Journal of Pediatric Neurology - Volume 5, issue 3

Authors: Jan, Mohammed M.S.

Article Type: Research Article

Abstract: Hypotonia in infants can be a confusing clinical presentation leading to inaccurate evaluation and unnecessary investigations. Hypotonia can result from a variety of central or peripheral causes. Therefore, hypotonia is a phenotype of many clinical conditions with variable prognosis. It is important to recognize that hypotonia is not equivalent to weakness. Infants with central causes, such as Down syndrome, may have severe hypotonia with normal muscle strength. Peripheral hypotonia is frequently associated with …weakness, which can be predominantly distal in neuropathies or predominantly proximal in myopathies. In general, central hypotonia is much more commonly encountered; however, the prognosis is worst for hypotonia secondary to neuromuscular pathology. The distinction between central and peripheral hypotonia is therefore critical for proper evaluation and management. Stepwise and accurate assessment is very important to reach the correct diagnosis promptly. In this review, I present a concise clinical approach for evaluating the hypotonic infant. Some practical tips and skills are discussed to improve the likelihood of obtaining an accurate diagnosis. Reaching a specific diagnosis is needed for providing appropriate therapy, prognosis, and counseling. Show more

Keywords: Infant, child, hypotonia, floppy, examination, approach

Citation: Journal of Pediatric Neurology, vol. 5, no. 3, pp. 181-187, 2007

Authors: Wisniewski, Tami | Brimacombe, Michael B. | Ming, Xue

Article Type: Research Article

Abstract: The overall design quality of studies conducted on psychotropic substances prescribed to autism spectrum disorder and attention deficit hyperactivity disorder children is reviewed. Searches were conducted on Medline and Cochrane Controlled Trials Register. Peer-reviewed, published studies, written in English were included. Only studies that focused on children, had a minimal sample size of ten participants, and were conducted between 1994 and 2004 were included in the review. Two hundred and eighty one studies were …identified and reviewed with 184 (65.5%) focused primarily on methylphenidate, with the remaining 97 studies examined stimulants, antipsychotics, antidepressants, alpha agonists, anxiolytics, and mood stabilizers. Of these, 152 (54.1%) did not employ optimal double blind placebo controlled design and 106 (37.7%) were conducted with less than 30 participants. The upper quartile for median study duration across all medications examined was 9.75 weeks for double-blinded studies and 16 weeks for all studies. Many pharmacotherapy studies conducted on children with autism spectrum disorder and attention deficit hyperactivity disorder are of limited duration, weak quality design and may be seriously underpowered. The issue of follow-up is rarely adequately addressed. These findings raise both safety and efficacy questions that have yet to be adequately studied. Show more

Keywords: Autism, ADHD, pharmacotherapy, design quality

Citation: Journal of Pediatric Neurology, vol. 5, no. 3, pp. 189-197, 2007

Authors: Tomoda, Akemi | Mizuno, Kei | Murayama, Nobuki | Joudoi, Takako | Igasaki, Tomohiko | Miyazaki, Makoto | Miike, Teruhisa

Article Type: Research Article

Abstract: The aim of this study was to investigate cognitive functions such as allocation and information processing in patients with childhood type chronic fatigue syndrome (CCFS) using event-related potentials. A total of 190 healthy children as controls and 414 patients with CCFS participated in this study. Both patients and controls had (i) event-related potentials (including P300, P100, N150 and P200 measurements); (ii) component analysis of electrocardiographic R-R interval to evaluate autonomic function; and (iii) …KANA-Pick-out test to evaluate frontal lobe function. The cutoff threshold defining abnormal P300 latency to target stimuli and amplitude to non-target stimuli was set at two standard deviations above the mean values of controls. Forty patients (classified as Type-I) had an abnormally-prolonged P300 latency to target stimuli, and 49 patients (classified as Type-II) had an abnormally-exaggerated P300 amplitude, and the remaining 325 patients whose P300 latency and amplitude ranged below the above-mentioned threshold were classified as Type-III. All patient groups had normal P100, N150 and P200 measurements. Component analysis of electrocardiographic R-R intervals revealed that the power of high-frequency components was lower in all patient groups, compared to controls. The score in KANA-Pick-out test was worse in all patient groups compared to controls; Type-I Group had the worst score and Type-II Group had the best score among the patients. Taken together, we speculate that abnormally-prolonged P300 latency to target stimuli might be associated with learning disability and abnormally-exaggerated P300 amplitude to non-target stimuli might be associated with hypersensitivity such as phobia in patients with CCFS. Psychosomatic symptoms in patients with CCFS might be associated with higher-order level cognitive dysfunction. Show more

Keywords: Event-related potential, P300, childhood type chronic fatigue syndrome, electrocardiographic R-R interval, frontal lobe function

