Autoimmune encephalitis in a resource-limited public health setting: a case series analysis / Encefalite autoimune em um hospital universitário público: análise de uma série de casos

Abstract Background Autoimmune encephalitis (AE) consists of a group of acquired diseases that affect the central nervous system. A myriad of phenotypes may be present at the onset. Due to the heterogeneity of clinical presentations, it is difficult to achieve uniformity for the diagnostic and therapeutic processes and follow-up strategies. Objective To describe a series of patients diagnosed with AE in a resource-limited public hospital in southern Brazil and to analyze therapeutics and outcomes. Methods We retrospectively reviewed the electronic medical records of patients diagnosed with AE at the Hospital de Clínicas de Porto Alegre from 2014 to 2022. Data collected included clinical presentation, neuroimaging, cerebrospinal fluid testings, electroencephalogram, autoantibodies, treatments, outcomes, follow-up time, degree of neurological impairment, and mortality. Results Data from 17 patients were retrieved. Eleven cases were classified as definite AE and 6 as possible AE. Autoantibodies were identified in 9 patients. Timing for diagnosis was impacted by the high costs associated with autoantibody testing. Most patients became functionally dependent (82.4%) and most survivors remained with autoimmune-associated epilepsy (75%). Five patients died during hospitalization, and one after a 26-month of follow-up. Conclusion In this resource-limited hospital, patients with AE had a worse clinical outcome than that previously described in the literature. Development of epilepsy during follow-up and mortality were greater, whilst functional outcome was inferior. Autoantibody testing was initially denied in most patients, which impacted the definitive diagnosis and the use of second-line therapies.

Resumo Antecedentes A encefalite autoimune (EA) consiste em um grupo de doenças adquiridas que afetam o sistema nervoso central. Objetivo Descrever uma série de pacientes diagnosticados com EA em um contexto de atenção terciária à saúde com recursos limitados e analisar a terapêutica e os resultados. Métodos Revisamos retrospectivamente os prontuários eletrônicos de pacientes diagnosticados com EA no Hospital de Clínicas de Porto Alegre de 2014 a 2022. Os dados coletados incluíram apresentação clínica, neuroimagem, exames de líquido cefalorraquidiano, eletroencefalograma, autoanticorpos, tratamentos, resultados, tempo de acompanhamento, grau de comprometimento neurológico e mortalidade. Resultados Dados de 17 pacientes foram coletados. Onze casos foram classificados como EA definitivo e seis como EA possível. Autoanticorpos foram identificados em nove pacientes. O tempo para o diagnóstico foi afetado pelos altos custos associados ao teste de autoanticorpos. A maioria dos pacientes tornou-se funcionalmente dependente (82,4%), e a maioria dos sobreviventes permaneceu com epilepsia autoimune associada (75%). Cinco pacientes faleceram durante a internação, e um após 26 meses de seguimento. Conclusão No hospital em questão, os pacientes com EA tiveram um desfecho clínico pior do que o previamente descrito na literatura. O desenvolvimento de epilepsia durante o acompanhamento e a mortalidade foram maiores, enquanto o desfecho funcional foi inferior. Os testes de autoanticorpos foram inicialmente negados para a maioria dos pacientes, o que impactou o diagnóstico definitivo e o uso de terapias de segunda linha.

POEMS syndrome: A rare entity

POEMS syndrome is a rare paraneoplastic syndrome due to an underlying plasma cell disorder. The diagnosis of POEMS syndrome can be a challenge. A good history, physical examination, and appropriate testing can aid in establishing its diagnosis. We are presenting the case of a 75-year-old man who was diagnosed with POEMS syndrome.

Linfoma de Hodgkin: neuronopatía sensitiva y autonómica como manifestación paraneoplásica / Hodgkin's lymphoma: sensitive and autonomic neuropa thy as a paraneoplastic manifestation

Resumen El linfoma de Hodgkin (LH) comprende un grupo heterogéneo de neoplasias linfoides cuyo origen radica en linfocitos B. Las manifestaciones neurológicas de dicha enfermedad son infrecuentes, pudiendo tener su origen por invasión directa de las células neoplásicas en el sistema nervioso, o indirectamente, a través de síndromes paraneoplásicos o como complicación del tratamiento. Dentro de los síndromes neurológicos paraneoplásicos que afectan a pacientes con LH, la degeneración cerebelosa paraneoplásica es la más frecuente. Otros reportados con menor frecuencia en series de casos o casos aislados incluyen encefalitis límbica, neuronopatía sensitiva, motora y autonómica. Estos pueden ser la manifestación inicial de la enfer medad neoplásica, y la falta de conocimiento de dicha asociación puede retrasar el diagnóstico, con inicio tardío del tratamiento y peor pronóstico. Reportamos el caso de una mujer con LH que presentó al inicio de su enfermedad neuronopatía sensitiva y autonómica como manifestaciones neurológicas paraneoplásicas. Una vez iniciado el tratamiento específico para su lin foma, la neuronopatía autonómica tuvo resolución casi completa a diferencia de la neuronopatía sensitiva, la cual demostró escasa recuperación.

