Autoimmune encephalitis in a tertiary hospital in the Philippines.

BACKGROUND: Autoimmune encephalitis (AE) is an emerging disorder in adults and children. Due to its potentially reversible nature, prompt recognition and intervention are of utmost importance. OBJECTIVE: To describe the clinical and paraclinical features, as well as treatment outcomes of patients with AE admitted in a Philippine tertiary hospital. METHODS: Retrospective case series of patients with definite AE. RESULTS: Eighteen (18) patients were included (12 adults, 6 children), majority of whom had anti-N-methyl-d-aspartate receptor (NMDAR) encephalitis. The median age of onset was 32 (IQR: 10.8) years old and 13 (IQR: 4.8) years old in the adult and pediatric population, respectively. In both age groups, most presented with psychiatric symptoms and normal imaging findings. Cerebrospinal fluid (CSF) pleocytosis was detected in 8/12 (66.7%) adults and 2/6 (33.3%) children, while CSF protein elevation was only seen in 6/12 (50%) adults. Most patients presented with seizures, and the most frequent electroencephalography (EEG) abnormality detected was slow activity (70.5%). A high proportion of patients received high dose steroids, alone (35.3%) or in combination with intravenous immunoglobulin (IVIG, 52.9%). Overall, 66.7% had improved outcomes, mostly seen in the pediatric population. CONCLUSION: This study highlighted the broad clinical phenotype, as well as the similarities and differences of AE manifestations in adults and children. It demonstrated the limited but supportive role of laboratory investigations in the diagnosis of AE. It also underscored the importance of early intervention in AE and highlighted factors influencing treatment practices and discharge outcomes in the local setting.

Cerebellar ataxia as a primary manifestation of neuropsychiatric systemic lupus erythematosus.

Acute cerebellar ataxia is a rare primary manifestation of neuropsychiatric systemic lupus erythematosus (NPSLE). We report a case of a 22-year-old woman who presented with gait instability, behavioural changes and new-onset seizures. The tempo of disease progression was explained by an autoimmune cause, eventually fulfilling the criteria for systemic lupus erythematosus. The patient's neurological symptoms improved markedly following administration of steroids and immunomodulators. A review of literature on cerebellar ataxia in NPSLE and a summary of all reported cases to date are also presented.

Treatment gaps and challenges in epilepsy care in the Philippines.

OBJECTIVE: Epilepsy is a neurologic disease that carries a high disease burden and likely, a huge treatment gap especially in low-to-middle income countries (LMIC) such as the Philippines. This review aimed to examine the treatment gaps and challenges that burden Philippine epilepsy care. MATERIALS & METHODS: Pertinent data on epidemiology, research, health financing and health systems, pharmacologic and surgical treatment options, cost of care, and workforce were obtained through a literature search and review of relevant Philippine government websites. RESULTS: The estimated prevalence of epilepsy in the Philippines is 0.9%. Epilepsy research in the Philippines is low in quantity compared with the rest of Southeast Asia (SEA). Inequities in quality and quantity of healthcare services delivered to local government units (LGUs) have arisen because of devolution. Programs for epilepsy care by both government and nongovernment institutions have been implemented. Healthcare expenditure in the Philippines is still largely out-of-pocket, with only partial coverage from the public sector. There is limited access to antiseizure medications (ASMs), mainly due to cost. Epilepsy surgery is an underutilized treatment option. There are only 20 epileptologists in the Philippines, with one epileptologist for every 45,000 patients with epilepsy. In addition, epilepsy care service delivery has been further impeded by the coronavirus disease of 2019 (COVID-19) pandemic. CONCLUSION: There is a large treatment gap in epilepsy care in the Philippines in terms of high epilepsy disease burden, socioeconomic limitations and inadequate public support, sparse clinico-epidemiologic research on epilepsy, inaccessibility of health care services and essential pharmacotherapy, underutilization of surgical options, and lack of specialists capable of rendering epilepsy care. Acknowledgment of the existence of these treatment gaps and addressing such are expected to improve the overall survival and quality of life of patients with epilepsy in the Philippines.

