Treatment of refractory partial status epilepticus with multiple subpial transection: case report.

Status epilepticus (SE) represents a medical emergency that annually affects 60,000--150,000 individuals in the United States. Selective neuronal loss in vulnerable areas has been pathologically demonstrated following convulsive SE primarily affecting the limbic system, thalamus and cerebellum. Morbidity in those cases that follow refractory SE (RSE) is poorly documented. There have been anecdotal reports of surgical treatment for this condition, especially secondary to brain lesions. We report a 6-year-old patient who was in RSE for 60 days, without a brain lesion documented by MRI. The patient underwent multiple subpial transection (MST) of the sensorimotor cortex, which by ictal EEG and ictal SPECT proved to be the epileptogenic zone. We conclude that MST should be considered as an alternative treatment for refractory partial SE.

Uso del estimulador vagal, en la epilepsia refractaria: una alternativa terapéutica / Vagus nerve stimulation in the management of intractable epilepsy

La utilización del estimulador vagal constituye una alternativa válida de tratamiento paliativo en aquellas epilepsias refractarias que no pueden ser tratadas mediante técnicas quirúrgicas a cielo abierto (lobectomias, lesionectomías, desconexiones). Se describen fundamentos del procedimiento, indicaciones del mismo, técnica quirúrgica y experiencia de los autores.

Uso del estimulador vagal, en la epilepsia refractaria: una alternativa terapéutica / Vagus nerve stimulation in the management of intractable epilepsy

La utilización del estimulador vagal constituye una alternativa válida de tratamiento paliativo en aquellas epilepsias refractarias que no pueden ser tratadas mediante técnicas quirúrgicas a cielo abierto (lobectomias, lesionectomías, desconexiones). Se describen fundamentos del procedimiento, indicaciones del mismo, técnica quirúrgica y experiencia de los autores. (AU)

Extratemporal epilepsy in children: candidate selection and surgical treatment.

From June 1988 to June 1998, 60 children with extratemporal epilepsies (EE), most of whom were symptomatic, underwent surgery. All patients were studied by means of CT scanning, MRI and scalp EEG. Video-telemetry was used in 40 cases. Intracranial electrodes were placed in 10. Intraoperative ECoG was used in the 35 children who underwent resective procedures and in the 25 in whom disconnection was performed. Surgical procedures were as follows: 24 lesionectomies, 25 disconnecting procedures, 7 polectomies and/or lobectomies, 3 corticectomies and 1 anatomical hemispherectomy. After at least 1 year's follow-up in 48 children, to date 38 are in Engel class I, 7 in class II, 1 in class III and 2 in class IV. That is to say, in 46 of the 48, surgical outcomes ranges from very good to at least worthwhile, as reflected in their classification in Engel class III.

[Rasmussen syndrome]. / Síndrome de Rasmussen.

INTRODUCTION: Rasmussen's encephalitis is a neurological disorder probably of immunological origin, characterized by intractable epilepsy, neurological deterioration and the presence of antibodies against R3 glutamate receptors. OBJECTIVE: To present the experience of an Argentinian Paediatric Neurology Service during the past 7 years. MATERIAL AND METHODS: We analyzed the clinical histories of 12 patients with RS as defined by the diagnostic criteria proposed by Hart et al. We evaluated the following parameters: neurological examination, neuropsychological assessment, types of crises, age of onset, frequency, distribution, electroencephalogram, video EEG, evoked potentials, neuro-radiological studies; CT and cerebral MR using gadolinium, SPECT, lumbar puncture and cerebral biopsy. RESULTS: The crises were of simple partial motor type in 12 cases, secondarily generalized partial crises in 7 cases, complex partial crises in 4 cases and partial and generalized motor states in 8 cases. RESULTS. The ictal EEGs during the continuous partial crises showed contralateral focal slow activity of the affected side of the body in 30% of cases. The neuro-radiological studies (CT and MR) showed signs of cerebral hemiatrophy in all 12 cases, hyper-intense images in the T2 sequences of the fronto-temporo-parietal regions in 5 cases, an asymmetric megacisterna magna in 1 case and hypoplasia of the cerebellar vermis in another case. CONCLUSIONS: The continuous partial crises showed little correlation with the electro-encephalography. Following administration of i.v. gammaglobulin there was a transient halt in progression of the neurological deterioration and control of the crises in 55% of the cases. Surgery continued to be the treatment recommended for control of the epileptic crises.