Abstract Hodgkin lymphoma (HL) comprises a heterogeneous group of lymphoid neoplasms whose origin lies in B lym phocytes. The neurological manifestations of this pathol ogy are infrequent, and may arise from direct invasion of neoplastic cells to the nervous system, or indirectly, through paraneoplastic syndromes or as a complication of treatment. Among the neurological paraneoplastic syndromes that affect patients with HL, paraneoplastic cerebellar degeneration is the most common. Other few cases include limbic encephalitis, sensory, motor, and autonomic neuronopathy. These syndromes can be the initial manifestation of neoplastic disease, and the lack of information regarding this association can lead to a delay in diagnosis and consequently in the initiation of therapy worsening the prognosis. We report the case of a woman with HL who presented sensory and autonomic neuronopathy at the onset of her disease as paraneo plastic neurological manifestations. After the initiation of the specific treatment for the lymphoma, the autonomic neuronopathy had almost complete resolution, unlike the sensory neuronopathy, which showed limited recovery.

A slow-release dexamethasone implant for cancer-associated retinopathy / Implante de liberacao lenta de dexametasona para retinopatia associada ao cancer

ABSTRACT This case report aims to show the anatomical and functional results of a patient diagnosed as having cancer-associated retinopathy treated with a controlled-release dexamethasone implant (Ozurdex). Anatomical outcomes were assessed using spectral domain optical coherence tomography; and functional outcomes, by measuring visual acuity, microperimetry, and mutifocal electroretinography. The follow-up period was 1 year.

RESUMO Este relato de caso tem como objetivo mostrar os resultados anatômicos e funcionais de um paciente com diagnóstico de retinopatia associada ao câncer tratado com implante de liberação controlada de dexametasona (Ozurdex®). Os resultados anatômicos foram avaliados por SD-OCT e os resultados funcionais por medida de acuidade visual, microperimetria e eletrorretinograma multifocal. O período de acompanhamento foi de um ano.

A case of nasopharyngeal carcinoma with autonomic nervous dysfunction and literature review / 中国医师杂志

Objective:A case of advanced nasopharyngeal carcinoma with autonomic dysfunction was reported and its pathophysiological mechanism was discussed.Methods:The diagnosis and treatment of a nasopharyngeal carcinoma patient with autonomic nervous dysfunction such as paroxysmal syncope was summarized, and the pathophysiological mechanism of this case was analyzed by searching related literature.Results:Nasopharyngeal carcinoma characterized by autonomic dysfunction was rare and had a poor prognosis. Autonomic dysfunction caused by nasopharyngeal carcinoma was associated with carotid sinus syndrome, parapharyngeal space syncope syndrome, glossopharyngeal nerve reflex and paraneoplastic neuropathy.Conclusions:Early detection and treatment is a key factor affecting the prognosis of nasopharyngeal carcinoma. Clinicians should consider nasopharyngeal carcinoma as one of the differential diagnoses in the diagnosis and treatment of patients with autonomic nervous dysfunction combined with cranial nerve damage.

A retrospective clinical analysis of 17 cases of renal cell carcinoma with Stauffer syndrome / 现代泌尿外科杂志

【Objective】 To explore the diagnosis, treatment and prognosis of renal cell carcinoma (RCC) patients with Stauffer syndrome. 【Methods】 The clinicopathological and follow-up data of 17 RCC patients with Stauffer syndrome who underwent operation during Sep.2014 and Aug.2019 were retrospectively analyzed. The survival was analyzed with Kaplan-Meier curve and log-rank, and related factors affecting the prognosis were determined with univariate and multivariate Cox regression model. 【Results】 The pathological results included clear cell RCC in 14 cases, papillary RCC in 2 cases, and poorly differentiated tissue in 1 case. The liver function recovered within 3 months after operation in 5 cases, within 6 months in 3 cases, within 1 year in 4 cases, and did not recover in 3 cases. During the follow up of 6 to 72 (average 54.1) months, the 1-, 3-, and 5-year survival rates were 88.2% (15/17), 76.5% (13/17) and 52.9% (9/17), respectively. Survival analysis showed that the cancer-specific survival (CSS) of RCC patients with Stauffer syndrome was low, and tumor size, AJCC stage, lymph node metastasis and Stauffer syndrome were predictors of poor prognosis. 【Conclusion】 The prognosis of RCC patients with Stauffer syndrome is poor, and early surgical intervention should be conducted. The liver function of most patients can return to normal gradually after surgery. Continuous examination of liver function has significant meaning for tumor recurrence, metastasis and prognosis.

