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Background: Temporal lobe epilepsy (TLE) has the highest probability of becoming resistant. One of the causes was Polymorphism in multidrug resistant-1 (MDR1) C3435T. In Dr. Cipto Mangunkusumo Hospital, potential drug-resistant epilepsy prevalence was 84.51%; 66.6% of them used carbamazepine (CBZ) as antiseizure medication. This comparative cross-sectional study aimed to investigate MDR1 C3435T polymorphism and CBZ plasma level (plCBZ) in Indonesian TLE patients. Methods: TLE patient was selected consecutively; divided into drug-responsive (DRV) and drugresistant (DRE) groups. Healthy subjects were included as a control for the gene polymorphism comparison. MDR1 was identified using the restriction fragment length polymorphism PCR technique; C allele at 159 and 57bp while T allele at 216bp. High-performance liquid chromatography was used to determine plCBZ. Results: There were 86 subjects; 61 in the study group and 25 controls. The genotype distribution between them was 0.58 vs 0.42, x2=0.54, p=0.000. In the study group, CBZ within therapeutic doses (dCBZ) had outreached the therapeutic plCBZ and found similar in all genotypes. DRE criteria were found in 37 subjects. Distribution of C and T in DRV was 0.63 vs 0.37, x2=10.4; and DRE 0.55 vs 0.45 x2=6.17 (p=0.019). In Tukey’s multiple comparison post hoc test, CT in DRV had significantly lower dCBZ (330,36 ± 174,91 mg) and plCBZ (7.15 ± 2.64 mcg/mL) compared to all genotypes in DRE. Whereas mean dCBZ was around 800mg and plCBZ outreached the toxic level; TT was the highest. Conclusion: The genotype MDR1 distribution was similar in the normal population and DRE. Therapeutic plCBZ was achieved using the low dose. CT genotype responds to lower dCBZ, while TT genotype outreached the highest toxic plCBZ.

Kuesioner Sindrom Terowongan Karpal Boston (KSTK-B) merupakan kuesioner dalam dikembangkan dalam Bahasa Inggris untuk menilai derajat keparahan dan status fungsional pada Sindrom Terowongan Karpal(STK). Penelitian ini bertujuan untuk mendapatkan kuesioner KSTK-B versi bahasa Indonesia yang valid danreliabel. Dilakukan adaptasi dan penerjemahan transkultural KSTK-B ke bahasa Indonesia, dilanjutkan ujivaliditas menggunakan uji korelasi Pearson dan uji reliabilitas menggunakan nilai alpha Cronbach. Penelitiandilakukan pada bulan Juli 2018 hingga September 2018. Subjek penelitian didapatkan sebanyak tiga puluhlima pasien dewasa dengan STK yang datang ke Poli Rawat Jalan Neurologi terdiri dari 31 perempuan, usiaberkisar antara 45 - 71 tahun dengan 32 subyek berusia = 50 tahun. Pada uji validitas domain derajat keparahangejala pada uji pertama nilai r antara 0,484-0,781; r pada uji kedua 0,482-0,760; untuk domain status fungsionaldidapatkan nilai r 0,495-0,825; dan 0,615-0,783 pada uji kedua. Hasil uji reliabilitas, domain derajat keparahangejala nilai alfa Cronbach 0,876 pada uji pertama dan 0,874 pada uji kedua, untuk uji reliabilitas domain statusfungsional pada uji pertama sebesar 0,857 dan 0,854 pada uji kedua. Kuesioner KSTK-B versi Bahasa Indonesiavalid dan reliabel dalam mengevaluasi keluhan serta gejala pada penderita dengan STK.

