Journal of Pediatric Neurology - Volume 5, issue 4

Authors: Gordon, Neil S.

Article Type: Research Article

Abstract: The symptoms and signs of Sandifer's syndrome are described, especially the abnormal movements, which can wrongly be diagnosed as some form of dystonia. They affect head, neck and chest, and are often accompanied by abnormal postures. The syndrome, may or may not, be associated with hiatus hernia, and gastro-intestinal symptoms are common. Neurologically impaired children are especially at risk, and can present particular difficulties in diagnosis. Barium swallow can confirm the diagnosis, and also help to …establish the cause. The possible reasons for the syndrome are considered, relief of abdominal discomfort being most plausible. Treatment can result in dramatic improvement, although sometimes surgery is needed. Show more

Keywords: Sandifer's syndrome, symptoms, investigations, treatment

Citation: Journal of Pediatric Neurology, vol. 5, no. 4, pp. 275-278, 2007

Authors: Lúquez, Carolina | Bianco, María I. | Sagua, María D. | Barzola, Cristian P. | de Jong, Laura I.T. | Degarbo, Stella M. | Fernández, Rafael A.

Article Type: Research Article

Abstract: Infant botulism is the most common form of human botulism in some countries including Argentina. However, its transmission has not been completely elucidated. In Argentina, 366 laboratory-confirmed infant botulism cases were reported between March 1982 and December 2005. The average annual incidence was 2.2 per 100,000 live births. All 108 cases diagnosed in our laboratory had botulinum toxin producing organisms in their feces, 96.3% also had botulinum toxin in feces, and 69% had detectable botulinum toxin …in their serum. Biochemical tests showed uniformity among the botulinum toxin-producing clostridia isolated from infant botulism cases and soil samples. A positive relationship between presence of botulinum spores in soil and illness incidence was observed in the Northeast and West regions but not in the Central, South and Northwest regions. In the Northwest and Central regions, there was a relatively high occurrence of botulinum spores in the soil but low incidence of the disease. Type A botulinum toxin was detected in all infant botulism cases and also it was the toxin type most prevalent in the soil. Despite the presence of B and F types in the soil, there were no reports of infant botulism cases by these types in Argentina between 1982 and 2005. Show more

Keywords: Botulinum toxin, soil, infant botulism, C. botulinum

Citation: Journal of Pediatric Neurology, vol. 5, no. 4, pp. 279-286, 2007

Authors: Fallah, Razieh | Gofrani, Mohammad

Article Type: Research Article

Abstract: Twenty children including nine boys and eleven girls with a mean age of 3.8 years (range: 0.1–12 years) and admitted with a diagnosis of refractory convulsive status epilepticus constituted the study group. Ten children among them were treated with intravenous lidocaine (group 1) and other ten received midazolam infusion (group 2) for control of seizures. Variables such as age, sex, neuroimaging, electroencephalographic findings, concomitant fever, neurodevelopmental delay, complications, seizure control and length of stay …(days) in the intensive care unit were carefully recorded. Two out of ten (20%) patients in group 1 responded while as five out of ten (50%) patients in group 2 responded (P=0.1). No paraclinical side effects occurred in any patients in both the groups. Two patients needed intubation in group 1 as against seven in group 2 (P=0.03). Mean length of stay in the intensive care unit was 4.6 days in group 1 and 9.2 days in group 2 (P=0.04). No significant differences were seen with regard to age, sex, electroencephalographic findings, neuroimaging abnormalities or neurodevelopmental delay between the two groups. In conclusion, lidocaine can be used in refractory status epilepticus treatment especially when respiratory care and intubation facilities are not present. Show more

Keywords: Status epilepticus, intravenous lidocaine, midazolam infusion

Citation: Journal of Pediatric Neurology, vol. 5, no. 4, pp. 287-290, 2007

Authors: Ahmed, Riaz

Article Type: Research Article

Abstract: Twenty-eight children with tonic clonic seizures and with mean age of 4.6 years received buccal midazolam in a dose of 0.2 mg/kg for control of seizures. Thirteen children were known epilepsy and were on regular antiepileptic treatment, eight had febrile seizures, four had idiopathic first onset seizures, two had meningitis and one hypocalcemia. The seizures were controlled in 64% midazolam and seven patients needed a second dose. These seven patients also received phenytoin/phenobarbitone infusion following the …2nd dose of buccal midazolam. The dose of buccal midazolam was not increased to more than 0.2 mg/kg in any of the patients. Low dose midazolam given by the buccal route is often sufficient to control seizures effectively. Show more

