Uncommon occipital ganglioglioma in a child: The critical role of MRI in diagnosis and management.

Epilepsy is a prevalent neurological disorder in children, with 30-35% of cases developing into intractable epilepsy despite appropriate anti-seizure medications (ASMs). Advanced imaging is crucial for early diagnosis, especially in detecting structural causes like gangliogliomas, which are associated with drug-resistant focal epilepsy. We report a 5-year-old boy with recurrent seizures, initially undetected by EEG. Persistent seizures led to the identification of an occipital lobe ganglioglioma through MRI. The tumor was successfully resected, significantly improving the patient's condition. This case emphasizes the limitations of relying solely on EEG for epilepsy diagnosis and highlights the necessity of integrating advanced imaging techniques. The successful outcome underscores the importance of a multidisciplinary approach, involving pediatric neurologists, radiologists, and neurosurgeons, in managing complex pediatric epilepsy cases.

N-methyl-D-aspartate receptor autoantibody levels in children with intractable and non-intractable epilepsy.

Objective: Intractable epilepsy in children is a prevalent neurological disorder that can pose serious risks. The involvement of the autoimmune system is a significant factor in the pathogenesis of the disease. The N-methyl-D-aspartate-receptor (NMDAR) is a glutamate receptor and ion channel present in neurons and is associated with the mechanism of autoimmune etiology in epilepsy. This study aims to compare the levels of NMDAR auto antibodies in children with intractable and non-intractable epilepsy. Methods: A prospective analytic study was conducted from June to September 2022. The study sample consisted of patients aged 1 month to 18 years diagnosed with epilepsy and receiving anti-seizure medication (ASM) therapy at Dr. Soetomo General Academic Hospital, Surabaya. The patients were divided into two groups, namely intractable epilepsy and non-intractable epilepsy. The NMDAR autoantibody levels were determined using enzyme-linked immunosorbent assay (ELISA). Statistical analysis employed the chi-squared and Wilcoxon-Mann-Whitney tests. Results: Seventy-five subjects were included in the study. Of these patients, 41.3 % with intractable epilepsy and 33.4 % with non-intractable epilepsy presented NMDAR auto antibodies. Analysis of the patient characteristics revealed a correlation between seizure frequency and NMDAR autoantibody positivity (P = 0.002) but not between the number of ASM and NMDAR autoantibody positivity (P > 0.05). The NMDAR autoantibody levels were not significantly different in children with intractable and non-intractable epilepsy (P = 0.157). Conclusion: The NMDAR autoantibody levels were numerically higher in children with intractable epilepsy compared with children with non-intractable epilepsy.

Validity of measurement of femoral anteversion angle using FEMORA® software based on two-dimensional radiographic imaging examination femur in children with cerebral palsy in Indonesia.

Introduction: Children with spastic cerebral palsy (CP) often show an increase in femoral anteversion angle (FAA). Computed tomography (CT) scan is the main modality for evaluating FAA in these patients, however, due to significant radiation exposure, it carries a high carcinogenic risk. FEMORA® software is expected to be able to accurately assess FAA even with conventional X-ray images that only require low radiation exposure. However, its validity has not been tested in various populations or CT devices. This study aimed to validate the FEMORA® software by comparing it to CT scans done on an Indonesian population. Material and methods: All spastic CP patients of the outpatient clinic at Dr. Soetomo Hospital between March and November 2022, were included. The FEMORA® Software evaluation was performed by three examiners. The calculation results were averaged and compared with those of the CT scan. Intraclass correlation coefficient (ICC), reliability, and correlation were be assessed. Results: There were 36 patients included in this study. Most were female (n = 22; 61,1 %) and the average age was 7,28 years old. Interobserver preoperative analysis using ICC showed good outcomes (p = 0.918; 95 % CI, 0.858-0.955). FAA measurement results using FEMORA® and CT scans were 41,71 ± 12,90 and 32,68 ± 11,85, respectively. Correlation coefficient between the two values is 0.634 (p < 0.001). Conclusion: FEMORA® software demonstrates a good and significant correlation with FAA measurement using CT scan.

RISK FACTORS OF VINCRISTINE-INDUCED PERIPHERAL NEUROPATHY IN ACUTE LYMPHOBLASTIC LEUKAEMIA CHILDREN.

