Journal of Pediatric Neurology - Volume 5, issue 1

Authors: Kumar, Raj | Kalra, Samir K.

Article Type: Research Article

Abstract: Craniovertebral junction is a complex transition zone between cranium and rostral spinal column. Many conditions affect and destabilize this junction with congenital atlantoaxial dislocation being the commonest. The unique anatomy of craniovertebral junction poses specific management difficulties especially in children. It requires a comprehensive understanding of the biomechanics and nuances of this complex entity to manage it efficiently. The problems in management are encountered at all stages starting from history, clinical examination, …diagnosis to surgery. An individualistic approach to each case is suggested for proper management. Show more

Keywords: Atlantoaxial dislocation, basilar invagination

Citation: Journal of Pediatric Neurology, vol. 5, no. 1, pp. 1-8, 2007

Authors: Kenney, Christopher J. | Hunter, Christine B. | Mejia, Nicte I. | Jankovic, Joseph

Article Type: Research Article

Abstract: We sought to evaluate the safety and efficacy of tetrabenazine (TBZ) in patients with moderate to severe Tourette syndrome (TS). TBZ, a monoamine-depleting drug, has been previously reported to be effective in the treatment of hyperkinetic movement disorders such as Huntington disease and tardive dyskinesia. TBZ has the distinct advantage over neuroleptics in that it does not cause tardive dyskinesia. We describe a retrospective, open-label study of 77 TS patients (75.3% male), and 14.8 ± 17.4 …years of age, treated with TBZ for an average of 2.0 years. TBZ showed a moderate to marked improvement in TS-related symptoms and functional improvement in 83.1% of patients. Adverse events included drowsiness/fatigue (36.4%), nausea (10.4%), depression (9.1%), insomnia (7.8%), and parkinsonism (6.5%). In this retrospective, open-label study, TBZ was found to be generally safe and well-tolerated in patients with TS. Show more

Keywords: Tourette syndrome, tics, tetrabenazine

Citation: Journal of Pediatric Neurology, vol. 5, no. 1, pp. 9-13, 2007

Authors: Nelson, Teresita Y. | Bost, Mandy T. | Lesser, Philip S.

Article Type: Research Article

Abstract: The objective of this study is to evaluate the effect of topiramate on tics in patients with Tourette syndrome (TS). TS is a complex neurobehavioral disorder that emerges in childhood with widely varying patterns of intermittent involuntary/semivoluntary movements expressed as motor and vocal tics. Although neuroleptics are effective anti-tic agents, side effects such as tardive dyskinesia, weight gain, and hepatotoxicity can limit their usefulness. Our use of topiramate followed reports of its effectiveness in …reducing involuntary movements in patients with essential tremor. Retrospective patient record was audited using a standardized data collection form in a general child neurology practice. Charts for topiramate-treated patients meeting diagnostic criteria for TS were reviewed. Data for tics, behavior disorders, medication history, and topiramate treatment were extracted. The Shapiro tic severity scale was used to quantify change from baseline. Of 39 children/adolescents with TS treated with topiramate, tic severity was reduced at least 50% in 23 (59%) patients, did not change in five (13%) patients and worsened in two (5%) patients. Topiramate was discontinued and not restarted due to side effects in seven (18%) patients. Topiramate appears to be useful in suppressing tics in patients with TS, but double-blind, randomized controlled studies are needed to document its efficacy. Show more

Keywords: Topiramate, Tourette syndrome, movement disorders, adolescent medicine, child psychiatry, developmental/behavioral pediatrics

