Journal of Pediatric Neurology - Volume 6, issue 2

Authors: Adamolekun, Bola | Wahba, Mervat

Article Type: Research Article

Abstract: Behavioral disorders and epileptic seizures are frequent co-morbidities in patients with intellectual disability (ID). This co-occurrence of behavioral disorders and epilepsy in patients with ID provides opportunities for the selection of antiepileptic drugs (AEDs) that not only show efficacy for the common seizure types in the ID population, but also are effective in the management of some of the behavioral disorders seen in this patient population. The effects of AEDs on behavioral disorders in patients …with intellectual disabilities and seizures, as well as the interactions between AEDs and psychotropic medications that may impact on behavior; are discussed in this communication. Show more

Keywords: Behavior, epilepsy, intellectual disability, antiepileptic drugs

Citation: Journal of Pediatric Neurology, vol. 6, no. 2, pp. 93-97, 2008

Authors: Castejón, Orlando J.

Article Type: Research Article

Abstract: Cortical biopsies of 13 infant patients with clinical diagnosis of congenital hydrocephalus, Arnold-Chiari malformation, and postmeningitis hydrocephalus were examined with transmission electron microscopy to study the synaptic plasticity and synaptic degenerative changes in hydrocephalic edema. The immature cortical neuropil of different cortical regions showed swollen nerve cell processes separated by enlarged extracellular space. Isolated and swollen presynaptic endings with few or numerous synaptic vesicles, disruption of limiting plasma membrane, and absent postsynaptic …partners were also observed. Activated flat and invaginated axodendritic and axospinodendritic asymmetric synaptic contacts showed synaptic vesicles anchored to the presynaptic membrane, and short or large synaptic active zones. The swollen and degenerated synaptic contacts, including axosomatic synapses exhibited enlargement of few synaptic vesicles and lack of pre- and postsynaptic densities. Synaptic disassembly was observed in elevated intracranial pressure- hydrocephalus. Megaspines making multiple asymmetric synaptic junctions were also distinguished. Phagocytic astrocytes engulfed the degenerated synapses. The potential role of hydrocephalic edema and ischemia, oxidative stress, increased calcium concentration, activation of N-methyl D-aspartate receptors, and disturbance of ion homeostasis are discussed in relation with the observed synaptic plasticity and synaptic degenerative changes. Show more

Keywords: Synaptic plasticity, synaptic degeneration, congenital hydrocephalus, cerebral cortex, electron microscopy

Citation: Journal of Pediatric Neurology, vol. 6, no. 2, pp. 99-107, 2008

Authors: Pakalnis, Ann | Tischner, Jessica | Colvin, Andrew | Kring, Donna

Article Type: Research Article

Abstract: Episodic tension type headaches are common in the pediatric population. Concurrent behavioral and emotional disorders could significantly impact treatment options and therapeutic response. Children and adolescents from our multi-disciplinary headache clinic with episodic tension type headaches (according to International Classification of Headache Disorders-II) criteria were evaluated prospectively for presence of concurrent psychologic disorders with standardized psychologic batteries and case controls were used for comparison. Parents frequently reported behavioral symptoms, but only …oppositional defiant disorder was significantly represented with anxiety disorders also very common. Presence of concurrent psychiatric disorders did not influence headache treatment response. Show more

Keywords: Pediatrics, headache, tension, behavior

Citation: Journal of Pediatric Neurology, vol. 6, no. 2, pp. 109-113, 2008

Authors: Mostafa, Gehan A. | Hamza, Rasha T. | El-Shahawi, Heba H.

