Journal of Pediatric Neurology - Volume 11, issue 3

Authors: Tomoum, Hoda Y. | Aly, Rasha H. | Youssef, Omneya I. | Abdelal, Hala | Nour, Mohamed U. | El Sharkasy, Amina H.

Article Type: Research Article

Abstract: To evaluate ictal serum levels of heart type fatty acid binding protein (H-FABP) in children with intractable epilepsy as a marker of cardiac ischemia in comparison to their levels in the interictal period and that of controls. The study included 23 seizure episodes recorded in 14 patients with intractable seizures. Serum levels of H-FABP in the ictal and interictal periods were done as well as simultaneous electrocardiogram, Holter and video electroencephalography recordings with evaluation of changes …in the heart rate (HR), and HR variability parameters. Levels of H-FABP were higher in the patients' group whether in the ictal or interictal periods compared to the control group. However, levels were not significantly different in the ictal period compared to the interictal one. Ictal tachycardia was recorded in 95% and bradycardia in 5% of the studied seizures episodes. Premature atrial and ventricular contractions were recorded in the 1st postictal hour of 8.7% and 30.4% of the recorded seizures, respectively. Interictal values showed significant increase minutes before the onset of the seizures and remained high 2 hours postictally. All time domain HR variability parameters were lower in the patients' group. Our results showed significant increase in the serum levels of H-FABP in our patients' group, which might suggest a degree of myocardial ischemia even in asymptomatic patients. Though bradycardia, prolonged QTc and arrhythmic changes were reported in few cases, the importance of the results warrants studying those changes at least in patients with refractory seizures. Show more

Keywords: Epilepsy, heart rate, heart rate variability, heart type fatty acid binding protein, Holter

DOI: 10.3233/JPN-130621

Citation: Journal of Pediatric Neurology, vol. 11, no. 3, pp. 149-157, 2013

Authors: Malekian, Armen | Barzegar, Mohammad | Malaki, Majid | Shiva, Siamak

Article Type: Research Article

Abstract: Guillan-Barré syndrome (GBS) is a debilitating illness with different subtypes depending on geographical area. In this study we review the GBS subtypes in Iranian children. One hundred and eight children below 15 yr entered the study during 7 yr (1998–2005), they were evaluated in 1st (95 cases) and 2nd wk (13 cases) diagnosed clinically, electrodiagnostically and their cerebrospinal fluid (CSF) were analyzed. Patients were divided into demyelinating and axonal forms. Data were analyzed by t test …for continuous measures and the Fisher's exact test for categorical variables in SPSS 16. P less 0.05 was significant. The means age ± SD of affected patients are 4.8 ± 2.7 yr (minimum 1 yr, maximum 14.5 yr). CSF protein and pleocytosis were more common in 2nd wk group (77 mg/dL in first week versus 63 mg/dL in second week); 2% (first week) and 30% CSF pleocytosis occurred (second week). Demyelinating form happens in 47% of patients, axonal 40% and combined form of axonal and demyelinating form happened in 9%. Normal electrodiagnostic tests in spite of clinically confirmed GBS were observed in 4%. CSF protein was higher in demyelinating compared to axonal form (71 mg/dL and 54 mg/dL). There is no sex predilection for GBS affection (59 males, and 49 females). Peak incidence of GBS in children is below age of 5 yr (65%); axonal form of GBS (sensory and motor) make a considerable proportion of GBS in children (40%) with lower CSF protein compared to demyelinating type, but it is not statistically significant (P > 0.05). Show more

