Long COVID: plasma levels of neurofilament light chain in mild COVID-19 patients with neurocognitive symptoms.

It is well known the potential of severe acute respiratory coronavirus type 2 (SARS-CoV-2) infection to induce post-acute sequelae, a condition called Long COVID. This syndrome includes several symptoms, but the central nervous system (CNS) main one is neurocognitive dysfunction. Recently it has been demonstrated the relevance of plasma levels of neurofilament light chain (pNfL), as a biomarker of early involvement of the CNS in COVID-19. The aim of this study was to investigate the relationship between pNfL in patients with post-acute neurocognitive symptoms and the potential of NfL as a prognostic biomarker in these cases. A group of 63 long COVID patients ranging from 18 to 59 years-old were evaluated, submitted to a neurocognitive battery assessment, and subdivided in different groups, according to results. Plasma samples were collected during the long COVID assessment and used for measurement of pNfL with the Single molecule array (SIMOA) assays. Levels of pNfL were significantly higher in long COVID patients with neurocognitive symptoms when compared to HC (p = 0.0031). Long COVID patients with cognitive impairment and fatigue symptoms presented higher pNfL levels when compared to long COVID patients without these symptoms, individually and combined (p = 0.0263, p = 0.0480, and 0.0142, respectively). Correlation analysis showed that levels of cognitive lost and exacerbation of fatigue in the neurocognitive evaluation had a significative correlation with higher pNfL levels (p = 0.0219 and 0.0255, respectively). Previous reports suggested that pNfL levels are related with higher risk of severity and predict lethality of COVID-19. Our findings demonstrate that SARS-CoV-2 infection seems to have a long-term impact on the brain, even in patients who presented mild acute disease. NfL measurements might be useful to identify CNS involvement in long COVID associated with neurocognitive symptoms and to identify who will need continuous monitoring and treatment support.

Post-COVID-19 olfactory dysfunction: carbamazepine as a treatment option in a series of cases.

Olfactory dysfunction is reported frequently in patients with coronavirus disease 2019. However, an effective treatment for this dysfunction is unknown. The present study evaluated carbamazepine as a treatment option for olfactory dysfunction based on its use in cases of neuralgia, especially of the V cranial nerve. The study included 10 patients with coronavirus disease with olfactory complaints who were part of a cohort of 172 coronavirus disease patients monitored for late neurological manifestations. Carbamazepine was administered for 11 weeks. The adverse effects reported were drowsiness (9/10) and dizziness (2/10); 9 of the 10 patients reported improved olfactory function after carbamazepine treatment. While the role of carbamazepine in the control of post-coronavirus disease olfactory dysfunction could not be confirmed in this study, the satisfactory response observed in most patients in this series suggests that further studies are warranted.

Cognitive Complaints Assessment and Neuropsychiatric Disorders After Mild COVID-19 Infection.

OBJECTIVES: This study aimed to analyze cognitive impairment associated with long-term coronavirus disease 2019 (COVID-19) syndrome and its correlation with anxiety, depression, and fatigue in patients infected with severe acute respiratory syndrome coronavirus. METHODS: This was a cross-sectional study of 127 patients with COVID-19. Tests to screen for neuropsychiatric symptoms included the Fatigue Severity Scale, Mini-Mental State Exam 2 (MMSE-2), Symbol Digit Modalities Test (SDMT), and Hospital Anxiety and Depression Scale. RESULTS: In cognitive tests, SDMT was abnormal in 22%, being more sensitive than MMSE-2 to detect cognitive changes. Furthermore, although manifestations such as fatigue, depression, and anxiety were frequent in the post-COVID-19 phase, these 3 conditions, known to contribute to cognitive impairment, were slightly correlated with worse performance on the rapid screening tests. CONCLUSIONS: In patients with mild COVID-19 and cognitive complaints, SDMT helped to confirm disturbances in the attention domain and processing speed.

HIV-associated painful neuropathy: where are we?

