Jacob Genizi

Primary headaches, attention deficit disorder and learning disabilities in children and adolescents

BackgroundPrimary headaches and Learning difficulties are both common in the pediatric population. The goal of our study was to assess the prevalence of learning disabilities and attention deficit disorder in children and adolescents with migraine and tension type headaches.MethodsRetrospective review of medical records of children and adolescents who presented with headache to the outpatient pediatric neurology clinics of Bnai-Zion Medical Center and Meyer Children’s Hospital, Haifa, during the years 2009–2010. Demographics, Headache type, attention deficit disorder (ADHD), learning disabilities and academic achievements were assessed.Results243 patients met the inclusion criteria and were assessed: 135 (55.6%) females and 108 (44.4%) males. 44% were diagnosed with migraine (35.8% of the males, 64.2% of the females, p = 0.04), 47.7% were diagnosed with tension type headache (50.4% of the males, 49.6% of the females). Among patients presenting with headache for the first time, 24% were formerly diagnosed with learning disabilities and 28% were diagnosed with attention deficit disorder (ADHD). ADHD was more prevalent among patients with tension type headache when compared with patients with migraine (36.5% vs. 19.8%, p = 0.006). Poor to average school academic performance was more prevalent among children with tension type headache, whereas good to excellent academic performance was more prevalent among those with migraine.ConclusionsLearning disabilities and ADHD are more common in children and adolescents who are referred for neurological assessment due to primary headaches than is described in the general pediatric population. There is an association between headache diagnosis and school achievements.

Childhood epilepsy with occipital paroxysms : Difficulties in distinct segregation into either the early-onset or late-onset epilepsy subtypes

The Commission on Classification and Terminology of the International League Against Epilepsy Childhood rigidly segregated epilepsy with occipital paroxysms into 2 separate syndromes with different predominant seizure types: early-onset seizure susceptibility type consisting of prolonged infrequent, nocturnal autonomic seizures and accompanied by eye deviation and ictal vomiting and late onset with short diurnal frequent seizures and visual ictal manifestations along with throbbing headaches. Epileptic clinical manifestations and electroencephalographic data were analyzed in 28 patients with suspected occipital lobe epilepsy in an attempt to segregate them into either the early or late forms according to the International League Against Epilepsy classification. Electroencephalography in 25 children demonstrated occipital epileptiform paroxysms compatible with the suspected epileptic syndrome. Only 14 (50%) children complied with the rigid criteria of either early-onset or late-onset presentations. The other 14 (50%) children presented with mixed diverse epileptic phenomena such as short-lived seizures in infancy or prolonged seizures during childhood, not complying with either rigid syndrome (ie, short-lived epileptic blindness at an early age or vomiting during later childhood). Despite present attempts to rigidly segregate childhood epilepsy with occipital paroxysms into 2 distinct epileptic syndromes, a high percentage of children still present with various mixed clinical phenomena. Therefore, clinicians should be aware of possible unique and unusual presentations of occipital lobe epilepsy at various ages.

Intractable Gelastic Seizures During Infancy : Ictal Positron Emission Tomography (PET) Demonstrating Epileptiform Activity Within the Hypothalamic Hamartoma

Gelastic seizures comprise a very rare form of epilepsy. They present with recurrent bursts of laughter voices without mirth and are most commonly associated with the evolution of a hypothalamic hamartoma. The purpose of this article is to describe the second reported ictal fluorodeoxyglucose-positron emission tomography study in a unique case of an infant with intractable gelastic seizures since the neonatal period associated with a hypothalamic hamartoma. The patient presented at 4 months old with recurrent, almost persistent, gelastic seizures consisting of laughter bouts without mirth. The seizures were noticeable at the first week of life and increased in frequency to last up to 12 hours, namely status gelasticus. These gelastic fits were accompanied with focal motor seizures, including unilateral right-eye blinking and mouth twitching. Developmental mile-stones were intact for age. Magnetic resonance imaging of the cortex demonstrated a large hypothalamic hamartoma within the third ventricle, hampering cerebrovascular fluid drainage of the lateral ventricles. An electroencephalography was nondiagnostic. Ictal fluorodeoxyglucose-positron emission tomography demonstrated a large circumscribed hypermetabolic region within the location of the hypothalamic hamartoma, representing localized intense epileptiform activity. The infant became instantly free of all seizure types given minute doses of oral benzodiazepine (clonazepam) and remains completely controlled after 12 months. Her overall development remains intact. This ictal fluorodeoxyglucose-positron emission tomography is the second reported study verifying that the main source of the epileptic activity inducing gelastic seizures originates from the hypothalamic hamartoma itself; therefore, a complementary fluorodeoxyglucose-positron emission tomography study should be considered in any patient presenting with intractable gelastic seizures, especially in those associated with hypothalamic hamartoma, in order to localize the region of epileptiform activity amenable to surgical resection if intensive drug therapy fails.

