Journal of Pediatric Neurology - Volume 4, issue 3

Authors: Kramer, Uri | Watemberg, Nathan | Zelnik, Nathanel | Shahar, Eli | Lerman-Sagie, Tally | Goldberg-Stern, Hadassah | Ben-Zeev, Bruria

Article Type: Research Article

Abstract: To evaluate the efficacy of steroid therapy in epileptic encephalopathies and refractory epilepsies other than West syndrome. Retrospective analysis of treatment and outcome data of patients treated with steroids for epileptic encephalopathies. Outcome was analyzed in terms of the patient's epileptic syndrome, age at seizure onset, duration of seizure disorder, age at treatment, abnormalities noted on neurological examination, developmental status and type of steroid. Fifty-eight consecutive patients from six pediatric neurology outpatient …clinics were included in the study. The epileptic syndrome and types were as follows: myoclonic epilepsy (n=16), epilepsy with continuous spike waves during slow-wave sleep (n=12), partial epilepsy (n=10), Landau-Kleffner syndrome (n=6), generalized epilepsy with multiple epileptic seizure types (n=5), Rasmussen's encephalitis (n=3), Lennox-Gastaut syndrome (n=2), atypical absence (n=2) and spasms (n=2). Sixteen patients (27%) had received adrenocorticotrophic hormone (ACTH) and 42 (7%) had received prednisone (22 of the latter patients were given initial pulse methylprednisolone). Complete cessation of seizures as well as disappearance of epileptic discharges in electroencephalogram were achieved in 16 patients (28%); 15 other patients (26%) had improvement of >50%. Of the 31 patients with >50% improvement and follow-up of at least 1 year, 16 (52%) subsequently relapsed. ACTH was found to be significantly more efficacious than prednisone in controlling seizures and improving function. Initiation of treatment with high-dose methylprednisolone did not increase efficacy. ACTH may be more efficacious than prednisone in treating epileptic encephalopathies and refractory epilepsies other than West syndrome. Show more

Keywords: Epilepsy, encephalopathy, steroids

Citation: Journal of Pediatric Neurology, vol. 4, no. 3, pp. 147-153, 2006

Authors: El-Salem, Khalid | Al-Shimmery, Ehsan | Horany, Khalid | Al-Refai, Ali | Khader, Yousef

Article Type: Research Article

Abstract: A multicenter retrospective analysis of the clinical characteristics of early onset multiple sclerosis (EOMS) in Jordan was conducted using multiple sclerosis (MS) database of three major referral centers. The database was formulated by collecting information from consecutive MS patients fulfilling the McDonald's criteria for clinically definite or clinically probable MS, attending neurology clinics in the participating hospitals in the period between August 2004 and September 2005. Patients with disease onset at or …before the age of 18 years were included and their data were statistically analyzed. EOMS was seen in 30 patients (13.4%) out of 230, in the Jordan MS database. Estimated prevalence of pediatric MS in Jordan was 5.25/100,000. Female to male ratio was 4:1 (24 females: six males). Twenty-eight (93.3%) subjects had a relapsing-remitting course, while two were primary progressive. None of the patients had a secondary progressive pattern. The majority of our patients (77%) were monosymptomatic at onset. Weakness at onset was seen in 23.3% of patients, which was higher than most of previously reported percentages. Optic neuritis and sensory disturbances were equally observed as presenting symptoms (23.3% each). About 13% of our total patient population had a positive family history of MS in a first degree relative. Six patients were treated with interferon before the age of 18, and tolerated the treatment well. A tendency towards less current disability was noted with earlier age of onset, use of interferon, a relapsing-remitting course and a positive family history of MS. We conclude that EOMS in the Jordanian population is clinically similar to adult onset MS, but slightly different from EOMS in other countries, with a largely monosymptomatic presentation, a higher number of patients with weakness at onset, and the absence of a secondary progressive pattern. This emphasizes the importance of genetic and environmental factors in MS onset. Show more

Keywords: Multiple sclerosis, early onset, Jordan

Citation: Journal of Pediatric Neurology, vol. 4, no. 3, pp. 155-160, 2006

Authors: Al-Haggar, Mohammad S. | Al-Naggar, Zienab A. | Abdel-Salam, Mohammad A.