HIV-associated sensory neuropathy (HIV-SN) affects 14–38% of HIV+ individuals stable on therapy with no neurotoxic drugs. Polymorphisms in CAMKK2, P2X7R and P2X4R associated with altered risk of HIV-SN in Indonesian and South African patients. The role of CaMKK2 in neuronal repair makes this an attractive candidate, but a direct role for any protein is predicated on expression in affected tissues. Here, we describe expression of CaMKK2, P2X7R and P2X4R proteins in skin biopsies from the lower legs of HIV+ Indonesians with and without HIV-SN, and healthy controls (HC). HIV-SN was diagnosed using the Brief Peripheral Neuropathy Screen. Biopsies were stained to detect protein gene product 9.5 on nerve fibres and CaMKK2, P2X7R or P2X4R, and were examined using 3-colour sequential scanning confocal microscopy. Intraepidermal nerve fibre densities (IENFD) were lower in HIV+ donors than HC and correlated directly with nadir CD4 T-cell counts (r = 0.69, p = 0.004). However, IENFD counts were similar in HIV-SN+ and HIV-SN− donors (p = 0.19) and so did not define neuropathy. CaMKK2+ cells were located close to dermal and epidermal nerve fibres and were rare in HC and HIV-SN− donors, consistent with a role for the protein in nerve damage and/or repair. P2X7R was expressed by cells in blood vessels of HIV-SN− donors, but rarely in HC or HIV-SN+ donors. P2X4R expression by cells in the epidermal basal layer appeared greatest in HIV-SN+ donors. Overall, the differential expression of CaMKK2, P2X7R and P2X4R supports the genetic evidence of a role for these proteins in HIV-SN.

Neurotoxic anti-retroviral therapy (ART) such as stavudine has now been replaced with safer therapies, reducing the prevalence of neuropathy from 34% to 15% in HIV+ Indonesians. However, it is unclear whether the residual cases display damage to small or large nerve fibres, and whether both are influenced by known risk factors, including alleles of CAMKK2 associated with neuropathy in HIV patients. The encoded protein influences the growth and repair of nerve fibres.HIV-positive adults on ART for > 12 months without exposure to stavudine were screened for neuropathy using the AIDS Clinical Trials Group Brief Peripheral Neuropathy Screen (BPNS). Large fibre neuropathy was assessed by nerve conduction (NC) and small fibre neuropathy using Stimulated Skin Wrinkling (SSW) applied to the fingers. CAMKK2 alleles were assessed by TaqMan OpenArray technology.Neuropathy diagnoses were more common with SSW than BPNS (49/173 vs 26/185, χ; p=0.0009), with poor alignment between these outcomes (p=0.60). NC and BPNS diagnosed neuropathy at similar frequencies (29/151 vs 26/185; p=0.12) and were aligned (p

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.

HIV-associated sensory neuropathy (HIV-SN) is a debilitating neurological complication of HIV infection potentiated by the antiretroviral drug stavudine. While stavudine is no longer used, HIV-SN now affects around 15% of HIV+ Indonesians. Here, we investigate whether polymorphisms within the P2X-block (P2X4R, P2X7R, CAMKK2) and/or ANAPC5 mark susceptibility to HIV-SN in this setting. As polymorphisms in these genes associated with HIV-SN in African HIV patients receiving stavudine, the comparison can identify mechanisms independent of stavudine. HIV patients who had never used stavudine (n = 202) attending clinics in Jakarta were screened for neuropathy using the AIDS Clinical Trials Group Brief Peripheral Neuropathy Screen. Open-array technology was used to type 48 polymorphisms spanning the four genes. Haplotypes were derived for each gene using fastPHASE. Haplogroups were constructed with median-joining methods. Multivariable models optimally predicting HIV-SN were based on factors achieving p

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).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.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).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.

