Journal of Child Neurology current issue

Methylphenidate in Children With Oppositional Defiant Disorder and Both Comorbid Chronic Multiple Tic Disorder and ADHD

Gadow, K. D., Nolan, E. E., Sverd, J., Sprafkin, J., Schneider, J. — 2008-09-30

Our primary objective was to determine if immediate-release methylphenidate is an effective treatment for oppositional defiant disorder diagnosed from mother's report in children with both chronic multiple tic disorder and attention-deficit hyperactivity disorder (ADHD). Children (n = 31) aged 6 to 12 years received placebo and 3 doses of methylphenidate twice daily for 2 weeks each under double-blind conditions and were assessed with ratings scales and laboratory measures. Results indicated significant improvement in both oppositional and ADHD behaviors with medication; however, the magnitude of treatment effect varied considerably as a function of disorder (ADHD > Oppositional behaviors), informant (teacher > mother), assessment instrument, and specific oppositional behavior (rebellious > disobeys rules). Drug response was comparable with that in children (n = 26) who did not have diagnosed oppositional defiant disorder, but comorbidity appeared to alter the perceived benefits for ADHD according to mother's report. Methylphenidate is an effective short-term treatment for oppositional behavior in children with comorbid ADHD and chronic multiple tic disorder.

Quality of Life and Seizure Outcome After Vagus Nerve Stimulation in Children With Intractable Epilepsy

2008-09-30

This study examined the effect of vagus nerve stimulation on quality of life in children with epilepsy using a validated quality-of-life scale and an empirical technique that accounts for measurement error in assessing individual change (the reliable change index). Participants were 34 children with severe intractable epilepsy who underwent vagus nerve stimulation and 19 children with intractable epilepsy who received medical management. Parent-completed epilepsy-specific and global ratings at baseline and after 1 year indicated that most children had no changes in quality of life following vagus nerve stimulation (52%-77%), similar to the comparison group. There was a trend for decreases to be less common in the vagus nerve stimulation group (14% vs 37%, P < .07), but there was no relation between improved quality of life and seizure control. The results raise questions about the mechanisms that underlie changes in quality of life after vagus nerve stimulation in this group of children.

Spinocerebellar Ataxia Type 2 Presenting With Cognitive Regression in Childhood

Ramocki, M. B., Chapieski, L., McDonald, R. O., Fernandez, F., Malphrus, A. D. — 2008-09-30

Spinocerebellar ataxia type 2 typically presents in adulthood with progressive ataxia, dysarthria, tremor, and slow saccadic eye movements. Childhood-onset spinocerebellar ataxia type 2 is rare, and only the infantile-onset form has been well characterized clinically. This article describes a girl who met all developmental milestones until age 31/2 years, when she experienced cognitive regression that preceded motor regression by 6 months. A diagnosis of spinocerebellar ataxia type 2 was delayed until she presented to the emergency department at age 7 years. This report documents the results of her neuropsychologic evaluation at both time points. This case broadens the spectrum of spinocerebellar ataxia type 2 presentation in childhood, highlights the importance of considering a spinocerebellar ataxia in a child who presents with cognitive regression only, and extends currently available clinical information to help clinicians discuss the prognosis in childhood spinocerebellar ataxia type 2.

Impact of Neurofibromatosis Type 1 on School Performance

2008-09-30

School functioning of 86 Dutch neurofibromatosis type 1 children (7-17 years) using teacher questionnaires was analyzed to determine the impact of neurofibromatosis type 1 on school performance. In all, 75% of the neurofibromatosis type 1 children performed more than 1 standard deviation below grade peers in at least one of the domains of spelling, mathematics, technical reading or comprehensive reading. Furthermore, neurofibromatosis type 1 children had a 4-fold increased risk for attending special education and a 6-fold increased risk for receiving remedial teaching for learning, behavior, speech, or motor problems. Children without apparent learning disabilities still frequently displayed neuropsychological deficits. Only 10% of the children did not show any school-functioning problems. Finally, it was found that the clinical severity of neurofibromatosis type 1 correlated with the cognitive deficits. Taken together, it was shown that neurofibromatosis type 1 has profound impact on school performance. Awareness of these problems may facilitate timely recognition and appropriate support.

Infected Intraparenchymal Dermoids: An Underestimated Entity

2008-09-30

Infection secondary to a dermal sinus most commonly occurs in the form of cutaneous, epidural, or subdural abscesses. Rarely, it can result in an intramedullary abscess as a result of a dermal sinus. This study presents a clinicoradiological profile of 19 cases harboring abscesses within the dermoids and highlights the importance of dermal sinus acting as a pathway for infections to enter the nervous system. Emergent exploration, pus drainage, and minimal abscess wall excision along with prolonged antibiotic administration remained the management of choice in all cases. Methicillin-sensitive Staphylococcus aureus was the commonest offending organism. In all, 7 patients recovered to normal neurological status, 5 showed no improvement, and 7 improved partially. Improvement in motor power was noted, albeit partially, but bladder functions failed to recover even at long-term follow-up. Even when such infective complications of dermal sinuses are rare, these are potentially serious and disabling.

