Journal of Pediatric Neurology - Volume 12, issue 4

Authors: El-Ansary, Afaf | Hassan, Sohair A. | Anwar, Mona | Shmais, Ghada Abu | Bhat, Ramesa Shafi | Hashish, Adel | Khalil, Rehab O. | Al-Ayadhi, Laila | Meguid, Nagwa A.

Article Type: Research Article

Abstract: Autism spectrum disorders are complex developmental disorders with increasing incidence and poorly understood etiology. Imbalance of amino acids profoundly influences brain function, and is thought to be one of the key players in the pathophysiology of autism. This study aimed to measure the plasma amino acid profiles of 20 Egyptian and 20 Saudi autistic patients in comparison to matching healthy controls to clarify the role of impaired amino acid concentrations in the etiology of autism. Plasma …amino acids profiles were measured using high performance liquid chromatography. While plasma levels of glutamic, aspartic, and glycine recorded the most significant percentage elevated amino acids, glutamine, asparagine, arginine, tyrosine and isoleucine recorded the most remarkable percentage decrease in autistic patients from both populations compared to controls. Among the calculated relative values, only acidic/basic, and glutamate/glutamine ratios were significantly higher in autistics compared to controls. Non-essential/essential and glucogenic/ketogenic ratios were unaltered in autistics compared to controls. Increased plasma glutamate/glutamine ratio, together with increased glycine, arginine, aspartate, aspargine levels, and acidic/basic amino acid ratio can serve as a predictive tools for the early detection of autism. These findings suggest that glutamatergic abnormalities in the brain may be associated with the pathobiology of autism. Show more

Keywords: Autism, amino acids profile, glutamate, glutamine

DOI: 10.3233/JPN-140660

Citation: Journal of Pediatric Neurology, vol. 12, no. 4, pp. 171-181, 2014

Authors: Sisman, Julide | Logan, J. Wells | Westra, Sjirk J. | Allred, Elizabeth N. | Leviton, Alan

Article Type: Research Article

Abstract: Although lenticulostriate vasculopathy (LSV) was first detected on a cranial ultrasound nearly 30 years ago, its clinical implications and significance remain unknown. The objective of this study was to evaluate the inter-rater reliability of cranial ultrasound readings of LSV, and to explore relationships with potential antecedents and developmental correlates in extremely low gestational age newborns. Of the 1506 infants enrolled during the years 2002–2004, 1450 had at least one set of ultrasound scans evaluated …for LSV and 939 had all three sets. To evaluate the inter-rater agreement for identifying LSV, we compared readings from two independent radiologists on days 1–4, 5–14, and on or after day 15. We then evaluated the relationships between LSV and maternal, antenatal, and postnatal characteristics. Our results showed that kappa values were 0.18, 0.33, and 0.36 on days 1–4, days 5–14, and day 15 or greater. Infants who were identified as LSV positive by two readers had higher Score for Neonatal Acute Physiology-II (an illness severity indicator), higher rates of tracheal infection and bacteremia, lower partial pressure of arterial oxygen and pH levels on 2 of the first 3 postnatal days, and they were more likely to have a lower psychomotor development index at age 2 years. Positive agreement on the presence of LSV was low, as was the kappa value, an index of inter-rater reliability. Infants with high illness severity scores and their correlates were at increased risk of developing LSV, while those who develop LSV appear to be at increased risk of motor dysfunction. Show more

Keywords: Lenticulostriate vasculopathy, cranial ultrasound, thalamus, basal ganglia

DOI: 10.3233/JPN-140661

Citation: Journal of Pediatric Neurology, vol. 12, no. 4, pp. 183-193, 2014

Authors: Kuti, Bankole Peter | Oseni, Saheed Babajide | Owa, Joshua Aderinsola

Article Type: Research Article

Abstract: The occurrence of seizures among ill newborns has been reported to increase morbidity and mortality. Recognition and prompt management/avoidance of risk factors associated with the occurrence of seizures in the newborn may improve survival. This study aims to determine the risk factors for seizures among admitted babies at the Wesley Guild hospital, Ilesa. Consecutive neonatal admissions over a 9 month period were prospectively observed for clinical seizures. Perinatal and neonatal factors were compared between babies with …and without seizures. Multiple logistic regression analysis was used to determine the independent predictors of the occurrence of seizures among the babies. Fifty-five (16.2%) of the 340 neonates admitted during the study period had clinical seizures. Male to female ratio of babies with seizures was 2.9:1, and 67.2% were admitted within 72 h of birth. Male sex (odds ratio [OR] 2.256; 95% confidence interval [CI] 1.146–7.167; P=0.024), parental low socioeconomic class (OR=2.211; 95% CI=1.427–9.281; P=0.007), delivery outside the hospital, particularly in mission homes (OR=4.173; 95% CI=1.590–14.040; P=0.005), and maternity homes (OR=2.005; 95% CI=1.183–9.692; P=0.003) were the independent risk factors associated with seizures among the babies. Delivery in private maternity and mission homes coupled with parental low socioeconomic class are major risk factors for seizures among admitted sick babies at the Wesley Guild hospital, Ilesa. Making delivery in standard health centers easily accessible and affordable may help reduce this burden. Show more

Keywords: Convulsions, ill newborns, predictors, risk factors

DOI: 10.3233/JPN-140662

Citation: Journal of Pediatric Neurology, vol. 12, no. 4, pp. 195-202, 2014

Authors: Dooley, Joseph M. | Chiasson, Emma K.