Citation: Journal of Pediatric Neurology, vol. 5, no. 3, pp. 199-208, 2007

Authors: Awaad, Yasser | Michon, Anne Marie | Minarik, Sarah

Article Type: Research Article

Abstract: Tics are a common disorder in children. Some available drugs have unacceptable side effects, and thus alternative treatments are needed. Levetiracetam is an antiepileptic drug reported to be useful for the treatment of tics. Patients age 18 years and younger with tics and Tourette syndrome were enrolled in this prospective, open-label study. Seventy patients were treated with levetiracetam as monotherapy for four years. The initial dose of levetiracetam was 250 mg/day. The dose was titrated over …three weeks to 1,000 to 2,000 mg/day. Clinical outcomes were assessed with the Clinical Global Impression Scale, Yale Global Tic Severity Scale, Revised Conners' Parent Rating Scale, and Revised Conners' Teacher Rating Scale. After four years of treatment with levetiracetam, all 70 patients showed some improvements based on the scales used. A total of 49 patients improved with regard to behavior and school performance. Levetiracetam was generally well tolerated. Despite the limitations of open-label studies, these results suggest that levetiracetam may be useful in treating tics in children and adolescents. Given its established safety profile, levetiracetam is a candidate for further study. Show more

Keywords: Levetiracetam, Tourette syndrome, tics, adolescents, children

Citation: Journal of Pediatric Neurology, vol. 5, no. 3, pp. 209-214, 2007

Authors: Peña, Joaquín A. | Cruz, Eduardo Mora La | Leendertz, Reinier | Faneite, Lysabella Soto | Montiel-Nava, Cecilia

Article Type: Research Article

Abstract: Aim of the study was to characterize findings on magnetic resonance imaging (MRI) and proton magnetic resonance spectroscopy (^{1} H-MRS) in children with late infantile neuronal ceroid lipofuscinosis (NCL) and the relationship between these results and duration of the disorder. Three children with late infantile NCL and six age-matched controls were examined with MRI and by localized ^{1} H-MRS. Voxel regions studied were periventricular frontal white matter, thalami and cerebellar …deep white matter. N-acetylaspartate/creatine (NAA/Cr) and choline/creatine (Cho/Cr) ratio was calculated. MRI showed various degrees of cortical atrophy and increased periventricular white matter signal intensity on T2- and -fluid attenuated inversion recovery-weighted images. There was no significant volume loss in the brainstem. Metabolic ratio (NAA/Cr and Cho/Cr) showed progressive decrease with longer disease duration in thalamic and cerebellar voxels and exhibited a negative relationship in frontal voxels. In conclusion, the most significant changes on neuroimaging in late infantile NCL are progressive cortical and cerebellar atrophy, which are more pronounced in the cerebellum. Decreased signal intensity in the thalami may also be observed as in other forms of NCL. ^{1} H-MRS is the most sensitive method for measuring metabolic changes. The decreased level of NAA although not specific, may still contribute significantly to the diagnosis of late infantile NCL. Show more

Keywords: Magnetic resonance imaging, lipofuscinosis, late infantile

Citation: Journal of Pediatric Neurology, vol. 5, no. 3, pp. 215-220, 2007

Authors: Al Jumah, Mohammed | Majumdar, Ramanath | Rehana, Zahra | Al Rajeh, Saad | Eyaid, Wafaa

Article Type: Research Article

Abstract: Spinal muscular atrophy (SMA), an autosomal recessive inherited disease, is a leading genetic killer under the age of two years. This disease is caused by mutation in the telomeric copy of the survival motor neuron gene (SMN1). Most carriers of SMA have one chromosome 5 with a normal SMN1 gene and one with a deleted SMN1 gene. Although no large-scale population-based studies has been done in Saudi Arabia, previous estimations have indicated that incidence of …SMA is much higher in Saudi population compared with other ethnic groups, partly because of high prevalence of consanguineous marriages. In this pilot study, we used a highly sensitive quantitative-polymerase chain reaction method to detect SMA carriers. Among 187 normal individuals not related to an SMA patient the carrier frequency was 5%, i.e. one carrier in each 20 persons compared to one in 50–80 in other parts of the world. Based on the results of this pilot study a new project is designed to further analyze the carrier status of SMA in Saudi population. This will shed more light on the magnitude of the problem of SMA in the countries with high rate of consanguinity. Show more

Keywords: Spinal muscular atrophy, carrier screening, gene deletion, Saudi population

