How often do neurological disorders lead to dizziness in childhood?

OBJECTIVE: The aim of the study was to determine the features and differential diagnosis of childhood dizziness and find out the prevalence of neurological diseases in children who were referred to the pediatric neurology outpatient clinic. A secondary aim was to evaluate the outcome of dizziness after 12 months. MATERIAL AND METHODS: The records of children with a complaint of dizziness that were referred to the pediatric neurology outpatient clinic were retrospectively reviewed, and detailed medical and family history, clinical characteristics, laboratory investigations including vitamin B12 levels, and neuroimaging tests were analyzed. Patients were grouped as neurological disorders and non-neurological disorders. Neurological disorders included vestibular migraine, benign paroxysmal vertigo, and epilepsy, and non-neurological disorders contained the remaining disorders. RESULTS: The study consisted of 60 children (36 females, 24 males) with a mean age of 11.7±4.1 years. The most common diagnoses were vestibular migraine (21.7%) and orthostatic hypotension (20%). We found that the incidence of neurological diseases was 40% (vestibular migraine, 21.7%; epilepsy, 10%; benign paroxysmal vertigo, 8.3%). When we compared the neurological disorders with non-neurological disorders, there was a significant difference in terms of age at onset and duration of attacks (p=0.001 for both), whereas no significant difference was detected in terms of gender, frequency of attacks, and vitamin B12 levels. We detected ongoing symptoms in 10% of the patients who were diagnosed with vestibular migraine and psychogenic dizziness. CONCLUSION: Both non-neurological and neurological diseases are common in etiology of children with dizziness.

Interictal epileptiform discharges on electroencephalography in children with methylenetetrahydrofolate reductase (MTHFR) polymorphisms.

OBJECTIVE: Methylenetetrahydrofolate reductase (MTHFR) is an important enzyme involved in folate metabolism. MTHFR C677T and A1298C polymorphisms are best-defined variants of MTHFR that were reported to be associated with epilepsy development. The aim of the study was to determine the incidence of interictal epileptiform discharges on electroencephalography (EEG) in asymptomatic children with C677T and A1298C polymorphisms who had no history of seizure. METHODS: Children with MTHFR C677T or A1298C polymorphisms who had normal neurological examination without a history of seizure were included in the study. Blood samples for serum folate, vitamin B12, and homocysteine levels were analyzed. Sleep and awake electroencephalograms (EEG) were recorded. RESULTS: A total of 102 children (50 girls and 52 boys) with a mean age of 59.4 ± 58.7 months were included in the study. Interictal epileptiform EEG discharges were detected in 3 children (2.9%). CONCLUSION: There was no increase in the prevalence of interictal epileptiform discharges in seizure-free and asymptomatic children with MTHFR C677T and A1298C polymorphisms.

DRESS Syndrome or Hematologic Malignancy?: A Case Report of a 4-Year-Old Boy.

Drug reaction with eosinophilia and systemic symptom (DRESS) is a serious idiosyncratic drug reaction. It is characterized by skin eruption, fever, hematologic abnormalities, and multi-organ involvement. Diagnosis is challenging because of the wide clinical spectrum. Its association with aromatic antiepileptic drugs, such as phenytoin, phenobarbital, and carbamazepine, has been well described in adults. There are few reports of DRESS syndrome in children, and knowledge about the relationship between new antiepileptic drugs such as oxcarbazepine and this syndrome is limited. The DRESS syndrome is a challenging entity and probably underdiagnosed because many of its clinical findings can mimic those of other serious systemic disorders such as infections and hematologic disorders. Virus reactivation and use of some drugs together with suspected drugs, such as amoxicillin, can trigger the symptoms of DRESS syndrome. This is a case report of a 4-year-old boy with oxcarbazepine-induced DRESS syndrome possibly triggered by amoxicillin; hematologic malignancy was included in the differential diagnosis.

The frequency of late-onset Pompe disease in pediatric patients with limb-girdle muscle weakness and nonspecific hyperCKemia: A multicenter study.

The aim of this multicenter study was to screen for late-onset Pompe disease in high-risk children with limb-girdle muscle weakness and nonspecific hyperCKemia using the dried blood spot (DBS) test. Seventy-two children from four pediatric neurology departments in Turkey were enrolled in the study: 37 with limb-girdle muscle weakness and 35 with nonspecific hyperCKemia. Acid α-glucosidase (GAA) activity was measured on DBS by tandem mass spectrometry. Six patients tested positively for Pompe disease. In three patients, one with the limb-girdle muscle weakness and two with nonspecific hyperCKemia, this was confirmed by genetic analysis. The overall frequency of late-onset Pompe disease in the study population was 4.2%. The c.1784C>T mutation found in one patient is a new mutation whereas the c.1655T>C mutation detected in the other two patients is not novel. In conclusion, Pompe disease should be suspected in patients with limb-girdle muscle weakness and nonspecific hyperCKemia. The DBS test is a safe and reliable method of diagnosis but must be confirmed by genetic analysis. In patients with a positive DBS test and negative genetic analysis, tissue assay of GAA should be considered.