A Case of Paraneoplastic Pemphigus with Malignant Thymoma and Obliterated Bronchiolitis / 罕见病研究

Paraneoplastic pemphigus (PNP) is a rare and life-threatening autoimmune blistering disease characterized by severe mucosal erosions and polymorphous cutaneous eruptions associated with lymphoproliferative neoplasmas. Castleman's disease, non-Hodgkin's lymphoma, thymoma, follicular dendritic cell sarcoma and chronic lymphocytic leukemia are the commonly associated neoplasmas in PNP, whereas malignant thymoma is rare. The prognosis of PNP is poor due to severe infections during immunosuppressive treatment, underlying malignancies and bronchiolitis obliterans mediated by autoimmunity. A 58-year-old woman who experienced recurrent oral erosive ulcer for 3 years, systemic erythema for 10 months, and dyspnea for 3 months was diagnosed with PNP with malignant thymoma and obliterated bronchiolitis by clinical, radiological, histopathological, and immunological examinations. She died of respiratory failure 3 weeks after resection of the malignant thymoma.

Melanoma uveal

Introducción: El melanoma uveal, es el cáncer intraocular más común en adultos, representa cerca del 3 % al 5 % de todos los melanomas; con un pico de incidencia en hombres en la sexta década de la vida. Objetivo: Describir la presentación clínica, diagnóstico y tratamiento de una paciente con melanoma uveal. Caso clínico: Paciente femenina de 39 años de edad, con antecedentes de salud, quien acudió a consulta por presentar dolor intenso de doce días de evolución localizado en el ojo derecho, asociado a pérdida de la visión, dolor a los movimientos oculares, astenia y anorexia. Al examen físico se encontró edema palpebral severo, abundantes secreciones mucopurulentas, quémosis conjuntival en 360 grados marcado e hiperemia cilio-conjuntival en el ojo derecho. Se realizaron estudios analíticos e imagenológicos. Al analizar el resultado de la exploración física y los complementarios se decidió la intervención quirúrgica consistente en la exenteración orbitaria derecha, esfenoidectomía y etmoidectomía transorbitaria derecha. Con una evolución satisfactoria fue egresada del centro hospitalario. La biopsia informó un melanoma coroideo. Conclusiones: El melanoma coroideo es una enfermedad ocular poco frecuente en mujeres jóvenes de piel negra. La sintomatología es extremadamente variable con las masas coroideas. Es importante conocer sus características, pues estos efectos clínicos sirven como un recordatorio para los oftalmólogos para incluirla en el diagnóstico diferencial de otras patologías oculares.

Introduction: Uveal melanoma is the most common intraocular cancer in adults, represents approximately 3 % to 5 % of all melanomas; with a peak incidence in men in the sixth decade of life. Objective : To describe the clinical presentation, diagnosis and treatment of a patient with uveal melanoma. Clinical case: A 39-year-old female patient, with a health history, who came to the consultation due to intense pain of twelve days of evolution located in the right eye, associated with loss of vision, pain with eye movements, asthenia and anorexy. Physical examination revealed severe palpebral edema, abundant mucopurulent secretions, marked 360-degree conjunctival chemosis, and cilio-conjunctival hyperemia in the right eye. Analytical and imaging studies were performed. When analyzing the results of the physical and complementary examination, the surgical intervention consisting of right orbital exenteration, sphenoidectomy and right transorbital ethmoidectomy was decided. With a satisfactory evolution, she was discharged from the hospital. Biopsy reported choroidal melanoma. Conclusions: Choroidal melanoma is a rare ocular entity in young black women. Symptomatology is extremely variable with choroidal masses. It is important to know its characteristics, since these clinical effects serve as a reminder for ophthalmologists to include it in the differential diagnosis of other ocular pathologies.

Vasculitis cutánea paraneoplásica asociada a linfomadifuso de célulasB grandes / Paraneoplastic cutaneous vasculitis associated with diffuse large B-cell non-Hodgkin's lymphoma

Resumen Los fenómenos cutáneosparaneoplásicos son heterogéneos, infrecuentes y adquiridos, caracterizados por la presencia subyacente de una neoplasia. La manifestacióncutánea más frecuentemente descripta de la vasculitis paraneoplásica es la púrpura palpable. También puede manifestarse como urticaria, eritema y úlceras de miembros inferiores. Se presenta el caso de un paciente de 38 años con diagnóstico de linfoma difuso de células B grandes y úlceras atípicas de aparición concomitantepor vasculitis leucocitoclástica como fenómeno paraneoplásico. Estas lesiones suelentener un curso paralelo a la neoplasia por lo que al realizar tratamiento de la misma pueden remitir, no así cuando se realiza únicamente tratamiento para las lesiones cutáneas. Remarcamos la importancia de conservar un lecho vital y en estado de granulaciónque acompañeal tratamiento general, para así favorecer una rápida epitelización y prevención de infecciones intercurrentes.

