Journal of Pediatric Neurology - Volume 4, issue 1

Authors: Massaro, An N. | Rothbaum, Rebecca | Aly, Hany

Article Type: Research Article

Abstract: Perinatal events and postnatal environmental factors are often the focus when identifying neurodevelopmental risk factors. However, the in utero environment may be an equally important factor for fetal brain development. The objective of this article is to describe in utero environmental factors that may impact fetal brain development and subsequent neurodevelopmental outcome. This is a literature review to examine maternal nutrition, exposures, and behaviors that impact the in utero environment and, consequently, fetal brain …development. A medline search of relevant articles published over the last 25 years was performed utilizing various keywords such as fetal brain development, individual vitamin/minerals, and specific exposures chosen by the authors that have been shown to impact in utero neurodevelopment. This article reviews important maternal nutritional deficiencies and toxin exposures that may negatively impact fetal brain development. Positive exposures to maternal voice, music, and exercise are also reviewed. Fetal brain development is a complex and intricate process. Neurodevelopmental delays detected postnatally are often multifactorial. In utero insults, postnatal illness, socioeconomic factors, and developmental environment may all attribute to poor outcomes. It is often difficult or impossible to determine which factor or factors contribute the most to morbidity. While reports on developmental delays often focus on clinical risk factors such as preterm birth or maternal/neonatal infection, perinatal events such as birth asphyxia, or known postnatal environmental factors such as low socioeconomic status or parental neglect, it is important to remember that in utero insults may be equally responsible. This article reviews early neurological development and a selection of various maternal exposures and behaviors that may impact the fetal brain during this critical developmental period. Show more

Keywords: Folate, vitamin B6, copper, iron, iodine, docosahexaenoic acid, arachidonic acid, toxin, exercise, music

Citation: Journal of Pediatric Neurology, vol. 4, no. 1, pp. 1-9, 2006

Authors: Gordon, Neil

Article Type: Research Article

Abstract: Williams syndrome is a rare genetically determined syndrome in which studies have shown a number of unusual physical and mental abnormalities. These may well lead to an increased understanding of the causes of the syndrome in the future. The symptoms and signs are described, especially the facial appearance, the incidence of hypercalcemia, and the cardiovascular complications. A number of studies have been done on the mental impairment of children with Williams syndrome, and their excessive friendliness …is often emphasized. However, in spite of their language fluency they may well have pragmatic disorders, which warrant treatment. The learning of visual-spatial material can often be a problem, and one that persists into adult life. The cerebral pathology shows a number of correlations with clinical findings, such as the learning difficulties, in particular visual processing; hyperacusis; and unexpected musical skills. The clinical findings will suggest the diagnosis, but this may well depend on having seen similarly affected children before; and will be confirmed by genetic studies. The prognosis can be favorable. Treatment can help the symptoms of hypercalcemia and hypothyroidism, and surgery for aortic supravalvular stenosis is sometimes possible. Show more

Keywords: Williams syndrome, symptoms, signs, pathology, diagnosis, treatment

Citation: Journal of Pediatric Neurology, vol. 4, no. 1, pp. 11-14, 2006

Authors: Al-Haggar, Mohammad | El-Baz, Rizk | Youseff, Hisham | Othman, Nierra | Ali, Rabab

Article Type: Research Article

Abstract: As inattention and hyperactivity are highly prevalent among school children, we aimed to introduce an overview on ADHD (attention deficit hyperactivity disorder) in Delta regions of Egypt, to evaluate its diagnostic accuracy, and to suggest a de novo method for diagnosis among Egyptian school children. This retrospective epidemiological study was conducted on cases registered in database files of Pediatrics Genetics unit, in Mansoura University Children's Hospital, which serves all Delta regions of Egypt. …Initial diagnosis of 356 registered cases was based upon detailed history, signs and symptoms without applying any strict criteria. Some cases were receiving stimulants drugs however almost all were under behavioral therapy. All cases were currently re-evaluated applying DSM-IV criteria (Diagnostic and Statistical Manual of Mental disorders, 4th edition) and Conners' Developmental approach. Diagnosis of ADHD was confirmed in 283 children excluding 73 cases (20.5%) they were 211 boys and 72 girls (boys/girls ratio 3/1), age range 7–12 years (mean 9.5 ± 3.13 years). Most cases whether at initial registration or during final assessment were associated with hyperactivity/impulsivity however predominantly inattentive cases constituted 39.04%. Half of excluded cases were predominantly inattentive (49.3%) and 61.7% of exclusion were due to clinical improvements. Other exclusionary causes included sleep disorders, hyperthyroidism, communication disorders, mental retardation and autism. Positive family history was reported in 49.8% mostly among inattentive and combined types. ADHD is not uncommon in our community; its frequency among cases referred for childhood diagnostic clinics approximates 79.5%, and prevalence would be much lower if evaluated among asymptomatic school children. Inattentive ADHD is frequently overlooked and its response to therapy is usually satisfactory. Show more