Article Type: Research Article

Abstract: We aimed to evaluate the efficacy of cognitive behavioral therapy (CBT) aided by biofeedback in rehabilitating Egyptian adolescents who were suffering from chronic fatigue syndrome (CFS). Out of 298 screened individuals with chronic fatigue, only 159 adolescents were eligible for study; of them 63 cases lost follow up and four cases were further excluded because of switch leaving only 92 cases with complete database. Age range of enrolled cases was 10–14 years and male/female ratio (1/2.5). …They were recruited from private schools and polyclinics in Eastern province, Saudi Arabia; some cases were referred by psychiatrists in private hospitals of the same area. All cases were diagnosed as CFS according to the recommendations of International CFS Study Group. Patients were randomly allocated to one of two groups; interventional group comprised 50 cases and underwent CBT aided with biofeedback over a period of 18 months applying two protocols according to patient's activity pattern. Forty-two cases were followed and treated symptomatically and used as control group. Data were processed and analyzed using SPSS version 10.0. The most common symptoms were unrefreshing sleep, headache and myalgia (95.8%, 67.7% and 50% respectively). Patients of interventional group showed marked improvement manifested by decrement of checklist individual strength (decreased 23.1%; 95% confidence interval 19.2–25.4%) and better school attendance (increased 31.5%; 95% confidence interval 29.8–36.6 hours/month) with the disappearance of some self-rated CFS symptoms. CBT aided by biofeedback could be very effective in treatment of adolescents suffering from CFS taking in consideration the stressors and precipitating factors during settings of psychotherapy. Show more

Keywords: Biofeedback, cognitive behavioral therapy, chronic fatigue syndrome, adolescents

Citation: Journal of Pediatric Neurology, vol. 4, no. 3, pp. 161-169, 2006

Authors: Sethi, Sujata | Setiya, Rajat | Lallar, Kuldeep

Article Type: Research Article

Abstract: Sydenham chorea is the most common form of acquired chorea in developing countries. It is an incapacitating illness lasting for months and requires drug therapy. In view of the significant side effects associated with the drugs commonly used for the treatment of Sydenham chorea, we evaluated the efficacy of olanzapine in six children with Sydenham chorea and found encouraging results with no adverse effects.

Keywords: Sydenham chorea, olanzapine, sodium valproate, carbamazepine

Citation: Journal of Pediatric Neurology, vol. 4, no. 3, pp. 171-174, 2006

Authors: Kumar, Raj

Article Type: Research Article

Abstract: Nine cases of treated medulloblastomas, who developed single or multiple supratentorial and other neuraxial disseminations at follow-up, were studied retrospectively regarding their course, route of spread, and outcome. These cases were encountered over a period of 9 years out of all treated pediatric medulloblastomas (posterior fossa) between 1999 and July 2005. The records of all the cases were maintained on a computer database under ICD-9 classification in the hospital records department. All nine cases developed …frontal or subfrontal metastases, while two developed small recurrences in the posterior fossa. Two of nine cases developed multiple spread within the neuraxis. Surgical excision of frontal metastases was performed in two cases and chemotherapy was offered in seven cases. The following inferences were drawn from this analysis. (i) Supratentorial or any neuraxial dissemination may occur as early as 6~months or may be delayed for up to 9.5 years. (ii) Dissemination of disease may not be associated with recurrence at the primary site in most of the cases. (iii) Even chemotherapy may not be promising in all the cases, but it may prolong survival in some cases, if it is tolerated well. (iv) Surgical excision of metastasis may have risk of further dissemination of disease. Show more

Keywords: Medulloblastoma, neuraxial dissemination, supratentorial metastases, subfrontal dissemination

Citation: Journal of Pediatric Neurology, vol. 4, no. 3, pp. 175-181, 2006

Authors: Denne, Christian | Kleines, Michael | Scheithauer, Simone | Heimann, Gerhard | Häusler, Martin

Article Type: Research Article

Abstract: We report a girl with opsoclonus-myoclonus syndrome related to cerebral rubella infection that relapsed during adrenocorticotropin monotherapy but stabilized with methotrexate add-on treatment. Methotrexate may be a new therapeutic option in opsoclonus-myoclonus syndrome, reducing the need for long-term high-dose adrenocorticotropin treatment and the risk of steroid-related side effects.

