Evaluation of Patients With Febrile Seizure: Risk Factors, Reccurence, Treatment and Prognosis.

INTRODUCTION: Febrile convulsion (FC) is the most common neurological disorder in childhood. The aim of this study was to determine the risk factors for recurrence and the development of epilepsy from the demographic data of these patients. METHODS: A retrospective study was made of 680 patients with FC who presented to our hospital. Patients with only FC were defined as group 1 and those who developed epilepsy after FC as group 2. Comparisons were made between the 2 groups of the demographic parameters, parental consanguinity, familial history of FCs or epilepsy, criteria for starting prophylactic treatment, response to treatment, risk factors for recurrence, and results of electroencephalogram. RESULTS: The study included a total of 680 patients comprising 399 males (59%) and 281 females (41%). A diagnosis of FC was made in 652 cases (95.8%, group 1), and epilepsy was diagnosed in 28 (4.2%, group 2). A positive familial history of FCs was determined statistically significantly higher in group 1. On the other hand, a history of pathological birth and parental consanguinity was found higher in group 2. Prophylactic treatment was administered to 89.3% of group 2 and 40.3% of group 1. CONCLUSIONS: Febrile convulsions create fear and panic in the family and are a significant health problem in Turkey. The conclusion that has been reached is that the unnecessary administration of prophylactic treatment could be prevented with education of the families on the subject of fever and correct identification of patients at risk of developing epilepsy.

A rare cause of mental motor retardation: recessive congenital methemoglobinemia type II.

Recessive congenital methemoglobinemia (RCM) is a very rare disorder caused by NADH- cytochrome b5 reductase (cytb5r) deficiency. It has been classified into four types. Type I presents with mild cyanosis due to a significant deficiency of cytb5r in erythrocytes only. In type II, the deficiency occurs in all tissues and causes growth and mental retardation and other neurological impairments. RCM types I and II are caused by a defect in a single gene, which is located on chromosome 22 (locus DIA 1: q 13.31-qter). Prenatal diagnosis is possible. Cyanosis can be well treated by 200-500 mg of ascorbic acid daily; there is no effective therapy for the progressive neurological impairments. This report presents two siblings with central cyanosis, growth retardation, mental retardation, microcephaly, dystonia and hypertonia diagnosed as RCM type II.

Attitudes of parents and physicians toward febrile seizures.

OBJECTIVES: Although febrile seizures are common in children, attitudes may change among parents. The management of a child may differ depending on the specialty of the attending physician. This study was carried out to analyze attitudes of Turkish parents and physicians toward febrile seizures. MATERIAL AND METHODS: 308 children with febrile seizure who were admitted to the Department of Pediatric Neurology at Dr Sami Ulus Children's Hospital and Gazi University in Turkey between January 2006 and March 2007 were enrolled. RESULTS: Prior to seizure, approximately half of the parents took appropriate steps in reducing fever. The data also showed that there was a wide variation of treatment practice depending on the specialty of the attending physician. DISCUSSION: Educational level and economic status are important variables affecting attitudes of parents toward fever and febrile seizure. The management of the child with a febrile seizure differs even within the same specialty in Turkish physicians.

Sinusitis in children and adolescents with chronic or recurrent headache: a case-control study.

The aim of this study was to determine the frequency of misdiagnosis of sinus headache in migraine and other primary headache types in the children and adolescents with chronic or recurrent headaches. Children with chronic or recurrent headaches (n = 310) were prospectively evaluated. Data collection for each patient included history of previously diagnosed sinusitis due to headache, and additional sinusitis complaints (such as fever, cough, nasal discharge, postnasal discharge) at the time of sinusitis diagnosis, and improvement of the headache following treatment of sinusitis. If sinus radiographs existed they were recorded. The study included 214 patients with complete data. One hundred and sixteen (54.2%) patients have been diagnosed as sinusitis previously and 25% of them had at least one additional complaint, while 75% of them had none. Sinusitis treatment had no effect on the headaches in 60.3% of the patients. Sinus graphy had been performed in 52.8%, and 50.4% of them were normal. The prevalence of sinus headache concomitant with primary headache, and only sinus headache was detected in 7 and 1%, respectively, in our study. Approximately 40% of the patients with migraine and 60% of the patients with tension-type headache had been misdiagnosed as "sinus headache". Children with chronic or recurrent headaches are frequently misdiagnosed as sinus headache and receive unnecessary sinusitis treatment and sinus graphy.

Prothrombotic risk factors in children with hemiplegic cerebral palsy.