Article Type: Research Article

Abstract: Etiology of autism has become an area of significant controversy. Allergy may play a role in the pathogenesis of autism wherein allergic immune responses to some proteins (e.g., dietary proteins and latex) may induce the production of brain autoantibodies, which are found in many autistic children. This study was conducted to investigate the frequency of allergic manifestations in autistic children. The relationship between allergy and disease characteristics in terms of disease severity, clinical findings and …electroencephalography (EEG) abnormalities was also studied. Fifty autistic children (30 had mild to moderate autism and 20 had severe autism) were studied in comparison to 50 age- and sex- matched children without neuropsychiatric manifestations serving as controls. Clinical evaluation was done with special emphasis on neuropsychiatric assessment and clinical manifestations of allergy. Serum total immunoglobulin E was measured in all studied subjects. In addition, EEG and assessment of mental age were done for all autistic children. Allergic manifestations (bronchial asthma, atopic dermatitis and/or allergic rhinitis) were found in 52% of autistic patients. This frequency was significantly higher than that of controls (10%; P < 0.001). There was a significant positive association between the frequency of allergic manifestations and disease severity, important clinical findings elicited in some autistic children (gastrointestinal symptoms and neurological manifestations) and EEG abnormalities. In conclusion, the frequency of allergic manifestations is increased in autistic children. The significant positive association between these manifestations and important disease characteristics (disease severity, gastrointestinal symptoms, neurological findings and EEG abnormalities) may shed light on the possible causal role of allergy in some autistic children. Indeed, we need to know more about the links between allergy, immune system and brain in autism. This is important to determine whether therapeutic modulation of immune function and allergic diseases are legitimate avenues for novel therapy in selected cases of autism or even for attempted primary prevention in genetically at risk subgroups. Show more

Keywords: Autism, allergy, autistic severity, serum IgE

Citation: Journal of Pediatric Neurology, vol. 6, no. 2, pp. 115-123, 2008

Authors: Ali, Mumtaz | Khan, Hamzullah | Khan, Khalid Mahmood | Khwanzada, Khalid | Ayub, Shahid | Rahman, Said

Article Type: Research Article

Abstract: To determine the clinical presentation and surgical outcome of cranioorbital hydatidosis. This retrospective study was performed in the department of neurosurgery Lady Reading Hospital, Peshawar from November 1999 to October 2006. Twenty-one cases with intracranial/orbital hydatidosis were operated. The patients were either admitted through out patient department or referred from other units. Computed tomography and/or magnetic resonance imaging were the imaging modalities to reach the diagnosis in addition to serological and hematological …tests. Surgery was the only treatment option used. Pericystic hydraulic method was the technique used for the excision of the hydatid cyst of brain. There were nine males and 12 females with male to female ratio of 1:1.3. All patients belonged to pediatric age group with age range of 4–14 years and mean age of 8.66 years. Headache, vomiting, papilledema were present in all the patients while seizures were the present in 16 patients. The lesion was removed surgically by adopting pericystic hydraulic method during craniotomy in all but two cases of orbital hydatid disease where lateral orbitotomy was performed to enucleate the cyst. There was no intraoperative morbidity except that the cyst ruptured in one case. No postoperative complications were noted and there was no mortality. Hydatid cyst of the brain presents clinically as intracranial space occupying lesion and is more common in children. Surgery is the treatment option with affordable morbidity and low mortality. Show more

