Journal of Pediatric Neurology - Volume 6, issue 1

Authors: Gordon, Neil

Article Type: Research Article

Abstract: Syncope and autonomic failure are defined. Then the symptoms are described. There are three types of neurally mediated syncope; situational, carotid sinus hypersensitivity, and vasovagal syncope, which is the commonest form. The diagnosis can most often be made by taking a detailed history, but the tilt table test will help to confirm this. Details of the test are given. The different terms that have been used to describe this form of syncope have caused confusion, but …neurally mediated syncope seems to be the best. There may often be difficulties in differentiating neurally mediated syncope from epilepsy, with all the problems a wrong diagnosis may cause, both personal and social, especially in the case of reflex anoxic seizures. The ocular compression test can help in making a definite diagnosis. Management will include reassurance, and advice on avoiding situations, which may result in an attack. Drugs are of limited value, and if syncope occurs frequently, cardiac pacemakers may have to be considered. This is justified, not only by the distress the attacks can cause to the individual, but also by such problems as driving and working with machinery. Show more

Keywords: Neurally mediated syncope

Citation: Journal of Pediatric Neurology, vol. 6, no. 1, pp. 1-4, 2008

Authors: Miller-Horn, Jill W. | Kaleyias, Joseph | Valencia, Ignacio | Melvin, Joseph J. | Khurana, Divya S. | Hardison, H. Huntley | Marks, Harold | Legido, Agustin | Kothare, Sanjeev V.

Article Type: Research Article

Abstract: Randomized controlled trials have shown that stimulants reduce symptoms of impulsivity and hyperactivity in children with attention deficit/hyperactivity disorder (ADHD); however, these rigid protocols show no advantage of one medication over another. Our study examined the question of differential efficacy and tolerability of five medications used for ADHD, in the open-label setting of our outpatient child neurology clinic. This retrospective study identified 137 children and adolescents (109 boys and 28 girls), with …a mean age of 10 years (range 4 to 19 years) treated for ADHD. Treatment options were amphetamine/dextroamphetamine extended release (adderall XR) in 19.0%, amphetamine/dextroamphetamine (adderall), osmotic controlled-released (OROS) formulation of methylphenidate (OROS-MPH, concerta) in 29.2%, atomoxetine (strattera) in 21.9% and methylphenidate standard release (MPH) in 16.8%. Global effectiveness was assessed for each medication. Overall, 78% of patients improved with medication, with no significant statistical difference in efficacy among the five medications. Side effects included decreased appetite (14.6%), insomnia (10.2%), headaches (7.3%), and tics (3.7%). The only difference in side effects was with atomoxetine showing a significantly lower incidence of headaches than amphetamine/dextroamphetamine XR, amphetamine/dextroamphetamine or OROS-MPH. In conclusion, our results in the open-label setting were comparable to those found in randomized controlled trials; the medications we examined were equally effective with minimal differences in side effect profiles. Show more

Keywords: ADHD, children, stimulants, non-stimulants, efficacy, side effect, tolerability