ANTI-NMDA RECEPTOR ENCEPHALITIS: ENCEPHALITIS PRESENTING WITH EARLY PSYCHOSIS SYMPTOMSABSTRACTAnti N-methyl-D-aspartate (NMDA) receptor encephalitis is seldom found in Indonesia. The atypical initial presentation of psychosis and cognitive disturbance results in underdiagnosed, or even undiagnosed case. This report described a 12-year-old girl presenting with initial psychiatric symptom followed by seizure and oropharyngeal dystonia. Anti-NMDA receptor antibody test carried out on both serum and cerebrospinal fluid (CSF) were positive and no other pathogen was detected in the CSF. Clinical improvement was seen following the third day of methylprednisolone administration. This case demonstrates the importance of considering anti-NMDA receptor encephalitis or the other autoimmune encephalitis as the differential of infective encephalitis. Anti-NMDA receptor antibody test, which can already be performed in Indonesia, will greatly guide to the definitive diagnosis.Keywords: Encephalitis, NMDA, oropharyngeal dystonia, psychiatricABSTRAKEnsefalitis antireseptor N-metil-D-aspartat (NMDA) masih jarang ditemukan di Indonesia. Gejala awal yang tidak khas berupa psikosis dan gangguan kognitif menyebabkan keterlambatan diagnosis, atau bahkan tidak terdiagnosis, ensefalitis jenis ini. Berikut dilaporkan kasus seorang perempuan 12 tahun dengan gejala awal berupa gejala psikiatrik yang diikuti dengan kejang dan munculnya distonia orofaring. Pemeriksaan antibodi antireseptor NMDA pada serum dan cairan serebrospinal (CSS) positif dan tidak ditemukan patogen lain pada pemerikaan CSS. Perbaikan klinis terlihat setelah pemberian metilprednisolon selama 3 hari. Kasus ini memperlihatkan pentingnya mempertimbangkan diagnosis ensefalitis antireseptor NMDA atau ensefalitis autoimun lainnya sebagai diagnosis banding pada kasus ensefalitis infeksi. Oleh karena itu, pemeriksaan antibodi antireseptor NMDA yang telah dapat dilakukan di Indonesia akan sangat membantu proses penegakan diagnosis.Kata kunci: Distonia orofaring, ensefalitis, NMDA, psikiatrik

Pendahuluan: Miastenia gravis (MG) merupakan penyakit autoimun kronik pada taut neuromuskular dengan gejala kelemahan fluktuatif. Kemajuan dalam diagnosis dan tatalaksana kasus MG akan meningkatkan angka harapan hidup pasien, sehingga evaluasi keberhasilan terapi tidak lagi hanya didasarkan pada mengatasi gejala, namun juga dalam mengevaluasi kualitas hidup pasien. The 15-item Myasthenia Gravis Quality of Life scale (MG-QOL15) merupakan kuesioner yang digunakan saat ini untuk mengevaluasi kualitas hidup pada pasien MG. Tujuan: Mendapatkan instrumen MG-QOL15 versi bahasa Indonesia yang valid dan reliabel. Metode: Empat puluh empat pasien penyakit MG di Poliklinik Neurologi Rumah Sakit Cipto Mangunkusumo yang memenuhi kriteria inklusi diikutsertakan dalam penelitian potong lintang ini. Pasien mengisi kuesioner MG-QOL15 sebanyak 2 kali dengan jarak waktu 2 hari. Konsep yang digunakan untuk uji validitas MG-QOL15 INA adalah validasi lintas budaya menurut metode World Health Organization (WHO). Uji reliabilitas dinilai menggunakan nilai alfa Cronbach. Hasil: MG-QOL15 INA telah melalui validasi lintas budaya menurut WHO dengan nilai koefisien korelasi Spearman berkisar antara 0,568-0,789 pada pemeriksaan pertama dan 0,574-0,763 pada retest. Nilai alfa Cronbach pada pemeriksaan pertama 0,917 dan 0,909 untuk re-test. Diskusi: MG-QOL15 INA valid dan reliabel untuk digunakan sebagai instrumen evaluasi kualitas hidup pasien MG. Kata Kunci: MG-QOL15, Miastenia gravis, kualitas hidup

