A Comparison of Pain Before and After Transfusion in Adult transfusion-dependent thalassemia (TDT) Using BPI-SF.

Background Pain is a complication in patients with transfusion-dependent thalassemia (TDT). There are several mechanisms underlying pain in people with thalassemia and low hemoglobin at the end of the transfusion cycle was the most etiology. Pain can develop into chronic pain and interfere with the quality of life. The Brief Pain Inventory Short Form (BPI-SF) can help identify pain in people with TDT. The present study aimed to compare pain before and after transfusion in adult TDT patients. Methods It was an analytical observational study using a cross-sectional design on adult TDT patients with pain who came to the Haemato-Oncology Clinic of Dr. Hasan Sadikin Hospital Bandung. This study was conducted from December 2020 to July 2021. All subjects were assisted in filling out the Indonesian version of the BPI-SF questionnaire hemoglobin levels were examined and before and after transfusion, then paired test analysis was performed using the Wilcoxon Test. Results This study is conducted on 60 adult TDT patients with symptoms of pain. The median value of pain intensity and pain interferes with life obtained from the Indonesian version of the BPI-SF score after transfusion decreased significantly compared to before transfusion (NRS 5 vs. 0 and 2.8 vs. 0; p=0.0001). Conclusion There is a significant difference in pain intensity and pain interfere with life in adults with TDT before and after transfusion. It is necessary to carry out pain assessments for thalassemia patients.

The number of risk factors increases the recurrence events in ischemic stroke.

PURPOSE: Stroke is a significant cause of disability worldwide and is considered a disease caused by long-term exposure to lifestyle-related risk factors. These risk factors influence the first event of stroke and recurrent stroke events, which carry more significant risks for more severe disabilities. This study specifically compared the risk factors and neurological outcome of patients with recurrent ischemic stroke to those who had just experienced their first stroke among patients admitted to the Hospital. PATIENTS AND METHODS: We observed and analyzed 300 patients' data who met the inclusion and exclusion criteria. This retrospective observational study was conducted on consecutive acute ischemic stroke patients admitted to the top referral hospital, West Java, Indonesia. The data displayed are epidemiological characteristics, NIHSS score at admission and discharge, and the type and number of risk factors. Data were then analyzed using appropriate statistical tests. RESULTS: Most patients had more than one risk factor with hypertension as the most frequent (268 subjects or 89.3%). In patients who experienced ischemic stroke for the first time, the average National Institutes of Health Stroke Scale (NIHSS) score was lower (6.52 ± 3.55), and the alteration of NIHSS score was higher (1.22 ± 2.26) than those with recurrent stroke (6.96 ± 3.55) for NIHSS score and 1.21 ± 1.73 for alteration of NIHSS score). We processed the data with statistical analysis and showed a positive correlation between age (P < 0.05) and the number of risk factors (P < 0.001) in the recurrent ischemic stroke group. CONCLUSIONS: Age and the number of risk factors correlate with recurrent ischemic strokes.

Brain MRI Volumetry Analysis in an Indonesian Family of SCA 3 Patients: A Case-Based Study.

Introduction: Spinocerebellar ataxia type-3 (SCA3) is an adult-onset autosomal dominant neurodegenerative disease. It is caused by expanding of CAG repeat in ATXN3 gene that later on would affect brain structures. This brain changes could be evaluated using brain MRI volumetric. However, findings across published brain volumetric studies have been inconsistent. Here, we report MRI brain volumetric analysis in a family of SCA 3 patients, which included pre-symptomatic and symptomatic patients. Methodology: The study included affected and unaffected members from a large six-generation family of SCA 3, genetically confirmed using PolyQ/CAG repeat expansion analysis, Sanger sequencing, and PCR. Clinical evaluation was performed using Scale for the Assessment and Rating of Ataxia (SARA). Subjects' brains were scanned using 3.0-T MRI with a 3D T1 BRAVO sequence. Evaluations were performed by 2 independent neuroradiologists. An automated volumetric analysis was performed using FreeSurfer and CERES (for the cerebellum). Result: We evaluated 7 subjects from this SCA3 family, including 3 subjects with SCA3 and 4 unaffected subjects. The volumetric evaluation revealed smaller brain volumes (p < 0.05) in the corpus callosum, cerebellar volume of lobules I-II, lobule IV, lobule VIIB and lobule IX; and in cerebellar gray matter volume of lobule IV, and VIIIA; in the pathologic/expanded CAG repeat group (SCA3). Conclusion: Brain MRI volumetry of SCA3 subjects showed smaller brain volumes in multiple brain regions including the corpus callosum and gray matter volumes of several cerebellar lobules.

