Focal Convulsions Research Articles

A Retrospective Review of Efficacy and Tolerability of Carbamazepine in Children with Focal and Generalized Epilepsy: A Single Center Experience

Abstract Background Epilepsy has usually been defined as ≥ 2 unprovoked seizures separated by at least 24 hours. This definition is based on the concept that the recurrence risk after two unprovoked seizures is about 80%, while the recurrence risk after a single unprovoked seizure is only about 50%. Aim of the Work to retrospectively evaluate the efficacy and tolerability of carbamazepine as monotherapy and as adjunctive therapy in treatment of children with focal and generalized epilepsies respectively, and how it compares to other drugs. Patients and Methods Records of 100 pediatric patients with epilepsy who were 16 years old or less & received carbamazepine treatment or derivative (Group A) and 100 pediatric patients with epilepsy who did not receive carbamazepine treatment or derivative (Group B) (total equal to 200 patients). Both Groups A and B were be further subdivided into 3 subgroups: (1) patients with focal epilepsy on monotherapy (A1) with 9 patients & (B1) with 11 patients. (2) patients with focal epilepsy on polytherapy (A2) with 27 patients & (B2) with 9 patients. (3) patients with generalized epilepsy on polytherapy (A3) with 64 patients & (B3) with 80 patients. The following data were collected from patients’ records (detailed history regarding neonatal history, family history, detailed history of AED, detailed history of epilepsy including GASE scale (The Global Assessment of Severity of Epilepsy (GASE), aspects of the scale were rated on a 7-point Likert scale with descriptors for 1 representing “none or never” and 7 representing “extremely frequent, severe or high” Comparison regarding efficacy and tolerability was done as below: Within each subgroup of Group A: before and after CBZ use. Between each subgroup of Group A and Group B. i.e A1 VS B1 A2 VS B2 A3VS B3 It included the below items : 1. Efficacy Assessment: Subjective/Clinical: Control of seizures – partial or complete expressed as a percentage from the baseline before adding the specific drug. And by detecting seizure frequency difference (by subtracting seizure frequency after treatment from this before treatment) Objective GASE score before and after treatment.. Results 2. Tolerability : detected by noticing different side effects of all AED administrated and whether it was severe enough to stop the drug. 1. Both groups with focal monotherapy (A1 & B1) showed improvement regarding frequency of seizures when comparing each group with itself before & after treatment (p value 0.011 & 0.0003 respectively), however, comparison between both groups showed statistically significant result regarding final GASE severity score difference with much improvement in group A1 (p value 0.044) 2. Both groups with generalized seizures (A3 & B3) showed significant GASE severity score and seizure frequency improvement when comparing before and after treatment per each group (p value in group A3 <0.001 & <0.001 respectively & p value in group B3 <0.001 & <0.001 respectively). However, comparing the results of both groups together showed statistically significant results regarding seizure frequency difference with better results in the group B3 (p value <0.001). 3. Regarding groups (A2 &B2); Comparing before and after treatment in group A2 showed significant GASE severity score improvement (p value 0.000) while comparing before and after treatment in the group B2 showed significant seizure frequency improvement (p value 0.018). However, comparison between both groups showed statistically significant results regarding seizure frequency difference with better improvement in the group B2 (P value <0.001). Conclusion carbamazepine and its derivatives had superior efficacy as monotherapy for treating focal convulsions than other AEDs; on the other hand it had inferior efficacy as polytherapy in treating children with generalized convulsions.