BACKGROUND: The purpose of the present paper was to investigate the prevalence of prothrombotic risk factors associated with hemiplegic cerebral palsy (CP). METHODS: Twenty-three hemiplegic CP patients were tested for inherited and acquired prothrombotic risk factors, except methylene tetrahydrofolate reductase (MTFR) polymorphism. RESULTS: A total of 56.5% of patients had at least one coagulation abnormality and 13% of them had two. Four patients (8.7%) had infection and congenital heart disease, who also had additional coagulopathy risk factors. Obstetric problems were detected in 56.5%. Coagulopathy risk factors were factor V Leiden mutation (21.7%), protein C deficiency (21.7%), elevated lipoprotein-a (13%), G20210A mutation of prothrombin (8.7%), and protein S deficiency (4.3%). CONCLUSION: Children with hemiplegic CP need to be evaluated for coagulopathic abnormalities.

Devic's neuromyelitis optica in an infant case.

Devic's neuromyelitis optica was orginally described as an acute severe monophasic syndrome characterised by myelitis and optic neuritis. The mean age at onset was reported to be around 40 years, with a wide range. However, Devic's neuromyelitis optica has also been seen in children. Prognosis of the syndrome was poor, and no satisfactory treatment was known. This article reports a 23-month-old boy with acute myelitis and optic neuritis who was diagnosed with Devic's neuromyelitis optica. The response of the patient to therapy was poor, and he developed severe sequelae.

Bilateral basal ganglia lesions after hypoglycemic coma in a 6-year-old child.

Imaging findings of brain damage due to neonatal hypoglycemia are known; however, the effect of childhood hypoglycemia on the brain has not been described well. The authors present the case of a 6-year-old girl who had seizures secondary to hypoglycemia followed up for 1 year as epilepsy. The patient had experienced a hypoglycemic coma attack about 1 year before. Brain magnetic resonance imaging showed atrophy of the cerebrum and cerebellum and bilateral symmetrically hyperintense lesions in the putamina. The patient was diagnosed with hypoglycemia due to hyperinsulinism.

Progressive vacuolar myelopathy and leukoencephalopathy in childhood acute lymphoblastic leukemia and transient improvement with vitamin B12.

Vacuolar myelopathy (VM) in leukemia is rare. We report a boy with leukemia who developed isolated central nervous system (CNS) relapse during reinduction therapy. 5 months after cranial radiotherapy, he gradually developed quadriparesis. Magnetic resonance imaging revealed an intramedullary lesion which extended through the cervical spine. Serum vitamin B12, folic acid, cerebrospinal fluid methyl malonic acid were normal. Viral screening by ELISA was negative. He had lymphopenia, and reduced immunoglobulins, from a cardiac arrest. Biopsy revealed VM. He responded to weekly vitamin B12 treatment but on the 6th week of the therapy he died after developing periventricular, gliotic, hyperintense lesions in the brain.

Fixation-off sensitivity and generalized epileptic EEG induced by eyes closed.

This report describes a female with eyelid fluttering with absence seizures, infrequent generalized tonic-clonic seizures, and mild mental retardation. Interictal and video-electroencephalography evaluations revealed normal activity while eyes were open but continuous generalized discharges with eyes closed (eyes closed induced abnormality), as well as fixation-off sensitivity. This patient is in the group of a pure and distinct clinical form of fixation-off sensitivity cryptogenic generalized epilepsy.

Nonconvulsive status epilepticus on electroencephalography in a case with subacute sclerosing panencephalitis.

Subacute sclerosing panencephalitis is a neurodegenerative disease with a poor prognosis. We report a case of a 5 1/2-year-old boy who had emotional lability, cognitive difficulties, and myoclonia after a mild closed head injury. The magnetic resonance image of the brain and computed tomographic scan of the head were normal. His electroencephalogram (EEG) showed continuous nonconvulsive status epilepticus activity, which could not be suppressed with intravenous diazepam. After treatment with phenytoin for 2 days, an EEG showed periodic high-amplitude sharp-and-slow-wave complexes, which were also not suppressed with intravenous diazepam. Since the patient had measles at 5 months of age, subacute sclerosing panencephalitis was considered, and the diagnosis was confirmed by the presence of measles antibodies in cerebrospinal fluid.

A comparison of buccal midazolam and rectal diazepam for the acute treatment of seizures.

In this study, the authors aimed to evaluate buccal midazolam as a practical and safe alternative medication for children who suffer from seizures in the emergency setting and in home practice or anywhere. The effects and side effects of buccal midazolam and rectal diazepam were compared in the treatment of acute convulsions in 43 children, ranging in age from 2 months to 12 years who were seen at the emergency service of the children hospital. Midazolam was given on the even days of the month and diazepam was given on the odd days. In the midazolam group, the seizures of 18/23 (78%) patients terminated in 10 minutes; however 5/23 (22%) patients did not respond. In the diazepam group 17/20 (85%) patients responded in 10 minutes, but 3/20 (15%) did not respond. Midazolam was found to be as effective as diazepam and the difference was not statistically significant (p<0.05). Response periods of the 2 drugs showed no significant difference (p>0.05). The need for a second drug for seizures that did not stop with the first drug was equal, and the difference was not statistically significant (p>0.05). They did not observe any serious complications. In conclusion, buccal midazolam is safe and as effective as rectal diazepam for the treatment of seizures.