Citation: Journal of Pediatric Neurology, vol. 5, no. 1, pp. 15-19, 2007

Authors: Sadoh, Wilson E. | Okunola, Olusola P. | Oviawe, Osa

Article Type: Research Article

Abstract: The human immunodeficiency virus (HIV) infection is a multi-systemic illness and often leads to acquired immunodeficiency syndrome (AIDS) in untreated cases. In children, neurologic manifestations are common. HIV encephalopathy is a neurological dysfunction arising from direct HIV infection of the brain. It ranges between 30% to 50% in untreated cases and 5% to 10% in those on Highly Active Anti-retroviral Therapy (HAART). There is a paucity of information on neurological manifestations of HIV/AIDS in children …from sub-Sahara Africa. We reviewed the records of all consecutive HIV infected non-neonatal children presenting to the pediatric department of the University of Benin Teaching Hospital, Benin City, Nigeria, between January 1999 and December 2004. Those who had neurologic manifestations were included in the study. Their biodata, the results of cerebrospinal fluid analysis, Mantoux tests and acid- and alcohol-fast bacilli (AAFB) determination were obtained. The HIV sero-status was determined using enzyme-linked immunosorbent assay test and confirmed by Western blot. In children less than 18 months, the Centers for Disease Control (CDC) criteria for surveillance case definition for AIDS were used. HIV encephalopathy was diagnosed based on CDC revised classification criteria. Of the 203 HIV seropositive children, 15 (7.4%) had neurologic manifestations. The most common manifestations were brisk deep tendon reflexes in 14 (93.3%), extensor plantar responses in 10 (66.7%) and cortical fisting in eight (53.3%). The male/female ratio was 1:2; mean age was 7.7 ± 4.8 months (range 3–18 months). As a result of cost, five (33.3%) patients had their CD4+ count determined on presentation and ranged between 30 and 300/μL. Of the 15 patients, 12 (80.0%) died, eight while in hospital and four at follow-up visit. Three patients were lost to follow-up. Five patients received HAART for a mean 11.3 ± 2.5 weeks (range 7–14 weeks). Children with HIV/AIDS present with severe neurologic manifestations and often delay in seeking appropriate medical care. It is envisioned that the promotion of the 'Prevention of Mother to Child Transmission' program coupled with early therapy with HAART, will prevent the high mortality currently associated with this condition. Show more

Keywords: Neurologic, encephalopathy, HIV, AIDS, Benin, Nigeria

Citation: Journal of Pediatric Neurology, vol. 5, no. 1, pp. 21-25, 2007

Authors: Ahmad Bhat, Mushtaq | Ali, Wajid | Mohidin, Khalid | Sultana, Mudasir

Article Type: Research Article

Abstract: In a prospective longitudinal study, children with severe breath holding spells (BHS) were enrolled to evaluate the effect of oral iron in decreasing the frequency of BHS and to document the natural history of such spells in children not treated and in non-responders to the therapy. The patients who were anemic (hemoglobin more than 2 SD below the mean for that particular age) were treated with oral iron (6 mg/kg/day) for 12 weeks. Other patients served …as controls. After 16 weeks from the initiation of treatment, the patients were evaluated for reduction in frequency of BHS. Fifty-nine children constituted the study group. The median frequency of BHS was weekly with 18% of patients having > 1 spell per day. The median age at peak frequency was 8–14 months. Anemia was present in 35/59 patients and these received oral iron therapy for 12 weeks. Twenty-four patients with no anemia constituted the control group. The treated group had a significant reduction in frequency of BHS (77% vs. (29%) compared with the control group (P<0.01). Among patients in control group and partial and non-responders in the treatment group (39/59), the last spell occurred at mean and median age of 36 months. Iron has a significant role in termination of BHS in anemic patients. Show more

Keywords: Breath holding spells, oral iron therapy

Citation: Journal of Pediatric Neurology, vol. 5, no. 1, pp. 27-32, 2007

Authors: Sobaniec, Wojciech | Sendrowski, Krzysztof | Florys, Bożena | Urban, Mirosława | Boćkowski, Leszek | Peczyńska, Jadwiga

