The real-world applicability of the 2023 international myelin oligodendrocyte glycoprotein antibody-associated disease criteria in a Latin American cohort.

BACKGROUND AND PURPOSE: The diagnostic criteria for myelin oligodendrocyte glycoprotein antibody (MOG-IgG)-associated disease (MOGAD) were published in 2023. We aimed to determine the performance of the new criteria in Latin American (LATAM) patients compared with the 2018 criteria and explore the significance of MOG-IgG titers in diagnosis. METHODS: We retrospectively reviewed the medical records of LATAM (Argentina, Chile, Brazil, Peru, Ecuador, and Colombia) adult patients with one clinical MOGAD event and MOG-IgG positivity confirmed by cell-based assay. Both 2018 and 2023 MOGAD criteria were applied, calculating diagnostic performance indicators. RESULTS: Among 171 patients (predominantly females, mean age at first attack = 34.1 years, mean disease duration = 4.5 years), 98.2% patients met the 2018 criteria, and of those who did not fulfill diagnostic criteria (n = 3), all tested positive for MOG-IgG (one low-positive and two without reported titer). Additionally, 144 (84.2%) patients met the 2023 criteria, of whom 57 (39.5%) had MOG-IgG+ titer information (19 clearly positive and 38 low-positive), whereas 87 (60.5%) patients had no MOG-IgG titer. All 144 patients met diagnostic supporting criteria. The remaining 27 patients did not meet the 2023 MOGAD criteria due to low MOG-IgG (n = 12) or lack of titer antibody access (n = 15), associated with the absence of supporting criteria. The 2023 MOGAD criteria showed a sensitivity of 86% (95% confidence interval = 0.80-0.91) and specificity of 100% compared to the 2018 criteria. CONCLUSIONS: These findings support the diagnostic utility of the 2023 MOGAD criteria in an LATAM cohort in real-world practice, despite limited access to MOG-IgG titration.

Gaps in clinical research in frontotemporal dementia: A call for diversity and disparities-focused research.

Frontotemporal dementia (FTD) is one of the leading causes of dementia before age 65 and often manifests as abnormal behavior (in behavioral variant FTD) or language impairment (in primary progressive aphasia). FTD's exact clinical presentation varies by culture, language, education, social norms, and other socioeconomic factors; current research and clinical practice, however, is mainly based on studies conducted in North America and Western Europe. Changes in diagnostic criteria and procedures as well as new or adapted cognitive tests are likely needed to take into consideration global diversity. This perspective paper by two professional interest areas of the Alzheimer's Association International Society to Advance Alzheimer's Research and Treatment examines how increasing global diversity impacts the clinical presentation, screening, assessment, and diagnosis of FTD and its treatment and care. It subsequently provides recommendations to address immediate needs to advance global FTD research and clinical practice.

Biomarkers for dementia in Latin American countries: Gaps and opportunities.

Limited knowledge on dementia biomarkers in Latin American and Caribbean (LAC) countries remains a serious barrier. Here, we reported a survey to explore the ongoing work, needs, interests, potential barriers, and opportunities for future studies related to biomarkers. The results show that neuroimaging is the most used biomarker (73%), followed by genetic studies (40%), peripheral fluids biomarkers (31%), and cerebrospinal fluid biomarkers (29%). Regarding barriers in LAC, lack of funding appears to undermine the implementation of biomarkers in clinical or research settings, followed by insufficient infrastructure and training. The survey revealed that despite the above barriers, the region holds a great potential to advance dementia biomarkers research. Considering the unique contributions that LAC could make to this growing field, we highlight the urgent need to expand biomarker research. These insights allowed us to propose an action plan that addresses the recommendations for a biomarker framework recently proposed by regional experts.

Diagnostic accuracy of brief cognitive screening tools to diagnose vascular cognitive impairment in Peru.

