Journal of Pediatric Neurology - Volume 12, issue 1

Authors: Ansari, Zarrin A. H.

Article Type: Research Article

Abstract: Age-specific distribution of epilepsy is bimodal, with the highest incidence being in childhood. Seizures can affect life as early as day one. There is a paramount variation of epilepsy in childhood and adolesence with regards to its etiology and prognosis. Antiepileptic drugs, being the mainstay of treatment, necessitates that their use in this vulnerable age group must be made after a thorough look into the available evidence. The data on levetiracetam (LEV), a second-generation antiepileptic, spans …from randomized controlled trials to case reports in various pediatric epilepsy and epileptic syndromes. This review is a compilation and critical analysis of the data on the differences in the pharmacokinetic properties of LEV in children versus adults, drug-interactions, efficacy of LEV in various contexts, namely, as add-on, as monotherapy, in partial and generalized seizures, in specific epilepsy syndromes such as benign epilepsy with centrotemporal spikes, epileptic syndromes with continuous spike and wave during sleep, Lennox-Gastaut syndrome, Jeavons syndrome and epilepsy associated with tuberous sclerosis. LEV is an effective option for the treatment of partial-onset seizures as both an add-on in refractory epilepsy and monotherapy in newly diagnosed focal epilepsies. There are preliminary but promising data available on various epileptic syndromes and epileptic encephalopathies that can nudge researchers towards further work in these areas in order to generate evidences that is more substantial. The safety and tolerability profile of LEV is favorable, though, behavioral and neuropsychiatric adverse events are reportedly higher in children versus adults. The neuropsychiatric adverse events are often observed against the background of previously existing behavioral issues and neurocognitive impairments. Show more

Keywords: Levetiracetam, pediatrics, safety, efficacy, pharmacokinetics, epilepsy syndromes, myoclonic, absence, refractory epilepsy

DOI: 10.3233/JPN-140633

Citation: Journal of Pediatric Neurology, vol. 12, no. 1, pp. 1-13, 2014

Authors: Tedla, Jaya Shanker

Article Type: Research Article

Abstract: The purpose of this study was to evaluate the effect of trunk and lower extremity strength-training exercises on muscle strength, balance and motor function in 5 to 14 yr old subjects with spastic diplegia cerebral palsy. In this randomized controlled trial 60 subjects with spastic diplegia were randomly allocated either to the experimental group or to the control group. The experimental group received strength-training in a circuit of workstations with principles of progressive resisted exercises. For …the control group only conventional physical therapy treatment was provided. Outcome measures were strength, balance and motor function. Paired t test was used to evaluate the differences before and after the interventions for strength, balance (pediatric balance scale), and function (gross motor functional measure). The Mann-Whitney U test was used to evaluate the effects between the experimental and the control groups due to intervention in strength, balance (pediatric balance scale), and function (gross motor functional measure). Outcome measures improved in both the groups due to intervention, but the improvements in the experimental group were better than the control group. We conclude that 6 wk of strength-training of the trunk and the lower extremity muscles along with conventional physiotherapy, or conventional physiotherapy alone is effective in improving overall strength, balance and function of the subjects with spastic diplegia studied. But strength-training is found to be of greater benefit than conventional physical therapy. Show more

Keywords: Cerebral palsy, spastic diplegia, strength training, balance, motor function

DOI: 10.3233/JPN-140634

Citation: Journal of Pediatric Neurology, vol. 12, no. 1, pp. 15-28, 2014

Authors: Kaytser, Victor | Berry-Kravis, Elizabeth | Ouyang, Bichun | Doll, Emily | Hall, Deborah A.

Article Type: Research Article

Abstract: The purpose of this study was to quantify the type and frequency of stereotypies in fragile X syndrome and to determine the relationship of observed stereotypies with gender, level of cognitive function, and parent-reported scores for repetitive behaviors. Subjects with fragile X syndrome were videotaped during the administration of a standardized conversation task, a standardized narrative task, and sitting alone. Two individuals rated the stereotypies on the videotapes (10 min each) using the …stereotyped behavior scale. Intelligence quotient scores were collected and parents/guardians completed the Vineland adaptive behavior scale and the aberrant behavior checklist – community edition. Thirty-eight subjects were tested (mean age 17.1 ± 7.1 yr). The mean total of stereotypies was 11 ± 11.7 movements in 10 min and the mean stereotyped behavior scale score was 5.8 ± 5.5. The most common type of movement was rubbing him/herself, followed by body movement and repetitive movement. There were correlations between the stereotyped behavior scale score and intelligence quotient (P=0.0006), the aberrant behavior checklist total score (P=0.01), and the stereotypy subscore (P=0.0007). Stereotypies are common in fragile X syndrome and more frequent in individuals with lower intelligence quotients and more severe behavior. The stereotyped behavior scale measure correlated with parent completed checklists, but showed poor to moderate reliability for measurement of stereotypies by a single rater and test retest in fragile X syndrome. Show more