Citation: Journal of Pediatric Neurology, vol. 6, no. 1, pp. 5-10, 2008

Authors: Toopchizadeh, Vahideh | Barzegar, Mohammad

Article Type: Research Article

Abstract: Guillain-Barré syndrome (GBS) is classified into acute inflammatory demyelinating polyneuropathy (AIDP), acute motor axonal polyneuropathy (AMAN), and acute motor sensory axonal polyneuropathy (AMSAN), but little is known about the incidence of the subtypes and electrophysiologic changes in Northwest of Iran. The aim of this study is to present the electrophysiologic pattern and incidence of various subtypes of childhood GBS. Electrophysiologic features of 72 children (aged 1–15 years old) were studied prospectively during …two years in Tabriz Children Hospital. Electrodiagnostic tests were performed in the first 2 weeks of the disease onset. Motor nerve conduction study was performed on the tibial, peroneal, median, and ulnar nerves. Sensory nerve conduction study was performed on the median and sural nerves, F-waves were also studied on the tibial, median, and ulnar nerves. Compound muscle action potential (CMAP) and sensory nerve action potential (SNAP) amplitudes, distal latencies, nerve conduction velocities and F-wave latencies were measured. In this concern, any conduction block and temporal dispersion were noted. Needle electromyography was performed, motor unit action potential changes, and spontaneous activity were studied. Patients were classified into AIDP, AMAN, and AMSAN on the basis of electrodiagnostic criteria. About half of the patients (47.2%) had AIDP form, 44.4% had AMAN, and 5.6% had AMSAN. Moreover, Fisher variant was observed in 2.8% of the cases. The most common electrophysiologic findings were low amplitude CMAP and abnormal F- waves, i.e. 97.2% and 88.9% respectively. Twelve patients (16.8%) required assisted ventilation, 75% of them had AMAN form of GBS. AMAN form of childhood GBS is relatively common in Iran in comparison to Western countries. Respiratory failure resulting in mechanical ventilation is more probable in this form. Show more

Keywords: Electrophysiologic tests, Guillain-Barré syndrome, subtypes, children

Citation: Journal of Pediatric Neurology, vol. 6, no. 1, pp. 11-16, 2008

Authors: Southern, Caroline L. | Beare, Nicholas A.V. | Falsini, Benedetto | Lochhead, Jonathan | Molyneux, Malcolm E. | Taylor, Terrie E. | Harding, Simon P.

Article Type: Research Article

Abstract: We aimed to investigate the visual evoked potential (VEP) in children unconscious with Plasmodium falciparum malaria, a common cause of death in Africa. Flash VEPs were carried out in Malawi during one peak malaria season. Children were included in the study if they had P. falciparum malaria and reduced consciousness – Blantyre Coma Score (BCS) 4 or less out of 5. Initial VEPs were performed after stabilising the patient and commencing treatment. To investigate optimal …VEP protocols, varying stimulus parameters were tested. Where possible, VEPs were repeated daily until the child recovered full consciousness (BCS 5). The initial traces of 40 children were included in the study and serial traces were obtained in 30 of these. Mean VEP latency was greater on admission than either on day 1 or at BCS 5 (paired t-test, P < 0.05) for all test protocols. There was a positive correlation between VEP latency on admission and parasite count and a negative correlation with blood glucose on admission (Spearman's rank correlation, P < 0.05 for all test protocols). Children with severe P. falciparum malaria have delayed VEP responses and recovery occurs in parallel with return to normal consciousness. VEP recordings are quick and reproducible and may find a place in assessing levels of coma and cerebral function in severe malaria. Show more

Keywords: Coma, malaria, visual evoked potential, children

Citation: Journal of Pediatric Neurology, vol. 6, no. 1, pp. 17-24, 2008

Authors: Lagunju, Ike A. | Adedokun, Babatunde O.

Article Type: Research Article

Abstract: Cerebral palsy (CP) is one of the leading causes of childhood disability worldwide. Children in the developing world are disproportionately affected with majority of the cases resulting from preventable causes. Various topographic classes of CP have been described in literature, with varying degrees of functional impairments and neuro-cognitive dysfunction. We aimed to compare the characteristics of children with quadriplegic and hemiplegic CP. Two hundred and thirty six consecutive cases of CP, 186 with spastic …quadriplegia and 50 with spastic hemiplegia seen over a period of 30 months were studied. Their demographic characteristics, functional and associated neuro-cognitive impairments were compared using the SPSS for windows software. The study showed relatively high proportions (20.3%) of post-neonatally acquired CP. Children with spastic quadriplegia were found to present at a relatively younger age than those with hemiplegia. Spastic hemiplegia was found to be associated with mild to moderate degrees of functional impairment (P < 0.001), and a significantly lower prevalence of hearing impairment (P < 0.001), speech defects (95% CI OR =1.50–7.19), mental retardation (0.026) and growth impairment (P< 0.001). Although epilepsy was a common associated deficit in quadriplegic CP, a higher prevalence was found in the hemiplegic group. We can conclude that hemiplegic CP is a less severe disease compared with quadriplegic CP. Post-neonatally acquired CP accounts for a significant proportion of CP in Nigeria, and majority are due to intracranial infections. Although, CP cannot be cured, prompt diagnosis of this condition and early identification of the associated deficits and institution of the necessary management will often improve the child's capabilities. Show more

