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Abstract Objective To outline the preliminary development and validation of a questionnaire for diagnosing epilepsy and distinguishing focal and generalized epilepsy among infants and children in Indonesia, where electroencephalography and pediatric neurologists are generally not available. Methods A 10‐question questionnaire comprising of 43 items was developed through literature review and expert panel discussions. Then, the questionnaire was administered by pediatricians to 75 children aged 1 month to 18 years old presenting with >1 episode of unprovoked seizures at an interval of >24 h. Subsequently, the questionnaire was assessed for content validity with item‐level and scale‐level content validity indices and ratio, construct validity with item‐total correlation tests, criterion validity with diagnostic parameter assessments, and inter‐rater reliability using Cohen's kappa (κ) and internal consistency with Cronbach's alpha (α) coefficient. Results The questionnaire exhibited favorable internal validity and reliability in diagnosing epilepsy and distinguishing focal and generalized epilepsy, with excellent content (both indices and ratio at 1) and construct validity (rcount > rtable at p

BACKGROUND: Myasthenia gravis (MG) is a rare autoimmune disease affecting neuromuscular junction involvement. The finding that T-reg level in MG patients was lower than that in normal persons leads to the idea that the primary pathology of the disease is T-reg dependent. The T-reg level of MG patients seems to be decreasing compared to that of normal persons. The study was conducted to observe the contribution of T-reg level in plasma injected into Swiss-Webster mice to develop clinically and pathologically myasthenic syndromes. METHODS: Swiss-Webster mice were grouped into three groups: the groups received plasma with normal, low, and high T-reg levels, respectively. The T-reg levels of the mice were measured with flow cytometry analysis and a human regulatory T-cell cocktail for T-cell surface cell marker. The motor function, interleukin (IL)-2, interferon (IFN)-γ, and thymus weight of mice were measured after the injection. Histopathological examination was performed to analyze mice’s muscles and thymus. RESULTS: The result identified that the motor function (2-week treatment group: p=0.021 and 3-week treatment group: p=0.032) and muscle width (p=0.014, p=0.032 and p≤0.001) were significantly lower in the low T-reg level plasma group compared to control and high T-reg level plasma groups. The thymus showed an increase in weight without an increase in the cortex-medulla ratio of the thymus, indicating hyperplasia. Both IL-2 and IFN-γ levels were lower in the low and high T-reg level groups compared with the control group, indicating the autoimmune process. CONCLUSION: Low T-reg level was associated with lower motor function, muscle width, increased thymus weight, as well as lower IL-2 and IFN-γ levels. T-reg level contributed to clinical myasthenic syndromes but not pathological findings. This research method is expected to be a basis for the development of animal models with Swiss-Webster mice. KEYWORDS: animal model, Myasthenia gravis, Swiss-Webster mice

Introduction. Due to their immunocompromised state from immunosuppressive therapy and respiratory efficiency, patients with Myasthenia Gravis (MG) are more likely to get infected with COVID-19. Currently, reports on MG patients exposed with COVID-19 in Indonesia are still limited. Therefore, this study was conducted to determine the clinical characteristics and outcomes of MG patients infected with COVID-19, especially in Jakarta, Indonesia. Methods. It is a retrospective study of MG patients in a National Referral Hospital in Indonesia from September 2021 to December 2022. The data obtained includes demographics, medical history, clinical characteristics, and severity of MG assessed by MG composite scale (MGCS), Myasthenia Gravis Foundation of America (MGFA) classification, symptoms, and therapy. Results. Of the 8 patients, 7 patients were female. The range of age at COVID-19 onset was 28-62 years old. On admission to the hospital, 2 patients were mild MG, 2 patients were moderate MG, 3 patients with severe MG, and one patient could not be identified because the patient came in an unconscious state. Five patients were classified as moderate COVID-19, two patients had severe COVID-19, and one patient was classified as critical COVID-19. Most patients experience MG exacerbation or require oxygen support during hospitalization. Only one patient deceased while receiving treatment at the emergency room, while the rest improved and were discharged home. Conclusion. MG patients exposed with COVID-19 tend to experience moderate, severe, and critical COVID-19 symptoms. However, with proper management of the infection and other comorbidities, MG patients with COVID-19 may achieve favorable outcome.

