Rational approach to treatment options for Lennox-Gastaut syndrome.

Lennox-Gastaut syndrome (LGS) is an intractable childhood-onset epileptic encephalopathy. Seizure freedom is rare in LGS. One of the hallmarks of LGS is medical intractability, with generally poor response to antiepileptic drugs (AEDs). Nevertheless, several treatment options are available that can mitigate the severity of seizures and curtail their frequency. New AEDs have been validated in randomized, controlled trials for the treatment of seizures in LGS. In some cases, nonpharmacologic options may be effective, although more data are needed to confirm efficacy outcomes. Comprehensive patient assessments are critical to achieve an optimal AED treatment regimen and minimize the potential for adverse effects.

Laparoscopic Thal fundoplication in mentally retarded children.

BACKGROUND: An increasing number of reports indicate that Thal fundoplication is the procedure of choice in mentally retarded children. With the advent of laparoscopy, Nissen's fundoplication seems to have been repopularized. However, the choice of the operative technique should be based on the merits of the procedure itself rather than the laparoscopic feasibility. The aim of this study is to determine if laparoscopic Thal fundoplication is beneficial for mentally retarded children. METHODS: Between November 1993 and 1994 laparoscopic Thal fundoplication was performed in 15 mentally handicapped children; 13 also had a feeding gastrostomy. Age varied from 1.5 to almost 17 years (mean 7 years). Mean weight was 18 kg (5-50 kg). All patients underwent an upper GI study and endoscopy as well as pre- and 3 months postoperative pH study. Indications for the procedure were reflux esophagitis in 11 and feeding problems with silent reflux in 4. RESULTS: The laparoscopic procedure was converted in the second patient because of bleeding in the hiatus. No further procedure-related intra- or postoperative complications occurred. The mean hospitalization was 3.7 days. No symptomatic postoperative gastroesophageal reflux has been observed. All children have undergone postoperative pH studies, which displayed silent reflux in two. Gastrostomy feeding is well tolerated. CONCLUSION: We conclude that Thal fundoplication can be performed laparoscopically in mentally retarded children. The laparoscopic results are comparable to the open Thal procedure.

[Stereotaxic brain operations in erethitic imbecility (author's transl)]. / Stereotaktische Hirnoperationen bei erethischer Imbecillität.

Long-term results of stereotaxic operations in 23 patients with severe erethitic (aggressive-destructive) imbecility were evaluated. Target areas in the thalamus, electrocoagulated in separate bilateral operations, were the dorsomedial nucleus and the internal medullary lamina. Definite tranquilization was achieved in 50% and acquiescence in 27% of patients, resulting in a positive effect on more than 75% of patients. The postoperative course showed a constant level of quiet behavior in only 25% of patients ("static course"), whereas 50% of patients showed an oscillating course leading to subsequent improvement and later stabilization ("undulant course"). The final outcome was improvement and later stabilization ("undulant course"). The final outcome was not improved in 25% of patients. Additional therapeutic measures involving adaptation of psychopharmacotherapy improvements in surroundings, and play and working therapy were occasionally effective (Fig 3). In the pneumoencephalograms of these patients a new distinctive variant was encountered that awaits confirmation: typically, a definite increase in thalamic height, a decrease in ventricular width, and a relative diminution of base line length was found. Stereotaxic thalamotomy in erethitic imbeciles is a therapeutic method with a 75% long-term effectiveness and should be considered a proven therapeutic alternative.