Keywords: Buccal midazolam, seizures, child

Citation: Journal of Pediatric Neurology, vol. 5, no. 4, pp. 291-293, 2007

Authors: Al-Futaisi, Amna | Al-Busaidi, Mujahid | Al-Abdwani, Raghad | Javad, Hashim | Koul, Roshan

Article Type: Research Article

Abstract: Acute disseminated encephalomyelitis is typically a monophasic multifocal autoimmune demyelinating disease of the central nervous system, which generally follows a febrile infection or a vaccination. It presents with a variety of clinical features reflecting the multifocal nature of the disease. The study was carried out at Sultan Qaboos University Hospital Oman, which is a tertiary care hospital, to see the pattern in this country. A review of inpatients files' records was done with the diagnosis of …acute disseminated encephalomyelitis. Inclusion criteria were acute onset, a preceding infection or vaccination, hyperattenuated lesions seen on T2 weighted magnetic resonance imaging, or computerized tomography findings consistent with white matter changes and rapid response to corticosteroids upon therapy. Twenty-three children met the clinical and imaging criteria. Fifteen (65%) were males and eight (35%) were females giving a ratio of 2:1. Their ages ranged from 9 months to 11 years with median age of 5 years. Eleven (48%) patients had a history of an acute respiratory tract infection two to 20 days before presentation. Two (9%) patients had chickenpox, and another two (9%) patients had fever with a rash. One (4%) patient had pneumonia with positive IgM for Mycoplasma pneumonia. None had received a vaccine prior to presentation. Pyramidal signs were seen in the majority of children (83%). Most children had involvement of the subcortical white matter (74%) followed by basal ganglia (24%) and thalamic lesions (24%). Corticosteroids were administered to 21 (91%) children. Eight (35%) of these children had received intravenous immunoglobulin after failure to respond adequately to steroid therapy. Full recovery was seen in 16 (70%) of the patients. The average period of recovery was around 3 weeks. Seven (30%) patients had persistent neurological deficits upon discharge as well as on follow up two years later. The clinical features, outcome and response to treatment were similar to other reported studies. This is the first study to show a clear-cut male predilection of 2:1. Show more

Keywords: ADEM, MRI, corticosteroid, Oman

Citation: Journal of Pediatric Neurology, vol. 5, no. 4, pp. 295-299, 2007

Authors: Laroia, Nirupama | Guillet, Ronnie | McBride, Margaret

Article Type: Research Article

Abstract: Felbamate, a non-selective anticonvulsant thought to act via blocking the N-methyl-D-aspartate receptor, has been shown in animal studies not only to block seizures, but also to be neuroprotective. Excessive discharge of the excitatory pathways, involving primarily the glutamate receptor system, is important in the cascade of neuronal injury in hypoxic ischemic encephalopathy (HIE). Because of renewed interest in adjunctive therapy in addition to hypothermia to minimize hypoxic ischemic injury in neonates, we …report the absorption and time to peak level of felbamate preliminary to testing its neuroprotective effects in this population. A single dose of either 45 mg/kg or 200 mg/kg of felbamate was given via nasogastric tube to four infants with severe HIE. We measured serum felbamate levels at 2, 4, 8, 12 and 24 hours after administration of the drug. Oral absorption of felbamate is slow. Peak levels of 40–64 μg/mL were achieved at 12 to 24 hours in the two infants who received 200 mg/kg dose. In conclusion, oral felbamate is unsuitable for infants with HIE as oral doses are unlikely to reach adequate neuroprotective levels in a timely fashion. Show more

Keywords: Neuroprotection, HIE, newborn, felbamate

Citation: Journal of Pediatric Neurology, vol. 5, no. 4, pp. 301-304, 2007

Authors: Badran, Eman F. | Masri, Amira T. | Hamamy, Hanan | Al-Qudah, Abdulkarim A.

Article Type: Research Article

Abstract: A prospective longitudinal study was conducted on all neonates admitted with a diagnosis of neonatal seizures in the neonatal intensive care unit of Jordan University Hospital Amman from April 2003 to March 2005 to examine the cause and clinical profile of seizures in a community, which culturally favors consanguineous marriages. Of 35 neonates studied, inborn errors of metabolism accounted for 28.6%, hypoxic ischemic encephalopathy for 17.1% and cerebral malformations for 14.3%. The consanguinity rate …among parents of affected neonates was 54%, and 22.9% of these probands had a similarly affected sibling. Compared with 78 matched controls, consanguinity proved to be a risk factor for neonatal seizures (P=0.007, odds ratios =3.02; 95% confidence intervals 1.22 to 7.51). The hospital-based incidence of seizures was 5.6 per 1000 live births. Poor outcome in the form of mortality or morbidity was present in 67.8% of the cases. Early-onset seizures and status epilepticus accounted in 40% and 22.9% of cases respectively, with subtle seizures being the commonest type. Based on our results, inborn errors of metabolism and cerebral malformations make a significant contribution to the etiology of clinical neonatal seizures. This is accounted for by the consanguineous population being at risk for a number of neurogenetic disorders leading to convulsions on one hand and by improved methods of diagnosis of neonatal seizures on the other. In future, genetic counseling may help to minimize the contribution of underlying genetic factors to neonatal seizures in our community. Show more