BACKGROUND: After the advent of combination antiretroviral therapy, infection with the human immunodeficiency virus (HIV) ceased to be a devastating disease, but sensory neuropathy resulting from the permanence of the virus and the side effects of treatment have worsened the morbidities of these patients. OBJECTIVE: To investigate the quality of life of 64 HIV-positive patients: 24 with painful neuropathy (case group) and 40 without painful neuropathy (control group). The impact of other factors on quality of life was also assessed. METHODS: To assess painful neuropathy, the Leeds Assessment of Neuropathic Symptoms and Signs (LANSS) scale, Douleur Neuropathique 4 (DN4) questions and Neuropathy Disability Score (NDS) were used. The Short Form Health Survey (SF-36) scale was used to assess quality of life. Factors related or unrelated to HIV were obtained through the medical history and analysis on medical records. RESULTS: The quality of life of patients with neuropathic pain was worse in six of the eight domains of the SF-36 scale. The number of clinical manifestations related to HIV, length of time with detectable viral load since diagnosis, length of time since the diagnosis of HIV infection and length of time of HAART use had a negative impact on quality of life. Higher levels of CD4, education and family income had a positive impact. CONCLUSIONS: Painful neuropathy related to HIV is a factor that worsens the quality of life of patients infected with this virus and should be included in the clinical evaluation.

Doença de Creutzfeltd-Jakob esporádica sem demência: relato de caso / Sporadic Creutzfeldt-Jakob disease without dementia: a case report

Resumo A doença de Creutzfeldt-Jakob é uma condição rara causada por príons. Embora a forma mais notória da doença seja a infecciosa, a forma mais comum é a chamada esporádica, onde ocorre a transformação de proteínas citoplasmáticas das células gliais em príons. Caracteriza-se por uma demência rapidamente progressiva cujo diagnóstico pode ser feito com grande precisão por meio de sinais clínicos, alterações típicas na ressonância magnética de crânio e o exame Real-Time Quaking-Induced Conversion (Rt-QuIC) no líquido cefalorraquidiano. Relatamos um caso da doença sem distúrbios cognitivos, mas com outros sinais clínicos comuns como anormalidades comportamentais, ataxia, reações extrapiramidais e mioclonia; observamos ainda alterações típicas na ressonância magnética do crânio (alterações de sinal afetando áreas dos lobos parietal e temporal) e um Rt-QuIC fortemente positivo. Entendemos que o relato do caso possa servir de alerta para que outros profissionais de saúde possam reconhecer a doença, aumentando as possibilidades de um diagnóstico mais preciso em casos semelhantes.

Abstract Creutzfeldt-Jakob disease is a rare condition caused by prions. Although the infectious form of the disease is the most well-known, the most common form is the so-called sporadic type, where the transformation of cytoplasmic proteins from glial cells into prions occurs. The disease is characterized by rapidly progressive dementia whose diagnosis can be made with great accuracy based on clinical signs, typical changes on magnetic resonance imaging and real-time quaking-induced conversion (Rt-QuIC) testing in cerebrospinal fluid. We report a case of the disease without cognitive disorders in the initial phase, but with other common clinical signs including behavioral abnormalities, ataxia, extrapyramidal features, and myoclonus; typical changes on magnetic resonance imaging of the skull (signal alterations affecting parietal and temporal lobes areas) and strongly positive Rt-QuIC test. This case report can serve to alert other health professionals on recognizing the disease and contribute to a more accurate diagnosis in similar cases.

HIV-associated painful neuropathy: where are we? / Neuropatia dolorosa associada ao HIV: onde estamos?

ABSTRACT Background: After the advent of combination antiretroviral therapy, infection with the human immunodeficiency virus (HIV) ceased to be a devastating disease, but sensory neuropathy resulting from the permanence of the virus and the side effects of treatment have worsened the morbidities of these patients. Objective: To investigate the quality of life of 64 HIV-positive patients: 24 with painful neuropathy (case group) and 40 without painful neuropathy (control group). The impact of other factors on quality of life was also assessed. Methods To assess painful neuropathy, the Leeds Assessment of Neuropathic Symptoms and Signs (LANSS) scale, Douleur Neuropathique 4 (DN4) questions and Neuropathy Disability Score (NDS) were used. The Short Form Health Survey (SF-36) scale was used to assess quality of life. Factors related or unrelated to HIV were obtained through the medical history and analysis on medical records. Results: The quality of life of patients with neuropathic pain was worse in six of the eight domains of the SF-36 scale. The number of clinical manifestations related to HIV, length of time with detectable viral load since diagnosis, length of time since the diagnosis of HIV infection and length of time of HAART use had a negative impact on quality of life. Higher levels of CD4, education and family income had a positive impact. Conclusions: Painful neuropathy related to HIV is a factor that worsens the quality of life of patients infected with this virus and should be included in the clinical evaluation.

