Diversidad sindromatica de la enfermedad de Creutzfeldt-Jakob: correlato neurofisiologico e histopatologico

Ferrer D, Sergio; Cartier R., Luis; Lolas S., Fernando; Perez F., Manuel

doi:10.1590/S0004-282X1982000100005

Resúmenes

Se estudian dos casos de enfermedad de Creutzfeldt-Jakob comprobados por examen necrópsico. Uno de ellos presentaba típicas descargas hipersincrónicas, periódicas en el electroencefalograma; el otro caso no mostraba estas descargas epileptiformes y evolucionó con una lentitud difusa progresiva. La espongiosis, gliosis y pérdida neuronal fué intensa en la corteza del caso con espigas y muy moderada en el caso sin espigas. El compromiso subcortical era similar en ambos casos. Se postula que la diferente expresión topográfica de su histopatología explicaria tambien la diferencia en el comportamiento de los potenciales evocados somatosensoriales y el diferente modelo electroencefalográfico del sueno penthotálico. La independencia de las descargas epileptiformes en cada hemisferio se prueba con el test de Wada. Se discute el origen de las espigas y su periodicidad. Se postula teóricamente que el predominio de las lesiones de las capas II a IV privaria a las células piramidales remanentes, de influencias inhibitorias, lo que explicaria la génesis de las espigas.

Two syndromatic forms of Creutzfeldt-Jakob disease are illustrated with data from two cases confirmed by necropsy and studied by means of electroencephalogram, evoked potential recording and neuropathological techniques. One of the cases presented the typical hypersynchronous periodic discharges thougth to be characteristic of this disease. The other did not exhibit epileptiform diischarges, showing an evolution in which progressive diffuse slowing was the prominent feature. Cortical spongyosis, gliosis, and neuronal lose were intense in the case showing spikes, and moderate in the one without spikes. Subcortical involvement was similar in both cases. This different topography of pathological involvement might explain, in addition to electroencephalographic findings, the different behavior of somatosensory evoked potentials, which were of larger amplitude in the case showing epileptiform discharges, although in both cases deterioration of clinical state was accompanied by a progressive amplitude decrement in the evoked potential. Sleep induced by barbiturates was disrupted in the case with spikes, its pattern being closer to normal in the case with less severe cortical involvement. A Wada test demostrated that discharges in the hemispheres were independent. Spike periodicity is discussed. It is postulated that the heavier involvement of cortical layers II to IV deprive the remaining pyramidal cells of inhibitory influences, a condition which might explain the generation of periodic spike discharges.

Diversidad sindromatica de la enfermedad de Creutzfeldt-Jakob: correlato neurofisiologico e histopatologico

Syndromatic heterogeneity of Creutzfeldt-Jakob disease: neurophysioíogical and histopathological correlates

Sergio Ferrer D.^I; Luis Cartier R.^II; Fernando Lolas S.^III; Manuel Perez F.^IV

^IProf, de Neurologia, Hospital Militar, Santiago de Chile

^IIJefe dei Laboratório de Neuropatología, Departamento de Ciências Neurológicas, Universidad de Chile, Area Oriente

^IIIProf, de Neurofisiología, Departamento de Fisiología de la Universidad de Chile, Area Norte

^IVAyudante de Clínica Neurológica, Hospital Militar

RESUMEN

Se estudian dos casos de enfermedad de Creutzfeldt-Jakob comprobados por examen necrópsico. Uno de ellos presentaba típicas descargas hipersincrónicas, periódicas en el electroencefalograma; el otro caso no mostraba estas descargas epileptiformes y evolucionó con una lentitud difusa progresiva. La espongiosis, gliosis y pérdida neuronal fué intensa en la corteza del caso con espigas y muy moderada en el caso sin espigas. El compromiso subcortical era similar en ambos casos.

Se postula que la diferente expresión topográfica de su histopatología explicaria tambien la diferencia en el comportamiento de los potenciales evocados somatosensoriales y el diferente modelo electroencefalográfico del sueno penthotálico. La independencia de las descargas epileptiformes en cada hemisferio se prueba con el test de Wada.

