Multiple sclerosis and related disorders · 2017
Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS): A pediatric case report with six year follow-up.
Клинический случайСтатьяНазвание и аннотация: Перевод в очереди
Обработка статьи · этапы, ошибки и управление
Загрузка состояния…
Ошибки и ограничения
История · было → стало
Состояние обновляется каждые 5 секунд, пока блок открыт. Процент показывается только там, где известен объём работы. Пауза останавливает продолжение этапа; текущий запрос к источнику может завершиться.
Veerapandiyan Aravindhan, Chaudhari Amit, Deo Prabhav, Ming Xue
Аннотация
BACKGROUND: Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) is an inflammatory disorder involving brainstem, pons in particular, characterized by a predominant T-cell pathology and responsiveness to glucocorticosteroids. We describe a difficult 6 year course of this rare syndrome in a 10-year-old girl.
CASE REPORT: Our patient presented with diplopia, spastic paraparesis, and unsteady gait. MRI showed characteristic punctate hyper-intensities with enhancement in the brain stem, cerebellar peduncles, and optic chiasm and diffuse nodular enhancement throughout the cervical and thoracic spinal cord. Brain biopsy revealed perivascular inflammatory lesions compatible with CLIPPERS. Pulse intravenous methylprednisolone followed by oral prednisolone resulted in significant clinical improvement. She had multiple clinical relapses associated with new brain and/or spinal cord lesions despite sequential maintenance methotrexate and rituximab with low dose steroids. Each relapse responded to pulse steroids.
CONCLUSION: Her overall clinical course was progressive but responsive to pulse steroids. Long-term treatment remains challenging.
CASE REPORT: Our patient presented with diplopia, spastic paraparesis, and unsteady gait. MRI showed characteristic punctate hyper-intensities with enhancement in the brain stem, cerebellar peduncles, and optic chiasm and diffuse nodular enhancement throughout the cervical and thoracic spinal cord. Brain biopsy revealed perivascular inflammatory lesions compatible with CLIPPERS. Pulse intravenous methylprednisolone followed by oral prednisolone resulted in significant clinical improvement. She had multiple clinical relapses associated with new brain and/or spinal cord lesions despite sequential maintenance methotrexate and rituximab with low dose steroids. Each relapse responded to pulse steroids.
CONCLUSION: Her overall clinical course was progressive but responsive to pulse steroids. Long-term treatment remains challenging.