Characteristics and management of Susac syndrome in an emergent country: a multi-center case series from Brazil.

BACKGROUND: Susac syndrome (SS) is a rare endotheliopathy with an estimated prevalence of 0.14-0.024 per 100,000. It is an important differential diagnosis in demyelinating disorders. There are few case series and no large randomized controlled trials, and most reports come from developed countries. We report six cases of SS in three centers in Brazil and discuss management challenges in emergent countries. METHODS: This is a retrospective case series of patients diagnosed with SS in three medical centers in Brazil between April 2018 and July 2021. The European Susac consortium (EuSaC) criteria were used for diagnosis of SS. Demographic data and clinical interventions were described and outcomes were assessed subjectively and by applying the modified Rankin Scale (mRS) on last follow-up. RESULTS: Six patients were diagnosed with SS (3 males, 3 females). Mean age at presentation was 36 years (range 17 to 54). The most common initial symptom was confusion, followed by visual impairment and hearing loss. Characteristic snowball lesions on magnetic resonance imaging (MRI) were present in four patients (66%). Retinal artery abnormalities were present in half (3/6) of patients, and sensorineural hearing loss was present in four patients (66%). Outcome was favorable (mRS ≤ 2) in five patients (86%). Patients treated early had a more favorable outcome. CONCLUSION: Emergent countries face challenges in the diagnosis and management of patients with SS, such as access to advanced tests (fluorescein angiography, serial MRI) and treatment drugs (rituximab, mycophenolate). Further research should consider particularities of patients with SS in emergent countries.

Clinical Features and Inflammatory Markers in Autoimmune Encephalitis Associated With Antibodies Against Neuronal Surface in Brazilian Patients.

Acute encephalitis is a debilitating neurological disorder associated with brain inflammation and rapidly progressive encephalopathy. Autoimmune encephalitis (AE) is increasingly recognized as one of the most frequent causes of encephalitis, however signs of inflammation are not always present at the onset which may delay the diagnosis. We retrospectively assessed patients with AE associated with antibodies against neuronal surface diagnosed in reference centers in Northeast of Brazil between 2014 to 2017. CNS inflammatory markers were defined as altered CSF (pleocytosis >5 cells/mm3) and/or any brain parenchymal MRI signal abnormality. Thirteen patients were evaluated, anti-NMDAR was the most common antibody found (10/13, 77%), followed by anti-LGI1 (2/13, 15%), and anti-AMPAR (1/13, 7%). Median time to diagnosis was 4 months (range 2-9 months). Among these 13 patients, 6 (46.1%) had inflammatory markers and when compared to those who did not present signs of inflammation, there were no significant differences regarding the age of onset, time to diagnosis and modified Rankin scale score at the last visit. Most of the patients presented partial or complete response to immunotherapy during follow-up. Our findings suggest that the presence of inflammatory markers may not correlate with clinical presentation or prognosis in patients with AE associated with antibodies against neuronal surface. Neurologists should be aware to recognize clinical features of AE and promptly request antibody testing even without evidence of inflammation in CSF or MRI studies.

A comunicação do diagnóstico "sombrio" na relação médico-paciente entre estudantes de Medicina: uma experiência de dramatização na educação médica / Communicating an unfavorable diagnosis during the physician-patient relationship: a role-playing experiment with medical students

O objetivo do trabalho foi apresentar a dramatização como prática para a humanização da relação médico- paciente entre estudantes do terceiro semestre do curso de Medicina da Universidade Estadual do Ceará (Uece). Por meio de metodologia qualitativa, aplicaram-se perguntas referentes aos sentimentos vivenciados enquanto na posição de médicos e de pacientes, e à vivência com a técnica, após a mesma ser apresentada em sala de aula (n=24).Os dados permitiram identificar que os temas abordados se referiam a doenças graves e perdas. Em relação aos sentimentos, no papel tanto de paciente quanto de médico, demonstraram angústia, impotência, apreensão,medo e ansiedade. Expressaram dificuldades frente ao diagnóstico e à transmissão de má notícia, ao mesmo tempo em que valorizaram a construção do vínculo médico-paciente. Frente à experiência, destacaram a técnica como geradora de auto conhecimento e vivência da futura profissão. Estudos prospectivos devem ser realizados para avaliar o impacto dessa estratégia na humanização da relação médico- paciente e educação médica, explorando o máximo de potencialidade frente às suas limitações.

The aim of this article is to present role-playing as apractice for humanization of the physician-patient relationship, as experienced by third-semester medical students at the State University of Ceará (UECE), Brazil. Using a qualitative methodology, questions were applied relating to the students' feelings while playing the roles of doctor and patient, respectively, and their experience with the technique after its presentation in the classroom (n = 24). According to the findings, the topics tha twere approached related to serious illnesses and losses. Both in the patient's and physician's role, the students felt anguish, powerlessness, apprehension, fear, and anxiety. They expressed difficulties towards both the diagnosis itself and communicating the bad news, even while they valued building and consolidating the physician-patient bond. As for the method, they highlighted its importance for generating self-knowledge and experience for their future profession. Prospective studies should be performed to evaluate the strategy's impact on the humanization of the physician-patient relationship and medical education, fully exploring its potential and limitations.