RESUMO Antecedentes: Após o advento da terapia antirretroviral combinada a infecção pelo vírus da imunodeficiência humana (HIV) deixou de ser uma doença devastadora, porém a neuropatia sensitiva consequente à permanência do vírus e ao efeito colateral do tratamento piora a morbidade desses pacientes. Objetivo: Investigar a qualidade de vida de 64 pacientes com HIV, 24 com neuropatia dolorosa (grupo caso) e 40 sem neuropatia dolorosa (grupo controle). Avaliou-se também o impacto de outros fatores relacionados e não relacionados ao HIV na qualidade de vida. Métodos: Para avaliação da neuropatia dolorosa foram utilizadas as escalas Leeds Assessment of Neuropathic Symptoms and Signs (LANSS), Douleur Neuropathique 4 (DN4) e Escore de Comprometimento Neuropático (ECN). Para avaliação da qualidade de vida foi utilizada a escala Short Form Health Survey (SF-36). Fatores relacionados e não relacionados ao HIV foram obtidos através da anamnese e análise de prontuário. Resultados: A qualidade de vida dos pacientes com dor neuropática foi pior em 6 dos 8 domínios da escala SF-36. O número de manifestações clínicas relacionadas ao HIV, tempo de carga viral detectável desde o diagnóstico, tempo de diagnóstico da infecção pelo vírus e tempo de uso de TARVC impactaram negativamente na qualidade de vida. Maior nível de CD4, da escolaridade e da renda familiar impactaram positivamente. Conclusões: A neuropatia dolorosa relacionada ao HIV é fator de piora da qualidade de vida dos pacientes infectados por esse vírus devendo ser incluída na avaliação clínica desses pacientes.

Xantomatose cerebrotendínea: uma doença rara e multissistêmica ainda pouco conhecida. Quando devemos suspeitar? / Cerebrotendinous xanthomatosis: a rare and multisystemic disease still little known. When should we suspect?

Xantomatose cerebrotendínea (XCT) é uma doença congênita autossômica recessiva rara multissistêmica do me-tabolismo do ácido biliar que leva ao acúmulo de intermediários do colesterol em diversos tecidos. A principal ma-nifestação da doença é o acometimento neurológico progressivo e irreversível, que inicia na infância e evolui com disfunção neurológica grave na fase adulta. Sintomas não neurológicos característicos como xantomas tendíneos, cataratas de início na infância e diarreia crônica infantil também podem estar presentes. No Brasil, não existe te-rapia medicamentosa para a doença. A principal abordagem terapêutica para retardar a progressão do quadro é o acompanhamento multidisciplinar com o objetivo de melhorar a qualidade de vida. Apesar dos sintomas iniciarem na infância, a maioria dos pacientes demora em média 16 anos para receber o diagnóstico, fase na qual o dano neurológico já é extenso e as abordagens terapêuticas não são mais eficazes. Neste estudo é relatado o caso de paciente de 47 anos com XCT que iniciou os sintomas na infância, com piora neurológica aos 38 anos e diagnóstico aos 44 anos, fase na qual a neurodegeneração já era grave e irreversível. Os testes laboratoriais e Imagem de Res-sonância Magnética indicaram alterações características da doença. Ressalta-se a importância de ter a XTC como diagnóstico diferencial na presença de um quadro neurológico progressivo, amplo e variado, associado com xanto-mas tendíneos e outros sinais e sintomas específicos. Por tratar-se de doença crônica e degenerativa, o diagnóstico precoce é essencial para que se possa instituir medidas que melhorem a qualidade de vida (AU)

Cerebrotendinous xanthomatosis (CTX) is a rare, multisystemic autosomal-recessive disease of biliary acid me-tabolism that leads to accumulation of cholesterol intermediates in multiple tissues. Its primary presentation is progressive and irreversible neurological damage, beginning in childhood and progressing to neurological dys-function in adulthood. There also are characteristic non-neurological symptoms, including tendinous xanthomas, cataracts beginning in childhood, and chronic infantile diarrhea. In Brazil, there is no available treatment for CTX. The primary therapeutic approach to slow disease progression is a palliative one, with multidisciplinary team. While CTX symptoms begin in childhood, most patients are diagnosed at approximately age 16, when neurological damage is extensive and therapeutic approaches are no longer effective. Here, we report a case of a 47-year-old female patient with CTX with symptoms beginning in childhood, with neurological worsening at the age of 38 and diagnosis at 44, at which neurodegeneration was already severe and irreversible. Laboratory tests and magnetic resonance imaging indicated characteristic symptoms. It is important to consider CTX as a differential diagnosis in the presence of a progressive, wide, and varied neurological picture, with tendinous xanthomas and other specific symptoms. Because it is a chronic and degenerative disease, early diagnosis is essential to establish measures to improve the quality of life (AU)

T-Cell Lymphoma in a Patient with Neurofibromatosis Type 1 and AIDS.

