Giant immature teratoma in a 15 year old causing anti-n-methyl-d-aspartate (anti-nmda) receptor encephalitis-like and Guillain-Barré Syndrome as paraneoplastic manifestations: A case report / Philippine Journal of Obstetrics and Gynecology

@#Germ cell tumor is the most prevalent ovarian tumor in young women between 10 and 30 years of age.[1] However, immature teratomas account for only 20% of the malignant ovarian tumors found in the adolescent age group.[2] More uncommon is the occurrence of immature teratoma causing anti‑N‑methyl‑D‑aspartate receptor (Anti‑NMDAR) encephalitis and Guillain‑Barré Syndrome (GBS). This is a case of a 15‑year‑old female, nulligravid, who initially presented sudden behavioral change and symmetrical weakness of both lower and upper extremities with concomitant seizure episodes and with palpable lower abdominal mass. The patient was diagnosed to have GBS and treated with intravenous immunoglobulin causing resolution of neurologic symptoms months after. During the management of GBS, the patient noted increasing abdominal girth. Tumor markers showed elevated alpha‑fetoprotein, CA125, and lactate dehydrogenase. An imaging study revealed predominantly solid ovarian mass hence malignancy was considered. Once the medical condition stabilized, the patient underwent fertility‑sparing surgery with the final histopathologic result of immature teratoma. The aim of this report is to present a case of anti‑NMDAR encephalitis and GBS as paraneoplastic manifestation of immature teratoma.

Reporte de un caso fatal de encefalitis autoinmunitaria asociada a teratoma ovárico / Report of a fatal case of autoimmune encephalitis associated with an ovarian teratoma

INTRODUCCIÓN: El teratoma del ovario es el tumor de células germinales más frecuente. Entre sus complicaciones se describen algunos síndromes paraneoplásicos, como la encefalitis por anticuerpos contra el receptor N-metil-D-aspartato (NMDA). CASO CLÍNICO: Mujer de 22 años sin antecedentes de importancia que consulta por cuadro clínico de 4 días de evolución caracterizado por síntomas psiquiátricos y un episodio convulsivo. Se considera un cuadro de meningoencefalitis y se indica manejo antibiótico de amplio espectro y antiviral. Ante el deterioro se sospecha una encefalitis autoinmunitaria, se identifica un tumor anexial en los estudios imagenológicos compatible con teratoma y se confirma el diagnóstico con el hallazgo de anticuerpos NMDA en el líquido cefalorraquídeo. A pesar de su resección y manejo sistémico, fallece a los 5 meses. DISCUSIÓN: La encefalitis autoinmunitaria asociada a un teratoma es inusual, pero es una complicación que debe sospecharse como diagnóstico de exclusión. La mayoría tienen un pronóstico favorable, aunque hasta una cuarta parte de los casos puede asociarse a daño irreversible en la corteza del hipocampo e incluso la muerte, principalmente cuando el diagnóstico y el tratamiento son tardíos. CONCLUSIONES: Este caso es un reto clínico que representa un vacío en la evidencia actual, puesto que no existe un estándar de manejo de los teratomas. Se propone que, una vez diagnosticado un teratoma, se realice una cistectomía ovárica. Son necesarios más estudios para validar esta recomendación.

INTRODUCTION: The ovarian teratoma is the most common cell germ tumor. Some paraneoplastic syndromes have been described, including the anti-N-methyl-D-aspartate (NMDA) receptor encephalitis as part of its complications. CASE REPORT: A 22 years old female patient with no important medical history, consults due to an acute psychotic disorder and a convulsion. A meningoencephalitis was considered and broad-spectrum antibiotics and antivirals were started. Faced with deterioration, an autoimmune encephalitis is considered as well. Imaging studies revealed an ovarian teratoma and diagnosis was confirmed with antibodies against NMDA receptor in cerebrospinal fluid. Despite its resection and systemic management, the patient dies after 5 months. DISCUSSION: The autoimmune encephalitis associated with an ovarian teratoma is rare, its a complication that must be suspected as an exclusion diagnosis and most have a favorable prognosis, however up to a quarter of cases can be associated with irreversible damage to the hippocampal cortex and even death, mainly when late diagnosis and treatment are made. CONCLUSIONS: This case is a clinical challenge, no evidence is available since there is no standard for teratoma management. It is proposed that once a teratoma is diagnosed, an ovarian cystectomy is performed. Further studies are necessary to validate this recommendation.