Keywords: Fragile X syndrome, stereotypies, FMR1, autistic disorder

DOI: 10.3233/JPN-140635

Citation: Journal of Pediatric Neurology, vol. 12, no. 1, pp. 29-34, 2014

Authors: Gowda, Vykuntaraju K.N. | Bannigidad, Ningappa B. | Kumar, Pragalath | Srikanteswara, Praveen-Kumar | Shivananda, | Govindraj, | Vykuntaraju, Sarala H. | Ramaswamy, Premalatha

Article Type: Research Article

Abstract: Objective: To study the etiology and clinical profile of non-traumatic coma in children at tertiary care center and to determine the predictors of outcome. Methods: One hundred and four consecutive children between 2 mo-12 yr were studied. The clinical signs at admission; vital signs, Glasgow coma scale, respiratory pattern, papillary reflex, extra-ocular movements, fundus picture and motor deficits were recorded. Etiology of coma was determined by clinical history, examination and relevant investigations. …Their progress was monitored clinically, biochemically and with multi-system monitors. Outcome was recorded as survived or died. Results: Etiology of coma in 65% cases was intracranial infections; other causes were metabolic (20%). Sixty-seven percent recovered completely, 16% had residual neurodeficits, 16% died. Survival was better in children with intracranial infections (13%) as compared to metabolic coma (33%). On multivariate logistic regression, bradycardia, hypotension, abnormal respiratory pattern (especially, ataxic type), duration of coma more than 48 h, Glasgow coma scale < 7 at admission, unequal and non-reactive pupils, papilledema, abnormal extra-ocular movements, motor deficits, signs of meningitis correlated with mortality. Requirement of ventilatory support and abnormal computerized tomography findings correlated with mortality. Conclusions: Intracranial infections were the most common cause of non-traumatic coma in children; the most common cause of death being metabolic coma. Simple clinical signs and relevant investigations served as prognostic indicators of outcome. Show more

Keywords: Coma, childhood, Glasgow coma scale, intracranial infections, non-traumatic coma

DOI: 10.3233/JPN-140636

Citation: Journal of Pediatric Neurology, vol. 12, no. 1, pp. 35-43, 2014

Authors: Alp, Hayrullah | Baysal, Tamer | Karaarslan, Sevim | Alpınar, Abdullah | Çaksen, Hüseyin

Article Type: Research Article

Abstract: Rhabdomyomas, the most common primary cardiac tumors seen in infants and children, are often associated with tuberous sclerosis. However, these cardiac rhabdomyomas may cause hemodynamic disturbances and arrhythmias. In the present case, we demonstrated an infant with electrocardiographic ST elevation suggesting myocardial ischemia associated with multiple cardiac rhabdomyomas.

Keywords: Rhabdomyoma, tuberous sclerosis, myocardial ischemia, mosque sign, ST segment elevation, infant

DOI: 10.3233/JPN-140637

Citation: Journal of Pediatric Neurology, vol. 12, no. 1, pp. 45-47, 2014

Authors: van Hoeve, Karen | Vanrykel, Katrien

Article Type: Research Article

Abstract: We report a 15-year-old girl with intractable hiccups unresponsive to high doses of proton pump inhibitors. Further investigations revealed an acute demyelinating neurological disorder on magnetic resonance imaging of the brain with a lesion in the brain stem responsible for the hiccups. Hiccups are a common and usually transient condition. It is rare for it to become intractable. Persistent hiccups may reflect a serious underlying disease, as in our case due to a demyelinating disorder. We …would like to highlight the underlying causes of an acute demyelinating neurological disorder and the characteristics to help with the differential diagnosis. Show more

Keywords: Acute demyelinating neurological disorder, acute disseminated encephalomyelitis, hiccups, clinically isolated syndrome

DOI: 10.3233/JPN-140638

Citation: Journal of Pediatric Neurology, vol. 12, no. 1, pp. 49-53, 2014

Authors: Gowda, Vykuntaraju K. | Reddy, Bharath | Reddy, Hamsa | Shivappa, Sanjay K. | Ramaswamy, Premalatha | Gowda, Sarala H.S. | Madivala, Bhaskar V.