Keywords: Cerebral hydatid cyst, ecchinococcosis, intracranial hydatidosis

Citation: Journal of Pediatric Neurology, vol. 6, no. 2, pp. 125-128, 2008

Authors: Fallah, Razieh | Golestan, Motahhareh

Article Type: Research Article

Abstract: Febrile seizures are the most common form of convulsions, occurring in 2–5% of children. In the approach to a convulsing febrile patient, discovering the cause of fever and excluding central nervous system infection, serious electrolyte imbalances and other acute neurologic illnesses are essential. History and physical examination may be helpful. The purpose of this study was to evaluate significance of laboratory diagnostic tests other than cerebrospinal fluid examination in first febrile seizures of children. In a …descriptive study, medical records of 139 children with first febrile seizure, admitted between March 2004 and August 2005 to Yazd Shaheed Sadoughi Hospital, were evaluated on all laboratory diagnostic done tests (other than cerebrospinal fluid examination), classification of laboratory tests results (normal, outside of normal range, or significantly abnormal), number of significant abnormal tests not predicted by history and physical examination, change in management plan based on biochemical abnormalities and type of febrile seizure. Febrile seizure type was complex in one-third of patients. The number of laboratory tests performed was 9.24 ± 2.4 (range one to 16). Significantly abnormal test results were seen in 12% of patients. Patients less than one year old and those with complex febrile seizures had the greatest number of significantly abnormal test results. Significantly abnormal tests results not predicted by history and physical examination were seen in three cases (one with asymptomatic hypocalcemia and two with urinary tract infection), all of whom were less than 1 year old. Routine blood chemistry analysis is not necessary in children with febrile seizures and only should be ordered based on the patient's condition or medical history. Show more

Keywords: Seizure, febrile seizure, laboratory evaluation

Citation: Journal of Pediatric Neurology, vol. 6, no. 2, pp. 129-132, 2008

Authors: Hamzat, Talhatu K. | Carsamer, Sandra | Wiredu, Edwin K.

Article Type: Research Article

Abstract: The aim of this study was to provide hospital-based epidemiological information about newborn brachial plexus palsy (NBPP) in Accra, Ghana. The records of all pediatric patients treated over a 5-year period (December 1999 and December 2004) were retrieved to identify those with NBPP. The records of those who had NBPP were further reviewed to document their sociodemography, diagnosis, labor and birth history, presentation at birth, mode and place of delivery. A total of 773 patients' records …were reviewed out of which 210 (27.2%) were cases of NBPP. Using the 1987 Narakas system of classification, majority (94.8%) of the NBPP cases were of group~I type brachial plexus injury or Erb's palsy, with a male predominance (61.4%), and most (79.5%) were delivered by normal vaginal delivery (52.9%) and most (70.9%) were cephalic in presentation at birth. About 55.2% cases were referred for physiotherapy within one month of diagnosis. The treatment disposition for majority (88.1%) of the NBPP patients was not documented and only (4.8%) was formally discharged from physiotherapy. The results indicate that birth weight exceeding 4.0 kg, vertex presentation and vaginal delivery were the noticeable co-existing factors for NBPP in this population. The Erb's type was the modal type of NBPP in the sample and majority were delivered in private clinics. The clinical, economic and sociocultural implications of this disorder, as well as the importance of better clinical documentation in physiotherapy and needs for a national survey for NBPP in Ghana was discussed. Show more

Keywords: Newborn brachial plexus, palsy, Ghana, prevalence

Citation: Journal of Pediatric Neurology, vol. 6, no. 2, pp. 133-138, 2008

Authors: Zaid, Afawi | Abu-Hammad, Talab | Shorer, Zamir | Grotto, Itamar | Mahajnah, Muhammad

Article Type: Research Article

Abstract: Since population-based data on epilepsy prevalence in Israel is not available, the aim of this study was to assess the prevalence of epilepsy in Bedouin children between the ages of 0–16 years living in Southern Israel. A cross-sectional study was conducted which included the collection of clinical and demographic data as well as the diagnosis of epilepsy in relatives (first/second-rank family connection). Data was collected from all 23 primary care clinics (Clallit health fund) in the …Bedouin sector, Southern Israel, and from the computerized files of the neurology clinic, Soroka University medical center, Beer-Sheva. The study population consisted of 68,578 Bedouin children from birth to 16 years, of which 275 were diagnosed with epilepsy. The epilepsy prevalence of 4.01, is less than world estimate (4.5–17 per 1000), with huge differences between various clinics and age subgroups, ranging from 0 to 11.10. Most children received care only from their family doctor rather than from a neurologist and the type of epilepsy was not classified. Twenty-four families (74 children) had more than one relative of first or second rank suffering from epilepsy, indicating the existence of definite genetic involvement. Failure to diagnose and/or to refer suspected episodes of epilepsy to Soroka neurology clinic may be explained by either the failure to recognize the episode as epileptic or non-compliance due to fear of social stigma. Further research is necessary to explain these findings. Show more

