Journal of Pediatric Neurology - Volume 3, issue 1
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Journal of Pediatric Neurology is an English multidisciplinary peer-reviewed medical journal publishing articles in the fields of child neurology, pediatric neurosurgery, pediatric neuroradiology, child psychiatry and pediatric neuroscience.
Journal of Pediatric Neurology encourages submissions from authors throughout the world. The following articles will be considered for publication: editorials, original and review articles, rapid communications, case reports, letters to the editor and book reviews. The aim of the journal is to share and disseminate knowledge between all disciplines that work in the field of pediatric neurology.
Abstract: Preventable risk factors for sudden infant death syndrome include the infant's environment and response characteristics. Various infant and environmental factors thus modify the vital cardiocirculatory, respiratory and arousal controls in healthy infants. Similar changes in cardiorespiratory and autoresuscitative responses have been found in the analysis of sleep recordings of victims of sudden infant death syndrome. It is not known why some infants die, while others show similar changes but survive in the first year of life.…The death could be due to the degree of the initial immature controls, to the severity of the additional challenge, or to a combined effect of inadequate autoresuscitative mechanisms and the cumulative influence of infant and/or environmental stressors.
Keywords: infant, sleep, sudden infant death syndrome, apnea
Abstract: Enuresis is a common problem affecting a large child population. Though not psychogenic in nature, it is often associated with psychosocial problems. Structural abnormalities as a cause are rare. Common causative factors include developmental delay, genetic factors, abnormalities of sleep-wake cycle and of anti-diuretic hormone secretion. Detailed history, physical examination and simple routine tests are usually sufficient to reach the diagnosis. Both pharmacological as well as non-pharmacological treatments alone or in combination…have been used with varying success rates.
Abstract: The aim of this study is to find the efficacy and safety of intermittent clobazam in febrile seizures (FS). The study was a double blind placebo control trial. Sixty patients of age group 6 months to five years presenting with febrile seizures were finally randomized to clobazam (group A; n = 30; male/female: 14:16) and placebo (group B; n = 30; male/female: 17:13) groups after exclusion of mental retardation, congenital malformations, intracranial infections, renal or hepatic…dysfunction or history of intake of antiepileptic drugs and afebrile seizures. The patients in group A were given intermittent prophylactic clobazam @0.75 mg/kg body weight twice daily and group B were given placebo, during the course of fever. Antipyretic (paracetamol) and cold sponging was advised to the guardian, if the fever exceeded 38°C. The patients were assessed primarily on the basis of recurrence of seizure per febrile episodes, pattern of seizures and safety from the adverse effects reported during the course of study and from the investigation done on the patient. The two groups had comparable clinical profile. Mean ages of patients in group A and B were 25.5 ± 13.38 and 18.43 ± 13.95 months, respectively. Thirteen cases in group A and 12 cases in group B had abnormal electroencephalogram at baseline. Five patients in group A and eight cases in group B had a family history of FS. Twenty seven patients in group A and 24 patients belonging to group B had generalized seizures at presentation whereas three and six patients presented with partial seizures at the time of enrolment in group A and B respectively. After treatment with clobazam seven and two patients had generalized and partial seizures respectively while the figures were 23 and 2 for group B patients. Thus, recurrence of FS was observed in 30% (nine) patients in group A and 83.3% in group B, this difference was statistically significant. There were 151 and 161 febrile episodes during the course of study in group A and B, respectively, 0.139 seizures per febrile episodes were observed in group A after treatment with clobazam and 0.820 seizures per febrile episodes in the placebo group. There was a significant reduction in the frequency of seizures per febrile episode with clobazam as compared to placebo. The average number of seizures preceding six months was 4.33 ± 2.78 in the clobazam and, this declined significantly to 0.7 ± 1.37 (P < 0.001), while in the placebo group no decline in seizure frequency was observed. There was a significant prolongation of seizure free interval in group A treated with clobazam on comparison to placebo group. No significant adverse effect except irritability was observed in four patients on clobazam. Clobazam is efficacious and well tolerated as intermittent therapy for FS and is superior to the use of intermittent antipyretics alone.
Abstract: This non-randomized, uncontrolled, single-center study was conducted to evaluate the effects of zonisamide on intractable epilepsy in a primarily pediatric population. Twelve patients ranging in age from 18 months to 23 years were included in the study. All patients were diagnosed with intractable seizures. Adjunctive therapy with zonisamide was initiated at 100~mg once a day and increased, if needed, by 50–100 mg after one week to reach the maximal or therapeutic dosage depending on patient age…and weight. Follow-up occurred in person or by phone at two weeks, one month, and every three months. All 12 patients responded favorably to adjunctive zonisamide. Five patients had subclinical status interrupted and stopped as evidenced by clinical examinations and electroencephalographic (EEG) findings. These patients also became awake, alert, more attentive, and more interactive. An additional six patients experienced clinical and EEG improvement in their seizure frequency, duration, and severity. One patient with autonomic seizures showed EEG improvement in seizure frequency, duration, and severity; however, seizure prodrome control was not obtained. Adjunctive therapy with zonisamide benefited all patients in this study, despite the variation in symptoms and etiology. Zonisamide is thought to have multiple mechanisms of action, which may benefit more patient types than antiepileptic drugs with a single mechanism of action. Zonisamide was well tolerated by all patients.