INCREASED TISSUE TRANSGLUTAMINASE LEVELS ARE ASSOCIATED WITH INCREASED EPILEPTIFORM ACTIVITY IN ELECTROENCEPHALOGRAPHY AMONG PATIENTS WITH CELIAC DISEASE

Background - Celiac disease is an autoimmune systemic disorder in genetically predisposed individuals precipitated by gluten ingestion. Objective - In this study, we aimed to determine asymptomatic spike-and-wave findings on electroencephalography in children with celiac disease. Methods - A total of 175 children with the diagnosis of celiac disease (study group) and 99 age- and sex-matched healthy children as controls (control group) were included in the study. In order to determine the effects of gluten free diet on laboratory and electroencephalography findings, the celiac group is further subdivided into two as newly-diagnosed and formerly-diagnosed patients. Medical histories of all children and laboratory findings were all recorded and neurologic statuses were evaluated. All patients underwent a sleep and awake electroencephalography. Results - Among 175 celiac disease patients included in the study, 43 were newly diagnosed while 132 were formerly-diagnosed patients. In electroencephalography evaluation of patients the epileptiform activity was determined in 4 (9.3%) of newly diagnosed and in 2 (1.5%) of formerly diagnosed patients; on the other hand the epileptiform activity was present in only 1 (1.0%) of control cases. There was a statistically significant difference between groups in regards to the presence of epileptiform activity in electroencephalography. Pearson correlation analysis revealed that epileptiform activity in both sleep and awake electroencephalography were positively correlated with tissue transglutaminase levels (P=0.014 and P=0.019, respectively). Conclusion - We have determined an increased epileptiform activity frequency among newly-diagnosed celiac disease patients compared with formerly-diagnosed celiac disease patients and control cases. Moreover the tissue transglutaminase levels were also correlated with the presence of epileptiform activity in electroencephalography. Among newly diagnosed celiac disease patients, clinicians should be aware of this association and be alert about any neurological symptoms.

Contexto - A doença celíaca é uma doença sistêmica auto-imune em indivíduos geneticamente predispostos, precipitada pela ingestão de glúten. Objetivo - Neste estudo, tivemos como objetivo determinar achados de picos e onda assintomáticos na eletroencefalografia de crianças com doença celíaca. Métodos - Foi incluído um total de 175 crianças com o diagnóstico de doença celíaca (grupo de estudo) com idade e sexo correspondentes a 99 crianças saudáveis como controles (grupo controle) com o fim de determinar os efeitos da dieta livre de glúten nos resultados laboratoriais e na eletroencefalografia. O grupo de doença celíaca é subdividido em dois, com pacientes recém diagnosticados e anteriormente diagnosticados. Foram registrados históricos médicos e resultados laboratoriais de todas as crianças e foram avaliados os estados neurológicos. Todos os pacientes foram submetidos a um eletroencefalografia em sono e acordado. Resultados - Dos 175 pacientes com doença celíaca incluídos no estudo, 43 foram recém diagnosticados, enquanto 132 foram diagnosticados anteriormente. Na avaliação de eletroencefalografia dos pacientes a atividade epileptiforme foi determinada em 4 (9,3%) de recém diagnosticados e em 2 (1,5%) dos pacientes anteriormente diagnosticados; por outro lado, a atividade epileptiforme estava presente em apenas 1 (1,0%) dos casos de controle. Houve uma diferença estatisticamente significativa entre os grupos no que diz respeito à presença de atividade epileptiforme em eletroencefalografia. Análise de correlação de Pearson revelou que atividade epileptiforme na eletroencefalografia tanto no sono como na vigília foram positivamente correlacionados com níveis de transglutaminase tecidual (P=0,014 e P=0,019, respectivamente). Conclusão - Determinamos uma frequência de atividade epileptiforme aumentada entre pacientes recém diagnosticados com doença celíaca em comparação com pacientes anteriormente diagnosticados e casos de controle. Além disso, os níveis de transglutaminase tecidual também foram correlacionados com a presença de atividade epileptiforme na eletroencefalografia. Os clínicos devem estar cientes dessa associação e alertas sobre algum sintoma neurológico entre pacientes recentemente diagnosticados de doença celíaca.

