Asian Study of Cerebral Venous Thrombosis.

BACKGROUND/OBJECTIVE: Most of the studies and registries related to cerebral venous thrombosis (CVT) are reported from European countries and the United States. The objective of the present study is to identify risk factors, presentation, and outcome of CVT in Asian patients. METHODS: Asian CVT registry is a prospective multinational observational study that included patients (aged > 16 years) with symptomatic CVT. RESULTS: Eight hundred and twelve patients (59% women) from 20 centers in 9 Asian countries were included. Mean age of the patients was 31 years. Motor weakness in limbs was present in 325 (40%) patients. One hundred and eighty (22.1%) patients had a normal Glasgow coma scale (GCS) at presentation, and another 529 patients (65%) had GCS between 11 and 14. The rest (103; 13%) had a GCS of less than 10 at presentation. Permanent risk factors were present in 264 (33%) patients, transient in 342 (42%) patients, both in 43 (5%) patients and no risk factors were found in 163 (20%) patients. Anemia was present in 51%, use of oral contraceptive pills (OCP) was present in 12% women and a hypercoaguable state was present in more than 40% of those tested. One hundred and forty-three cases (18%) were in women who were either pregnant (18; 2%) or in the puerperium (up to 6 weeks postpartum; N = 125; 15%). A total of 86 (10.5%) patients were diagnosed with infection in any part of the body. The most common MRI finding was local brain edema or ischemia (53.3%) followed by hemorrhage (26.7%). Twenty-seven patients (3.3%) died during hospital stay. The mRS score at discharge was available for 661 (81%) patients. Of these, 577 (87.3%) had good functional outcome at discharge. Motor weakness at presentation, GCS of 9 or less and mental status disorder were the strongest independent predictors of mortality at last follow-up among patients with CVT. CONCLUSIONS: Important differences were identified as compared to western data including younger age, high frequency of anemia, low use of OCP, and high frequency of hypercoaguable states. Functional outcome at discharge was good.

Occurrence of Hashimoto's thyroiditis among the patients with Sydenham's chorea.

AIM: The purpose of this study was to examine the possible association of Hashimoto's thyroiditis (HT) with Sydenham's chorea (SC). MATERIALS AND METHODS: A total of 25 SC patients and 25 patients with the diagnosis of HT were included in the study. Neurological, cardiac, radiological abnormalities, clinical findings, and biochemical analysis were evaluated. RESULTS: Heart murmur as a result of mitral valve deformation was present in all SC group patients. No neurologic and cardiac abnormalities were noted in HT group. Serum thyroid-stimulating hormone (TSH), anti-thyroid peroxidase, and anti-thyroglobulin levels were found to be high in 4 patients of the SC group and called as SC with HT group. Significant elevation of serum TSH levels in SC with HT group (31.75 ± 3.71 µU/ml) was observed when compared to HT group (12.60 ± 4.24 µU/ml, p < 0.05). CONCLUSION: These results showed that HT can be occurred among the patients with SC with cardiac involvement.

Prevalence of resistant occipital lobe epilepsy associated with celiac disease in children.

PURPOSE: Celiac disease (CD) is a chronic, inflammatory autoimmune disorder caused by intolerance to ingested gluten. Increased frequency of CD has been reported in occipital lobe epilepsy. The aim of the present study is to investigate the frequency of CD among children followed up due to epilepsy and diagnosed with epileptic activity in the occipital lobe in at least one electroencephalography (EEG) test. METHODS: For this research, 90 pediatric epilepsy patients with epileptic activity in the occipital lobe were enrolled in the study group, while the control group comprised of 100 healthy children. In addition to the EEG examination, tissue transglutaminase (tTG) antibody was determined on duodenal biopsy. RESULTS: None of the healthy children in the control group was positive in terms of the tTG antibody test used to scan CD. In the group with epileptic activity in the occipital lobe, two patients out of 90 were tTG antibody positive. The seroprevalence was 1/45 (2.22 %) in this group. These two patients were diagnosed with CD based on the endoscopic duodenal biopsy. In these patients, the seizures were uncontrollable through monotherapy. CONCLUSIONS: Our results showed that the prevalence of CD is observed to be higher than the normal population among the patients with occipital lobe epilepsy. This type of seizure disorder seems to be more resistant to monotherapy, compared with other types of occipital epilepsy. Therefore, screening for CD is recommended in children with resistant epileptic activity in the occipital lobe.

Headache in Cerebral Venous Thrombosis: incidence, pattern and location in 200 consecutive patients.