Keywords: Quadriplegia, hemiplegia, cerebral palsy

Citation: Journal of Pediatric Neurology, vol. 6, no. 1, pp. 25-30, 2008

Authors: Asadi-Pooya, Ali Akbar | Asadi-Pooya, Ahmad | Rosen, David

Article Type: Research Article

Abstract: Hearing impairment is the most common complication of meningitis. This prospective study was designed to determine the prevalence of hearing impairment after aseptic and bacterial meningitis in children. All patients 5–15 years of age with bacterial or aseptic meningitis admitted in Shiraz University Hospitals were included in a 40-month period study. Pure tone audiometry was arranged at the time of discharge. In total, 115 patients were studied. Sensorineural hearing impairment was detected in 35/83 of aseptic …meningitis patients (42.2%) and 14/32 of bacterial meningitis patients (46.8%). There were 13 patients with bilateral hearing loss in aseptic meningitis group and eight patients in bacterial meningitis group (P=0.389). There were seven patients with moderate to severe hearing loss in bacterial meningitis group and four patients in aseptic meningitis group (P=0.014). This study demonstrated high prevalence of sensorineural hearing impairment following both bacterial and aseptic meningitis. Bilateral hearing impairment was common in both groups. Hearing impairment was more severe among bacterial meningitis patients. Show more

Keywords: Child, hearing impairment, meningitis

Citation: Journal of Pediatric Neurology, vol. 6, no. 1, pp. 31-34, 2008

Authors: Al-Futaisi, Amna | Abdwani, Raghad | Fathalla, Mahmoud | Al Zakwani, Ibrahim | Javad, Hashem | Al-Zuhaibi, Sana | Ganesh, Anuradha | Koul, Roshan

Article Type: Research Article

Abstract: Idiopathic intracranial hypertension (IIH) is a relatively rare disease in children. It is defined as raised intracranial pressure in the absence of clinical, laboratory or radiological evidence of an intracranial space occupying lesion. We aimed to evaluate the clinical and therapeutic features of IIH in a presenting group of Omani pediatric population. We conducted a retrospective chart review of patients below 18 years of age diagnosed with IIH between 1992 and 2006 according to Dandy's Modified …Criteria at Sultan Qaboos University Hospital, Oman. Statistical analyses were conducted using McNemar's test. Nineteen patients were included in the study, 11 (58%) were males and 8 (42%) were females, with a ratio of 1.4:1. The median age at presentation was 8.5 years. The average body mass index of the patients was 18.1 with only 2 (11%) children being obese, both males and pubertal. All the patients except 1 (5%) were symptomatic at presentation. The commonest symptom was headache found in 16 (84%) patients. This was followed by visual symptoms in half of them in the form of diplopia (16%) or transient visual obscuration (37%). All patients had bilateral papilledema, 7 (37%) had visual field defects, 4 (21%) had an enlarged blind spot and 3 (17%) had restricted visual field. Secondary causes of intracranial hypertension were not identified but the associated conditions were sickle cell disease, Behcet disease, neurofibromatosis type 1, varicella infection, and brucellosis. Seventeen (89%) patients showed good response to treatment with acetazolamide, steroids or both, with resolution of symptoms and signs (p<0.001). Two (11%) had deterioration in their vision despite aggressive medical treatment and were referred for surgical intervention. The epidemiologic features in the studied pediatric population were similar to that of other studies. The prompt recognition, evaluation, and treatment of IIH are needed to alleviate symptoms and to preserve visual function. There was a favorable response to medical treatment in this study cohort. Show more

Keywords: Idiopathic intracranial hypertension, papilledema, headache

Citation: Journal of Pediatric Neurology, vol. 6, no. 1, pp. 35-38, 2008

Authors: Omran, Mohammadreza Salehi | Khalilian, Elham | Mehdipour, Elnaz | Juibary, Ali Ghabeli