Keywords: Neonates, causes, seizures, consanguinity, Jordan

Citation: Journal of Pediatric Neurology, vol. 5, no. 4, pp. 305-309, 2007

Authors: AL-Zwaini, Essam J.

Article Type: Research Article

Abstract: Prospective study of 169 children with febrile seizures (FS) was carried out from 1st February 2002 to 30th of January 2003. The proportion of patients with FS among all admitted patients was 7.6%. The mean age of patients with FS was 25.8 months, and the peak age of our patients was 18–19 months. Male to female ratio was 1.3:1. The mean age of the 1st attacks was 23.5 months, and that for recurrent attacks was 29.8 months. …The mean temperature for the 1st attack was 39.7°C and that for recurrent attacks was 38.2°C. There was a history of prematurely in 13%, a family history of FS in 35% and epilepsy in 17%. Complex FS were seen in 27% and febrile status epilepticus in 3%. Upper and lower respiratory tract infection was the cause of fever in 67% of patients and the majority of them were admitted in the cold months of year. Lumbar puncture was done in 37% of patients. The commonest anticonvulsant used to terminate the seizures was intravenous diazepam in 78.7% followed by rectal diazepam in 8.9%. Intermittent diazepam (45%) was the most widely used anticonvulsant for prophylaxis, followed by phenobarbital (23%). Show more

Keywords: Febrile seizures, criteria, epidemiology, clinical features

Citation: Journal of Pediatric Neurology, vol. 5, no. 4, pp. 311-315, 2007

Authors: Singhi, Pratibha | Mahajan, Vikas | Hiremath, Gurumurthy M.

Article Type: Research Article

Abstract: Joubert syndrome is an autosomal recessive complex malformation of brain particularly involving the cerebellum and brain stem. Agenesis or dysgenesis of cerebellar vermis and the presence of "molar tooth sign" on axial magnetic resonance imaging are characteristically seen. The main clinical features include hypotonia, developmental delay, abnormal respiratory pattern of alternating episodic tachypnea, hyperpnea and apnea and oculomotor apraxia. We present clinical characteristics of five cases of Joubert syndrome with a review …of literature. Show more

Keywords: Joubert syndrome, child, India

Citation: Journal of Pediatric Neurology, vol. 5, no. 4, pp. 317-321, 2007

Authors: Karremann, Michael | Sojka, Steffen | Huehn, Regina | Preiss, Uwe | Schmidt, Frank

Article Type: Research Article

Abstract: We report on a seventeen-year-old boy presenting with postural headache caused by intracranial hypotension following surgical treatment of juvenile scoliosis. The clinical presentation, diagnostic findings and treatment are described and a review of the relevant literature is given. It is concluded that physicians should be aware of intracranial hypotension syndrome as a possible, though rare, complication after spine surgery.

Keywords: Adolescence, caffeine, intracranial hypotension syndrome, postural headache, spine surgery

Citation: Journal of Pediatric Neurology, vol. 5, no. 4, pp. 323-325, 2007

Authors: Hussain, Nahin | Chieng, Siik Kwong | Rickett, Andrew | Gosalakkal, Jayprakash

Article Type: Research Article

Abstract: Cerebral sinovenous thrombosis (CSVT) is a rare and under-diagnosed complication of childhood nephrotic syndrome. With the advent of magnetic resonance imaging and magnetic resonance venography scans, CSVT cases are now being diagnosed more frequently. We report case of a three-year-old male child, known case of steroid responsive nephrotic syndrome, who presented with a 3-week history of headache and sudden onset intermittent convergent squint. He was diagnosed to have CSVT on magnetic resonance imaging and magnetic resonance …venography of the brain. Devastating cerebral infarction was probably prevented with early institution of low molecular weight heparin. Nevertheless, randomized placebo-controlled studies are necessary for assessing its efficacy and absence of risk in children. Show more