Article Type: Research Article

Abstract: Diabetes is a common metabolic disorder, which causes multi-organ complications. Sensory dominant polyneuropathy and autonomic nervous system neuropathy are recognized as the most common clinical pictures of the nervous system disorders caused by diabetes. Pathological changes in the central nervous system in diabetic patients are relatively unknown and can be referred to as diabetic encephalopathy. The mechanisms underlying this complication remains still poorly understood. Measurable subclinical manifestations are electrophysiological changes, including the …impairment of the somatosensory evoked potentials. Aim of the study was to detect possible subclinical features of diabetic neuropathy and/or encephalopathy by investigating peripheral and central somatosensory conduction in children with newly diagnosed type I diabetes. Thirteen children with newly diagnosed type I diabetes (suffering from diabetes no longer than 24 months) were involved in the study of somatosensory evoked potentials. Latencies of potentials N9, N13, N20, P25, peripheral conduction time and central conduction time were analyzed. The control group consisted of 24 age-matched children with tension type headache. All of the analyzed evoked potentials parameters were in the normal range. No significant differences between diabetic and control children were found. Our results indicate that during the first 2 years from diabetes diagnosis in children no subclinical signs of central nervous system impairment and peripheral nervous system as well, can be detected using somatosensory evoked potentials examination. Show more

Keywords: Somatosensory evoked potentials, diabetes, diabetic encephalopathy, diabetic neuropathy, children

Citation: Journal of Pediatric Neurology, vol. 5, no. 1, pp. 33-37, 2007

Authors: Mishra, Om Prakash | Singhal, Deepak | Upadhyay, Ram Sanmukh | Prasad, Rajniti | Atri, Divya

Article Type: Research Article

Abstract: The exact etiopathogenesis of febrile seizures (FS) is unknown. The present study was conducted to evaluate the cerebrospinal fluid (CSF) zinc (Zn), magnesium (Mg), copper (Cu) and gamma-aminobutyric acid (GABA) levels in children with FS. The study subjects included 20 cases of FS, 26 patients of encephalitis and 22 children of fever with meningismus. The Zn, Mg and Cu levels in CSF and serum were analyzed by atomic absorption spectrophotometry and GABA was estimated by paper …chromatography method. The mean CSF Zn, Mg and Cu values were significantly decreased in FS in comparison to encephalitis and fever with meningismus cases (P<0.05). The mean serum Zn and Mg levels were also decreased in FS patients when compared with other two groups (P<0.05). No significant changes were observed in serum Cu levels among the three groups. There was no significant difference in the mean levels of CSF and serum GABA between FS and encephalitis, but the values were found to be significantly decreased when compared with fever with meningismus (P<0.05). Patients of FS having higher body temperature (>37.9°C) had significantly decreased levels of CSF (P<0.05) and serum GABA (P<0.01) in comparison to those having temperature of 37.2–37.8°C. Significant positive correlations were found between CSF and serum Zn, Mg and GABA values. The CSF Zn also had significant positive correlations with CSF Mg and GABA levels. These findings suggest a relationship between low levels of Zn, Mg and GABA and predisposition to FS in children. Show more

