Alper I. Dai

Cerebral Venous Thrombosis: Analysis of a Multicenter Cohort From the United States

OBJECTIVE The data regarding diagnosis, prognosis, management, and outcome of patients with cerebral venous thrombosis are limited from the United States. METHODS Patients with diagnosis of cerebral venous thrombosis were identified by International Classification of Diseases, Ninth Revision coding system at 10 centers in the United States during a 10-year period by retrospective chart review (1991-1997) or prospective enrollment (1997-2001). In all, 232 patients were screened for study inclusion and 182 of these patients were included in the study. RESULTS The age range was 13 to 82 years (mean 38 years). Hypercoagulable state was the most common predisposing factor followed by pregnancy, malignancy, and homocystinemia. Neurologic examination revealed normal findings in 69 patients (38%); 37 (20%) were comatose, 59 (32%) had papilledema, and 71 (39%) had hemiparesis. In all, 61 patients (33%) had evidence of hemorrhage by computed tomography/magnetic resonance imaging. A total of 27 patients (15%) were treated with thrombolysis and 124 (68%) with anticoagulation. Overall mortality was 13% (n = 24). One-year follow up was available for 96 patients (53%). Of these, 26 (27%) were healthy, 43 (45%) were ambulatory with assistance, and 27 (28%) were still bedridden. On multivariate analysis, the best predictors of a poor outcome were coma at presentation (odds ratio 15.2 [95% confidence interval; 1.5-66]) and intracerebral hemorrhage (odds ratio 8.7 [95% confidence interval; 1.3-34.5]). CONCLUSION Clinical and radiologic presentation of cerebral venous thrombosis in the United States is not much different from other parts of world but spectrum and frequency of predisposing factors are different. Number of patients treated with thrombolysis is higher as compared with other reported series of such patients. Coma at presentation and intracerebral hemorrhage were the strongest predictors of poor outcome, which is comparable with other series.

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.

Ischemic Stroke in Young Asian Women: Risk Factors, Subtypes and Outcome

Background and Objective: The objective of our study was to describe risk factors, mechanisms and outcome of young Asian women with ischemic stroke. Methods: Twelve tertiary-care centers in 8 Asian countries participated. Women aged 15–45 years were included if they had an ischemic stroke supported by neuroimaging. Data on age, risk factor history, stroke mechanism and discharge status were collected. Results: A total of 958 subjects were included, their mean age was 34 years. Large-vessel thrombosis comprised 24%, cerebral venous thrombosis 21%, cardioembolism 19% and small-vessel thrombosis 15%. The stroke risk factors included hypertension (29%), diabetes (14%), pregnancy (11%), valvular heart disease (10%) and cigarette smoking (3%). Anemia was found in 42%, and mortality was 4%; at discharge, 17% had modified Rankin score (mRS) >4 and 83% mRS 0–3. Conclusion: Unlike among Caucasians, large-vessel thrombosis, cerebral venous thrombosis and cardioembolism are common among young Asian women with stroke. A high proportion are pregnancy-related. More studies are needed.

Thiamine-responsive megaloblastic anemia syndrome

Thiamine-responsive megaloblastic anemia (TRMA) syndrome usually associated with diabetes mellitus, anemia and deafness, due to mutations in SLC19A2, encoding a thiamine transporter protein. The onset of disease is usually seen during infancy or at early childhood and most of the TRMA patients are originated from consanguineous families. In this case, we report a 5-month-old boy who had diagnosis of TRMA during evaluations for his anemia and thrombocytopenia. The diagnosis of TRMA should be kept in mind in differential diagnosis of megaloblastic anemia especially in the populations where the consanguinity is frequent.

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.

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. (J Child Neurol 2005;20:834—836).

Diffusion-weighted magnetic resonance imaging in superior sagittal sinus thrombosis.

Background and Purpose. Diffusion‐weighted imaging (DWI) has shown high sensitivity in the diagnosis of acute arterial strokes. The pathophysiology of cerebral venous thrombosis with associated venous stroke appears to differ from that of arterial strokes. The purpose of this study was to describe DWI findings in venous strokes. Methods. The authors reviewed 3 adults with superior sagittal sinus thrombosis who underwent DWI and magnetic resonance imaging within 24 hours of symptom onset. DWI was obtained at 1.5 T using the multishot echo planar technique (TR = 8000, TE = 97, field of view = 30 ?19 cm, slice thickness = 6.0 mm, interslice gap = 0.5 mm, matrix 128 ?128, NEX = 1). The diffusion gradients were applied in 3 orthogonal directions with 3 increasing b values (0–1000 s/mm2) to create average (trace) DWI images. Apparent diffusion coefficient (ADC) values were calculated on a pixel‐by‐pixel basis and displayed as ADC maps. Results. DWI showed hyperintensities in patients 1 and 2 and hypointensity in patient 3 in corresponding to parenchymal lesions on conventional images. As compared to the homologous uninvolved location in the contralateral hemisphere, ADC values were decreased (0.53 ?10−3 mm2/s [patient 1] and 0.68 −3 mm2/s [patient 2]) and increased (1.1 ?10−3 mm2/s [patient 3]). The ADC ratio of the lesion in the involved to uninvolved side was 88% (patient 1), 81% (patient 2), and 120% (patient 3). Conclusion. Acute cerebral venous strokes may contain cytotoxic edema and/or vasogenic edema on DWI scans. DWI may be helpful in diagnosing cerebral venous thrombosis in cases with cryptic presentations.

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.

Prevalence and Clinical Significance of Antithyroid Antibodies in Children with Immune Thrombocytopenic Purpura

Background and objective: To determine the prevalence and the clinical significance of thyroid autoantibodies and their influence on treatment response in children with idiopathic thrombocytopenic purpura (ITP). Patient and Method: We retrospectively analyzed the antithyroglobulin (anti-TG) and antithyroid peroxidase (anti-TPO) antibodies from the records of 151 ITP patients who were admitted to the Pediatric Hematology Department of Gaziantep University between 2009 and 2012. Results: Anti-TPO and/or anti-TG was found positive in 38 (36.8%) of 103 patients whose thyroid autoantibody levels were measured. The comparison of positivity ratios of autoantibodies between acute and chronic ITP patients showed no significant difference. However, the separate comparison of each group of ITP patients with control group showed significantly high positivity ratios of autoantibodies in ITP patients. The initial mean platelet count of anti-TPO positive patients at diagnosis was significantly less than that of the negative patients (P = .008). One month after treatment, platelet count of anti-TPO positive patients was significantly less than that of the negative patients (P = .01). Moreover, the mean platelet counts of anti-TPO positive patients were significantly less than those of the negative patients after intravenous immunoglobulin treatment (P < .001). Conclusion: We demonstrated that the thyroid-autoimmune-diseases-related autoantibodies are frequently found in childhood ITP. Although no recommendation is found in international guidelines regarding screening for thyroid autoantibodies in patients with ITP, in view of the high incidence of antithyroid antibodies and their potential negative effect on treatment response, screening these patients for such antibodies would be recommended.

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.