Pioderma gangrenoso ampollar como forma de presentación de leucemia mieloide agudo: informe de un caso / Acute myeloid leukemia presented in the form of bullous pyoderma gangrenosum: a case report

El pioderma gangrenoso ampollar es una variedad infrecuente de pioderma gangrenoso, que se asocia en el 50-70% de los casos con trastornos oncohematológicos. Se comunica el caso de una paciente de 59 años, que consultó por fiebre y ampollas purpúricas de rápida progresión, con compromiso cutáneo mucoso. Con sospecha de una enfermedad neutrofílica, ampollar, o infección por gérmenes oportunistas, se realizó biopsia de piel para estudio histopatológico, inmunofluorescencia directa y cultivo. Los cultivos y la inmunofluorescencia directa fueron negativos, y la anatomía patológica reveló un denso infiltrado inflamatorio con predominio neutrofílico en dermis. Ante el diagnóstico de pioderma gangrenoso ampollar, se realizó una punción-aspiración de médula ósea cuyo resultado fue compatible con leucemia mieloide aguda. Se instauró tratamiento con corticosteroides sistémicos, a pesar de lo cual la paciente evolucionó desfavorablemente y falleció a los 15 días de su ingreso hospitalario. Este caso ilustra la asociación de esta enfermedad cutánea con trastornos oncohematológicos y el mal pronóstico que esto implica a corto plazo. (AU)

Bullous pyoderma gangrenosum is an infrequent type of pyoderma gangrenosum, associated with onco hematological diseases in 50-70% of cases. We present the case of a 59-year-old patient with fever and mucocutaneous hemorrhagic bullous of rapid progression. A biopsy for histopathology, direct immunofluorescence (DIF) and skin culture was made, considering the possibility of neutrophilic dermatoses, bullous dermatosis or an opportunistic infection. The results of both the culture and the DIF were negative. The histopathological examination of the specimen revealed a dense dermal polymorphic infiltrate composed primarily of neutrophils. Considering bullous pyoderma gangrenosum as a potential diagnosis, a bone-marrow biopsy was performed. This study revealed an acute myeloid leukemia. Although systemic corticosteroid therapy was begun, the patient presented an unfavorable evolution that led to her death 15 days after her admission at the hospital. This case shows the association between bullous pyoderma gangrenosum and onco hematological diseases. In addition, it highlights the poor prognosis related to these diseases in the short term. (AU)

La dermatomiositis paraneoplásica como una presentación inusual de cáncer de mama / Paraneoplastic dermatomyositis as an unusual presentation of breast cancer

Introducción: La dermatomiositis es una inflamación muscular autoinmune con presencia de rash, se manifiesta con debilidad muscular proximal, asociado a complicaciones cardiacas como miocarditis y/o trastornos de la conducción. En algunas ocasiones puede ser la única manifestación clínica de una neoplasia maligna oculta, por lo cual su detección temprana puede tener grandes repercusiones en el pronóstico del tumor oculto. Objetivo: Describir el caso de una paciente con un síndrome paraneoplásico dado por una dermatopolimiositis como presentación inusual de un cáncer de mama. Caso clínico: Se trata de una paciente en quinta década de la vida sin antecedentes personales de importancia, que debuta con una debilidad en miembros superiores de predominio proximal incapacitante, fiebre y elevación importante de la creatinquinasa (CPK), bajo un diagnóstico de una dermatopolimiositis como manifestación principal de un cáncer de mama infiltrante. Conclusiones: La dermatomiositis en mujeres de mediana edad debe hacer sospechar en una patología neoplásica oculta. Lo más importante es descastar el cáncer de mama(AU)

Introduction: Dermatomyositis is an autoimmune muscle inflammation exhibited rash, and proximal muscle weakness, associated with cardiac complications such as myocarditis and / or conduction disorders. In some cases, it can be the only clinical manifestation of hidden malignancy, so its early detection can have great repercussions on the prognosis of the hidden tumor. Objective: To describe the case of a patient with a paraneoplastic syndrome caused by dermatopolymyositis as an unusual presentation of breast cancer. Clinical case report: This is a patient in her fifties with no significant clinical personal history, who had disabling proximal weakness, predominantly in her upper limbs, fever and significant elevation of creatine kinase (CPK), under a diagnosis of dermatopolymyositis as the main manifestation of an infiltrating breast cancer. Conclusions: Dermatomyositis in middle-aged women should make us suspect an occult neoplastic pathology. The most important is to rule out breast cancer(AU)