Keywords: Febrile seizures, zinc, magnesium, copper, GABA

Citation: Journal of Pediatric Neurology, vol. 5, no. 1, pp. 39-44, 2007

Authors: Basu, Sriparna | Ramchandran, Uma | Thapliyal, Anna

Article Type: Research Article

Abstract: Neurocysticercosis (NCC) is a very common pediatric neurological problem in Western Nepal. Its magnitude is not adequately known as in most situations it remains as an iceberg. To define the problem a retrospective study of the clinical profile, response to treatment and follow-up of the pediatric cases was done. Hospital records of inpatients admitted in the last 4 years (2000–2003) with the diagnosis of NCC were reviewed. Diagnosis was primarily based on clinical features, computed tomography …(CT) scan features and exclusion of other causes. All patients were treated with 28 days' course of albendazole, anti-edema drugs and anticonvulsants. Follow-up period varied from 1 to 3 years. The medical records of 124 children with NCC were evaluated. The most common age group affected was 10–12 years with the youngest patient of 11 months. Partial seizure was the most prominent presentation. The most frequent finding in CT scan was single ring-enhancing lesion with perilesional edema affecting the parietal lobe. Twenty patients were lost to follow up. Ninety-eight patients had complete clinical response and 87 of them had complete disappearance of lesion in CT scan at the end of 1 year. Recurrence of seizure was the only residual symptom found in six (4.8% patients, all of them having calcified lesions in CT scan. In areas where NCC is endemic, partial seizure in the pediatric age group is highly suggestive and should be considered as a potential indicator of the disease. Considering the lower cost and easy availability, CT scan remains the best investigation for confirmation of diagnosis in a developing country like Nepal. The disease is usually completely curable. Recurrent partial seizure is the only residual symptom, which may occur more commonly in patients with calcified lesions. Show more

Keywords: Children, Nepal, neurocysticercosis, seizure

Citation: Journal of Pediatric Neurology, vol. 5, no. 1, pp. 45-52, 2007

Authors: Tubbs, R. Shane | Loukas, Marios

Article Type: Research Article

Abstract: Masses of the cavernous sinus and Meckel's cave are uncommon. We report a patient who presented with facial pain. His exam at the most recent presentation was normal other than a diminished corneal reflex on the right side and hyperreflexia throughout. Imaging demonstrated an enhancing lesion of the left occipital lobe, left gyrus rectus, right ventricular trigone, right cavernous sinus and Meckel's cave, and diffuse intracranial meningeal enhancement. The histopathological examination and clinical course of this …patient are discussed. Show more

Keywords: Brain tumor, neurofibromatosis, phacomatosis, schwannoma

Citation: Journal of Pediatric Neurology, vol. 5, no. 1, pp. 53-56, 2007

Authors: Yüceer, Nurullah | Özer, Erdener

Article Type: Research Article

Abstract: The authors report a child with a cerebellar ganglioglioma and large cyst. An 8-year-old boy presented with headache and ataxia. Computerized tomography and magnetic resonance imaging (MRI) studies demonstrated a large cystic component associated with intense contrast enhancement of a mural nodule in the left cerebellar hemisphere. A preoperative diagnosis of cystic astrocytoma was made. The tumor was removed via a midline suboccipital approach. Pathological examination of the tumor specimen revealed a …ganglioglioma. The postoperative course was uneventful. There was no tumor recurrence on repeat MRI. At most recent follow-up, the patient was well. Although rare, cerebellar cystic gangliogliomas should be included in the differential diagnosis of posterior fossa tumors. Show more

Keywords: Cerebellum, childhood, ganglioglioma, magnetic resonance imaging, surgery

Citation: Journal of Pediatric Neurology, vol. 5, no. 1, pp. 57-60, 2007

Authors: Kumar, Raj | Giri, Pramod | Kumari, Niraj | Kalra, Samir K.

Article Type: Research Article

Abstract: Malignant rhabdoid tumors of the brain are extremely malignant, highly aggressive neoplasms found in infants and young children. These are rare and not associated with renal neoplasm as believed earlier. We report three cases of malignant rhabdoid tumors with reference to their clinicoradiological aspect and fatal outcome despite a multimodality approach of management (aggressive surgical resection and chemoradiotherapy). The first case was a two and one half-year-old girl who presented with seizures, raised intracranial …pressure and right hemiparesis. Contrast magnetic resonance imaging showed a huge heterointense tumor of the left hemisphere, which was a soft, suckable, and very vascular, infiltrative mass with areas of necrosis. Postoperatively, the girl had a difficult course with multiple metastases detected on the 45th day and death on the 52nd day post diagnosis. The second patient was admitted with large and infiltrative posterior fossa mass that required ventilatory support in postoperative period for 3 weeks until his death. The third patient was operated for a large, vascular and friable mass of the temporal lobe, which recurred in 5 weeks. He was offered chemoradiotherapy but the length of his life was only 8 months. Show more

