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Washington University Experience | MYELIN (NON-IMMUNE MEDIATED) | Metachromatic Leukodystrophy | 4A0 Case 4 History
Case 4 History ---- The patient was diagnosed with CIDP at 4 years of age when she presented with weakness, difficulty walking, headaches, increased falls and muscle tremors. She was treated with cyclosporine and Imuran and received one dose of IV IG in the hospital without significant improvement. Her neurologic illness was progressive with both severe central and peripheral neurologic impairment and she became non-ambulatory. She also had five “episodes” of left sided weakness and numbness between September and November, 1994. In addition, her parents and school teachers had noted mental status changes (e.g., not understanding commands on request or decreased volume and amount of speech). She had trouble feeding herself with a spoon, difficulty coloring and had increased bowel and bladder incontinence with some episodes of blank staring. A subsequent workup included nerve and muscle biopsy at which time she received a diagnosis of metachromatic leukodystrophy on the basis of prismatic bodies diagnostic of that disease.