Pendahuluan: Antibodi reseptor asetilkolin (anti-AChR) merupakan antibodi utama dalam patogenesis penyakit miastenia gravis (MG). Pemeriksaan antibodi ini merupakan salah satu pemeriksaan penting dalam menegakkan diagnosa. Gambaran klinis MG dapat terbagi menjadi MG okular, MG generalisata dan MG bulbar. Tiap individu dapat memiliki derajat keparahan yang berbeda yang dinilai berdasarkan MG composite score (MG-cs). Tujuan: Untuk mengetahui adanya hubungan kadar anti-AChR dengan derajat keparahan penyakit MG berdasarkan MG-cs di RS Cipto Mangunkusumo (RSCM). Metode: Penelitian ini merupakan studi potong lintang yang melibatkan pasien MG di RSCM pada bulan Januari 2017- November 2017. Kriteria inklusi subjek ialah pasien yang telah didiagnosa MG berusia 18-75 tahun. Pemeriksaan kadar anti-AChR dengan metode ELISA. Penilaian MG-cs dilakukan bersamaan dengan pengambilan sampel darah. Hasil: Didapatkan 72 subjek yang sesuai kriteria inklusi. Rerata usia subjek adalah 43 tahun (SD 12,56) dengan jenis kelamin perempuan lebih banyak (2,5:1). Gejala okular merupakan gejala terbanyak saat awitan (79,2%) dan early onset MG lebih banyak ditemukan (77,8%). Sebanyak 59,7% subjek memiliki seropositif anti-AChR. Tidak ada perbedaan bermakna seropositifitas anti-AChR pada kelompok berdasarkan jenis kelamin, usia awitan, tipe MG, dan pemberian terapi imunosupresan. Tidak didapatkan hubungan bermakna antara kadar anti-AChR dengan MG-cs (p=0,727). Diskusi: Kadar anti-AChR tidak berhubungan dengan derajat keparahan penyakit MG. Kata kunci: Antibodi reseptor asetilkolin, derajat keparahan MG, MG-cs

HORMONE AND EPILEPSY INTERACTIONABSTRACTHormones are signaling chemical molecules between cells that circulated in blood within the human body and play important roles for human being. Unfortunately, not all their functions are known, especially in epilepsy. Studies reported interactions between hormones and epilepsy, also with antiepileptic drugs (AEDs). For example, seizures may be induced by estrogen, whileAEDs can decrease hormone level by inducing enzyme in P450 cytochrome and accelerating hormone clearance.These conditions are known as tridirectional interactions. Understanding the interactions between hormones, epilepsy and AEDs would raise an awareness and insight for clinicians especially neurologist for basic knowledges as considerations in treating patients with epilepsy.Keyword: Antiepileptic drugs, epilepsy, hormone, seizureABSTRAKHormon merupakan molekul pembawa pesan kimiawi antar sel yang berada dalam tubuh manusia, beredar di dalam darah dan sangat penting bagi kehidupan manusia. Perannya pada epilepsi belum seluruhnya diketahui, akan tetapi berbagai penelitian yang ada menunjukan adanya interaksi antara hormon dan epilepsi begitu juga terhadap obat antiepilepsi (OAE). Sebagai contoh, bangkitan epilepsi dapat dicetuskan oleh estrogen, namun beberapa OAE yang merupakan penginduksi enzim sitokrom P450 di hati, dapat menurunkan kadar hormon di dalam tubuh dan juga mempercepat pembersihan hormon terutama hormon steroid seks. Hubungan ketiga faktor ini menciptakan hubungan yang disebut hubungan tridireksional. Dengan memahami mengenai interaksi yang terjadi antara hormon, epilepsi dan OAE, diharapkan dapat membuka wawasan bagi neurologis untuk memiliki pengetahuan dasar sebagai pertimbangan dalam tata laksana pasien epilepsi.Kata kunci: Bangkitan,epilepsi, hormon, obat antiepilepsi

