Journal of Pediatric Neurology - Volume 9, issue 2

Authors: Zeng, Linan | Luo, Rong | Zhang, Lingli

Article Type: Research Article

Abstract: To determine the influence of dose on the efficacy and safety of adrenocorticotropic hormone (ACTH) for infantile spasms. We searched six relative databases. Randomized controlled trials and crossover studies, which meet our inclusion criteria were considered for inclusion. The quality of included trials was evaluated with respect to sequence generation, allocation concealment, blinding and incomplete data. We performed both direct and indirect meta-analysis to evaluate the efficacy and safety of high-dose and …low-dose of ACTH. Results: A total of 168 reports were retrieved, and after scrutiny seven reports of trials containing three head to head comparisons, two vigabatrin comparisons and two prednisone comparisons were included. In direct comparison meta-analysis, studies were homogenous (P > 0.10). There was a trend towards more complete cessation of spasms in the low-dose ACTH treated group (RR: 0.86, 95% confidence interval [CI]: 0.57–1.31), though statistically not significant. Relapse was more frequent in low-dose ACTH groups (RR: 0.69, 95% CI: 0.22–2.19) but without demonstrating statistic difference. No difference was present for disappearance of hypsarrhythmia between high and low-dose ACTH (RR: 0.93, 95% CI: 0.57–1.54). In indirect meta-analysis the comparison of complete cessation of spasms between high-dose and low-dose ACTH from indirect meta failed to show any significant difference. There was no significant difference between two dosages with respect to relapse rates. High-dose ACTH produced more adverse effects than low-dose group. A low-dose ACTH therapy might be preferable for its comparative efficacy and lower risk of adverse effects. Show more

Keywords: Infantile spasms, ACTH, dosage, systematic review

DOI: 10.3233/JPN-2011-0469

Citation: Journal of Pediatric Neurology, vol. 9, no. 2, pp. 141-149, 2011

Authors: Caylak, Emrah

Article Type: Research Article

Abstract: Developmental dyslexia (DD) or reading disability is characterized by unexpected problems in reading acquisition, despite average intelligence. It is considered the most common neurodevelopmental disorder, and many researchers aimed to identify the cognitive, neurobiological and genetic etiology of DD. At the cognitive level, the hypothesis of auditory temporal processing deficit has been widely investigated. The aim of this review is to present some of the most exciting researches on DD in the domains of this theory …that will help future studies to follow. Show more

Keywords: Dyslexia, deficit, theory, auditory temporal processing, temporal order

DOI: 10.3233/JPN-2011-0455

Citation: Journal of Pediatric Neurology, vol. 9, no. 2, pp. 151-168, 2011

Authors: Winner, Paul

Article Type: Research Article

Abstract: To assess the feasibility, efficacy, and tolerability of early intervention with sumatriptan tablets formulated with RT Technology™ (sumatriptan RT) for adolescent migraine. Conventional triptan tablets, a treatment of choice for migraine in adults, have not been demonstrated reliably effective in adolescents in prospective, placebo-controlled studies. Neither the early-intervention approach nor sumatriptan RT, a fast-disintegrating/rapid-release oral tablet, has previously been studied in adolescent migraineurs. Males or females 12 to 17-year-old diagnosed with …migraine with or without aura were asked to treat four migraine attacks over a 6 mo period with sumatriptan RT 100 mg as early intervention (i.e., administered within 30 min of attack onset while pain was still mild). Of the 35 patients who enrolled, 32 treated at least one migraine attack, and 23 treated all four-migraine attacks. Pain-free response 2 hr postdose (primary endpoint) was reported in 71% of the 112 attacks treated. Migraine-free response (i.e., no pain; no nausea, vomiting, photophobia, or phonophobia; and no use of rescue medication) 2 hr postdose was reported in 69% of attacks. Response rates were consistent from attack to attack. Rescue medication was used in 19% of migraine attacks. In 112 attacks, a total of 25 adverse events (none serious) were reported in nine patients. The most common adverse events were worsening of symptoms (n=5), neck pain (n=4), and chest tightening (n=4). Early intervention with sumatriptan RT constitutes a promising approach to treating migraine in adolescents and warrants further assessment in controlled clinical trials. Show more

