Avinoam Shuper

Attention-Deficit Disorders and Epilepsy in Childhood: Incidence, Causative Relations and Treatment Possibilities

At least 20% of children with epilepsy have clinical attention-deficit hyperactivity disorder (ADHD) compared to 3% to 7% of the general pediatric population. Several mechanisms may account for the high prevalence, such as a common genetic propensity, noradrenergic system dysregulation, subclinical epileptiform discharges, or even seizures, antiepileptic drug effects, and psychosocial factors. At the same time, children with attention-deficit hyperactivity disorder have a higher than normal rate of electroencephalography abnormalities (5.6-30.1% vs. 3.5%). Methylphenidate treatment is equally efficient in children with isolated attention-deficit hyperactivity disorder and in children with attention-deficit hyperactivity disorder and epilepsy (70%-77%). Electroencephalography screening in patients with attention-deficit hyperactivity disorder in the absence of other clinical indications or before starting methylphenidate treatment is not currently indicated. Methylphenidate is considered safe for use in children who are seizure-free. However, the few reports of seizure aggravation in methylphenidate-treated children with uncontrolled epilepsy have raised concern.

Methotrexate treatment protocols and the central nervous system: significant cure with significant neurotoxicity.

Methotrexate can influence the central nervous system through several metabolic toxic pathways. These effects can be categorized as immediate, acute to subacute, or chronic neurologic syndromes. The acute to subacute syndrome occurs frequently in acute lymphoblastic leukemia treatment protocols, generally manifesting with focal neurologic signs and changes seen on magnetic resonance imaging and single photon emission computed tomography. While in some patients the neurotoxicity is transient and benign and allows for continuation of chemotherapy, in others it can be quite severe and debilitating, leading to permanent neurologic deficits. The need to modify the treatment protocols when neurotoxicity appears is not fully established. It is also unknown whether the use of sufficient amounts of leucovorin can overcome the toxic effects of the drug. (J Child Neurol 2000;15:573-580).

Neuropsychological aspects of benign childhood epilepsy with centrotemporal spikes

PURPOSE To establish whether the disability in benign epilepsy with centrotemporal spikes (BECTS) is the result of the number of seizures, the anti-epileptic therapy or is an inherent characteristic of the syndrome itself. METHODS Thirty-six children with BECTS were tested for cognitive functions prior to commencing treatment with anti-epileptic drugs, and the findings were compared with those in 15 children with normal electroencephalograms, performed for unrelated reasons. The data in the study group were further correlated with the laterality of the epileptic focus and the number of seizures. RESULTS Scores for verbal functioning on neuropsychological tests were significantly lower in the study group than the control group. There was no relationship between the neuropsychological scores in the patients and either lateralization of the epileptic focus or number of seizures. DISCUSSION Children with BECTS have an impaired ability to process verbal information. The deficiency is apparently a result of the pathological electrical discharges that are part of the syndrome and are not dependent on the epileptic focus laterality, the number of seizures, or the anti-epileptic treatment.

Prevalence of epilepsy and attention-deficit hyperactivity (ADHD) disorder: a population-based study.

Epilepsy and attention-deficit hyperactivity disorder (ADHD) were reported to co-occur at rates higher than expected for coincidental findings. This study investigated the prevalence of both disorders in community-based primary care practice. The central database of the second-largest health maintenance organization in Israel was searched for all children aged 6 to 13 years (n = 284 419; 51.5% males) diagnosed as having ADHD according to the physicians’ records and/or the filling of at least 10 prescriptions for antiepileptic medications according to pharmacy records. The prevalence of epilepsy in the total population was 5 out of 1000 children, and the prevalence of ADHD was 12.6%. More than one-fourth (27.7%) of the epileptic children were also diagnosed as having ADHD. On multivariate analysis, children with ADHD had almost twice the risk of epilepsy than children without ADHD. This study supports hospital-based findings of a strong interrelationship between ADHD and epilepsy. The high rate of ADHD in Israeli children warrants further investigation.

Pediatric brain stem gliomas: an update

Abstract It has become evident that pediatric brain stem gliomas are a heterogeneous pathology and should be classified according to clinical and radiological criteria. This classification has contributed to better treatment and greatly improved prognosis. Based on a review of the literature, we describe the different types of brain stem astrocytomas reported, which are: cervicomedullary, exophytic, cystic, focal and diffuse. Particular attention is paid to therapeutic modalities. For the first three lesions named the treatment is surgical and oncological therapy should be evaluated only for regrowth of the mass. Focal tumors of the medulla and pons are still treated empirically (surgically and/or with radiotherapy), because a definitive therapeutic protocol has not yet been elaborated; on the other hand it is well estabilished that if the focal mass is in the midbrain this should merely be monitored by means of serial MRI, while radiotherapy should be applied in the event of the tumors growth. Diffuse gliomas are treated with oncological therapy, and surgery (for biopsy or tumor excision) is not indicated. Five illustrative cases from our department are presented.

