Factors correlating to decisions for prescribing pharmacological treatment and referrals in suspected peripheral neuropathy cases in chat consultation-based application.

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 < 0.01). Chronic symptoms (p < 0.01) and previous laboratory/other tests (p = 0.01) decreased the likelihood of medication prescription. Referrals were more likely to be given to chronic onset (p = 0.02), hypertension and heart disease (p < 0.01), and previous laboratory/other tests (p = 0.02). The opposite was true for age≥65 years, female (p = 0.04), and neurologists or other specialists as responders (p < 0.01). Conclusion: We identified several factors that influence the treatment decision such as female patients and onset. Meanwhile, age, sex, chronic symptoms, history of hypertension and heart disease, and previous laboratory/other tests may influence the referral decisions. General practitioners were more likely to refer the patients whereas neurologists or other specialists were more likely to give treatment. Chat-based telemedicine services can still be developed in the future to be better.

Pulmonary Function Assessment in Myasthenia Gravis Patients in a National Referral Hospital in Indonesia.

Purpose: Myasthenia gravis (MG) can cause respiratory muscle weakness and the need of ventilator support. Spirometry as the gold standard for pulmonary function examination has limited availability, especially in our hospital which is only available in outpatient clinic during work hours (not in emergency room or patient room). Furthermore, all primary healthcare in Indonesia do not have spirometry equipment, thus other alternatives are required. This study aimed to analyze the relationship between a single breath counting test (SBCT), peak flow meter (PFM), and spirometry to assess pulmonary function in MG patients in a national referral hospital in Indonesia. Patients and Methods: A single-center, cross-sectional study was conducted and SBCT, PFM, and spirometry examination were performed in MG patients and healthy controls. Results: Seventy patients, aged 47.7 ± 13.4 years old, participated in this study. SBCT, forced vital capacity first second (FVC1), and forced expiratory volume first second (FEV1) value between MG patients and healthy controls showed a significant difference, in which healthy controls have higher SBCT, FVC1, and FEV1 values (p = 0.000, p = 0.000 and p = 0.001 respectively). There was a significant difference between PFM with SBCT and FVC1 value in MG patients. Strong correlation was found between SBCT and FVC1 in MG patients. Conclusion: MG patients had worse pulmonary function compared to healthy controls. SBCT and PFM examination have a significant correlation with FVC1 in MG patients. Therefore, SBCT and PFM can be used as a bedside tool to detect respiratory impairment in MG patients.

Characteristics of menstrual disorders and reproductive hormones in women with epilepsy at an Indonesian national referral hospital.

Objective: Menstrual 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. Methods: A 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. Results: A 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). Significance: Women 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.

The stigma paradox: Perception of quality-of-life in people with epilepsy among themselves, the family, and the general population in Indonesian urban areas.

OBJECTIVE: To understand quality of life (QoL) perceptions of people with epilepsy (PWE) through knowledge, attitude, and behavior (KAB); PWE; their families (PWEf); and the general population (GPop). METHODS: This descriptive study was conducted in Jakarta and its surrounding cities from January to December 2019. PWE were recruited from outpatient clinics. PWEf were caregivers who lived with PWE. GPop were age matched, randomly selected, and interviewed for public events. The perception of QoL was scaled from 1 to 5 (1 =very poor to 5 =very good). KAB was obtained from open- and closed-ended questionnaires, scaled from 1 to 5 (1 =strongly disagree to 5 =strongly agree). The differences in each group were analyzed using t-tests and analysis of variance. RESULTS: We interviewed 371 participants, predominantly female and senior high school graduates. Unemployment and singlehood were higher in PWEs. QoL perception in PWE was similar to GPop (3.01 [0.75] vs. 3.07 [0.76], p = 0.49), yet lower in PWEf (2.78 [0.76]; p < 0.05). According to PWE and PWEf, not being stigmatized and support from family were essential, while GPop emphasized the medical perspective. Overall, the knowledge section had the lowest score and behavior had the highest. The GPop was uncertain about the cause of epilepsy in K2 (3.73 [1.05]), K4 (3.35 [1.24]), and K7 (2.93 [1.08]); p < 0.001. Despite positive behavior, GPop were unwilling to marry (B4) nor had PWE as their in-laws (B5); (2.83 [0.73] and 2.78 [0.77]; p < 0.001). Moreover, PWEf were still doubtful about GPop's acceptance (B1) (3.86 [0.38] vs 4.00 [0.40]; p < 0.05). CONCLUSION: The perception of QoL in PWE did not seem to be directly associated with KAB. Despite similar knowledge of PWE and PWEf, better perceptions came from PWE and GPop. The reluctance to form deeper bonds between GPop and PWE, along with PWEf's skepticism, could lead to low self-esteem, unemployment, and unmarried rates. Further studies are required to elaborate on these issues.