Keywords: Headache, migraine, adolescent, sumatriptan, triptan

DOI: 10.3233/JPN-2011-0471

Citation: Journal of Pediatric Neurology, vol. 9, no. 2, pp. 169-175, 2011

Authors: Praveen-Kumar, Srikanteshwara | Sinha, Sanjib | Taly, Arun B. | Bharath, Rose Dawn | Bindu, Parayil S. | Murthy, Subha S. | Ravi, Vasanthapuram

Article Type: Research Article

Abstract: Reports of magnetic resonance imaging (MRI) findings in subacute sclerosing panencephalitis (SSPE) with clinical and electroencephalography (EEG) correlation are few. To describe the MRI features of SSPE and correlate with its clinical features and EEG. Clinical, MRI and EEG data of 33 patients (male/female 19/14; age: 11.4 ± 5.3 yr) were reviewed. All patients had raised IgG anti-measles antibody (⩾ 1:625) in cerebrospinal fluid. Their clinical features were myoclonus (100%), seizures (42%), visual disturbances …(18%), pyramidal signs (30%), extrapyramidal symptoms (27%), and cognitive impairment (79%) and behavioral changes (21%). EEGs were universally abnormal: diffuse slowing of background activity-all; periodic complexes-27 (symmetrical): High voltage delta waves (55%) and sharp & slow wave discharges (45%). MRI findings included – normal (9%), cerebral atrophy (78%), white matter (76%) and basal ganglia (6%) changes. Restricted diffusion in periventricular/subcortical region was noted in 4/23 patients (stage 2). Magnetic resonance spectroscopy showed decreased N-acetyl aspartate peaks and elevated choline peaks in 75% and lactate peak in 25% (stage 3) of patients. Correlation of background delta activity among patients with MRI signal abnormalities and those without any signal change was noted (P=0.02). There was significant correlation between visual deficits and bilateral parieto-occipital white matter signal changes (P=0.03). There was no other EEG-MRI or clinical-MRI correlation. MRI abnormalities in SSPE are diverse and may or may not reflect underlying pathology. Visual deficits were observed in patients with parieto-occipital white matter signal change. Restricted diffusion of signal abnormalities and lactate peak in magnetic resonance spectroscopy were uncommon and it may add to better understanding. Show more

Keywords: EEG, MRI, periodic complexes, SSPE

DOI: 10.3233/JPN-2011-0457

Citation: Journal of Pediatric Neurology, vol. 9, no. 2, pp. 177-185, 2011

Authors: Jain, Kapil | Gupta, Priyanka | Srivastava, Arun Kumar | Kumar, Raj

Article Type: Research Article

Abstract: Ventriculo-peritoneal (VP) shunt and endoscopic third ventriculostomy (ETV), both are well-established therapeutic modalities for the management of hydrocephalus. However, the treatment of choice is still controversial due to non-uniformity of the pre-existing data. It was a single institute retrospective cum prospective study enrolling 68 pediatric patients of congenital aqueductal stenosis (neonate to 18-year-old, mean age 48.2 ± 67.9 mo). The present study dealt with a uniform population of congenital aqueductal stenosis excluding …secondary causes like post-infectious, post-hemorrhagic and tumor-related aqueductal stenosis. There were 54 patients in the VP shunt group and 14 in the ETV group. In the VP shunt group, there was no intra-operative or post-operative mortality and none had cerebrospinal fluid leak. Technical failure rate (shunt malfunction within 5 days of surgery) was 1.8% after primary shunting procedure and 4.5% after shunt revisions. Among 38 patients with a minimum available follow-up of ⩾ 6 mo (mean 34.5 ± 29.3 mo), the overall incidence of shunt obstruction after primary VP shunt was 36.8% out of which 21.1% blocked within a year. The shunt infection rate was 5.3%, which presented clinically within 3 mo of surgery. Two (5.3%) patients developed symptomatic slit ventricles and 13.2% patients had new-onset seizures. In the ETV group, there was one (7.1%) technical failure where the stoma could not be made due to severe bleeding. Two (14.2%) patients had intra-operative bleeding and the post-operative mortality was 7.1% (cause of death: ventriculitis). Follow-up ranging from 6–52 mo (mean 15.4 ± 13.6 mo) was available in 10 patients. Stoma blocked in 50% patients and all of them presented by around 1 mo period. Although precise statistical comparison could not be done due to small and disparate number of cases in the two groups, the data is still valuable in the Indian context. After ETV, there was no long-term morbidity whereas 61% patients of VP shunt continued to have some or other problem in the form of shunt obstruction, infection, new-onset seizures or slit ventricle syndrome. We conclude that depending on the expertise, ETV should be offered as the treatment of choice in congenital aqueductal stenosis because of its less frequent association with long-term morbidities. Show more