OBJECTIVE HIV-associated sensory neuropathy (HIV-SN) remains common in HIV+ individuals receiving antiretroviral therapy (ART), even though neurotoxic antiretroviral drugs (e.g. stavudine) have been phased out of use. Accumulating evidence indicates that the neuropathy is immune-mediated. We hypothesize that chemokines produced locally in the skin promote migration of macrophages and T cells into the tissue, damaging cutaneous nerves causing HIV-SN. DESIGN We assessed chemokine receptor expression on infiltrating CD14 and CD3 cells around cutaneous nerves in standardized skin biopsies from HIV-SN+ patients (n = 5), HIV-SN- patients (n = 9) and healthy controls (n = 4). METHODS The AIDS Clinical Trials Group Brief Peripheral Neuropathy Screen was used to assess Indonesian HIV+ patients receiving ART without stavudine (case definition: bilateral presence of at least one symptom and at least one sign of neuropathy). Distal leg skin biopsies were stained to visualize chemokine receptors (CCR2, CCR5, CXCR3, CXCR4, CX3CR1), infiltrating CD3 and CD14 cells, and protein-gene-product 9.5 on nerves, using immunohistochemistry and 4-colour confocal microscopy. RESULTS Intraepidermal nerve fibre density was variable in patients without HIV-SN and generally lower in those with HIV-SN. CX3CR1 was more evident on CD14 cells whereas CCR2, CCR5, CXCR3 and CXCR4 were more common on CD3 cells. Expression of CX3CR1, CCR2 and CCR5 was more common in HIV-SN+ patients than those without HIV-SN. CXCR3 and CXCR4 were upregulated in all HIV+ patients, compared with healthy controls. CONCLUSION Inflammatory macrophages expressing CX3CR1 and T cells expressing CCR2 and CCR5 may participate in peripheral nerve damage leading to HIV-SN in HIV+ patients treated without stavudine. Further characterization of these cells is warranted.

HIV-associated sensory neuropathy (HIV-SN) is a disabling complication of HIV disease and antiretroviral therapies (ART). Since stavudine was removed from recommended treatment schedules, the prevalence of HIV-SN has declined and associated risk factors have changed. With stavudine, rs1799964*C (TNF-1031) associated with HIV-SN in Caucasians and Indonesians but not in South Africans. Here, we investigate associations between HIV-SN and rs1799964*C and 12 other polymorphisms spanning TNF and seven neighboring genes (the TNF-block) in Indonesians (n = 202, 34/168 cases) and South Africans (n = 75, 29/75 cases) treated without stavudine. Haplotypes were derived using fastPHASE and haplotype networks built with PopART. There were no associations with rs1799964*C in either population. However, rs9281523*C in intron 10 of BAT1 (alternatively DDX39B) independently associated with HIV-SN in Indonesians after correcting for lower CD4 T-cell counts and &gt, 500 copies of HIV RNA/mL (model p = 0.0011, Pseudo R2 = 0.09). rs4947324*T (between NFKBIL1 and LTA) independently associated with reduced risk of HIV-SN and African haplotype 1 (containing no minor alleles) associated with increased risk of HIV-SN after correcting for greater body weight, a history of tuberculosis and nadir CD4 T-cell counts (model: p = 0.0003, Pseudo R2 = 0.23). These results confirm TNF-block genotypes influence susceptibility of HIV-SN. However, critical genotypes differ between ethnicities and with stavudine use.