Abstract Paraneoplastic cutaneous phenomena are heterogeneous, infrequent and acquired, characterized by the underlying presence of a neoplasm. The most frequently described cutaneous manifestation of paraneoplastic vasculitis is palpable purpura. It can also manifest as urticaria, erythema and ulcers of the lower limbs. We present the case of a 38-year-old patient with a diagnosis of diffuse large B-cell lymphoma and atypical ulcers of intercurrent onset due to leukocytoclastic vasculitis as a paraneoplastic phenomenon. These lesions usually have a parallel course to the neoplasm, so when the neoplasm is treated they may remit, but not when only the cutaneous lesions are treated. We emphasize the importance of preserving a vital tissue in a state of granulation that accompanies the general treatment, in order to favor a rapid epithelialization and prevention of intercurrent infections.

Acute Myeloid Leukemia in a Geriatric Patient with a History of Minimal Change Disease

Background: The increase in the incidence of malignancies globally, and the increase in the usage frequency and types of new anti-cancer drugs, have made onconephrology more important in our clinical practice. Paraneoplastic glomerulonephritis constitutes an important part of this approach as well. Purpose: The association of AML-nephrotic syndrome is relatively less defined in the literature compared to other hematological malignancies. Case presentation: In this article, we present a case of acute myelocytic leukemia in a patient who was diagnosed with minimal change disease many years ago. Discussion and Conclusion: Hematological malignancies-MCD association, is one of the best described examples of paraneoplastic glomerulonephritis. We know that cancer can be clinically diagnosed years after the detection of renal disease in paraneoplastic glomerulonephritis. In this case; rationality of follow-up, not only during the diagnosis of glomerulonephritis but also periodically in the long term, especially in clinical situations such as MCD that occur in geriatric patients, should be discussed.

Introducción: El aumento en la incidencia de neoplasias malignas a nivel mundial, y el aumento en la frecuencia de uso y tipos de nuevos medicamentos contra el cáncer, han hecho que la onconefrología sea más importante en nuestra práctica clínica. Asimismo, la glomerulonefritis paraneoplásica también constituye una parte importante de este enfoque. Propósito: La asociación de LMA-síndrome nefrótico está relativamente menos definida en la literatura a comparación de otras neoplasias malignas hematológicas. Presentación del caso: En este artículo presentamos un caso de leucemia mielocítica aguda en un paciente al que se le diagnosticó enfermedad de cambios mínimos hace años. Discusión y Conclusión: La asociación de neoplasias hematológicas malignas-MCD, es uno de los ejemplos mejor descritos de glomerulonefritis paraneoplásica. Sabemos que el cáncer puede diagnosticarse clínicamente años después de la detección de la enfermedad renal en la glomerulonefritis paraneoplásica. En este caso, debe discutirse la racionalidad del seguimiento, no solo durante el diagnóstico de glomerulonefritis, sino también periódicamente a largo plazo especialmente en situaciones clínicas como la ECM que se presenta en pacientes geriátricos.

Mielopatias inflamatórias / Inflammatory myelopathies / Mielopatías inflamatorias

Mielopatia inflamatória ou mielite transversa é uma síndrome neurológica potencialmente incapacitante com uma variedade de etiologias. Episódios únicos ou recorrentes podem resultar em dependência de cadeira de rodas. O quadro clínico de fraqueza, alteração de sensibilidade e disfunção autonômica de início agudo ou subagudo é marca dessa síndrome. Esse cenário é comum às diferentes etiologias, que podem ser de natureza desmielinizante, por doença autoimune sistêmica, paraneoplásica ou infecciosa. A ressonância magnética de coluna é o exame de neuroimagem de escolha. Exames complementares como avaliação do líquido cefalorraquidiano, testes sorológicos e pesquisa de anticorpos dão suporte à investigação. A depender da etiologia, há tratamentos específicos a fim de reduzir incapacidade e chance de novos surtos, além de diferentes prognósticos. Este trabalho objetiva uma revisão de literatura sobre mielopatias inflamatórias e suas principais etiologias, a partir de dados obtidos na plataforma eletrônica PubMed. Para a discussão, foram revisadas as etiologias desmielinizantes (encefalomielite disseminada aguda, esclerose múltipla, doença do espectro, neuromielite óptica e neurite óptica, encefalite e mielite associadas ao MOG-IgG); doenças autoimunes (lúpus eritematoso sistêmico e síndrome de Sjögren); síndromes paraneoplásicas e mielopatias infecciosas (neuroesquistossomose, mielite por HIV e por HTLV-1 e neurossífilis). Concluiu-se com este estudo que a mielopatia inflamatória é uma condição de gravidade variável que produz potencial incapacidade, causada por diferentes etiologias, porém com quadro clínico comum entre elas. Por isso, é importante conhecer cada uma dessas causas, a fim de promover o melhor e mais precoce tratamento e reduzir sequelas.

