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Case No. 639
2022-11-01~2022-11-30
출제자 : 김슬기
Hit : 418
| Period | 2022-11-01~2022-11-30 |
|---|---|
| Diagnosis | CLIPPERS (Chronic Lymphocytic Inflammation with Pontine Perivascular Enhancement Responsive to Steroids) |
| Gender |
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| Age | 21 |
| Clinical information | 21/M C.C>Truncal ataxia (5 days ago), Binocular horizontal diplopia (2 months ago), Left side hypesthesia (2 months ago) 진단은? |
| Discussion | 진단: CLIPPERS (Chronic Lymphocytic Inflammation with Pontine Perivascular Enhancement Responsive to Steroids) 고찰 • Rare disorder first described in 2010 as a distinct form of brainstem encephalitis with predominant T – cell pathology • Pathology Dense lymphocytic inflammation with perivascular predominance and parenchymal diffuse infiltration T-cells predominating infiltration (CD4 > CD8) with variable macrophage component • Clinical presentation Subacute, wide variety of brainstem symptoms Cognitively intact and do not suffer from other signs of inflammation (e.g. fever or sweats) Often, relapsing-remitting course without treatment • MRI Unique, characterized by multiple punctate, patchy and linear regions of contrast enhancement relatively confined to the pons (“peppering the pons”) May extend laterally into cerebellar peduncels or hemispehres, caudally into medulla, spinal cord, rostrally into midbrain. Faint, patchy or speckled punctate hyperintensities on T2/FLAIR Relatively little edema / minimal or no mass effect |
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