Se discute el origen de las espigas y su periodicidad. Se postula teóricamente que el predominio de las lesiones de las capas II a IV privaria a las células piramidales remanentes, de influencias inhibitorias, lo que explicaria la génesis de las espigas.

SUMMARY

Two syndromatic forms of Creutzfeldt-Jakob disease are illustrated with data from two cases confirmed by necropsy and studied by means of electroencephalogram, evoked potential recording and neuropathological techniques. One of the cases presented the typical hypersynchronous periodic discharges thougth to be characteristic of this disease. The other did not exhibit epileptiform diischarges, showing an evolution in which progressive diffuse slowing was the prominent feature.

Cortical spongyosis, gliosis, and neuronal lose were intense in the case showing spikes, and moderate in the one without spikes. Subcortical involvement was similar in both cases. This different topography of pathological involvement might explain, in addition to electroencephalographic findings, the different behavior of somatosensory evoked potentials, which were of larger amplitude in the case showing epileptiform discharges, although in both cases deterioration of clinical state was accompanied by a progressive amplitude decrement in the evoked potential. Sleep induced by barbiturates was disrupted in the case with spikes, its pattern being closer to normal in the case with less severe cortical involvement. A Wada test demostrated that discharges in the hemispheres were independent.

Spike periodicity is discussed. It is postulated that the heavier involvement of cortical layers II to IV deprive the remaining pyramidal cells of inhibitory influences, a condition which might explain the generation of periodic spike discharges.

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Servicio de Neurologia, Hospital Militar - Plaza de los Leones s/n - Santiago - Chile.

