Standard therapeutic regimens of praziquantel for neurocysticercosis use daily doses of 50 mg/kg for 15–21 days, with prolonged remission being achieved in 60–80% patients. In this prospective study, 100 mg/kg daily was used for 10 days in 13 patients aged 32 ± 15 years (mean ± SD) with severe intra-, extraparenchymal or mixed forms of neurocysticercosis. Patients were monitored with computerized tomography and cerebrospinal fluid (CSF) examination on days 1, 5 and 10. Full blood count, sedimentation rate, blood sugar, urea, creatinine, bilirubin, liver transaminases, alkaline phosphatase, urine analysis and electrocardiogram were carried out before and after treatment. Doses of dexamethazone and of other drugs used concomitantly were controlled. There was no toxicity, clinical or detected by the methods employed in the study. After 22 ± 5 (mean ± SD) months follow-up, 6 patients needed ventriculoperitoneal shunting, 2 had died, 7 were improved and led useful lives and 4 were in prolonged remission. There was no correlation between serum or CSF praziquantel correlation and outcome of treatment. The proposed regimen is well tolerated, may be as efficient as previously advocated regimens, requires less hospitalization time and may be adopted routinely for therapy of neurocysticercosis.

Relato do caso de paciente do sexo feminino com 40 anos de idade, com quadro progressivo de diminuição de força, disfonia, disfagia, dispnéia e hiperidrose. O exame físico revelava contratura em flexão das mãos, déficit de força muscular, fasciculações, mioquimia facial e pseudomiotonia nas mãos, A eletromiografia mostrou atividade elétrica contínua em repouso. O estudo histoquímico muscular revelou atrofia de fibras do tipo II, enquanto a microscopia eletrônica mostrou dilatação importante das cisternas do retículo sarcoplasmático. O diagnóstico de síndrome de Isaacs foi firmado. Excelente resposta clínica ao emprego de carbamazepina foi observada. São discutidos e revistos vários aspectos relacionados a essa rara síndrome.

The wide clinical spectrum of neurocysticercosis has led to many attempts at clinical, radiological, CSF and other classifications. Based on an objective review of the relevant literature and on a prospective study of 42 patients with active neurocysticercosis, a new classification is proposed, based on clinical, tomographic, magnetic resonance and CSF evidence of viability of cysts. The first step is to define whether the disease is active or not. Inactive disease may be parenchymal calcifications or hydrocephalus. Active disease may be intraparenchymal, extraparenchymal or mixed. Statistical analysis of 42 cases with active disease shows intraparenchymal disease to occur in younger patients, perhaps more frequently in females, and to have a better prognosis than extraparenchymal of mixed disease. The latter appears to have the worst prognosis. Therapeutic implications are that only active disease warrants etiological therapy. There remain doubts about the best therapy for some infrequent subtypes of extraparenchymal and mixed disease.

No abstract

Cognitive function of patients on monotherapy specific for their epileptic syndrome has been studied infrequently. We evaluated 7 patients with symptomatic localised epilepsies (SEL) on phenytoin aged 30 +/- 12 (mean +/- standard deviation) years, 8 with idiopathic generalised epilepsies on sodium valproate aged 18 +/- 4 years, 16 with SEL on carbamazepine aged 28 +/- 11 years, and 35 healthy controls aged 27 +/- 11 years. All subjects were of normal intelligence, educated appropriately to age, and led productive lives in the community. Two of the patients on carbamazepine and one on valproate had less than five partial, absence or myoclonic seizures monthly, the remaining were controlled. Carbamazepine serum concentrations were 12 +/- 5 micrograms/ml, phenytoin were 23 +/- 7, and valproate were 62 +/- 23 (mean +/- sd). Tests included immediate recall and recognition for pictures, Stroop test, delayed recall and recognition of pictures. Patients on phenytoin and valproate performed significantly worse than controls on immediate recall, and patients on carbamazepine performed significantly worse than controls in Stroop test (p < 0.01). The results indicate relatively minor effects of the epileptic syndromes and of phenytoin, carbamazepine and valproate on cognition of patients with controlled epilepsy leading productive lives in the community. We conclude that the cognitive deficit found in chronic epileptic patients on poly-therapeutic drug regimen must be multifactorial, and that future studies need to control for all possible variables in order to achieve meaningful results.

Between 1982 and 1988 24 women and 6 men with myasthenia gravis were included in a prospective and standardized diagnostic and therapeutic protocol. Age varied between 10 and 74 years (34 +/- 16, mean +/- standard deviation). Three patients with the ocular form were treated with pyridostigmine. Four patients with the generalized form and advanced age received steroids and/or azathioprine. Twenty-three patients with the generalized form underwent thymectomy followed by prednisone and/or azathioprine. One patient died after complex thymectomy for invasive thymoma. Another died soon after admission in myasthenic/cholinergic crisis. Two other patients had minor complications of thymectomy. Of 19 patients followed for 1-60 (mean 24) months, 11 (58%) are in remission, one of them without any medication.

Barbiturates are considered first line antiepileptic drugs in third world countries due to traditional and economic reasons. This prospective uncontrolled study of 52 patients aged 15 to 64 years (mean 24) demonstrates that patients who become refractory to barbiturates are mainly those with partial seizures with or without generalization or with a focal EEG abnormality (71%). Seizures tend to become refractory approximately 6 years after barbiturates were started. Progressive barbiturate withdrawal over a period of two to 8 months (mean 5) with institution of treatment with carbamazepine, phenytoin or sodium valproate allowed complete barbiturate withdrawal in 42 of the 52 patients (81%). Furthermore monthly seizure frequency in those in whom barbiturates were withdrawn decreased from 7.1 to 1.7 per patient. An improvement in mental status was observed but not measured. These results show that barbiturates should not be first-choice drugs in patients who have a chronic disease such as epilepsy, and indicate a schedule for barbiturate withdrawal which is safe and independent of hospitalization or monitoring of antiepileptic drug serum concentrations.

Serum and CSF concentrations of praziquantel were analyzed in 8 patients with active neurocysticercosis aged 35 ± 16 (mean ± sd) years, in order to determine factors that may improve the therapeutic ratio. Praziquantel was given orally at 6‐h intervals for 10 days at a daily dose of 100 mg/kg. Serum concentrations were determined at 1, 2 and 4 h, and CSF concentrations at 2 h after the morning dose. Peak serum concentrations occurred between and 2 h after administration. There was a trend towards a drop in serum concentration from Days 1 through 5 to 10 of therapy. A fourfold increase in oral dose led to an eightfold rise in serum and CSF concentration, indicating saturation of hepatic metabolism. There were linear correlations (p < 0.01) between serum and CSF concentrations of praziquantel, indicating free flow across the blood‐brain barrier, above an apparent threshold, which may be related to occupation of plasma protein‐binding sites. The results indicate that monitoring of serum concentrations may be clinically useful.