Rosenthal fiber encephalopathy presenting with demyelination and Rosenthal fibers in a solvent abuser: adult Alexander's disease?
We report a neuropathological case of the brain of a 22-year-old male solvent abuser. He had also been diagnosed as suffering from schizophrenia. Neurohistology revealed the presence of Rosenthal fibers (RFs) in subpial and perivascular collections in the medulla, the floor of the 3rd and 4th ventricles, and also in the spinal cord. There was mild demyelination in the frontal, temporal, and parietal lobes, as well as the left dorsal column in the spinal cord. There was bilateral basal ganglia calcification. Demyelination and RFs have been the hallmark of the diagnosis of adult Alexander's disease. We urge that these are insufficient criteria for this nosological category, and suggest the alternative and more appropriate label of Rosenthal fiber encephalopathy. In view of the known toxic, clinical effects of chronic solvent abuse, and the lack of knowledge of the neuropathological effects, we also tentatively note the association of the solvent abuse with the neuropathology, but are unable to assert any causal link.
Wilson
S P
SP
Department of Neuropathology, Institute of Pathology, Leeds General Infirmary, UK.
Al-Sarraj
S
S
Bridges
L R
LR
D000328:Adult; D002493:Central Nervous System Diseases / Q000139:chemically induced / Q000175:diagnosis*; D003711:Demyelinating Diseases / Q000139:chemically induced / Q000175:diagnosis*; D003937:Diagnosis, Differential; D004804:Eosinophils / Q000473:pathology*; D006801:Humans; D008297:Male; D012997:Solvents; D019966:Substance-Related Disorders / Q000150:complications*
D012997:Solvents