Clinical features and outcomes of nonconvulsive status epilepticus in a developing country: A 5-year retrospective study.

BACKGROUND: This study aimed to determine the frequency of electrographically confirmed nonconvulsive status epilepticus (NCSE) in a cohort suspected with this condition and to determine the demographic/clinical profile, treatment, and outcomes of these patients in the context of a developing country, the Philippines. METHODS: We conducted a retrospective study among patients with suspected with NCSE admitted in the Philippine General Hospital from 2014 to 2019. Using the Salzberg 2013 criteria to diagnose NCSE, three electroencephalographers independently reviewed the electroencephalogram (EEG) tracings of suspected patients and were blinded from the clinical information. Then, we obtained pertinent clinical data from the medical records of EEG-confirmed NCSE cases. RESULTS: Out of 89 patients suspected with NCSE and with available EEG tracings, information from a total of 14 patients (15.7%) with electrographically confirmed cases were included in the analysis. Median age was 52 ranging from 22 to 77 and female-to-male ratio was 1.3:1. The following conditions were associated with NCSE: intracranial tumor (n = 4), metabolic encephalopathy (n = 4), autoimmune encephalitis (n = 3), intracranial hemorrhage (n = 3), sepsis (n = 3), cardiac arrest (n = 2), hypoxic-ischemic injury (n = 2), antiepileptic withdrawal (n = 1), intracranial abscess (n = 1), head trauma (n = 1), and meningitis (n = 1). Three patients (21.4%) had relatively good clinical outcomes (mRS 0-2) while 6 patients (42.8%) had poor outcomes (mRS 3-5) at discharge. Five patients (35.7%) died due to medical/neurological complications. Our review of the literature showed that the profile of NCSE cases identified in our resource-limited institution strengthens the findings in other populations. CONCLUSION: Our data showed that approximately 1 in 6 patients who are suspected with NCSE may have electrographic evidence of NCSE in our setting. The most common etiologies associated with NCSE were intracranial tumors and metabolic conditions. Further studies may entail a prospective collection of data to validate the estimates of our study.

Electroencephalographic findings in antileucine-rich glioma-inactivated 1 (LGI1) autoimmune encephalitis: A systematic review.

BACKGROUND: Antileucine-rich glioma-inactivated 1 (LGI1) autoimmune encephalitis is a distinct syndrome of early onset faciobrachial dystonic seizures (FBDS) and other focal seizures followed by neuropsychiatric symptoms. The electroclinical syndrome of this condition is yet to be fully understood. The main objective of this study was to determine the frequencies of electroencephalogram (EEG) findings in patients diagnosed with anti-LGI1 autoimmune encephalitis through a systematic review of published relevant articles. METHODS: Major electronic healthcare databases were searched for relevant published articles until July 2020. RESULTS: From 327 articles, 23 case reports and 14 case series were included in the review after satisfying the eligibility criteria. Data from a total of 151 cases of definite anti-LGI1 encephalitis were analyzed in this systematic review. Epileptiform discharges were the most frequent finding at 57.3%. Focal electroencephalographic abnormalities including slow wave (59.3%) and epileptiform activities (53.5%) most commonly arise from the temporal region. Faciobrachial dystonic seizures often have no ictal electroencephalographic correlate but would show electrodecremental events when seizure duration is prolonged. CONCLUSION: Electroencephalogram abnormalities are seen in anti-LGI1 autoimmune encephalitis with epileptiform discharges as the most common finding followed by focal slowing. These findings were most frequently seen arising in the temporal region. Further studies using a standardized protocol focusing on electroencephalographic findings in anti-LGI1 encephalitis with clinical correlation to disease stage and treatment outcomes in terms of seizure control and memory and cognitive function are needed. Particular attention on ictal electrodecremental activity during FBDS should be done in studies with a larger sample size.

Electroencephalographic findings in COVID-19 patients: A systematic review.