INTRODUCTION: To evaluate the diagnostic accuracy of three brief cognitive screening (BCS) tools, Peruvian version of Addenbrooke's Cognitive Examination (ACE-Pe), of INECO Frontal Screening (IFS-Pe) and of the Mini-Mental State Examination (MMSE-Pe), for the diagnosis of vascular cognitive impairment (VCI) and its non-dementia stages (VCI-ND) and vascular dementia (VD) in patients with cerebral stroke in Lima-Peru. MATERIALS AND METHODS: A cohort analysis to evaluate the diagnostic accuracy of three BCS for VCI. RESULTS: Two hundred and four patients were evaluated: 61% Non-VCI, 30% VCI-ND and 9% VD. To discriminate patients with VCI from controls, the area under the curve (AUC) of ACE-Pe, IFS-Pe and MMs-Pe were 0.99 (95% confidence interval [CI] 0.98-0.99), 0.99 (95%CI 0.98-0.99) and 0.87 (95%CI 0.82-0.92), respectively. Of the three BCS, the IFS-Pe presented a larger AUC to discriminate VCI-ND from VD (AUC = 0.98 [95%CI 0.95-1]) compared to ACE-Pe (AUC = 0.84 [95%CI 0.74-0.95]) and MMSE-Pe (0.92 [95%CI 0.86-0.99]). The IFS-Pe presented a higher sensitivity (S), specificity (Sp), and positive (+LR) and negative likelihood ratios (-LR) (S = 96.72%, Sp = 89.47%, +LR = 9.1 and -LR = 0.03) than ACE-Pe (S = 96.72%, Sp = 63.16%, +LR = 2.62 and -LR = 0.05) and MMSE-Pe (S = 90.16%, Sp = 78.95%, +LR = 4.28 and -LR = 0.12). In the multiple regression analysis, the IFS-Pe was not affected by age, sex or years of schooling. CONCLUSION: The IFS-Pe has the best diagnostic accuracy for detecting VCI and discriminating between pre-dementia (VCI-ND) and dementia (VD) stages.

The Neuropsychiatric Features of Behavioral Variant Frontotemporal Dementia.

Behavioral variant frontotemporal dementia (bvFTD) is a syndrome defined by a set of core clinical criteria, which include disinhibition; apathy or inertia; loss of sympathy or empathy; perseverative, stereotyped, or compulsive/ritualistic behavior; and hyperorality. The clinical features of bvFTD overlap substantially with those of psychiatric disease, particularly major depressive disorder and bipolar affective disorder. The similarities between bvFTD and primary psychiatric disease results in a significant diagnostic challenge for clinicians. Understanding the neuropsychiatric aspects of bvFTD may assist in differentiating bvFTD from a primary psychiatric disorder.

Occurrence of area postrema syndrome during follow-up: phenotype and influence over NMOSD activity in LATAM in real-world settings.

INTRODUCTION: We aimed to assess the frequency, duration, and severity of area postrema syndrome (APS) during follow-up in neuromyelitis optica spectrum disorder (NMOSD) patients, as well as its association with inflammatory activity and prognostic factors of APS severity in a real-world setting. METHODS: We conducted a retrospective study on a cohort of Latin American (LATAM) NMOSD patients who had experienced APS during their follow-up. Patients from Mexico, Peru, Brazil, Colombia, Panama, Chile and Argentina patients who met 2015 NMOSD criteria were included. We evaluated data on symptom type (nausea, vomiting and/or hiccups), frequency, duration, severity (measured by APS severity scale), association with other NMOSD core relapses, and acute treatments (symptomatic and immunotherapy or plasmapheresis). Logistic regression was conducted to evaluate factors associated with APS severity (vs. mild-moderate). RESULTS: Out of 631 NMOSD patients, 116 (18.3%) developed APS during their follow-up. The most common APS phenotype was severe. Inflammatory activity (i.e., relapses) significantly decreased after the onset of APS. Half of the patients experienced isolated APS with a median duration of 10 days, and the most frequently used acute treatment was IV steroids. All three symptoms were present in 44.6% of the patients. APS symptoms resolved following immunotherapy. Logistic regression did not identify independent factors associated with the severity of APS. CONCLUSIONS: Our findings indicate that 18.3% of NMOSD patients developed APS during the follow-up period, with most patients fulfilling criteria for severe APS. The inflammatory activity decreased after the onset of APS compared to the previous year.

Parasite antigen in serum predicts the presence of viable brain parasites in patients with apparently calcified cysticercosis only.

