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.

Estimulador del nervio vago: tratamiento en 158 pacientes pediátricos con un largo seguimiento / Vagus nerve stimulation: treatment of 158 pediatric patients with a long-term follow-up

Objetivo. Describir una población pediátrica de pacientes con epilepsia farmacorresistente tratada con estimulador del nervio vago en un hospital nacional de pediatría, evaluando la eficacia, la tolerabilidad y la seguridad del tratamiento. Pacientes y métodos. Se realizó un análisis retrospectivo de 158 pacientes pediátricos seguidos por epilepsia refractaria al tratamiento farmacológico y no farmacológico, incluida la cirugía, que fueron tratados con estimulador del nervio vago entre los años 2001 y 2015. Se excluyeron pacientes con encefalopatías evolutivas y cardiopatías congénitas. Resultados. Se incluyeron 158 pacientes (80 varones) con una edad media de implante de 11,4 años y un tiempo de evolución de epilepsia preimplante de 9,5 años. El tiempo de seguimiento fue de 1-15 años (mediana: 6,9 años); la edad actual de los pacientes, 2-31 años (mediana: 14,1 años). A los 24 meses postimplante, un 66,5% de los pacientes presentó una mejoría mayor o igual al 50% de las crisis previas. Sólo tres pacientes (1,8%) presentaron efectos adversos graves, 26 (16,4%) mostraron efectos adversos menores y 129 (81,8%) no mostraron efectos adversos al tratamiento. Conclusión. La terapia con estimulador del nervio vago en esta serie pediátrica con epilepsia refractaria fue eficaz, bien tolerada y segura (AU)

Aim. To describe a series of patients with drug resistant epilepsy treated with vagus nerve stimulation in a national pediatric hospital, evaluating efficacy, safety and tolerability. Patients and methods. A retrospective analysis of 158 pediatric patients with epilepsy resistant to pharmacological and non pharmacological treatment including surgery that were treated with vagus nerve stimulation between 2001-2015. Patients with progressive encephalopathies, and congenital heart disease were excluded. Results. 158 patients (80 male) were included, with a mean age at implantation of 11.4 years and a mean age at evolution of epilepsy of 9.5 years. Time of follow-up: 1-15 years (median: 6.9 years). Patient’s age at this time: 2-31 years (median: 14.1 years). Effectiveness: 66.5% of patients showed more or equal at 50% of seizure control at 24 months of implant. Just three patients showed severe side effects (1.8%). Minor side effects were seen in 26 patients (16.4%). Without side effects: 129 (81.8%). Conclusion. Vagus nerve stimulation is an effective, tolerable and safe therapy in our pediatric series with refractory epilepsy (AU)

Ketogenic diet in patients with epileptic encephalopathy with electrical status epilepticus during slow sleep.

UNLABELLED: Epileptic encephalopathy with electrical status epilepticus during slow sleep (ESES) belongs to the group of epileptic encephalopathies that often prove refractory to AED treatment. The ketogenic diet (KD) has been used as an alternative to antiepileptic drugs (AEDs) for patients with refractory epileptic encephalopathies. PURPOSE: In this retrospective study we assess the efficacy and tolerability of the KD in patients with ESES syndrome. METHODS: Between March 1, 1990 and April 1, 2013, 65 patients who met diagnostic criteria of ESES syndrome were seen at our department. Twelve of them were placed on the KD and followed for a minimum of 18 months. RESULTS: The children had previously received a mean of 5.5 different AEDs and were on a mean of 3 AEDs when the diet was started. Eighteen months after initiating the diet, seven of the initial patients (58%) remained on the diet; one patient (8.3%) had become seizure free, one (8.3%) had a 75-99% decrease in seizures, two (16.6%) had a 50-74% decrease in seizures, and the remaining three children (24.9%) had a <50% decrease in seizures. In the patient who had become seizure free and in the one who had a 75-99% seizure decrease AEDs were reduced. CONCLUSION: The KD is a well-tolerated treatment option for patients with ESES syndrome, not only for structural cases but also for those with an unknown etiology. The diet should be considered in the management of this syndrome.