Congenital neurosyphilis presenting as neonatal sepsis.

INTRODUCTION: Congenital syphilis involves any organs with various symptoms, including neurological signs. Neurosyphilis is a severe syphilis complication that can develop at any stage of illness. CASE PRESENTATION: A 2,520 g male infant was spontaneously born at term from an untreated syphilis mother. Physical examination revealed decreased consciousness, respiratory distress, seizure, but without neurologic abnormality sign. The serum and cerebrospinal fluid Venereal Disease Research Laboratory and Treponemal Pallidum Hemagglutination Assay TPHA tests titters were 1:16 and 1:1,280, respectively. The diagnosis at admission was respiratory failure and neonatal sepsis. The infant was mechanically ventilated and treated with early management of sepsis. Blood culture was sterile later on. Then, the infant was administered intramuscular benzathine penicillin G (50,000 units/kg/dose) for a total of three weeks. The infant's condition was improved during the treatment. CONCLUSIONS: There are many challenges associated with screening and monitoring neurosyphilis in congenital syphilis. Congenital syphilis presenting as sepsis is easily misdiagnosed as bacterial sepsis.

Periventricular hypodensity is associated with the incidence of pre-shunt seizure in hydrocephalic children.

BACKGROUND: The seizure incidence in hydrocephalic children has been acknowledged in a lot of studies previously; nonetheless, seizure pathogenesis in these children remains unclear. Its high proportion of hydrocephalic children who underwent shunt surgery suggests that the seizure might be associated with the protocol of shunt placement and/or the shunt existence intracranially; however, this hypothesis could not explain the pre-shunt seizure incidence in hydrocephalic children. OBJECTIVE: This study aims to evaluate the patients' characteristics and CT findings in pre-shunt hydrocephalic children to identify the possible seizure etiology in these patients. METHODS: Three hundred and thirty-four children with hydrocephalus were included in this study, including 147 hydrocephalic children with the pre-shunt seizure history and 187 hydrocephalic children presented without the pre-shunt seizure history. The following information was retrieved from the patients' medical records: gender, age, pediatric Glasgow Coma Scale (pGCS) upon admission, and hydrocephalus diagnoses. CT findings were re-evaluated to assess the compression association of sulci and gyri, Sylvian fissures, cisterns, FH/ID ratio, Evan's ratio, and periventricular hypodensity with pre-shunt seizure. RESULTS: The results show that the pre-shunt seizure incidence is significantly higher in hydrocephalic children aged 1 to 5 years old (63/113 (55%), p = 0.0001), diagnosed with communicating hydrocephalus (97/163 (59%), p = 0.0001) or infectious hydrocephalus (80/109 (73%), p = 0.0001). The presence of periventricular hypodensity is significantly associated with the pre-shunt seizure incidence (132/205 (64.3%), p = 0.0001). Results from univariate analyses suggest significant association between periventricular hypodensity in every location and pre-shunt seizure (p < 0.0001). Multivariate analyses identify that temporal horn in the right lateral ventricle as the location of periventricular hypodensity has the strongest association with the pre-shunt seizure. CONCLUSION: The presence of periventricular hypodensity in head CT scan is significantly associated with the pre-shunt seizure incidence. Further investigation to confirm this finding and evaluate the possible roles of inflammation in the pre-shunt seizure in hydrocephalic children is important to seek its possible implication on the treatment of pre-shunt seizure in these children.

Clinical and genetic profile in index patients with spinocerebellar ataxia type 3 in Indonesia: case report.