Variante "no clásica" o "atípica" del síndrome de Stauffer en paciente con carcinoma renal de células claras: reporte de caso / "Non-classical" or "atypical" variant of Stauffer syndrome in a patient with clear cell renal carcinoma: a case report

La manifestación paraneoplásica conocida como síndrome de Stauffer tiene una presentación atípica, caracterizada por ictericia y colestasis intrahepática. Presentamos el caso de un paciente de 53 años de edad, con antecedente de una masa renal derecha en plan de resección quirúrgica programada, con cuadro de evolución de dolor abdominal en hipocondrio derecho e ictericia. A su ingreso se documentó hepatoesplenomegalia, elevación de bilirrubinas a expensas de la directa, y de fosfatasa alcalina junto con elevación de transaminasas. Se descartaron causas obstructivas a nivel de vía biliar intra y extrahepática. No se documentaron metástasis o lesiones focales a nivel de parénquima, ni lesiones de etiología vascular que explicaran el cuadro. También se descartó hepatitis B, C e infección por VIH, por lo cual se consideró un probable síndrome de Stauffer. Fue llevado a nefrectomía intrahospitalaria, con posterior diagnóstico patológico compatible con carcinoma de células claras. Luego del procedimiento se normalizó la bioquímica hepática y se corrigió la ictericia. Es importante reconocer que la afectación hepática en el contexto de neoplasias, no es solo atribuida a metástasis a distancia, sino también a la existencia de síndromes paraneoplásicos como condicionantes.

The paraneoplastic manifestation known as Stauffer syndrome has an atypical presentation, characterized by jaundice and intrahepatic cholestasis. We present the case of a 53-year-old patient, with a history of a right renal mass with a planned surgical resection, who developed abdominal pain in the right upper quadrant and jaundice. Upon admission, hepatosplenomegaly, elevated bilirubin, at the expense of direct bilirubin, alkaline phosphatase and elevated transaminases were documented. Intra- and extrahepatic bile ducts obstruction were ruled out. There were no documented metastases or focal lesions at the level of the parenchyma, or lesions of vascular etiology that could explain the condition. Hepatitis B, C and HIV infection were also ruled out, and a probable Stauffer syndrome was considered. In-hospital nephrectomy was performed, with subsequent pathology compatible with clear cell carcinoma. After the procedure, liver biochemistry was normalized and jaundice was corrected. It is important to recognize that liver involvement in the context of neoplasms is not only attributed to distant metastases but to the existence of paraneoplastic syndromes as determining factors.

Case for diagnosis. Hyperpigmented and excoriated papules and nodules in a diabetic patient

Abstract Reactive perforating collagenosis is a rare perforating dermatosis clinically characterized by intensely pruritic hyperpigmented papules, plaques, and nodules with a central keratotic plug. Histopathology reveals transepidermal elimination of collagen fibers. Its pathophysiology is still under investigation, but the acquired form has been linked to systemic conditions such as diabetes mellitus and chronic kidney disease. However, it has also been described as a paraneoplastic syndrome. The authors present the case of a 65-year-old diabetic patient in which a myeloproliferative neoplasm was suspected.

Dermatomiositis paraneoplásica como presentación de linfoma. Reporte de caso y revisión de la literatura / Paraneoplastic dermatomyositis as presentation of lymphoma. Case report and literature review

RESUMEN Los síndromes paraneoplásicos neurológicos se presentan en menos del 1% de los tumores sólidos y son infrecuentes en linfomas. Se asocian a tumores con alta actividad biológica y condicionan deterioro funcional y discapacidad. La dermatomiositis se asocia a cáncer, por tanto obliga al estudio de neoplasias ocultas; su diagnóstico como síndrome paraneoplásico se establece con criterios específicos. El pronóstico funcional depende del diagnóstico oportuno, control del cáncer y de la regulación de la respuesta inmunológica. Se presenta el caso de una mujer de 65 años con dermatomiositis en el curso de un linfoma B marginal variante convencional de primario cutáneo.