Encephalopathy with status epilepticus during sleep: unusual EEG patterns.

PURPOSE: To retrospectively analyze the electroclinical characteristics, etiology, treatment, and prognosis of patients with epileptic encephalopathy with status epilepticus during sleep (ESES) with unusual EEG features and to corroborate if this series of patients is part of the ESES syndrome. METHOD: Charts of 17 patients with typical clinical manifestations of the ESES syndrome with focal ESES of non-REM sleep at onset and during the focal ESES phase, or bilateral synchronic and asynchronic ESES with a symmetric or asymmetric morphology, continuous or subcontinuous and sometimes multifocal paroxysms with or without slow-wave activity during slow sleep seen between 2000 and 2012 were analyzed. RESULTS: Mean patient follow-up from onset was 7.5 years. An idiopathic cause was found in seven patients, a structural cause in eight, and etiology was unknown in the remaining two. The median age at onset of the unusual ESES syndrome was 7 years. During the ESES phase, 15 children developed new seizure types, negative myoclonus was observed in seven patients, positive myoclonus in five, and absences in nine. Six patients had motor impairment, two had auditory verbal agnosia, and two had motor speech impairment. Attention deficit hyperactivity disorder was observed in four, aggressiveness in six, memory deficit in two, and impaired temporospatial orientation in four. The patients with focal ESES in the frontal region showed behavioral disturbances and/or motor deterioration, and in those with temporo-occipital involvement the dominant clinical manifestations were language and/or behavioral disturbances. CONCLUSION: Our patients with typical clinical manifestations of ESES syndrome but with unusual EEG patterns may be variants of this syndrome.

Ketogenic diet in pediatric patients with refractory focal status epilepticus.

UNLABELLED: The ketogenic diet (KD) has been used as an alternative treatment for patients with refractory status epilepticus (SE). PURPOSE: In this retrospective study we assess the efficacy and tolerability of the KD in patients with refractory SE. METHODS: Between March 1, 2010 and January 1, 2014, 10 patients who met the diagnostic criteria of refractory SE seen at our department were placed on the KD and followed for a minimum of 6 months. RESULTS: Ketonuria was reached within 2-4 days (mean 3 days) for all patients. Seizures stopped in two patients and five patients had a 50-75% seizure reduction within 2-5 days (mean 2.5 days) following the onset of ketonuria and within 5-7 days (mean 5 days) following the onset of the diet. Three patients had a <50% seizure reduction and all of them had severe adverse events so the diet was discontinued. Seven patients remained on the diet for 6 months to 3 years (mean 1.5 years). In all seven patients within 4 months the seizures recurred, but their quality of life did not worsen. The frequency of the seizures consisted of weekly seizures in two, monthly seizures in two, occasional seizures in two, and isolated seizures in one. All of them kept a good tolerability of the diet. CONCLUSION: The KD is an effective and well-tolerated treatment option for patients with refractory SE. In patients with focal SE secondary to inflammatory or probably inflammatory diseases, the KD should be considered earlier in the course of the treatment.

Myoclonic epilepsy in infancy: an electroclinical study and long-term follow-up of 38 patients.

PURPOSE: Myoclonic epilepsy in infancy (MEI) is characterized by brief generalized myoclonic seizures associated with generalized spike-wave paroxysms without other seizure types occurring in the first 3 years of life in developmentally normal children. In this study we analyze the electroclinical features, treatment, and outcome of 38 patients with MEI. METHODS: A retrospective chart review was conducted in 38 patients followed at the Neurology Department of the Pediatric Hospital Juan P. Garrahan in Buenos Aires, Argentina, between 1990 and 2012. KEY FINDINGS: A total of 24 boys and 14 girls were identified. The mean and median ages at seizure onset were 16 and 18 months, respectively (range 3-40 months). Ten patients (28.9%) had a family history of epilepsy, and six (15.8%) had a family history of febrile seizures. All patients had several daily brief and isolated myoclonic seizures during wakefulness and predominantly in the first two stages of sleep. Twelve children (31.5%) had reflex myoclonus, triggered by a tactile stimulus in 10 and additionally by noise and light in 2. The remaining two had photosensitive myoclonic jerks. The interictal electroencephalography (EEG) recordings evidenced generalized spike waves, polyspikes, and polyspike-wave paroxysms. The interictal EEG was normal in 12 patients. The abnormalities on the ictal EEG were similar to those on the interictal EEG. Most of the patients responded well to valproic acid. After a mean follow-up of 13.5 years, 24 patients (63%) were without treatment. At the last examination, 32 patients had normal neurologic and neuropsychological evaluations. Two patients (5.2%) had significant cognitive impairment (an IQ of 60 and 63, respectively) despite good seizure control. Four patients (10.4%) had significant learning impairment, two of whom also had attention deficit hyperactivity disorder. SIGNIFICANCE: MEI is a well-defined epileptic syndrome of unknown etiology, but likely of a genetic cause. It is self-limited and pharmacosensitive mainly to valproic acid.