Keywords: Aqueductal stenosis, ventriculo-peritoneal shunt, endoscopic third ventriculostomy

DOI: 10.3233/JPN-2011-0456

Citation: Journal of Pediatric Neurology, vol. 9, no. 2, pp. 187-193, 2011

Authors: Standish, John C. | Hilmas, Elora | Falchek, Stephen J.

Article Type: Research Article

Abstract: The purpose of this study was to investigate if levetiracetam (LEV) is an effective agent in the treatment of status epilepticus (SE) in children, evaluate the dosing of LEV being used, and to record the incidence of adverse effects. A retrospective chart review was done for patients who had received LEV for the treatment of SE and grand mal seizure at a 180-bed academic pediatric teaching hospital. Patients met inclusion criteria if they had received LEV …for the treatment of SE, and if the dose, administration time, and administration route were documented in their charts. The primary outcome was the rate of seizure cessation attributed to treatment with LEV. Additional data collected included overall rate of seizure cessation regardless of agent used, rate of seizure cessation based on age, type of seizure(s) experienced, history of intractable seizures, and where in the sequence of therapy LEV was used. A total of 20 of 51 patients met inclusion criteria and treatment with LEV resulted in seizure cessation in 15 patients (75%). The average dose received by patients was 37.5 mg/kg (range: 16–98.8 mg/kg) as an intravenous bolus. The adverse effects experienced were similar to what is described in the literature. LEV may be a successful first line or adjunct treatment for pediatric patients in SE regardless of the patients' age, type of seizure, or history of intractable epilepsy. It also appears to be a safe treatment option. Optimal dosing still needs to be defined. Show more

Keywords: Levetiracetam, status epilepticus

DOI: 10.3233/JPN-2011-0458

Citation: Journal of Pediatric Neurology, vol. 9, no. 2, pp. 195-201, 2011

Authors: Masri and, Amira | Shanab, Mayada Abu

Article Type: Research Article

Abstract: Paroxysmal non-epileptic disorders (PNEDs) are often misdiagnosed as epilepsy. This study describes cases of misdiagnosed PNEDs. In addition, it identifies aspects of the clinical assessment that may assist in reaching a correct diagnosis. A total of 100 patients (58 boys and 42 girls) were included. Misdiagnosed PNEDs included: breath-holding spells (37%); excessive jitteriness and/or atypical hyperekplexia (15%); vasovagal attack (12%); psychogenic seizures (11%); gastroesophageal reflux (10%); hyperekplexia (5%); masturbation (3%); head nodding …(2%); tic (2%); paroxysmal torticollis (1%); migraine (1%); vitamin B12 deficiency-induced tremor (1%). Forty-four (44%) patients were misdiagnosed with epilepsy and parents presented for a second opinion, while 56 (56%) patients were referred with a suspicion of epilepsy. Forty-two (42%) patients received antiepileptic treatment before presenting to us. History emerged as the most helpful diagnostic tool. Examination was only useful in hyperekplexia, while mobile phone video camera emerged as a potentially useful but underutilized diagnostic tool and were present in 85% of the patients. PNEDs are often misdiagnosed as epilepsy. Elements that were helpful in making a correct diagnosis included history, examination, and witnessing the attack in clinic or on mobile phones. Show more

Keywords: Paroxysmal non-epileptic events, breath-holdings spells, hyperekplexia, vasovagal syncope, masturbation, mobile phone

DOI: 10.3233/JPN-2011-0477

Citation: Journal of Pediatric Neurology, vol. 9, no. 2, pp. 203-208, 2011

Authors: Dogan, Asuman | Yıldırım, Adem | Karabay, Ilkay | Dost, Gulseren | Ozgirgin, Nese a