Introduction. Parsonage Turner Syndrome (PTS) is a rare syndrome that may occur in healthy subjects. The clinical presentation might not be typical and risk factors attributed to the development of disease may not be easily depicted. We present a case of abrupt onset of unilateral shoulder pain, followed by progressive neurologic weakness. Result. A 54-year-old male presented with abrupt onset of shoulder pain mainly on the right side. Pain was felt in both shoulder and thigh which progressed to weakness, dysesthesia, and numbness in the right upper arm. In 4 days, he could barely lift his right upper arm. There was no history of trauma nor infection on the affected area. On physical examination, there were limitation of shoulder abduction and external rotation. His right arm strength from proximal to distal was 1344. Other extremities showed no significant weakness. Sensory involvement did not match dermatomal and peripheral sensory distribution. Nerve Conduction Velocity (NCV) showed axonal motor neuropathy of right axillary nerve. Electromyography (EMG) showed no Motor Unit Action Potential (MUAP) on deltoid muscle. His CK was 457, and there was an increase in LDH and CRP. Cervical MRI was unremarkable. Steroids were immediately given, and the response was fast and satisfactory. After 1 day, his motor strength increased, and the pain subsided. EMG showed single MUAP on deltoid. After 10 days, his motor strength returned to near normal. NCV study showed decreased amplitude of right axillary nerve, and recruitment was slightly decreased on right deltoid muscle. He underwent physical therapy and was making marked progress. Conclusion. PTS generally involves one upper limb, and axillary nerve is one the most commonly involved. PTS should be considered as a differential diagnosis in cases of shoulder pain and arm weakness without any clear etiology.

Introduction. Repetitive Nerve Stimulation (RNS) has a sensitivity of 50-80% in predicting Myasthenia Gravis (MG). This wide range is affected by type and severity of MG, muscle tested, frequency of stimulation and exercise. There has been a previous study describing these factors but did not confirmed it with the result of Single Fiber (SF) and Acetylcholine Receptor antibody (AChR). Methods. This was a cross-sectional study. Data was from RNS results of MG patients in Electromyography laboratories, Cipto Mangunkusumo National Hospital January to December 2019. Muscle tested on RNS were abductor digiti minimi (ADM) muscle, trapezius (Tr) muscle and orbicularis occuli (OrbOc) muscle. Slow RNS used 3, 5, 7 and 10 Hz stimulation followed by 3 Hz stimulation immediate, 1 minute (min) and 2 min post exercise. Decrease of Compound Muscles Action Potential (CMAP) amplitude > 10% is considered as positive RNS. Results. As much as 47 patients were included in this study. The average age of patients is 43 years old. As much as 44.7% had a positive RNS test, and 70% of the case was positive from OrbOc, followed by Tr (60%) and ADM (30%). Frequency of stimulation that obtained the positivity the most is at 5 Hz, and the 4th wave revealed the lowest point of decreament, followed by the 5th wave. Single Fiber was positive in 57.4% of the case, and AChR was positive in 51.1%. There are 11 patients (23.4%) that has a mismatch results between SF and AChR. Conclusion. RNS without confirmation of SF and AChR may yield a false negative result. Orbicularis Oculi shows the most sensitive muscle tested in RNS, with stimulation of 5 Hz and the 4th waveform the lowest decrement. SF and AChR are still important in confirming strong suspicion patients with MG despite negative results in RNS.

HIV-associated sensory neuropathy (HIV-SN) remains common in HIV patients receiving anti- retroviral therapy (ART). Neuropathies resulting from HIV infection itself and toxic side effects of ART have similar presentations and include neuropathic pain, tingling and numbness. Some nucleoside analogue reverse transcriptase inhibitors used to treat HIV were strongly associated with HIV-SN. The prevalence of HIV-SN in patients receiving stavudine at Cipto Mangunkusumo Hospital was 34% and had strong association with age and height. As stavudine has been phased out recently, some patients still have HIV-SN. In 2016, the prevalence of HIV-SN had been significantly reduced became 14.2% and associated factors was viral load >500 copies HIV RNA/mL. It is an evidence that lack of stavudine exposure is central to the lower neuropathy prevalence. One of clinical manifestation of HIV-SN is neuropathic pain. Neuropathic pain can be found as solely symptom without other sensory neuropathy symptoms (numbness) and signs (decreased physiological reflex and vibration). Prevalence of neuropathic pain (NP) of HIV patients was 6.6% at Cipto Mangunkusumo Hospital, smaller than HIV-SN. Our study showed that HIV patients with NP had shorter duration of ART than patients with HIV-SN, and may be an early symptom of small fiber neuropathy in HIV patients. Some evidences indicate that HIV-SN is immune-mediated. Intra-epidermal fiber density of HIV-SN patients is lower than HIV-noSN and healthy control. Low fiber density in HIV-SN seems to be corelated with low nadir CD4+ counts. CD14+ macrophages were evident at surrounding intra-epidermal nerve of HIV-SN and HIV-noSN patients. Some chemokine receptors such as CX3CR1, CCR2 and CCR5 was more common in HIV-SN patients. These evidences showed that inflammatory macrophages expressing chemokine receptors may participate in peripheral nerve damage leading to HIV-SN in HIV patients treated without stavudine.