BACKGROUND: Computed tomography (CT) remains the standard neuroimaging screening exam for neurocysticercosis, and residual brain calcifications are the commonest finding. Magnetic resonance imaging (MRI) is more sensitive than CT but is rarely available in endemic regions. Enzyme-linked immunoelectrotransfer blot (EITB) assay uses antibody detection for diagnosis confirmation; by contrast, enzyme-linked immunosorbent assay (ELISA) antigen detection (Ag-ELISA) detects circulating parasite antigen. This study evaluated whether these assays predict undetected viable cysts in patients with only calcified lesions on brain CT. METHODS: Serum samples from 39 patients with calcified neurocysticercosis and no viable parasites on CT were processed by Ag-ELISA and EITB. MRI was performed for each patient within 2 months of serologic testing. Conservatively high ELISA and EITB cutoffs were used to predict the finding of viable brain cysts on MRI. RESULTS: Using receiver operating characteristic-optimized cutoffs, 7 patients were Ag-ELISA positive, and 8 had strong antibody reactions on EITB. MRI showed viable brain cysts in 7 (18.0%) patients. Patients with positive Ag-ELISA were more likely to have viable cysts than Ag-ELISA negatives (6/7 vs 1/32; odds ratio, 186 [95% confidence interval, 1-34 470.0], P < .001; sensitivity 85.7%, specificity 96.9%, positive likelihood ratio of 27 to detect viable cysts). Similar but weaker associations were also found between a strong antibody reaction on EITB and undetected viable brain cysts. CONCLUSIONS: Antigen detection, and in a lesser degree strong antibody reactions, can predict viable neurocysticercosis. Serological diagnostic methods could identify viable lesions missed by CT in patients with apparently only calcified cysticercosis and could be considered for diagnosis workup and further therapy.

Clinical and paraclinical profile of neuromyelitis optic spectrum disorder in a peruvian cohort.

BACKGROUND: Neuromyelitis Optica Spectrum Disorders (NMOSD) is an autoimmune, inflammatory disorder of the Central Nervous System that typically involves the spinal cord and the optic nerves. Recently, the clinical and radiological spectrum of NMOSD has been increasing in Latin America. In Peru, there have only been a few clinical reports on NMOSD published. For this reason, we aimed to assess the clinical and paraclinical characteristics of patients with NMOSD from a tertiary-level neurological center in Lima-Peru. METHODS: This is a descriptive study. We assessed medical reports of patients with NMOSD based on the 2015 diagnostic criteria attended in a goverment institute (Instituto Nacional de Ciencias Neurologicas) from Peru between 2013-2019. Those patients who met diagnostic criteria were selected and analyzed. We analyzed continuous data among groups (AQP4-IgG seropositive and AQP4-IgG seronegative/unknow). RESULTS: We identified 58 clinical records that met the selection criteria and were included in the study. The highest percentage of patients (53%) were born in the north of Peru (from parallel 0°01'48''S - 6°56'38''S). NMOSD were more prevalent in women (86%), the male:female ratio was 1:6, the mean age at diagnosis was 50 years. AQP4-IgG antibodies were tested in (63.8%), 62% of whom were seropositive and 38% seronegative. The frequency of EO-NMO and LO-NMO was 34.8% and 65.2% in AQP4-IgG seropositive patients, respectively. Unknown AQP4-IgG was found 21 patients. In LO-NMOSD group, AQP4-IgG seropositive was found in a higher percentage. Optic neuritis was the first clinical event at 40% . In the patients who presented myelitis as the first clinical event, 18.2% were AQP4-IgG seropositive, while only 4.8% was found in the rest of the patients. 17% had other associated autoimmune diseases and 16% had anti-nuclear antibodies. 79% of patients had low vitamin D-25(OH) levels (<30ng/ml). Orbit MRI showed unilateral optic neuritis in 46.6%. Spinal cord MRIs showed extensive longitudinal myelitis in 52% of patients and the thoracic segment was the most frequently affected (47%). CONCLUSIONS: In the present study of a peruvian NMOSD cohort, we found a higher frequency of unilateral optic neuritis cases, and a higher percentage of AQP4-IgG seropositive patients among those older than 50.

Clinical characteristics and plasma exchange response in Guillain-Barré patients. / Características clínicas y respuesta al recambio plasmático terapéutico en los pacientes con síndrome de Guillain Barré.

The objective of the study was to describe the clinical characteristics, treatment response and possible associated factors of patients with Guillain-Barré syndrome at the National Institute of Neurological Sciences. A descriptive study on hospital discharges was conducted during the period 2017-2019. Treatment response was evaluated based on Hughes' disability scale. From 31 patients 61.3% were males and the mean age was 50 years. At admission, 87.1% of patients were on grade 3 or 4 of Hughes scale, most of them with axonal compromise which was associated to disability. Only 22 patients received plasma exchange; 6 months thereafter, 90.9% of patients decreased by at least one degree in Hughes scale and 42.8% were left without disability. In conclusion, a male and axonal subtype predominance was found, been the latter associated to disability.