1. AYALA, G. F.; MATSUMOTO, H. & GUMNIT, R. J. - Excitability changes and inhibitory mechanisms in neocortical neurons during seizures. J. Neurophysiol. 33:73, 1970.
2. AYALA, G. F.; DICHTER, M.; GUMNIT, R. J.; MATSUMOTO, H. & SPENCER, W. A. - Genesis of epileptic interictal spikes: new knowlegde of cortical feedback system suggests a neurophysiological explanation of brief paroxysms. Brain Res. 52:1, 1973.
3. BALL, G. J.; GLOOR, P. & THOMPSON, C. J. - Computed Unit-EEG correlations and laminar profiles of spindle waves in the EEG of cats. Electroenceph. Clin. Neurophysiol. 43:330, 1977.
4. BECK, E.; DANIEL, P. M.; GAJDUSEK, D. C. & GIBBS, C. J. Jr. - Subacute degenerations of the brain transmissible to experimental animals: a neuropathological evaluations. VI Congrés International de Neuropathologie. Paris, 1970. Masson et Cie, Paris, 1971.
5. BURGER, L. J.; ROWAN, J. & GOLDENSHON, E. S. - Creutzfeldt Jacob disease: an electroencephaphic study. Arch. Neurol. (Chicago) 26:428, 1972.
6. CAPON, A.; COLIN, F.; DELTENRE, P.; HUBERT, J. P. & FLAMET-DURANT, J. - Etude électrophysiologique de deux cas de maladie de Creutzfeldt-Jakob. Rev. Electroencephalogr. Neurophysiol. (Paris) 6:130, 1976.
7. CARTIER, L.; FERRER, S.; STUARDO, A. & PEREZ, M. - Correlación clínica, anatomopatológica y electroencefalográíca en la enfermedad de Creutzfeldt-Jakob. II Congreso Ibero-Americano de Electroencefalografia y Neurofisiologia Clínica, (Lima), 1979.
8. CARTIER, L. & GALVEZ, S. - Intranuclear corpuscles in the neurone of substantia nigra: anatomoclinical report of 11 cases of Jakob-Creutzfeldt disease. Neuro-cirugfa (Chile) 35:1, 1977.
9. CLARK, D. L. & ROSNER, B. S. - Neurophysiologic effects of general anesthetics. Anesthesiology 38:564, 1973.
10. CREUTZFELDT, O. D.; GAREY, L. J.; KURODA, R. & WOLF J. R. - The distribution of degenerating axons after small lesions in the intact and isolated visual cortex of the cat. Exp. Brain Res. 27:419, 1977.
11. CHIAPPA, K. & YOUNG, R. - The E.E.G. as a definitive diagnostic tool early in the course of Creutzfeldt-Jakob diisease. Electroenceph. Clin. Neurophysiol. 45:26, 1978.
12. GATTER, K. C. & POWELL, T. P. S. - The intrinsic connections of the cortex of area 4 of the monkey. Brain 513:101, 1978.
13. GATTER, K. C; SLOPER, J. J. & POWELL T. P. S. - An electron microscopic study of the termination of intracortical axons upon Betz cells in are 4 of the monkey. Brain 543:101, 1978.
14. GIBBS, C. J. Jr. & GAJDUSEK, D. C. - Experimental subacute spongiform virus encephalophaties in primates and other laboratory animals. Science (Washington) 182:67. 1973.
15. GLOOR, P.; KALABAY, O. & GIARD, N. - The electroencephalogram in difuse encephalopathies: electroencephalographic correlates of grey and white matter lesions. Brain 91:779, 1968.
16. GOTO, K.; UMEZAKI, H. & SUETSUGU, M. - Electroencephalographic and clnico-pathological studies on Creutzfeldt-Jakob syndrome. J. Neurol. Neurosurg. and Psychiat. 39:931, 1976.
17. HALLIDAY, A. M. - The electrophysiological study of myoclonic in man. Brain 90:241, 1967.
18. JONES, D. P. & NEVIN, S. - Rapidly progressive cerebral degeneration (subacute vascular encephalopathy) with mental disorder; focal disturbances and myoclonic epilepsy. J. Neurol. Neurosurg. and Psychiat. 17:148, 1954.
19. KIRSCHBAUM, W. R. - Jakob-Creutzfeldt Disease. Elsevier (New York) 1968.
20. LAMPERT, P. W.; GAJDUSEK, D. C. & GIBBS, C. J. Jr. - Pathology of dendrites in subacute spongiform virus encephalopathies. Advances in Neurology (New York) 12:465, 1975.
21. LEE, R. G. & BLAIR, R. D. G. - Evolution of EEG and visual evoked response changes in Jakob-Creutzfeldt disease. Electroenceph. Clin. Neurophysiol. 35:133, 1973.
22. MASTERS, C. L. & RICHARDSON, E. P. - Subacute spongiform encephalopathy (Creutzfeldt-Jakob disease). Brain 101:333, 1978.
23. MATSUMOTO, H. & AJMONE-MARSAN, C. - Cortical cellular phenomena in experimental epilepsy: interictal manifestation. Exp. Neurol. 9:286, 1964.
24. MATSUMOTO, H. & AJMONE-MARSAN, C. - Cortical cellular phenomena in experimental epilepsy: ictal manifestations. Exp. Neurol. 9:305, 1964.
25. MORRELL, F.; BERGEN, D.; HOEPPNER, T.; LOLAS, F.; FEXEIRA, C; BECK, E. & SMITH, D. - Epileptiform activity, steady potential shift and visual evoked response in Creutzfeldt-Jakob disease. Electroenceph. Clin. Neurophysiol. 45:26, 1978.
26. NEVIN, S.; MC MENEMEY, W. H.; BEHRMAN, S. & JONES, D. P. - Subacute spongiform encephalopathy: a subacute form of encephalopatty attributable to vascular dysfunction (Spongiform cerebral atrophy). Brain 36;762, 1960.
27. PEDLEY, T. A. - The pathophysiology of focal epilepsy neurophysiological considerations. Ann. Neurol. (Boston) 3:2, 1978.
28. PRINCE, D. A. - The deporalization shift in epileptic neurons. Exp. Neurol. 21:467, 1968.
29. PRINCE, D. A. - Electrophysiology of epileptic neurons spike generations. Electroenceph. Clin. Neurophysiol. 26:476, 1969.
30. RAYPORT, M. - Electroencephagraphic, corticographic and intracerebral potentials in two anatomicaly verified cases of Creutzfeldt-Jakob disease. Electroenceph. Clin. Neurophysiol. 15:921, 1963.
31. SZENTAGOTHAI, J. - Architecture of the Cerebral Cortex in Basic Mechanisms of Epilepsies. Little Brown and Co., Boston, 1969, pp. 13.
32. TESANER, D. & FOUCHER, A. - Corrélations entre les variations de la vigilance, l'activite EEG, la respiration et les clonies dans a cas de maladie de Creutzfeldt-Jakob. Rev. Electroencephalogr. Neurophysiol. 8:354, 1978.