Article Type: Research Article

Abstract: We determined the developmental level of 107 children (mean age, 66.41 ± 32.92 mo) with cerebral palsy in an inpatient rehabilitation program and assessed both the difference between calendar age and developmental age and the effect of developmental level on motor function improvement. The Denver II test was used to reveal the difference between calendar age and developmental age, and we determined whether there was a statistically significant difference between the admission and discharge motor function …measurements of the subjects. The effect of the Denver II developmental levels of language, fine-motor, personal-social, and gross-motor skills on motor function was investigated. We found a statistically significant difference between the developmental levels and calendar ages of the study subjects. Denver II test results revealed a statistically significant difference between the personal-social, gross-motor, and fine-motor levels of the subjects. We found no difference only between personal-social and language levels. Gross-motor development was the most delayed value by calendar age. Regardless of developmental level, there was a statistically significant difference between motor activity levels at admission and discharge as revealed by the Gross-motor Functional Classification System and the Gross-motor Function Measure. The developmental ages of the subjects were much lower than their calendar ages. We concluded that the Denver II language and fine-motor developmental level did not have an effect on functional gains but that the subjects with good personal-social and gross-motor function developmental levels exhibited relatively better motor function gains at the time of discharge. Show more

Keywords: Cerebral palsy, Denver II, motor function

DOI: 10.3233/JPN-2011-0459

Citation: Journal of Pediatric Neurology, vol. 9, no. 2, pp. 209-214, 2011

Authors: Bhave, Anupama | Bhargava, Roli | Kumar, Rashmi

Article Type: Research Article

Abstract: Identification of cognitive impairment in the community is vital for need based planning of rehabilitative services. There is a need for a screening questionnaire to detect subnormal intelligence in older children in the community. The aim of this study was to validate an indigenously developed screening tool for detecting intellectual disability in the community amongst children aged 9 to 15 yr. This study was conducted in Pediatric Outpatient Department of Chhatrapati Shahuji Maharaj Medical University Lucknow, …India. A screening questionnaire was developed with learning, language, cognition and social domains. This was pretested and checked for reliability. It was then validated on 144 children aged 9 to 15 yr attending the hospital who were not acutely ill and whose parents consented for the study. A literate layperson with some training administered the screening questionnaire to parents of such children. Validation was done by a Child Psychologist using the Binet Kamat Test (Indian version of Stanford Binet test) and Seguin Form Board for intelligence quotient and Vineland Social Maturity Scale (Malin's-Indian version) for social quotient (SQ). Validation was considered positive if both intelligence quotient and SQ were less than 85 or if used alone SQ was less than 85. Sensitivity and specificity were calculated as 86.2% and 81.7% respectively. This study concludes that the Lucknow Intelligence Screening Test is a valid parent-administered intellectual disability-screening instrument for easy and effective use in the community. Show more

Keywords: Screening instrument, Lucknow intelligence screening test, intelligence screening, parent-administered screening tool, reliability, sensitivity, specificity

DOI: 10.3233/JPN-2011-0472

Citation: Journal of Pediatric Neurology, vol. 9, no. 2, pp. 215-220, 2011

Authors: Awaad, Yasser | Rizk, Tamer

Article Type: Research Article

Abstract: Dystonia is a syndrome of sustained muscular contractions of opposing muscles with various etiologies. The currently available symptomatic treatment strategies are quite effective for some of the various types of dystonia. They help in decreasing involuntary movements, correcting abnormal posture, preventing contractures, reducing pain, improving function and quality of life. Intrathecal baclofen and deep brain stimulation were proved to be fairly effective in controlling dystonia when used separately. We are reporting a …synergetic effect of intrathecal baclofen and deep brain stimulation when used simultaneously in two cases of primary generalized dystonia with excellent control of dystonia. Show more

Keywords: Dystonia, intrathecal baclofen, deep brain stimulation

DOI: 10.3233/JPN-2011-0460

Citation: Journal of Pediatric Neurology, vol. 9, no. 2, pp. 221-225, 2011

Authors: Malhotra, Reenu K. | Florez, Luisa | White, Dollett | Papasozomenos, Sozos | Covinsky, Michael | Bhattacharjee, Meena | Wang, Min