El objetivo del estudio fue describir las características clínicas, la respuesta al tratamiento y posibles factores asociados de los pacientes con síndrome de Guillain Barré en el Instituto Nacional de Ciencias Neurológicas. Se realizó un estudio descriptivo sobre egresos hospitalarios durante el periodo 2017-2019. La respuesta al tratamiento se evaluó mediante la escala de discapacidad de Hughes. De los 31 pacientes el 61,3% eran varones, y la edad promedio fue de 50 años. Al ingreso, el 87,1% de pacientes se encontraban en el grado 3 o 4 de la escala de Hughes, la mayoría con compromiso axonal, el cual estuvo asociado a discapacidad. Solo 22 pacientes recibieron recambio plasmático; luego de seis meses el 90,9% disminuyó al menos en un grado en la escala de Hughes y el 42,8% quedaron sin discapacidad. En conclusión, se encontró un predominio del sexo masculino y del compromiso axonal, este último asociado a discapacidad.

Autoimmune neurology: Co-occurrence of anti-NMDAR encephalitis and anti-MOG associated disease, report of a case.

We report the case of a patient with symptoms of anti-NMDAR encephalitis and anti-MOG associated disease simultaneously, in whom the identification of antibodies guided to a more aggressive treatment strategy, resulting in a good clinical outcome. MRI is an important tool to diagnose this kind of patients. The co-occurrence of both diseases in infrequent, but atypical symptoms should increase our awareness of the possibility of an overlap syndrome.

Brainstem syndrome at onset is related to an early MS diagnosis in Peru: A national referral center cohort.

BACKGROUND: MS is unpredictable regarding clinical symptoms; however, certain symptoms represent the preferred localization of white matter lesions such as brainstem, spinal cord; or optic nerve. OBJECTIVES: To investigate the epidemiological, clinical, and imaging characteristics of MS patients in a national referral center in Peru, and to evaluate whether the type of symptom at onset relates with the time to making an MS diagnosis. METHODS: Retrospective study of MS patients at the Instituto Nacional de Ciencias Neurológicas between January 2010 and December 2018. Four different syndromes were selected for analysis as symptom onset (optic neuritis, brainstem syndrome, myelitis, and others). RESULTS: we identified 268 patients for whom a diagnosis of MS had been given; after excluding misdiagnosed patients (33 Neuromyelitis optica), lost or incomplete records, 121 patients were included. The majority of patients (46.6%) were born in Lima. Female to male ratio was 1.37 to 1, mean age at diagnosis was 31 years. At onset, myelitis was present in 35% of RRMS patients, followed by brainstem syndrome (25%) and optic neuritis (18%). Brainstem syndrome was statistically significant predictor for earlier diagnosis (adjusted HR: 2.09; p = 0.015). CONCLUSION: Brainstem syndrome as an initial presentation of MS in Peru is related to an earlier diagnosis.

Neuropsychiatric Symptoms in Patients With Alzheimer's Disease During SARS-COV-2 Pandemic in Peru.

To evaluate neuropsychiatric symptoms in patients with Alzheimer's disease (AD) and their association with cognition and functionality during lockdown of the COVID-19's first wave. We included 91 patients and caregivers of people with AD from a memory clinic. The RUDAS, M@T, and CDR were administered to patients and NPI/ADCS-ADL to caregivers. Baseline and lockdown measurements scales were analyzed to compare the frequencies at baseline versus lockdown and conditional Odds Ratio (ORc) was calculated for the neuropsychiatric symptoms. During the pandemic, significant increase in the number of cases was observed in depression (23%), agitation (36.8%), aberrant motor activity (12%), sleep disorders (26.3%), and appetite change (12.1%). In worsening of pre-existing symptoms, the most frequent were delusions (75%), followed by sleep disorders (71.7%). Lockdown induces a rapid increase of neuropsychiatric symptoms affecting cognitive symptoms and functionality of Peruvian patients with AD.

Knowledge and Attitudes for the Management of Behavioral Variant of Frontotemporal Dementia.