Epidemiological, Clinical and Therapeutic Aspects of Neuropaediatric Emergencies in Children Aged 1 Month to 15 Years in the Paediatric Emergency Department of CHU-Gabriel Toure

Introduction: Paediatric neurological emergencies are a major public health problem worldwide, in Africa and in Mali. Despite the scale and complexity of paediatric neurological emergencies, there is little data in the literature in Africa and particularly in Mali on these neurological disorders in paediatrics. Aim: The aim of our study was to examine the epidemiological, clinical and therapeutic aspects of neuropaediatric emergencies in children aged between 1 month and 15 years in the paediatric department of the CHU-Gabriel Touré in 2021. Patients and Methods: This was a cross-sectional study conducted over a twelve-month period (01 January to 31 January 2021) in children aged 1 month to 15 years hospitalised in the paediatric emergency department for a neurological emergency. Results: The frequency of hospitalization was 18.35%. The age group 12 to 60 months accounted for 43.7%. The sex ratio was 1.44. Convulsion was the most frequent reason for hospitalisation (49.3%). Delayed psychomotor development was noted in 4%. Around 3% of the children had not been vaccinated. On clinical neurological examination, the signs observed were : Altered consciousness (88.3%), convulsion (61%), and axial hypotonia (29%). Focal convulsions accounted for 59%, and 45.7% were febrile. The main diagnoses were neuromalaria, hyperpyretic convulsion and meningitis (68.3%, 7% and 6.3% respectively). The death rate within the first 24 hours was 48%. The average length of hospitalisation was 5.03 +/-4.5 days, with extremes of 1 and 21 days. Conclusion: Neuropaediatric emergencies are a major paediatric health problem. They are frequent and contribute substantially to infant and child mortality in our context.

Isolated Cortical Vein Thrombosis: A Case Report

Isolated cortical vein thrombosis (ICVT) is a rare entity of cerebral venous thrombosis (CVT) that represents a third leading cause of death during pregnancy and puerperium. IVCT manifests with wide spectrum of symptoms including headache, seizure, focal neurological deficits and changed mental status, makes the diagnosis challenging. Pregnancy, puerperium, lumbar puncture, trauma and infection, genetic predisposing and acquired prothrombotic causes, all are well recognized risk factors for ICVT. Although no established universal consensus for management, IVCT generally treated by anticoagulant. In this paper, a case of IVCT reported in 25 years old postpartum mother, delivered by cesarean using epidural anesthesia. The case presents by persisting non-orthostatic headache in first few days post-delivery that treated as post dural puncture headache but not improved. On day 7 post-delivery developed multiple episodes of focal convulsion progressed to generalized tonic-clonic convulsion associated by left hemi-paresis. MRI gradient echo sequences showed liner hypo-signal lesion with configuration of cortical vein over right high parietal area. The MRV showed multiple small filling defects along the right cortical vein in favor IVCT. The patient treated successfully by enoxaparin (according her body weight) followed by warfarin for 3 months with complete resolution of the neurological deficit.

A Narrative Review of the Neurological Manifestations of Human Adenosine Deaminase 2 Deficiency

Deficiency of human adenosine deaminase type 2 (DADA2) is a complex systemic autoinflammatory disorder characterized by vasculopathy, immune dysregulation, and hematologic abnormalities. The most notable neurological manifestations of DADA2 are strokes that can manifest with various neurological symptoms and are potentially fatal. However, neurological presentations can be diverse. We here present a review of the neurological manifestations of DADA2 to increase clinical awareness of DADA2 as the underlying diagnosis. We reviewed all published cases of DADA2 from 1 January 2014 until 19 July 2022 found via PubMed. A total of 129 articles describing the clinical features of DADA2 were included in the analysis. Six hundred twenty-eight patients diagnosed with DADA2 were included in the review. 50.3% of patients had at least signs of one reported neurological event, which was the initial or sole manifestation in 5.7% and 0.6%, respectively. 77.5% of patients with neurological manifestations had at least signs of one cerebrovascular accident, with lacunar strokes being the most common and 35.9% of them having multiple stroke episodes. There is a remarkable predilection for the brain stem and deep gray matter, with 37.3% and 41.6% of ischemic strokes, respectively. Other neurological involvement included neuropathies, focal neurological deficits, ophthalmological findings, convulsions, and headaches. In summary, neurological manifestations affect a significant proportion of patients with DADA2, and the phenotype is broad. Neurological manifestations can be the first and single manifestation of DADA2. Therefore, stroke, encephalitis, posterior reversible encephalopathy syndrome, mononeuropathy and polyneuropathy, and Behçet’s disease-like presentations should prompt the neurologist to exclude DADA2, especially but not only in childhood.