Keywords: Guillan-Barré syndrome, children

DOI: 10.3233/JPN-130622

Citation: Journal of Pediatric Neurology, vol. 11, no. 3, pp. 159-163, 2013

Authors: Kwon, Soonhak | Choe, Jaeyoung | Seo, Hyeeun

Article Type: Research Article

Abstract: Sleep is an essential physiologic process for health and analyzing sleep architecture helps us not only to diagnose sleep disorders, but also to understand the pathophysiology of these diseases. Therefore, this study was aimed to analyze the sleep structure in children with sleep disorders. Thirty-four children diagnosed with obstructive sleep apnea, parasomnia, or narcolepsy participated in this study from March 2008 to September 2011. Their polysomnographs were reviewed and their sleep parameters were analyzed. Their …mean age was 9.3 ± 4.1 yr (range: 3–12-year-old) and male to female ratio was 3.8. Patients with obstructive sleep apnea showed increased sleep latency and total arousal index and a decreased N3 sleep compared to other groups. In children with parasomnia, apnea hypopnea index and periodic limb movement index were not increased and rapid eye movement (REM) sleep, REM sleep latency were decreased. Patients with narcolepsy showed decreased sleep latency and REM sleep latency. Among the three groups, there was no difference except apnea hypopnea index. The result showed that sleep architectures in children with sleep disorders were relatively similar to previous studies from Western countries. In addition, it was judged that this study would provide fundamental knowledge about sleep structure of Korean children. However, further studies will be required to provide better understanding about the sleep architectures in children with sleep disorders. Show more

Keywords: Sleep architecture, polysomnography, sleep disorder

DOI: 10.3233/JPN-130616

Citation: Journal of Pediatric Neurology, vol. 11, no. 3, pp. 165-170, 2013

Authors: Assadi, Mitra | Zerafati, Gazelle | Velazquez, Yadira | Leone, Paola

Article Type: Research Article

Abstract: The frequency of probable migraine (PM) remains elusive; some of the published literature estimate PM to be at least as prevalent as definite migraine (DM) while others report PM to affect about 5% of the population or less. We conducted a cross sectional survey of female high school students using a validated questionnaire which covered the diagnostic features of DM, PM and tension type headaches as specified by the international classification of headache disorders. The survey …also inquired about subjects' treatment history and analgesic use. Headache related disability was ascertained using headache impact test 6 (HIT-6). The participants included 309 girls with a mean age of 16.0 yr. The prevalence of DM and PM in our cohort were 18% and 25% respectively. Overall, headaches were more frequent in the DM group although this did not reach statistical significance (P < 0.06). There was no statistical significance between DM and PM in respect to seeking medical care (P < 0.30). The individuals with tension type headaches however, were less likely to seek medical care compared to the ones with PM or DM (P < 0.001). The preventative medications were under-utilized in all three groups. Moreover, individuals in the DM group reported higher frequencies of analgesic use (P < 0.001). The girls with DM experienced higher degrees of headache related disability as measured by HIT-6 (P < 0.0001). PM appears to be at least as prevalent of DM in our cohort of teenage girls. We propose that PM may produce a milder phenotype indicated by significantly lower HIT-6 scores. Show more

Keywords: Probable migraine, definite migraine, headache impact test 6, tension type headaches

DOI: 10.3233/JPN-130617

Citation: Journal of Pediatric Neurology, vol. 11, no. 3, pp. 171-175, 2013

Authors: Chauhan, Lokesh | Rana, Kamer S. | Sharma, Monica

Article Type: Research Article

Abstract: Comparison of internal anal sphincter pressures in spastic cerebral palsy (CP) children with constipation and without constipation. Our study included 65 spastic CP children between 1–14 yr of age. Children having multiple congenital anomalies and with other causes of constipation were excluded. Basal anorectal pressures at the internal anal sphincter at rest were recorded. Majority (67.7%) of cases were in the age group of 1–5 yr. Majority (50.7%) were spastic quadriplegic. Perinatal risk factors accounted for …86.2% of the cases, birth asphyxia being the commonest (67.9%). Seizures were significantly high (P=0.019) in children with constipation. The mean of maximum, (P < 0.002), mean of minimum (P < 0.005) and the mean of mean anorectal pressures (P < 0.002) were significantly high in spastic children with constipation. Increased anorectal pressures may be an important cause of constipation in children with spastic CP and anorectal manometry should be carried out in these children to rule out increased pressures at the anorectal level. Show more

Keywords: Spastic cerebral palsy, anorectal pressure, anorectal manometry

DOI: 10.3233/JPN-130619

Citation: Journal of Pediatric Neurology, vol. 11, no. 3, pp. 177-181, 2013

Authors: Kashyape, Pawan S. | Arunachalam, Ramamurthy | Kennedy, Colin R.