Article Type: Research Article

Abstract: Boys with Duchenne muscular dystrophy typically lose the ability to walk independently between the ages of 10 and 12 yr, although this may be prolonged with steroid therapy. The loss of ambulation primarily reflects progressive weakness, although other factors may play a role. We describe a boy with Duchenne muscular dystrophy whose ability to walk was jeopardized at age 10 yr. He had contractures of both Achilles tendons and tight anterior compartments in both legs with …discomfort over the anterior compartments bilaterally. Following tendon lengthening and fasciotomy of the anterior compartments his gait, level of functioning and discomfort greatly improved. He continues to ambulate for short distances without assistance at 18 years of age. Anterior compartment syndrome should be considered in boys with Duchenne dystrophy, especially if they have leg pain and are losing the ability to walk earlier than anticipated. Show more

Keywords: Duchenne, muscular dystrophy, compartment, fasciotomy

DOI: 10.3233/JPN-140663

Citation: Journal of Pediatric Neurology, vol. 12, no. 4, pp. 203-205, 2014

Authors: Campos, Jos\'e Guevara | González-Guevara, Lucía | González, José Guevara

Article Type: Research Article

Abstract: The clinical significance of occipital intermittent rhythmic delta activity (OIRDA) on the electroencephalogram (EEG) has not been fully established. Over the years, it has been suggested that the interpretation of the OIRDA electroencephalographic pattern may be related to metabolic problems, structural lesions, infections and epilepsy. Recent studies suggested that this pattern occurs almost exclusively in children and is probably of epileptic origin in most case. It has been associated with primary generalized epilepsy …syndromes, such as childhood absence. An 8-year-old schoolgirl, attending the 3rd grade, with easy distractibility, inattention, low school performance,and microcephaly is described. EEG was performed awake during hyperventilation revealed occipital intermittent rhythmic delta with a frequency of 3 Hz, bilateral and synchronous, with 2nd and 4th sec of duration. Antiepileptic therapy with valproic acid was begun. After being treated for 8 months, no OIRDA was detected on follow up EEG. The presence of OIRDA activity on EEG in a child should alert us to the presence of absence epilepsy. Show more

Keywords: Occipital rhythmic, absences, electroencephalogram, epilepsy, delta activity

DOI: 10.3233/JPN-140664

Citation: Journal of Pediatric Neurology, vol. 12, no. 4, pp. 207-211, 2014

Authors: Güngör, Serdal | Arslan, Müjgan | Karakurt, Cemşit | Raif, Sabiha Güng\"or

Article Type: Research Article

Abstract: Ventriculoperitoneal and ventriculoatrial shunting methods are used for the surgical treatment of hydrocephalus. However, many years or months after the treatment, complications can develop. In this article, we report on a patient with perforation of the tricuspid valve and tricuspid insufficiency, which developed as a complication of the ventriculoatrial shunt. This is a previously unreported complication in pediatric patients with a ventriculoatrial shunt. Because of fairly common and life-threatening cardiac complications, ventriculoatrial …shunted patients should be monitored closely by echocardiography. Show more

Keywords: Child, perforation of tricuspid valve, ventriculoatrial shunt

DOI: 10.3233/JPN-140665

Citation: Journal of Pediatric Neurology, vol. 12, no. 4, pp. 213-215, 2014

Authors: Dell'Orto, Valentina | Bernasconi, Enos | Pezzoli, Valdo | Queirolo, Sara | Ramelli, Gian Paolo

Article Type: Research Article

Abstract: A 14-year-old girl diagnosed with Epstein-Barr Virus encephalitis with generalized status epilepticus and cardiac arrest. The pathogenic role of Epstein-Barr Virus was identified by a polymerase chain reaction analysis of the patient's cerebrospinal fluid. Cranial computerized tomography and magnetic resonance imaging revealed no abnormalities. After being admitted to the intensive care unit, the patient exhibited progressive improvement until complete recovery from the disease. Despite the severity of the disease, the patient did …not experience long-term sequelae. Show more

Keywords: Epstein-Barr Virus, encephalitis, status epilepticus

DOI: 10.3233/JPN-140666

Citation: Journal of Pediatric Neurology, vol. 12, no. 4, pp. 217-219, 2014

Authors: Yelsangikar, Kalpana Ramesh | Srikanteswara, Praveen-kumar

Article Type: Research Article

Abstract: Kikuchi-Fujimoto disease (KFD) is a benign and self-limiting condition primarily affecting the cervical lymph nodes. Central nervous system involvement with acute cerebellar symptoms is a rare presentation in KFD. We report a 15-year-old girl presenting with intentional tremor, dysarthria and gait ataxia following episodes of recurrent fever and cervical lymphadenopathy. The diagnosis of KFD was made based on histopathology. Brain magnetic resonance imaging showed lesions in mid-pons and bilateral middle cerebellar peduncles. …The patient's symptoms spontaneously resolved without any sequelae. A diagnosis of KFD should be considered when enlarged cervical lymphnodes are observed in patients with acute cerebellar symptoms. Selective inflammation of cerebellum and related structures by viral or immunological responses may be attributed to this rare presentation in KFD. Show more

Keywords: Hemophagocytosis, cervical lymphadenopathy, cerebellar ataxia

DOI: 10.3233/JPN-140667

Citation: Journal of Pediatric Neurology, vol. 12, no. 4, pp. 221-224, 2014

Authors: Chakrabarty, Biswaroop | Singh, Manoj | Yoganathan, Sangeetha | Kumar, Atin | Gulati, Sheffali

Article Type: Research Article

DOI: 10.3233/JPN-140668

Citation: Journal of Pediatric Neurology, vol. 12, no. 4, pp. 225-226, 2014

Article Type: Other

Citation: Journal of Pediatric Neurology, vol. 12, no. 4, pp. 227-230, 2014