Tranexamic acid-induced focal convulsions after spinal surgery: a rare case report and literature review on side effects of accidental spinal administration of tranexamic acid.

Convulsions are a neurological illness that has complexity. In clinical treatment, drug-induced convulsions appear from time to time. Drug-induced convulsions often begin as isolated acute seizures but may progress to persistent seizures. In orthopedics, topical administration of tranexamic acid is commonly used in conjunction with intravenous drip to achieve hemostasis during artificial joint replacement surgery. However, side effects induced by tranexamic acid accidental spinal administration should be taken seriously. We report a case of a middle-aged male treated with tranexamic acid locally in combination with intravenous drip for intraoperative hemostasis when undergoing spinal surgery. The patient had involuntary convulsions in both lower limbs after the operation. After symptomatic administration, the symptoms of convulsions gradually resolved. During the follow-up, the convulsions never occurred again. We reviewed the literature on cases with side effects of local tranexamic acid application in spinal surgery and discussed the mechanism of tranexamic acid-induced convulsions. Tranexamic acid is associated with an increased incidence of postoperative seizures. However, many clinicians are unaware that tranexamic acid causes seizures. This rare case summarized the risk factors and clinical features of these seizures. Moreover, it highlights several clinical and preclinical studies that offer mechanistic insights into the potential causes and treatments for tranexamic acid-associated seizures. A clear understanding of tranexamic acid-induced convulsions-related adverse reactions can help the first-line clinical screening of causes and adjustment of drug treatment. This review will aid the medical community by increasing awareness about tranexamic acid-associated seizures and translating scientific findings into therapeutic interventions for patients.

Convulsion in Children with Down syndrome at Benghazi Children Hospital (2020-2021)

Introduction: Down syndrome (DS) (trisomy 21) is the most frequent chromosomal cause of mental retardation, with a frequency rate of about 1 in 700 births. The prevalence of epilepsy in subjects with DS is reported to range from 1% to 13%, higher than in the general population but lower than in patients with mental retardation. Aim: To find the prevalence of convulsion in Down syndrome patients attending pediatric hospital at Benghazi and to find the associated factors related to convulsion in Down syndrome patients. Methodology: cross –sectional study included all Down syndrome patients admitted to pediatric hospital during two years period ( 2020- 2021) , and Patients attending both genetic and neurological clinics during the same years. Results: Total number of cases was 150; History of convulsion was positive in (18)12% of Down syndrome patients. Majority (89%) of patients had generalized tonic clonic, 5.5% had focal convulsion, and 5.5 % had infantile spasm, all patients were in treatment and had good response. Males constitute to 61% of all patients with male to female ratio 1.6:1. Nearly half (50.4%) of male patients their age was 1- 5years, while 43.8% of females was in same age group. Majority of mothers age were > 32years, 88% of all mothers their age 30years or above. All patients with convulsion had no complication during delivery, while 9% of patients without convulsion had complications. More than half (61.5%) of the patients the convulsion started before the age of one year. Results of EEG were abnormal in 25% of patients with convulsion. Conclusion: Normal ECHO was found in 33% of all cases, Atrial septal defect was recorded in 27% of patients with convulsion and 19.5% of patients without convulsion, Ventricular septal defect and Atrial septal defect in 15.4% of patients with convulsion and 10.3% of patients without convulsion, Patent ductus arterious was in 11.5% of patients with convulsion and 3.2%of patients without convulsion. More than .........

High Concentration of Protein Oxidation Biomarker O-Tyr/Phe Predicts Better Outcome in Childhood Bacterial Meningitis.