Deep convolutional neural network-based algorithm for muscle biopsy diagnosis.

Histopathologic evaluation of muscle biopsy samples is essential for classifying and diagnosing muscle diseases. However, the numbers of experienced specialists and pathologists are limited. Although new technologies such as artificial intelligence are expected to improve medical reach, their use with rare diseases, such as muscle diseases, is challenging because of the limited availability of training datasets. To address this gap, we developed an algorithm based on deep convolutional neural networks (CNNs) and collected 4041 microscopic images of 1400 hematoxylin-and-eosin-stained pathology slides stored in the National Center of Neurology and Psychiatry for training CNNs. Our trained algorithm differentiated idiopathic inflammatory myopathies (mostly treatable) from hereditary muscle diseases (mostly non-treatable) with an area under the curve (AUC) of 0.996 and achieved better sensitivity and specificity than the diagnoses done by nine physicians under limited diseases and conditions. Furthermore, it successfully and accurately classified four subtypes of the idiopathic inflammatory myopathies with an average AUC of 0.958 and classified seven subtypes of hereditary muscle disease with an average AUC of 0.936. We also established a method to validate the similarity between the predictions made by the algorithm and the seven physicians using visualization technology and clarified the validity of the predictions. These results support the reliability of the algorithm and suggest that our algorithm has the potential to be used straightforwardly in a clinical setting.

Case series: COVID-19 in patients with mild to moderate myasthenia gravis in a National Referral Hospital in Indonesia.

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.

Two Japanese LGMDR25 patients with a biallelic recurrent nonsense variant of BVES.

We report two Japanese patients with autosomal recessive limb-girdle muscular dystrophy type R25 (LGMDR25), harboring a novel recurrent homozygous nonsense variant of BVES. Muscle symptoms manifested from childhood to adulthood, initiated in the proximal or distal muscles of the lower limbs, and displayed asymmetric muscle involvement. Similar to the patients in previous reports, these patients also lost ambulation in late middle age. The posterior compartment of the lower limb muscles (biceps femoris, adductor magnus, gastrocnemius, and soleus) was preferentially affected as was the paraspinal muscle. Muscles in the anterior compartment of the thigh were affected in more advanced stages. Both patients had symptomatic atrioventricular block. The POPDC1 protein was undetectable in the muscles of the patients. As observed by transmission electron microscopy, one of the patient samples had fewer caveolae along the sarcolemma than a control sample.

[A case of Emery-Dreifuss muscular dystrophy with slight joint contracture].

A 42-year-old man with a history of two previous coronary embolisms was referred to our hospital. He had been experiencing muscle weakness since he was around 40 years old. He had muscle atrophy of the scapula, upper arm, and lower extremities, and electromyography revealed myogenic changes in the limb muscles. Histopathological analysis of the muscle biopsy specimen revealed a complete deficiency of emerin protein, and genetic examination revealed a mutation in the emerin (EMD) gene, resulting in a diagnosis of Emery-Dreifuss muscular dystrophy (EDMD). EDMD is a muscular disorder with three symptoms: joint contracture at early onset, muscle weakness and atrophy, and cardiac dysfunction. Although this patient showed no obvious joint contracture, the course and clinical symptoms vary among patients. Therefore, in patients in whom clinical diagnosis is difficult, muscle biopsy and genetic testing should be performed for EDMD in order to prevent sudden death due to this disease.

Screening of Generalized Anxiety Disorder in Patients with Epilepsy: Using a Valid and Reliable Indonesian Version of Generalized Anxiety Disorder-7 (GAD-7).

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 (p<0.01) and Cronbach's alpha value of 0.867. The best cutoff value to detect GAD in Indonesian PWE was >6 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.

Adverse Events of Antiepileptic Drugs Using Indonesian Version of Liverpool Adverse Events Profile.

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.