Keywords: Venous thrombosis, cerebral, nephrotic syndrome

Citation: Journal of Pediatric Neurology, vol. 5, no. 4, pp. 327-330, 2007

Authors: Yilmaz, Kutluhan | Yilmaz, Mustafa | Sirikci, Akif

Article Type: Research Article

Abstract: Positron emission tomography (PET), as a functional neuroimaging technique, provides biochemical and molecular information about brain and tumor tissues. We present the findings of FDG (2-deoxy-2[18F]-fluoro-D-glucose)-PET and magnetic resonance imaging in a 7-year-girl with tuberous sclerosis. Cranial magnetic resonance imaging revealed multiple cortical tubers, subependymal nodules and a subependymal giant cell astrocytoma. On FDG-PET images, the regions of cortical tubers were hypometabolic and the tumor also demonstrated hypometabolism implying a low-grade tumor. …PET has been studied in tuberous sclerosis for many purposes including epilepsy and mental retardation. The present report suggests that FDG-PET could be useful in patients with tuberous sclerosis and intraventricular tumor as well. Show more

Keywords: Tuberous sclerosis, positron emission tomography

Citation: Journal of Pediatric Neurology, vol. 5, no. 4, pp. 331-334, 2007

Authors: Kolthof, Henk Jan | Tjeenk-Kalff, Ariane C.

Article Type: Research Article

Abstract: Cerebral disorders are nowadays seen as a feature of nephropathic cystinosis. Describing specific cognitive profiles in children with cystinosis may help to elucidate the cause of the impairments seen. A 9-year-old girl with nephropathic cystinosis had a progressive decline in mental functioning, in particular severe memory problems. None of the cases reviewed described memory problems at such a young age, so it is difficult to ascertain the possible cause of the impairment. Although few cases with …dementia are reported, there is apparently no direct association between cystinosis and memory problems. Neither has the medication cysteamine been associated with memory loss in humans. Show more

Keywords: Cystinosis, memory problems, complications

Citation: Journal of Pediatric Neurology, vol. 5, no. 4, pp. 335-341, 2007

Authors: Arantes, Mavilde | Perdigao, Sandra | Pereira, Jorge Resende | Costa, Manuela

Article Type: Research Article

Abstract: Schizencephaly is an uncommon disorder of cell migration characterized by communication clefts between ventricular system and subarachnoid space. The clefts may be unilateral or bilateral and may be closed or separated. Patients with bilateral clefts typically present with severe mental deficits, motor anomalies and seizures. We report a 16-year-old girl whose cranial magnetic resonance imaging revealed bilateral open-lip schizencephaly, but with an atypical clinical course. She had minimally recognizable neurological deficits and …epilepsy for last one year. Such mild clinical phenotypes were not proportional to the magnetic resonance imaging of the cerebral malformation. Show more

Keywords: Migration disorder, schizencephaly, magnetic resonance image

Citation: Journal of Pediatric Neurology, vol. 5, no. 4, pp. 343-345, 2007

Authors: Singhi, Pratibha | Mahajan, Vikas

Article Type: Research Article

Abstract: Paroxysmal dyskinesias are neurologic conditions characterized by sudden episodes of abnormal involuntary movements. Paroxysmal exertion induced dyskinesia is a rare condition characterized by abnormal involuntary movements on exercise, which get relieved with rest. In general, these movements are poorly responsive to medical treatment. We report one family in which all three male members are affected and had good response to phenytoin.

Keywords: Dyskinesia, paroxysmal, exercise, phenytoin

Citation: Journal of Pediatric Neurology, vol. 5, no. 4, pp. 347-350, 2007

Authors: Wani, Abrar Ahad | Babu, Makhan Lal | Kirmani, Altaf | Ramzan, Altaf Umer | Bhat, Abdul Rashid | Alam, Syed Shafiq | Ahmad, Altaf | Baba, Khalil Mohammad

Article Type: Research Article

Abstract: Giant cell tumor of the bone accounts for 4–5% of all primary bone tumors. Spine is the fourth common site for their occurrence and axis is a relatively rare site. A case of giant cell tumor of axis in a 17-year-old male is hereby reported. The patient underwent transoral decompression and posterior fusion. Postoperatively patient had no neurological deficit and biopsy revealed the tumor as giant cell tumor.

Keywords: Giant cell tumor, axis

Citation: Journal of Pediatric Neurology, vol. 5, no. 4, pp. 351-354, 2007

Authors: Yoshikawa, Hideto

Article Type: Other

Citation: Journal of Pediatric Neurology, vol. 5, no. 4, pp. 355-356, 2007

Authors: Kumar, Raj | Das, Nitu Kumar | Mahapatra, Ashok Kumar

Article Type: Other

Citation: Journal of Pediatric Neurology, vol. 5, no. 4, pp. 357-359, 2007