Alhan Shamseddine

Quality of life after surgery for intractable partial epilepsy in children: A cohort study with controls

PURPOSE Investigate if quality of life (QOL) normalizes on long-term follow-up after surgery for partial epilepsy in children. METHODS This is a cohort study with controls in which a consecutive cohort of nineteen 2-14-year-old children who underwent focal resections for intractable partial seizures between 1996 and 2006, were matched with 19 non-surgery intractable partial epilepsy patients, and with 19 healthy subjects. The two epilepsy groups were matched for age, sex, socio-economic status (SES), cognitive level, seizure type, and seizure frequency. The healthy group was matched with the two epilepsy groups for age, sex, SES, and cognitive level. QOL was assessed using the QOLCE (Quality of Life in Childhood Epilepsy Questionnaire). RESULTS In the surgery group (follow-up 3.84+/-2.26 years), 78.9% had Engel class-I versus 21.1% in non-surgery (p=0.01) (follow-up 3.44+/-2.95 years). Surgery patients were similar to healthy subjects in the social, emotional, cognitive, behavioral, and overall QOL (p>0.05) but had lower scores in the total QOL, physical, and health domains (p<0.05). Surgery patients scored better than non-surgery in the behavioral domain and the HASES (Hague Side Effects Scale) score (p<0.05). Non-surgery patients scored worse than healthy in total QOL, physical, behavioral, health, and overall QOL (p<0.05). IQ, HASS (Hague Seizure Severity Scale), and HASES scores were positively associated with total QOL score (p<0.05). Subgroup analysis on seizure-free surgery patients showed that they did not differ from healthy subjects in any of QOL domains (p>0.05, power>0.8). CONCLUSION Our data indicate that epilepsy surgery for partial seizures in children is associated with better QOL as compared to children with intractable epilepsy who are not operated on, and suggest that in those who achieve seizure freedom normal QOL may at least potentially be possible.

Marked benefits in physical activity and well-being, but not in functioning domains, 2 years after successful epilepsy surgery in children.

In this first study comparing epilepsy-specific quality-of-life measures of children after epilepsy surgery (2.4 years after focal resection) with those of a matched comparison group of nonoperated patients, seizure severity, medication side effects, overall quality of life, general health, physical activity, and well-being were better in surgical patients (70.6% seizure free vs 8.3%). Cognitive, social, and behavioral functioning did not differ, suggesting that these may require additional interventions during postsurgical follow-up.

Stages of status epilepticus in the developing brain.

BACKGROUND Adult rats undergo five distinct electrographic stages during status epilepticus (SE). Whether developing animals manifest those stages is not yet known. GOALS Determine in the kainic acid (KA) model: (1) the EEG stages of SE in P15 and P35 rats; (2) the relative susceptibilities of these two age groups to develop SE; and (3) the effect of phenobarbital on SE stages. MATERIALS AND METHODS Experiment 1: Three groups of P15, and three of P35 rats received intraperitoneally (i.p.) low (5 mg/kg), intermediate (10 mg/kg), or high (15 mg/kg) KA doses. Experiment 2: One group of P35 rats received KA (12 mg/kg), and one KA and phenobarbital (70 mg/kg i.p.). EEGs were recorded through intracranial electrodes and were reviewed and staged blindly. RESULTS Both age groups manifested the five EEG stages of SE, but these occurred at the low dose in the P15 rats, and at the intermediate and high doses in the P35 rats. Unlike P35 rats, P15 rats were less likely to progress through all five stages, and had different behavioral manifestations that did not segregate into distinct stages. Phenobarbital caused an initial increase in paroxysmal beta discharges and in tonic activity and scratching. It subsequently resulted in less severe and shorter stages of SE. CONCLUSION Both P15 and P35 rats can progress through the five distinct electrographic stages of SE. However, P15 rats are less likely to progress through all these stages. They also have a lower seizure threshold and different behavioral manifestations that do not segregate into separate stages. Phenobarbital shortens and modifies the behavioral and electrographic stages of SE.

Pattern-induced partial seizures with repetitive affectionate kissing: An unusual manifestation of right temporal lobe epilepsy

We report a case of recurrent partial seizures that were often precipitated by looking up a flight of stairs and included spitting as well as repetitive affectionate kissing automatisms. These seizures were shown by long-term video/EEG monitoring to be of right temporal origin and completely subsided after right temporal lobectomy. This case is unique because: (1) The patient had partial rather than primarily generalized pattern-induced seizures. (2) Affectionate kissing automatisms were a part of his partial seizures and, to our knowledge, have not been reported in the literature before.

