Michel Pierre Lison

Aneurisma intracraniano na síndrome de Marfan: a case report

Vascular malformations in hereditary connective tissue diseases are reviewed. Intracavernous aneurysm of the internal carotid artery in a patient with Marfans syndrome is reported.

Estudo clínico-eletrencefalográfico longitudinal em pacientes epilépticos tratados com Ro 5-4023

Twenty two epileptic patients, with daily seizures (5 had typical absences, 3 had psychomotor seizures, 5 had myoclonic absences, one had intentional myoclonia and 8 had Lennox syndrome) were treated with 7-nitro-5-(2chlorophenyl)-3H-1,4 benzodiazepine-2(1H)-one or Ro 5-4023. Twenty of these patients had received previous treatment with other anticonvulsivant drugs with no satisfactory results. A clinical and electroencephalographical longitudinal study was made on these patients. With the use of Ro 5-4023 the seizures were completely controlled since the first day in 14 patients; in 6 there was a rapid and significant decrease in frequency and intensity of seizures; in one, out of these 6, seizures disappeared during the second week of treatment. Recurrence of seizures were observed in 8 patients. The drug is effective in a large spectrum of generalized seizures (typical absences, myoclonic absences); partial seizures, in particular the psychomotor seizures of infancy with slow spike-waves; the severe epileptic encephalopathy of infancy, Lennox type; and the degenerative epileptic syndrome. Side effects due to the drug were present in 11 patients, being of short duration in 9. In two patients there was precipitation of generalized tonic-clonic seizures; anyhow it seems that the occurrence of this complication is more frequent when one uses other types of benzodiazepines. Electroencephadographic studies showed improvement of the previous abnormalities; there was disappearance or decrease of bilateral diffuse discharges, when spontaneous or provoked by intermitent light stimuli, onset of diffuse, fast rhythm, persistance or onset of slow focal abnormalities or irritative type of discharges.

Bissincronia secundária na síndrome de Lennox

The relations between epileptogenic focus seated outside the temporal lobe and childhood epileptic encephalopathies with diffuse slow spike-waves were studied. Eleven patients (3 with clinical and electroencephalographic evidences of cortico-mesial lesions and 8 with evidences of diffuse lesions in one of the hemispheres) were selected among 32 patients suffering from Lennox syndrome. Clinical history, findings of neurological examination and focal electroencephalographic abnormalities are important factors for ascertaining the localization of the primary cerebral lesion. No physiopathogenic datum permits to exclude from the Lennox syndrome the sequels of myoclonic encephalopathy with hypsarrhythmia or the cases with clinical and electroencephalographic signs of cortical or hemispheric localization when secondary bi-synchronous electroencephalographic discharges are present and the characteristic clinical symptoms and response to therapy are observed.

Complicação rara e reversível de síndrome convulsiva: hipotonia generalizada e alterações da atividade intelectual

A case of transitory hypotonic syndrome with disturbances of the intelectual capacity is reported. The syndrome developed during the course of epileptic seizures in a child one year old. The physiopathogenic interpretation of clinical and electroencephalographic signs is attempted on the light of the present knowledge of some reticular formation disfunctions and their bioelectric significance.

Síndrome de Lennox: atualização

Present concepts on the clinical and electroencephalographical pictures of the Lennox syndrome are reviewed. The therapeutic perspectives are outlined.Present concepts on the clinical and electroencephalographical pictures of the Lennox syndrome are reviewed. The therapeutic perspectives are outlined.

Forma miopática da cisticercose

A case of pseudohypertrophic myopathy due to Cysticercus cellulosae infestation is reported. The clinical and paraclinical findings are discussed and confrontation is made with the four cases which were previously reported in the literature.

