Adult onset leukodystrophy with neuroaxonal spheroids and demyelinating plaque-like lesions

摘要: Adult onset leukodystrophy with neuroaxonal spheroids is an uncommon cause of dementia. Both hereditary (autosomal dominant) and sporadic cases have been described. A 41-year-old African woman presented inappropriate behavior personality change consistent frontal lobe dysfunction. MRI demonstrated diffuse frontoparietal white matter signal abnormality volume loss, as well focal enhancing lesions, while CT scan showed calcifications. She had gradually deteriorating over the last 5 years, diagnosed having progressive demyelinating illness. died recurrent chest infections. There was no familial history. The brain prominent symmetrical changes greyish discolorization mainly affecting parietal lobes, less involvement temporal only mildly occipital matter. Histology revealed deep atrophy many labelled by neurofilament β-amyloid precursor protein. In addition, scattered inactive plaque-like lesions were found in periventricular areas, brainstem cervical spinal cord. This case typical features adult spheroids. However, we also which has not previously possibility a origin contributing to may be considered pathogenesis this condition.