Background: The diagnosis of the behavioral variant of frontotemporal dementia (bvFTD) can be especially challenging and is relatively underdiagnosed. There is scarce information on training and attitudes from care providers facing bvFTD in settings with limited resources. We aim to describe clinical knowledge and attitudes facing bvFTD from neurologists, psychiatrists, and residents in Peru. Methods: Potential participants received invitations by email to complete an online questionnaire. In addition, we reviewed 21 curricula from undergraduate medical schools' programs offered by the main schools of medicine in Peru during 2020 and 2021. Results: A total of 145 participants completed the survey. The responders were neurologists (51%), psychiatrists (25%), and residents in neurology or psychiatry (24%). Only 26% of the respondents acknowledged receiving at least one class on bvFTD in undergraduate medical training, but 66.6% received at least some training during postgraduate study. Participants identified isolated supportive symptoms for bvFTD; however, only 25% identified the possible criteria and 18% the probable bvFTD criteria. They identified MoCA in 44% and Frontal Assessment Battery (39%) as the most frequently used screening test to assess bvFTD patients. Memantine and Acetylcholinesterase inhibitors were incorrectly indicated by 40.8% of participants. Seventy six percentage of participants indicated that they did not provide education and support to the caregiver. The dementia topic was available on 95.2%, but FTD in only 19%. Conclusion: Neuropsychiatry medical specialists in Peru receive limited training in FTD. Their clinical attitudes for treating bvFTD require appropriate training focused on diagnostic criteria, assessment tools, and pharmacological and non-pharmacological management.

Diagnostic Utility of Measuring Cerebral Atrophy in the Behavioral Variant of Frontotemporal Dementia and Association With Clinical Deterioration.

Importance: The presence of atrophy on magnetic resonance imaging can support the diagnosis of the behavioral variant of frontotemporal dementia (bvFTD), but reproducible measurements are lacking. Objective: To assess the diagnostic and prognostic utility of 6 visual atrophy scales (VAS) and the Magnetic Resonance Parkinsonism Index (MRPI). Design, Setting, and Participants: In this diagnostic/prognostic study, data from 235 patients with bvFTD and 225 age- and magnetic resonance imaging-matched control individuals from 3 centers were collected from December 1, 1998, to September 30, 2019. One hundred twenty-one participants with bvFTD had high confidence of frontotemporal lobar degeneration (FTLD) (bvFTD-HC), and 19 had low confidence of FTLD (bvFTD-LC). Blinded clinicians applied 6 previously validated VAS, and the MRPI was calculated with a fully automated approach. Cortical thickness and subcortical volumes were also measured for comparison. Data were analyzed from February 1 to June 30, 2020. Main Outcomes and Measures: The main outcomes of this study were bvFTD-HC or a neuropathological diagnosis of 4-repeat (4R) tauopathy and the clinical deterioration rate (assessed by longitudinal measurements of Clinical Dementia Rating Sum of Boxes). Measures of cerebral atrophy included VAS scores, the bvFTD atrophy score (sum of VAS scores in orbitofrontal, anterior cingulate, anterior temporal, medial temporal lobe, and frontal insula regions), the MRPI, and other computerized quantifications of cortical and subcortical volumes. The areas under the receiver operating characteristic curve (AUROC) were calculated for the differentiation of participants with bvFTD-HC and bvFTD-LC and controls. Linear mixed models were used to evaluate the ability of atrophy measures to estimate longitudinal clinical deterioration. Results: Of the 460 included participants, 296 (64.3%) were men, and the mean (SD) age was 62.6 (11.4) years. The accuracy of the bvFTD atrophy score for the differentiation of bvFTD-HC from controls (AUROC, 0.930; 95% CI, 0.903-0.957) and bvFTD-HC from bvFTD-LC (AUROC, 0.880; 95% CI, 0.787-0.972) was comparable to computerized measures (AUROC, 0.973 [95% CI, 0.954-0.993] and 0.898 [95% CI, 0.834-0.962], respectively). The MRPI was increased in patients with bvFTD and underlying 4R tauopathies compared with other FTLD subtypes (14.1 [2.0] vs 11.2 [2.6] points; P < .001). Higher bvFTD atrophy scores were associated with faster clinical deterioration in bvFTD (1.86-point change in Clinical Dementia Rating Sum of Boxes score per bvFTD atrophy score increase per year; 95% CI, 0.99-2.73; P < .001). Conclusions and Relevance: Based on these study findings, in bvFTD, VAS increased the diagnostic certainty of underlying FTLD, and the MRPI showed potential for the detection of participants with underlying 4R tauopathies. These widely available measures of atrophy can also be useful to estimate longitudinal clinical deterioration.

