Journal of Pediatric Neurology - Volume 4, issue 4

Authors: Ray, Pulak | Jallo, George I. | Kim, Richard Y. H. | Kim, Bong-Soo | Wilson, Sean | Kothbauer, Karl | Abbott, Rick

Article Type: Research Article

Abstract: Endoscopic third ventriculostomy (ETV) is a common alternative method for the management of hydrocephalus in selected patients. A retrospective chart review was conducted to review our experience with ETV for patients with a variety of causes for hydrocephalus. A total of 67 children with median age of 10.8 years (range=8 weeks to 21 years) underwent 76 ETVs from July 1992 to June 2003. Two procedures were abandoned due to anatomical distortion. The medical records, operative reports, …and imaging studies, were retrospectively reviewed with regard to outcome, complications and patency rate. Treatment failure was defined as the need to shunt the patient within 4 weeks of performing the ETV. The overall success rate was 83% with a mean follow-up 40.8 months. Nine patients underwent 11 repeat ETVs at an average interval period of 24 months with a patency rate of 90% following the second procedure. Ten of eleven patients who underwent re-ETV were ultimately shunt-independent. The highest success rates were achieved for obstructive hydrocephalus from midbrain/tectal tumor (100%, n =4), pineal tumor (100%, n=3), intraventricular tumor or cyst (100%, n=2), and post-infectious hydrocephalus (100%, n=1). Lower patency rates were noted in patients with Chiari malformation (0%, n=1) and posterior fossa tumors (63%, n=8). Due to the efficacy of ETV, it should be considered as the primary procedure, rather than ventricular peritoneal shunts, in carefully selected children. The reliability rate is dependent upon the age and etiology of hydrocephalus. Show more

Keywords: Endoscope, third ventriculostomy, hydrocephalus, outcome

Citation: Journal of Pediatric Neurology, vol. 4, no. 4, pp. 221-232, 2006

Authors: Waring, Rosemary | Naidu, Sakkubai | Skjeldal, Ola | Klovrza, Liba | Reichelt, Karl L.

Article Type: Research Article

Abstract: Although mutations in the MECP2 gene are associated with many cases of Rett syndrome (RS), the phenotype remains unexplained. At least in the early stages, RS and autism have common features; urinary analysis of autistic children has shown an abnormal profile of excretion of sulphur-containing anions, with high levels of sulphite, sulphate and thiosulphate and low values for thiocyanate ions. These parameters were therefore studied in an RS population. Levels of urinary peptides, protein, cysteine, free …sulphate, conjugated sulphate, sulphite, thiosulphate and thiocyanate were determined in female RS patients (n=21) and in controls n=21), using standard methods. The urinary volumes were adjusted to standard creatinine levels (200 nmol/mL). Children with RS had higher urinary peptides and protein excretion. Free sulphate values and total sulphate excretion were higher in RS (P<0.001) than in controls. Cysteine, sulphite and thiosulphate excretion were elevated (P<0.001) while thiocyanate excretion was greatly reduced (P<0.001). These results are consistent with reduced expression or activity of the enzyme rhodanese (thiosulphate cyanide sulphurtransferase). Other mutation(s) in genes involved in metabolism of sulphur anions may also contribute to the RS phenotype. Show more

Keywords: Rett syndrome, sulphur anions, metabolism, rhodanese

Citation: Journal of Pediatric Neurology, vol. 4, no. 4, pp. 233-237, 2006

Authors: Bener, Abdulbari | Al-Suweidi, Ebtesam E.K. | Bessisso, Mohammed | Al-Gazali, Lihadh I. | Al-Khider, Ali

Article Type: Research Article

Abstract: The aim of this study was to determine genetic and environmental risk factors associated with febrile convulsions among children in the United Arab Emirates. This study was based on matched case-control studies. Subjects were collected from the Al-Ain Medical Health District and the Tawam and Al-Ain Teaching Hospitals of Faculty of Medicine and Health Sciences. Seventy patients with febrile seizure were identified and were matched to 70 control febrile patients without seizure with the same age …range (3–36 months), who attended the same hospitals during the same period of time. Mothers of studied patients and controls were interviewed by telephone. If the mother was not available, the father was interviewed. Potential risk factors were investigated. The mean and standard deviation for age were 17.0 ± 7.9 months for studied patients and 18.0 ± 9.1 months for controls. The highest frequency of cases with febrile seizure was in children aged 1–2 years. Forty six cases (65.7% had a febrile seizure for the first time and 24 cases (34.3%) had it before (recurrent febrile seizure). The Mantel Haenszel test revealed that sex, respiratory infection, positive family history, and birth weight were significant predictors for febrile seizure. It is recommended to establish health educational programs for mothers regarding febrile seizures and how to deal with their children during such attacks. Show more