ABSTRACT Neurological paraneoplastic syndromes occur in less than 1% of solid tumours and are uncommon in lymphomas. They are related to tumours with high biological activity and cause functional impairment and disability. Dermatomyositis is associated with cancer, and requires the study of hidden neoplasms. Its diagnosis as a paraneoplastic syndrome is established with specific criteria. Functional prognosis depends on early diagnosis, cancer control, and regulation of the immune response. The case is presented of a 65 year-old woman with dermatomyositis during the course of a conventional variant of a primary cutaneous B marginal lymphoma.

Síndrome de persona rígida, presentación de un caso clínico y actualidad en el tratamiento / Stiff person syndrome, presentation of a clinical case and an update on the treatment

RESUMEN El síndrome de persona rígida afecta el sistema nervioso central. Los signos clínicos relevantes son la rigidez, los espasmos musculares y sensibilidad incrementada a los estímulos externos, que inducen las contracciones musculares. Las mujeres son afectadas de 2 a 3 veces más con relación a los hombres. Hay marcadores de tipo clínico y electrofisiológico característicos. La etiología se asocia con la mediación por anticuerpos y puede ser la expresión de un síndrome paraneoplásico. El tratamiento farmacológico se realiza con medicamentos relajantes musculares y medicamentos con mecanismo inmunomodulador o inmunosupresor. Adicionalmente, se requiere un plan complementario de rehabilitación. El propósito del grupo es hacer una descripción del caso clínico, que consideramos es relevante por su baja frecuencia de presentación y realizar una actualización sobre el tema.

A B S T R A C T Stiff person syndrome affects the central nervous system. Relevant clinical signs are stiffness, muscle spasms, increased sensitivity with external stimuli that increase muscle contractions. Women are affected twice to three times more, in comparation with the men. There are characteristic clinical and electrophysiological type markers. The etiology is associated with mediation by antibodies and may be the expression of a paraneoplastic syndrome. Pharmacological treatment is focused on muscle relaxant-type medications, drugs with immunomodulatory or immunosuppressive mechanism. In adition, complementary rehabilitation treatment is required. The purpose of the group is to make the description of the clinical case that is relevant due to the low frequency of presentation and to carry out an update of the topic.

Manifestaciones paraneoplásicas a propósito de un caso con sarcoma de partes blandas y metástasis ósea / Paraneoplastic manifestations regarding a case with soft tissue sarcoma and bone metastases

Introducción: El sarcoma de tejidos blandos (STB) constituye un grupo variado de más de 60 neoplasias que solo suponen 1 por ciento del total de los tumores. Objetivo: Describir el caso de un paciente con sarcoma de partes blandas con metástasis ósea y manifestaciones paraneoplásicas. Caso clínico: Se presenta el caso de un paciente masculino de 55 años de edad con antecedentes de diabetes mellitus tipo 2 e hipertensión arterial que refiere que hace 50 días inició con decaimiento marcado, luego de permanecer 1 mes con este síntoma inició con dolor en la columna en la parte más baja de la espalda, conjuntamente iniciando con un cuadro febril. Además, tuvo pérdida de la fuerza principalmente en las piernas, y hablaba cosas incoherentes sin sentido, gran inapetencia, con pérdida de peso de 50 lb en 20 días. Ante cuadro de pérdida de la conciencia y de la fuerza principalmente en las piernas se decidió su remisión hacia el Hospital Clínico Quirúrgico Lucía Íñiguez Landín de Holguín donde se concluyó como sarcoma de partes blandas con manifestaciones paraneoplásicas y metástasis ósea. Conclusiones: El caso presentado de sarcoma de partes blandas pretende aportar información sobre esta enfermedad tan infrecuente en nuestro medio(AU)

Introduction: Soft tissue sarcoma (STB) constitutes a varied group of more than 60 malignancies that only accounts for 1% of all tumors. Objective: To report a case of a patient with soft tissue sarcoma with bone metastases and paraneoplastic manifestations. Clinical case report: We report the case of a 55-year-old male patient with a history of type 2 diabetes mellitus and hypertension, who stated that 50 days ago he began to feel markedly listless. And after a month he began to suffer pain in the spine, in the lower back, and have a fever. In addition he had loss of strength mainly in the legs, and spoke inconsistently, nonsense, great loss of appetite, with weight loss of 50 lbs in 20 days. Given the condition of loss of consciousness and strength, mainly in the legs, it was decided to refer him to Lucía Iñiguez Landín Surgical Clinical Hospital in Holguín, where a soft tissue sarcoma with paraneoplastic manifestations and bone metastases was diagnosed. Conclusions: The report of this case of soft tissue sarcoma aims to provide information about this disease, which is so rare in our setting(AU)