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16 Ago 2012
Fecha del número
Mar 1982

This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License.

[1] 1. AYALA, G. F.; MATSUMOTO, H. & GUMNIT, R. J. - Excitability changes and inhibitory mechanisms in neocortical neurons during seizures. J. Neurophysiol. 33:73, 1970.

[2] 2. AYALA, G. F.; DICHTER, M.; GUMNIT, R. J.; MATSUMOTO, H. & SPENCER, W. A. - Genesis of epileptic interictal spikes: new knowlegde of cortical feedback system suggests a neurophysiological explanation of brief paroxysms. Brain Res. 52:1, 1973.

[3] 3. BALL, G. J.; GLOOR, P. & THOMPSON, C. J. - Computed Unit-EEG correlations and laminar profiles of spindle waves in the EEG of cats. Electroenceph. Clin. Neurophysiol. 43:330, 1977.

[4] 4. BECK, E.; DANIEL, P. M.; GAJDUSEK, D. C. & GIBBS, C. J. Jr. - Subacute degenerations of the brain transmissible to experimental animals: a neuropathological evaluations. VI Congrés International de Neuropathologie. Paris, 1970. Masson et Cie, Paris, 1971.

[5] 5. BURGER, L. J.; ROWAN, J. & GOLDENSHON, E. S. - Creutzfeldt Jacob disease: an electroencephaphic study. Arch. Neurol. (Chicago) 26:428, 1972.

[6] 6. CAPON, A.; COLIN, F.; DELTENRE, P.; HUBERT, J. P. & FLAMET-DURANT, J. - Etude électrophysiologique de deux cas de maladie de Creutzfeldt-Jakob. Rev. Electroencephalogr. Neurophysiol. (Paris) 6:130, 1976.

[7] 7. CARTIER, L.; FERRER, S.; STUARDO, A. & PEREZ, M. - Correlación clínica, anatomopatológica y electroencefalográíca en la enfermedad de Creutzfeldt-Jakob. II Congreso Ibero-Americano de Electroencefalografia y Neurofisiologia Clínica, (Lima), 1979.

[8] 8. CARTIER, L. & GALVEZ, S. - Intranuclear corpuscles in the neurone of substantia nigra: anatomoclinical report of 11 cases of Jakob-Creutzfeldt disease. Neuro-cirugfa (Chile) 35:1, 1977.

[9] 9. CLARK, D. L. & ROSNER, B. S. - Neurophysiologic effects of general anesthetics. Anesthesiology 38:564, 1973.

[10] 10. CREUTZFELDT, O. D.; GAREY, L. J.; KURODA, R. & WOLF J. R. - The distribution of degenerating axons after small lesions in the intact and isolated visual cortex of the cat. Exp. Brain Res. 27:419, 1977.

[11] 11. CHIAPPA, K. & YOUNG, R. - The E.E.G. as a definitive diagnostic tool early in the course of Creutzfeldt-Jakob diisease. Electroenceph. Clin. Neurophysiol. 45:26, 1978.

[12] 12. GATTER, K. C. & POWELL, T. P. S. - The intrinsic connections of the cortex of area 4 of the monkey. Brain 513:101, 1978.

[13] 13. GATTER, K. C; SLOPER, J. J. & POWELL T. P. S. - An electron microscopic study of the termination of intracortical axons upon Betz cells in are 4 of the monkey. Brain 543:101, 1978.

[14] 14. GIBBS, C. J. Jr. & GAJDUSEK, D. C. - Experimental subacute spongiform virus encephalophaties in primates and other laboratory animals. Science (Washington) 182:67. 1973.