Keywords: Genetics, environmental, risk factor, febrile seizures

Citation: Journal of Pediatric Neurology, vol. 4, no. 4, pp. 239-243, 2006

Authors: Masri, Amira T.

Article Type: Research Article

Abstract: We aimed to determine the incidence, types of neural tube defects (NTDs), seasonal variation, complications, and follow up of a sample of children managed at Jordan University Hospital. This retrospective study covered a 10-year period (1993–2002) and included all patients with NTDs managed at Jordan University Hospital. The medical files were reviewed and data were analyzed using Statistical Package of Social Science. One hundred and nineteen patients had NTDs, 31 cases (26%) were born at our …hospital; with incidence estimated to be 1.1/1000 among 28,301 live births during that period. There was no seasonal variation with male to female ratio (1.2:1). The most common type of NTD was myelomeningocele, followed by anencephaly. Sixty patients out of 91 (66%) with myelomeningocele were admitted to our neonatal intensive care unit, seven (12%) died while in the unit. Lower limb paralysis was reported in 77 patients (84%), neurogenic bladder in 48 (52.7%), club feet in nine cases (10%). Urine cultures were positive for bacterial growth in 42 patients (60%). Thirty six patients (42.8%) from those who had their follow up in our hospital were readmitted at least once; the most common cause of readmission was shunt infection. This study shows a high incidence of NTDs in Jordan and emphasizes the need for multidisciplinary clinics to follow and support these children and their families. Show more

Keywords: Anencephaly, myelomeningocele, neural tube defects, Jordan

Citation: Journal of Pediatric Neurology, vol. 4, no. 4, pp. 245-249, 2006

Authors: Ajlouni, Saleh F. Al | Aqrabawi, Manar | Oweis, Nabieh | Daoud, Azhar S.

Article Type: Research Article

Abstract: This study was conducted to evaluate the clinical profile and predisposing factors of cerebral palsy (CP) in Jordanian children. Two hundred Jordanian children born between 1990 and 2005 with CP were reviewed in three teaching hospitals to study prospectively and retrospectively their clinical profile, possible etiological factors, and associated problems. A data collection sheet was developed to collect information from the children's parents, or occasionally from the chart regarding the demographic characteristics, …detailed history, findings of the physical, developmental and neurological examination. Spastic CP was the predominant type (64.5%) with a quadriplegic CP being the most common (36%). Dyskinetic CP was present in 10.5%, while ataxic in 18%. In congenital CP, birth asphyxia constituted a significant possible cause (33.5%). Epilepsy is one of the most common associated problems among CP children constituting 56%. The results of our magnetic resonance imaging findings showed that only 15.5% of the children were normal. This was the first study which detailed the clinical spectrum of CP in Jordan. We concluded that the clinical spectrum of CP in our developing countries may differ from that of the developed countries. Show more

Keywords: Cerebral palsy, clinical spectrum, Jordan

Citation: Journal of Pediatric Neurology, vol. 4, no. 4, pp. 251-255, 2006

Authors: Abdulla, Jamila K. | Al-Tawari, Asma A. | Cindro-Heberle, Lada | Gopinath, Musuwadi S. | Neubauer, David

Article Type: Research Article

Abstract: Neurocysticercosis is the most common parasitic central nervous system disease and the major cause of epilepsy in non-Muslim developing countries. Cases also occur in non-endemic regions reflecting pattern of immigration from highly endemic areas. Intending to increase awareness of cysticercosis even in countries where the religion doesn't allow pork intake we report seven cases of autochtonous infection diagnosed in Kuwait in the period from 2000 to 2004. Six children presented with seizures, three of them also …had headache and vomiting. None was given cysticidal treatment however all showed complete resolution of ring enhancing lesions on neuroimaging and a favorable outcome. In a family with two affected members, another asymptomatic sibling showed a ring lesion that resolved spontaneously. Show more