Article Type: Research Article

Abstract: Febrile seizure (FS) is the most common neurological disorder in the pediatric population. The clinical characteristics of 630 patients who had episodes of FS were retrospectively reviewed. No preference was seen for either sex (1.2:1). In those cases with FS, 514 (81.6%) convulsions were simple and 116 (18.4%) were complex. A family history of FS was present in 25.7% of the cases reviewed. Generalized seizure types accounted for 99% of the cases. Age of seizure onset …peaked at 10 month. The etiology of fever was identified in 85.3% of cases. Gastroenteritis was the first etiology of fever accounting for 27.3% of the cases. Recurrent FS was observed in 33.5%. All of patients had complete blood count and electrolytes including sodium, potassium, calcium and glucose level in their records. Show more

Keywords: Febrile seizures, fever, infection, Iran

Citation: Journal of Pediatric Neurology, vol. 6, no. 1, pp. 39-42, 2008

Authors: Hundozi-Hysenaj, Hajrie | Boshnjaku-Dallku, Iliriana

Article Type: Research Article

Abstract: Cerebral palsy (CP) is often associated with epilepsy. We sought to determine the frequency with which epilepsy is associated with CP and to define the relationship in terms of gender, etiology, pathophysiology, and topographical parameters. This was a retrospective analysis of children with CP treated at the University Clinical Centre of Kosova (UCCK) in Prishtina, over a 5-year period, 2000–2005. A total of 154 children (aged 7–15 years) with CP, treated at the UCCK were enrolled. …CP was associated with epilepsy in 63 cases (40.9%), 29 (46.0%) of which had spastic quadriparetic CP; 17 (27.0%), diplegic CP; and 15 (23.8%), hemiparetic CP. For the quadriparetic children, there was no significant difference in the number associated with epilepsy between the genders (female, 45.2%; male, 46.9%). Epilepsy was frequently associated with CP, with the spastic quadriparetic group being most affected. There was no large difference in the number associated with epilepsy between the right and left hemiparetic CP cases. Tonic-clonic epilepsy was more frequently associated with hemiparetic CP, whereas partial-type epilepsy was more often associated with quadriparetic CP. Show more

Keywords: Cerebral palsy, epilepsy

Citation: Journal of Pediatric Neurology, vol. 6, no. 1, pp. 43-46, 2008

Authors: Akuma, Akuma O. | Sharples, Peta M. | Jardine, Philip E.

Article Type: Research Article

Abstract: Two teenage girls with episodic neuropsychiatric symptoms, altered consciousness, collapse and seizures requiring intensive care admissions are presented. Both were euthyroid at presentation and had markedly elevated anti-thyroperoxidase antibody. Extensive neurometabolic tests and cranial imaging were normal. However, psychometric testing showed evidence of memory and cognitive difficulties. Both were diagnosed as Hashimoto encephalopathy and one improved after steroid treatment while the other was lost to follow-up after defaulting scheduled elective hospital admission …for steroid therapy. Show more

Keywords: Hashimoto encephalopathy, antithyroid antibodies, status epilepticus

Citation: Journal of Pediatric Neurology, vol. 6, no. 1, pp. 47-51, 2008

Authors: Mordekar, Santosh R. | Rittey, Christopher D. | Jaspan, Timothy | Connolly, Daniel J. | Whitehouse, William P.

Article Type: Research Article

Abstract: Acute disseminated encephalomyelitis is an acute demyelinating disorder of the central nervous system, characterized by an encephalopathy, multifocal grey matter and white matter involvement. Pediatric high-grade gliomas represent a heterogeneous group of tumors accounting for 15–20% of all pediatric brain tumors. We describe two children who were incorrectly diagnosed as acute disseminated encephalomyelitis initially, but subsequently found to have glioblastoma multiforme. The cases would exemplify the diagnostic difficulties in acute disseminated encephalomyelitis.

