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Case No. 354
2014-11-01~2014-11-30
출제자 : 김은희
Hit : 335
| Period | 2014-11-01~2014-11-30 |
|---|---|
| Diagnosis | Neurodegenerative LCH |
| Clinical information | 45/F 20년전 Diabetes insipidus 증상, 이후 Langerhans cell histiocytosis로 진단 2년전부터 insidious onset of gait disturbance, dysarthria, ataxia 발생 |
| Discussion | Neurodegenerative LCH 1. Langerhans cell histiocytosis (LCH): a rare systemic granulomatous disease of the dendritic system with a variable clinical course that may be encountered at any age 2. Neurodegenerative LCH: MR SI abnormalities in the cerebellum, pons, BG pathology: neuronal loss and axonal degeneration along with T cell inflammation 3. Imaging features - Cerebellum gray matter (dentate nu): T2 high, T1 low SI - Pons: T2 high SI in the pontine tegmentum or pontine pyramidal tracts - BG: T1 high SI, T2 variable SI 4. Neurologic Sx: subtle deficit~profound ataxia, dysarthria 5. The increase of SI abnormalities in the cerebellum and BG does not correlate with a clinical deterioration AJNR 28:1022-28, Jun-Jul 2007 |
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