Neuronal damage in bacterial meningitis (BM) partly stems from the host´s inflammatory response and induced oxidative stress (OS). We studied the association of cerebrospinal fluid (CSF) biomarkers indicating oxidative damage to proteins with course of illness and outcome in childhood BM in Angola. Ortho-tyrosine/phenylalanine (o-Tyr/Phe), 3-chlorotyrosine/para-tyrosine (3Cl-Tyr/p-Tyr), and 3-nitrotyrosine/para-tyrosine (3NO2-Tyr/p-Tyr) concentration ratios were measured in 79 BM admission CSF samples, employing liquid chromatography coupled to tandem mass spectrometry. Besides death, disease outcomes were registered on Day 7 of treatment and one month after discharge (control visit). The outcome was graded according to the modified Glasgow Outcome Scale (GOS), which considers neurological and audiological sequelae. Children with a o-Tyr/Phe ratio below the median were more likely to present focal convulsions and secondary fever during recovery and suboptimal outcome (GOS < 5) on Day 7 and at control visit (odds ratio (OR) 2.85; 95% CI 1.14-7.14 and OR 5.23; 95% CI 1.66-16.52, respectively). Their most common sequela was ataxia on Day 7 and at control visit (OR 8.55; 95% CI 2.27-32.22 and OR 5.83; 95% CI 1.12-30.4, respectively). The association of a higher admission CSF o-Tyr/Phe ratio with a better course and outcome for pediatric BM points to a beneficial effect of OS.

MRI Abnormalities of the Brain After Complex Febrile Seizures in Children.

Background Though simple febrile seizures do not cause significant and lasting neurological deficits, complex febrile seizures (CFS) can result in neurologic sequelae.Because CFS causes cortical focal injuries and other brain lesions, itneeds to be evaluated. Objective The objective of this study was to evaluate the MRI brain changes in CFS, the incidence of seizures in children aged six months to five years, and their severity in relation to MRI findings of the brain. Methods In this observational study, 36 children aged six months to five years, having fever with seizure, and fulfilling the criteria of CFS were enrolled within 48 hours of the episode. Detailed clinical and neurological examinations were performed with MRI scans to find out the probable CNS lesions of CFS. Two radiologists separately evaluated all MRI brains. Results MRI lesions of the brain were found in11 (30.56%) CFS patients. Generalized tonic-clonic seizures (GTCS) (n=31, 86.11%) were the most common type of seizure among CFS cases. Cortical focal hyperintensity (CFH) (42.1%) was the most common MRI presentation. Positive MRI findings were significantly associated with focal convulsions (n=5, 100%) as compared to GTCS cases (n=6, 19%) (p=0.001). Conclusions CFH is the most common abnormality in the MRI brain amongCFS cases. CFS patients with focal seizures or prolonged seizures in 24 hours have higher abnormal neuroimaging findings. MRI should be considered a preferred investigatory tool for detecting CNS pathology in CFS cases. Follow-up studies are needed to determine the long-term outcomes of CNS lesions in children with CFS.

