| Period | 2021-11-01~2021-11-30 |
|---|---|
| Diagnosis | CLIPPERS (Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids) |
| Gender |
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| Age | 21 |
| Clinical information | 21세 남자 1달전부터 지속된 sensory disturbance, binocular horizontal diplopia를 주소로 내원. 증상은 steroid 치료 후 호전되었다가 이후 악화를 반복하였다. 가장 가능성 높은 진단은? |
| Discussion | CLIPPERS (Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids) Clinical issues - Subacute brainstem symptoms such as gait ataxia, diplopia, facial paresthesia, and nystagmus - mean age at onset: 40-50 years, minor male predominance - CNS symptoms responsive to steroid therapy Radiology features - T2: faint, patchy or speckled punctate hyperintensities - minimal or no mass effect - T1 C+ (Gd): punctate/curvilinear enhancing foci peppering pons and cerebellum - Location: predominantly pons, cerebellum - may extend to medulla/spinal cord or midbrain - 60%: have CNS lesions outside pontocerebellar region (basal ganglia, hemispheric white matter, cortex) |
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Correct Answer
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