Epilepsy classification and additional definitions in occipital lobe epilepsy.

AIM: To evaluate epileptic children with occipital lobe epilepsy (OLE) in the light of the characteristics of Panayiotopoulos syndrome and late-onset occipital lobe epilepsy of Gastaut (OLE-G). METHODS: Patients were categorized into six groups: primary OLE with autonomic symptoms (Panayiotopoulos syndrome), primary OLE with visual symptoms (OLE-G), secondary OLE with autonomic symptoms (P-type sOLE), secondary OLE with visual symptoms (G-type sOLE), and non-categorized primary OLE and non-categorized secondary OLE according to characteristic ictal symptoms of both Panayiotopoulos syndrome and OLE-G, as well as aetiology (primary or secondary). Patients were compared with regards to seizure symptoms, aetiology, cranial imaging, EEG, treatment and outcome. RESULTS: Of 108 patients with OLE (6.4±3.9 years of age), 60 patients constituted primary groups (32 with Panayiotopoulos syndrome, 11 with OLE-G, and 17 with non-categorized primary OLE); the other 48 patients constituted secondary groups (eight with P-type sOLE, three with G-type sOLE, and 37 with non-categorized sOLE). Epileptiform activity was restricted to the occipital area in half of the patients. Generalized epileptiform activity was observed in three patients, including a patient with Panayiotopoulos syndrome (PS). Only one patient had refractory epilepsy in the primary groups while such patients made up 29% in the secondary groups. CONCLUSION: In OLE, typical autonomic or visual ictal symptoms of Panayiotopoulos syndrome and OLE-G do not necessarily indicate primary (i.e. genetic or idiopathic) aetiology. Moreover, primary OLE may not present with these symptoms. Since there are many patients with OLE who do not exhibit the characteristics of Panayiotopoulos syndrome or OLE-G, additional definitions and terminology appear to be necessary to differentiate between such patients in both clinical practice and studies.

INCREASED TISSUE TRANSGLUTAMINASE LEVELS ARE ASSOCIATED WITH INCREASED EPILEPTIFORM ACTIVITY IN ELECTROENCEPHALOGRAPHY AMONG PATIENTS WITH CELIAC DISEASE.

BACKGROUND: Celiac disease is an autoimmune systemic disorder in genetically predisposed individuals precipitated by gluten ingestion. OBJECTIVE: In this study, we aimed to determine asymptomatic spike-and-wave findings on electroencephalography in children with celiac disease. METHODS: A total of 175 children with the diagnosis of celiac disease (study group) and 99 age- and sex-matched healthy children as controls (control group) were included in the study. In order to determine the effects of gluten free diet on laboratory and electroencephalography findings, the celiac group is further subdivided into two as newly-diagnosed and formerly-diagnosed patients. Medical histories of all children and laboratory findings were all recorded and neurologic statuses were evaluated. All patients underwent a sleep and awake electroencephalography. RESULTS: Among 175 celiac disease patients included in the study, 43 were newly diagnosed while 132 were formerly-diagnosed patients. In electroencephalography evaluation of patients the epileptiform activity was determined in 4 (9.3%) of newly diagnosed and in 2 (1.5%) of formerly diagnosed patients; on the other hand the epileptiform activity was present in only 1 (1.0%) of control cases. There was a statistically significant difference between groups in regards to the presence of epileptiform activity in electroencephalography. Pearson correlation analysis revealed that epileptiform activity in both sleep and awake electroencephalography were positively correlated with tissue transglutaminase levels (P=0.014 and P=0.019, respectively). CONCLUSION: We have determined an increased epileptiform activity frequency among newly-diagnosed celiac disease patients compared with formerly-diagnosed celiac disease patients and control cases. Moreover the tissue transglutaminase levels were also correlated with the presence of epileptiform activity in electroencephalography. Among newly diagnosed celiac disease patients, clinicians should be aware of this association and be alert about any neurological symptoms.

Epidemiological study of self-reported sleep problems in Turkish high school adolescents.