Keywords: Opsoclonus, myoclonus, methotrexate, ACTH, rubella

Citation: Journal of Pediatric Neurology, vol. 4, no. 3, pp. 183-185, 2006

Authors: Besnard, Stéphane | Beaume, Anne | Gil, Roger | Pluchon, Claudette | Oriot, Denis | Loiseau, Marie-Noelle

Article Type: Research Article

Abstract: This case report presents a 14-month follow-up of a case of Hashimoto's encephalopathy (HE) treated with immunoglobulins for the first time in an 11-year-old adolescent. The diagnostic criteria were: (a) a drop in school performance and psychological withdrawal for several weeks, (b) an euthyroid state with a past medical history of autoimmune thyroiditis treated with thyroxin, (c) a first episode of tonic-clonic seizure with clinical and electroencephalographic (EEG) findings of encephalopathy, (d) a high serum …concentration of antithyroglobulin and antithyroid peroxidase antibodies, (e) negative results of cerebrospinal fluid and blood studies for viral and bacterial infections. Her acute epileptic symptoms resolved with clonazepam. Immunoglobulin therapy (400 mg/kg/day for 5 days every 6 weeks) was given for 14 months. Her cognitive function was greatly improved but a light-to-mild cognitive deficit remained. An EEG performed at her 14-month follow-up showed a correlation with her cognitive improvement. We suggest that immunoglobulin can be used as a first-line therapy in HE to avoid side effects or relapses as has been described with long-term steroid therapy. Show more

Keywords: Encephalopathy, thyroiditis disease, immunoglobulin

Citation: Journal of Pediatric Neurology, vol. 4, no. 3, pp. 187-193, 2006

Authors: Smyth, Matthew D. | Narayan, Prithvi | Tubbs, R. Shane | Leonard, Jeffrey R. | Park, Tae Sung | Grabb, Paul A.

Article Type: Research Article

Abstract: We present two clinical cases of infants with hydrocephalus requiring multiple cerebrospinal fluid (CSF) shunt revisions and diagnostic computerized tomography (CT) scans that developed head and neck malignancies during their late teen years. These represent the first reported cases of possible diagnostic imaging-induced malignancy in children. The first patient had undergone at least 23 CT scans and 25 plain skull radiographs prior to the diagnosis of a neck Hodgkin's lymphoma. The second patient had undergone at …least 14 CT scans and 15 plain skull radiographs prior to the diagnosis of a left parietal poorly differentiated gliosarcoma. Although it is not possible to prove that the multiple diagnostic studies these patients underwent caused their malignancies, these illustrative cases and the epidemiological literature describing malignancy risk and radiation exposure should give clinicians pause when considering requesting multiple diagnostic CT studies in children during the evaluation of possible CSF shunt dysfunction and other clinical problems. "Shunt magnetic resonance imaging" protocols should be considered for these patients and used whenever possible to minimize exposure to ionizing radiation. Show more

Keywords: Pediatrics, diagnostic CT, CT risk, induced malignancy, hydrocephalus

Citation: Journal of Pediatric Neurology, vol. 4, no. 3, pp. 195-201, 2006

Authors: Ray, Munni | Kothur, Kavitha

Article Type: Research Article

Abstract: We report a 12-year-old child from North India with cysticercosis presenting as an ulnar entrapment neuropathy due to a compressive mass in the flexor digitorum profundus. Magnetic resonance imaging suggested an inflammatory mass compressing the neurovascular bundle. The histologic diagnosis was consistent with cysticercosis. Such solitary presentation of entrapment neuropathy with cysticercosis is extremely rare, and to our knowledge, this is the only case reported in pediatric literature.

Keywords: Ulnar nerve, entrapment neuropathy, cysticercosis

Citation: Journal of Pediatric Neurology, vol. 4, no. 3, pp. 203-206, 2006

Authors: Tabarki, Brahim | Kamel, Areej | Anjum, Sher Bahadur | Ezeldin, Kamel | Amin, Adnan

Article Type: Research Article

Abstract: Postinfectious immune-mediated brainstem encephalitis associated with herpes simplex virus infection is believed to be rare. We report a seven-year-old previously healthy boy who developed brainstem encephalitis with excellent response to acyclovir and methylprednisolone, after herpetic gingivostomatitis. No relapse was noted during a 24-month follow-up period.

Keywords: Herpes simplex virus type 1, brainstem encephalitis, immune-mediated process, steroid therapy

Citation: Journal of Pediatric Neurology, vol. 4, no. 3, pp. 207-209, 2006