We analyzed the incidence, pattern and location of headache in consecutive 200 patients with proven diagnosis of CVT to identify an association between localization of headache and site of sinus involvement. Headache was present in 136 (68%) patients. The duration of headache (reported in 128 patients) was reported as acute (1-3 days), 81 patients (60%); sub-acute (4-14 days), 33 patients (24%); and chronic (more than 14 days), 14 patients (10%). The quality of headache (reported in 72 patients) was reported as throbbing 12 (9%), band like 27 (20%), thunderclap 7 (5%), and other (pounding, exploding, stabbing, etc.) 26 (20%). The location of headache (reported in 101 patients) was reported as unilateral (one side of head) 48 (37%), localized (frontal, temporal, occipital, and neck) 25 (19%), and diffuse (whole head) 28 (20%). 43 (32%) patients had normal neurological examination (normal mental status, cranial nerves, motor and sensory examination with down going planters). 93 (68%) patients have abnormal findings on neurological examination including papilledema 29 (15%) patients, altered mental status 38 (19%), and focal neurological deficit 45 (22%) patients. There was no association between headache and presence of hemorrhage on CT and MRI (P = 0.1) or hydrocephalus (P = 0.09). There was no association between localization of headache and site of sinus thrombosis except sigmoid sinus thrombosis, where 17 out of 28 patients with involvement of sigmoid sinus alone or in combination with transverse sinus had pain in the occipital and neck region (P < 0.05). There was no association between lateralization of pain and site of thrombosis (P = 0.66).

Effectiveness Oral Theophylline, Piracetam, and Iron Treatments in Children With Simple Breath-Holding Spells.

Breath-holding spells (BHS) are common nonepileptic paroxysmal events in children. This is a retrospective study to compare the effectiveness of oral theophylline, piracetam, and iron treatments in children with simple BHS. A total of 146 children (75 girls and 71 boys) with simple BHS were included to this retrospective study. Children were divided into 4 groups: nontreated (no anemia and no treatment), oral theophylline (10 mg/kg/d as a single daily dose), piracetam (40 mg/kg/d in 2 divided doses), and elementary iron (3 mg/kg/d as a single daily dose) treatments. Iron therapy had been given only in children with iron deficiency anemia. Neurologic, cardiologic, and biochemical evaluations were performed for all children. The majority of the patients had cyanotic spells (83.6%). The frequency of attacks/month was markedly decreased with iron (58.8%) and theophylline (82.9%) treatments, but not with piracetam therapy (8.8%) and nontreated group (4.7%). Satisfaction of the parents/caregivers was found to be high in the theophylline group (P < .001). Our results showed that theophylline was the most effective therapy to decrease the frequency of simple BHS in children.

Cerebral venous sinus thrombosis in children: a multicenter cohort from the United States.

This study presents a large multicenter cohort of children with cerebral venous thrombosis from 5 centers in the United States and analyzes their clinical findings and risk factors. Seventy patients were included in the study (25 neonates, 35%). The age ranged from 6 days to 12 years. Thirty-eight (55%) were younger than 6 months of age, and 28 (40%) were male. Presenting features included seizures (59%), coma (30%), headache (18%), and motor weakness (21%). Common neurological findings included decreased level of consciousness (50%), papilledema (18%), cranial nerve palsy (33%), hemiparesis (29%), and hypotonia (22%). Predisposing factors were identified in 63 (90%) patients. These included infection (40%), perinatal complications (25%), hypercoagulable/hematological diseases (13%), and various other conditions (10%). Hemorrhagic infarcts occurred in 40% of the patients and hydrocephalus in 10%. Transverse sinus thrombosis was more common (73%) than sagittal sinus thrombosis (35%). Three children underwent thrombolysis, 15 patients received anticoagulation, and 49 (70%) were treated with antibiotics and hydration. Nine (13%) patients (6 of them neonates) died. Twenty-nine patients (41%) were normal, whereas 32 patients (46%) had a neurological deficit at discharge. Seizures and coma at presentation were poor prognostic indicators. In conclusion, cerebral venous thrombosis predominantly affects children younger than age 6 months. Mortality is high (25%) in neonatal cerebral venous thrombosis. Only 18 (25%) patients were treated with anticoagulation or thrombolysis.

Association of Neutrophil/Lymphocyte Ratio With Intravenous Immunoglobulin Treatment in Children With Guillain-Barré Syndrome.

