| Period | 2020-10-01~2020-10-31 |
|---|---|
| Diagnosis | Adult-onset leukoencephalopathy with axonal spheroids and pigmented glia (ALSP) |
| Clinical information | 42세/남자 요양병원 입원 중 GTC type seizure를 보여 응급실 내원함. 가능성이 높은 진단은? |
| Discussion | • A rare inherited autosomal dominant disease characterized by an adult-onset leukodystrophy • Image finding - Bilateral, asymmetric patchy or confluent areas of subcortical and deep white matter signal change (predominant involvement of frontal and parietal lobes). - Corticospinal tract involvement is common. - Progression leads to a severe atrophy of the supratentorial white matter. - Persistent diffusion restriction spots in white matter are relatively characteristic finding. |
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