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. 1974 Aug;37(8):879–887. doi: 10.1136/jnnp.37.8.879

Jakob-Creutzfeldt disease

Modification of clinical and electroencephalographic activity with methylphenidate and diazepam

F Elliott 1,1, C Gardner-Thorpe 1,2, D D Barwick 1, J B Foster 1,3
PMCID: PMC494801  PMID: 4607657

Abstract

The electroencephalogram in three patients with Jakob-Creutzfeldt disease showed two separate abnormalities—namely, progressive background suppression and periodic generalized synchronous triphasic sharp wave complexes which evolve to a uniform morphology and periodicity. The abnormalities, when found in the EEG of a patient in middle-age with a dementing illness, should not be confused with other periodic electroencephalographic phenomena. Since the neuropathological abnormalities of Jakob-Creutzfeldt disease are non-specific, the electroencephalogram is essential for the recognition of this disorder, although serial recordings may be necessary to establish the diagnosis. Modification of the electroencephalographic abnormalities occurs with afferent stimuli and with methylphenidate or diazepam, suggesting that the phenomenon of background suppression is independent of the presence of the periodic complexes. Modification of clinical activity with methylphenidate suggests that some degree of reversibility of function exists in this inexorably fatal disorder. Further detailed studies of the electroencephalogram in cases of Jakob-Creutzfeldt disease are indicated.

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Selected References

These references are in PubMed. This may not be the complete list of references from this article.

  1. Barrett R. E. Dementia in adults. Med Clin North Am. 1972 Nov;56(6):1405–1418. doi: 10.1016/s0025-7125(16)32329-x. [DOI] [PubMed] [Google Scholar]
  2. Förster C. EEG changes in Jakob-Creutzfeldt's disease under the influence of diazepam. Electroencephalogr Clin Neurophysiol. 1970 Aug;29(2):218–218. [PubMed] [Google Scholar]
  3. Förster C., Kugler J. EEG differential diagnosis of Jakob-Creutzfeldt disease. Electroencephalogr Clin Neurophysiol. 1970 Mar;28(3):327–327. [PubMed] [Google Scholar]
  4. Gajdusek D. C., Gibbs C. J., Alpers M. Experimental transmission of a Kuru-like syndrome to chimpanzees. Nature. 1966 Feb 19;209(5025):794–796. doi: 10.1038/209794a0. [DOI] [PubMed] [Google Scholar]
  5. Gibbs C. J., Jr, Gajdusek D. C., Asher D. M., Alpers M. P., Beck E., Daniel P. M., Matthews W. B. Creutzfeldt-Jakob disease (spongiform encephalopathy): transmission to the chimpanzee. Science. 1968 Jul 26;161(3839):388–389. doi: 10.1126/science.161.3839.388. [DOI] [PubMed] [Google Scholar]
  6. Goldhammer Y., Braham J. EEG changes in subacute encephalopathy (Creutzfeldt-Jakob disease). Electroencephalogr Clin Neurophysiol. 1969 Aug;27(2):217–217. [PubMed] [Google Scholar]
  7. Goldhammer Y., Bubis J. J., Sarova-Pinhas I., Braham J. Subacute spongiform encephalopathy and its relation to Jakob-Creutzfeldt disease: report on six cases. J Neurol Neurosurg Psychiatry. 1972 Feb;35(1):1–10. doi: 10.1136/jnnp.35.1.1. [DOI] [PMC free article] [PubMed] [Google Scholar]
  8. Gubbay S. S., Barwick D. D. Two cases of accidental hypothermia in Parkinson's disease with unusual E.E.G. findings. J Neurol Neurosurg Psychiatry. 1966 Oct;29(5):459–466. doi: 10.1136/jnnp.29.5.459. [DOI] [PMC free article] [PubMed] [Google Scholar]
  9. HIRANO A., KURLAND L. T., KROOTH R. S., LESSELL S. Parkinsonism-dementia complex, an endemic disease on the island of Guam. I. Clinical features. Brain. 1961 Dec;84:642–661. doi: 10.1093/brain/84.4.642. [DOI] [PubMed] [Google Scholar]
  10. HIRANO A., MALAMUD N., KURLAND L. T. Parkinsonism-dementia complex, an endemic disease on the island of Guam. II. Pathological features. Brain. 1961 Dec;84:662–679. doi: 10.1093/brain/84.4.662. [DOI] [PubMed] [Google Scholar]
  11. Hauser-Dumur F., Radvanyi M. F. EEG evolution of two cases of late Jakob-Creutzfeldt syndrome. Electroencephalogr Clin Neurophysiol. 1970 Jun;28(6):644–644. [PubMed] [Google Scholar]
  12. Hirano A., Malamud N., Elizan T. S., Kurland L. T. Amyotrophic lateral sclerosis and Parkinsonism-dementia complex on Guam. Further pathologic studies. Arch Neurol. 1966 Jul;15(1):35–51. doi: 10.1001/archneur.1966.00470130039004. [DOI] [PubMed] [Google Scholar]
  13. JONES D. P., NEVIN S. Rapidly progressive cerebral degeneration (subacute vascular encephalopathy) with mental disorder, focal disturbances, and myoclonic epilepsy. J Neurol Neurosurg Psychiatry. 1954 May;17(2):148–159. doi: 10.1136/jnnp.17.2.148. [DOI] [PMC free article] [PubMed] [Google Scholar]
  14. May W. W. Creutzfeldt-Jakob disease. 1. Survey of the literature and clinical diagnosis. Acta Neurol Scand. 1968;44(1):1–32. doi: 10.1111/j.1600-0404.1968.tb07440.x. [DOI] [PubMed] [Google Scholar]
  15. NEUMANN M. A., GAJDUSEK D. C., ZIGAS V. NEUROPATHOLOGIC FINDINGS IN EXOTIC NEUROLOGIC DISORDERS AMONG NATIVES OF THE HIGHLANDS OF NEW GUINEA. J Neuropathol Exp Neurol. 1964 Jul;23:486–507. doi: 10.1097/00005072-196407000-00007. [DOI] [PubMed] [Google Scholar]
  16. Robinson N. Creutzfeldt-Jakob's disease: a histochemical study. Brain. 1969;92(3):581–588. doi: 10.1093/brain/92.3.581. [DOI] [PubMed] [Google Scholar]
  17. SIEDLER H., MALAMUD N. CREUTZFELDT-JAKOB'S DISEASE. CLINICOPATHOLOGIC REPORT OF 15 CASES AND REVIEW OF THE LITERATURE (WITH SPECIAL REFERENCE TO A RELATED DISORDER DESIGNATED AS SUBACUTE SPONGIFORM ENCEPHALOPATHY). J Neuropathol Exp Neurol. 1963 Jul;22:381–402. [PubMed] [Google Scholar]
  18. Torvik A. Aspects of the pathology of presenile dementia. Acta Neurol Scand. 1970;46(Suppl):19+–19+. doi: 10.1111/j.1600-0404.1970.tb02154.x. [DOI] [PubMed] [Google Scholar]

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