Guillain-Barré syndrome (GBS) is an acute immune-mediated inflammatory polyneuropathy of the peripheral nervous system. The authors aimed to investigate whether the neutrophil/lymphocyte (N/L) and platelet/lymphocyte (P/L) ratios are the parameters that associated with the drug treatment or severity of GBS. Twenty-seven children with GBS were retrospectively analyzed from the medical records of patients who attended to the Pediatric Neurology Department of the Gaziantep University Hospital. Biochemical and hematologic parameters were measured. Leukocytes, neutrophils counts and N/L ratio were significantly higher before the intravenous immunoglobulin treatment ( P < .001). However, there were no marked differences in platelet count and P/L ratio. In addition, marked correlation was observed between the N/L ratio after treatment and duration of weakness. The results of the study showed that N/L ratio is significantly higher in GBS patients, and reduces following with intravenous immunoglobulin treatment.

Idiopathic epilepsy of childhood and potassium ion channels.

The etiology of common idiopathic epileptic syndromes is genetically determined, but the complex pattern of inheritance suggests an epistatic interaction of several susceptibility genes. Mutations in over 70 genes now define biological pathways leading to rare monogenic forms of epilepsy in humans and animals. Recognizing the molecular basis of an ion-channel disease has provided new opportunities for screening, early diagnosis, and therapy of these conditions. Potassium can affect the development of common seizure type and can be defined seizure susceptibility allele. The existence of inward-rectifying potassium (Kir) channels was first recognized half a century ago. The biophysical fingerprint of Kir channels is inward rectification in the current-voltage relationship , which limits potassium efflux at depolarizing membrane potentials. Kir channels are essential in the control of resting membrane potential, coupling of the metabolic cellular state with membrane excitability, and maintenance of potassium homeostasis. The critical interval contains several candidate genes, one of which, KCNJ1O, exhibits a potentially important polymorphism with regard to fundamental aspects of seizure susceptibility. Deletion of KCNJ1O as a seizure susceptibility gene that code for inward rectifier potassium ion channels imparts protection against seizures results in spontaneous seizures and increased seizure susceptibility. The unique role of Kir channels in membrane physiology coupled with previous strong association between ion channel gene mutations and seizure phenotypes puts even greater focus on KCNJ1O. The major challenge of the future will be to recognize the molecular basis of a Kir-mediated channelopathy in order to screen, diagnose and treat these ion channel diseases.

Serial Casting as an Adjunct to Botulinum Toxin Type A Treatment in Children With Cerebral Palsy and Spastic Paraparesis With Scissoring of the Lower Extremities.

The purpose of this study was to examine whether combination therapy of serial casting and botulinum toxin type A injection can further enhance the effects of botulinum toxin type A in children with cerebral palsy with scissoring of both legs. This study was a prospective and randomized trial. The children were divided into 2 groups, one of which received serial casting after botulinum toxin type A (n = 40), and the other which only received botulinum toxin type A (n = 40). Serial casting started 3 weeks after the botulinum toxin type A. Both groups received physiotherapy. Groups were assessed at baseline then compared at 6 and 12 weeks following the intervention. Significant improvements in Gross Motor Function Measure-66 and Caregiver Health Questionnaire were recorded in both groups ( P < .001). The modified Ashworth scale improved significantly following botulinum toxin type A in the serial casting group ( P < .05), but not in botulinum toxin type A only group. These results suggest that serial casting after botulinum toxin type A can enhance the benefits of botulinum toxin type A in children with cerebral palsy.

Paediatric cerebral venous thrombosis.

Cerebral venous thrombosis (CVT) in children is a multifactorial serious disease. It is being increasingly diagnosed, mainly because of more sensitive diagnostic procedures and increasing clinical awareness. The clinical manifestations can be life-threatening and cause long-term neurological deficits. Thromboembolism in children is a multifactorial disorder in which both genetic and acquired risk factors play a role. CVT occurs in various clinical settings, including infection, dehydration, renal failure, trauma, cancer and haematological disorder with multiple risk factors. Clinical manifestations of CSVT are nonspecific and may be subtle. Most of the clinical scenarios occur at all ages and the clinician should consider this diagnosis in a wide range of acute neurological presentations in childhood. CVT can have an extremely variable clinical presentation, mode of onset, imaging appearance and outcome. Its prognosis remains largely unpredictable. Diffusion and perfusion MRI may play a role in detecting venous congestion and CT or MR venography are now the methods of choice for investigation of cerebral venous thrombosis. The options for treatment of infants and children include standard or low molecular weight heparin for 7-10 days followed by oral anticoagulants for 3-6 months. Specific treatment with anticoagulation is controversial in children, but has been established as appropriate therapy in adults. Anticoagulant treatment with heparin is probably safe and beneficial for children with sinus thrombosis, even those with intracranial haemorrhages.