Efeitos de derivados benzodiazepines na epilepsia: estudos eletrencefalográficos longitudinais

The modifications of the electroencephalic patterns in 24 childrens with daily epileptic seizures treated by benzodiazepine derivatives were studied. The electroencephalographic findings during the longitudinal therapeutic follow-ups disclose important modifications of the subcortical mechanisms involved in the production of the convulsive seizures and paroxistical discharges. These facts are in accordance with the hypothesis that benzodiazepine derivatives act upon the non-specific thalamic reticular system. The benzodiazepine derivatives are more active upon the inhibitory system related to the thalamocortical projection system. Two hypothesis are discussed: 1 — the drugs act upon chemoreceptors present in the thalamic reticular system; 2 — the act indirectly, depressing some elements of the limbic system and modifying in this way the feed-back of subcortical circuits which discharge upon hyperactive centrencephalic neurons.

Valium endovenoso no tratamento do estado de mal epiléptico

Twenty five patients in status epilepticus were submitted to treatment by intravenous Valium. Twenty of them were afflicted by chronic cerebral disorders and the others by acute cerebral disorders. The treatment proved to be extremely efficient in the control of the seizures, specially in the cases with chronic lesions. Respiratory depression was observed in only one patient afflicted by acute cerebral lesion. Electroencephalograms obtained during the administration of Valium showed complete extinction of the paroxystic activity, which was replaced, in the normal hemisphere, by well developed beta rythm. In the injured hemisphere the background activity was quite depressed or desorganized and presented little or no beta activity. These findings and those previously reported by others allow the following conclusions: 1) intravenous Valium is presently the drug of choice for the control of any type of status epilepticus; 2) in patients with acute cerebral disorders, the administration of Valium should be made with care, because of its undersirable collateral affects; 3) the electroencephalographic examination during the administration of Valium may be important for the determination of which cerebral hemisphere is injured.

Sindrome de Lennox-Gastaut com inicio tardio: II - crises epilepticas simples e tonico-clonicas

O seguimento de 12 pacientes apresentando sindrome de Lennox-Gastaut com inicio apos os 6 anos de idade, durante o periodo medio de 2 anos e 6 meses, permitiu a identificacao dos seguintes tipos de crises epilepticas: tonicas, hemitonicas, atonicas, mioclono-atonicas, ausencias atipicas, parciais com generalizacao secundaria, tonico-clonicas, parciais com automatismos, versivas e ausencias tipicas. Durante o primeiro ano de seguimento houve predominio de crises tonicas, ausencias atipicas, crises tonico-clonicas e atonicas, respectivamente. No terceiro ano de crises tonicas, tonico-clonicas, hemitonicas e ausencias atipicas. O estudo das crises segundo a frequencia evidenciou predominio de uma ou mais crises por dia e de uma a 6 por semana. Na evolucao, as modificacoes de frequencia ocorreram, em geral, no sentido da diminuicao do numero de crises. A comparacao de nossos achados com os da literatura sobre sindrome de Lennox-Gastaut com inicio tardio evidencia, em nossa casuistica, frequencia mais elevada das crises tonicas, ausencias atipicas e das crises hemigeneralizadas. Essas manifestacoes sao, mais comumente, encontradas nos pacientes com sindrome de Lennox-Gastaut de inicio anterior aos 6 anos.

Efeito de medicamentos sobre tipos eletroclínicos de crises epilépticas na síndrome de Lennox-Gastaut

Analisamos os resultados terapeuticos obtidos com o uso de diferentes drogas (diazepam, nitrazepam, clonazepam, difenilhidantoina, barbituricos - fenobarbital e primidona -, dipropilacetato de sodio e ACTH) em 29 pacientes com sindrome de Lennox-Gastaut. Discutimos a acao de cada droga sobre as crises epilepticas (tonicas, tonico-clonicas, clonicas, mioclonicas ou mioclonoatonicas, atonicas e ausencias atipicas) durante o primeiro mes de cada esquema terapeutico. Delimitamos os campos de acao especifica de cada medicacao e verificamos a possibilidade de reduzir a incidencia de crises epilepticas retirando ou diminuindo as dosagens das drogas administradas.