A patient with biopsy-proven Alexander disease presented with progressive head enlargement and clinical evidence of increased intracranial pressure. Lumbar puncture under sedation confirmed increased intracranial pressure with otherwise normal cerebrospinal fluid values. The association of megalencephaly, increased intracranial pressure and neuroimaging evidence of leukoencephalopathy should alert pediatric neurologists to the possibility of Alexander disease. (J Child Neurol 1992;7:168-171).
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References
1.
Alexander WS : Progressive fibrinoid degeneration of fibrillary astrocytes associated with mental retardation in a hydrocephalic infant. Brain1949;72:373-381.
2.
Russo LS, Aron A., Anderson PJ: Alexander's disease: A report and reappraisal. Neurology1976;26:607-614.
3.
Matalon R., Michals K., Sebesta D., et al: Aspartoacylase deficiency and N-acetylaspartic aciduria in patients with Canavan's disease. Am J Med Genet1988 ;29:463-471.
4.
Ozand PT, Gascon GG, Dhalla M.: Aspartoacylase deficiency and Canavan's disease in Saudi Arabia. Am J Med Genet1990;35:266-268.
5.
Farrell K., Chuang S., Becker LE: Computed tomography in Alexander's disease. Ann Neurol1984;15:605-607.
6.
Cole G., De Villiers F, Proctor Nsf, et al: Alexander's disease: Case report including histopathological and electron microscopic features. J Neurol Neurosurg Psychiatry1979;42:619-624.
7.
Towfighi J., Young R., Sassani J., et al: Alexander's disease: Further light- and electronic-microscopic observations. Acta Neuropathol (Berl)1983;61:36-42.
8.
Borrett D., Becker LE: Alexander's disease. A disease of astrocytes. Brain1985;108:367-385.