Dissecting the role of Amerindian genetic ancestry and the ApoE ε4 allele on Alzheimer disease in an admixed Peruvian population.

Alzheimer disease (AD) is the leading cause of dementia in the elderly and occurs in all ethnic and racial groups. The apolipoprotein E (ApoE) ε4 is the most significant genetic risk factor for late-onset AD and shows the strongest effect among East Asian populations followed by non-Hispanic white populations and has a relatively lower effect in African descent populations. Admixture analysis in the African American and Puerto Rican populations showed that the variation in ε4 risk is correlated with the genetic ancestral background local to the ApoE gene. Native American populations are substantially underrepresented in AD genetic studies. The Peruvian population with up to ~80 of Amerindian (AI) ancestry provides a unique opportunity to assess the role of AI ancestry in AD. In this study, we assess the effect of the ApoE ε4 allele on AD in the Peruvian population. A total of 79 AD cases and 128 unrelated cognitive healthy controls from Peruvian population were included in the study. Genome-wide genotyping was performed using the Illumina Global screening array v2.0. Global ancestry and local ancestry analyses were assessed. The effect of the ApoE ε4 allele on AD was tested using a logistic regression model by adjusting for age, gender, and population substructure (first 3 principal components). Results showed that the genetic ancestry surrounding the ApoE gene is predominantly AI (60.6%) and the ε4 allele is significantly associated with increased risk of AD in the Peruvian population (odds ratio = 5.02, confidence interval: 2.3-12.5, p-value = 2e-4). Our results showed that the risk for AD from ApoE ε4 in Peruvians is higher than we have observed in non-Hispanic white populations. Given the high admixture of AI ancestry in the Peruvian population, it suggests that the AI genetic ancestry local to the ApoE gene is contributing to a strong risk for AD in ε4 carriers. Our data also support the findings of an interaction between the genetic risk allele ApoE ε4 and the ancestral backgrounds located around the genomic region of ApoE gene.

Neuromyelitis optica in patients with myasthenia gravis: Two case-reports.

Neuromyelitis optica spectrum disorders (NMOSD) and myasthenia gravis (MG) are disorders that affect the central nervous system and the neuromuscular junction respectively. Although both conditions are rare, reports of the coexistence of these two pathologies are increasing worldwide. Rarely, patients with MG develop aggressive forms of neuromyelitis optica (NMO) after thymectomy. Here, we describe two Peruvian patients with the association of MG and NMO.

[Autoimmune encephalitis mediated by antibodies against the N-Nethyl-N-Aspartate receptor: report of four cases in Peru]. / Encefalitis autoinmune mediada por anticuerpos contra el receptor N-Metil-N-Aspartato: reporte de cuatro casos en Perú.

Autoimmune encephalitis with antibodies against the N-methyl-D-aspartate receptor (anti-NMDAR) is a disorder mediated by antibodies against neural surface antigens, whose early diagnosis and timely treatment improve the prognosis of the disease. Four cases with the definitive diagnosis of autoimmune encephalitis by anti-NMDAR are presented, treated at the National Institute of Neurological Sciences in Lima-Peru. All patients had epileptic seizures and three cases developed a refractory epileptic state. In addition, three patients presented neuropsychiatric alterations, dyskinesias, and dysautonomia. Two cases required ventilatory support. All presented an abnormal electroencephalogram; two cases had pleocytosis in cerebrospinal fluid, and only one showed brain abnormalities on MRI. Regarding treatment, all patients received methylprednisolone immunotherapy and only two of them required plasma exchange for an ineffective response to corticosteroid treatment. After 12 months of hospital discharge, three patients were free of epileptic seizures and only one case did not achieve functional independence. These cases show that anti-NMDAR encephalitis is a treatable condition and its early recognition together with appropriate treatment (immunotherapy/plasmapheresis) are essential for a favorable evolution.