Erythroderma as first manifestation of squamous cell lung cancer: rare case report

Abstract Erythroderma as the first manifestation of a solid organ malignancy is rare. The underlying cancer is a challenging condition to diagnose. There are a few cases of erythroderma in cancer patients reported in the literature. We here describe the case of a 70-year-old man who presented with asthenia, weight loss, dry cough and total body erythema with desquamation over the past month. A chest computed tomography scan showed a nodular lesion, which was finally diagnosed as a squamous cell lung carcinoma. To our knowledge, as an erythroderma presentation, only 13 cases have been reported in the literature. This case report demonstrates the need to search for a neoplasm in patients presenting with erythroderma, particularly in the presence of accompanying debilitating symptoms.

Paraneoplastic pemphigus: a clinical, laboratorial, and therapeutic overview

Abstract: Paraneoplastic pemphigus is a rare and severe autoimmune blistering disease characterized by mucocutaneous lesions associated with benign and malignant neoplasms. Diagnostic criteria include the presence of chronic mucositis and polymorphic cutaneous lesions with occult or confirmed neoplasia; histopathological analysis exhibiting intraepidermal acantholysis, necrotic keratinocytes, and vacuolar interface dermatitis; direct immunofluorescence with intercellular deposits (IgG and C3) and at the basement membrane zone (IgG); indirect immunofluorescence with intercellular deposition of IgG (substrates: monkey esophagus and simple, columnar, and transitional epithelium); and, autoreactivity to desmogleins 1 and 3, desmocollins 1, 2, and 3, desmoplakins I and II, envoplakin, periplakin, epiplakin, plectin, BP230, and α-2-macroglobulin-like protein 1. Neoplasias frequently related to paraneoplastic pemphigus include chronic lymphocytic leukemia, non-Hodgkin lymphoma, carcinomas, Castleman disease, thymoma, and others. Currently, there is no standardized treatment for paraneoplastic pemphigus. Systemic corticosteroids, azathioprine, mycophenolate mofetil, cyclosporine, rituximab, cyclophosphamide, plasmapheresis, and intravenous immunoglobulin have been used, with variable outcomes. Reported survival rates in 1, 2, and 5 years are 49%, 41%, and 38%, respectively.

Leucemia mieloide aguda en adulto mayor comórbido. A propósito de un caso / Acute myeloid leukemia in a comorbid adult. About a case

Caso clínico: paciente masculino de 81 años de edad, con cuadro de 24 horas de evolución secundario a caída, caracterizado por somnolencia, desorientación en persona, tiempo y espacio, incontinencia urinaria y dificultad para la deambulación, motivo por el cual se le efectúa una tomografía simple de cráneo encontrándose hematomas subdurales bilaterales, presenta episodios de sangrado recurrente y leucocitosis sostenida , en frotis de sangre periférica se observó 28% de blastos por lo que se realiza una biopsia de medula ósea descubriéndose un cariotipo medular compatible con leucemia mieloide aguda, debido a sus características clínicas y a su mala evolución fue catalogado como paciente paliativo. Conclusiones: la leucemia mieloide aguda es una patología hematológica cuya evolución al no ser detectada genera un alto grado de mortalidad, sobre todo en el adulto mayor comórbido.

It was presented a clinical case of a male patient of 81 years of age, with a 24-hour of secondary evolution to a fall. It was characterized by drowsiness, disorientation in person, time and space, urinary incontinence and difficulty in walking, that was why a simple skull tomography was performed, in which bilateral subdural hematomas were found. The patient presented episodes of recurrent bleeding and sustained leukocytosis. Peripheral blood smear showed 28% of blasts, so a bone marrow biopsy was performed, revealing a medullary karyotype compatible with acute myeloid leukemia. The patient was classified as palliative due to its clinical characteristics and evolution. The hematological pathology above was potentially fatal. If this one was not detected early, it would generate an accelerated unfavorable evolution, especially in the elderly comorbid, as occurred in the case presented.