Neurofibromatosis type 1 (NF1) and AIDS are risk factors for the development of malignant neoplasms, including hematological malignancies, such as non-Hodgkin lymphoma. NF1 is an autosomal dominant disease that primarily manifests as café-au-lait spots, dermal neurofibromas, axillary and/or inguinal ephelides or freckles, plexiform neurofibromas, Lisch nodules, and bone deformities. In this report, we present a 38-year-old female patient with NF1 from childhood and AIDS who developed peripheral T-cell lymphoma with good response to chemotherapeutic treatment.

Neurological implications of SARS-CoV-2 infection: review of literature / Implicações neurológicas da infecção por SARS-CoV-2: revisão da literatura

The infection caused by the new coronavirus had its first case described in December 2019, in Wuhan, China, and reached a pandemic status in March 2020. Since then, knowledge about the different aspects of this infection has evolved, as well as increased reports concerning related neurological manifestations. Thus, the neurologist assumes a fundamental role in the care of these patients, who may have a clinical phenotype that goes beyond respiratory aspects. In the present study, we highlight the data available in the literature so far regarding the main neurological implications related to COVID-19 infection, in addition to calling attention for some aspects related to patients with previous neurological diseases who contract this infection.

A infecção causada pelo novo Coronavírus teve seu primeiro caso descrito em dezembro de 2019, em Wuhan, China e alcançou o status de pandemia em março de 2020. Desde então, o conhecimento sobre os diferentes aspectos da referida infecção evolui assim como aumentam relatos de manifestações neurológicas relacionadas. Assim, o neurologista assume papel fundamental na assistência desses pacientes, que podem ter um fenótipo clínico que ultrapassa os aspectos respiratórios. No presente estudo, destacamos os dados disponíveis na literatura até o presente momento no tocante às principais implicações neurológicas relacionadas à infecção pelo COVID-19, além de destacar alguns aspectos relativos aos pacientes com doenças neurológicas prévias que contraem a referida infecção.

Palm to Finger Ulnar Sensory Nerve Conduction.

Ulnar neuropathy at the wrist (UNW) is rare, and always challenging to localize. To increase the sensitivity and specificity of the diagnosis of UNW many authors advocate the stimulation of the ulnar nerve (UN) in the segment of the wrist and palm. The focus of this paper is to present a modified and simplified technique of sensory nerve conduction (SNC) of the UN in the wrist and palm segments and demonstrate the validity of this technique in the study of five cases of type III UNW. The SNC of UN was performed antidromically with fifth finger ring recording electrodes. The UN was stimulated 14 cm proximal to the active electrode (the standard way) and 7 cm proximal to the active electrode. The normal data from amplitude and conduction velocity (CV) ratios between the palm to finger and wrist to finger segments were obtained. Normal amplitude ratio was 1.4 to 0.76. Normal CV ratio was 0.8 to 1.23.We found evidences of abnormal SNAP amplitude ratio or substantial slowing of UN sensory fibers across the wrist in 5 of the 5 patients with electrophysiological-definite type III UNW.

Polineuropatia desmielinizante como manifestação inicial isolada de hanseníase: relato de caso / Demyelinating polyneuropathy as an isolated initial manifestation of leprosy: case report

RESUMO: Modelo do Estudo: Relato de caso. Importância do problema: No mundo, mais de três milhões de pessoas estão vivendo com deficiência física devido à hanseníase. O Brasil é o segundo país com o maior número de casos novos registrados.A magnitude e o alto risco de incapacidade mantêm a doença como problema de saúde pública. O diagnóstico de hanseníase em geral é simples. Porém, quadros com ausência de lesões cutâneas características, somente com alterações neurais, representam um desafio para o diagnóstico diferencial com outras doenças neurológicas. Comentários: Relatamos o caso de um paciente encaminhado ao serviço de neurologia com história clínica e eletroneuromiografia compatíveis com polineuropatia desmielinizante, sem qualquer lesão cutânea ao exame de admissão. O raciocínio clínico inicial foi direcionado para o diagnóstico das polineuropatias desmielinizantes inflamatórias adquiridas como Polineuropatia Desmielinizante Inflamatória Crônica (CIDP) e suas variantes. No entanto, após anamnese e exame físico detalhados, chamou a atenção a ausência do componente atáxico e a presença predominante de alterações sensitivas de fibra fina, espessamento de nervo e importante fator epidemiológico para hanseníase, motivando a suspeita e a in-vestigação desta enfermidade por meio da biópsia de nervo que foi sugestiva de hanseníase. Após três meses, em novo exame do paciente para biopsiar áreas de anestesia para reforçar o diagnóstico, observou-se o surgimento de extensas lesões levemente hipocrômicas no tronco e membros inferiores, cuja biópsia definiu o diagnóstico de hanseníase. (AU)