Keywords: ADEM, encephalopathy, glioma, children

Citation: Journal of Pediatric Neurology, vol. 6, no. 1, pp. 53-56, 2008

Authors: Gupta, Vijay Kumar | Dewan, Vivek | Nandan, Devki | Kumar, Praveen | Bahl, Dheeraj | Kaur, Nirmaljeet

Article Type: Research Article

Abstract: Although abnormal cerebrospinal fluid findings similar to aseptic meningitis are reported in up to 80% infants with congenital syphilis, overt symptomatic meningeal syphilis is quite a rare presentation, especially at an early age. We report a neonate who presented at 60 hours of life with irritability, poor activity, seizures and bulging fontanel. He was confirmed to have acute syphilitic meningitis.

Keywords: Congenital syphilis, meningitis, newborn

Citation: Journal of Pediatric Neurology, vol. 6, no. 1, pp. 57-60, 2008

Authors: Kaleyias, Joseph | Bharucha, Diana X. | Yorns, William | Miller-Horn, Jill W. | Finck, Christine | Piatt, Joseph H. | Kothare, Sanjeev V.

Article Type: Research Article

Abstract: We present a case of a 4-month-old girl with a history of a huge axillary cystic hygroma since birth, with elective surgical resection of the lesion and subsequent development of infection at the surgery site. During hospitalization for the infection, she developed seizures, a bulging fontanelle, increased head circumference, and widely separated cranial sutures. Brain imaging demonstrated new, large, bilateral subdural fluid collections with membrane enhancement around the left-sided collection. She was managed by …subdural tap, bilateral craniotomy, broad-spectrum antibiotics, and subduroperitoneal shunt insertion. Ultimately, she had complete recovery without complications. This case adds to the spectrum of intracranial conditions that have been associated with cystic hygroma. Show more

Keywords: Subdural hygroma, axillary cystic hygroma, neonate

Citation: Journal of Pediatric Neurology, vol. 6, no. 1, pp. 61-64, 2008

Authors: Yuceer, Nurullah | Kurul, Semra

Article Type: Research Article

Abstract: A premature infant with posthemorrhagic hydrocephalus associated with bronchopulmonary dysplasia is presented in this study. Firstly ventriculosubgaleal shunt surgery was performed. Then, ventriculosubgaleal shunt was converted to a ventriculoperitoneal shunt. Postoperative courses of both ventriculosubgaleal shunt surgery and ventriculoperitoneal shunt surgery were uneventful. This illustrative case suggests that ventriculosubgaleal shunt can be an effective method for treating posthemorrhagic hydrocephaus associated with bronchopulmonary dysplasia.

Keywords: Premature infant, posthaemorrhagic hydrocephalus, ventriculoperitoneal, ventriculosubgaleal shunt

Citation: Journal of Pediatric Neurology, vol. 6, no. 1, pp. 65-68, 2008

Authors: Jha, Sanjeev | Agarwal, Vikas

Article Type: Research Article

Abstract: Arsenic poisoning has multiple causes including exposure to pesticides, rodenticides, paints, pigments, alloys and consumption of water contaminated with arsenic. In India, traditional home made, herbal remedies are widely consumed to enhance potency and vitality, and they are often contaminated with toxic ingredients, including arsenic. We report a case of chronic arsenic poisoning, due to prolonged consumption of such a homemade vitaliser that contained arsenic as chief ingredient. Our patient presented with …polyneuropathy and cutaneous lesions that recovered significantly with dimercaprol therapy, despite delayed diagnosis. Show more

Keywords: Arsenic, polyneuropathy, home made-vitaliser, Mees' line, quadriparesis

Citation: Journal of Pediatric Neurology, vol. 6, no. 1, pp. 69-71, 2008

Authors: Onal, Hasan | Alhaj, Safa | Ozdil, Mine | Ozyilmaz, Isa | Altun, Gurkan | Aydin, Ahmet