La encefalitis autoinmune por anticuerpos contra el receptor N-metil-D-aspartato (anti-NMDAR) es un desorden mediado por anticuerpos contra antígenos de superficie neuronal, cuyo diagnóstico temprano y tratamiento oportuno mejoran el pronóstico de la enfermedad. Se presentan cuatro casos con el diagnóstico definitivo de encefalitis autoinmune por anti-NMDAR, tratados en el Instituto Nacional de Ciencias Neurológicas en Lima-Perú. Todos los pacientes presentaron crisis epilépticas y tres casos desarrollaron un estado epiléptico refractario. Asimismo, tres pacientes presentaron alteraciones neuropsiquiátricas, discinesias y disautonomías. Dos casos requirieron soporte ventilatorio. Todos presentaron un electroencefalograma anormal, dos casos tuvieron pleocitosis en líquido cefalorraquídeo, y sólo uno mostró anormalidades cerebrales en la resonancia magnética. Respecto al tratamiento, todos los pacientes recibieron inmunoterapia con metilprednisolona y sólo dos de ellos requirieron plasmaféresis por respuesta ineficaz al tratamiento con corticoides. A los 12 meses del alta hospitalaria, tres pacientes quedaron libre de crisis epilépticas y sólo un caso no logró la independencia funcional. Estos casos muestran que la encefalitis anti-NMDAR es una condición tratable y su reconocimiento temprano junto con un tratamiento adecuado (inmunoterapia/plasmaféresis) son esenciales para una evolución favorable.

Primer caso de esclerosis múltiple primaria progresiva tratada con ocrelizumab en el Perú / First case of Primary Progressive Multiple Sclerosis treated with Ocrelizumab in Peru

RESUMEN La esclerosis múltiple es una enfermedad crónica, inflamatoria, desmielinizante, de etiología autoinmune que afecta al sistema nervioso central. Es la causa más común de discapacidad neurológica no traumática en adultos jóvenes. El 10 % de pacientes con esta enfermedad son diagnosticados con la forma esclerosis múltiple primaria progresiva (EMPP) que, hasta la aparición del anticuerpo monoclonal anti-CD20 ocrelizumab, no tenía una terapia específica. Se presenta el primer caso de EMPP tratado con ocrelizumab en el sistema público peruano. El paciente presentó una tolerabilidad aceptable y una respuesta clínica adecuada, medida con la Escala Expandida del Estado de Discapacidad (EDSS). Se destaca que, en la legislación peruana, la esclerosis múltiple es considerada una enfermedad rara que requiere una evaluación ad hoc para la autorización de financiamiento público para terapias específicas.

ABSTRACT Multiple Sclerosis is a chronic, demyelinating, autoimmune, neuroinflammatory disease. Known as the most common cause of non-traumatic neurological disability in young adults. Ten per cent of patients with Multiple Sclerosis are diagnosed with the Primary Progressive form (PPMS) which, until the emergence of the anti-CD20 monoclonal antibody Ocrelizumab, had no specific therapy. The first case treated with Ocrelizumab in the Peruvian public healthcare system is reported. The patient presented an acceptable tolerability and an adequate clinical response, as measured by the EDSS scale. Of note, under Peruvian legislation, Multiple Sclerosis is considered a rare disease and, therefore, requires an ad hoc evaluation for the authorization of public funding for specific therapies.

Factors associated with depression in patients with Parkinson's disease A multicenter study in Lima, Peru.

The diagnosis and treatment of depression in patients with Parkinson's disease (PD) is inadequate, often contributing to a reduced quality of life, rapid disease progression, higher cognitive impairment, and an increased burden of care for family members of patients with PD. OBJECTIVE: To determine the factors associated with depression in PD and to examine the frequency of depressive symptoms among patients with PD. METHODS: This study was an observational, analytical, multicenter study of a cross-sectional cohort, conducted between July 2016 and May 2017. PD patients were recruited from neurology clinics in Lima, Peru. All statistical analyses were performed using descriptive statistics. Bivariate and multivariate logistic regression analyses were calculated using STATA. RESULTS: Out of 124 patients (average age: 68.7 years; 58% males) included in the study 60.5% (75/124) presented with symptoms of depression; only 20% (25/124) received antidepressants. Factors associated with depression in PD included: unemployment, falls, freezing of gait, involuntary movements micrographia, stooped posture, hyposmia, movement disorders in sleep, rapid disease progression, and the use of MAOIs. Furthermore, statistically significant differences were found in disease duration, UPDRS and MMSE scores, Hoehn and Yahr (HY) stage, and length of time taking L-dopa between PD patients with and without depressive symptoms. CONCLUSION: Factors associated with depressive symptoms in patients with PD were hyposmia, rapid progression of the disease, the use of L-dopa, and use of MAOIs. The frequency of depressive symptoms in patients with PD is high; early diagnosis and prompt treatment are needed to improve their quality of life and the family environment.