Keywords: Bedouins, childhood epilepsy, epidemiology, cross-sectional study

Citation: Journal of Pediatric Neurology, vol. 6, no. 2, pp. 139-143, 2008

Authors: Idowu, Olufemi Emmanuel | Disu, Elizabeth Aruma | Anga, Alexandra Laraba | Fabanwo, Olusegun Adetokunbo

Article Type: Research Article

Abstract: Cranial and spinal dysraphisms have different incidence in different parts of the world depending on the geographic region, ethnicity, socioeconomic status of the parents, maternal age and parity. Our aim was to determine the current state of neural tube defects in our environment where there is no policy of food supplementation with folic acid. A prospective study of 47 consecutive patients (and their parents) with neural tube defects who presented at the pediatric and neurosurgical unit …of Lagos State University Teaching Hospital, Ikeja, Lagos between August 2006 and July 2007 were studied. Most patients were males. The male to female ratio for myelomeningocoele and encephalocele was 2:1 and 1:1.5, respectively. The most common dysraphism was open spinal dysraphism (77%) and this occurred usually at the lumbosacral region (67%). None of the infants was offspring of consanguineous marriage. Most patients were second born. Presentation of patients to the hospital was after 48 hours of delivery in majority of cases. The average maternal age at conception was 27 years (18–37 years). There was no history of preconception use of folic acid in all the mothers of the patients. There is a need to consider an aggressive approach to periconceptional folic acid supplementation for our women and early referral of patients with cranial and spinal dysraphisms. Policy to encourage fortification of a staple food with folic acid is urgently needed. Show more

Keywords: Dysraphisms, clinical profile, child

Citation: Journal of Pediatric Neurology, vol. 6, no. 2, pp. 145-150, 2008

Authors: Fusco, Carlo | Giustina, Elvio Della

Article Type: Research Article

Abstract: A 4-year-old child presented with peripheral left facial palsy. Neurological examination was normal except for deep tendon areflexia in the lower limbs. Serologic evaluation and cerebral magnetic resonance imaging to rule out post-infectious causes were normal. Electroneurography (ENG) showed absent sensory action potentials. After 15 days, the patient experienced a relapse of peripheral left facial palsy; ENG was unchanged, and somatosensory evoked potentials showed posterior column involvement. Genetic analysis demonstrated GAA repeat …expansion on both alleles (860 and 900 GAA triplets) of the frataxin gene, and the child was diagnosed with Friedreich ataxia. Neurological findings at the last examination, performed at 7 years of age, were unchanged except for mild bilateral pes cavus. Peripheral seventh cranial nerve palsy could be an early and unusual sign of central nervous system degeneration and axonal sensory neuropathy. Its occurrence in this patient provides further evidence that Friedreich ataxia may have a varied clinical presentation at onset. In accordance with other studies, we believe that the size of both GAA expansions affect the age at onset of the disease, despite atypical phenotype. Show more

Keywords: Facial palsy, axonal neuropathy, Friedreich ataxia, child

Citation: Journal of Pediatric Neurology, vol. 6, no. 2, pp. 151-153, 2008

Authors: Godinho, Savio I. | Khanna, Paritosh C. | Srinivasan, Kaliappan G. | Khademian, Zarir | Krishnamoorthy, Divya

Article Type: Research Article

Abstract: Megalencephalic leukodystrophy with subcortical cysts is a rare, recently described condition. Infantile macrocephaly, initially normal development and gradual neurological deterioration and relative preservation of mental abilities predominate. Magnetic resonance imaging demonstrates an edematous brain with diffuse white-matter abnormalities and subcortical cysts. Megalencephalic leukodystrophy with subcortical cysts has been described as having a predilection for some communities. We report what is thought to be a sporadic case of two siblings of a consanguineous …marriage, not belonging to known susceptible communities, who presented with characteristic features. Show more

Keywords: Megalencephalic leukodystrophy with subcortical cysts, magnetic resonance imaging

Citation: Journal of Pediatric Neurology, vol. 6, no. 2, pp. 155-158, 2008

Authors: Hirschl, Robert | Baig, Mirza N. | Karas, Chris | Gasser, Ryan | Elton, Scott W.