Heart rate variability and autonomic dysfunction in SSPE.

Autonomic nervous system involvement in subacute sclerosing panencephalitis was studied in 29 patients by analysis of heart rate variability and compared with a control group which consisted of 20 age- and sex-matched healthy subjects. Holter recordings for 24 hours were obtained, and all recordings were analyzed using time-domain parameters. The patients with subacute sclerosing panencephalitis were found to have significantly lower values of standard deviation of all normal sinus intervals and triangular index when compared with the control group. Of 23 patients who had regular follow-up, 15 died in a period ranging from 1 to 13 months. Twelve of these patients had heart rate variability parameters lower than normal, but no significant difference was observed between the heart rate variability values of patients who survived or died. Also, there was no association between brain magnetic resonance imaging findings and heart rate variability indices. It is concluded that patients with subacute sclerosing panencephalitis have autonomic dysfunction; and this was thought to be mainly due to central involvement.

Brown-Vialetto-van Laere syndrome; the first Turkish case.

We describe a 14-year-old female patient with progressive ponto-bulbar palsy and deafness. The first symptom was present at the age of 9 as a difficulty in walking and then she was stable with mild clumsy walking till 14 year-old. It was noticed that she had rapidly progression gait disorder, hearing loss, difficulty in swallowing and speaking in a period of 2.5 months. Clinically, there were bilateral facial weaknesss, atrophic tongue with fasciculations, poor gag reflex, deafness, axial and appendicular hypotonia, severe muscular weakness involving muscles of neck, shoulder, and upper arms, hands with thenar and hypothenar amyotrophy. Hearing loss was documented by brainstem auditory evoked potentials. Other laboratory investigations, screening tests and imaging studing were normal. These clinical features are consistent with the Brown-Vialetto-van Laere syndrome.

Subacute sclerosing panencephalitis: a cause of acute vision loss.

A 4-year-old male presented with only acute vision loss. His neurologic examination, funduscopic examination, and pupils were normal. Cranial magnetic resonance imaging revealed abnormal hyperintense, bilaterally symmetric lesions (on T(2)-weighted and fluid-attenuated inversion recovery, images) in bilateral optic radiations, pulvinar region in the thalami, crus posterior of internal capsules, periventricular white matter, and unilaterally left anterior pons. Elevated measles antibody titers in the cerebrospinal fluid confirmed the diagnosis of subacute sclerosing panencephalitis. Vision loss improved and cranial magnetic resonance imaging findings regressed,but myoclonic jerks and deterioration began 7 months later. The diagnosis of subacute sclerosing panencephalitis should be considered in cases with acute vision loss resulting from cortical blindness even when classical findings of the central nervous system do not exist.

Central pontine and extrapontine myelinolysis owing to disequilibrium syndrome.

Neurologic disorders can be seen in patients with end-stage renal failure owing to complications of hemodialysis or peritoneal dialysis. The disequilibrium syndrome can be seen, usually soon after or toward the end of dialysis. We report a patient with central pontine and extrapontine myelinolysis owing to disequilibrium syndrome. The patient had depressed consciousness, agitation, tremor, stupor and hyperactive deep tendon reflexes toward the end of the second peritoneal dialysis. A brain computed tomographic (CT) scan showed hypodense lesions in pontine and extrapontine locations without radiocontrast medium enhancement After 2 days, the patient had only minimal memory deficits. A control brain CT scan 1 week later showed a decrease of the lesions in central pontine and extrapontine locations. Central pontine and extrapontine myelinolysis should be suspected and investigated in the acute neurologic disorders of dialysis patients.

Combined treatment with subcutaneous interferon-alpha, oral isoprinosine, and lamivudine for subacute sclerosing panencephalitis.

We compared patients with subacute sclerosing panencephalitis who received treatment according to our protocol for at least 6 months (19 patients) with the patients who could not receive any treatment (13 patients). The treatment protocol consisted of oral isoprinosine (100 mg/kg/day), subcutaneous interferon alpha-2a (10 mU/m2/three times a week), and oral lamivudine (10 mg/kg/day). There were no statistical differences between the two groups according to Neurological Deficit Index, clinical stage, and average age on admission and also on the final evaluation after treatment. The mortality rates of both groups were similar: 3 (15.7%) for the treatment group and 6 (46%) for controls. The remission rates for the treatment and control groups were 7 of 19 (36.8%) and 0 of 13 (0%), respectively, and the difference was statistically significant (P = .036). The mean survival period of the treatment group was significantly longer than that of the control group (P = .01). In conclusion, this combination treatment protocol resulted in higher remission rates and longer survival periods when compared with controls, as well as a remission rate that was better than the spontaneous remission rate of 5%. For this reason, and as well as because interferon-alpha therapy has an easier route of application and a higher family compliance, we have considered this an alternative protocol for patients with subacute sclerosing panencephalitis.