Keywords: Malignant, rhabdoid tumor, multimodality, recurrence

Citation: Journal of Pediatric Neurology, vol. 5, no. 1, pp. 61-66, 2007

Authors: Batra, Anil | Mishra, Devendra

Article Type: Research Article

Abstract: Meningitis is a medical emergency and lumbar puncture in a child with seizures and fever is crucial for guiding further management. However, seizures themselves occasionally alter the cerebrospinal fluid (CSF), making it difficult to interpret the CSF findings. Postictal pleocytosis is one such phenomenon that confuses the interpretation of CSF analysis. In areas with limited availability of investigative facilities, meningitis cannot be definitively ruled out and a diagnosis of postictal pleocytosis remains just a …clinical suspicion. Show more

Keywords: Cerebrospinal fluid analysis, lumbar puncture, meningitis

Citation: Journal of Pediatric Neurology, vol. 5, no. 1, pp. 67-69, 2007

Authors: Per, Hüseyin | Kumandaş, Sefer | Gümüş, Hakan | Konuşkan, Bahadır | Öztürk, Mustafa Kürşad

Article Type: Research Article

Abstract: Salmonella typhi is a Gram-negative, motile aerobic bacillus with non-encapsulated, non-sporulating rods from the Enterobacteriaceae family. Salmonella can localize in any organ or tissue. Focal intracranial infections caused by Salmonella species are uncommon manifestations of salmonellosis in developed countries and are often associated with diarrheal diseases and malnutrition. Salmonella meningitis is a serious disease that could lead to death and damage in infants. We report a case of a 4-month-old infant with …meningitis and subdural empyema caused by Salmonella typhi. Management included treatment with neurosurgical drainage and a prolonged course of antibiotics. Show more

Keywords: Salmonella, meningitis, subdural empyema

Citation: Journal of Pediatric Neurology, vol. 5, no. 1, pp. 71-74, 2007

Authors: Al-Mosawi, Aamir Jalal

Article Type: Research Article

Abstract: The oculocerebrorenal syndrome (OCRS) is a rare hereditary disorder characterized by ocular and cerebral defects, hypotonia, hyporeflexia and renal dysfunction. The aim of this study is to report the phenotype OCRS in four Iraqi children. From 1994 to 2005, four patients (two boys and two girls) with OCRS phenotype were observed at Al-Kadhimiyia University Hospital. All described cases had congenital cataract(s), hypotonia and hyporeflexia and renal dysfunction. It was possible to study and follow three of …them for several months, while the fourth case was observed only twice and then lost to follow up. Ocular manifestations in the three cases were in the form of cataract and glaucoma; one boy had immature cataracts not affecting the vision, and bilateral congenital chorioretinal hypoplasia. Two boys had nystagmus during infancy, which was undetectable at time of referral of one case. All the patients had hypotonia and hyporeflexia, which developed after 3 years in one girl. Three patients had global developmental delay; however, intelligence and language was well developed in one girl. All the children had end stage renal failure with evidence of glomerular defect. None had any renal tubular dysfunction or Fanconi syndrome. Uremia was the cause of death in the three cases followed up during their first decade. Congenital chorioretinal hypoplasia, which had never been described in OCRS, was present in one among our cases. Show more

Keywords: Oculocerebrorenal, lowe syndrome, Iraqi children

Citation: Journal of Pediatric Neurology, vol. 5, no. 1, pp. 75-78, 2007

Authors: Kumar, Raj

Article Type: Other

Citation: Journal of Pediatric Neurology, vol. 5, no. 1, pp. 79-81, 2007

Article Type: Other

Citation: Journal of Pediatric Neurology, vol. 5, no. 1, pp. 83-85, 2007