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We aimed to determine seizure characteristics of pediatric patients with epilepsy, and evaluate if Semiological Seizure Classification (SSC) system is applicable in this cohort. We retrospectively studied 183 patients, aged between 3 months-18 years, admitted to the video-EEG monitoring unit (VEMU). Most patients suffered from intractable epilepsy with comorbidities, and had structural lesions. Seizures were classified based on ictal video-EEG recordings by using SSC system; 157 patients had only one seizure type, 26 had more than one seizure types. Overall 211 seizures and 373 semiologies were analyzed; 114 seizures (54%) had more than one semiological subtype. The most frequent semiology was motor seizures (78%), followed by dialeptic seizures (12%). The most common subtypes were simple motor seizures (49%); tonic seizures constituted (28.4%) of all semiologies. We conclude that SSC system is applicable for children with epilepsy admitted to VEMU; complementary EEG and imaging data are required for evaluation of patients with epilepsy. [ABSTRACT FROM AUTHOR]

We report magnetic resonance (MR) imaging findings including diffusionweighted imaging and proton MR spectroscopy findings in a patient with infantile Refsum disease. The initial diagnosis was made on the basis of history, clinical findings and biochemical studies. Bilateral and symmetrical involvement of the peritrigonal white matter, centrum semiovale, thalami, corpus callosum and corticospinal tracts as assessed by increased T2 signal was highly suggestive of a peroxisomal disorder. Facilitated diffusion was observed in diseased parenchyma. Long echo-time (TE: 270 ms) MRS showed decreased N-acetyl-aspartate/creatine and elevated choline/creatine and lactate; short echo-time MRS (TE: 30 ms) revealed increased myoinositol at 3.56 ppm and lipid peaks at 0.9 and 1.3 ppm. A major contribution to the differential diagnosis came from MR imaging and proton MRS, as discussed in this report. [ABSTRACT FROM AUTHOR]

The value of video-electroencephalographic monitoring (EEG-VM) in evaluating patients with epileptic disorders constitutes a major research field. This study investigates the usefulness of inpatient long-term EEG-VM for pediatric neurology patients under four headings: pre-surgical evaluation; seizure classification; epileptic seizure and non-epileptic paroxysmal event differentiation; and antiepileptic drug (AED) treatment modification. A retrospective study of 101 patients over a one-year period was carried out. The results showed that following EEG-VM, 57.4% of the patients were referred for discussion to the epilepsy surgery conference regarding resective surgery, and of these, 31% were deemed to be surgical candidates. The seizure classification assigned to the patients before EEG-VM changed in 73.3% of the patients after EEG-VM. Regarding the differentiation between epileptic seizure and non-epileptic paroxysmal events, a diagnosis of psychogenic non-epileptic seizure (PNES) was made in 4% of the patients after EEG-VM. EEG-VM outcomes led to the modification of AED treatment in 68.3% of the patients. These significant alterations demonstrate the usefulness of EEG-VM in the management of pediatric neurology patients. [ABSTRACT FROM AUTHOR]

Conversion disorder (CD) in children remains a major challenge both in pediatric and mental health clinics and is still a prevalent psychiatric disorder in developing countries. The authors describe a 10-year-old boy with the complaints of inability to walk, speak or eat, excessive drooling, urinary and fecal incontinence, disturbance from light and sound, and expression of needs only by eye movements. The patient diagnosed with CD was followed by the Department of Child and Adolescent Psychiatry with play therapy, individual psychotherapy and family therapy. At the end of three months, the patient was discharged. This is one of the most challenging cases of CD in children. The most important aim of the treatment is to understand the need for conversion symptoms and to constitute a healthy psychological environment for the child rather than to remove the physical symptoms. [ABSTRACT FROM AUTHOR]