Article Type: Research Article

Abstract: A 14-year-old girl with a suprasellar mixed malignant germ cell tumor was treated with complete surgical resection, chemotherapy, and radiation therapy. We performed a literature search to explore the current treatment options and prognosis of such a diagnosis. In reviewing the literature on mixed germ cell tumors, it appears that platinum-based chemotherapy regimens combined with radiation therapy provide the best outcome in terms of survival rates. Although controversial and debated, initial therapy should include surgical …resection or a stereotactic biopsy when resection is not possible. By obtaining tissue for histopathology, one can tailor treatment plans accordingly and provide greater prognostic accuracy. In this case report, all three therapeutic modalities were used in the treatment of a mixed malignant germ cell tumor. Thus far, 20 months posttreatment, the patient is doing well and is neurologically intact, but diagnosis and treatment were relatively recent. Only time will tell the efficacy of this treatment plan. Show more

Keywords: Mixed malignant germ cell tumor, suprasellar, pediatric neurosurgery, germ cell tumor

Citation: Journal of Pediatric Neurology, vol. 6, no. 2, pp. 159-163, 2008

Authors: Berhouma, Moncef | Jemel, Hafedh | Khaldi, Moncef

Article Type: Research Article

Abstract: Growing skull fractures are rare complications of head injury, affecting almost exclusively young children. This disease is characterized by the progression of a skull fracture with an underlying dural tear, leading to large cranial vault defects and parenchymal porencephalic damages. This report on three cases with a radiological follow-up depicts the difficulty in the management of neglected cases. Three young patients (two months, two years and six years old) were managed for growing skull fracture in …our department since 2001. Even if the three patients were diagnosed early, the therapeutic management was complex because of a long delay due to several reasons. While a cranioplasty and dural repair were performed in all patients, one required a shunt procedure for a large leptomeningeal cyst associated with a unilateral hydrocephalus. The clinical and radiological features are reviewed, as well as pathophysiological hypotheses and therapeutic principles. Show more

Keywords: Leptomeningeal cyst, growing skull fracture, head trauma, encephalomalacia, cranioplasty, head injury sequelae

Citation: Journal of Pediatric Neurology, vol. 6, no. 2, pp. 165-169, 2008

Authors: Mah, Emily Siew Ling | Muridan, Roziah | Cheng, Peh Suat | Ariffin, Hany | Muthurangam, Ramanujam Tindivanam

Article Type: Research Article

Abstract: Primary intracranial primitive neuroectodermal tumors are rare malignant small cell embryonal neoplasms of neuroectodermal origin, accounting for approximately 2.5% of childhood brain tumors and 0.46% of those in adults. There are very few reported cases of extracranial metastases from these tumors. We report a very rare case of primary intracranial primitive neuroectodermal tumor with metastasis to the lungs.

Keywords: PNET, metastasis, thorax

Citation: Journal of Pediatric Neurology, vol. 6, no. 2, pp. 171-175, 2008

Authors: Makino, Keishi | Yano, Shigetoshi | Ohta, Kazutaka | Kuratsu, Jun-ichi

Article Type: Research Article

Abstract: Loculated hydrocephalus (LH) is caused, most frequently, by meningitis or intraventricular hemorrhage. However, uniloculated hydrocephalus caused by neonatal intraventricular hemorrhage is uncommon. We report the case of an infant who developed LH associated with antenatal intraventricular hemorrhage during the neonatal period. LH was suspected by routine ultrasonographic examination at 38 weeks' of gestation. A computerized tomography scan on the 2nd day, after the child was born, confirmed dilatation of the right lateral …ventricle. Furthermore, magnetic resonance imaging examination on the 6th day suggested subependymal hemorrhage in right lateral ventricle. At 5 months' of age, the infant underwent endoscopic septum pellucidum perforation for an increase in ventriculomegaly and also the size of the head circumference. However, 2 months after surgery, a ventriculo-peritoneal shunt was placed because of reappearance of ventriculomegaly. Examination of the child, when she was 2 years old, showed improvement of ventriculomegaly and the child was making appropriate developmental progress. Show more

