Alexander's disease with Rosenthal fibre formation in an adult.
The case of a woman who had a mild mental and physical handicap all her life is described. At the age of 39 years she began a progressive deterioration which terminated with her death after a year. CT scanning revealed basal ganglia calcification and at necropsy there was evidence of widespread generalised myelin loss with extensive Rosenthal fibre formation. The hypothesis that this may be an adult form of Alexander's disease is discussed.
D001921:Brain / Q000473:pathology; D001927:Brain Diseases / Q000473:pathology*; D002114:Calcinosis / Q000473:pathology; D002421:Caudate Nucleus / Q000473:pathology; D003704:Dementia / Q000473:pathology*; D005260:Female; D006801:Humans; D008607:Intellectual Disability / Q000473:pathology*; D008875:Middle Aged; D009186:Myelin Sheath / Q000648:ultrastructure; D009413:Nerve Fibers, Myelinated / Q000648:ultrastructure*; D009454:Neurofibrils / Q000648:ultrastructure; D013116:Spinal Cord / Q000473:pathology; D014057:Tomography, X-Ray Computed