Citation: Journal of Pediatric Neurology, vol. 5, no. 3, pp. 221-224, 2007

Authors: Kumar, Rashmi | Kumar, Chandra Bhushan | Chandrakanta, | Rao, Yashwant

Article Type: Research Article

Abstract: Meningoencephalitides (ME) are an important group of disorders that constitute a medical emergency in any age group, more so in children. The physician uses the lumbar puncture (LP) as a diagnostic test to confirm or exclude the diagnosis of ME. Of all the LPs ordered, ME is found in only a small proportion. The need for LP is often alarming to the family. The diagnostic accuracy of different clinical symptoms and signs may therefore help the …physician in being more discriminative in use of LP. Setting: Pediatric wards of a teaching hospital in Northern India. Methods: Consecutive children between one month and 12 years of age undergoing LP on the suspicion of ME on three preselected weekdays were enrolled in the study and all relevant clinical features, total and differential leukocyte counts in blood were recorded. Outcome measurement was presence or absence of ME diagnosed on the basis of cerebrospinal fluid (CSF) examination for cells, protein, sugar and bacterial culture. Clinical features, total and differential leukocyte counts were compared in ME and nonME groups. Sensitivity and specificity of clinical features were computed and logistic regression and stratified analysis by age performed. Results: One hundred and nineteen patients were enrolled, of which 35 (29.4% had ME. Only one symptom, headache, was significantly more common in ME. All the meningeal signs were more common in ME with sensitivity being highest for Vincent's sign (76.9%. Tense, bulging anterior fontanelle, focal neurological deficit, and mean polymorphonuclear (PMN) leukocyte percentage in blood were also significantly higher in ME. Logistic regression showed that only neck rigidity and PMN leukocyte percentage were significant independent predictors of ME. In children beyond 24 months of age, fever was present in 19 out of 20 cases of ME. Presence of fever and headache achieved high sensitivity while neck rigidity, Vincent's sign and focal deficits had high specificity in this age group. In children below 12 months of age, none of the signs achieved adequate accuracy. Conclusions: In hospitalized children undergoing LP on suspicion of ME, neck stiffness along with high PMN leukocyte percent (>80%) in peripheral blood are highly suggestive of ME. Absence of fever beyond two years of age almost rules out ME. However, no clinical features are adequately discriminative for the diagnosis of ME in infants. Show more

Keywords: Meningoencephalitis, meningeal signs, lumbar puncture, sensitivity, specificity

Citation: Journal of Pediatric Neurology, vol. 5, no. 3, pp. 225-230, 2007

Authors: Asadi-Pooya, Ali Akbar | Ghetmiri, Ehsan

Article Type: Research Article

Abstract: The aim of this case-control study was to determine whether there is any association between idiopathic generalized epilepsy and allergic disorders in children. All children with idiopathic epilepsy attending the Motahary Clinic, Shiraz, Iran, in a 7-month period were included in the study. Neurologically normal children, matched for age and sex, were considered as controls. The required data including age, gender, history of allergic disorders, history of smoking in the family members and breast versus formula …feeding in the first six months of life were collected. The total number of participants was 110 patients and 100 controls. History of allergic disorders was positive in 28.2% of the patients and 36% of the controls (P=0.225). When only patients with idiopathic generalized epilepsy were considered (100 patients), allergic disorders were reported in 28%. The prevalence of allergic disorders in patients with idiopathic generalized epilepsy was not significantly different from that in controls (P=0.225). Family history of allergic disorders was positive in 34.5% of the patients and 45% of the controls (P=0.122). Our study failed to demonstrate any association or link between idiopathic generalized epilepsy and allergy. Show more

Keywords: Allergy, epilepsy, children

Citation: Journal of Pediatric Neurology, vol. 5, no. 3, pp. 231-232, 2007

Authors: Mentzel, Hans-Joachim | Kentouche, Karim | Güllmar, Daniel | Reichenbach, Jürgen R. | Gruhn, Bernd | Kaiser, Werner A.

Article Type: Research Article

Abstract: We describe changes in apparent diffusion coefficient (ADC) and fractional anisotropy (FA) in tacrolimus associated encephalopathy. ADC was clearly increased and FA decreased in the involved regions. There was a strong local contrast-enhancing effect along the cortical surface. Repeat magnetic resonance imaging five days later showed an increase in FA; but no contrast enhancement. The observed ADC and FA changes in the acute phase suggest that vasogenic edema may play a pivotal role in tacrolimus induced …neurotoxicity. The contrast-effect can be explained by disturbed venous drainage caused by thrombotic microangiopathy. Show more

Keywords: Magnetic resonance imaging, diffusion tensor imaging, fractional anisotropy, apparent diffusion coefficient, tacrolimus, thrombotic thrombocytopenic purpura, stem cell transplantation

Citation: Journal of Pediatric Neurology, vol. 5, no. 3, pp. 233-238, 2007

Authors: Lai, Chi-Chieh | Huang, Wen-Chin | Kao, Hung-Wen | Lee, Chuen-Ming

Article Type: Research Article

Abstract: Single fetal death for a twin pregnancy is often associated with multiple organ damage for the surviving twin, especially involving the central nervous system. The surviving twin may reveal normal neural function incipiently and its condition can easily remain undiagnosed. Herein, we present the case of a surviving twin complicated with asymptomatic unilateral cerebral infarction, and featuring the concomitant intrauterine demise of the other fetus, magnetic resonance angiography for the survivor revealing no evidence of …narrowing of cerebral vessels. Show more

Keywords: Cerebral infarction, intrauterine death of twin fetus, twin pregnancy

Citation: Journal of Pediatric Neurology, vol. 5, no. 3, pp. 239-241, 2007