Keywords: Loculated hydrocephalus, intraventricular hemorrhage, ventriculo-peritoneal shunt

Citation: Journal of Pediatric Neurology, vol. 6, no. 2, pp. 177-182, 2008

Authors: Bhat, Mushtaq Ahmad | Kawoosa, Masarat Sultana | Bhat, Javeed Iqbal | Ali, Wajid

Article Type: Research Article

Abstract: Inadequate exclusive breast-feed is associated with many complications in neonates, which sometimes become life threatening. Hypernatremia is one of the commonest complications, which in severe cases may be associated with recurrent seizures, sinus thrombosis, arterial thrombosis etc. However, intracranial hemorrhage is rarely seen. We present three neonates with severe hypernatremia secondary to inadequate exclusive breast-feed, two of whom developed intracranial hemorrhage during hospital stay with review of literature.

Keywords: Breast-feeding, hypernatremia, intracranial complications

Citation: Journal of Pediatric Neurology, vol. 6, no. 2, pp. 183-187, 2008

Authors: Bettinelli, Alberto | Zucca, Claudio | Radice, Lucia | Stucchi, Sara | Tettamanti, Alberto | Bianchetti, Mario G.

Article Type: Research Article

Abstract: A 10-year-old severely disabled Italian boy on long-term treatment with valproic acid presented with tubular proteinuria, generalized aminoaciduria, normoglycemic glucosuria, and phosphate wasting. Since no other explanation for these renal tubular abnormalities was found, the treatment with valproic acid was discontinued. The abnormalities resolved within 5 months after discontinuing this anticonvulsant. We recommend screening patients on long-term anticonvulsant therapy with valproate periodically for renal tubular dysfunction, either via urinalysis or via measurement …of urinary lactate/creatinine ratio, if clinical suspicion is high. Show more

Keywords: Fanconi syndrome, kidney disease, valproic acid, epilepsy

Citation: Journal of Pediatric Neurology, vol. 6, no. 2, pp. 189-190, 2008

Authors: Ibrahim, Shahnaz | Khan, Shamshad Gulab

Article Type: Research Article

Abstract: We report a 3-year-old girl with characteristic clinical, electroencephalographic and neuroradiological features of Rett syndrome (RS) – an X-linked dominant, neurodevelopmental disorder caused by mutation in methyl-CpG binding protein 2 (MECP2) gene. It predominantly affects females and is one of the commonest causes of mental retardation in females. It is characterized by progressive loss of intellectual functioning, fine and gross motor skills and communicative abilities, deceleration of head growth, and the development …of stereotypic hand movements, occurring after a period of normal development. A 3-year-old girl was brought from our neighboring country with progressive loss of milestones. She had a normal development till one year of age when she gradually lost her motor milestones and started socially withdrawing from her surroundings. She also started excessive hand playing which were stereotypical and repetitive. She was microcephalic and had marked postural hypotonia. Her electroencephalography and magnetic resonance imaging showed the characteristic change associated with RS. These clinical features support the diagnosis of RS even though genetic analysis was not done. This is the first case report of RS from Pakistan. Show more

Keywords: Rett syndrome, stereotypic hand movements

Citation: Journal of Pediatric Neurology, vol. 6, no. 2, pp. 191-194, 2008

Authors: Lancon, John A.

Article Type: Research Article

Citation: Journal of Pediatric Neurology, vol. 6, no. 2, pp. 195-195, 2008