Article Type: Research Article

Abstract: Vein of Galen aneurysmal malformations (VGAMs) are rare congenital vascular, malformation. The malformation usually develops between the 6th to 11th wk of gestation. We report three autopsy cases, all prenatally diagnosed by ultrasound or fetal magnetic resonance imaging with VGAM with associated high-output cardiac failure. Prenatal fetal echocardiogram on two patients showed cardiomegaly, ventricular dilatation, pulmonary hypertension and reversed aortic flow. The cause of death in all the three patients was high-output …cardiac failure due to VGAM. The autopsy findings confirmed feeding arteries from posterior cerebral arteries to VGAM in all patients. Other significant neuropathologic findings at autopsy were severe hydrocephalus, polymicrogyria, germinal matrix hemorrhage, periventricular leukomalacia, and microcalcification. The findings support that VGAM may be due to abnormal arterial venous communication and the most common cause of death in these patients is high-output cardiac failure. The presence of other associated brain abnormalities indicates a poor prognosis. Show more

Keywords: Vein of Galen, aneurysm, malformation, cardiac failure, diagnosis, autopsy

DOI: 10.3233/JPN-2011-0475

Citation: Journal of Pediatric Neurology, vol. 9, no. 2, pp. 227-232, 2011

Authors: Walker, Jennifer C. | Brussel, Wim | Erasmus, Corrie E.

Article Type: Research Article

Abstract: We present a case of a boy with recurrent left-sided hemiparesis. Early magnetic resonance imaging showed features suggesting acute disseminated encephalomyelitis (ADEM). However, he deteriorated neurologically on high dose methylprednisolone. A follow-up cerebral magnetic resonance imaging showed an extensive infarction due to carotid artery dissection. Distinguishing features between ADEM and acute stroke in childhood are described, but at early stage, it might be difficult to distinguish ADEM from acute stroke.

Keywords: ADEM, carotid artery dissection, clinical phenomena, MRI, diffusion-weighted imaging

DOI: 10.3233/JPN-2011-0464

Citation: Journal of Pediatric Neurology, vol. 9, no. 2, pp. 233-237, 2011

Authors: Karkare, Shefali | Merchant, Sabiha | Solomon, Gail E.

Article Type: Research Article

Abstract: Epileptic negative myoclonus (ENM) is an infrequent seizure type seen in association with a variety of epileptic syndromes. It has been reported to occur in benign focal epilepsies of childhood and can be worsened by a number of antiepileptic medications. Levetiracetam widely used to treat positive myoclonus in diverse clinical situations has also been reported as a therapeutic option. We report two children with partial epilepsy that demonstrated induction of ENM in one and worsening of …ENM in the second patient on levetiracetam. Show more

Keywords: Epileptic negative myoclonus, levetiracetam, child

DOI: 10.3233/JPN-2011-0461

Citation: Journal of Pediatric Neurology, vol. 9, no. 2, pp. 239-242, 2011

Authors: Mohan, Suyash | Verma, Ashish | Sharma, Gagan | Phadke, Rajendra Vishnu

Article Type: Research Article

Abstract: We describe the case of a 4-month-old infant with evolving spontaneous thrombosis of vein of Galen malformation. It is an anomaly that occurs most often in children, with a high mortality due to the accompanying severe cardiac failure. Spontaneous thrombosis of vein of Galen malformation is rare. There are several proposed mechanisms of spontaneous thrombosis notable ones being slow flow shunts and severe obstruction of the venous outflow. The absence of blood flow at the deep …cerebral venous system due to malformation thrombosis, along with absence of signs or symptoms of intracranial hypertension led us to conservative treatment of the patient presented here. Show more

Keywords: Vein of Galen malformation, spontaneous thrombosis, transcranial ultrasound, color Doppler

DOI: 10.3233/JPN-2011-0476

Citation: Journal of Pediatric Neurology, vol. 9, no. 2, pp. 243-246, 2011

Authors: Sinha, Rahul | John, Biju | Raju, Uma | Singh, Daljit | Sodhi, Kirandeep