Spinocerebellar ataxia (SCA) is an autosomal dominant hereditary disease with progressive course, and no causal therapy. Diagnostics are still challenging, due to facility and protocols, and so as in Indonesia. As a national referral center, Dr. Hasan Sadikin Central General Hospital has received a lot of patients from all over Indonesia, particularly from Western Java. Study related to SCA (including clinical and genetic profile) is still limited in Indonesia. We identified index patients from three families with ataxia, hence intend to determine their clinical and genetic pattern. The hereditary pattern is autosomal dominant. Scale for the assessment and rating of ataxia (SARA) shows mild and moderate ataxia. Inventory of non-ataxia signs (INAS) scores of the patients were 3, 5 and 6. Montreal cognitive assessment-Indonesian version (MOCA-INA) shows only one patient has mild cognitive impairment, despite young age. Barthel index shows 1 subject has moderate dependency. Mutation in Ataxin3 polyQ repeats shows pathologically long CAG repeats, 72,10; 72,10; and 72,23 respectively in mutant and wild type allele. We diagnosed the index patients with spinocerebellar ataxia type 3. This study is the first case series study in Indonesia. The hereditary pattern is clearly shown as an autosomal dominant ataxia. The clinical and genetic profile was varied, and the symptom is progressive and deteriorates overtime, including wide based gait, speech problem, motor and sensor complaint, and cognitive decline complaint. Despite the same polyQ stretch length, the onset and clinical characteristics of patients are diverse.

The Lymphocyte Depletion in Patients with Acute Ischemic Stroke Associated with Poor Neurologic Outcome.

PURPOSE: Inflammation plays an important role and is involved in all stages of acute ischemic stroke. One of these stages involves the recruitment of leukocytes from the peripheral circulation into the ischemic tissue. Lymphocytes as a subtype of leukocytes are important mediators and can become a predictor of neurological outcome. Several studies have been conducted regarding the correlation between differential lymphocyte counts and acute ischemic stroke. Most of these studies analyzed lymphocyte ratio to other leukocyte subtypes such as neutrophils and monocytes. This study specifically observed the role of lymphocytes as an indicator of the inflammatory response in patients with acute ischemic stroke. This study aimed to observe the correlation among risk factors, infarct location, leukocyte counts, lymphocyte value and neurologic output in acute ischemic stroke patients. PATIENTS AND METHODS: We observed and analyzed 193 patients' data from medical record which met the inclusion and exclusion criteria with a diagnosis of acute ischemic stroke at the Department of Neurology of Dr. Hasan Sadikin General Bandung. Data were then analysed using appropriate statistical tests. RESULTS: Most patients have more than one risk factor with a leukocyte count of less than 10,000 cell/mm3. Infarct was mostly located in subcortical area (basal ganglia), with moderate average NIHSS values at admission and at discharge. The number of lymphocytes decreased in the subject group with more than 10,000 cell/mm3 leukocytes. Subsequently, data were analyzed using Spearman's test and there was a correlation between NIHSS on admission and lymphocyte depletion. CONCLUSION: The lymphocyte depletion in patients with leukocytosis is a predictor of poor NIHSS.

Neurodevelopmental outcomes after ventriculoperitoneal shunt placement in children with non-infectious hydrocephalus: a meta-analysis.