Inflammatory myelopathy or transverse myelitis is a potentially disabling neurological syndrome with various etiologies. Single or recurrent episodes can result in wheelchair dependence. A clinical picture of weakness, altered sensitivity, and autonomic dysfunction with acute or subacute onset is characteristic of this syndrome. This scenario is common to different etiologies, which can be of a demyelinating nature, due to systemic, paraneoplastic, or infectious autoimmune disease. Spine MRI is the neuroimaging test of choice. Complementary tests such as cerebrospinal fluid evaluation, serological tests and antibody research support the investigation. Depending on the etiology, there are specific treatments to reduce disability and the chance of new episodes, and different prognoses. This study is a literature review on inflammatory myelopathies and their main etiologies, based on data obtained from the PubMed database. Demyelinating etiologies (acute disseminated encephalomyelitis, multiple sclerosis, neuromyelitis optic spectrum disease and optic neuritis, MOG-IgG-associated encephalitis and myelitis), autoimmune diseases (systemic lupus erythematosus and Sjögren's syndrome), paraneoplastic syndromes and infectious myelopathies (neuroschistosomiasis, HIV and HTLV-1 myelitis, and neurosyphilis) were reviewed for discussion. In conclusion, inflammatory myelopathy is a condition of variable severity that produces potential disability, caused by different etiologies, but with a common clinical picture between them. Thus, knowledge on each of these causes is important to promote the best and earliest treatment and reduce sequelae.

La mielopatía inflamatoria o mielitis transversa es un síndrome neurológico potencialmente incapacitante con una variedad de etiologías. Los episodios únicos o recurrentes pueden tener como consecuencia dependencia de silla de ruedas. El cuadro clínico de debilidad, sensibilidad alterada y disfunción autonómica de inicio agudo o subagudo es distintivo de este síndrome. Esto es común a diferentes etiologías, que pueden ser de naturaleza desmielinizante, debido a enfermedades autoinmunes sistémicas, paraneoplásicas o infecciosas. La resonancia magnética de columna es la prueba de neuroimagen de elección. Las pruebas complementarias, como la evaluación del líquido cefalorraquídeo, las pruebas serológicas y la investigación de anticuerpos respaldan la investigación. Dependiendo de la etiología, existen tratamientos específicos para reducir la discapacidad y la posibilidad de nuevos brotes, además de diferentes pronósticos. Este trabajo tiene como objetivo revisar la literatura sobre mielopatías inflamatorias y sus principales etiologías desde los datos obtenidos de la base de datos electrónica PubMed. Se revisaron las etiologías desmielinizantes (encefalomielitis aguda diseminada, esclerosis múltiple, enfermedad del espectro, neuromielitis óptico y neuritis óptica, encefalitis y mielitis asociadas a MOG-IgG), las enfermedades autoinmunes (lupus eritematoso sistémico y síndrome de Sjögren), los síndromes paraneoplásicos y mielopatías infecciosas (neurosquistosomiasis, mielitis por VIH y HTLV-1 y neurosífilis). Se concluyó que la mielopatía inflamatoria es una condición de severidad variable, que produce potencial discapacidad causada por diferentes etiologías, pero tiene un cuadro clínico común entre ellas. Por ello, es importante conocer cada una de las causas para promover el mejor y más precoz tratamiento, además de reducir las secuelas.

Síndrome POEMS: desafios no diagnóstico de um caso atípico / POEMS syndrome: challenges in the diagnosis of an atypical case

Introdução: Síndrome POEMS trata de um raro evento paraneoplásico, sem relato atual na literatura sobre sua real prevalência. A maior parte dos casos ocorre em homens de meia idade. Relato do Caso:Paciente masculino, 65 anos, com queixa edema e parestesia em pernas que evoluiu para plegia, associada a hiporexia e fadiga. Investigação ambulatorial inicial evidenciou Polirradiculoneuropatia Inflamatória Desmielinizante Crônica (PIDC) de etiologia indefinida. Excluídos secundarismos, o paciente foi diagnosticado com hipotireoidismo primário, hipogonadismo severo, lesões hipercrômicas em pele, ascite, derrame pleural e trombocitose, além de gamopatia monoclonal IgA Lambda por imunofixação sérica. Sorologias virais negativas. Excluída a possibilidade de Mieloma Múltiplo e outras gamopatias, foi aventada a hipótese de Síndrome POEMS, sendo realizada dosagem de VEGF plasmática (425 pg/mL; VR = <96.2). Trata-se de um caso atípico na medida em que, lesões ósseas, presentes em até 97% dos casos, não foram evidenciadas no paciente em questão, tornando desafiador o diagnóstico. Conclusões: O diagnóstico de síndromes raras, embora desafiante, traz ao clínico um olhar mais amplo do paciente na medida em que incrementa o raciocínio clínico. [au]