BACKGROUND: The aim of the present study was to investigate the frequency and correlates of a variety of sleep problems in adolescents. METHODS: A representative school-based sample of 3485 8th-12th graders was selected according to the cluster sampling technique. A sleep questionnaire and the Strengths and Difficulties Questionnaire were completed by the students along with their parents. Logistic regression analysis was used to identify the independent variables of insomnia, parasomnia and excessive daytime sleepiness (EDS). RESULTS: Initiation insomnia, maintenance insomnia, non-restorative sleep (NRS) and EDS were described by 12.4%, 10.7%, 9% and 9.7%, respectively. At least one parasomnia with a frequency above the median was reported by 23.4% (nightmare, 12.8%; sleep terror, 3.6%; sleepwalking, 2.5%; bruxism, 2.5%; and sleep talking, 10.9%). Insomnias and parasomnias were highly associated with each other. Female gender was related to maintenance insomnia, NRS and night terror. Emotional problems correlated with initiation insomnia, maintenance insomnia, NRS, EDS, nightmares and bruxism; hyperactivity/inattention with initiation insomnia, NRS and sleep talking. Conduct problems were associated with NRS, whereas poor school performance was associated with initiation insomnia. CONCLUSION: Self-reported sleep problems were prevalent and highly associated with each other and behavioral and emotional difficulties in Turkish adolescents.

Prevalence of celiac disease in Turkish children with idiopathic epilepsy.

OBJECTIVE: This study has examined the prevalence of celiac disease in Turkish children with idiopathic epilepsy. METHODS: Children with idiopathic epilepsy were screened for celiac disease using the IgA anti-tissue transglutaminase antibody and compared with the healthy control group in order to find the association of celiac disease (CD) with idiopathic epilepsy. Upper gastrointestinal endoscopy and small intestinal biopsies were offered to all antibody-positive patients. Findings : A total of 214 children with the diagnosis of idiopathic epilepsy and 166 healthy children as control group were studied. Of the patients recruited, 55.1% had generalized epilepsy, and 44.9% had partial epilepsy. In 33 patients with partial epilepsy, electroclinical features were consistent with a diagnosis of childhood partial epilepsy with occipital paroxysms (CPEO). Two of 33 patients with CPEO had positive IgA anti-tissue transglutaminase antibodies in serology. Pathological examination of small intestinal biopsy specimens showed total villous atrophy in both of them. The prevalence of celiac disease among children with idiopathic epilepsy and CPEO was 0.9% and 6%, respectively. CONCLUSION: The results of the present study revealed that prevalence of CD is increased in children with epilepsy. On the other hand, as high as 6% prevalence of CD among patients with CPEO found in this study should be kept in mind and the clinicians should be aware of this association.

A case of congenital disorder of glycosylation ia presented with recurrent pericardial effusion.

BACKGROUND: Inherited deficiency of phosophomannomutase (PMM2) causes a human glycosylation disorder known as Congenital Disorder of Glycosylation Ia. CASE PRESENTATION: Herein, we describe a case of congenital disorder of glycosylation Ia, presented with recurrent pericardial effusion and unusual findings of inverted nipples, fat pads, reduced deep-tendon reflexes and multisystem involvement. CONCLUSION: Congenital Disorder of Glycosylation Ia should be considered in children with developmental delay, those with multi-system disease involving neurologic, gastrointestinal, ophthalmologic, cardiac or endocrine systems. On the other hand, severe cardiac involvement may also be a feature of Congenital Disorder of Glycosylation Ia and diagnosed patients should also be evaluated in this respect.

Colchicine treatment in children with familial Mediterranean fever: is it a risk factor for neuromyopathy?

BACKGROUND: We cared for a 17-year-old adolescent with familial Mediterranean fever under colchicine treatment. Because of the increased creatinine kinase level (3937 U/L) observed in this individual, we planned to assess all pediatric patients with familial Mediterranean fever under colchicine treatment to detect any resultant neuromyopathy. METHODS: The study included 88 children with familial Mediterranean fever who were receiving colchicine. The patient with myopathy was not included in the study. Serum creatinine kinase levels were measured and nerve conduction studies were carried out in all patients. RESULTS: The study included 88 patients (47 female, 53.4%) with an average age of 10.1 ± 3.35 years. The average period of colchicine use was 28.25 ± 17.66 months. Side effects of colchicine were detected in 10 patients (11%)--as diarrhea in eight patients, leukopenia in one patient, and hair loss in one patient. Nerve conduction studies determined incidental carpal tunnel syndrome in only one patient. CONCLUSIONS: Our study did not suggest an elevated risk of neuromyopathy associated with the use of colchicine for familial Mediterranean fever.

Congenital cytomegalovirus infection and finger anomaly.