Validation of the Presence of Comorbidities in a Danish Clinical Cohort of Children With Tourette Syndrome

Mol Debes, N. M. M., Hjalgrim, H., Skov, L. — 2008-09-30

Tourette syndrome (TS) is characterized by the presence of motor and vocal tics and is often accompanied by comorbid symptoms. We assessed the frequency of the comorbid symptoms obsessive-compulsive disorder obsessive-compulsive disorder, attention-deficit hyperactivity disorder (ADHD), rage attacks, sleeping disturbances, and depressive symptoms in a Danish clinical cohort of 314 children with TS using validated diagnostic instruments. For the assessment of symptoms of seasonal affective disorder and stuttering, we used a nonvalidated systematic interview. In total, only 10.2% of the children did not have any comorbid symptoms at all. If ADHD and/or obsessive-compulsive disorder were present, the rates of the comorbidities rage, symptoms of seasonal affective disorder, sleep disturbances, and depressive symptoms were significantly higher than if ADHD and/or obsessive-compulsive disorder were absent. The most severe tics were found in the group for which both ADHD and obsessive-compulsive disorder were present. Furthermore, there was a tendency toward more severe tics if other comorbid symptoms were present.

Neurologic Manifestations of Pediatric Chikungunya Infection

2008-09-30

Chikungunya virus, a mosquito-borne arbovirus, was responsible for a massive epidemic in La Réunion Island during 2005 to 2006. The disease is usually benign, but neurologic involvement, with sometimes fatal outcome, has been described. We report a retrospective hospital-based pediatric series of 30 children (23 boys and 7 girls) who presented neurologic manifestations of chikungunya such as encephalitis (n = 12), febrile seizures (n = 10), meningeal syndrome (n = 4), and acute encephalopathy (n = 4). Cerebrospinal fluid biological and cytological analyses (n = 23) were unremarkable except for 1 case of acute disseminated encephalomyelitis. The presence of viral genome in cerebrospinal fluid was inconstantly positive. Brain magnetic resonance imaging (MRI) scans (n = 14) were abnormal in 5 cases. Electroencephalography was nonspecific. Two patients died. At discharge and 6 months later, 5 children had neurologic sequelae. Patients with initial severe neurologic presentation and having pathological brain MRI had more sequelae or fatal disease.

Attitudes Toward Attention-Deficit Hyperactivity Disorder (ADHD) Treatment: Parents' and Children's Perspectives

Berger, I., Dor, T., Nevo, Y., Goldzweig, G. — 2008-09-30

Attitudes toward pharmacological treatment may be a major factor contributing to adherence to such treatment. In the current study, attitudes toward methylphenidate treatment among 50 children diagnosed with attention-deficit hyperactivity disorder (ADHD) and their parents were assessed. Authors of this study have found that the study population is concerned and suspicious toward methylphenidate treatment. Most participants were exposed to negative information even before treatment initiation, which caused many participants to consult other sources and postpone the treatment initiation. Although experiencing methylphenidate as safe and effective (after 23.5 months of treatment), the leading cause of negative attitudes is the concern regarding long-term effects. The single most effective factor regarding the attitude toward methylphenidate treatment is the neurologist's explanation. It is concluded that the pediatric neurologist has a crucial role in affecting attitudes of children and parents toward methylphenidate treatment.

Profound Biotinidase Deficiency in a Child With Predominantly Spinal Cord Disease

Chedrawi, A. K., Ali, A., Al Hassnan, Z. N., Faiyaz-Ul-Haque, M., Wolf, B. — 2008-09-30

Biotinidase deficiency is an autosomal recessively inherited disorder that manifests during childhood with various cutaneous and neurological symptoms particularly seizures, hypotonia, and developmental delay. Spinal cord disease has been reported rarely. We describe a 3-year-old boy with profound biotinidase deficiency who presented with progressive spastic paraparesis and ascending weakness in the absence of the usual characteristic neurological manifestations. Supplementation with biotin resulted in resolution of paraparesis with persistent mild spasticity in the lower limbs. DNA mutation analysis revealed that he was homozygous for a novel missense mutation (C>T1339;H447Y) in the BTD gene. This case indicates that biotinidase deficiency should be included in the differential diagnosis of subacute myelopathy and emphasizes the importance of a prompt diagnosis to prevent irreversible neurological damage.

