Pediatric Neurology Unit Research Articles

Sulthiame in refractory paediatric epilepsies: An experience of an ‘old’ antiepileptic drug in a tertiary paediatric neurology unit

Purpose Sulthiame is an old antiepileptic drug primarily used in a few European countries for the treatment of benign epilepsy of childhood with central temporal spikes. Other studies suggest that it might be effective in children and adults with a range of refractory seizure types. Methods A retrospective case note review was undertaken to evaluate the efficacy and safety of sulthiame as adjunctive therapy in children with refractory epilepsies. Results Twenty patients (10 female) were evaluated, aged 10.7 (range 2.1–17) years. The median duration of treatment with sulthiame was 18 (range 2–37) months. Fifty five percent of patients showed at least a 50% reduction in seizure frequency and two patients were seizure-free at the end of follow-up. Patients with focal seizures responded best. Seven patients reported side effects, leading to withdrawal of the drug in two (10%). Conclusion Sulthiame was reasonably effective and well-tolerated in a heterogeneous group of 20 children with refractory epilepsies. Although an ‘old’ antiepileptic drug it should be considered in a similar population.

Pattern of Paediatric Neurological Disorders in Port Harcourt, Nigeria

Background: Paediatric Neurological disorders in developing countries are very challenging. This is due to its chronicity, late presentation and unavailability of modern diagnostic facilities in developing countries like Nigeria. Lack of these modern technology and manpower contribute significantly to increased morbidity and mortality. This study demonstrates the pattern of neurological disorders and the challenges in management in a developing country. Materials and Method: This was a retrospective hospital based analysis of neurological disorders seen in the Paediatric neurology unit of the University of Port Harcourt Teaching hospital, Nigeria from January 2004 to December 2009. Descriptive statistics was used to present the result. Result: A total of 35,473 patients were seen in the Paediatric unit. Of these 2,379 had neurological disorders. This gave a prevalence of 6.7% of Paediatric neurological disorders. There were 1,431 males and 948 females (male: female ratio of 1.51:1.0). The age ranged from 3 months to 15 years. The age group 1->5 years accounted for the most affected age group constituting 87.7%. The most frequent Paediatric neurological disorders were epilepsy (24.6%), cerebral palsy (15.4%), and central nervous system infections (9.5%). Conclusions/Recommendation: Wide spectrums of neurological disorders occur in our environment. The high incidence of epilepsy and cerebral palsy suggests that effort should be geared towards educating the populace about early diagnosis and prompt management.

Mild Wolf–Hirschhorn phenotype in a girl with unbalanced t(4p;12p) translocation without seizures

Mild Wolf–Hirschhorn Phenotype in a Girl With Unbalanced t(4p;12p) Translocation Without Seizures Cinzia Galasso,* Adriana Lo-Castro, Nadia El-Malhany, Marcella Zollino, Marina Murdolo, Daniela Orteschi, Maria Luisa Manca Bitti, and Paolo Curatolo Pediatric Neurology Unit, Department of Neuroscience, ‘‘Tor Vergata’’ University of Rome, Rome, Italy Department of Medical Genetics, Catholic University of Rome, Rome, Italy Department of Paediatrics, ‘‘Tor Vergata’’ University of Rome, Rome, Italy

Safety of botulinum toxin type A in children younger than 2 years

Background Botulinum toxin type A (BoNT-A) has been used in many indications and is licensed for the treatment of spasticity in children older than 2 years. However, there are few reports of BoNT-A treatment in patients younger than 2 years of age. Aims To review retrospectively the safety data from all infants treated with botulinum toxin type A (BoNT-A) before 2 years of age in a paediatric neurology unit. Methods There were 74 infants: 28 received the first dose before 1 year of age, and 46 between the ages of 1 and 2 years. Results In the first year of life, the most frequent indication was obstetric brachial palsy (OBP) (71.4% of cases) and in the second year, cerebral palsy (CP) (73.9%). Both Botox ® and Dysport ®, the two commercially-available BoNT-A products in Spain, were used. The average starting dose by session was 6.55 U/kg body weight Botox in infants in their first year of life, and 8.4 U/kg body weight Botox and 21.1 U/kg body weight Dysport in the second year of life. Only 3.6% of cases treated in the first year and 6.5% of those treated in the second experienced adverse events (AEs), which consisted of mild weakness or tiredness lasting 1–4 days. Conclusions BoNT-A has a good safety profile in infants younger than 2 years old. AEs are similar to those found in older children.