[15] 15. GLOOR, P.; KALABAY, O. & GIARD, N. - The electroencephalogram in difuse encephalopathies: electroencephalographic correlates of grey and white matter lesions. Brain 91:779, 1968.

[16] 16. GOTO, K.; UMEZAKI, H. & SUETSUGU, M. - Electroencephalographic and clnico-pathological studies on Creutzfeldt-Jakob syndrome. J. Neurol. Neurosurg. and Psychiat. 39:931, 1976.

[17] 17. HALLIDAY, A. M. - The electrophysiological study of myoclonic in man. Brain 90:241, 1967.

[18] 18. JONES, D. P. & NEVIN, S. - Rapidly progressive cerebral degeneration (subacute vascular encephalopathy) with mental disorder; focal disturbances and myoclonic epilepsy. J. Neurol. Neurosurg. and Psychiat. 17:148, 1954.

[19] 19. KIRSCHBAUM, W. R. - Jakob-Creutzfeldt Disease. Elsevier (New York) 1968.

[20] 20. LAMPERT, P. W.; GAJDUSEK, D. C. & GIBBS, C. J. Jr. - Pathology of dendrites in subacute spongiform virus encephalopathies. Advances in Neurology (New York) 12:465, 1975.

[21] 21. LEE, R. G. & BLAIR, R. D. G. - Evolution of EEG and visual evoked response changes in Jakob-Creutzfeldt disease. Electroenceph. Clin. Neurophysiol. 35:133, 1973.

[22] 22. MASTERS, C. L. & RICHARDSON, E. P. - Subacute spongiform encephalopathy (Creutzfeldt-Jakob disease). Brain 101:333, 1978.

[23] 23. MATSUMOTO, H. & AJMONE-MARSAN, C. - Cortical cellular phenomena in experimental epilepsy: interictal manifestation. Exp. Neurol. 9:286, 1964.

[24] 24. MATSUMOTO, H. & AJMONE-MARSAN, C. - Cortical cellular phenomena in experimental epilepsy: ictal manifestations. Exp. Neurol. 9:305, 1964.

[25] 25. MORRELL, F.; BERGEN, D.; HOEPPNER, T.; LOLAS, F.; FEXEIRA, C; BECK, E. & SMITH, D. - Epileptiform activity, steady potential shift and visual evoked response in Creutzfeldt-Jakob disease. Electroenceph. Clin. Neurophysiol. 45:26, 1978.

[26] 26. NEVIN, S.; MC MENEMEY, W. H.; BEHRMAN, S. & JONES, D. P. - Subacute spongiform encephalopathy: a subacute form of encephalopatty attributable to vascular dysfunction (Spongiform cerebral atrophy). Brain 36;762, 1960.

[27] 27. PEDLEY, T. A. - The pathophysiology of focal epilepsy neurophysiological considerations. Ann. Neurol. (Boston) 3:2, 1978.

[28] 28. PRINCE, D. A. - The deporalization shift in epileptic neurons. Exp. Neurol. 21:467, 1968.

[29] 29. PRINCE, D. A. - Electrophysiology of epileptic neurons spike generations. Electroenceph. Clin. Neurophysiol. 26:476, 1969.

[30] 30. RAYPORT, M. - Electroencephagraphic, corticographic and intracerebral potentials in two anatomicaly verified cases of Creutzfeldt-Jakob disease. Electroenceph. Clin. Neurophysiol. 15:921, 1963.

[31] 31. SZENTAGOTHAI, J. - Architecture of the Cerebral Cortex in Basic Mechanisms of Epilepsies. Little Brown and Co., Boston, 1969, pp. 13.

[32] 32. TESANER, D. & FOUCHER, A. - Corrélations entre les variations de la vigilance, l'activite EEG, la respiration et les clonies dans a cas de maladie de Creutzfeldt-Jakob. Rev. Electroencephalogr. Neurophysiol. 8:354, 1978.

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Diversidad sindromatica de la enfermedad de Creutzfeldt-Jakob: correlato neurofisiologico e histopatologico

Syndromatic heterogeneity of Creutzfeldt-Jakob disease: neurophysioíogical and histopathological correlates

Resúmenes

Fechas de Publicación