Intraoral acanthosis nigricans and its clinical relevance as a manifestation of a paraneoplastic syndrome: a case report / Acantosis nigricans intraoral y su relevancia clínica como manifestación de un síndrome paraneoplásico: reporte de un caso

Malignant-or-paraneoplastic acanthosis nigricans is a verrucous and hyperpigmented tumor affecting the mucosa and skin. In most cases malignant acanthosis nigricans is a distant manifestation of an intra-abdominal primary cancer. While the diagnosis of malignant acanthosis nigricans is challenging, some specific clinical and histopathological findings could lead to an accurate diagnosis. A rare clinical case of a 59-year-old female, who was referred to the maxillofacial surgery service due to a painful oral lesion in the palatine region, is presented. Upon examination, papillomatous lesions were observed on the hard palate, that were later diagnosed as intraoral malignant acanthosis nigricans secondary to gastric cancer. Both local and systemic evaluations are discussed, highlighting the relevance of a multidisciplinary approach consistent with the fact that these manifestations, although infrequent, should generate suspicion among clinicians and therefore motivation to perform a diligent and complete study since it can reveal the presence of a malignant pathology.

La acantosis nigricans maligna o paraneoplásica es un tumor verrugoso e hiperpigmentado que afecta la mucosa y la piel. En la mayoría de los casos, la acantosis nigricans maligna es una manifestación distante de un cáncer primario intraabdominal. Si bien el diagnóstico de acantosis nigricans maligna es desafiante, algunos hallazgos clínicos e histopatológicos específicos podrían conducir a un diagnóstico preciso. Se presenta un caso clínico raro de una mujer de 59 años, que fue derivada al servicio de cirugía maxilofacial debido a una lesión oral dolorosa en la región palatina. En el examen, se observaron lesiones papilomatosas en el paladar duro, que posteriormente se diagnosticaron como acantosis nigricans maligna intraoral secundaria a cáncer gástrico. Se discuten tanto las evaluaciones locales como las sistémicas, destacando la relevancia de un enfoque multidisciplinario consistente con el hecho de que estas manifestaciones, aunque poco frecuentes, deberían generar sospecha entre los clínicos y, por lo tanto, motivación para un estudio diligente y completo, ya que puede revelar la presencia de una patología maligna.

Hermann Oppenheim, a pioneer of modern German neurology / Hermann Oppenheim, um pioneiro da moderna neurologia alemã

Hermann Oppenheim (1858-1919) was a leading fgure of the modern German neurology. In spite of the antisemitic ofcial policy, besides his complex personality, he had achieved widespread recognition of his professional qualifcation that attracted neurologists from all around the world to his private clinic. However, he did not held prominent positions at University milieu, in spite of being the main assistant to Karl Westphal (1833­1890) at the Charité-Hospital, in Berlin. Oppenheim was the author of an encyclopedic book of neurology titled "Lehrbuch der Nervenkrankheiten für Ärzte und Studierende" ("Textbook of Nervous Diseases for Doctors and Students"), frst ed., 1894. He also published signifcant works on several disorders, including "traumatic neurosis" (1889) that was criticized by Jean-Martin Charcot (1825­1893), among others. He was clinically responsible for the frst successful removal of brain tumors, including pineal tumor. He coined the term "dystonia musculorum deformans", and he led to several other achievements such as amyotonia congenita ("Oppenheim's disease"), besides Oppenheim's reflex.

Hermann Oppenheim (1858-1919) foi uma fgura importante da moderna neurologia alemã. Apesar da política ofcial anti-semita, além de sua personalidade complexa, ele alcançou amplo reconhecimento de sua qualifcação profssional que atraiu neurologistas de todo o mundo para sua clínica particular. No entanto, ele não ocupou posições de destaque no meio universitário, apesar de ser o principal assistente de Karl Westphal (1833-1890) no Charité-Hospital, em Berlim. Oppenheim foi o autor de um livro enciclopédico de neurologia intitulado "Lehrbuch der Nervenkrankheiten für Ärzte und Studierende" ("Livro Didático de Doenças Nervosas para Médicos e Alunos"), editado em 1894. Ele também publicou trabalhos signifcativos sobre vários distúrbios, incluindo "neurose traumática" ( 1889) que foi criticado por Jean-Martin Charcot (1825­1893), entre outros. Ele foi clinicamente responsável pela primeira remoção bem sucedida de tumores cerebrais, incluindo o tumor pineal. Ele cunhou o termo "distonia musculorumdeformans" e levou a outras várias conquistas como a amiotonia congênita ("doença de Oppenheim"), além do reflexo de Oppenheim.