ALCOHOL WITHDRAWAL IS COMMONLY ENCOUNTERED IN GENERAL HOSPITAL SETTINGS – A FIVE-YEAR EXPERIENCE IN A MEDICAL COLLEGE

Background: Alcohol withdrawal seizures (AWS) are dened as adult-onset seizures that occur in the setting of chronic alcohol dependence. alcohol withdrawal syndrome (AWS) is a combination of varied physiological manifestations that occur as a response to the sudden cessation or reduction of alcohol consumption. A total of 250 consecutive patients presenting wit Materials and methods: h seizures to either emergency room or out-patient to department of General Medicine, SVS Medical College Mahabubnagar, Telangana state for ve years from rst August 2014 to 31st July 2019. All the cases were thoroughly investigated with neuroimaging and electroencephalogram apart from a detailed history and clinical examination. 74 cases (29.6%) fell in the category of alcohol withdrawal seizures. Patients were initially screened for alcohol use, using AUDIT, a 10-item questionnaire designed to detect hazardous and harmful drinkers that has been validated and is extensively used. The data was analyzed using SPSS 20.0 package for Windows. 42 male and 32 female were included in this Results: study. The mean age was 22.26 [10.46] while mean duration of alcoholism was 14 years [8.98]. 41 were from rural background, 60 were earning while 14 were dependent; 24 cases were unmarried and 42 were from nuclear family. 62 cases used to consume Indian made foreign liquor (IFL). These demographic differences did not have any change the presentation pattern of AWS. Anxiety agitation (51) was most noted followed by disorientation (48) and headache (46). GTCS was noticed in 68 (91.89 %) and focal ts were recorded in only 6 patients. Five cases of GTCS turned to status epilepticus as only focal convulsion landed in status epilepticus. CT scan of brain demonstrated atrophy in 22. All the cases responded well to parental benzodiazepines. Conclusion: 74 Cases of seizures were reported in this present study. 32(43.24%) female cases also presented with seizures as against no case in female reported earlier. Cerebral atrophy was recorded in this series in 22 while only 8 had seizure like activity in EEG recording.

Neonatal Encephalitis and White Matter Injury in an Early Neonate: Cause or Association With COVID-19 Infection?

Coronavirus disease 2019, caused by severe acute respiratory syndrome coronavirus 2 (SARS CoV-2) has demanded world’s attention. Though this disease affects all age groups, for reasons unknown at this time, neonates and children seem to be at a lower risk of severe infection. We report the first case of neonatal SARS CoV-2 encephalitis in Pune, India and review the related published articles. A 4-day-old, previously healthy, full-term neonate was hospitalized for poor feeding and repeated episodes of focal convulsions since 104 h of life. Keeping in mind the rampant community transmission, SARS-CoV-2 rapid antigen detection and polymerase chain reaction testing was done, where both turned out to be positive. There were elevated inflammatory markers, cerebrospinal fluid pleocytosis, and acute splenial lesions in the magnetic resonance imaging of the brain consistent with neonatal SARS CoV-2 encephalitis. The baby recovered completely with no recurrence and was discharged home in 2 weeks.

Clinical characteristics, treatment, and long-term outcomes in children suffering from benign convulsions with mild gastroenteritis: a retrospective study

BackgroundBenign convulsions with mild gastroenteritis (BCWG) is a common condition in children in Asia and is generally not associated with pH or electrolyte imbalances. When BCWG is diagnosed, a lumbar puncture is usually recommended to rule out potential intracranial infections. This study examined the clinical characteristics of BCWG and evaluated the necessity of lumbar puncture.MethodsMedical records of children admitted to the First Hospital of Jilin University with BCWG between January 2018 and May 2019 were reviewed and analyzed. Children were stratified by rotavirus positivity or lumbar puncture status. Clinical characteristics and long-term outcomes were compared between groups.ResultsA total of 51 children were included in the analyses (55.1% rotavirus [HRV] positive). The average age of convulsion onset was 21.12 ± 7.44 months, the male-to-female ratio was 1.8:1, and convulsions occurred primarily between October 2018 and April 2019. The main clinical presentations of BCWG were convulsions, vomiting, diarrhea, and fever. Convulsions occurred predominantly two days after diagnosis of gastroenteritis, were mainly generalized tonic-clonic with 88.2% of children having ≤ 3 convulsions per episode, and had a mean duration of 2.0 minutes (interquartile range [IQR]: 1.0, 3.0). Children with BCWG had mild metabolic acidosis (HCO3− 17.82 ± 3.63 mmol/L) with an elevated anion gap (AG; 20.98 ± 3.00 mmol/L), mild hyponatremia (134.56 ± 2.85 mmol/L), and slightly increased levels of creatine kinase myocardial band (CKMB). HRV + children had more severe acidosis and higher CKMB levels. Cerebrospinal fluid (CSF) samples collected via lumbar puncture were normal. No developmental abnormalities were noted as assessed by the Social Life Ability Scale.ConclusionsBCWG is a situation-related seizure, with clinical presentations of tonic-clonic or focal convulsions and mild gastroenteritis (vomiting, diarrhea). Mild metabolic acidosis and hyponatremia may exist. The prognosis of the disease is favorable; lumbar puncture and long-term antiepileptics are unnecessary and should not be recommended.