Introduction: POEMS syndrome is a rare paraneoplastic event, with no current report in the literature about its real prevalence. Most cases occur in middle-aged men. Case Report: Male patient, 65 years old, complaining of edema and paresthesia in the legs that evolved to plegia, associated with hyporexia and fatigue. Initial outpatient investigation revealed Chronic Inflammatory Demyelinating Polyradiculoneuropathy (CIDP) of undefined etiology. Excluding secondaries, the patient was diagnosed with primary hypothyroidism, severe hypogonadism, hyperchromic skin lesions, ascites, pleural effusion and thrombocytosis, in addition to monoclonal IgA Lambda gammopathy by serum immunofixation. Viral serologies was negative. Excluding the possibility of Multiple Myeloma and other gammopathies, the hypothesis of POEMS Syndrome was raised, and plasma VEGF measurement was performed (425 pg/mL; RV = <96.2). This is an atypical case in that bone lesions, present in up to 97% of cases, were not evidenced in the patient in question, making the diagnosis challenging. Conclusions: The diagnosis of rare syndromes, although challenging, brings the clinician a broader view of the patient as it increases clinical reasoning. [au]

Síndrome de hipersecreción de lipasa y lesiones óseas en el cáncer de células acinares del páncreas / Lipase hypersecretion syndrome and bone lesions in acinar cell cancer of the pancreas. Report of one case

Lipase hypersecretion syndrome (LHS) is a rare paraneoplastic syndrome, associated with acinar cell carcinoma of the pancreas (ACCP) in 10% to 15% of patients. Clinically, LHS manifests itself with the appearance of subcutaneous fibrocystic nodules, associated with trophic changes in the overlying skin, such as ulcers or fistulas that are difficult to manage, mainly affecting the lower extremities. Additionally, lipolysis near the joints and in the intraosseous adipose tissue can cause bilateral arthralgias, especially of the knees and ankles. We report a 57-year-old man, with a history of insulin resistance and allergic rhinitis, who presented in June 2019 with multiple subcutaneous nodules in the lower extremities, predominantly in both ankles, associated with arthralgia in that region. Additionally, a CT scan of the abdomen revealed a significant abdominal mass, measuring approximately 17 cm and in contact with the body and tail of the pancreas, pathologically compatible with an ACCP. Treatment with capecitabine was started with a favorable progression. The patient currently presents a small left lateral retro malleolar fistula, which, given the analyzes, studies and reviewed literature is concluded to be a lesion in the context of LHS.

A critical review and update on autoimmune encephalitis: understanding the alphabet soup / Revisão crítica e atualizações em encefalites autoimunes: entendendo a sopa de letrinhas

ABSTRACT Autoimmune encephalitis (AE) comprises a group of diseases mediated by antibodies against neuronal cell surface or synaptic antigens, such as ion channels or neurotransmitter receptors. New clinical syndromes and their associated antibodies were and are still being characterized over the last two decades. The fact that their main clinical features are interdisciplinary, - encompassing neuropsychiatric symptoms, cognitive dysfunction, epileptic seizures, movement and sleep disorders - has led to a surge of interest in this field. Some of these diseases present with a well-defined syndrome, being recognizable on clinical grounds. Correct diagnosis is important since AE are potentially treatable diseases, despite their severity. On the other hand, an increasing number of neuronal antibodies being described casts doubt upon the way we should utilize antibody testing and interpret results. In this article we review, summarize and update the current knowledge on antibody mediated encephalitis.

RESUMO As encefalites autoimunes compreendem um grupo de doenças mediadas por anticorpos contra antígenos de superfície neuronal ou sinapse, como canais iônicos ou receptores de neurotransmissores. Novas síndromes clínicas e os anticorpos a elas associados foram e ainda estão sendo caracterizados ao longo das últimas duas décadas. Dado que suas principais características clínicas são interdisciplinares, isto é, incluem -se sintomas neuropisquiátricos, disfunção cognitiva, crises epilépticas, distúrbio do movimento e do sono, há uma grande onda de interesse sobre esse campo de conhecimento. Algumas dessas doenças apresentam-se com uma síndrome bem definida, sendo possível reconhecê-las clinicamente. Diagnosticá-las corretamente é importante uma vez que se trata de doenças potencialmente tratáveis apesar da gravidade que lhes é característica. Por outro lado, o número crescente de anticorpos sendo descritos causam dúvida frequente sobre qual o melhor teste a solicitar e como interpretá-los. Nós aqui apresentamos uma revisão atualização resumida do conhecimento atual sobre as encefalites mediadas por anticorpos.

