Summary
Two cases of Alexander's disease are described. One case of infantile onset died at the age of 6 months and the second case was of the juvenile type with onset at 2 years and death at 10 years. A clinical diagnosis of this disease is difficult since signs can vary according to the age of the patient. The severity of the pathological changes can also depend upon the age of onset of this disease, but they are restricted to the central nervous system. The Rosenthal fibre is the characteristic feature of Alexander's disease and we have examined for the first time its ultrastructure and immunocytochemical characteristics at the electron microscopical level and demonstrated coexpression of anti-glial fibrillary acidic protein and anti-ubiquitin antisera.
Similar content being viewed by others
References
Alexander WS (1949) Progressive fibrinoid degeneration of fibrillary astrocytes associated with mental retardation in a hydrocephalic infant. Brain 72:373–381
Arend RO, Leary PM, Rutherford GS (1991) Alexander's disease: a case report with brain biopsy, ultrasound, CT scan and MRI findings. Clin Neuropathol 10:122–126
Blakemore WF, Franklin RMJ (1991) Transplantation of glial cells into the CNS. Trends Neurosci 14:232–327
Borret D, Becker LS (1985) Alexander's disease: a disease of astrocytes. Brain 108:367–385
Cole G, DeVilliers F, Proctor NSE, Freiman I, Bill P (1979) Alexander's disease: Case report including histopathological and electron microscopic features. J Neurol Neurosurg Psychiatry 42:619–624
DeCoo IF, Gabreels EJM, Renier WO, DePont JHM, VanHaelstu GJM, Veerkamp J, Trijbels JMF, Jaspar HHJ, Renkawek K (1991) Canavans disease: neuromorphological and biochemical analysis of a brain biopsy specimen. Clin Neuropathol 10:73–78
Dina AK, Sarkar C, Roy S (1990) Rosenthal fibers: an immunohistochemical ultrastructural and immunoelectron microscopic study. Acta Neuropathol 79:456–460
Farrell K, Chuang S, Becker LE (1984) Computed Tomography in Alexander's disease. Ann Neurol 15:605–607
French TA, Bower BD, Cameron AH (1976) Alexander's disease presenting as an astrocytoma. J Neurol Neurosurg Psychiatry 39:803–809
Goldman JE, Corbin E (1988) Isolation of a major protein component of Rosenthal fibers. Am J Pathol 130:569–577
Goldman JE, Corbin E (1991) Rosenthal fibers contain ubiquitinated β-crystallin. Am J Pathol 139:933–938
Gutmann DH (1991) Chromosome 11q23.3-qter deletion and Alexander's disease. Am J Med Genet 39:226
Herndon R, Rubenstein L, Freeman J, Mathesion G (1970) Light and electron microscopic observations in Alexander's disease and in multiple sclerosis. J Neuropathol Exp Neurol 29:524–551
Horoupian DS, Kress Y, Yen SH (1982) Nickel induced changes and reappraisal of Rosenthal fibers in focal CNS lesions. J Neuropathol Exp Neurol 41:664–676
Iwaki T, Kume I, Liem RKM, Goldman JE (1989) β-Crystallins expressed in non-lenticular tissues and accumulates in Alexander's disease brains. Cell 57:71–79
Lowe J, Blanchard A, Morrell K, Lennox G, Reynolds L, Billett M, Landon M, Mayer RJ (1988) Ubiquitin is a common factor in intermediate filament inclusion bodies of diverse type in man, including those of Parkinson's disease, Pick's disease and Alzheimer's disease, as well as Rosenthal fibres in cerebellar astrocytomas, cytoplasmic bodies in muscle and Mallory bodies in alcoholic liver disease. J Pathol 155:9–15
Lowe J, Morrell K, Lennox G, Landon M, Mayer RJ (1989) Rosenthal fibers are based on the ubiquitination of glial filaments. Neuropathol Appl Neurobiol 15:45–53
Peiffer J (1968) Alexander's disease. Really a leukodystrophy? Pathol Eur 3:305–312
Raff MC (1989) Glial cell diversification in the rat optic nerve. Science 243:1450–1454
Russell D, Rubenstein L (1977) Pathology of tumours of the central nervous system, 4th edn. Edward Arnold, London, p 161
Russo D, Aron A, Anderson J (1976) Alexander's disease: a report and reappraisal. Neurology 26:607–614
Schohet SS, Lampert PW, Earle KM (1968) Alexander's disease: a case report with electron microscope observations. Neurology 18:543–549
Seil FJ, Schohet SS, Earle KM (1968) Alexander's disease in an adult. Arch Neurol 19:494–502
Sherwin M, Bethrong M (1970) A case of Alexander's disease with sudanophilic leukodystrophy. Arch Pathol 89:321–328
Singhrao S, Cole G, Henderson WJ, Newman GR (1990) LR White embedding allows a multi-method approach to the analysis of brain tissue from patients with Alzheimer's disease. J Histochem 22:257–268
Vogel FS, Hallervorden JV (1962) Leukodystrophy with diffuse Rosenthal fiber formation. Acta Neuropathol (Berl) 2:126–143
Walls TJ, Jones RA, Cartlidge NEF, Saunders M (1984) Alexander's disease with Rosenthal fiber formation in an adult. J Neurol Neurosurg Psychiatry 47:399–403
Wohwill F, Bernstein P, Yakovlev P (1957) Case of unusual presumably familial leukodystrophy with megalencephaly. J Neuropathol Exp Neurol 16:129–130
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Neal, J.W., Cave, E.M., Singhrao, S.K. et al. Alexander's disease in infancy and childhood: a report of two cases. Acta Neuropathol 84, 322–327 (1992). https://doi.org/10.1007/BF00227826
Received:
Revised:
Accepted:
Issue Date:
DOI: https://doi.org/10.1007/BF00227826