Keywords: Attention deficit, hyperactivity, epidemiology, Egypt

Citation: Journal of Pediatric Neurology, vol. 4, no. 1, pp. 15-18, 2006

Authors: Seguti, Vladimir F. | Schappo, Andrea | Sobrinho, José A.N. | Ferreira, Lisiane S.

Article Type: Research Article

Abstract: Schizencephaly is an uncommon structural disorder of cerebral cortical development. There are few comparative studies evaluating epilepsy in that condition. The aim of this study is to analyze which factors are associated with the development of epilepsy in patients with schizencephaly. The study is a longitudinal, observational and comparative retrospective study of 35 pediatric patients with schizencephaly. The patients were divided into two groups: a group of 10 children who developed epilepsy and another group of …25 who had no clinical epileptic manifestations. Both groups were followed up and the data collected to analyze potential risk factors included demographic and prenatal data (prenatal event, family history of epilepsy), radiological features (location of the cleft, unilateral or bilateral, extent of the lesion, number of impaired lobes, open-lip or closed-lip types, presence of associated anomalies), and neurological and electroencephalographic data. No significant difference was found in the association between epilepsy and any of the examined factors (P>0.05). Neither the features of the lesion (cleft size location, type of lip, number of impaired lobes, associated anomalies), nor electroencephalography abnormalities, nor the family history of epilepsy or prenatal events could predict the occurrence of epilepsy in patients with schizencephaly. Show more

Keywords: Schizencephaly, epilepsy, outcome

Citation: Journal of Pediatric Neurology, vol. 4, no. 1, pp. 19-26, 2006

Authors: Mentzel, Hans-Joachim | Karadag, Demet | Güllmar, Daniel | Löbel, Ulrike | Reichenbach, Jürgen R. | Sedlacik, Jan | Seidel, Jörg | Brandl, Ulrich | Kaiser, Werner A.

Article Type: Research Article

Abstract: To evaluate by means of diffusion tensor magnetic resonance imaging (DTI) if changes in apparent diffusion coefficient (ADC) and diffusion tensor fraction anisotropy (FA) in unidentified bright objects (UBOs) and perilesional normal appearing brain regions of children with neurofibromatosis type 1 (NF1) can be observed. Ten children with NF1 underwent conventional magnetic resonance imaging and DTI. The ADC and FA in UBOs and perilesional normal appearing brain of NF1 patients were compared with the same regions …of the brain of controls. The ADC increased significantly for UBOs and surrounding normal appearing brain tissue of the brain in children with NF1. We observed no significant differences in FA between the UBOs and perilesional areas or that of controls. Although there is limited neuropathologic information about UBOs, detection of diffusion properties of UBOs can be used to understand tissue characteristics of UBOs, noninvasively. Show more

Keywords: Diffusion tensor imaging, fraction anisotropy, apparent diffusion coefficient, neurofibromatosis, unknown bright objects, phacomatoses

Citation: Journal of Pediatric Neurology, vol. 4, no. 1, pp. 27-31, 2006

Authors: Suzuki, Motomasa | Hayakawa, Fumio | Okumura, Akihisa | Watanabe, Kennichi | Fukumoto, Yukiko | Kato, Toru | Watanabe, Kazuyoshi

Article Type: Research Article

Abstract: The value of diffusion-weighted imaging (DWI) in children with acute central nervous system (CNS) symptoms has not been fully established. We aimed to elucidate whether DWI gives additional information in children with acute CNS disorder. The subjects were eight children with acute CNS symptoms, whose cranial DWI showed signal abnormality. Two patients were suffered from cerebral infarction, and one had systemic lupus erythematosus with CNS involvement, four had acute encephalopathy, and one had relapsing encephalopathy. We …retrospectively compared (1) the size of signal abnormality, (2) latency until appearance of signal abnormality, and (3) ability to differentiate old lesions between DWI and fluid-attenuation inversion recovery (FLAIR). In addition, we analyzed cerebral blood flow (CBF) changes in lesions detected by DWI, using single-photon emission computed topography (SPECT). In seven of eight patients, the size of signal abnormality was larger in DWI compared to FLAIR. In four of five patients with magnetic resonance imaging obtained during the very acute phase of CNS symptoms, the latency until appearance of signal abnormality was shorter on DWI compared to FLAIR. Combination of DWI and FLAIR successfully differentiated new lesions from old ones in all three subjects with a history of old lesions. DWI revealed signal abnormality not during the very acute phase but on 5th day of CNS symptoms in a patient with encephalopathy. In seven patients with SPECT available, lesions detected by DWI showed decreased CBF in four patients, increased CBF in two, normal CBF in one. DWI may provide useful additional information in children with acute CNS symptoms. Signal abnormalities on DWI may represent ischemic lesions or inflammatory lesions with increased or normal CBF on SPECT. Show more