Article Type: Research Article

Abstract: Congenital pain insensitivity and anhidrosis is an extremely rare disorder characterized by lack of pain sensation, painless injuries of the arms, legs and oral structures, hyperthermia during hot weather because of inability to sweat, mental retardation, infection and scarring of the tongue, lips and gums, chronic infections of bones and joints, bone fractures, multiple scars, osteomyelitis and joint deformities. We report this rare case in a 2-year-old girl who presented with recurrent episodes of high-grade fever …with anhidrosis, dry fissured skin, self-mutilation, tooth loss. She also had three episodes of generalized tonic-clonic seizure during afebrile period. This case report highlights the rare association of congenital insensitivity to pain and anhidrosis with seizure disorder. Show more

Keywords: CIPA, seizure, anhidrosis

DOI: 10.3233/JPN-2011-0470

Citation: Journal of Pediatric Neurology, vol. 9, no. 2, pp. 247-249, 2011

Authors: Konanki, Ramesh | Sharma, Suvasini | Garg, Ajay | Gulati, Sheffali | Kabra, Madhulika

Article Type: Research Article

Abstract: A 1-year-old boy who presented with global developmental delay, spastic quadriparesis, seizures and squint with similar history in an elder sibling has been described. Magnetic resonance imaging of brain revealed features consistent with bilateral fronto-parietal polymicrogyria, which is a cerebral cortical malformation with characteristic clinical and neuroimaging findings.

Keywords: Polymicrogyria, bilateral fronto-parietal polymicrogyria

DOI: 10.3233/JPN-2011-0465

Citation: Journal of Pediatric Neurology, vol. 9, no. 2, pp. 251-253, 2011

Authors: Değerliyurt, Aydan | Ceylaner, Gülay | Ceylaner, Serdar

Article Type: Research Article

Abstract: The XYY chromosome abnormality is a frequently encountered numerical chromosome abnormality characterized by behavioral problems, developmental retardation and average or lower than average intelligence. A strong association with epilepsy has not been reported for the XYY anomaly unlike other chromosomal abnormalities where concurrence with seizures or epilepsy has been reported in a few studies. In this manuscript, we describe a patient with XYY karyotype, mild mental retardation and epilepsy.

Keywords: Chromosome XYY abnormality, epilepsy, mild mental retardation

DOI: 10.3233/JPN-2011-0479

Citation: Journal of Pediatric Neurology, vol. 9, no. 2, pp. 255-258, 2011

Authors: Tringler, Natalia | Flesler, Santiago | Caraballo, Roberto

Article Type: Research Article

Abstract: Opsoclonus-myoclonus syndrome (OMS) is an unusual entity of early-childhood onset characterized by variable, irregular, abnormal movements in the trunk and limbs, myoclonus, and chaotic eye movements (opsoclonus). In half of the patients, OMS presents as a paraneoplastic syndrome secondary to tumors of the neural crest (neuroblastoma), but the etiology may be multiple. OMS has been reported to occur in association with viral infections. We describe three patients with clinical features of OMS who developed paroxystic …episodes compatible with stimulus-sensitive myoclonus. The presence of these paroxysmal phenomena does not rule out OMS. We suggest that in patients with OMS the presence or absence of stimulus-sensitive myoclonus should be considered to recognize the true prevalence of these particular electroclinical events. Show more

Keywords: Myoclonus, opsoclonus, stimulus-sensitive, subcortical, syndrome

DOI: 10.3233/JPN-2011-0466

Citation: Journal of Pediatric Neurology, vol. 9, no. 2, pp. 259-263, 2011

Authors: Kumar, Raj | Kawal, Priyanka | Mehrotra, Anant | Mahapatra, Ashok K.

Article Type: Research Article

Abstract: Astrocytomas are the most common pediatric brain tumors and account for 40.9% of cerebral and 7.7% of all childhood cancers. Here we present a 16 yr male child who was diagnosed and operated for astrocytoma (grade II) of medulla oblongata; he received radiotherapy for residual tumor postoperatively. The patient developed large anaplastic astrocytoma (grade III) in splenium of corpus callosum and bilateral parieto-occipital region after 3 yr of first operation. It seems this tumor disseminated from …brainstem, though primary site remained silent because of the effect of postoperative radiation therapy. It might have disseminated through ventricular cerebrospinal fluid pathways. Little is known about low-grade astrocytoma with dissemination and their malignant transformation to high-grade glioma. The optimum therapy or combinations of therapies for such tumors remains unclear. Few aspects of dissemination and malignant transformation of low-grade astrocytoma to high-grade or appearance of fresh tumor etc are discussed in this report. Show more

