Journal of Pediatric Neurology - Volume 10, issue 2
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The
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.
The
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: Working memory (WM) performance is considered to change according to the nature of the task by adequate and prompt activation of corresponding functional connectivity in the brain. In the present study, we examined continuous prefrontal hemodynamic changes depending on reciprocal disposition of WM and non-WM tasks using two-channel near-infrared spectroscopy. To investigate possible functional connectivity deficits in autism spectrum disorder (ASD) during these tasks, relative concentration changes in oxygenated hemoglobin (Hb), deoxygenated…Hb, and total Hb were compared between high-functioning ASD subjects (n=11) and controls (n=22). Instant evoked cerebral blood oxygenation changes were observed in response to the task switch in controls but not in ASD subjects, although the task performance rate was almost equivalent. Delayed or altered response of functional connectivity to incoming stimuli is considered a characteristic feature of ASD.
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Keywords: Autism, executive function, working memory, default mode network
Abstract: To describe the group of patients under the age of 18 who have neuromuscular disorders and are from a region with 1.65 million inhabitants in South-Eastern Norway. Patients with known or suspected neuromuscular disorders were identified by neuropediatricians/rehabilitation teams in the region. We registered a total of 141 patients under the age of 18 who had a neuromuscular diagnosis, giving a prevalence of 35.6 × 10^{-5} in this population. It was possible to…check the diagnoses of 122 patients. Duchenne muscular dystrophy was the largest group, followed by peripheral neuropathies, myotonic dystrophy and spinal muscular atrophy. The prevalence figures for the different diagnostic entities are discussed on the basis of previously reported studies from the same area, from a comparable Swedish area and from other countries. We registered patients with a broad spectrum of neuromuscular diagnoses in our health region. The classical, well-known entities were the most prevalent conditions. It was difficult to make a very specific diagnosis in quite a few cases.
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Abstract: There is high prevalence rate of attention deficit/hyperactive disorders (ADHD), and there is vague relation between it and epileptiform abnormalities. The objective of this study is to assess the relation between epileptiform abnormalities and ADHD. We studied 50 ADHD children and 25 sex and age matched controls. We used 20 channels electroencephalography under standard condition for assessing patients and control. ADHD rating scale was used for assessing patients. Epileptiform abnormalities were detected in 15 (30%) of…ADHD children, in comparison with two (8%) of control group (P < 0.005). While, we did not find any correlation between ADHD subtypes and pattern of epileptiform discharge.
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Abstract: The stigma on Nigerian children (aged 6–18 year) living with epilepsy attending the Pediatric Neurology Clinic in Calabar was studied. The survey was conducted between September 2010 and February 2011 on children having established epilepsy and was attending the Neurology Clinic of the University of Calabar Teaching Hospital, Calabar. A questionnaire with open-ended responses was drawn up and administered on the epileptic children. Information was collected from the children privately in the absence of the…parents. The children were interviewed by the authors and the data recorded into the form by the latter. Forty percent of the children reported being regarded as demon-possessed, some were said to be mad and suffering under a family curse. Sixty percent of the children claimed being made objects of ridicule. Fifty percent of the children would wish to withdraw from school while 46% would change school because of embarrassment. Fifty-six percent knew that friends and schoolmates usually run away from them during attacks, 29.4% claim their teachers will not help but only watch them while fitting at school. Twenty-four percent of the victims, all in the age bracket 12–18 yrs, expressed suicide wish. Stigma associated with epilepsy is a problem of children in Calabar. Education of parents and the entire populace is advocated as this could change the society's misconception of epilepsy.