Use of Intravenous Tissue Plasminogen Activator in a 16-Year-Old Patient with Basilar Occlusion

Heil, J. W., Malinowski, L., Rinderknecht, A., Broderick, J. P., Franz, D. — 2008-09-30

Intravenous tissue plasminogen activator (t-PA) is currently approved by the US Food and Drug Administration (FDA) for the treatment of ischemic stroke in patients > 18 years of age who present within 3 hours of stroke onset and meet certain criteria. We report a case of a 16-year-old, previously healthy female who presented with a basilar artery occlusion and pontine ischemic stroke. She was treated with intravenous t-PA approximately 4 hours after the onset of symptoms. The patient demonstrated a remarkable recovery 6 hours after onset of her symptoms and had minimal deficits on discharge from the hospital 1 week later. She was found to have a lupus anticoagulant and was heterozygous for the prothrombin gene G2010A mutation. These were likely contributing causes for her stroke. She was also homozygous for plasminogen activator inhibitor 1 (PAI-1) 4G/4G, which at present is a controversial stroke risk factor.

Simultaneous Toxicities in a Child on Multiple Anticonvulsants

Delima, S. I. R., Walsh, L. E., Golomb, M. R. — 2008-09-30

It is rare to develop simultaneous toxicities while on anticonvulsants. This article presents a 31/2-year-old child on valproic acid, lamotrigine, and phenytoin who developed simultaneous hepatotoxicity and bone marrow toxicity during a parainfluenza virus type 3 infection. These toxicities resolved after the cessation of anticonvulsants, and her seizures were managed acutely with scheduled lorazepam. This article discusses the possibility that simultaneous use of valproic acid, lamotrigine, and phenytoin could give this combination of toxicities and that concurrent viral infection may increase this risk.

Divergent Ependymal Tumor (Ependymoblastoma/Anaplastic Ependymoma) of the Posterior Fossa: An Uncommon Case Observed in a Child

2008-09-30

We report a divergent ependymal tumor of the posterior fossa (ependymoblastoma/anaplastic ependymoma) observed in an 8-year-old boy. The tumor showed the histological pattern typical of an ependymoblastoma (tubular-papillary fetaloid architecture with stratification of the tumor cells) next to areas in which findings typical of an anaplastic ependymoma were detected. The immunohistochemical study confirmed our diagnostic suspicion, allowing us to establish a differential diagnosis with other entities such as medulloblastoma, medulloepithelioma, atypical rhabdoid/teratoid tumor, or metastases.

Clinical Observation of Self-Injurious Behavior Correlated With Changes in Scalp Morphology in a Child With Congenital Hydrocephalus

Hartman, E. C., Gilles, E., McComas, J. J., Danov, S. E., Symons, F. J. — 2008-09-30

We report the case of a 12-year-old girl born with cerebral dysgenesis and congenital hydrocephalus first shunted shortly after birth. She had severe tissue-damaging self-injurious behavior, profound mental retardation, quadriparesis, as well as multiple cranial anomalies including turricephaly. After stage 1 cranial remodeling, a bone window was left pending second stage remodeling. Episodic changes in fluctuation of the scalp overlying the bone window were easily observed. During the course of a behavioral assessment for her self-injury, it was observed that the overall frequency of occurrence of self-injury increased significantly (P < .01) when the scalp was protruding and bulging compared with when the scalp was flush with the skull table. Periods of increased scalp protrusion were also associated with higher scores on a pain scale developed for children with communicative impairments associated with severe neurological impairment. After shunt replacement, there was remarkable improvement in functional status and decreased episodic self-injury.

Acute Epidural Hematoma With Infarction of the Right Hemisphere in a 5-Month-Old Child: Case Report With a Long-Term Follow-Up and a Review of the Literature

Ulrich, P. T., Fuessler, H., Januschek, E. — 2008-09-30

Two hours after a fall, a 5-month-old girl was admitted to our hospital because of an extended galea hematoma and restlessness. Five hours after the trauma, a left hemiparesis developed. The child became drowsy. The hematocrit had fallen to 7.1 g/dL. Cranial computerized tomography disclosed a huge frontoparietal epidural hematoma on the right side and a hypodensity in the territories of the middle and posterior cerebral arteries. Immediately before surgery, the right pupil dilated. After evacuation of the epidural hematoma and ligation of the middle meningeal artery, the girl recovered satisfactorily. Nevertheless, magnetic resonance imaging showed a vast defect zone in the territories of the right middle and posterior cerebral arteries. Six weeks after the injury, the visual-evoked potentials were unavailable on both sides. Four years after the accident, visual assessment revealed normal acuity and stereopsis. Cognitive and neuromotor development were undisturbed and appropriate to the age.

