Hamed A. El-Khayat

Physical Growth and Endocrinal Disorders during Pubertal Maturation in Girls with Epilepsy

Summary:  Purpose: This study investigated the effect of epilepsy and/or antiepileptic drugs (AEDs) on the physical growth, pubertal development, and androgenic status of girls with epilepsy between ages 8 and 18 years.

Physical and Hormonal Profile of Male Sexual Development in Epilepsy

Summary:  Purpose: This study was designed to investigate the effect of epilepsy and antiepileptic drugs (AEDs) on both the physical and hormonal aspects of the sexual development of male patients with epilepsy.

Melatonin and Sleep-Related Problems in Children With Intractable Epilepsy

Children with epilepsy have high rates of sleep problems. Melatonin has been advocated in treatment of sleep disorders, and its beneficial effect has been confirmed in insomnia. The aim of this study was to assess melatonin levels in children with intractable epilepsy and its relation to pattern of sleep and characteristics of seizure disorder, as well as the effect of melatonin therapy on those parameters. The study was conducted on 23 children with intractable epilepsy and 14 children with controlled seizures. Patients were evaluated by psychometric sleep assessment and assay of diurnal and nocturnal melatonin levels. Children with intractable epilepsy received oral melatonin before bedtime. They were reassessed after 3 months. Children with intractable epilepsy had higher scores for each category of sleep walking, forcible teeth grinding, and sleep apnea. At the end of therapeutic trial, patients with intractable epilepsy exhibited significant improvement in bedtime resistance, sleep duration, sleep latency, frequent nocturnal arousals, sleep walking, excessive daytime sleepiness, nocturnal enuresis, forcible teeth grinding, sleep apnea, and Epworth sleepiness scores. There was also significant reduction in seizure severity. Thus, use of melatonin in patients with intractable seizures was associated with improvement of both many sleep-related phenomena and the severity of seizures.

Reproductive hormonal changes and catamenial pattern in adolescent females with epilepsy

Purpose: We aimed to evaluate the effect of epilepsy on the reproductive hormones levels among female patients, and to investigate the frequency of catamenial pattern of seizures.

Growth hormone levels in children and adolescents with epilepsy.

BACKGROUND Patients with epilepsy often complain of symptoms that may be caused by disturbances in their hormonal balance. Disturbances in physical growth has been previously described. The aim of this study was to evaluate the effect of epilepsy and/or anti-epileptic drugs on the physical growth of patients with idiopathic epilepsy, as well as on the growth hormone (GH) and insulin growth factor-1 (IGF-1) status in those patients. METHODS The study comprised 40 children and adolescents with idiopathic epilepsy on either valproate or carbamazepine. Anthropometric measurements [occipitofrontal circumference, weight, height, body mass index, span, and midarm circumference] were taken. Serum levels of GH before and after provocation with L-dopa and of IGF-1 were assessed. Results were compared to a matched control group. RESULTS The height measurements were reduced in patients with epilepsy compared to the controls group. Though weight values were not significantly different, the body mass indices of the patients were significantly higher than controls, especially in patients on valproate therapy. Basal GH levels showed no significantly variation between patients and controls. However, post provocation GH and IGF-1 levels were significantly lower in patients. The type of epilepsy, disease duration, and the degree of seizure control had no significant effect on the studied parameters. In conclusion, physical growth seems to be affected in patients with epilepsy. This may be due to hormonal imbalance as evident by reduced post provocation GH levels and IGF-1 levels in the included group of patients.

Fetal brain disruption sequence versus fetal brain arrest: A distinct autosomal recessive developmental brain malformation phenotype

The term fetal brain disruption sequence (FBDS) was coined to describe a number of sporadic conditions caused by numerous external disruptive events presenting with variable imaging findings. However, rare familial occurrences have been reported. We describe five patients (two sib pairs and one sporadic) with congenital severe microcephaly, seizures, and profound intellectual disability. Brain magnetic resonance imaging (MRI) revealed unique and uniform picture of underdeveloped cerebral hemispheres with increased extraxial CSF, abnormal gyral pattern (polymicrogyria‐like lesions in two sibs and lissencephaly in the others), loss of white matter, dysplastic ventricles, hypogenesis of corpus callosum, and hypoplasia of the brainstem, but hypoplastic cerebellum in one. Fetal magnetic resonance imaging (FMRI) of two patients showed the same developmental brain malformations in utero. These imaging findings are in accordance with arrested brain development rather than disruption. Molecular analysis excluded mutations in potentially related genes such as NDE1, MKL2, OCLN, and JAM3. These unique clinical and imaging findings were described before among familial reports with FBDS. However, our patients represent a recognizable phenotype of developmental brain malformations, that is, apparently distinguishable from either familial microhydranencephaly or microlissencephaly that were collectively termed FBDS. Thus, the use of the umbrella term FBDS is no longer helpful. Accordingly, we propose the term fetal brain arrest to distinguish them from other familial patients diagnosed as FBDS. The presence of five affected patients from three unrelated consanguineous families suggests an autosomal‐recessive mode of inheritance. The spectrum of fetal brain disruption sequence is reviewed.