Keywords: DWI, SPECT, CNS, child

Citation: Journal of Pediatric Neurology, vol. 4, no. 1, pp. 33-39, 2006

Authors: Ganesh, Ramaswamy | Deenadayalan, Munirathinam | Janakiraman, Lalitha | Viswanathan, Venkataraman

Article Type: Research Article

Abstract: Childhood strokes are not so rare as once thought and its incidence is equal to pediatric brain tumors. The recent advent of more accurate diagnostic techniques has lead to a surge of interest, which may lead in the near future to strategies for treatment and prevention. We retrospectively analyzed the prevalence of first episode of arterial stroke in children. This study was conducted in a private tertiary care hospital for children from June 2002 to May …2004 involving 12 children with first episode of arterial stroke out of 23,125 admissions. Majority (42%) of the children was between 1 month and 5 years and hemiplegia/hemiparesis was the common clinical presentation. Ten children had infarct and two had hemorrhage. The causes identified after detailed investigations were tetralogy of Fallot (2), protein C deficiency (1), homocystinuria (1), nonspecific vasculitis (1), hypercholesterolemia (1), factor XIII deficiency (1), late hemorrhagic disease of the newborn (1) and idiopathic (4). All the children recovered though some had neurological deficit and there was no mortality. Ischemic stroke was more common than hemorrhagic stroke. Prognosis of pediatric stroke is good and the treatment is largely supportive. Show more

Keywords: Stroke, ischemia, hemorrhage, CT, MRI

Citation: Journal of Pediatric Neurology, vol. 4, no. 1, pp. 41-44, 2006

Authors: Ahmed, Riaz | Varghese, Thomas

Article Type: Research Article

Abstract: An unusual presentation of absence seizure in a boy in the form of "throttling sensation" has been described. He has responded well to valporate after confirming the diagnosis by electroencephalogram. This is to emphasis the pediatricians about the importance of referral to neurologists for appropriate diagnosis if they encounter such atypical symptoms among their patients.

Keywords: Absence seizures, electroencephalogram, throttling sensation, spike and wave

Citation: Journal of Pediatric Neurology, vol. 4, no. 1, pp. 45-47, 2006

Authors: Masri, Amira

Article Type: Research Article

Abstract: Abnormal movements including tremor and myoclonus have been described in patients with vitamin B12 deficiency before or upon initiation of treatment with vitamin B12. We report here a case of vitamin B12 deficiency in an infant who presented first with chaotic rotatory eye movements (myoclonus) followed by the appearance of tremors in the tongue, palate, upper and lower limbs. All the abnormal movements disappeared spontaneously within a period of six weeks. To our knowledge, this is …the first report of vitamin B12 deficiency presenting with myoclonus in the eyes. Show more

Keywords: Vitamin B12, eye myoclonus, tremor, abnormal movements

Citation: Journal of Pediatric Neurology, vol. 4, no. 1, pp. 49-51, 2006

Authors: Amit, Rami | Amit, Yali | Amit, Daniel J.

Article Type: Research Article

Abstract: Seizures and electroencephalography (EEG) paroxysms induced by eye closure. Are rare but a recognized form of stimulus induced epilepsy. We report a case of a 15-year-old male diagnosed with juvenile absence epilepsy in whom EEG paroxysms in the form of generalized three per second spike and wave bursts generated by sustained eye closure were consistently blocked during mental activity in the form of skip counting. To the best of our knowledge, this phenomenon has not been …previously reported. We propose two possible mechanisms to account for this blockage, both based on reduction of excitability: (1) blockage of the seizures in the brainstem at the level of the reticular activating system, by raising the level of attention, via the thalamo-cortical circuitry, (2) task-specific, based on the model mechanism of "winner-takes-all" which generates enhanced inhibition to produce cognitive selectivity. Show more

Keywords: Seizures, reflex epilepsy, eye closure

Citation: Journal of Pediatric Neurology, vol. 4, no. 1, pp. 53-56, 2006