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Abstract: A cross sectional study was undertaken to assess the knowledge and attitudes of secondary education teachers towards schoolchildren with epilepsy. Questionnaires were administered to three randomly selected schools and systematic random sampling method applied to select teachers in these schools. Their demographic characteristics and responses to the questions were analyzed. Out of the 105 teachers that returned completed forms, majority (97) were university graduates. Sixty-nine (65.7%) are aware that epilepsy may be…caused by brain infection, 14 (13.3%) thought it is due to head trauma, while eight (7.6%) associates epilepsy with evil spirits; three (2.9%) thought that epilepsy follows a family curse and 11 (10.5%) were not sure of the cause. Concerning treatment, 89 (84.8%) believed the disease is curable but only 52.4% recommended orthodox treatment. Eighty-seven (82.9%) of the respondents will assist a convulsing child while 18 (17.1%) would either run away or not touch a convulsing child. Forty-nine (46.7%) of the teachers believed that epilepsy is contagious. There is a statistically significant relationship between the teachers who believed epilepsy is contagious and the action taken when a child is convulsing (χ ^{2} = 21.06, P= 0.00). Sixty (57.1%) of teachers thought that children with epilepsy are intellectually deficient, nevertheless, only 37.1% recommended special schools for children with epilepsy and another 11.4% suggested total exclusion of the children from school. There is relative lack of knowledge on epilepsy leading to misconceptions and stigmatization. These teachers can hand on same misconceptions to their pupils and by extension the entire community. We therefore, recommend an elaborate educational campaign on epilepsy by health professionals.
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Abstract: Having a child with cerebral palsy (CP) in developing countries of the world is particularly burdensome. This study investigated psychosocial impact on the family caring for a child with CP, examined possible association of severity of gross motor function impairment on the psychosocial impact and compared impact scores of families with CP child with that of families with no CP child. Participants consisted of 117 parents/caregivers of children with CP and 117 parents/caregivers of children without…CP whose children attended purposively selected secondary and tertiary health institutions in South-West region of Nigeria. Impact on family (IOF) scale was used to assess psychosocial impact while gross motor function classification system for CP was used to rate severity of motor function impairment in children with CP. Participants were mostly of low income 59 (25.2%) and middle income 85 (36.3%) categories. Children were mostly in the 1–3 year age range 116 (49.6%). Birth asphyxia was most reported cause of CP and no social support service was reported. Significantly higher mean subscale and total IOF scores were observed in families with CP child (37.4 ± 8.91) compared with families with no CP child (20.8 ± 4.98) P=0.000. Significant differences did not exist in mean total IOF scores and severity of motor function impairment in children with CP (P=0.16). Presence of a child with CP imposes tremendous psychosocial challenges on the family irrespective of severity of motor function impairment. Implementation of an effective and accessible social support system by Nigerian policy makers is imperative.
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Keywords: Cerebral palsy, psychosocial impact, family, children
Abstract: Neonatal subgaleal hematomas, rare but potentially fatal accumulations of blood beneath the galea aponeurotica of the scalp, are often associated with instrumental deliveries. We report an unusual case of disseminated intravascular coagulation and massive subgaleal hematoma in a neonate secondary, to placental abruption and disseminated intravascular coagulation in the mother. The neonate presented with subgaleal and subarachnoid hemorrhage and hypovolemic shock soon after birth and developed severe hyperbilirubinemia later on requiring multiple…exchange transfusions and aspiration of the hematoma.
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Abstract: Subacute combined degeneration of spinal cord is a rare neurological manifestation of vitamin B12 deficiency due to inadequate dietary intake in children. We report a case of 4.5-year-old girl who was vegan with developmental regression, hyperpigmentation over extremities and features of subacute combined degeneration of spinal cord, a rarity in children. Laboratory investigations and neuroimaging were confirmatory. She responded well to treatment and she is having no complaints at present.
Keywords: Vitamin B12, subacute combined degeneration of cord, hyperpigmentation
Abstract: After 18 months, the open anterior fontanel (AF) persistence has been described in several neurological, genetic and metabolic disorders. Nevertheless, there are few healthy children published reports where the fontanel closure occurred later than expected. The case of a 4.2-year-old male child will be reported with persistent AF and it was perceived as a normal variation after anamnesis, physical exam and laboratory investigations. It is important to know the several syndromes, diseases and exposure to toxic…substances that may cause AF closure delay; since through clinical evaluation, few laboratory tests and neuroimaging we can confirm the possibility of a simple but rare normal variation.
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Abstract: The presence of an alpha pattern on electroencephalography is well described in patients with coma following trauma, hypoxia, metabolic dysfunction and cardiorespiratory arrest. This is generally regarded as an adverse prognostic factor especially the non-reactive complete form. We report a 15-year-old girl with complete alpha coma following head injury, who subsequently made a good physical recovery. We believe that this case should help pediatric neurologists, intensivists and neurophysiologists in the future,…who have to make difficult management decisions and will inform their discussions with the child's parents or guardians.