Introduction: Since the COVID-19 pandemic, there has been increasing use ofchat-based telemedicine, including for patients with neuropathy complaints. It is imperative to learn how to effectively use telemedicine. This study describes the characteristics of patients with neuropathy complaints in chat-based telemedicine services in Indonesia and their influence on treatment decisions and referrals. Methods: This is a retrospective cross-sectional study during the COVID-19 pandemic era (March 2020 to December 2021) using anonymous secondary data from patient chat databases on Indonesian application-based telemedicine services (Halodoc, Alodokter, Good Doctor, and Milvik). We applied bivariate and multivariate analysis. Results: We obtained 1051 patients with suspected peripheral nerve complaints (4 per 10,000) from a total of 2,199,527 user consultations, with the majority being 40–64 years old females and diabetes mellitus was the leading comorbid (90.7%). Most patients received treatment (90.7%) and only 11.4% patients were referred. Multivariate analysis showed that treatment was more likely to be given by a neurologist (p

Objective Neuropsychiatric SLE (NPSLE) has a broad spectrum and to date, there is no gold-standard biomarker. The diagnosis relies on clinical assessment, supporting examinations and exclusion of other possible aetiologies. One method that can be used to establish NPSLE is to conduct a re-evaluation by involving several fields of medical science. This study aims to reassess SLE cases with neuropsychiatric (NP) manifestations through multidisciplinary re-evaluation and determine the final diagnosis of NPSLE or non-NPSLE.Methods This retrospective cross-sectional study used medical record data from patients with SLE with NP manifestations. Inclusion criteria included patients diagnosed with SLE, who had clinical manifestations of NP and were >18 years old. Multidisciplinary re-evaluation was conducted and agreed upon the diagnosis of NPSLE or non-NPSLE.Results We included 94 subjects with a total of 132 NP events consisting of 69 NPSLE and 63 non-NPSLE. After re-evaluating NPSLE events, 33.3% were still concluded to be NPSLE. Meanwhile, from the non-NPSLE group, 22.2% were then declared as NPSLE. There were no significant differences in demographic characteristics between the NPSLE and non-NPSLE groups. The proportion of NP events in both groups was almost the same except for cerebrovascular disease manifestations which were more common in the NPSLE group. Higher Mexican SLE Disease Activity Index scores with (p

Paraneoplastic syndrome is a broad spectrum of signs and symptoms due to neoplasm, attributed to substances produced by tumor cells, or in response to it. Myasthenia gravis (MG) is a well-known paraneoplastic neurological syndrome (PNS), frequently associated with thymic abnormalities, but rarely reported in patients with lymphoplasmacytic lymphoma. This study presents the case of a 52-year-old Indonesian male patient who was diagnosed with Waldenstrom macroglobulinemia (WM), a rare B-cell neoplasm, after developing a new onset of MG with myasthenic crisis. the patient’s MG features improved with Ibrutinib as a treatment targeted toward cancer. This is the first case report presenting the treatment response of Ibrutinib in WM with myasthenic crisis. The literature was reviewed to explain the possibility of MG as a paraneoplastic syndrome of WM and the treatment response of Ibrutinib for this patient, as well as summarizing previous case reports of concomitant MG and WM. MG should be considered a paraneoplastic malignancy syndrome, including WM, during diagnostic workup. Ibrutinib should also be considered when available to patients, due to its adequate response in both previously treated and treatment naïve patients.

Abstract Background Despite a considerable number of articles regarding neurological manifestations associated with severe acute respiratory syndrome coronavirus 2 infection, reports on transverse myelitis and encephalitis are scarce. Case presentation We report a 35-year-old Asian Arab female presenting with longitudinally extensive transverse myelitis within 3 weeks after being diagnosed with mild coronavirus disease 2019 infection. Administration of high-dose methylprednisolone led to significant clinical improvement. However, 2 days after discharge, the patient was readmitted with encephalitis manifestations, consisting of fever and loss of consciousness, along with deterioration in myelitis symptoms. Severe acute respiratory syndrome coronavirus 2 antibody was detected in cerebrospinal fluid, but DNA of severe acute respiratory syndrome coronavirus 2 was not found. Clinical recovery was achieved after the administration of intravenous immunoglobulin. Conclusion Longitudinally extensive transverse myelitis can be a neurological manifestation of coronavirus disease 2019 and can be followed by encephalomyelitis episodes. High-dose steroids and intravenous immunoglobulin as an immunomodulator are possible effective treatment options.

Background. Brain tumor is a rare tumor with low incidence. Although it is a rare tumor, the mortality of brain tumor is disproportionately high. Many countries have already published epidemiology of brain tumor. However, the epidemiology of brain tumor in Indonesia remains to be investigated. This article aimed to provide descriptive epidemiology of brain tumor. Methods. The data of brain tumor acquired from Department of Neurology and Neurosurgery Cipto Mangunkusumo General Hospital from 2014 to 2016. The histopathology classification of primary intracranial tumors was based on WHO classification of CNS tumors 2016 while brain metastasis was classified based on other histopathological types. The variables were analyzed and presented descriptively. Results. There were 316 subjects acquired in this study. Most of the subjects (68%) were women. The mean age of this study was 43.8 (11.7). Most of the subjects (86.1%) had primary histopathology. Lung cancer was the most commonly found brain metastasis in this study (34.1%). Conclusions. This is a pilot study of brain tumor epidemiology in Indonesia. Collaboration with other centers in Indonesia is needed to give a more representative insight of brain tumor in Indonesia.