Congenital cytomegalovirus (CMV) infection is the most common cause of intrauterine infection. It affects the central nervous system and causes microcephaly, hydrocephalus, mental retardation, hearing disorder, chorioretinitis and rarely finger anomalies. We present the case of a 4-month-old girl with an agenesis of the middle finger and syndactyly of the fourth and fifth fingers as a result of congenital CMV; this is the first such case in the literature. Here, congenital CMV infection is examined along with finger anomalies.

L-2 hydroxyglutaric aciduria presenting with anxiety symptoms.

l-2 Hydroxyglutaric aciduria is a rare autosomal recessively inherited metabolic disorder of organic acid metabolism. Cerebellar and pyramidal signs with progressive neurological syndromes, mental deterioration, tremors, seizures, epilepsy and rarely macrocephaly are clinical findings of the disease. The diagnosis depends on increased levels of l-2 hydroxyglutaric acid in urine, plasma and cerebrospinal fluid. Brain MRI shows peripheral white matter abnormalities in cerebral hemispheres, bilateral symmetrically abnormal signal intensity in basal ganglia and dentate nuclei. In this case report, we present a 13-year-old patient who presented with tremors and anxiety symptoms and was diagnosed as l-2 hydroxyglutaric aciduria after consultation with the child neurology department. We present a patient suffering from psychiatric symptoms with a metabolic disorder.

Cerebral multicystic lesions in a child with neurofibromatosis.

Neurofibromatosis type 1 (NF-1) is an autosomal dominant neurocutaneous syndrome, with frequent involvement of the central nervous system (CNS). As well as abnormal cellular differentiation, disordered cell migration during development is the most common cause of the various brain lesions. Cystic lesions are rarely observed in neurocutaneous diseases, and the origin of the cysts is not known. This paper presents a rare case, a child at the age of 3, who was diagnosed as NF-1 and was observed to have asymptomatic cystic lesions in right temporal lobe in radiological examination of CNS. This study draws attention to the relationship between these rare cystic lesions of unknown origin and neurocutaneous diseases.

Celiac disease with pulmonary haemosiderosis and cardiomyopathy.

Celiac disease or pulmonary haemosiderosis can be associated with several distinguished conditions. Pulmonary haemosiderosis is a rare, severe and fatal disease characterised by recurrent episodes of alveolar haemorrhage, haemoptysis and anaemia. Association of pulmonary haemosiderosis and celiac disease is extremely rare. We describe a case of celiac disease presented with dilated cardiomyopathy and pulmonary haemosiderosis without gastrointestinal symptoms of celiac disease. In addition, vitamin A deficiency was detected. This case suggests that celiac disease should be considered in patients with cardiomyopathy and/or pulmonary haemosiderosis regardless of the intestinal symptoms of celiac disease.

Pseudohypoparathyroidism presenting with ventricular arrhythmia: a case report.

Pseudohypoparathyroidism (PHP) is a rare disorder characterized by varying degrees of unresponsiveness to parathyroid hormone. Patients usually present with hypocalcemia-induced seizures or tetany, whereas no case of hypocalcemia-induced cardiac arrhythmia in PHP has been described to date. In this paper, we report the case of a male adolescent with PHP type 1a who presented with hypocalcemia-induced ventricular extrasystoles (bigeminy, trigeminy) and mild corrected QT interval prolongation. The patient had brachydactyly and his second fingers and toes were longer than the others, a finding consistent with PHP. Laboratory tests detected hypomagnesemia, as well as elevated levels of creatine kinase and lactate dehydrogenase. Ventricular arrhythmia and abnormal laboratory tests improved with calcium supplementation and vitamin D treatment. The findings in this patient suggest that hypomagnesemia may make patients with PHP more susceptible to hypocalcemia and may thus prompt a state of hypocalcemia-induced arrhythmia or other cardiac complications.

[Two cases of rare cerebral hydatid cyst]. / Nadir Serebral Yerlesimli Hidatik Kist Olgusu.

Hydatid cyst disease (Echinococcosis) is a parasitic illness that is rarely located in the brain. Primary cerebral hydatid cyst disease is rarely seen. We report here rare two cases presenting with sixth cranial nerve palsy with increased intracranial pressure syndrome due to primary cerebral hydatid cyst. A 5-year-old female and a 13-year-old boy complained of headache, strabismus, nausea, and vomiting. Neurological examination revealed sixth nerve palsy and papilloedema. The diagnosis was cerebral hydatid cyst disease and was confirmed with radiological and pathological investigations. Both cases were operated on. The cysts were removed without rupture, and therapy was completed with albendazole for a period of six months. They were symptom-free during the follow-up period. In conclusion, cerebral hydatid cyst disease should be kept in mind in the differential diagnosis of increased intracranial pressure syndrome.