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. Keywords: Neural leprosy, anti-PGL-1, systemic corticosteroid, multidisciplinary approach Normal 0 false false false EN-US X-NONE X-NONE

Background: Nonconvulsive status epilepticus (NCSE) is often underdiagnosed in patients with metabolic encephalopathy (ME). The diagnosis of ME should be made specifically to recognize the underlying etiology. Delay in seizure identification and making a diagnosis of NCSE contributed to the poor outcome. Objective: This study aimed to find the incidence and outcome of NCSE in patients with ME. Methods and Material: This was an observational prospective cross-sectional study in patients with ME in emergency and critical care units in Cipto Mangunkusumo General Hospital. The diagnosis of NCSE was based on EEG using Salzburg Criteria for Nonconvulsive Status Epilepticus (SCNC). The outcome was assessed within 30 days after the NCSE diagnosis has been made. Results: A total of 50 patients with ME were involved in this study. NCSE was confirmed in 32 subjects (64%). The most common etiology of ME was sepsis (58%). The mortality rate in the NCSE and non-NCSE group was 40.6% vs 44.4%. Multiple aetiologies were risk factors to poor outcome in the NCSE group. Conclusions: The incidence of NCSE among patients with ME at our hospital was high. Despite the anti-epileptic treatment of the NCSE group, the underlying cause of ME is still the main factor that affected the outcome. Therefore, aggressive treatment of anti-epileptic drug (AED) should be very carefully considered knowing the possible side-effect that might worsen the outcome of patients with ME.

Lower limb neuropathic pain in HIV patients is a common manifestation of sensory neuropathy (HIV-SN), but can be seen in patients who do not meet standard definitions of HIV-SN. The drug stavudine is a risk factor for HIV-SN, but some patients treated without stavudine experience HIV-SN, and the prevalence and risk factors influencing neuropathic pain in this setting are unknown. A cross sectional study at Cipto Mangunkusumo Hospital Jakarta tested 197 HIV patients treated for >12 months without stavudine. HIV-SN was defined using the AIDS Clinical Trial Group Brief Peripheral Neuropathy Screening Test (ACTG-BPNST). A validated Indonesia translation of Douleur Neuropathique en 4 (DN4) questionnaire was used to assess lower limb neuropathic pain. Nerve conduction studies assessed large nerve fiber function and Stimulated Skin Wrinkle (SSW) tests were performed to assess small nerve fibers. The prevalence of neuropathic pain was 6.6%. BPNST+HIV-SN was diagnosed in 14.2% of the cohort and 38.5% of patients with pain. Use of protease inhibitors and ART duration

Introduction Erasmus GBS Outcome Score (EGOS) is a model to predict the outcome of patients after GBS. EGOS studies were conducted in well developed countries, which have different patient characteristics and medical facility than in the middle-income country like Indonesia. In the middle-income country, the prognosis could be different because of limited medical facility and the lack of patient’s awareness. Methods This is a retrospective study of GBS patients who was admitted to Cipto Mangunkusumo Hospital (CMH) from 2010 to 2015. We reviewed demographic and clinical characteristics, EGOS, and the outcome of GBS patients. Results GBS patient who was admitted to CMH were 11.6 cases/year. There was no seasonal variation of GBS onset. Male-to-female ratio was 1.2: 1. Mean age of patients was 41.7 years old. The most frequent variant of GBS was AIDP (37.5%), followed by AMSAN (25%), AMAN (25%) and MFS (12.5%). Median duration of onset – to hospital was 8.5 days. In 83.3% patients the first plasma exchange was started at more than two weeks after the onset. Proportion of patients with good outcome was 74.4%. Higher EGOS tend to have higher proportion of patients with poor outcome. Conclusion This study shows EGOS can be applied at middle income country with limited resources and still have good trend to predict the outcome of the GBS even if the immunotherapy was started more than two weeks after the onset.