Recurrencia de crisis febriles en una población chilena

Febrile seizures recurrence in Chilean children Objective: Evaluate the clinical presentation and risk factors for recurrence of first febrile seizure (FS) in patients at Pediatric Emergency Department or Neurology Units at Clinical Hospital Universidad Catolica (UC) and Hospital Herminda Martin from Chillan (HM). Method: Descriptive-observational study of children with age average of 2.9 years-old, with first febrile seizure between January 1, 2003 and December 31, 2005. Results: Total 158 patients; 76% presented a simple FS and 24% a complex FS as first episode, only 1 case with febrile epileptic status. In 22% and 18% respectively, there was a FS history and epilepsy in first degree relatives. 34% of patients presented a second episode; 36% of these cases present a third FS and 9% more than 3 episodes. The average time of recurrence was 6.9 months. The most important risk factors for recurrence were: epilepsy history in first degree relatives and type of febrile seizure, with hazard ratio of 2.5 (p = 0.001) and 1,8 (p = 0.03) respectively. When fitting both variables, only family history of epilepsy was significant. Conclusions: Most of FS episodes are simple and 34% present recurrence. Family history of epilepsy and type of febrile seizure are associated recurrence risk factors. The follow-up of these patients does not predict their future risk of epilepsy. (Key words: febrile seizures, recurrence, epilepsy). Rev Chil Pediatr 2008; 79 (5): 488-494

Management of Neonatal Seizures

The generally accepted clinical approaches to neonatal seizures were determined by questionnaires completed by pediatric neurologists and neonatologists representing all the pediatric neurology units and all departments of neonatology in Isrrael, and were evaluated at Tel Aviv Sourasky Medical Center.

An audit of the utility of sleep EEGs in a tertiary paediatric neurology unit

An audit of the utility of sleep EEGs in a tertiary paediatric neurology unit

Plasma magnesium and calcium levels in children with epilepsy in Lagos

Magnesium and calcium have been found to have increasing roles in the patho-physiology of epilepsy. Hypomagnesaemia and hypocalcaemia cause hyper-exitability of neurons and have been associated strongly with seizures in adults and children. To determine if hypomagnesaemia or hypocalcaemia is present in Nigerian children with epilepsy and to determine the relationship between serum magnesium and calcium levels and frequency and control of epilepsy. A prospective case control study at the Lagos University Teaching Hospital. 45 children with epilepsy and 45 controls were recruited from the paediatric neurology unit of the Lagos University Teaching Hospital. Serum magnesium and calcium were measured by spectrophotometric methods. Serum magnesium and calcium was significantly lower in the patients compared to the controls; Magnesium - 0.98 (0.0005) Vs 1.2 (0.04) mmol/L, p< 0.0001 and Calcium - 2.29 (0.04) Vs 2.3 (0.02) mmol/L, p<0.05 respectively. No significant differences were noted in the plasma phosphorus and albumin concentrations. Magnesium and calcium levels are lower in the epileptic children compared to the controls during the seizure-free periods. More studies are needed to evaluate these electrolytes during seizures and the effect of the different anticonvulsant drugs on these electrolytes.

Anoxic-epileptic seizures: observational study of epileptic seizures induced by syncopes

Aims: To describe a large series of children with anoxic-epileptic seizures (AES)—that is, epileptic seizures induced by syncopes. Methods: Retrospective case-note review in a tertiary paediatric neurology unit. For all...

Conversion disorder in childhood

In his excellent review (September 2003 JRSM1) Professor Leary says that, in a paediatric neurology unit in the west of England, the prevalence of conversion disorder approaches 10%. In an Indian study on children and adolescents, the prevalence was 31% among inpatients and 15% among outpatients.2 This could be related to the fact that, in this culture, children with 'medical' symptoms are more readily brought for consultation than those with psychiatric symptoms alone. Moreover, conversion disorders are over-represented in the lower socio-economic groups.3 Professor Leary does not offer a reason for the higher incidence in girls than in boys. This might be related to sexual or physical abuse, a known predisposing factor.4 A common method of diagnosing conversion disorder is the resolution of symptoms in response to a placebo injection. However, this method is not advisable since it reinforces the 'medical' model for symptoms and may hamper the response to psychotherapy later. Though these children should not be extensively investigated at presentation they do need regular follow-up. In one early series, 46% of children with conversion disorder were later found to have organic explanations for their symptoms.5

Paraparesia espástica tropical/mielopatía asociada al HTLV-I en la infancia. Caso clínico y revisión bibliográfica

HTLV-I associated myelopathy is characterised by a clinical picture of slowly progressive spastic paraparesis that generally presents when the patient is at an age somewhere between his or her thirties and sixties; few cases have been reported involving children. It is a pathology that is prevalent in tropical regions that are endemic for HTLV-I (southern Japan, the Caribbean, Central and South America, and some areas of Africa). The authors report the case of a 9-year-old child from Guinea who was admitted to the Paediatric Neurology Unit in the Hospital de Santa Maria with a two-year-old clinical history of spastic paraparesis. Computerised tomography and magnetic resonance imaging did not show any alterations to the spinal cord. Somatosensory evoked potentials revealed a lesion in the posterior dorsolumbar spinal cord. The exclusion of other (infectious, metabolic and demyelinating) pathologies and the confirmation of infection by HTLV-I (by means of PCR) led to a diagnosis of myelopathy associated to this virus. Therapy was established with interferon alfa, but no appreciable significant improvement was observed. This case stands out because of the uncommonness of this pathology at the paediatric age. We review the most relevant aspects of this disorder at the earliest ages, above all with regard to its epidemiology, transmission, clinical symptoms and complementary diagnostic examinations. Known therapeutic options (corticoids, interferon alfa, antiretroviral agents, among others) and prognosis are also discussed.

