Sulthiame add-on therapy in children with Lennox-Gastaut syndrome: A study of 44 patients.

PURPOSE: The aim of this study was to evaluate efficacy and tolerability of sulthiame as an add-on treatment in 44 patients with Lennox-Gastaut syndrome (LGS) refractory to other antiepileptic drugs and/or non-pharmacological treatment. METHODS: Patients were selected according to the following criteria: (1) age 4 years or older, (2) a diagnosis of LGS refractory to at least four previous antiepileptic drugs, alone or in combination. Neurologic examinations, brain magnetic resonance imaging, and repeated prolonged electroencephalography (EEG) or video-EEG studies were performed in all cases. Data on school achievements and/or neuropsychological evaluations were obtained during the follow-up of 1-3 years. Sulthiame was added in doses ranging from 5 to 30 mg/kg/day. RESULTS: Twenty-seven of 44 patients (61%) who received sulthiame as add-on therapy had a greater than 50% seizure decrease after a mean follow-up period of 20 months. Complete seizure freedom was achieved in one patient (2%). Four patients (9%) had a 25-50% seizure decrease, while seizure frequency remained unchanged in 12 (25%), and was increased in one (2%). Hyperpnoea and dyspnoea were observed in four patients, and nausea, drowsiness, and headache were seen in one patient each; however, these manifestations were transient and discontinuation of sulthiame was not necessary. Two other patients had decreased appetite, skin rash, and irritability. The adverse effects were mild and transient in these nine cases. CONCLUSION: Sulthiame as an adjunctive therapy achieved a more than 50% seizure reduction in 27 of 44 patients with LGS with only mild or moderate adverse effects.

Epilepsy of infancy with migrating focal seizures: three patients treated with the ketogenic diet.

We present three patients with epilepsy of infancy with migrating focal seizures treated with the ketogenic diet. Between February 1, 2012 and January 31, 2014, three patients who met the diagnostic criteria for migrating focal seizures in infancy at our department were placed on the ketogenic diet and followed for a minimum of seven months. Two of the three children responded well to the ketogenic diet. One of these patients became seizure-free and his neuropsychological performance also significantly improved. The other child had a seizure reduction of 75% to 99% with only weekly seizures and moderate psychomotor improvement. For these two patients who responded well to the ketogenic diet, hospital admission was not required. The remaining patient had a seizure reduction of less than 50%. Tolerability of the diet was good in all three patients. Early treatment with the ketogenic diet should be considered for epilepsy of infancy with migrating focal seizures to control seizures and status epilepticus, and avoid progressive cognitive impairment.

Experiencia inicial con cinacalcet en Argentina / Inicial experience with cinacalcet in Argentina

Introducción: el hiperparatiroidismo secundarioes una complicación frecuente de laenfermedad renal crónica. Cinacalcet, un moduladoralostérico del receptor sensor del calcio incrementasu sensibilidad a la activación por partedel calcio iónico extracelular, demostró ser efectivoen reducir los niveles de PTH. Objetivo:Evaluar la eficacia de cinacalcet en pacientes enhemodiálisis con HPTS. Material y métodos: Se realizó un estudio retrospectivo, multicéntrico, observacional, en 76 pacientes que recibieronal menos 3 meses de cinacalcet como tratamientodel HPTS...

Introduction: secondary Hyperparathyroidism is a frequent chronic renal disease complication. Cinacalcet, an allosteric modulator of the calcium sensing receptor, increases its sensitivity to activation by extracellular calcium ions, proved to be effective in reducing PTH levels. Objetive: To evaluate cinacalcet effectiveness in hemodialysis patients with HPTS. Methods: A retrospective, multicenter, observational study was carried out, on 76 patients who received Cinacalcet for at least 3 months, as a treatment for HPTS...

Type II focal cortical dysplasia: electroclinical study and surgical outcome in 31 pediatric patients.

OBJECTIVE: The aim of this study was to analyze the electroclinical features and surgical outcome of 31 pediatric patients with focal cortical dysplasia (FCD) type II. MATERIAL AND METHODS: We conducted a retrospective, descriptive study of 31 patients with FCD type II followed between 1998 and 2011. We included patients with FCD type II confirmed by histopathological examination with abnormal magnetic resonance imaging and at least 1 year of follow-up. RESULTS: All patients had severe focal epilepsy; in infancy, four of them had also had epileptic spasms, associated with hypsarrhythmia in three. Focal status epilepticus occurred in five patients (16 %) and epilepsia partialis continua in one (3.2 %). Seizures occurred during sleep in 20 (64.5 %) and in clusters in 19 (61.3 %) patients. Neurological examination showed a mild motor deficit in seven (22.8 %) patients. Interictal abnormalities were characterized by rhythmic spikes and polyspike discharges, increasing during sleep in 13 (41.9 %) patients. Average time of follow-up after surgery was 4.7 years with a median time of 4 years and a range from 1 to 9 years. Engel classification class I was found in 20 (67.7 %) and class II in 3 cases (9.6 %). There were no significant changes after an average time of follow-up of 4.7 years. CONCLUSION: Our results confirm that surgery is the best treatment option for pediatric patients with refractory focal epilepsy due to type II FCD. A statistically significant correlation was found between a good prognosis and age at epilepsy onset older than 2 years.

Encephalopathy with status epilepticus during sleep or continuous spikes and waves during slow sleep syndrome: a multicenter, long-term follow-up study of 117 patients.

PURPOSE: To retrospectively analyze the electroclinical features, etiology, treatment and prognosis of 117 patients with encephalopathy with status epilepticus during sleep (ESES) or continuous spike and waves slow sleep (CSWSS) syndrome with a long-term follow-up. METHODS: Charts of 117 patients with ESES/CSWSS syndrome followed between 1990 and 2012 were analyzed. Inclusion criteria were: (1) focal seizures or apparently generalized seizures and focal EEG epileptiform discharges; (2) further occurrence of atypical absences, and myoclonic, atonic, and/or generalized seizures; (3) cognitive impairment and/or behavior disturbances; (4) continuous spike-and-wave discharges during slow sleep in more than 85% of non-REM sleep. Patients with spike-and-wave discharges in less than 85% of slow sleep were also analyzed. KEY FINDINGS: 'Mean follow-up from onset of ESES/CSWSS was 13 years (range, 2-22 years) in the symptomatic/structural and non-idiopathic group consisting of 79 children and 10.5 years (range, 2-21 years) in the idiopathic group consisting of 38 children. The comparison of clinical findings and localization of paroxysmal EEG abnormalities (focal, multifocal, or generalized) at the different stages (before, during, and after ESES/CSWSS) and the percentage of spike-wave index during ESES/CSWSS between the symptomatic/structural and non-idiopathic and the idiopathic group was not statistically significant. SIGNIFICANCE: ESES/CSWSS syndrome is an epileptic encephalopathy with similar electroclinical findings in children with a >85% spike-wave index and those with a <85% spike-wave index. In this series of patients, the most commonly used treatments were clobazam, ethosuximide, sulthiame, alone or in combination. In refractory cases, high-dose steroids were administered. Among the AED responders, the idiopathic cases returned to normality and the structural cases returned to baseline cognitive development.