The cross- ethnic variations in the prevalence of headache and other somatic complaints among adolescents in Northern Israel

BackgroundHeadache is the most common cause for chronic or recurrent pain in childhood and adolescence. Chronic pain may have a long-term effect on adolescents. It might contribute to functional limitations, such as poor school attendance, and it may adversely affect development of healthy social relationships. The aim of our study was to examine the cross- ethnic variation in the prevalence of headache in a non- clinical sample of adolescents in Northern Israel and to learn about its association to other somatic complaints.MethodsA self-administered, anonymous questionnaire was presented to 2,088 tenth grade students attending 19 high-schools in Northern Israel (all the public high schools within two districts). Participants were Jews and Arabs, the latter including Muslim, Christians, and Druze, aged 15 to 16. Parental and student consent was obtained from all participants. The study was approved by the IRB of our institution.ResultsAll 2088 questionnaires were returned although only 2019 were usable and analyzed. Arab adolescents comprised 55% (1117) of the analyzed sample and Jews 45% (902), 56% of participants were girls. Of the Arab participants, 18.6% reported having frequent headaches (girls 25.3%, boys 9.1%, P<0.0001) much less than their Jewish peers (P<0.0001) among whom 27.9% reported having frequent headaches (girls 35.6%, boys 19% P<0.0001). Other somatic complaints such as abdominal pain, palpitations, disordered sleep and fatigue were more frequent in adolescents (Jews and Arabs, girls and boys) who suffered from headaches than in their peers who did not report having headaches (P<0.0001), the same pattern observed in the Jewish and the Arab group.ConclusionsHeadache is a frequent complaint among adolescents in Northern Israel. Jewish adolescents reported having headaches more frequently than their Arab peers. Those who suffered from frequent headaches also reported having significantly more other somatic complaints than adolescents without headaches. Girls had more somatic complaints then boys in the two ethnic groups.

The complementary value of sleep-deprived EEG in childhood onset epilepsy.

BACKGROUND Although EEG is an important diagnostic tool in suspected childhood onset epilepsy, as many as 50% of wakefulness records remain normal. Sleep-deprived EEG has been reported in adults to serve as an activator of epileptic discharges but such effect is still not agreed upon in children reporting small series. PURPOSE Assess the complementary diagnostic value of sleep deprivation on the induction of epileptic discharges in childhood onset epilepsy having a normal awake record within a period of 5 years. EEG recording was performed during the awake, drowsiness and sleep states following sleep deprivation of 6h. BACKGROUND RESULTS: Fifty five children of whom the initial record failed to detect epileptiform discharges, were assessed at age 5-17 years (mean: 10+/-3.7), 27 boys and 28 girls. Sleep occurred in 51 (92.7%) after sleep deprivation and in only 1 (1.8%) during an awake record. Epileptic discharges were detected in 15 of 55 (27.2%) previous non-epileptic awake records during the sleep-deprived EEG either during wakefulness and more frequent during sleep. Eight abnormal records were detected in 18 (44%) children presenting with a focal seizure and 7 of 35 (20%) associated with generalized seizures. Epileptic discharges were recorded mainly and more frequent during sleep. CONCLUSIONS Our data suggests that sleep deprivation imposes an apparent activating impact uncovering epileptic discharges children corroborating with overt clinical seizures even beyond the sampling effect of repeat records.

Frequency of pediatric migraine with aura in a clinic‐based sample

To assess the prevalence and risk factors for pediatric migraine with aura (MWA) among patients presenting to pediatric neurology clinics.