ABSTRACT: Study: Case report. Importance: Worldwide over three million people are living with disabilities due to leprosy. Brazil is the second country with the highest number of new cases registered. The magnitude and high risk of disability make the disease a public health problem. The diagnosis of leprosy can be simple. However, in the absence of skin lesions and with many possibilities of neurological impairment, diagnosis can become a challenge. Comments: We report the case of a patient referred to the neurology service with a clinical history and electrophysiological tests compatible with demyelinating polyneuropathy, without any skin lesion at admission examination. The initial clinical research was directed to the diagnosis of acquired inflammatory demyelinating polyneuropathies such as Chronic Inflammatory Demyelinating Polyneuropathy (CIDP)and its variants. However, after anamnesis and detailed physical examination, the absence of the ataxic component and the predominant presence of sensory alterations of fine fiber, nerve thickening and important epidemiological factor for leprosy,led to the suspicion and investigation of this disease by nerve biopsy that was suggestive of leprosy. After three months, in a new patient examination "to perform a biopsy in areas of anesthesia" to reinforce the diagnosis, there was the appearance of extensive slightly hypochromic lesions in the trunk and lower limbs, whose biopsy defined the diagnosis of leprosy.(AU)

Pyoderma Gangrenosum Simulating Necrotizing Fasciitis.

Pyoderma gangrenosum received this name due to the notion that this disease was related to infections caused by bacteria in the genus Streptococcus. In contrast to this initial assumption, today the disease is thought to have an autoimmune origin. Necrotizing fasciitis was first mentioned around the fifth century AD, being referred to as a complication of erysipelas. It is a disease characterized by severe, rapidly progressing soft tissue infection, which causes necrosis of the subcutaneous tissue and the fascia. On the third day of hospitalization after antecubital venipuncture, a 59-year-old woman presented an erythematous and painful pustular lesion that quickly evolved into extensive ulceration circumvented by an erythematous halo and accompanied by toxemia. One of the proposed etiologies was necrotizing fasciitis. The microbiological results were all negative, while the histopathological analysis showed epidermal necrosis and inflammatory infiltrate composed predominantly of dermal neutrophils. Pyoderma gangrenosum was considered as a diagnosis. After 30 days, the patient was discharged with oral prednisone (60 mg/day), and the patient had complete healing of the initial injury in less than two months. This case was an unexpected event in the course of the hospitalization which was diagnosed as pyoderma gangrenosum associated with myelodysplastic syndrome.

RRH: envenoming syndrome due to 200 stings from Africanized honeybees.

Envenoming syndrome from Africanized bee stings is a toxic syndrome caused by the inoculation of large amounts of venom from multiple bee stings, generally more than five hundred. The incidence of severe toxicity from Africanized bee stings is rare but deadly. This report reveals that because of the small volume of distribution, having fewer stings does not exempt a patient from experiencing an unfavorable outcome, particularly in children, elderly people or underweight people.

RRH: envenoming syndrome due to 200 stings from Africanized honeybees / RRH: síndrome de envenenamento por 200 ferroadas de abelhas africanizadas

Envenoming syndrome from Africanized bee stings is a toxic syndrome caused by the inoculation of large amounts of venom from multiple bee stings, generally more than five hundred. The incidence of severe toxicity from Africanized bee stings is rare but deadly. This report reveals that because of the small volume of distribution, having fewer stings does not exempt a patient from experiencing an unfavorable outcome, particularly in children, elderly people or underweight people.

A síndrome de envenenamento por ferroadas de abelhas africanizadas é causada pela inoculação de uma grande quantidade de peçonha por múltiplas ferroadas de abelhas, geralmente acima de quinhentas. A incidência de uma intoxicação severa por ferroadas de abelhas africanizadas é rara, porém letal. Este relato de caso aponta que, devido a um menor volume de distribuição do veneno, um número menor de ferroadas por abelhas africanizadas não exime o paciente de apresentar envenenamentos com desfecho desfavorável, principalmente em crianças, idosos e pessoas com baixo peso.