To observe the clinical presentation of seizure disorder in 0-1 month age group

This prospective study was conducted over the period of one year at M.Y. Hospital &amp; Chacha Nehru Bal Chikitsalaya, Indore, in the Department of Pediatrics, M.G.M. Medical College, Indore.Detailed history of present illness with duration of convulsion were enquired. A detailed history of mother during antenatal, natal and post natal period was taken. Type of delivery (vaginal/ LSCS ; full term / preterm), enquired about the indication of LSCS. History of labour with particular ref to birth injury, asphyxia (delayed cry), neonatal history regarding jaundice &amp; feeding history, detailed family history in relation to epilepsy convulsion and mental diseases were recorded. Out of 100 cases 64% were males and 36% were females. Male female ratio was 1.77:1. Male cases were predominantly present in all 3 age groups. Majority of seizures occurred within 24 hrs. P value is not significant. Out of 100 cases 43 had tonic convulsions, 38 had subtle, and 19 had focal convulsions.43 neonates had seizures in 1st 24 hrs. Subtle seizures (60%) were most common followed by tonic seizures (32.5%) and then focal seizures (7.5%). In 2 – 7 day age group tonic seizures (42.8%) were most common followed by focal seizures (38%). In &amp;#62; 7 day age group also tonic seizures (73.6%) were most common. P value was &amp;#60; 0.001. Detailed clinical examination was done &amp; investigations were carried out in all patients. Cranial USG, EEG, &amp; CT scan were done as per requirement. Out of total 100 cases 74 were full term &amp; 26 were preterm. In our study maximum no. of cases developed seizure within 1st 24 hrs (43%) of life followed by 42% in 2–7 day age group &amp; 15% after 7 days. Convulsions were more common in males (64%) in all age group.

Subdural hematomas in young children: clinical and electrophysiological features

Subdural hematoma is a sufficiently rare, but serious pathology of the brain in infants, which can lead to severe neurological deficit or result in death. Subdural hematomas are detected in 2025 per 100,000 children under 1 year. A retrospective analysis of autopsy material revealed that subdural hematomas were diagnosed in 72% of children who died before the age of 5 months from intracranial hemorrhage. According to localization, subdural hematomas are divided into supra- and subtentorial, mainly associated localization, which are located along the tent of cerebellum and sickle of the brain, mainly. The main mechanism of development of subdural hematomas is associated with the rupture of the bridge veins of the subdural space as a result of their tension, both traumatic etiology and nontraumatic brain damage, accompanied by progressive cerebral atrophy. It is believed that perinatal hypoxic-ischemic brain damage is one of the leading etiological factors of developed subdural hematomas in young children. In addition, the formation of subdural hematomas in young children can be observed with intraamniotic infections, congenital fermentopathies, and above all, in children with aciduria. In some infants, subdural hematomas occur without clinical manifestation, but in most cases are accompanied by the development of neurological disorders, both in acute and in distant periods. In the main, subdural hematomas in the acute period manifest with focal convulsions with secondary generalization of seizures, behavioral disturbances, respiration, and symptoms of intracranial hypertension. During the formation of chronic subdural hematoma, development of structural epilepsy (up to 20%), microcephaly, impaired psychomotor development is observed. In 55% of young children with acute subdural hematomas, the formation of chronic subdural hematomas is observed.

A Case in Which Focal Convulsion Was the Initial Sign of Fatal Aortic Dissection.

Focal convulsion as an initial sign of aortic dissection is extremely rare. Type A aortic dissection involves the aortic arch, which may result in seizure either through the extension of the dissection into the common carotid arteries or through thromboembolism or cerebral hypoperfusion. Physicians should perform whole body computed tomography to determine whether or not dissection is present when treating patients with convulsion and a high level of fibrin degradation products.