Eritema multiforme como manifestación paraneoplásica de un adenocarcinoma de colon

RESUMEN: El eritema multiforme es una enfermedad autoinmune vesicular-ampollosa y autolimitada, se presenta de forma aguda afectando la piel y mucosa. En 2015, Lee E. y Freer J., incluyeron esta enfermedad dentro de los síndromes dermatológicos paraneoplásicos, pero actualmente no existen muchas publicaciones de esta entidad como manifestación a una neoplasia subyacente. El objetivo de esta investigación es presentar el eritema multiforme como una manifestación paraneoplásica inusual. En este caso una paciente de 64 años de edad que cursaba con adenocarcinoma de colon exhibió el eritema multiforme oral; cuyas manifestaciones bucales involucionaron gracias a la farmacoterapia local y sistémica en conjunto con la excéresis de la lesión cancerosa en el colon. Es importante reconocer la presencia de eritema multiforme como una manifestación paraneoplásica cuando no se exhiben los factores etiológicos frecuentes de esta patología y, por lo tanto, centrarse en la búsqueda subyacente de neoplasias malignas que no son muy evidentes.

ABSTRACT: Erythema multiforme is a self-limiting, blistering, autoimmune disease that presents acutely, affecting the skin and mucosa. In 2015, Lee E. and Freer J. included this disease in paraneoplastic dermatological syndromes, but there are currently not many publications about this entity as a manifestation of an underlying malignancy. The objective of this research is to present the erythema multiforme as an unusual paraneoplastic manifestation. In this case, a female 64-year-old patient with colon adenocarcinoma exhibited oral multiform erythema; whose oral manifestations regressed thanks to local and systemic pharmacotherapy in conjunction with the exeresis of the cancerous lesion in the colon. It is important to recognize the presence of erythema multiforme as a paraneoplastic manifestation when the frequent etiological factors of this pathology are not exhibited and, therefore, to focus on the underlying search for malignant neoplasms that are not very evident.

Comportamiento de las dermatosis paraneoplásicas en pacientes oncológicos. Cienfuegos, 2016-2017

RESUMEN Fundamento Las dermatosis paraneoplásicas son poco frecuentes, y acerca de ellas no existen estudios previos en la provincia de Cienfuegos. De su correcto reconocimiento depende en ocasiones el diagnóstico oportuno de una posible neoplasia interna. Objetivo describir el comportamiento de las dermatosis paraneoplásicas en pacientes oncológicos. Métodos estudio descriptivo, realizado en pacientes con neoplasias malignas, atendidos durante el período de junio de 2016 a diciembre de 2017, en el Hospital Dr. Gustavo Aldereguía Lima, de Cienfuegos. Fueron incluidos 59 pacientes con dermatosis paraneoplásicas, para los cuales se analizó tipo de dermatosis, momento de aparición y su asociación con cada tipo de neoplasia. La información se obtuvo a través del exámen clínico dermatológico y las historias clínicas. Resultados se identificaron 17 tipos de dermatosis paraneoplásicas, con destaque del signo Leser Trélat (21,0 %), seguido del Síndrome de Sweet (16,1 %) y el pioderma gangrenoso (11,3 %). En 25 (42,4 %) pacientes estas fueron detectadas antes del diagnóstico de neoplasias, entre las cuales el linfoma (86,7 %) fue la más representativa, seguido por las neoplasias de colon y mamas, aunque también se asociaron a Síndrome de Sweet y a pioderma gangrenoso. Conclusión Reconocer las lesiones características de las dermatosis paraneoplásicas facilita la detección precoz de un cáncer oculto, cuyas manifestaciones propias, probablemente, aparecerían meses después, incluso años.

ABSTRACT Background Paraneoplastic dermatoses are rare, and about them there are no previous studies in the Cienfuegos province. The timely diagnosis of a possible internal neoplasm sometimes depends on its correct recognition. Objective to describe the behavior of paraneoplastic dermatoses in cancer patients. Methods descriptive study, carried out in patients with malignant neoplasms, treated from June 2016 to December 2017, at the Dr. Gustavo Aldereguía Lima Hospital, in Cienfuegos. Fifty-nine patients with paraneoplastic dermatosis were included, for which the type of dermatosis, time of appearance and its association with each type of neoplasia were analyzed. The information was obtained through the clinical dermatological examination and the medical records. Results 17 types of paraneoplastic dermatoses were identified, highlighting the Leser Trélat sign (21.0%), followed by Sweet's syndrome (16.1%) and pyoderma gangrenosum (11.3%). In 25 (42.4%) patients these were detected before the diagnosis of neoplasms, among which lymphoma (86.7%) was the most representative, followed by colon and breast neoplasms, although they were also associated with Syndrome of Sweet and a pyoderma gangrenosum. Conclusion Recognizing the characteristic lesions of paraneoplastic dermatoses facilitates the early detection of a hidden cancer, whose own manifestations would probably appear months later, even years.

