Unilateral mesial temporal atrophy after a systemic insult as a possible etiology of refractory temporal lobe epilepsy: case report

Cukiert, Arthur; Andrioli, Mario Sergio Duarte; Caner-Cukiert, Ana Regina

doi:10.1590/S0004-282X1997000400023

Abstracts

Mesial temporal sclerosis is the main pathological substrate present in refractory temporal lobe epilepsy and its presence is often related to the occurrence of febrile seizures in infancy. There is an on-going discussion on the nature of mesial temporal sclerosis as it related to epilepsy: cause or consequence. A previously normal child developed hyperosmolar coma after abdominal surgery at the age of 6. Three months afterwards he developed simple and complex partial seizures with an increasing frequency and refractory to multiple mono-and polytherapic drug regimens. He was evaluated for surgery at the age of 13. Ictal and interictal recordings showed left temporal lobe abnormalities. Early CT scaning suggested left temporal atrophy. MRI showed mesial temporal sclerosis. Neuropsichological testing showed verbal memory deficits and he passed a left carotid artery amytal injection. He was submitted to a cortico-amygdalo-hippocampectomy and has been seizure-free since then. The clinical data obtained from this patient suggest that at least in this case mesial temporal sclerosis would be related to the cause of epilepsy and not resultant from repeated seizure activity.

epilepsy; mesial temporal sclerosis; systemic insult; febrile convulsions

A esclerose mesial temporal é o principal substrato anatomo-patológico envolvido na epilepsia refratária do lobo temporal e está frequentemente associada à ocorrência de convulsões febris na infância. Persiste até o momento intensa discussão se a esclerose mesial seria causa ou consequência da síndrome epiléptica. Uma criança previamente normal, desenvolveu coma hiperosmolar após intercorrência em cirurgia abdominal aos 6 anos de idade. Após 3 meses iniciaram-se crises parciais simples e complexas em frequência ascendente e refratárias a múltiplos esquemas terapêuticos em mono- e politerapia. Ele realizou investigação pré-operatória para epilepsia aos 13 anos de idade. Registros eletrencefalográficos de superfície ictais e interictais mostraram anormalidades temporais esquerdas. TC de crânio antiga, próxima ao insulto sistêmico, já sugeria atrofia do lobo temporal. RMN demonstrou esclerose mesial temporal. Testagem neuropsicológica mostrou déficit de memória verbal e este paciente apresentou o padrão passa-ipsi/falha contralateralmente no teste de amital sódico. Ele foi submetido a córtico-amígdalo-hipocampectomia e está sem crises desde então. Os achados anátomo-patológicos são de esclerose mesial. Os dados obtidos neste paciente sugerem que, ao menos neste caso, a esclerose mesial temporal está possivelmente relacionada à causa da epilepsia e não à presença de crises recorrentes.

epilepsia; esclerose mesial temporal; insulto sistêmico; convulsão febril

Unilateral mesial temporal atrophy after a systemic insult as a possible etiology of refractory temporal lobe epilepsy: case report

Esclerose mesial temporal unilateral após insulto sistêmico como possível etiologia de epilepsia refratária do lobo temporal: relato de caso

Arthur Cukiert^I; Mario Sergio Duarte Andrioli^II; Ana Regina Caner-Cukiert^III

^IServiço de Cirurgia de Epilepsia do Hospital Brigadeiro (SCEHB) e Clínica de Diagnóstico e Terapêutica das Epilepsias (CDTE), São Paulo: Neurocirurgião, Chefe do SCEHB

^IIServiço de Cirurgia de Epilepsia do Hospital Brigadeiro (SCEHB) e Clínica de Diagnóstico e Terapêutica das Epilepsias (CDTE), São Paulo: Neurocirurgião, SCEHB

^IIIServiço de Cirurgia de Epilepsia do Hospital Brigadeiro (SCEHB) e Clínica de Diagnóstico e Terapêutica das Epilepsias (CDTE), São Paulo: Psicóloga, Chefe do Setor de Neuropsicologia, CDTE

ABSTRACT

Mesial temporal sclerosis is the main pathological substrate present in refractory temporal lobe epilepsy and its presence is often related to the occurrence of febrile seizures in infancy. There is an on-going discussion on the nature of mesial temporal sclerosis as it related to epilepsy: cause or consequence. A previously normal child developed hyperosmolar coma after abdominal surgery at the age of 6. Three months afterwards he developed simple and complex partial seizures with an increasing frequency and refractory to multiple mono-and polytherapic drug regimens. He was evaluated for surgery at the age of 13. Ictal and interictal recordings showed left temporal lobe abnormalities. Early CT scaning suggested left temporal atrophy. MRI showed mesial temporal sclerosis. Neuropsichological testing showed verbal memory deficits and he passed a left carotid artery amytal injection. He was submitted to a cortico-amygdalo-hippocampectomy and has been seizure-free since then. The clinical data obtained from this patient suggest that at least in this case mesial temporal sclerosis would be related to the cause of epilepsy and not resultant from repeated seizure activity.