BACKGROUND: Hydrocephalus is diagnosed when an accumulating amount of cerebrospinal fluid (CSF) fails to circulate and/or absorbed in the ventricular system. Based on its etiology, hydrocephalus can be classified into infectious and non-infectious hydrocephalus. In children, non-infectious hydrocephalus includes congenital hydrocephalus, posthemorrhagic hydrocephalus, neural tube defect-related hydrocephalus, and tumor-related hydrocephalus. Regardless of the cause, a CSF diversion device is placed to divert the excess fluid from the ventricles into peritoneal cavity. Among all, ventriculoperitoneal (VP) shunt is arguably the most commonly used CSF diversion device to date. Until now, the long-term neurodevelopmental impact of VP shunt placement in non-infectious hydrocephalus patients remained unclear. OBJECTIVE: This study aims to evaluate the neurodevelopmental outcomes in children with non-infectious hydrocephalus who had VP shunt placement. MATERIALS AND METHODS: Systematic searches were performed using PubMed, Google Scholar, Scopus databases, and reference lists. Publications that fulfilled the inclusion criteria were included in the meta-analysis. Calculation of Mantel-Haezel risk ratio (RR) was applied, and heterogeneity index (I2) test was used to evaluate the existence of heterogeneity in all studies. Risk of bias was assessed based on the criteria from the Newcastle-Ottawa Scale (NOS). RESULTS: Of the 1929 studies identified, 12 publications were concluded to have fulfilled the inclusion criteria. Results from the meta-analysis showed that the risks of cerebral palsy, visual and hearing impairment, epilepsy, or seizures are significantly higher in children with non-infectious hydrocephalus who already had VP shunt placement (shunted non-infectious hydrocephalus, S-NIH) compared to that of the healthy control. The meta-analysis on intelligent quotient (IQ) and mental development index (MDI) showed that S-NIH children tend to score lower IQ and acquire risk of having mental development delay. On motoric development, S-NIH children scored lower motoric score and have significantly higher risk of motor development delay compared to control. Although normal children tend to have more internalizing behavior compared to S-NIH children, overall assessment on the risk of behavioral abnormalities showed that the differences between these two groups are insignificant. CONCLUSION: S-NIH children have significantly higher risks of disabilities and mental and motoric development delays; thus, planning on continuous rehabilitation for children with non-infectious hydrocephalus who already had placement of VP shunt is important to acquire their optimum potentials and quality of life.

D-Dimer Level Associates with the Incidence of Focal Neurological Deficits in Cerebral Venous Thrombosis Patients.

PURPOSE: D-dimer is one of the main coagulation factors that plays a role in some diseases. Cerebral venous thrombosis (CVT) is a rare cerebrovascular disease with various clinical presentations, and the D-dimer might contribute to its clinical progress. Our study's objective was to explore the association between D-dimer level and focal neurological deficits in selected CVT patients. PATIENTS AND METHODS: The patients' data were retrospectively enrolled if they showed venous thrombus features in the digital subtraction angiography examination. Data collected were then evaluated using appropriate statistical tests. RESULTS: In a total of 30 patients, 24 patients had focal neurological deficits with abnormal D-dimer (mean 1.13±1.359 mg/L). We continued analysis to determine the association between the visual analog scale (VAS), a tool to measure the patient's pain, and coagulation factors. There was no significant association between the VAS score and all the coagulation factors. Interestingly, a positive association was found between focal neurological deficits and abnormal D-dimer levels (p=0.009). CONCLUSION: The abnormality of D-dimer levels in CVT's patients associates with the incidence of focal neurological deficits.

Microbiological Testing of Gastric Aspirate Improves the Diagnosis of Pulmonary Tuberculosis in Unconscious Adults with TB Meningitis.

Conventional sputum collection for TB diagnosis is difficult in TB meningitis patients since most of them are admitted with decreased consciousness. It is assumed that unconscious patients swallow their sputum; therefore, gastric aspiration can replace sputum collection in unconscious patients. A prospective study was conducted to see whether examining gastric aspirate could increase the diagnosis certainty of pulmonary TB in such subjects. The inclusion criteria were age 18-60 years, decreased level of consciousness, and use of a nasogastric tube. Subjects who had taken antituberculosis drugs for more than 3 days were excluded. Gastric lavage was performed in the morning after an overnight fast. Specimens were examined for direct smear, culture, and rapid molecular testing. Demographic, clinical, chest X-ray, and laboratory data were also recorded. During the study period, 31 subjects were available. The positivity rates for microbiological tests were 19.3%, 41.9%, and 48.4% for smear, culture, and rapid molecular testing, respectively. All positive smears were confirmed by either culture or rapid molecular testing. Gastric lavage can be considered a tool for improving extraneural TB diagnosis in unconscious patients.