Article Type: Research Article

Abstract: We report a case of a previously unreported complication of an intracranial Ommaya reservoir catheter causing par-oxysmal episodes of bradycardia-asystole and syncope as a result of migration of the catheter tip into the left temporal lobe. These episodes were captured on ambulatory electroencephalography as syncopal events, but with interictal left temporal epi-leptiform discharges. These events promptly resolved after repositioning the catheter. There was a recurrence of these events 1 yr later, associated …with remigration of the catheter tip into the left temporal lobe, necessitating removal of the Ommaya reservoir, rendering the patient symptom free. This is the first reported case of an iatrogenic and possibly ictal bradycardia-asystole syn-drome and underpins the importance of considering migration of indwelling intracranial catheters into sensitive regions of the cortex as a possible cause of paroxysmal episodes. Show more

Keywords: Intracranial catheter, iatrogenic, bradycardia-asystole

DOI: 10.3233/JPN-130614

Citation: Journal of Pediatric Neurology, vol. 11, no. 3, pp. 183-187, 2013

Authors: de Souza, Rodrigo Becco | de Aguiar, Guilherme Brasileiro | Daniel, Jefferson Walter | Conti, Mário Luiz Marques | Veiga, José Carlos Esteves

Article Type: Research Article

Abstract: Pourfour du Petit syndrome, or reverse Horner syndrome, is described as an overactive sympathetic nervous system, being characterized by mydriasis, eyelid retraction, and hyperhidrosis. We described a case of Pourfour du Petit syndrome after cervical injury by gunshot, with a little review about this rare syndrome. Angiography revealed dissection and formation of pseudo aneurysm of the left carotid artery. We believe that this lesion caused hyper-stimulation of the left cervical sympathetic chain, resulting in reverse Horner …syndrome or Pourfour du Petit syndrome. There was reversal of symptoms spontaneously after 3 wk. Show more

Keywords: Horner syndrome, autonomic nervous system, Pourfour du Petit syndrome, carotid artery dissection

DOI: 10.3233/JPN-130615

Citation: Journal of Pediatric Neurology, vol. 11, no. 3, pp. 189-191, 2013

Authors: Choudhary, Sandeep | Alam, Anwer | Sachan, Deepak | Bijarnia, Ritu

Article Type: Research Article

Abstract: Metachromatic leukodystrophy (MLD) is a progressive white matter disease caused by arylsulfatase A deficiency. Demyelination in the nervous system is detected by cerebral magnetic resonance imaging and neurophysiological studies. We present here a child with late infantile MLD, who had initially difficulties in standing and walking with generalized dystonia and clubfoot and later on became unable to stand and sit even with support. Protein levels in cerebral spinal fluid were elevated and nerve …conduction studies revealed slowing down of motor and sensory nerve conduction velocity. Initial cerebral computerized tomography scan showed hypo dense lesion in bilateral globus pallidus. Magnetic resonance imaging was done subsequently that showed diffuse white matter signal changes. Enzyme assay for arylsulfatase A further confirmed the diagnosis of MLD. We conclude that in young children extrapyramidal symptomatology and demyelinating sensorimotor polyneuropathy may be a presenting feature of the onset of MLD. Show more

Keywords: Metachromatic leukodystrophy, arylsulfatase A, neurodegenerative disorder

DOI: 10.3233/JPN-130620

Citation: Journal of Pediatric Neurology, vol. 11, no. 3, pp. 193-196, 2013

Authors: Das, Suman | Mazumdar, Jayitri | Chatterjee, Kaushani | Sarkar, Nirmalya | Aich, Bholanath

Article Type: Research Article

Abstract: Holoprosencephaly (HPE) sequence is a rare spectrum of cerebral and facial malformations resulting from incomplete division of embryonic prosencephalon into diencephalon and telencephalon. De Meyer classified three ranges in order of increasing severity: lobar, semilobar and alobar HPE. A subtype called middle inter-hemispheric fissure variant has also been described. Arnold-Chiari III malformation is extremely rare and comprises of occipital encephalocele and associated cerebellar and brain stem abnormalities. This case report presents a …male infant with lobar HPE and Arnold-Chiari III malformation without any facial defects and chromosomal abnormalities. Show more

Keywords: Holoprosencephaly, Arnold-Chiari III malformation, agenesis of corpus callosum, occipital encephalocele

DOI: 10.3233/JPN-130618

Citation: Journal of Pediatric Neurology, vol. 11, no. 3, pp. 197-200, 2013