Article Type: Research Article

Abstract: Idiopathic intracranial hypertension is rare in children and associated with high cerebrospinal fluid pressure, with no evidence of intracranial malformation. The etiology is unknown but it is associated with endocrine disorders, drugs, toxins and viral infections. Investigations such as lumbar puncture and imaging are usually normal. We report a child who presented with features of elevated intracranial pressure following measles vaccination. His investigations were all found to be normal and was treated with …acetazolamide following which he recovered. Show more

Keywords: Idiopathic intracranial hypertension, measles vaccine, pseudotumor cerebri

DOI: 10.3233/JPN-140639

Citation: Journal of Pediatric Neurology, vol. 12, no. 1, pp. 55-58, 2014

Authors: Vergano, Samantha A. | Chisholm, Elizabeth

Article Type: Research Article

Abstract: Glucose transporter deficiency syndrome type 1 (Glut-1 DS) is a rare disease of abnormal glucose transport. Diagnosis is most often made in infancy with the presentation of epileptic seizures, gross motor delays, and microcephaly. We report a case of a 17-year-old male seen in our outpatient clinic with a life-long history of developmental delays and seizures. He began having apneic episodes with limb jerking at approximately 4 months of age. Several antiepileptics were tried before the …frequency of seizures decreased in early adolescence. Developmental delays became apparent in infancy. The proband's chief complaint at the time of presentation was abrupt episodes of confusion, fatigue, and ataxia, with body stiffening, occurring monthly, sometimes weekly, with minimal recollection of the event. At the time of his current evaluation, a review of laboratory findings revealed a low cerebrospinal fluid glucose level obtained during an emergency room evaluation for a headache several years prior. This finding raised concern for Glut-1 DS. Subsequent molecular analysis confirmed the diagnosis. While rare, this case emphasizes the importance of considering Glut-1 DS in the differential of seizures and cognitive delays, even in older children. Treatment with a ketogenic diet, despite at a later age of introduction, may still be effective in ameliorating symptoms. Show more

Keywords: Glucose-1-transporter deficiency, hypoglycorrhachia, seizures, developmental delay

DOI: 10.3233/JPN-140640

Citation: Journal of Pediatric Neurology, vol. 12, no. 1, pp. 59-61, 2014

Authors: Çelik, Tanju | Ünalp, Aycan | Berksoy, Emel Ataş | Yılmaz, Ünsal | Akıncı, Gülçin | Güzel, Orkide | Oztürk, Aysel

Article Type: Research Article

Abstract: Vitamin B12 deficiency may cause a wide variety of signs and symptoms such as macrocytic anemia, neurodevelopmental delay or regression, cerebral atrophy, polyneuropathy, paresthesias, degeneration of spinal cord, ataxia, irritability, weakness, hypotonia, apathy, tremor, seizures, and neuropsychiatric disorders such as schizophrenia and dementia. However, its association with epileptic spasms has been reported in only one patient before. Thus, the role of coincidence or association remains controversial. This article presents a 10-month-old girl …with B12 deficiency presenting with epileptic spasms, psychomotor regression, and suppression-burst pattern on electroencephalography. After exclusion of other causes of epileptic spasms such as hypoxic ischemic encephalopathy, cortical malformations, or neurodegenerative diseases through extensive neuroimaging and laboratory investigations, epileptic spasms were regarded as the presenting clinical picture of underlying vitamin B12 deficiency. Epileptic spasms may be a neurologic manifestation of underlying vitamin B12 deficiency. Show more

Keywords: Epileptic spasm, vitamin B12 deficiency

DOI: 10.3233/JPN-140641

Citation: Journal of Pediatric Neurology, vol. 12, no. 1, pp. 63-66, 2014

Article Type: Other

DOI: 10.3233/JPN-140632

Citation: Journal of Pediatric Neurology, vol. 12, no. 1, pp. 67-68, 2014