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Abstract: Diphtheritic neuropathy seldom has been observed nowadays in India beyond 1980 probably due to effective immunization. We report an 11-year-old adolescent girl, not adequately immunized who had pharyngitis with bull neck and subsequently presented with palatal weakness, accommodation paralysis, lower cranial nerve palsy and peripheral neuropathy, features characteristic of diphtheritic neuropathy. She was managed with supportive treatment. The specific treatment (diphtheria antitoxin) was not useful in our child as the child presented…4 wk after diphtheria infection. This case report will increase the awareness of sporadic occurrence of a rare disease among fellow pediatricians.
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Abstract: Leukemia is the most common malignancy in children. There were different atypical presentations of acute leukemia described in literature like cardiac metastasis, maxillofacial mass etc. Macrocephaly as a presentation of acute lymphoblastic leukemia is reported earlier. We describe a 3-year-old male who presented with macrocephaly and chronic raised intracranial pressure, which was subsequently diagnosed to be case of acute promyelocytic leukemia. This pointed out that macrocephaly could even be a rare and early manifestation of…acute myeloid leukemia in children.
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Abstract: In this paper, we report a case of a 5-year-old girl whose intractable seizures ceased after introduction of a lactose free diet. Because of epileptic seizures, antepileptic agents and vagal nerve stimulation treatment were applied to the patient, who had mental and motor retardation, over a period of 4 years. Despite treatments, seizures were refractory. An electroencephalogram showed a continuous pattern of generalized discharges briefly attenuated by short periods of suppression. A lactose tolerance test…was performed and lactose intolerance was diagnosed. A lactose-free diet was given and after this diet was applied, no further seizures were seen. Galactose deficiency due to lactose intolerance might have been the cause of motor and mental retardation in this patient. We speculate that lactose intolerance should be considered in the differential diagnosis of intractable seizures. Early diagnosis of lactose intolerance may prevent motor and mental retardation.
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Abstract: Our aim is to describe the difficulties in the diagnosis and treatment of a child with continuous unilateral headache. We present the case of a 12-year-old child who had a two year history of continuous dull pain behind the right ear with frequent episodes of severe sharp pain as well as ipsilateral color change on the cheek. We assess the role of international classification of headache disorders in establishing the diagnosis and we assess the treatment…options. The international classification of headache disorders criteria for the diagnosis of hemicrania continua are, based on experience with adults and may not be adequate in children. The child had a very poor quality of life due to his illness and failed to respond to indomethacin among many other medications. However, he responded well to treatment with botulinum toxin type-A on two occasions. Childhood hemicrania continua may have a different phenotype than that in adults. Botulinum toxin type-A may be an option for treatment in some patients.
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Abstract: Kleine-Levin syndrome~is an exceptionally rare disorder characterized by recurrent episodes of hypersomnia associated with varying degrees of behavioral or cognitive disturbances and compulsive eating behavior. The disorder occurs predominantly in adolescent males with a benign clinical course and spontaneous remission as long as other neurological or psychiatric diseases are excluded. A viral or an autoimmune etiology has been suggested as up to 70% of patients have flu-like symptoms at the onset of their symptoms.…In this report, we present a 9-year-old female patient with this rare disorder and review the relevant pediatric literature. She was previously healthy and presented with a 1-month history of generalized fatigue, excessive sleep and behavioral change. She would sleep for most of the day even while sitting and would resist being awakened. During brief periods of wakefulness, her mood will fluctuate from apathy to anger, sadness and aggression for minimal reasons. She would eat and drink excessively with a notable weight gain. Clinical examination and detailed investigations, including brain computerized tomography scan and electroencephalography were within normal limits. Remarkable improvement of her symptoms was noted after initiating methylphenidate and carbamazepine. We conclude that Kleine-Levin syndrome is an interesting rare disorder that needs to be diagnosed after excluding other causes of excessive sleep. Early recognition would result in appropriate treatment and shortened period of hospitalization.
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