Neurometabolic diseases diagnosed by cerebrospinal fluid (CSF) examination are GLUT1 deficiency, serine-deficiency syndromes, glycine encephalopathy, cerebral folate deficiency, neonatal vitamin-responsive epileptic encephalopathies, disorders of monoamine metabolism, and y-amino butyric acid (GABA) metabolism. We retrospectively analyzed and compared the demographic, clinical, laboratory, and neuroimaging features of 62 patients in whom CSF examination was performed. Of the 62 patients, 16 (25.8%) had a final diagnosis, including succinic semialdehyde dehydrogenase (SSADH) deficiency (n=4), aromatic amino acid decarboxylase (AADC) deficiency (n=4), L-dopa-responsive dystonia (n=3), glycine encephalopathy (n=2), pyridoxal-phosphate-dependent seizures (n=1), cerebral folate deficiency (n=1), and serine biosynthesis defect (n=1). Parental consanguinity was present in all patients except one. Positive yield of a diagnostic lumbar puncture (LP) for the diagnosis of inherited neurotransmitter metabolism disorder was 25.8% overall. Oculogyric crisis (50%), diurnal variation (81.8%) and consanguinity (93.8%) were the only statistically significant variables between patients with and without a specific diagnosis. It is challenging to diagnose neurotransmitter defects, since there is no ideal set of clinical symptoms. In our cohort, consanguinity, diurnal variation and abnormal ocular movements were the most significant findings associated with a diagnosis of a specific neurometabolic disorder based on CSF examination. Early diagnosis is of great importance not only for specific treatment, but also for genetic counseling and prenatal diagnosis. [ABSTRACT FROM AUTHOR]

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We studied the efficacy and side effect profile of lamotrigine (LTG) in children with intractable epilepsy. We reviewed the database of our prolonged video-EEG laboratory to screen patients with intractable epilepsy who were on LTG either alone or in combination for three months or more. Of 75 patients, 28 patients (37%) had primary generalized seizures, 42 patients (56%) had partial onset seizures with or without secondary generalization, and 5 patients (7%) had two or more types of seizures. LTG reduced seizure frequency by at least 50% in 57.1% of patients with partial seizures and in 53.6% of patients with primary generalized seizures. The incidence of adverse events was relatively low (15%); the most common was skin rash. LTG should be considered in the treatment of pediatric patients with both partial onset and primary generalized seizures refractory to the major older antiepileptic drugs. LTG has a favorable side effect profile. [ABSTRACT FROM AUTHOR]

Osteoid osteoma is an osteoblastic benign lesion of the bone. The pathognomonic symptom is significant pain, which responds well to nonsteroidal antiinflammatory drugs. When typical clinical and radiological features are present, the diagnosis is not difficult. However, if the lesion is in an area not clearly seen on plain radiographs, or clinical features are atypical, then diagnosis becomes difficult. We present a case of osteoid osteoma with delayed diagnosis that presented itself with neurological signs. Prominent features present in the patient included pain that responded well to medication and muscle atrophy, which led to a wider differential diagnosis. Diagnosis was made approximately two years after the onset of his initial symptoms, after having been investigated and treated both in our own hospital and elsewhere. This case illustrates clinical and radiological diagnostic problems of osteoid osteoma, demonstrating that it can present itself with neurological signs. Correct diagnosis then requires detailed history and clinical awareness. [ABSTRACT FROM AUTHOR]

L-2-Hydroxyglutaric aciduria (LHGA) is a rare autosomal recessively inherited neurodegenerative disorder. It is characterized by psychomotor retardation, progressive ataxia and typical magnetic resonance imaging findings, and presents in early infancy. On the other hand, medulloblastomas are very common solid tumors of childhood and infancy. We present a 3-year-old boy with LHGA who developed a medulloblastoma during the course of the disease. There has been no previous report of the coexistence of medulloblastomas with LHGA. Central nervous system tumors are encountered in children with other metabolic neurodegenerative disorders. The aim of this paper is to focus on the difficulties in the diagnosis and treatment of an intracranial tumor in a child already neurologically impaired due to metabolic neurodegenerative disease.Copyright © 2002 S. Karger AG, Basel [ABSTRACT FROM AUTHOR]

The aim of this study was to investigate the adverse effects of the intraocular injection of botulinum toxin in rabbits. Intravitreal injections of botulinum toxin A in five doses, 1.25, 2.5, 5, 10, 25 units, were given into five rabbit eyes. The same volume of saline was injected into the second eye of the rabbit as a control. External examination, ophthalmoscopy, visual evoked potentials and electroretinography were done before injection and repeated at the first and second weeks after the injection. There were no significant differences in retinal function between toxin- and saline-injected eyes, neither ophthalmoscopically nor electrophysiologically. Ipsilateral mydriasis developed in the eyes injected with botulinum toxin. This study suggests that botulinum toxin has no harmful effect on retinal function. [ABSTRACT FROM AUTHOR]