Wernekink comissure syndrome: a rare midbrain syndrome secondary to stroke.

Wernekink commissure involves the decussation of superior cerebellar peduncle (SCP) in midbrain. We report an elderly hypertensive, diabetic man who developed slurred speech, ataxia, and internuclear ophthalmoplegia. MRI examination revealed an unusual ischemic stroke involving Wernekink commissure. This rare stroke pattern involving decussation of SCP occurs in the setting of small arterial disease. The association between the anatomic location of the stroke and clinical findings is noteworthy.

The Clinical Implications of Todd Paralysis in Children With Benign Rolandic Epilepsy.

The aim of this study was to describe the clinical and electroencephalographic (EEG) findings of postictal Todd paralysis in benign rolandic epilepsy of childhood and find out the possible correlation with migraine. Based on International Headache Society pediatric migraine criteria, patients were investigated for migraine, and 12 of the 108 patients with benign rolandic epilepsy (6 girls and 6 boys, 11.1%) were found to have postictal Todd paralysis. Ten of these 12 patients (83.3%) had pediatric migraine based on the diagnostic criteria. We showed comorbidity of migraine and benign rolandic epilepsy with postictal Todd paralysis in children. Increased incidence of migraine in the present study suggest that children who have benign rolandic epilepsy and postictal Todd paralysis are more likely to have migraines.

Comparison of Efficacy and Side Effects of Oral Baclofen Versus Tizanidine Therapy with Adjuvant Botulinum Toxin Type A in Children With Cerebral Palsy and Spastic Equinus Foot Deformity.

This retrospective study aimed to compare the therapeutic response, including side effects, for oral baclofen versus oral tizanidine therapy with adjuvant botulinum toxin type A in a group of 64 pediatric patients diagnosed with static encephalopathy and spastic equinus foot deformity. Following botulinum toxin A treatment, clinical improvement led to the gradual reduction of baclofen or tizanidine dosing to one-third of the former dose. Gross Motor Functional Measure and Caregiver Health Questionnaire scores were markedly elevated post-botulinum toxin A treatment, with scores for the tizanidine (Gross Motor Functional Measure: 74.45 ± 3.72; Caregiver Health Questionnaire: 72.43 ± 4.29) group significantly higher than for the baclofen group (Gross Motor Functional Measure: 68.23 ± 2.66; Caregiver Health Questionnaire: 67.53 ± 2.67, P < .001). These findings suggest that the combined use of botulinum toxin A and a low dose of tizanidine in treating children with cerebral palsy appears to be more effective and has fewer side effects versus baclofen with adjuvant botulinum toxin A.

Postictal paresis in children with benign rolandic epilepsy.

Following a search for the presence of postictal paresis in a cohort of 70 patients with benign rolandic epilepsy of childhood, the symptomatology of the seizures and the presence of postictal paresis were reviewed. All children underwent a neurologic evaluation, including electroencephalography (EEG) and neuroimaging. Eight of the 70 patients (3 girls and 5 boys) were found to have postictal paresis. All patients had partial motor seizures involving predominantly the upper extremities and, to a lesser degree, the face and lower extremities. In all eight patients, the motor deficits resolved within 60 minutes. Follow-up neurologic examination was nonfocal in all patients. Seven of the eight patients experienced postictal paresis once, and one patient had two such episodes. Three of the eight patients experienced a brief speech arrest. The EEG in all patients demonstrated centrotemporal sharp waves. In seven patients, the sharp waves were bilateral and independent, and in one patient, the rolandic sharp waves were unilateral. A horizontal dipole with positivity at the central region was found in all patients using an average montage. In conclusion, we found an 11.5% association of postictal paresis in children with benign rolandic epilepsy of childhood, whereas 38% of children also had a brief speech arrest. The EEG was characteristic for benign rolandic epilepsy of childhood with bilateral asynchronous discharges in seven of eight patients (83%) and the presence of dipole in all patients. The presence of postictal paresis should not exclude the diagnosis of benign rolandic epilepsy of childhood.

Maternal Iron Deficiency Anemia as a Risk Factor for the Development of Retinopathy of Prematurity.