Conversion disorder in childhood--diagnosed too late, investigated too much?

Conversion disorder is defined in the Diagnostic and Statistical Manual of Mental Disorders (DSM-IV)1 as a condition in which symptoms and deficits in voluntary motor function suggest a neurological or other physical condition which is in fact not present. Child psychiatrists have reported a prevalence of 1–3% in the patients they see.2,3 Among new outpatients at a paediatric neurology unit in the west of England the prevalence is closer to 10%. These cases place considerable demands on consulting time and diagnostic resources. In this paper a case is made for early recognition and for management by the general paediatric team.

Risk factors of status epilepticus in children.

Although there is abundant literature about the morbidity and mortality rates of status epilepticus (SE), little is known about the risk factors of this medical emergency. The aim of the present study is to assess the risk factors of SE in children. The authors reviewed the medical records of 83 patients admitted to the Pediatric Neurology Unit of Trakya University Hospital, Edirne, Turkey from January 1994 to December 2001 with the diagnosis of SE. Eighty-three patients were compared with 166 controls who were admitted to the same unit due to non-status epilepticus (non-SE) seizure. The univariate analysis demonstrated that SE episodes were significantly associated with a history of birth asphyxia, neonatal seizure, discontinuation of antiepileptic medication, epilepsy, partial seizure evolving to secondary generalized seizures, myoclonic seizure, generalized abnormalities in the neurological examination, neuromotor retardation, generalized background abnormalities on electroencephalogram (EEG), generalized abnormalities on neuroimaging and polypharmacy than non-SE episodes. Logistic regression was used to test the independence of these parameters as predictors of SE risk. Four parameters emerged as significant independent predictors of SE in children in multiple logistic regression: polypharmacy (Odds ratio (OR) 5.17, P = 0.0004), discontinuation of antiepileptic medication (OR 4.04, P = 0.0095), neuromotor retardation (OR 4.03, P = 0.0016) and generalized background abnormalities on EEG (OR 2.48, P = 0.0419). Polypharmacy, discontinuation of antiepileptic medication, neuromotor retardation and generalized background abnormalities on EEG are indicators in children of a higher risk of SE.

Pediatric neurology services in Canada: demand versus supply.

As the practice of pediatric neurology has continued to evolve and expand, concerns have emerged regarding a perceived trend toward increasing clinical demand and decreasing manpower. Quantitative data are lacking, however. To address this, a questionnaire was sent to hospital-based pediatric neurology units in Canada, with all 18 centers responding. A total of 63 full-time-equivalent pediatric neurologists were disproportionately spread across the country, giving an overall ratio of 2.1 per million population (or 1.1 per 100,000 children). Waiting times for nonurgent consultations showed a median of 12.5 weeks. The number of weekly clinics per population was not proportional to either the number of specialists per center or to the waiting times. The regional variations in the level of service do not correlate with the regional manpower figures, reflecting different individual profiles of clinical and academic activities.

Malformation de Chiari de type I de l’enfant : modes de révélation de 34 observations

Incidental diagnoses of Chiari I malformation are more and more frequent in pauci or asymptomatic children. The value of neurophysiological investigations for surgical indications is discussed. Objective. – To determine clinical presentations of Chiari I malformation and relative frequencies of severe and incidental forms, and to evaluate the usefulness of neurophysiological investigations for surgical indications. Methods. – This retrospective study included 34 patients admitted from 1984 to 2000, with Chiari I malformation diagnosis in different pediatric (intensive care, neurology and neurosurgery) units of a university hospital. Results. – The clinical presentation was severe (two children died) in four cases (12%). Signs of brain stem compression were found in 41% of cases, scoliosis in 21%, and incidental diagnosis in 26%. The polysomnography, performed in 12 cases, showed sleep-apneas in six cases. One child with an incidental diagnosis of Chiari I malformation had many sleep-apneas which required a surgical treatment. A surgical decompression was performed in 69 % of cases, with clinical improvement in 77%. Conclusion. – The high incidence (12%) of severe forms revealing Chiari I malformations justifies systematic neurophysiological investigations for the initial evaluation and the follow-up of Chiari 1 malformations, including the less symptomatic forms.

Clinical Significance of Generalized Spike/Wave EEG

The clinical correlates of 154 children with epilepsy and generalized spike-and-wave (SW) EEG patterns were analyzed at the pediatric neurology units, HU de Getafe and Hospital 12 de Octubre, Madrid, Spain.

Acute isolated sphenoid sinusitis in children.

Acute isolated sphenoid sinusitis in children.

Cerebellar Ataxia, Opsoclonus, and Neuroblastoma

A 20 month-old girl with cerebellar ataxia and opsoclonus associated with neuroblastoma is reported from the Pediatric Neurology Unit, Tel Aviv Medical Center, Israel.

Pediatric Neurology Unit, Chaim Sheba Medical Center, Tel Hashomer, Israel; and Division of Clinical Pharmacology, The Hospital for Sick Children, Toronto, Ontario, Canada

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