title: Improved peripheral nerve conduction, EEG and verbal IQ after bone marrow transplantation for adult metachromatic leukodystrophy.
abstract: A 28-year-old woman with a 4 year history of slowly progressing 'frontal dementia' was diagnosed as having adult metachromatic leukodystrophy and was followed for 4 years after bone marrow transplantation (BMT). MRI, neurophysiological tests (EEG, ENeG, VEP, SEP and BAEP) and neuropsychological assessment were performed before, and repeatedly after BMT. MRI showed symmetrical white matter lesions in the frontal and parieto-occipital lobes and in the corpus callosum. EEG showed frontal and temporal slow wave abnormalities and nerve conduction was slow. Neuropsychological tests showed cognitive impairment in executive functions, decline in visuospatial-constructive and spatial memory tasks and disorganized thinking. IQ was low (52), with slightly better values for verbal IQ than for performance IQ. After BMT, the patient was followed for 4 years. Clear improvements were seen in EEG, in peripheral nerve conduction and in neuropsychological tests (especially in verbal IQ). MRI findings were unchanged. We believe that the improvement in our patient resulted from the bone marrow transplantation.
authors.affiliation: Department of Neurology and Clinical Neurophysiology, Huddinge University Hospital, Sweden.
authors.forename: G
authors.initials: G
authors.lastname: Solders
authors.forename: G
authors.initials: G
authors.lastname: Celsing
authors.forename: L
authors.initials: L
authors.lastname: Hagenfeldt
authors.forename: P
authors.initials: P
authors.lastname: Ljungman
authors.forename: B
authors.initials: B
authors.lastname: Isberg
authors.forename: O
authors.initials: O
authors.lastname: Ringdén
mesh_terms: D000328:Adult; D016026:Bone Marrow Transplantation; D001921:Brain / Q000473:pathology; D002553:Cerebroside-Sulfatase / Q000097:blood / Q000172:deficiency; D004569:Electroencephalography; D005260:Female; D006801:Humans; D007360:Intelligence; D007966:Leukodystrophy, Metachromatic / Q000503:physiopathology* / Q000523:psychology / Q000628:therapy*; D008279:Magnetic Resonance Imaging; D009431:Neural Conduction; D010525:Peripheral Nerves / Q000503:physiopathology*; D013997:Time Factors
chemical_list: D002553:Cerebroside-Sulfatase