Síndrome Paraneoplásico: Dermatomiositis secundaria a carcinoma de vesícula biliar: Reporte de un caso / Paraneoplastic Syndrome. Dermatomyositis secondary to gallbladder carcinoma: A case report

RESUMEN La dermatomiositis paraneoplásica, y en particular vinculada a carcinoma de vesícula biliar es una entidad infrecuente, con menos de diez casos reportados a nivel mundial. Presentamos un paciente de 74 años con manifestaciones clínicas y dermatológicas asociadas a dermatomiositis, lo que impulsó a realizar estudios más específicos, los cuales permitieron diagnosticar finalmente un carcinoma de vesícula biliar. Haremos un repaso de esta patología en relación con el caso presentado, destacando la importancia del examen dermatológico completo como primer eslabón en el diagnóstico de neoplasias de órganos internos.

ABSTRACT Paraneoplastic dermatomyositis, particularly associated with gallbladder carcinoma, is an infrequent entity, with less than 10 cases reported worldwide. We present a 74-year-old patient with clinical and dermatological manifestations associated with dermatomyositis, which prompted more specific studies, to finally find gallbladder carcinoma. We will review this pathology in relation to the case presented, highlighting the importance of the complete dermatological examination as the first link in the diagnosis of neoplasms of internal organs.

Clinical analysis of 11 cases of lymphoma complicated with paraneoplastic neurological syndrome / 中华血液学杂志

Objective: To evaluate the clinical characteristics, treatment, and prognosis of patients with paraneoplastic neurological syndrome (PNS) associated with lymphoma. Methods: Between January 2012 and May 2021, the clinical data of 11 patients with lymphoma complicated with PNS treated at Peking Union Medical College Hospital were retrospectively reviewed. Results: Among the 11 patients (8 male and 3 female) , the median onset age was 61 (range, 33-78) years. The symptoms of PNS preceded lymphoma in 10 patients. The median time from the onset of PNS to the diagnosis of lymphoma was 4 months. Of the 11 patients, one had Hodgkin's lymphoma, 8 had B-cell non-Hodgkin's lymphoma, and 2 had peripheral T-cell lymphoma. Seven patients were evaluated for onconeural antibody, of whom 2 were positive (1 for anti-Ma2 antibody and 1 for anti-Yo antibody) . Of the 11 patients, the PNS symptoms of 3 patients were located in the central nervous system, 4 were located in the peripheral nervous system, and 3 were located in the muscle. Eight of the 11 patients were treated with glucocorticoid-based immunosuppressive therapy before the diagnosis of lymphoma. Patients with central nervous system involvement and dermatomyositis responded well to glucocorticoid, whereas patients with peripheral neuropathy did not significantly benefit. All 11 patients were treated with chemotherapy after the diagnosis of lymphoma. The efficacy of chemotherapy was assessed in 9 patients, 7 cases achieved complete remission, 1 case was evaluated as stable disease, and 1 case was evaluated as disease progression. The PNS symptoms of the patients who achieved complete response were almost completely recovered. The median follow-up time was 42 (range, 4-95) months. At the end of the follow-up period, 6 of the 11 patients survived, 3 were lost to follow-up, and 2 died. The median overall survival of the whole group was not reached. Conclusions: PNS can involve various parts of the nervous system and can be associated with different types of lymphoma. Through early diagnosis and treatment, the PNS symptoms could improve in most patients who achieve complete remission of lymphoma.

Primary hepatic carcinoma with paraneoplastic syndrome: research progresses on clinical types and their basic mechanisms / 中华肝胆外科杂志

The onset of primary hepatic carcinoma (PHC) is usually occult, and early symptoms are not obvious. Most patients are at advanced stages of disease at diagnosis, and the prognosis is poor. Paraneoplastic syndrome (PNS) refers to the clinical manifestations indirectly caused by tumor metabolites or abnormal immune reactions that cannot be explained by the primary lesion, local tumor spread or distant metastasis. Hypercholesterolemia, hypercalcemia and hypoglycemia are the most commonly seen clinical presentations of PNS in PHC patients. Adequate understanding of PNS is of great importance in early diagnosis and treatment of PHC. In this review, we summarized the clinical manifestations and prognostic mechanisms of PNS in patients with PHC.

Anti-amphiphysin-associated paraneoplastic brainstem encephalitis with esophageal neuroendocrine carcinoma: a case report / 中华神经科杂志

Paraneoplastic neurological syndromes (PNS) are heterogeneous disorders caused by autoimmune responses of cancer, which can affect any part of the nervous system. Anti-amphiphysin antibody is one of the high-risk PNS antibodies, which is usually associated with small cell lung cancer and breast cancer. However, extrapulmonary neuroendocrine carcinoma is rare in patients with anti-amphiphysin antibody. A case of anti-amphiphysin-associated paraneoplastic brainstem encephalitis with esophageal neuroendocrine carcinoma is reported. The tumor was detected by fluorine 18 fluorodeoxyglucose positron emission tomography and pathologically confirmed by gastroscopic biopsy. The patient′s neurological symptoms were partially improved after treatment of intravenous immunoglobulin and glucocorticoids. However, the disease prognosis is closely related to the accompanying tumor.