Apparent Life-Threatening Events: Neurological Correlates and the Mandatory Work-Up

The purpose of this study was to estimate the prevalence of neurological causes for apparent life-threatening events in infants and assess the neurological work-up mandatory to diagnose such diagnosis, hypothesizing that acute neurological disorders constitute the most common etiology for such an acute event. A retrospective chart review allocated 93 infants fulfilling the criteria of the National Institutes of Health presenting at 1 week to 8 months with apnea, color changes, gagging, unresponsiveness, and alterations of body tone. An underlying neurological etiology was detected in 18 (19%), whereby 15 had seizures and 3 had central apnea. Only one electroencephalography demonstrated epileptic discharges, and brain imaging was intact. Thus, it has been concluded that neurological impairment, mainly that of seizures inducing an apparent life-threatening event, are relatively uncommon. As such, recommendations are that history taking and repeat physical examination still remain the major diagnostic tools before resorting to extensive laboratory studies.

Effect of high-dose methyl-prednisolone on brainstem encephalopathy and basal ganglia impairment complicating cat scratch disease

Cat scratch disease (CSD) is a zoonotic illness caused by the Gram negative bacillus Bartonella henselae characterized by a small skin lesion at the site of a bite, lick or scratch by a cat, commonly followed by regional lymphadenopathy 1 or 2 weeks later. We report herein on severe neurological complications of CSD combining brainstem encephalopathy and basal ganglia impairment. This 12-year-old female acutely presented to a local hospital with profound coma and a prolonged tonic posturing of extremities. On the neurological examination she was deeply comatose with pin-point pupils and lack of vestibulo-ocular responses, suggestive of brainstem encephalopathy, along with marked rigid hypertonicity suggestive also of basal ganglia impairment. Initially suspecting Herpes simplex encephalitis or acute disseminated encephalomyelitis she was promptly started with high-dose methyl-prednisolone and acyclovir. Her parents apparently reported that she was scratched by a kitten some 4 weeks prior to her present admission and as such, suspecting CSD, she was begun with doxycycline and rifampicin. Her serology had proven positive for IgM antibodies to Bartonella henselae establishing the diagnosis. She regained consciousness after 4 days and the signs of brainstem and extra-pyramidal impairment also gradually abated and disappeared after 10 days. A follow-up exam after a month disclosed mild extra-pyramidal abnormalities which disappeared after 3 months. Although extremely rare, CSD should be also considered in a patient presenting with a severe encephalopathy and associated basal ganglia impairment. The prompt administration of high-dose methyl-prednisolone upon admission may have contributed to the favorable outcome in our patient and therefore should be advocated in any patient presenting with profound encephalopathy regardless the underlying etiology recovered later.

Family quality of life among families with a child who has a severe neurodevelopmental disability: Impact of family and child socio-demographic factors

We aimed to examine family quality of life (FQOL) of Northern Israeli families having a child with a severe neurodevelopmental disability and its relation to socio-demographics. The cohort included caregivers of 70 children ages (mean ± standard deviation) 5.36 ± 3.53 years. Families were two-parent (85.7%), lived in the periphery (67.1%) and included Jews (60%), Muslims (18.6%), Druze (14.3%) and Christians (7.1%). Religiosity included: secular (38.6%), traditional (31.4%), religious (30%). Childrens diagnosis included autistic spectrum disorder (41.4%), intellectual disability (21.4%), cerebral palsy (17.1%), genetic syndromes (17.1%) and sensorineural hearing loss (2.9%). Degree of support (1-minimal,5-greatest) required by the child was 3.67 ± 1.28 for physical and 3.49 ± 1.36 for communication. Primary caregivers completed the FQOL Survey. Domain scores were highest for family relations and lowest for financial well-being. Dimension scores were highest for importance and lowest for opportunities. Overall FQOL approximated average. Jewish families and residents of a major urban area reported higher and more religious families reported lower overall FQOL. Regression analysis found ethnicity contributing to overall FQOL and domain scores with residence contributing to support from services. Ethnicity and child dependence contributed to dimension scores. Northern Israeli families having a child with a severe neurodevelopmental disability report average FQOL scores. However, family and child dependence characteristics affect FQOL scores. Professionals working with these families should consider FQOL information when making recommendations.

Legionella pneumophila and Pneumocystis jirovecii coinfection in an infant treated with adrenocorticotropic hormone for infantile spasm: case report and literature review.

We describe an 8-month-old infant with infantile spasms treated with adrenocorticotropic hormone (ACTH) who presented with fatal Legionella pneumophila and Pneumocystis jirovecii infection. Emphasis is placed on the ensuing immunosuppression and infectious sequelae of ACTH therapy. Given that ACTH therapy may increase the risk of fatal infection, patients undergoing such treatment should be closely monitored, with particular attention paid to the functioning of the immune system.