Breastfeeding May Protect from Developing Attention-Deficit/Hyperactivity Disorder

INTRODUCTION Breastfeeding has a positive influence on physical and mental development. Attention-deficit/hyperactivity disorder (ADHD) is a common neurobehavioral disorder with major social, familial, and academic influences. The present study aimed to evaluate whether ADHD is associated with a shorter duration of breastfeeding. SUBJECTS AND METHODS In this retrospective matched study, children 6-12 years old diagnosed at Schneiders Children Medical Center (Petach Tikva, Israel) with ADHD between 2008 and 2009 were compared with two control groups. The first one consisted of healthy (no ADHD) siblings of ADHD children; the second control group consisted of children without ADHD who consulted at the otolaryngology clinic. A constructed questionnaire about demographic, medical, and perinatal findings, feeding history during the first year of life, and a validated adult ADHD screening questionnaire were given to both parents of every child in each group. RESULTS In children later diagnosed as having ADHD, 43% were breastfed at 3 months of age compared with 69% in the siblings group and 73% in the control non-related group (p=0.002). By 6 months of age 29% of ADHD children were breastfed compared with 50% in the siblings group and 57% in the control non-related group (p=0.011). A stepwise logistic regression that included the variables found to be significant in univariate analysis demonstrated a significant association between ADHD and lack of breastfeeding at 3 months of age, maternal age at birth, male gender, and parental divorce. CONCLUSIONS Children with ADHD were less likely to breastfeed at 3 months and 6 months of age than children in the two control groups. We speculate that breastfeeding may have a protective effect from developing ADHD later in childhood.

Jitteriness Beyond the Neonatal Period: A Benign Pattern of Movement in Infancy

Jitteriness is a frequent, well-recognized phenomenon in neonates. Its occurrence in early infancy, beyond the neonatal period, is much less documented. Thirty-eight full-term infants who were jittery after 6 weeks of age were followed until the jitteriness disappeared and then reevaluated at the age of 3 years. The jittery movements had already been observed during the neonatal period in 16 babies but not before 6 weeks of age in 22. In 34 infants (89%), 1- and 5-minute Apgar scores were 9 or 10. At the initial examination, a mildly increased muscle tone was found in 12 infants, and hyperactive deep-tendon reflexes were found in 19. The jitteriness resolved at a mean age of 7.2 ± 3.4 months. At 3 years, 92% of the infants had a normal neurodevelopmental examination, while in the rest only minor, transient disturbances were found. This study indicates that jitteriness as a sole presentation in infants beyond the neonatal period is a benign phenomenon, associated with an excellent prognosis. Its etiology is unknown but seems most likely to be related to a maturational process within the central nervous system. (J Child Neurol 1991;6:243-245).

Osteopathia striata, cranial sclerosis with cleft palate and facial nerve palsy

Osteopathia striata (OS) is a rare bone dysplasia characterized by longitudinal sclerotic striations of the long bones. It is of no clinical importance, but OS associated with cranial sclerosis represents a separate entity with a high incidence of palatine malformations and deafness. Only 19 cases of this entity have been reported in the literature. One patient of this series also had facial nerve paralysis. This paper presents a second case of OS, cranial sclerosis, palatine pathology and recurrent facial nerve paralysis. This incidence of 2/20 (10%) does not seem to be coincidental but raises the possibility that facial nerve palsy is one of the clinical manifestations of this specific bone abnormality.

Retarded skeletal maturation in children with primary enuresis

Primary nocturnal enuresis (PNE) is a common paediatric problem of multifactorial aetiology. Growth and skeletal maturation were studied in 35 otherwise healthy children with PNE, 26 boys and 9 girls aged 6–14 years, and comparison was made with a control (CTR) group of 19 boys and 3 girls aged 6–13 years of similar ethnic origin. There was no significant difference between the mean height and weight centiles of the two groups. Bone age (BA) determined by the TW-2 method showed a significant lag behind chronological age (CA); the CA-BA difference being 1.46±1.56 years in the PNE group and -0.08±0.8 years in the CTR group (P<0.001). In 11 of the PNE group (31%) the BA retardation was greater than 24 months: in 4 it was between 24 to 36 months and in 7 the difference was greather than 36 months. In all these children T4 and TSH were found to be normal.It is hypothesised that the retarded bone age in children with PNE may reflect delayed maturation of regulatory CNS functions.

Task Switching After Cerebellar Damage

The authors of this study investigated task switching following cerebellar damage. The study group consisted of 7 children and adolescents (M age=13.8 years) who underwent surgical removal of a benign posterior fossa tumor. They were tested at a sufficient interval after surgery (M lag=6.13 years) for restoration of normal cognitive skills and intelligence. Although all showed normal learning of the task compared with control participants, when rapid behavioral changes were required (short preparation time), they exhibited behavioral rigidity manifested by enhanced switching cost. These results are in line with another study on serial reaction time with the same patients (A. Berger et al., in press). They have important implications for our understanding of the cognitive sequelae of early cerebellar damage as well as the involvement of the cerebellum in task switching.