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Case No. 543
2020-02-01~2020-02-29
출제자 : 배윤정
Hit : 327
| Period | 2020-02-01~2020-02-29 |
|---|---|
| Diagnosis | CLIPPERS (Chronic Lymphocytic Inflammation with Pontine Perivascular Enhancement Responsive to Steroid) |
| Clinical information | M/61Y Symptom: Ataxia, gait disturbance, dysarthria ** Symptoms and the lesions on the MRI were completely disappeared after steroid therapy. What is your diagnosis? |
| Discussion | CLIPPERS (Chronic Lymphocytic Inflammation with Pontine Perivascular Enhancement Responsive to Steroid) Definitions: -Recently described inflammatory CNS disorder, predominantly involving brainstem, adjacent rhombencephalic structures -Clinical, imaging response to glucocorticosteroids (Key to diagnose) Clinical presentations: -Mostly variable symptoms related to brainstem and cerebellar involvement, including ataxia, dysarthria, face tingling, vertigo, etc. -May have symptoms referable to long tract affections and/or spinal cord syndrome, pseudobulbar affections, cognitive dysfunction, or headache, etc. -But, generally, cognitively intact without signs of inflammation (e.g. fever, sweat) MR Imaging findings: -Hallmark feature: multiple punctate and/or curvilinear enhancement peppering the pons, with or without spread into the cerebellar peduncles and the cerebellum -Faint on T2-WI and FLAIR -Relatively little edema or positive mass effect -No angiographic changes Treatment and prognosis: -Respond rapidly to glucocorticosteroid -But, has a tendency to relapse if immunosuppression is ceased -Hydroxychloroquine has been reported to induce and maintain remission of symptoms. |
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