ObjectiveMenstrual disorders are more common in women with epilepsy than in those without epilepsy. This study aimed to examine the characteristics of reproductive function in women with epilepsy at an Indonesian national referral hospital.MethodsA case-control study was conducted from March 2020 to March 2021. Women with and without epilepsy aged ≥18 years were enrolled. All women were premenopausal before epilepsy diagnosis. Data on demographic characteristics, menstrual profiles, epileptic syndrome, seizure type, seizure frequency, etiology, localization, and anticonvulsant medication were collected. Hormone levels (follicle stimulating hormone, luteinizing hormone, prolactin, and estradiol) were measured.ResultsA total of 72 women with and 50 without epilepsy (controls) were included. Dysmenorrhea was more common in women with epilepsy than in those without (59.7 vs. 20%, p < 0.001; odds ratio: 5.931 [95% confidence interval: 2.566–13.709]). Marriage rates were higher in women without epilepsy (82 vs. 45.8%, p < 0.001). No difference was found in hormone levels between the groups. The frequency of seizures was associated with prolactin and estradiol levels (p < 0.001). Polytherapy with clobazam was associated with menstrual cycle regularity. In women with epilepsy with menstrual disorders, valproic acid was associated with higher estradiol levels (p = 0.001) and lamotrigine with lower follicle stimulating hormone levels (p = 0.008).SignificanceWomen with epilepsy experienced more dysmenorrhea. A higher frequency of seizures associated with lower prolactin and estradiol levels. Polytherapy with clobazam was associated with irregular menstrual cycles, while valproic acid and lamotrigine was associated with estradiol and follicle stimulating hormone levels.

Introduction: Moderate-to-severe traumatic brain injury (msTBI) can cause non-convulsive status epilepticus (NCSE). Electroencephalography (EEG) is employed as a diagnostic tool due to the non-specificity of clinical symptoms. This study aimed to identify clinical and EEG features related to NCSE in patients with msTBI. Methods: This was a cross-sectional study. Suspected NCSE in msTBI was examined using EEG data collected in consecutive patients from January 2017 to December 2019 at Dr. Cipto Mangunkusumo Hospital, Jakarta. Diagnoses of NCSE were made based on clinical manifestations and EEG features using the modified Salzburg Consensus Criteria for NCSE (mSCNC). Results: Of the 39 msTBI patients, 19 were diagnosed with NCSE; only two fulfilled the definitive criteria, and the remaining were possible NCSE. Delirium and perceptual impairment were only found in NCSE, while psychomotor agitation was higher (12.8% vs. 5.1% in NCSE vs. non-NCSE). The most common EEG feature was rhythmic activity (>0.5 Hz) without fluctuation, which improved with anti-epileptic drug administration. The Glasgow Coma Scale (GCS) score at onset and at hospitalisation discharge was significantly lower in patients with NCSE. The lesions in NCSE mostly originated from the temporal lobe. Injury to the temporal lobe had a significant relationship with NCSE occurrence (p = 0.036, odds ratio 11.45 [95% confidence interval 1.17–111.6]). Discussion: Post-traumatic NCSE can manifest as an alteration in mental status that could lead to missed diagnosis. In this study, delirium, perceptual impairment, and psychomotor agitation were confirmed as NCSE using EEG. The most common discharge originated from the injured temporal lobe, and this site was a significant factor associated with NCSE in patients with msTBI. Conclusion: NCSE can be found in msTBI cases with clinical manifestations of altered mental status, psychomotor agitation, and hallucination. An injured temporal lobe was a susceptible site for the development of NCSE.

Background: During the COVID-19 pandemic, patients with myasthenia gravis (MG) are most likely to be affected by this situation. Corticosteroids and immunosuppressant agents increase the risk of severe infection. Furthermore, viral infection and some medications in COVID-19 may exacerbate MG symptoms. Case description: We presented three patients with MG who contracted COVID-19. All of the patients had a favourable outcome. Only one patient who was not treated with corticosteroids or immunosuppressant therapy experienced deterioration of MG symptoms, while the other patients who received immunosuppressant therapy did not develop MG exacerbation. Surprisingly, azithromycin did not provoke myasthenic crisis (MC) in patients with normal MGFA classification. Conclusion: Using immunosuppressant agents may not lead to MG deterioration and may not be related to unfavourable outcomes.