Dysembryoplastic neuroepithelial tumor: morphological, immunocytochemical, and deoxyribonucleic acid analyses in a pediatric series.

Overtreatment by radiotherapy and/or chemotherapy for central nervous system tumors in infancy and childhood may be deleterious, so the recognition of surgically curable clinicopathological entities is mandatory. The dysembryoplastic neuroepithelial tumor is a complex multinodular lesion consisting of glial nodules, associated with a specific glioneuronal element and/or with focal cortical dysplasia, and occurring in young patients presenting with intractable, mostly complex partial, seizures without neurological deterioration. We report on 14 patients; 9 were from a series of 600 pediatric patients with intracranial central nervous system tumors studied at a single institution from 1988 to 1993, and 5 were referred from other pediatric hospitals. Six tumors were frontal, six were temporal, one was parietal, and one was occipitoparietal. Computed tomographic scans disclosed hypodense lesions with cystic appearances in 4 patients and slight focal postcontrast enhancements in only 2 patients, whereas magnetic resonance imaging, available for 7 of 14 patients, showed hypointense lesions in T1-weighted images and hyperintense lesions in T2-weighted images. Deformities of the overlying cranium were also observed in five patients. The age range at the time of surgery (excluding a 20-year-old male patient who underwent surgery at the main pediatric hospital) was 2.6 to 13 years, with a mean of 6.68 years. The male to female patient ratio was 10:4, and the duration of symptoms was 0.2 to 6 years.(ABSTRACT TRUNCATED AT 250 WORDS)

Siamesas pigópagas. Su evolución 14 años después de la separación quirúrgica / Pygopagus twins. Fourteen years after surgical separation

Se presenta la descripción , tratamiento quirúrgico y evolución de un par de gemelas pigópagas , asistidas en 1980.Las niñas compartían el sacro y la columa desde la 3º vértebra lumbar, el saco dural y el periné posterior,presentando un orificio anal común.Estaban unidas por una amplia superficie ,sin otras malformaciones.Pesaron al nacer 4.800 gr. Luego de efectuárseles colostomía , fueron separadas a los 45 días de vida.el efecto de la piel en ambas,se cubrió con reoperaciones ,obteniendo finalmente una aceptable continencia anal;su hermana en cambio, a quien se asignó un ano común ,tiene continencia defectuosa.Ambas presentaron vejiga neurogénica: una de ellas conserva buena función renal a expensas de un único riñón funcionante y requiere cateterismo intermitente; la otra evolucionó a la insuficiencia renal, es tratada con hemodiálisis y será transplantada próximamente.Esta última ,de menor talla y peso ,presenta moderada escoliosis y diferencia de longitud de miembros que altera su marcha,por asimetría de la pelvis.el desarrollo intelectual y social fue hasta el momento ,normal en ambas

Siamesas pigópagas. Su evolución 14 años después de la separación quirúrgica / Pygopagus twins. Fourteen years after surgical separation

Se presenta la descripción , tratamiento quirúrgico y evolución de un par de gemelas pigópagas , asistidas en 1980.Las niñas compartían el sacro y la columa desde la 3§ vértebra lumbar, el saco dural y el periné posterior,presentando un orificio anal común.Estaban unidas por una amplia superficie ,sin otras malformaciones.Pesaron al nacer 4.800 gr. Luego de efectuárseles colostomía , fueron separadas a los 45 días de vida.el efecto de la piel en ambas,se cubrió con reoperaciones ,obteniendo finalmente una aceptable continencia anal;su hermana en cambio, a quien se asignó un ano común ,tiene continencia defectuosa.Ambas presentaron vejiga neurogénica: una de ellas conserva buena función renal a expensas de un único riñón funcionante y requiere cateterismo intermitente; la otra evolucionó a la insuficiencia renal, es tratada con hemodiálisis y será transplantada próximamente.Esta última ,de menor talla y peso ,presenta moderada escoliosis y diferencia de longitud de miembros que altera su marcha,por asimetría de la pelvis.el desarrollo intelectual y social fue hasta el momento ,normal en ambas