Cryptogenic Stroke in a Boy With Atrial Septal Defect and Hyperhomocysteinemia

Indolfi, G., Bartolini, E., Trapani, S., Azzari, C., Resti, M. — 2008-09-30

In children, clinical recognition of stroke and determination of its exact etiology are extremely difficult. The authors described a case of cryptogenic stroke in a 14-year-old-boy with asymptomatic atrial septal defect and hypercoagulable state. Paradoxical embolism was hypothesized as the responsible etiopathological mechanism. It is crucial to increase clinician awareness of stroke in children.

Epstein-Barr Virus--Associated Meningoencephalomyelitis: Intrathecal Reactivation of the Virus in an Immunocompetent Child

2008-09-30

Neurologic complications, including meningoencephalitis, transverse myelitis, and peripheral neuropathy, have been reported in patients with acute infectious mononucleosis. Chronic active Epstein-Barr virus and human immunodeficiency virus infections occasionally induce central nervous system lymphoma. On the other hand, central nervous system disease alone associated with Epstein-Barr virus rarely occurs in previously healthy individuals. A 15-year-old girl who developed acute disseminated encephalomyelitis-like disease presenting fever, anuresis, diplopia, and muscle weakness is described here. Clinical and neuroimaging studies led to the diagnosis of encephalomyelitis. Despite the absence of infectious mononucleosis-like symptoms, anti-Epstein-Barr virus antibody titers in serum and cerebrospinal fluid showed the virus reactivation. The copy number of Epstein-Barr virus DNA increased in cerebrospinal fluid but not in peripheral blood. Ganciclovir and repeated methyl-prednisolone pulse therapy resulted in complete resolution. Central nervous system disease on the limited intrathecal reactivation of Epstein-Barr virus in immunocompetent children should be differentiated from acute disseminated encephalomyelitis.

Severe Ataxia as a Complication of Human Parvovirus B19 Acute Encephalitis in a Child

2008-09-30

Human parvovirus B19 generally causes erythema infectiosum in childhood, but it can be associated with unusual findings, particularly in immunocompromised patients. This is a report about an immunocompetent 4-year-old female child affected with acute encephalitis by parvovirus B19, documented by polymerase chain reaction performed on cerebrospinal fluid, who was treated with intravenous immunoglobulins and dexamethasone and who developed a cerebellar syndrome with ataxia, dysmetria, and dysarthria. To the best of the authors' knowledge, this may be the first report of human parvovirus B19 encephalitis complicated by severe ataxia in childhood.

Transient Dysautonomia and Cerebellitis in Childhood Enteric Fever

Thapa, R., Banerjee, P., Akhtar, N., Jain, T. S. — 2008-09-30

A case of childhood enteric fever complicated by transient dysautonomia and cerebellitis is reported. The child was treated with intravenous antibiotics, and the complications were managed conservatively. Dysautonomia and cerebellitis resolved by day 5 and day 8 after admission, respectively. Results of a neurologic examination at the end of 6 months were normal. Dysautonomia complicating the course of childhood enteric fever is previously unreported.

Book Review: Neurological Assessment in the First Two Years of Life. By Giovanni Cioni and Eugenio Mercuri (eds). Mac Keith Press, Tettenhal, Wolverhampton, UK, 2008. DOI: 10.1177/0883073808320757

Johnsen, S. D. — 2008-09-30

Does Ghrelin Really Increase in Epileptic Children Treated With Valproate?

Aydin, S., Dag, E. — 2008-09-30

Correspondence on "Childhood Epilepsy With Occipital Paroxysms: Difficulties in Distinct Segregation Into Either the Early-Onset or Late-Onset Epilepsy Subtypes"

Covanis, A. — 2008-09-30

Response to Correspondence on "Childhood Epilepsy With Occipital Paroxysms: Difficulties in Distinct Segregation Into Either the Early-Onset or Late-Onset Epilepsy Subtypes"

Shahar, E. — 2008-09-30

Correct Definition and Reference of the Term "Status Gelasticus"

Ng, Y.-t. — 2008-09-30

Correspondence on "Developmental Regression and Mitochondrial Dysfunction in a Child with Autism"

Poling, J. S. — 2008-09-30

Conflict of Interest Statement Concerning "Developmental Regression and Mitochondrial Dysfunction in a Child With Autism"

Frye, R. E., Zimmerman, A. W., Shoffner, J. M. — 2008-09-30

The Appalling Poling Saga

Brumback, R. A. — 2008-09-30

"Worshiping False Idols: The Impact Factor Dilemma": Correcting the Record

Pringle, J. — 2008-09-30

Response to Correspondence, "`Worshiping False Idols: The Impact Factor Dilemma': Correcting the Record"

Brumback, R. A. — 2008-09-30