A study of risk factors and clinical outcome of stroke in children

Background: Stroke is a significant cause of neurological morbidity and mortality in children. Early recognition permits timely therapeutic intervention aimed at preventing recurrence and improving long-term outcome. The objective of this study was to determine the risk factors, clinical outcome of stroke in children and to evaluate any association between them.Methods: This was a prospective, observational study, conducted over one and a half year at a tertiary care centre. Children between 1 month to 12 years and diagnosed to have stroke were enrolled. The clinical profile, investigations and outcome of stroke were studied. Data collected was analysed using Pearson chi-square test.Results: Of 55 children enrolled, definite etiology could be determined in 44 (80%) children. The common risk factors identified were vasculitis (30.9%), vasculopathy (23.6%), hypercoagulable states (18.1%), cardiac diseases (12.7%) and bleeding diathesis (7.2%). Focal neurologic deficits (69%), convulsions (58%), altered sensorium (41.8%), fever (45.4%) and aphasia (34.5%) were the chief presenting features. The overall mortality was 30.9% and neurologic deficits were observed in 54.5%. Children with undetermined etiology had a favorable outcome compared to those with definite etiology (p=0.007). Poor outcome was witnessed with vasculitis and cardiac diseases; recurrence was common with Moyamoya disease. Presence of either of altered sensorium (p=0.0005) or convulsions (p=0.046) or visual complaints (p=0.032) or headache (p=0.042) were associated with poor outcome whereas presence of focal neurologic deficits alone predicted a better outcome (p=0.036).Conclusions: The nature of risk factor and the clinical presentation significantly influence the outcome of stroke in children.

Progression in size of an arterio-venous malformation

We report a case of a young girl who was admitted with history of sudden onset left hemiplegia, when she was 5 years old. Investigations revealed no vascular malformation or abnormality. On conservative treatment, she progressively improved and had residual hemiparesis. She continued to have intermittent episodes of generalized and focal convulsions. For persistent convulsions, she was investigated again with MRI and angiography after 5 years of first presentation. Investigations at this time revealed a large arteriovenous malformation in the left posterior frontal region. The arteriovenous malformation was resected by surgery. Following surgery, the episodes of convulsions reduced. Arteriovenous malformations are generally considered to be congenital lesions and their size and physical architecture generally remains constant. The presented case clearly demonstrated de novo appearance or growth of the size of the arteriovenous malformation. Such a pattern of development and growth of a arteriovenous malformation is rare and only infrequently reported.

Brain tuberculoma, an unusual cause of stroke in a child with trisomy 21: a case report

BackgroundTuberculosis remains a public health problem in developing countries and is associated with lethal central nervous system complications. Intracranial tuberculomas occur in 13% of children with neurotuberculosis. Patients with trisomy 21 have an increased risk for stroke, which usually stems from cardiovascular defects.Case presentationWe report a case of a 12-year-old Sudanese boy with trisomy 21 who was presented to our hospital with focal convulsions and right-sided weakness. The results of neuroimaging and histopathological examinations were consistent with cerebral tuberculoma. The patient had a good initial response to antituberculosis drugs and steroids. To the best of our knowledge, this is the first case report of multiple brain tuberculomas described in a child with trisomy 21.ConclusionsPatients with trisomy 21 have an increased risk for stroke. Our patient had an exceptional case of stroke caused by tuberculoma. The present case emphasizes the need to consider tuberculomas in the differential diagnosis of children with neurological symptoms living in areas of high tuberculosis incidence.

An unusual cause for focal convulsions: Menkes kinky hair disease

A 6-month-old male infant was admitted to hospital with the history of repeated attacks of focal convulsions since 1 day prior to admission. He was also suffering from frequent attacks of cough and cold since 5 months of age, which was marked prior to admission. The infant had fair complexion, sparse fuzzy woolly hair with marked trunkal hypotonia. Serum copper and ceruloplasmin levels were low, magnetic resonance imaging showed diffuse cerebral atrophy with ill-defined areas of encephalomalacia. Microscopic examination of hair revealed pili torti. The patient was diagnosed as Menkes disease and treated symptomatically. For lack of facilities, we were not able to do magnetic resonance angiography and genetic study.