Small and large amplitude saccades serve different functions in daily life, with the latter accompanied more frequently by head movements and blinks. They are presumably controlled differently by the frontal eye fields. To investigate the frontal cortical regions responsible for the initiation and inhibition of small and large amplitude saccades, we investigated the effects of transcranial magnetic stimulation (TMS) on antisaccades of different sizes (5° and 30°). While the subjects performed a specific task, we stimulated the bilateral frontal cortices at various locations overlying the frontal oculomotor regions, 100 ms after the target presentation. A hemispheric asymmetry in TMS effect was noted, such that 5° saccades were delayed by TMS over both bilateral frontal cortices, whereas 30° saccades were delayed by TMS only over the left lateral frontal cortex. TMS also reduced the amplitude of 5° saccades over the bilateral frontal cortex, but 30° saccades were not significantly changed in amplitude. The frequency of erroneous prosaccades to targets was slightly decreased by TMS at 5°, but increased at 30°. The results suggest that 5° saccades are more susceptible to the effect of TMS than are 30° saccades. Furthermore, while bilateral frontal areas are involved in the initiation of antisaccades, especially of small amplitudes, the left lateral frontal cortex may subserve coordination of blinks and saccades, both of large and small amplitudes.

Magnetic round coil stimulation over the spinal enlargement activates the spinal nerves at the neuro-foramina level. However, activation of the cauda equina in the spinal canal has never been described in the literature. This study, for which 40 healthy subjects were recruited, activated the cauda equina using a round 20-cm-diameter coil designated as a Magnetic Augmented Translumbosacral Stimulation (MATS) coil. Magnetic stimulation placing the edge of the coil over the L1 and L3 spinous processes elicited compound muscle action potentials (CMAPs) from the abductor hallucis muscle. The CMAPs were compared with those elicited through high-voltage electrical stimulation. The CMAP latencies to L1 level MATS coil stimulation were not significantly different from those evoked by electrical stimulation at the same level. The CMAP latencies to L3 level MATS coil stimulation were varied in each subject. In fact, the L1 level MATS coil stimulation is considered to activate the cauda equina at the root exit site from the conus medullaris; the L3 level MATS coil stimulation activates some mid-part of the cauda equina or the distal cauda equina by spreading current. The MATS coil facilitates evaluation of spinal nerve conduction in the cauda equina.

Objective The aim of this paper is to develop a reliable method for supramaximal magnetic spinal motor root stimulation (MRS) for lower limb muscles using a specially devised coil. Methods For this study, 42 healthy subjects were recruited. A 20-cm diameter coil designated as a Magnetic Augmented Translumbosacral Stimulation (MATS) coil was used. Compound muscle action potentials (CMAPs) were recorded from the abductor hallucis muscle. Their CMAPs were compared with those obtained by MRS using a conventional round or double coil and with those obtained using high-voltage electrical stimulation. Results The MATS coil evoked CMAPs to supramaximal stimulation in 80 of 84 muscles, although round and double coils elicited supramaximal CMAPs in only 15 and 18 of 84 muscles, respectively. The CMAP size to the MATS coil stimulation was the same as that to high-voltage electrical motor root stimulation. Conclusions MATS coil achieved supramaximal stimulation of the lumbosacral spinal nerves. Significance The CMAPs to supramaximal stimulation are necessary for measurement of the amplitude and area for the detection of conduction blocks. The MATS coil stimulation of lumbosacral motor roots is a reliable method for measuring the CMAP size from lower limb muscles in spinal motor root stimulation.