Key-words: epilepsy, mesial temporal sclerosis, systemic insult, febrile convulsions

RESUMO

A esclerose mesial temporal é o principal substrato anatomo-patológico envolvido na epilepsia refratária do lobo temporal e está frequentemente associada à ocorrência de convulsões febris na infância. Persiste até o momento intensa discussão se a esclerose mesial seria causa ou consequência da síndrome epiléptica. Uma criança previamente normal, desenvolveu coma hiperosmolar após intercorrência em cirurgia abdominal aos 6 anos de idade. Após 3 meses iniciaram-se crises parciais simples e complexas em frequência ascendente e refratárias a múltiplos esquemas terapêuticos em mono- e politerapia. Ele realizou investigação pré-operatória para epilepsia aos 13 anos de idade. Registros eletrencefalográficos de superfície ictais e interictais mostraram anormalidades temporais esquerdas. TC de crânio antiga, próxima ao insulto sistêmico, já sugeria atrofia do lobo temporal. RMN demonstrou esclerose mesial temporal. Testagem neuropsicológica mostrou déficit de memória verbal e este paciente apresentou o padrão passa-ipsi/falha contralateralmente no teste de amital sódico. Ele foi submetido a córtico-amígdalo-hipocampectomia e está sem crises desde então. Os achados anátomo-patológicos são de esclerose mesial. Os dados obtidos neste paciente sugerem que, ao menos neste caso, a esclerose mesial temporal está possivelmente relacionada à causa da epilepsia e não à presença de crises recorrentes.

Palavras-chave: epilepsia, esclerose mesial temporal, insulto sistêmico, convulsão febril.

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Aceite: 02-maio-1997.

Dr. Arthur Cukiert - R. Nova York 744 apto 131 - 04560-001 - São Paulo SP - Brasil.

1. Babb TL, Brown WJ. Pathological findings in epilepsy. In Engel J (ed). Surgical treatment of the epilepsies; New York: Raven Press, 1987:511-540.
2. Babb TL, Kupfer WR, Pretorius JK. Synaptic reorganization by mossy fibers in human epileptic fascia dentata. Neuroscience 1991;42:351-363.
3. Babb TL, Lieb JP, Brown WJ. Distribution of pyramidal cell density and hyperexcitability in the epileptic human hippocampal formation. Epilepsia 1984;25:721-728.
4. Cendes F, Andermann F, Dubeau F, Gloor P, Evans A, Jones-Gotman M, Olivier A, Andermann E, Robitaille Y, Lopes-Cendes I, Peters T., Melanson, D. Early childhood prolonged febrile convulsions, atrophy and sclerosis of mesial structures, and temporal lobe epilepsy: an MRI volumetric study. Neurology 1993;43:1083-1087.
5. Falconer MA, Cavanagh JB. Clinico-pathological considerations on temporal lobe epilepsy due to small focal lesions. Brain 1959;82:483-504.
6. Falconer MA Serafetinides EA, Corsellis JA. Etiology and pathogenesis of temporal lobe epilepsy. Arch Neurol 1964;10:233-248.
7. Gloor P. Mesial temporal sclerosis: historical background and an overview from a modem perspective. In Luders H. (ed.) Epilepsy surgery; New York: Raven Press, 1991,689-703.
8. Maher J, McLachlan RS. Febrile convulsions: is seizure duration the most important predictor of temporal lobe epilepsy? Brain 1995;118:1521-1528.
9. Mathern GW, Pretorius JK, Babb T. Influence of the type of initial precipitating injury and at what age it occurs on course and outcome in patients with temporal lobe seizures. J Neurosurg 1995;82:220-227.
10. Sagar HJ, Oxbury JM. Hippocampal neuron loss in temporal lobe epilepsy: correlation with early childhood convulsions. Ann Neurol 1987;22:234-340.

Publication Dates

Publication in this collection
18 Oct 2010
Date of issue
Sept 1997

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

[1] 1. Babb TL, Brown WJ. Pathological findings in epilepsy. In Engel J (ed). Surgical treatment of the epilepsies; New York: Raven Press, 1987:511-540.

[2] 2. Babb TL, Kupfer WR, Pretorius JK. Synaptic reorganization by mossy fibers in human epileptic fascia dentata. Neuroscience 1991;42:351-363.

[3] 3. Babb TL, Lieb JP, Brown WJ. Distribution of pyramidal cell density and hyperexcitability in the epileptic human hippocampal formation. Epilepsia 1984;25:721-728.

[4] 4. Cendes F, Andermann F, Dubeau F, Gloor P, Evans A, Jones-Gotman M, Olivier A, Andermann E, Robitaille Y, Lopes-Cendes I, Peters T., Melanson, D. Early childhood prolonged febrile convulsions, atrophy and sclerosis of mesial structures, and temporal lobe epilepsy: an MRI volumetric study. Neurology 1993;43:1083-1087.

[5] 5. Falconer MA, Cavanagh JB. Clinico-pathological considerations on temporal lobe epilepsy due to small focal lesions. Brain 1959;82:483-504.

[6] 6. Falconer MA Serafetinides EA, Corsellis JA. Etiology and pathogenesis of temporal lobe epilepsy. Arch Neurol 1964;10:233-248.

[7] 7. Gloor P. Mesial temporal sclerosis: historical background and an overview from a modem perspective. In Luders H. (ed.) Epilepsy surgery; New York: Raven Press, 1991,689-703.

[8] 8. Maher J, McLachlan RS. Febrile convulsions: is seizure duration the most important predictor of temporal lobe epilepsy? Brain 1995;118:1521-1528.

[9] 9. Mathern GW, Pretorius JK, Babb T. Influence of the type of initial precipitating injury and at what age it occurs on course and outcome in patients with temporal lobe seizures. J Neurosurg 1995;82:220-227.

[10] 10. Sagar HJ, Oxbury JM. Hippocampal neuron loss in temporal lobe epilepsy: correlation with early childhood convulsions. Ann Neurol 1987;22:234-340.