PP15.9 – 2371: Pseudotumor cerebri: A single center experience in 24 patients

Objective Pseudotumor cerebri is a syndrome characterized by signs and symptoms of high intracranial pressure without radiological evidence of intracranial mass. Our aim is to present data of patients diagnosed with pseudotumor cerebri in our department. Methods We analyzed the epidemiology, etiology and follow-up of 24 consecutive patients. Results Twenty-four patients were included in the study. There were 14 girls (58.3%), with an average age at presentation of 10.2±3.9 years (3–17 years). The major symptom at presentation was headache (84%, n=21). Two patients (8%) presented with focal convulsion only and six patients (25%) had unilateral abducens paralysis at presentation. Five of these patients (20%) had other complaints like blurred vision, vomiting or convulsion in addition to headache. When classified according to etiology, 10 patients (41.7%) were overweighted. Nine of these patients were prepubertal girls. Sinus vein thrombosis was detected in five patients (20.8%) and two of these patients were males diagnosed with Behcet's disease. Etiology was unknown in nine patients, one of whom was diagnosed with Coffin Siris Syndrome. The average number of lumber puncture (LP) performed per patient was two (range 1–10). The average time between two LPs was 4.1 months (0.3–10 m). Acetozolamide was used in all of the patients. Four patients (16.7%) underwent ventriculoperitoneal shunt procedure. Mean follow up time was 10.3 months (0.5–36 m). Mean time to resolution of symptoms was 90 days (7–300 d). Patients who had surgical operation had complete recovery. Patients under medical treatment had exacerbations and resolution periods. No patient had permanent visual loss. Conclusion With increased awareness, patients with complaints of headache or the other symptoms of increased intracranial pressure are more commonly referred to pediatric neurology clinics.

Nitric oxide synthase expression correlates with death in an experimental mouse model of dengue with CNS involvement

BackgroundThe clinical presentation of dengue is classified by the World Health Organization into dengue without warning signs, dengue with warning signs and severe dengue. Reports of neurological disease caused by Dengue virus (DENV) are becoming frequent, with symptoms that include reduced consciousness, severe headache, neck stiffness, focal neurological signs, tense fontanelle and convulsions. However, the immune mechanisms involved in neurovirulence remain poorly understood. Here we present a mouse model in which one genotype of DENV is inoculated by the intracranial route and infects C57/BL6 mice and replicates in the brain, causing death of mice.MethodsMice were infected with different serotypes/genotypes of DENV by the intracranial route to evaluate viral replication, host cytokine and nitric oxide synthase 2 (Nos2) expression in the brain via real-time PCR. Histological analysis of the brain tissues was also performed. An analysis of which cells were responsible for the expression of cytokines and Nos2 was performed using flow cytometry. Survival curves of infected animals were also generatedResultsDENV 3 genotype I infected mice and replicated in the brain, causing death in our murine model. The increased levels of NOS2 could be the cause of the death of infected mice, as viral replication correlates with increased Nos2 and cytokine expression in the brain of C57BL/6 mice. In Nos2−/− mice that were infected with DENV, no clinical signs of infection were observed and cytokines were expressed at low levels, with the exception of interferon gamma (Ifng). Additionally, the Ifng−/− mice infected with DENV exhibited a severe and lethal disease, similar to the disease observed in C57BL/6 mice, while the DENV- infected Nos2−/− mice did not display increased mortality. Analyses of the brains from infected C57BL/6 mice revealed neuronal degeneration and necrosis during histopathologic examination. IFNg and NOS2 were produced in the brains of infected mice by CD4+ T cells and macrophages, respectively.ConclusionThe neurovirulence of DENV 3 genotype I is associated with a deleterious role of NOS2 in the brain, confirming this murine model as an appropriate tool to study DENV neurovirulence.