BACKGROUND: Growing evidence of neurologic involvement seen in COVID-19 infection necessitates the pooling of neurodiagnostic findings like electroencephalography (EEG) that may guide clinical management. The objective of this study was to review the EEG findings in patients diagnosed with COVID-19 infection through a systematic review of published articles. METHODS: We systematically searched until July 25, 2020 for published articles that reported on descriptive EEG findings in patients diagnosed with COVID-19 in PUBMED by Medline, EMBASE, and CENTRAL by the Cochrane Library. RESULTS: From a total of 94 identified records, 29 relevant articles were included in this review. A total of 177 patients with COVID-19 with descriptive EEG reports were analyzed. The most common indication for EEG was unexplained altered mental status. Disturbances of background activity such as generalized and focal slowing were seen as well epileptiform abnormalities and rhythmic or periodic discharges. There were no consistent EEG findings specific to COVID-19 infection. CONCLUSION: The EEG findings in COVID-19 appear to be non-specific. Further research on the relationship of the EEG findings to the clinical state and short- or long-term prognosis of COVID-19 patients may be conducted to help clinicians discern which patients would necessitate an EEG procedure and would eventually require treatment.

Efficacy and tolerability of intravenous brivaracetam for status epilepticus: A systematic review.

BACKGROUND: Status epilepticus (SE) is an emergent neurologic condition that carries a high risk of morbidity and mortality. Intravenous brivaracetam (IV BRV) may be an alternative anticonvulsant against status epilepticus, although the sparseness of controlled studies on the topic limits its recommendation for this indication. OBJECTIVES: This systematic review aimed to determine the efficacy and safety of IV BRV in the treatment of status epilepticus. METHODS: A comprehensive literature search was conducted until December 2019 through several electronic databases (PubMed, Google Scholar, Scopus, OpenGrey, ScienceDirect, HERDIN, Epistemonikos, CENTRAL, ClinicalTrials.gov) to identify relevant studies. Studies that involved adult patients with SE who were given IV BRV were considered for inclusion in this review. RESULTS: From a total of 34 studies identified, 5 uncontrolled studies with 77 patients were included in this review. Thirty-seven out of 77 patients (48%) with SE responded to IV BRV. Reported time to seizure cessation may be immediate from a few minutes to several hours after IV BRV treatment. Patients manifested with significant disability on Glasgow outcome scale (Median: 3) and modified Rankin scale (Mode: 5). Six patients [somnolence (5), worsening seizures (1)] had treatment emergent adverse events. CONCLUSIONS: Limited evidence from 5 uncontrolled studies involving a limited number of patients suggests that IV BRV may be efficacious and safe in terminating seizures among patients with SE or refractory SE. Further studies employing either prospective, controlled trials or registry-based study designs are essential to determine the definitive role of IV BRV in patients with SE.

Chronic inflammatory demyelinating polyneuropathy with tongue fasciculation: A case report.

Chronic inflammatory demyelinating polyneuropathy is one of the immune-mediated polyneuropathies responsive to immunotherapy. Its usual clinical presentation is a chronic course of symmetric sensorimotor affectation of both proximal and distal extremities with signs of demyelination on electrophysiologic studies. Cranial nerve signs in this condition is not commonly encountered, particularly involvement of the hypoglossal nerve. To date, there are only three published cases of hypoglossal involvement in this condition. This case report presents a patient with tongue fasciculation, which is an uncommon finding in chronic inflammatory demyelinating polyneuropathy. This paper highlights the importance of considering chronic inflammatory demyelinating polyneuropathy in the differential diagnoses of a patient with tongue fasciculation as it has been found to be responsive to immunotherapy in comparison to other lower motor neuron syndromes.

Autoimmune encephalitis associated with two antibodies.

•In patients presenting with clinical manifestations of encephalitis without clinical or laboratory signs of infection, an autoimmune etiology should be suspected.•Antibodies for various neural antigens may coexist, thus a complete and clinically-guided autoimmune panel must be done in suspected cases of autoimmune encephalitis.•Tumor resection, if applicable, combined with high dose steroids and immunotherapy are effective treatment strategies for autoimmune encephalitis with coexisting antibodies.