Keywords: zonisamide, intractable seizures, children
Abstract: Children with seizures may also have behavioral problems including hyperactivity. The management of hyperactivity in these cases is complicated by the fact that pharmacotherapeutic interventions may worsen the seizures. In this study 10 children with epilepsy and attention deficit hyperactivity disorder were treated with risperidone in an open label fashion. The dose was titrated up from 0.5 mg to a maximum of 3 mg per day based on clinical efficacy and tolerability. Clinical improvement was noted…in all the 10 patients. Tiredness was reported by one patient, and drowsiness by another, but none experienced extra pyramidal side effects. Furthermore, there were no untoward effects on the frequency or intensity of the seizures. Our findings suggest that risperidone in combination with anticonvulsant medication is safe and effective in treating children with seizures and hyperactivity. Controlled studies are needed to determine long-term efficacy and safety of this drug in this special population.
Abstract: A retrospective study was conducted to assess the effectiveness and tolerability of quetiapine in children and adolescents with subaverage replaced with IQ. Through a chart review conducted in a specialized outpatient clinic, five patients were identified (three males, two females; mean age = 16 ± 3.7 years); three were judged to be responders. Patients were included if concomitant medications remained unchanged during the titration and maintenance periods. Treatment duration was 20 ± 4 weeks; dose was…620 ± 205 mg/day. Improvements were observed in the conduct and hyperactivity subscales of the Conners Parent Rating Scale, routinely completed by the same caregiver. No differences were noted in anxiety, inattention, learning, and psychosomatic subscales. None of the patients discontinued the medication due to adverse events, but two experienced sedation. Quetiapine may be beneficial for treating associated symptoms of mental retardation; however, prospective and controlled studies are needed to assess further its efficacy and safety.
Abstract: A 4-month-old female infant presented with paroxysmal clonic or tonic hemifacial contractions. Afterward, she sometimes had clonic hemibody contractions. Nystagmoid eyeball movements were also occasionally seen. These evolved to stereotypic clinical patterns and frequencies, despite administration of antiepileptic drugs. Magnetic resonance imaging revealed a mass in the right cerebellar hemisphere, involving the right middle peduncles and effacing the fourth ventricles. Video-scalp electroencephalogram monitoring revealed a left fronto-temporal irregular spike slow wave that…at times propagated to the contralateral hemisphere. The lesion was removed and pathologic diagnosis was ganglioneurocytoma. Postoperatively, the infant did not present any new seizures but she died eight days after surgery due to severe duodenal hemorrhage. We suggest that cerebellar ganglioneurocytoma may cause epileptic hemifacial seizures of subcortical origin as was previously described.
Abstract: We report the case of a 14-year-old boy with acute disseminated encephalomyelitis who, rapidly deteriorated while on intravenous methylprednisolone. Emergency hemicraniectomy had to be done to control the raised intracranial pressure when the conventional methods failed to achieve a reduction in intracranial pressure.
Abstract: In moyamoya disease, the anterior blood circulation in the brain is occluded and the characteristic appearance of the disease is seen in angiography. Moyamoya disease may be primary or secondary to diseases or syndromes such as Down syndrome. A 20-month-old girl with Down syndrome was admitted because of weakness and convulsions. She had left-sided hemiparesis. Cranial magnetic resonance imaging showed hyperintense lesions in both cerebral hemispheres in T2-fluid attenuated inversion recovery, and cranial angiography…showed approximately 80–90% stenosis in the proximal region of the right middle cerebral artery fork, and left middle cerebral artery occlusion in the proximal region; left middle cerebral artery vascularization was provided by collateral arteries. These findings were consistent with those of moyamoya disease. Some proteins affecting arterial physiology are encoded on chromosome 21. These proteins are probably responsible for moyamoya disease in patients with Down syndrome. Our patient is one of the youngest with reported moyamoya syndrome and Down syndrome, and may point to the early onset of the disease.
Keywords: moyamoya syndrome, Down syndrome, infarction, angiography
Abstract: Pseudoseizures (PS) resemble epileptic seizures. They are strictly linked to personality disorders and may involve all ages, with a higher incidence during adolescence. Patients experience episodes of loss of consciousness, twitching or jerking, and unusual emotional states, such as intense feelings of fear or "déjá vu". The episodes may last 20 minutes, but unlike epileptic seizures, they are not associated with electrical abnormalities in the brain. Even for trained medical professionals, differentiating…epileptic seizures from PS is difficult. Physicians believe PS are psychological defense mechanisms induced by stress or episodes of severe emotional trauma; in fact, they tend to occur when patients try to avoid or forget the trauma. Twenty percent of patients with seizures are ultimately diagnosed with PS but the diagnosis is difficult to make on the first examination. Patients with PS are often referred to the Emergency Department because of their poor response to conventional anticonvulsant drugs. Due to the apparently critical condition of these patients on presentation, they are often referred to a Pediatric Intensive Care Unit (PICU) and may be over-treated during their difficult and long diagnostic course. We report two cases admitted to our PICU for apparent status epilepticus, in which the final diagnosis was PS.
Keywords: pseudoseizures, pediatric intensive care unit, status epilepticus, adolescence