year Follow-up
Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) is a rare inflammatory disorder involving brainstem, pons in particular, characterized by a predominant T-cell pathology and responsiveness to immunosuppression with glucocorticosteroids (GCS). Characteristic MRI features include ‘punctate’ and/or ‘curvilinear’ gadolinium enhancement ‘peppering’ the pons. Similar lesions can be seen in cerebellum, midbrain, medulla, spinal cord, deep grey matter, and cerebral white matter. Early and vigorous pulse GCS with maintenance immunosuppressive therapy seem helpful.
Objectives:
Describe a five year clinical course of a youngest with CLIPPERS
Methods:
Case report
Results:
10 year old girl presented with progressive lower extremity weakness, unsteady gait, and diplopia. Neurological exam revealed restricted abduction of both eyes, distal > proximal lower extremity weakness, and pyramidal tract signs. MRI showed hyper-intensities with enhancement in the midbrain, pons, medulla, cerebellar peduncles, and optic chiasm and diffuse nodular enhancement throughout the cervical and thoracic spinal cord. Lesion biopsies were compatible with CLIPPERS. Pulse intravenous methylprednisolone followed by oral prednisolone resulted in clinical improvement. She was treated with prednisolone again three years later for exacerbation of the neurologic deficits. Subsequent MRI showed interval resolution of scattered nodular enhancement, and atrophy of the pons, midbrain and cerebellum. She regained ambulatory function. Prednisolone was replaced by methotrexate due to intolerable side effects. Her neurological function maintained with no relapses on methotrexate for a year albeit radiological relapse with new multiple punctate enhancing lesions within the periventricular white matter and brainstem. Four months later, she demonstrated new symptoms of upper extremity weakness, dysarthria, and dysphagia which coincided with MRI progression of the nodular enhancing lesions throughout the brain. Clinical improvement achieved again with pulse steroids.
Conclusions:
Our patient’s clinical course was progressing but remitting to pulse steroid treatment. Optimal therapeutic proceedings and precise pathogenesis remain to be investigated