Neural leprosy is characterized by neurological deficit without skin lesions, with a prevalence ranging from 1% to 17.7%. Diagnosis might be difficult and need a multidisciplinary approach. This is a case of axonal type motor and sensory polyradiculoneuropathy of the peripheral facial nerve. A 26-year-old woman was referred from the neurology clinic with facial paralysis, suspected as leprosy. Physical examinations were as follows: no skin lesions, left eye lagophthalmos, thickening of right lateral peroneal and bilateral posterior tibial nerves, sensory impairment, peripheral bilateral facial palsy, and wasting of bilateral distal small muscles of the hands, with normal autonomic function. Nerve Conduction Study revealed multiple demyelinating mononeuropathy of upper and lower extremities. Her serum anti-PGL-1 IgM level was 1721 μ/mL, but after three months of treatment with MDT-PB regimen, it increased to 2815μ/mL. Therefore, the treatment was switched to MDT-MB regimen and 30 mg prednisone. The patient is still undergoing treatment. There has been a slight improvement after treatment with MDT-MB regimen. Nerve biopsy is the gold standard for diagnosis but has its limitations. However, serological test of anti PGL-1 can be a marker and a useful tool as an additional test to confirm the diagnosis, especially for patients with nerve impairments. Difficulties are due to the absence of skin lesions and neuropathy which may be caused by other diseases. Both diagnosis and treatment require multidisciplinary approach. Treatment given is intended to correct nerve damage and prevent further disabilities.

Introduction. Generalized anxiety disorder (GAD) is one of the most common types of anxiety disorder in epilepsy population, comprising 21.9%, that would further impair patients’ quality of life. Generalized Anxiety Disorder-7 (GAD-7) is the only screening tool for GAD that has been validated in patients with epilepsy (PWE). It is a self-reporting instrument that can be completed in less than three minutes; hence, its usage is appropriate in primary healthcare and neurology outpatient clinic. This study aimed to obtain a valid and reliable Indonesian version of GAD-7, assess its accuracy, and finally evaluate the prevalence of GAD in Indonesian PWE along with its contributing factors. Methods. A cross-sectional study was conducted in Cipto Mangunkusumo General Hospital, Jakarta. The GAD-7 was translated and adapted using World Health Organization (WHO) steps. Validity, reliability, test-retest reliability, and diagnostic accuracy were evaluated. Then, epilepsy outpatients were screened for GAD using the Indonesian version of GAD-7. Results. Internal validity and reliability for Indonesian version of GAD-7 were satisfactory with validity coefficient of 0.648 to 0.800 (p6 with the sensitivity, specificity, negative predictive value, and positive predictive value of 100%, 84.4%, 100%, and 55.8%, respectively. ROC analysis showed the area under the curve of 0.98 (95% CI: 0.96–0.99). The total subjects screened with the validated Indonesian version of GAD-7 were 146, and 49% were screened as having GAD. Sociodemographic and clinical characteristics had no statistically significant association with the presence of GAD. Conclusion. The Indonesian version of GAD-7 was a valuable screening tool to detect GAD in PWE. GAD was screened in a quite high proportion of PWE. Sociodemographic and clinical characteristics were not proven to play role in its development.

Introduction. Adverse events (AEs) associated with antiepileptic drugs (AEDs) affect people with epilepsy’s (PWE) quality of life. A study conducted in 15 European countries showed that the AEs prevalence of AEDs in PWE was up to 80%. To date, there are no validated screening instruments to detect AEs of AEDs in Indonesian PWE. Therefore its epidemiology is currently unknown. This study aimed to validate the Indonesian version of Liverpool Adverse Events Profile (LAEP), consequently increasing physicians’ awareness toward the probability of AEs and its necessary evaluation. Furthermore, this study was intended to determine the AEs prevalence of AEDs in Indonesian PWE. Methods. The questionnaire was translated from English into Indonesian version. The validity and reliability were tested using Spearman correlation and Cronbach’s alpha measurement. An observational cross-sectional study was carried out on consecutive PWE in outpatient clinic, Cipto Mangunkusumo Hospital. We analyzed duration of epilepsy, onset of epilepsy, seizure frequency, type of epilepsy, etiology and epilepsy syndrome, number of AEDs, duration of AED use, and comorbidity. Results. All of the 19 items in the questionnaire were valid, with correlation coefficient ranging from 0.465 to 0.690 (moderate-strong correlation). Cronbach’s alpha value was 0.846 (good consistency). The total of 90 subjects were enrolled with 91% screened as having AEs using LAEP questionnaire. The most common AEs were tiredness (67.8%), sleepiness (66.7%), memory problems (62.2%), and difficulty in concentrating (56.7%). The only clinical variable that influenced AEs was polytherapy. Conclusion. The Indonesian version of LAEP was a valid and reliable instrument to screen AE of AEDs in PWE. Almost all the subjects in this study were suspected having AEs. Polytherapy was the independent factor of AE.