Epilepsy surgery in a developing country (Lebanon): ten years experience and predictors of outcome

We present our 10-year experience and preoperative predictors of outcome in 93 adults and children who underwent epilepsy surgery at the American University of Beirut. Presurgical evaluation included video-EEG monitoring, MRI, neuropsychological assessment with invasive monitoring, and other tests (PET, SPECT, Wada). Surgeries included temporal (54%), extratemporal (22%), and multilobar resections (13%), hemispherectomy (4%), vagal nerve stimulation (6%), and corpus callosotomy (1%). Mesial temporal sclerosiswas the mostcommonaetiology (37%). After resective surgery, 70% had Engel class I, 9% class II, 14% class III, and 7% class IV. The number of antiepileptic drugs before surgery was the only preoperative factor associated with Engel class I (p=0.005). Despite the presence of financial and philanthropic aid, many patients could not be operated on for financial reasons. We conclude that advanced epilepsy presurgical workups, surgical procedures, and favourable outcomes, comparable to those of developed countries, are achievable in developing countries, but that issues of financial coverage remain to be addressed.

Refractory Calculation‐induced Idiopathic Generalized Epilepsy: A Case Report and Review of the Literature

Summary:  We report a case with calculation‐induced idiopathic generalized epilepsy (IGE) that, unlike most patients with IGE, was refractory to medications. This patient had a family history of (1) a similar condition in a relative of hers who, however, did not have identical manifestations, and (2) a mother who had migraine. Our observations illustrate that the occurrence of IGE in families usually follows rather complex patterns of inheritance and that some of them can be refractory to therapy.

Two new familial severe infantile spasm syndromes in males

We describe two new familial severe infantile spasm syndromes (ISSs) unrelated to Aristaless-related homeobox (ARX) gene mutation. Family A contains two male siblings each with dysmorphism, profound psychomotor delay, gastroesophageal reflux, infantile spasms, hypsarrhythmia, prominent independent central apneas, and early death. Family B contains two male siblings with dysmorphism, profound psychomotor delay, ambiguous genitalia, macular hypoplasia, neurosensory hearing deficit, gastroesophageal reflux, infantile spasms, no hypsarrhythmia, apneas, and early death in one sibling. Etiologic workup and ARX gene sequencing were negative. This indicates that several familial ISSs exist but are not genetically characterized.

Epilepsy surgery in a developing country (Lebanon)

We present our 10-year experience and preoperative predictors of outcome in 93 adults and children who underwent epilepsy surgery at the American University of Beirut. Presurgical evaluation included video-EEG monitoring, MRI, neuropsychological assessment with invasive monitoring, and other tests (PET, SPECT, Wada). Surgeries included temporal (54%), extratemporal (22%), and multilobar resections (13%), hemispherectomy (4%), vagal nerve stimulation (6%), and corpus callosotomy (1%). Mesial temporal sclerosiswas the mostcommonaetiology (37%). After resective surgery, 70% had Engel class I, 9% class II, 14% class III, and 7% class IV. The number of antiepileptic drugs before surgery was the only preoperative factor associated with Engel class I (p=0.005). Despite the presence of financial and philanthropic aid, many patients could not be operated on for financial reasons. We conclude that advanced epilepsy presurgical workups, surgical procedures, and favourable outcomes, comparable to those of developed countries, are achievable in developing countries, but that issues of financial coverage remain to be addressed.

Landau kleffner syndrome

INTRODUCTION: The Landau Kleffner syndrome (SLK) is associated with paroxystic alterations of the electroencephalogram which are intensified during sleep, with acquired aphasia and epilepsy, in 75 83% of the cases. The syndrome is associated with other features, such as personality disorders presenting as autistic behaviour, cognitive regression and in some cases, motor dysfunction. The epileptic activity appears to be responsible for the disorder. Treatment with anti epileptic drugs is ineffective in many cases, although there may be periods of spontaneous improvement, or there may be permanent sequelas of language. Design. A systematic revision of one case. CASE REPORT: A five year old boy with no previous clinical history had, at the age of four years, presented with behaviour changes and aphasia, accompanied by paroxystic changes on the EEG and nocturnal polysomnogram. On cranial CT there was a mid line cyst. He had had no seizures. Treatment. Treatment with carbamazepine led to clinical improvement in behaviour, reduction in the paroxysms and appearance of sleep spindles, but little effect on the degree of aphasia. CONCLUSIONS: The case described is a variant of SLK, with no epileptic seizures, some improvement on carbamazepine and a mid line cyst.