Anti-Ma2-Associated Encephalitis with Axonal Sensorimotor Polyneuropathy

Anti-Ma2-associated encephalitis is one of the paraneoplastic limbic and brainstem encephalitis characterized by decreased consciousness, parkinsonism and the limitation of vertical eye movement. It is usually associated with non-small cell lung cancer in male and female or germ cell tumor in male. Herein, we report a case of atypical anti-Ma2-associated encephalitis which presented with axonal sensorimotor polyneuropathy.

Paraneoplastic Chorea Associated with Anti-Hu Antibody and Small Cell Lung Carcinoma

No abstract available.

A Man Presenting with Sudden Weakness and Pain of the Right Hand, by Non-Small Cell Lung Cancer with Brain Metastases

Unexplained pain and weakness, i.e., without obvious predisposing factors, are often encountered by physiatrists and efforts should be made to determine the cause. A 63-year-old male presented with radiating pain in his right arm and mild weakness of the right hand. An electrodiagnostic examination revealed distal symmetric sensory polyneuropathy in the upper and lower extremities, and denervation potentials in the forearm muscles, which were inconsistent with the cervical spine MRI images and symptoms. A predisposing undiscovered disease was revealed, i.e., squamous cell carcinoma in the lung; brain metastasis affecting the left primary motor cortex was also detected. Therefore, we concluded that the pain and weakness were related to paraneoplastic syndrome and brain metastases of the hand knob. The observed denervation potentials were characterized as trans-synaptic changes in the brain metastasis. This case highlights the importance of unexplainable focal pain and weakness in the increasing prevalence of cancer.

Concurrence of ovarian cancer and dermatomyositis: a propensity score analysis / 부인종양

OBJECTIVE: To analyze the clinical characteristics, prognosis and parallel clinical course of ovarian cancer (OC) and dermatomyositis (DM). METHODS: The medical records of 23 consecutive patients who were diagnosed with OC and DM and were treated at Peking Union Medical College Hospital (PUMCH) between 2002 and 2017 were reviewed. Propensity score matching method was used to match control group (OC patients without DM) at a ratio of 1:5. The correlation between OC and DM was measured using the Pearson correlation scatter plot and Pearson's r. Kaplan-Meier survival analysis and Cox proportional hazard regression analysis were performed to evaluate the prognostic factors. RESULTS: After matching, 23 patients who have the concurrence of OC and DM (DM group) and 115 patients diagnosed with OC alone (No DM group) were included. The 5-year overall survival rates (71.6% vs. 51.8%, p=0.020) and 5-year progression-free survival (30.5% vs. 0%, p=0.018) were poorer in DM group. Correlation between serum cancer antigen 125 (CA 125) and creatine kinase (CK) level was observed in 12 patients. The time between OC and DM diagnosis is significant through univariable analysis (p=0.021) but not in multivariable analysis in patients who have the concurrence of OC and DM. CONCLUSION: The concurrence of OC and DM as a paraneoplastic syndrome is rare and has a poor prognosis. The risk for patients diagnosed with DM is highest within 3 years before or after OC diagnosis. A correlation and a parallel clinical course exist between these 2 diseases.

POEMS Syndrome: Bone Marrow, Laboratory, and Clinical Findings in 24 Korean Patients

POEMS syndrome is a rare paraneoplastic syndrome, which includes polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes due to plasma cell (PC) neoplasm. Diagnosis of this disease is challenging because of its rarity and complex clinical manifestations. We attempted to identify the key clinical features and characteristic bone marrow (BM) findings of POEMS syndrome, by reviewing the medical records and BM analyses of 24 Korean patients. Frequent clinical manifestations included polyneuropathy (100%), monoclonal gammopathy (100%), organomegaly (92%), extravascular volume overload (79%), and endocrinopathy (63%). The BM analyses revealed mild PC hyperplasia (median PCs: 5.5%) and frequent megakaryocytic hyperplasia (88%), megakaryocyte clusters (88%), and hyperlobation (100%). Flow cytometry of BM aspirates using CD138/CD38/CD45/CD19/CD56 showed normal (67%, 4/6) or neoplastic PC immunophenotypes (33%, 2/6). A diagnosis of POEMS syndrome must be considered when a patient suspected of having PC dyscrasia shows the above clinical presentation and BM findings.