Mesial temporal lobe epilepsy with hippocampal sclerosis: study of 42 children.

PURPOSE: We present the electroclinical features, treatment, and evolution of patients with mesial temporal lobe epilepsy with hippocampal sclerosis (MTLE-HS). MATERIAL AND METHODS: We analyzed the charts of forty-two patients who met the diagnostic criteria of MTLE-HS. The mean follow-up after seizure onset was 10.5 years. RESULTS: According to age, we defined three groups. The first group included nine patients that started with seizures before 2 years of age. Motor seizures were the hallmark clinical manifestation. All patients of this group also presented with motor arrest and oro-alimentary automatisms. In three of them, the interictal EEG recordings showed bilateral paroxysms predominantly in anterior regions, in addition to focal abnormalities, and two had an apparently generalized ictal pattern. The second group included 17 patients that started with seizures between 2 and 10 years of age. In this group the automatisms were also oroalimentary, but more complex and the patients had less motor manifestations. The interictal EEG recordings showed temporal abnormalities. The ictal EEG recordings showed lateralized abnormalities with a maximum in the temporal electrodes. The third group included 16 patients that started with seizures between 10 and 16 years of age. The most common clinical manifestation was abdominal aura followed by oroalimentary, gestural, and verbal automatisms. The interictal and ictal EEG recordings showed well-localized abnormalities in temporal lobes. Thirty-eight patients underwent surgical treatment. Thirty-five patients are seizure free. CONCLUSION: MTLE-HS represents a well-defined and distinct symptomatic epileptic syndrome. Surgical treatment was successful in most patients.

Benign infantile focal epilepsy with midline spikes and waves during sleep: a new epileptic syndrome or a variant of benign focal epilepsy?

OBJECTIVE: To analyze the electroclinical features and evolution of seven infants with benign infantile focal epilepsy with midline spikes and waves during sleep (BIMSE). MATERIAL AND METHODS: Seven patients were examined at our department between February 2003 and February 2009, with onset of seizures between six and 13 months of age (mean, 10.2 months; median, 11 months). Patients with cryptogenic and symptomatic focal epilepsies were excluded. Sex, age, familial history, type of seizures and AED treatment were noted and EEG monitoring, MRI and CT scanning, and developmental and psychomotor evolution were investigated. RESULTS: Patients included five males and two females. All patients suffered from seizures during wakefulness. Two of the patients (29%) did not have a recurrence. Five (71%) had sporadic seizures (ranging between two and five). One of the seven patients (14%) presented with seizures in clusters. During seizures, staring was observed in six (86%), motion arrest in five (71%), stiffening in five (71%), cyanosis in three (42%), automatisms in one (14%) and lateralizing signs in four (57%). Two patients (29%) had secondary generalisation. The duration of the seizures ranged between 30 seconds and five minutes. No status epilepticus was observed. The interictal EEG recording during sleep showed low-voltage unilateral or bilateral spikes located in the central and vertex regions, followed by slow waves in all patients. Outcome was excellent in all patients. CONCLUSION: We believe that BIMSE is a new syndrome rather than an early presentation of benign epilepsy of childhood with centrotemporal spikes, Panayiotopoulos syndrome, or a late presentation of benign focal infantile seizures.