Objective : This study analysed Vincristine-induced peripheral neuropathy (VIPN) risk factors in Acute Lymphoblastic Leukaemia (ALL) children. Method : This cross-sectional study design was performed at Dr. Soetomo Hospital, Surabaya, Indonesia, from August to October 2019. It included ALL children, aged 4-18 years, undergoing the 2013 or 2018 ALL Indonesian Protocol of Chemotherapy, with a cumulative vincristine dose ≥ 12 mg / m2. VIPN diagnosis is based on complaints, the Total Neuropathy Score Pediatric Vincristine (TNS-PV), and nerve conduction studies (NCS). The examined risk factors were sex, age, ALL classification, nutritional status, impaired liver function, and cumulative vincristine dose. Results : There were 52 ALL children : median age 7 years, 59.6% boys, 59.6% ALL standard risk, 44.2% experienced impaired liver function at initial ALL diagnosis. Based on a single parameter for diagnosis, 26.9% had VIPN based on complaints, 76.9% had it based on the TNS-PV, and 100% had it based on NCS. VIPN was diagnosed in 25% of children, with predominantly motor impairment and located in lower extremities. Impaired liver function is a risk factor for VIPN in ALL children (p = 0.046, prevalence ratio (PR) 2.84). Conclusion : Impaired liver function is a significant risk factor for VIPN in ALL children. J. Med. Invest. 68 : 232-237, August, 2021.

Correlation of serum S100B levels with brain magnetic resonance imaging abnormalities in children with status epilepticus.

PURPOSE: To evaluate the association between elevated S100B levels with brain tissue damage seen in abnormalities of head magnetic resonance imaging (MRI; diffusion tensor imaging [DTI] sequence) in patients with status epilepticus (SE). METHODS: An analytical observational study was conducted in children hospitalized at Dr Soetomo Hospital, Surabaya, from July to December 2016. The patients were divided into 2 groups: SE included all children with a history of SE; control included all children with febrile seizure. Blood samples of patients were drawn within 24 hours after admission. SE patients also underwent cranial MRI with additional DTI sequencing. The Mann-Whitney test and Spearman test were used for statistical analysis. RESULTS: Fifty-three patients were enrolled the study. In the 24 children with SE who met the inclusion criteria, serum S100B and cranial MRI findings were assessed. Twenty-two children admitted with febrile seizures became the control group. Most patients were male (66.7%); the mean age was 35.8 months (standard deviation, 31.09). Mean S100B values of the SE group (3.430±0.141 µg/L) and the control group (2.998±0.572 µg/L) were significantly different (P<0.05). A significant difference was noted among each level of encephalopathy based on the cranial MRI results with serum S100B levels and the correlation was strongly positive with a coefficient value of 0.758 (P<0.001). CONCLUSION: In SE patients, there is an increase of serum S100B levels within 24 hours after seizure, which has a strong positive correlation with brain damage seen in head MRI and DTI.

Rare Case Of Primary Congenital Glaucoma With Hypoplasia Corpus Callosum.

Primary congenital glaucoma is a rare disease that causes elevated intraocular pressure within the first three years of life. Few studies have explored the association of primary congenital glaucoma with malformation of corpus callosum. We report on a six-month-old female presenting with unilateral primary congenital glaucoma associated with hypoplasia of corpus callosum in Indonesian infant. The patient had already undergone trabeculectomy surgery. However, there no obvious improvement following the procedure given the severity of the condition. CONCLUSION: The failure rate of surgery in severe primary congenital glaucoma conditions is still very high, and therapy can usually preserve vision if early identification of mild or moderate form is made.

Uncommon Pathogen Bacillus Cereus Causing Subdural Empyema in a Child.

BACKGROUND: Subdural empyema (SDE) in children is a severe intracranial infection. Many pathogens can cause SDE. CASE DETAILS: In this articlewe present a 15-month old Indonesian boy diagnosed as SDE based on the clinical symptoms and neuroimaging. A complete blood count showed white blood cell count of 13.800/mm3 and the CRP level was 8.3 mg/L. Craniotomy following burr hole drainage procedure was performed to decrease intracranial pressure. The liquor culture indicated Bacillus cereus. A meropenem injection and metronidazole infusion were administered appropriately with antibiotic sensitivity for several weeks. Immediate initiation of appropriate antibiotic therapy related to an accelerated surgical drainage can improve the outcome. CONCLUSION: The combination of medication and surgery treatment for Bacillus cereus in SDE resulted in a good response, leaving neurological sequelae.

Acanthamoeba Infection in a Drowning Child.

BACKGROUND: Acanthamoeba infection is a potential life-threatening complication of drowning. The management of drowning-associated Acanthamoeba infection remains controversial. Survival reports on Acanthamoeba infection have been on case reports only. CASE DETAILS: A 2-year-old, previously healthy Indonesian boy presented with decreased consciousness and inadequate breathing, followingdrowning. The event was unsupervised with unknown estimated time of submersion. Resuscitation was commenced and mechanical ventilated was applied. Sputum specimen revealed alive Acanthamoeba with pseudopods and cysts. Sputum culture resulted in Pseudomonas aeruginosa and cerebrospinal fluid was supportive for Acanthamoeba with 1-3 trophozoites and cyst. Imaging of the head showed cerebral edema and encephalitis. The patient received intravenous ceftazidime, metronidazole, fluconazole and rifampicin. The patient's consciousness was unsatisfactory regained resulting in an altered mental status. CONCLUSION: No treatment so far has given a succesful outcome for Acanthamoeba. In this case, management with metronidazole, rifampicin and fluconazole showed regain of consciousness resulting in altered mental status.