Keywords: Neurocysticercosis, ring enhancing lesion, non-endemic Muslim countries, partial seizures, EITB assay

Citation: Journal of Pediatric Neurology, vol. 4, no. 4, pp. 257-260, 2006

Authors: Kato, Zenichiro | Saito, Kayoko | Isogai, Koji | Kondo, Naomi

Article Type: Research Article

Abstract: Fukuyama-type congenital muscular dystrophy (FCMD) is an autosomal recessive disorder characterized by congenital muscular dystrophy and associated with neuropathological anomalies such as polymicrogyria and pachygria. Cerebral abnormalities in FCMD have been well documented by neuropathological examinations and cranial imaging studies. However, the pathologic mechanism of white matter lesions remains controversial. In the present study, magnetic resonance imaging (MRI) of a 7-month-old boy with FCMD showed the previously reviewed characteristic morphological features such …as thick cortices with shallow sulci corresponding to polymicrogyria involving the frontal lobe. MRI also showed markedly prolonged T1 and T2 signals in the white matter, while diffusion-weighted images showed no abnormalities. The results of magnetic resonance spectroscopy showed an increase in choline and N-acetylaspartate resonances but normal myo-inositol resonance. The simulation of these findings with those of merosin-negative congenital muscular dystrophy should suggest a further study with larger population using a combination of different imaging techniques. Show more

Keywords: Fukuyama-type congenital muscular dystrophy, magnetic resonance imaging, spectroscopy

Citation: Journal of Pediatric Neurology, vol. 4, no. 4, pp. 261-264, 2006

Authors: Zage, Peter E. | Chopra, Monisha | McRae, Wes | Goldman, Stewart

Article Type: Research Article

Abstract: Retinoblastoma is the most common intraocular tumor of childhood and can be either unilateral or bilateral and either hereditary or non-hereditary. Retinoblastoma is associated with mutations of the Rb gene on chromosome 13q, and chromosome 13q deletions and translocations in association with retinoblastoma have been described. Retinoblastoma can be associated with developmental delay, growth retardation, and congenital malformations. The authors describe a child with congenital hypotonia who developed retinoblastoma and was …found to have a t(X;13) chromosomal translocation. The occurrence of hypotonia in association with retinoblastoma suggests an etiological link between the two disorders. Show more

Keywords: Retinoblastoma, congenital hypotonia, t(X;13) translocation

Citation: Journal of Pediatric Neurology, vol. 4, no. 4, pp. 265-270, 2006

Authors: Otero, Soraya

Article Type: Research Article

Abstract: This paper describes a 14-year-old boy presenting both extrapyramidal syndrome and a depressive episode after the initiation of flunarizine treatment for migraine. A detailed case history was obtained using traditional assessment and a semi-structured interview. A Medline search was done for information on adverse effects of flunarizine and co-morbidity between migraine and anxiety-depression symptoms. Some patients could have a predisposition to anxiety-depression as a global response. The intake of certain drugs, such …as flunarizine, affecting central receptors, could precipitate the onset of depressive symptoms. Show more

Keywords: Flunarizine, adverse effects, depression, extrapyramidal syndrome, children, adolescents

Citation: Journal of Pediatric Neurology, vol. 4, no. 4, pp. 271-273, 2006

Authors: Ray, Munni | Kothur, Kavitha | Khandelwal, Niranjan

Article Type: Research Article

Abstract: Acute bilateral striatal necrosis is considered to be a rare heterogeneous entity, in view of the variability of its clinical presentation. We report a 4-year-old girl presented with typical clinical features of measles associated with acute bilateral striatal necrosis. The clinical symptoms were an abrupt onset of seizures, hemiplegia, expressive aphasia and extrapyramidal signs on the seventh day of illness. Serum was positive for measles IgG and IgM. Cranial magnetic resonance imaging showed hyperintensities on T2 …weighted images affecting the putamen and caudate nuclei bilaterally. Neurologic recovery occurred within six weeks following therapy with corticosteroids. Repeated magnetic resonance imaging in follow up showed significant resolution of the lesions. Show more

Keywords: Infantile bilateral striatal necrosis, measles

Citation: Journal of Pediatric Neurology, vol. 4, no. 4, pp. 275-277, 2006