O diagnóstico e tratamento da depressão em pacientes com doença de Parkinson (DP) é inadequado, frequentemente contribuindo para a redução da qualidade de vida, progressão rápida da doença, maior comprometimento cognitivo e aumento da carga de cuidado aos familiares de pacientes com DP. OBJETIVO: Determinar os fatores associados à depressão na DP e examinar a frequência de sintomas depressivos entre pacientes com DP. MÉTODOS: Este estudo foi um estudo observacional, analítico, multicêntrico, de uma coorte transversal conduzida entre julho de 2016 e maio de 2017. Os pacientes com DP foram recrutados em clínicas de neurologia em Lima, Peru. Todas as análises estatísticas foram realizadas por meio de estatística descritiva. Análises de regressão logística bivariada e multivariada foram calculadas usando STATA. RESULTADOS: Dos 124 pacientes (idade média: 68,7 anos; 58% homens) incluídos no estudo, 60,5% (75/124) apresentaram sintomas de depressão; apenas 20% (25/124) receberam antidepressivos. Os fatores associados à depressão na DP incluíram: desemprego, quedas, congelamento da marcha, movimentos involuntários micrografia, postura inclinada, hiposmia, distúrbios do movimento no sono, progressão rápida da doença e uso de inibidores da MAO. Além disso, houve diferenças estatisticamente significativas encontradas em: duração da doença, escores nas escalas UPDRS e MMSE, estágio Hoehn e Yahr (HY) e tempo de duração da L-dopa entre os pacientes com DP, entre aqueles com e sem sintomas depressivos. CONCLUSÃO: Fatores associados a sintomas depressivos em pacientes com DP foram hiposmia, rápida progressão da doença, uso de L-dopa e uso de IMAOs. A frequência de sintomas depressivos em pacientes com DP é alta; o diagnóstico precoce e o tratamento imediato são necessários para melhorar a qualidade de vida e o ambiente familiar.

Susac syndrome: the first case report in Peru. / Síndrome de Susac: primer reporte de caso en el Perú.

Susac Syndrome is a rare entity, characterized by a triad of subacute encephalopathy, retinal artery occlusion and sensorineural hearing loss. It is more common in women and the age of onset fluctuates between 9-58 years of age. The pathogenesis is presented as microangiopathic changes at the cerebral, retinal and cochlear levels associated with an autoimmune mechanism. We present the case of a 31-year-old woman who started with a diffuse headache, puerile behavior, bradylalia and somnolence. As the disease progressed, she had auditory deficit and arterial obstruction of the right temporal retinal branch in retinal fluorescein angiography. Brain magnetic resonance showed rounded hyperintense lesions in the corpus callosum, periventricular region and cerebellum. This is the first reported case of Susac Syndrome in Peru, presented with the classic triad, which is an infrequent presentation. However, cases that show incomplete forms should be evaluated in a timely manner to initiate timely treatment and avoid irreversible consequences.

El síndrome de Susac es una entidad rara, caracterizada por la triada clásica de encefalopatía subaguda, oclusión de la arteria retiniana e hipoacusia neurosensorial. Es más frecuente en mujeres, la edad de inicio fluctúa entre los nueve y los 58 años de edad. La patogénesis se plantea como un cuadro microangiopático a nivel cerebral, retiniano y coclear asociado a un mecanismo autoinmune. Presentamos el caso de una mujer de 31 años de edad que inició con cefalea holocraneana, conducta pueril, bradilalia y somnolencia. En la angiografía con fluoresceína de retina presentó en la evolución un déficit auditivo y obstrucción arterial de la rama temporal retiniana derecha. La resonancia magnética cerebral mostró lesiones redondeadas hiperintensas en el cuerpo calloso, región periventricular y cerebelo. Se reporta el primer caso de síndrome de Susac definido en Perú, el que se manifestó con la triada clásica, que es de presentación poco frecuente. Sin embargo, también los casos que muestran formas incompletas deben ser evaluados oportunamente para iniciar un tratamiento oportuno y evitar secuelas irreversibles.