BACKGROUND: Retinopathy of prematurity is a proliferative vascular disease affecting premature newborns and occurs during vessel development and maturation. The aim of this study was to evaluate the maternal iron deficiency anemia as possible risk factors associated with the development of retinopathy of prematurity among premature or very low birth weight infants. METHODS: In this study, mothers of 254 infants with retinopathy of prematurity were analyzed retrospectively, and their laboratory results of medical records during pregnancy were reviewed for possible iron deficiency anemia. RESULTS: In a cohort of 254 mothers of premature infants with retinopathy of prematurity, 187 (73.6%) had iron deficiency, while the remaining 67 (26.4%) mothers had no deficiency. Babies born to mothers with iron deficiency anemia with markedly decreased hemoglobin, hematocrit, mean corpuscular volume, serum iron, and ferritin levels were more likely to develop retinopathy of prematurity. CONCLUSIONS: Our results are the first to suggest that maternal iron deficiency is a risk factor for the development of retinopathy of prematurity. Our data suggest that maternal iron supplementation therapy during pregnancy might lower the risk of retinopathy of prematurity.

Contribution of KCNJ10 gene polymorphisms in childhood epilepsy.

The purpose of this study was to investigate the possible association between childhood epilepsy and KCNJ10 gene polymorphisms (rs61822012 and rs2486253). A total of 200 epileptic cases and 200 healthy controls enrolled to this study. Genomic DNAs from the patients and control cases were analyzed by polymerase chain reaction (PCR) and restriction fragment length polymorphism methods. There were significant associations between the G/T genotype of KCNJ10 gene rs2486253 polymorphism in the idiopathic generalized epilepsy group (P = .037) and in subjects with generalized tonic-clonic seizures (P = .0015). T allele was also increased in patients with generalized tonic-clonic seizures (P = .0158). However, no statistically significant association was found between rs61822012 polymorphism and epilepsy. Our data suggest that G/T genotype of the KCNJ10 gene rs2486253 polymorphism affects risk for development of common types of childhood epilepsy. The T allele of this polymorphism was found to be a seizure-susceptibility allele for tonic-clonic epilepsy.

Supratentorial primitive neuroectodermal tumors of infancy: clinical and radiologic findings.

One hundred ninety-eight children were entered on POG 8633, "Prolonged Postoperative Chemotherapy and Delayed Radiation for Children <3 years of age with Malignant Brain Tumors" (1986-1990). Thirteen manifested supratentorial nonpineoblastoma primitive neuroectodermal tumors, making this the second most common supratentorial tumor in the study. Symptoms and signs included seizures, nausea, vomiting, lethargy, irritability, headache, focal motor weakness, and increased head circumference. Twelve of 13 had symptoms for less than 1 month before diagnosis. The average tumor size was 5.96 cm (+/- 0.37) x 5 cm (+/- 0.28) x 5.15 cm (+/- 0.31). Eight tumors were predominantly hemispheral, and five were midline. Computed tomographic scans on nine patients revealed tumor hyperdensity (nine), midline shift (eight), hydrocephalus (seven), cysts (six), well-defined borders (five), and calcification (four). No patients exhibited peritumoral edema. Contrast enhancement, primarily heterogeneous, was present in all patients. Magnetic resonance imaging appearance of the tumor (six patients) demonstrated midline shift (four), well-defined margins (four), necrosis (two), cysts (three), and hemorrhage (two). No peritumoral edema was present. Most enhanced heterogeneously. The diagnosis of supratentorial nonpineoblastoma primitive neuroectodermal tumors should be suspected when a large, sharply marginated, hyperdense supratentorial mass is observed in a young child, particularly when no peritumoral edema is present.

Botulinum toxin type A with oral baclofen versus oral tizanidine: a nonrandomized pilot comparison in patients with cerebral palsy and spastic equinus foot deformity.

The objective of this study was to compare the effectiveness of baclofen versus tizanidine as adjuvant treatment of botulinum toxin type A botulinum toxin type A in the management of children with spasticity. Thirty children with gastrocnemius spasticity were retrospectively reviewed at Gaziantep University Hospital, Gaziantep, Turkey. All patients were treated with localized botulinum toxin injections and baclofen or tizanidine for spasticity and were followed at 2- to 4-week intervals and evaluated for a total of 12 weeks; 17 children (57%) received baclofen and 13 (43%) received tizanidine. The mean score of Gross Motor Functional Measurement (76.63 +/- 5.88 vs 68.17 +/- 1.99; P < .001) and caregiver questionnaire scores (70.23 +/- 4.76 vs 66.59 +/- 3.53; P = .03) for the tizanidine group were significantly higher as compared with the baclofen group. This study suggests that combination of botulinum toxin type A with oral tizanidine is more effective with fewer side effects than combination of botulinum toxin type A and oral baclofen for spastic cerebral palsy.