Keywords: Metastasis in glioma, malignant transformation of low-grade glioma, brain stem gliomas

DOI: 10.3233/JPN-2011-0468

Citation: Journal of Pediatric Neurology, vol. 9, no. 2, pp. 265-269, 2011

Authors: Yanamandala, Rama | Pungavkar, Sona A. | Lawande, Malini A. | Patkar, Deepak P. | Merchant, Rashid

Article Type: Research Article

Abstract: Non-ketotic hyperglycinemia (NKH) is an uncommon metabolic disorder with an autosomal recessive inheritance due to abnormal glycine metabolism. We present the pre-therapy and post-therapy magnetic resonance imaging, biochemical and clinical features of a child with NKH with manifestation in the early neonatal period. The initial diagnosis of NKH was made on the biochemical evidence of elevated cerebrospinal fluid (CSF) glycine levels and CSF: plasma glycine ratio. Pre-therapy magnetic resonance imaging revealed thinned out …corpus callosum, intracranial hemorrhages, prominent ventricular system, few cystic changes in bilateral gangliocapsular region and abnormal T2 hyperintensities in the white matter. Magnetic resonance spectroscopy did not reveal glycine peak in our case. Post-therapy, there was clinical improvement and reduction in CSF glycine levels. In the follow up scan, myelination had progressed with residual leukomalacic changes and volume loss of white matter. Show more

Keywords: Non-ketotic hyperglycinemia, MRS, MRI, glycine

DOI: 10.3233/JPN-2011-0478

Citation: Journal of Pediatric Neurology, vol. 9, no. 2, pp. 271-275, 2011

Authors: Méziou, Meriam | Kamoun, Fatma | Ellouz, Emna | Hsairi, Ines | Mnif, Zeineb | Delalande, Olivier | Boudawara, Mohamed Zaher | Triki, Chahnez

Article Type: Research Article

Abstract: Rasmussen syndrome is a rare cryptogenic progressive brain disorder causing severe neurological problems. Surgery, particularly hemispherectomy in any of its varied forms, has an excellent effect in controlling seizures. We report two Tunisian patients with classically sporadic and unilateral childhood form. They are both treated successfully with vertical parasagittal hemispherotomy. This paper discusses and reviews the advances in epilepsy surgery for Rasmussen syndrome.

Keywords: Rasmussen syndrome, hemispherectomy, hemispherotomy, vertical approch

DOI: 10.3233/JPN-2011-0474

Citation: Journal of Pediatric Neurology, vol. 9, no. 2, pp. 277-280, 2011

Authors: Gupta, Priyanka | Jain, Kapil

Article Type: Research Article

Abstract: Meralgia paresthetica is relatively a common condition in adults but rare in children. Here we report the presentation, clinical features and response to conservative treatment in a 12-year-old boy affected with this condition. The etiologies and differential diagnosis of this entity are also discussed.

Keywords: Lateral cutaneous nerve of thigh, entrapment neuropathy, mononeuropathy, obesity, children

DOI: 10.3233/JPN-2011-0473

Citation: Journal of Pediatric Neurology, vol. 9, no. 2, pp. 281-283, 2011

Authors: Choudhary, Anita | Sharma, Suvasini | Arya, Ravindra | Gulati, Sheffali

Article Type: Research Article

DOI: 10.3233/JPN-2011-0462

Citation: Journal of Pediatric Neurology, vol. 9, no. 2, pp. 285-286, 2011

Authors: Beri, Sushil | Emmanuel, Joanne | Chow, Gabriel

Article Type: Research Article

DOI: 10.3233/JPN-2011-0467

Citation: Journal of Pediatric Neurology, vol. 9, no. 2, pp. 287-288, 2011

Authors: Choudhary, Anita | Sharma, Suvasini | Vashisht, Naginder | Gulati, Sheffali

Article Type: Research Article

DOI: 10.3233/JPN-2011-0463

Citation: Journal of Pediatric Neurology, vol. 9, no. 2, pp. 289-290, 2011