Abstract
A 29 year old male presented with epileptic fits, progressive left sided focal seizures and epilepsia partialis continua, increasing left hemiparesis and mental slowing. Death occurred 2 years after the onset of the illness. Lesions were limited to the right cerebral hemisphere. Hypertrophic astrocytosis was diffuse throughout the gray and white matter but was more severe in the deep cortical layers and U fibres, where it was associated with vacuolar changes and capillary proliferation. Sparse perivascular lymphocytic cuffs, rod shaped microglia and microglial nodules were present. No inclusion bodies were found. These clinico-pathologic features were similar to the cases described by Rasmussen. Only five necropsy cases of this rare disease have been reported previously, all in children. The aetiology is unknown.
Full text
PDFImages in this article
Selected References
These references are in PubMed. This may not be the complete list of references from this article.
- AGUILAR M. J., RASMUSSEN T. Role of encephalitis in pathogenesis of epilepsy. Arch Neurol. 1960 Jun;2:663–676. doi: 10.1001/archneur.1960.03840120069008. [DOI] [PubMed] [Google Scholar]
- Bancaud J., Bonis A., Trottier S., Talairach J., Dulac O. L'épilepsie partielle continue: syndrome et maladie. Rev Neurol (Paris) 1982;138(11):803–814. [PubMed] [Google Scholar]
- Diringer H., Rahn H. C., Bode L. Antibodies to protein of scrapie-associated fibrils. Lancet. 1984 Aug 11;2(8398):345–345. doi: 10.1016/s0140-6736(84)92708-9. [DOI] [PubMed] [Google Scholar]
- Friedman H., Ch'ien L., Parham D. Virus in brain of child with hemiplegia, hemiconvulsions, and epilepsy. Lancet. 1977 Sep 24;2(8039):666–666. doi: 10.1016/s0140-6736(77)92540-5. [DOI] [PubMed] [Google Scholar]
- Goldring S. A method for surgical management of focal epilepsy, especially as it relates to children. J Neurosurg. 1978 Sep;49(3):344–356. doi: 10.3171/jns.1978.49.3.0344. [DOI] [PubMed] [Google Scholar]
- Gupta P. C., Rapin I., Houroupian D. S., Roy S., Llena J. F., Tandon P. N. Smoldering encephalitis in children. Neuropediatrics. 1984 Oct;15(4):191–197. doi: 10.1055/s-2008-1052365. [DOI] [PubMed] [Google Scholar]
- Gupta P. C., Roy S., Tandon P. N. Progressive epilepsy due to chronic persistent encephalitis. Report of four cases. J Neurol Sci. 1974 May;22(1):105–120. doi: 10.1016/0022-510x(74)90058-6. [DOI] [PubMed] [Google Scholar]
- Johnson R. T., Herndon R. M. Virologic studies of multiple sclerosis and other chronic and relapsing neurological diseases. Prog Med Virol. 1974;18(0):214–228. [PubMed] [Google Scholar]
- Juul-Jensen P., Denny-Brown D. Epilepsia partialis continua. Arch Neurol. 1966 Dec;15(6):563–578. doi: 10.1001/archneur.1966.00470180003001. [DOI] [PubMed] [Google Scholar]
- Merz P. A., Rohwer R. G., Kascsak R., Wisniewski H. M., Somerville R. A., Gibbs C. J., Jr, Gajdusek D. C. Infection-specific particle from the unconventional slow virus diseases. Science. 1984 Jul 27;225(4660):437–440. doi: 10.1126/science.6377496. [DOI] [PubMed] [Google Scholar]
- RASMUSSEN T., GOSSMAN H. EPILEPSY DUE TO GROSS DESTRUCTIVE BRAIN LESIONS. RESULTS OF SURGICAL THERAPY. Neurology. 1963 Aug;13:659–669. doi: 10.1212/wnl.13.8.659. [DOI] [PubMed] [Google Scholar]
- RASMUSSEN T., OLSZEWSKI J., LLOYDSMITH D. Focal seizures due to chronic localized encephalitis. Neurology. 1958 Jun;8(6):435–445. doi: 10.1212/wnl.8.6.435. [DOI] [PubMed] [Google Scholar]
- Rasmussen T. Further observations on the syndrome of chronic encephalitis and epilepsy. Appl Neurophysiol. 1978;41(1-4):1–12. doi: 10.1159/000102395. [DOI] [PubMed] [Google Scholar]
- Rasmussen T., McCann W. Clinical studies of patients with focal epilepsy due to "chronic encephalitis". Trans Am Neurol Assoc. 1968;93:89–94. [PubMed] [Google Scholar]
- Sternberger L. A., Hardy P. H., Jr, Cuculis J. J., Meyer H. G. The unlabeled antibody enzyme method of immunohistochemistry: preparation and properties of soluble antigen-antibody complex (horseradish peroxidase-antihorseradish peroxidase) and its use in identification of spirochetes. J Histochem Cytochem. 1970 May;18(5):315–333. doi: 10.1177/18.5.315. [DOI] [PubMed] [Google Scholar]
- Thomas J. E., Reagan T. J., Klass D. W. Epilepsia partialis continua. A review of 32 cases. Arch Neurol. 1977 May;34(5):266–275. doi: 10.1001/archneur.1977.00500170020003. [DOI] [PubMed] [Google Scholar]
- Wilfert C. M., Buckley R. H., Mohanakumar T., Griffith J. F., Katz S. L., Whisnant J. K., Eggleston P. A., Moore M., Treadwell E., Oxman M. N. Persistent and fatal central-nervous-system ECHOvirus infections in patients with agammaglobulinemia. N Engl J Med. 1977 Jun 30;296(26):1485–1489. doi: 10.1056/NEJM197706302962601. [DOI] [PubMed] [Google Scholar]