Article Type: Research Article

Abstract: Combined methylmalonic aciduria and homocystinuria is a very rare disease caused by a defect in the synthesis of two cofactors in cobalamin dependent reactions, i.e. adenosylcobalamin and methylcobalamin, active forms of vitamin B12. Early onset disease consists of neurological, hematological and gastrointestinal abnormalities seen in the first year of life and some minor face abnormalities (long philtrum, wide forehead, big and low set ears, long face). Here we report a 4-month-old male with failure to thrive, …pancytopenia and neurological disturbance. Show more

Keywords: Vitamin B12, methylmalonic aciduria, homocystinuria

Citation: Journal of Pediatric Neurology, vol. 6, no. 1, pp. 73-76, 2008

Authors: Kumar, Raj | Vaid, Vivek Kumar | Kalra, Samir Kumar | Mahapatra, Ashok Kumar

Article Type: Research Article

Abstract: Intracranial aneurysms are extremely uncommon in children (⩽ 18 years old) and their incidence increases with age. Their epidemiology is poorly understood and certain features like location, morphology and presentation make them unique in comparison to adults. The internal carotid artery bifurcation is the most frequent site for aneurysm in this age group. We report a case of an 8-year-old boy with history of sudden unconsciousness followed by generalized tonic clonic seizures. The computerized …tomography scan revealed subarachnoid hemorrhage with blood seen in the left Sylvian fissure, along tentorium cerebelli and posterior falx. There was focal intracerebral hematoma in the left temporal lobe abutting the Sylvian fissure and blood could also be seen in the fourth, third and both lateral ventricles with mild ventriculomegaly. The digital subtraction angiography revealed a large intracranial aneurysm arising from the left proximal anterior cerebral artery just distal to left internal carotid artery bifurcation. The aneurysm was clipped using standard microsurgical techniques. The child did well postoperatively. Intracranial aneurysms are rare in childhood and proximal artery anterior cerebral artery aneurysms are amongst the rarer sites. This report highlights one such rare case. Show more

Keywords: Intracranial aneurysm, pediatric aneurysm, anterior cerebral artery aneurysm

Citation: Journal of Pediatric Neurology, vol. 6, no. 1, pp. 77-80, 2008

Authors: Unalp, Aycan | Dizdarer, Ceyhun | Ozcan, Tugrul | Bilgili, Gökmen

Article Type: Research Article

Abstract: Adrenoleukodystrophy is a neurodegenerative disease with X-linked recessive inheritance, which rapidly leads to neurological dysfunctions in childhood. It could only be treated by bone marrow transplantation at early stage. Here we report a case of X-linked adrenoleukodystrophy who was admitted to our hospital with status epilepticus and subclinical adrenal insufficiency. A 13-year-old boy, who had initially presented to a local hospital with seizures, was transferred to our hospital upon having generalized tonic-clonic …seizures, which were followed by focal seizures. He had been introverted and amnesiac for the previous 2 months; hence, he had been receiving medication for major depression. However, his clinical condition worsened despite the medication. Although the patient lacked typical symptoms, he was diagnosed as having adrenoleukodystrophy upon detection of hyperintense lesions in occipitoparietal white matter by brain magnetic resonance imaging and increased serum very long chain fatty acid concentrations. Thus, we would like to emphasize the importance of awareness of physicians, particularly the pediatricians, neurologists and psychiatrists for clinical and laboratory characteristics of adrenoleukodystrophy. Show more

Keywords: Adrenoleukodystrophy, adolescent, magnetic resonance imaging, adrenal insufficiency

Citation: Journal of Pediatric Neurology, vol. 6, no. 1, pp. 81-84, 2008

Authors: Kumar, Raj | Singhal, Namit | Kumar, Mahapatra Ashok

Article Type: Research Article

Citation: Journal of Pediatric Neurology, vol. 6, no. 1, pp. 85-87, 2008

Authors: Kumar, Raj | Kumar, Mahapatra Ashok

Article Type: Other

Citation: Journal of Pediatric Neurology, vol. 6, no. 1, pp. 89-90, 2008

Article Type: Other

Citation: Journal of Pediatric Neurology, vol. 6, no. 1, pp. 91-92, 2008