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.

Encefalitis límbica: clínica, patogenia y problemas diagnósticos / Limbic encephalitis: clinic, pathogenesis and diagnostic problems

La encefalitis límbica es una enfermedad infrecuente y potencialmente grave, que puede o no ser paraneoplásica y se caracteriza por déficit de la memoria reciente, alteraciones psiquiátricas y convulsiones. De origen autoinmunitario, está asociada a anticuerpos séricos e intratecales contra antígenos neuronales intracelulares y de superficie, con especial afectación de zonas límbicas. En este artículo se revisan aspectos históricos y epidemiológicos, patogenia, síndromes más frecuentes y mejor delimitados, histopatología y estudios complementarios. Se repasan también las dificultades del diagnóstico diferencial y la necesidad de descartar siempre un tumor subyacente. La detección de autoanticuerpos neuronales es importante para el diagnóstico, la planificación terapéutica y el pronóstico. La inmunoterapia y, si corresponde, el tratamiento de la neoplasia son cruciales para lograr una recuperación neurológica sustancial. La encefalitis límbica es una entidad probablemente subdiagnosticada, con un pronóstico más favorable si se trata de forma temprana. El actual conocimiento de su patogenia puede además aportar claridad para la mejor comprensión de otros síndromes neurológicos y psiquiátricos que puedan compartir mecanismos autoinmunitarios, como algunos trastornos psicóticos y epilepsias farmacorresistentes. (AU)

Limbic encephalitis is a rare and potentially serious disease, which may or may not be paraneoplastic and is characterized by recent memory deficits, psychiatric disturbances and seizures. Of autoimmune origin, it is associated with serum and intrathecal antibodies against intracellular and surface neuronal antigens, with special involvement of limbic areas. This article reviews historical and epidemiological aspects, pathogenesis, more frequent and better defined syndromes, histopathology and complementary studies. The difficulties of differential diagnosis and the need to always rule out an underlying tumor are also reviewed. Detection of neuronal autoantibodies is important for diagnosis, therapeutic planning and prognosis. Immunotherapy and, if appropriate, neoplasm treatment, are crucial to achieve substantial neurological recovery. Limbic encephalitis is probably an underdiagnosed entity, with a more favorable prognosis if treated early. The current knowledge of its pathogenesis may also provide clarity for a better understanding of other neurological and psychiatric syndromes that may share autoimmune mechanisms, such as some psychotic disorders and drug-resistant epilepsies. (AU)

Two Cases of Seronegative Paraneoplastic Neurologic Syndrome with Opsoclonus Nystagmus / 대한이비인후과학회지

The dizziness associated with paraneoplastic neurologic syndrome is hard to diagnose clinically because the prevalence of disease is rare, and radiologic and serologic examination result may come out normal. Opsoclonus-myoclonus symdrome is a representative of classical paraneoplastic neurologic syndromes. In this paper, we report 2 cases of paraneoplastic neurologic syndromes with negative serologic auto-antibody test and no brain lesion on MRI. Both cases were eventually diagnosed through PET. Patients with opsoclonus-myoclonus type nystagmus should be evaluated for paraneoplastic neurologic syndrome even if their radiologic and serologic findings are normal.

Cáncer de mama metastásico oculto: a propósito de un caso / Hidden Metastatic Breast Cancer: A Case Report

El cáncer de mama triple negativo es un tumor agresivo que, muy pocas veces en la clínica, se presenta con un síndrome neurológico paraneoplásico asociado a un carcinoma metastásico de origen desconocido. Este artículo reporta una paciente con tal asociación y que su caso fue discutida en Clínica Patológica Institucional(AU)

Triple negative breast cancer is an aggressive tumor that, in the clinic, rarely presents with a paraneoplastic neurological syndrome associated with a metastatic carcinoma of unknown origin. This article reports a patient with such an association and that was discussed in Institutional Pathological Clinic(AU)

Partial spontaneous remission of small cell lung carcinoma with neurologic symptom / 영남의대학술지

Small cell lung carcinoma (SCLC) is a cancer that shows aggressive behavior, early spread to distant sites, and frequent association with distinct paraneoplastic syndromes. Spontaneous remission of cancer, particularly of SCLC, is a rare biological event. Cases involving spontaneous regression of SCLC were reported, and were associated with paraneoplastic syndromes of the nervous system. This article reports on a 78-year-old man with SCLC in remission, with neurological symptoms. The patient visited the hospital because of generalized weakness, and imaging studies revealed a mass in the lower lobe of the left lung, pathological evaluation showed SCLC. The patient refused oncologic treatment and was treated only with conservative care. In follow-up study the diameter of the mass had decreased from initial 32 mm, 9 months after admission to 20 mm, 17 months after admission to 13 mm. The patient kept complaining of generalized weakness, dizziness, and paresthesia of limbs. We assumed that, in this case, the spontaneous remission of lung cancer was related to the immunologic response directed against the tumor, which is believed to be an important factor in the pathogenesis of paraneoplastic neurologic syndromes.

Partial spontaneous remission of small cell lung carcinoma with neurologic symptom / 영남의대학술지

Small cell lung carcinoma (SCLC) is a cancer that shows aggressive behavior, early spread to distant sites, and frequent association with distinct paraneoplastic syndromes. Spontaneous remission of cancer, particularly of SCLC, is a rare biological event. Cases involving spontaneous regression of SCLC were reported, and were associated with paraneoplastic syndromes of the nervous system. This article reports on a 78-year-old man with SCLC in remission, with neurological symptoms. The patient visited the hospital because of generalized weakness, and imaging studies revealed a mass in the lower lobe of the left lung, pathological evaluation showed SCLC. The patient refused oncologic treatment and was treated only with conservative care. In follow-up study the diameter of the mass had decreased from initial 32 mm, 9 months after admission to 20 mm, 17 months after admission to 13 mm. The patient kept complaining of generalized weakness, dizziness, and paresthesia of limbs. We assumed that, in this case, the spontaneous remission of lung cancer was related to the immunologic response directed against the tumor, which is believed to be an important factor in the pathogenesis of paraneoplastic neurologic syndromes.

Síndrome de kinsbourne / Kinsbourne syndrome

Antecedentes: El Síndrome Kinsbourne es un desorden neurológico raro caracterizado por movimientos oculares irregulares, involuntarios y multidireccionales (opsoclonos), polimioclonias difusas y ataxia. Puede ser de etiología paraneoplásica (neuroblastoma) en el 50% de pacientes, pero existen múltiples causas dentro de ellas, las para y post infecciosas. Caso clínico: Masculino de 1 año de edad, con inestabilidad de la marcha. Como único antecedente proceso respiratorio y gastrointestinal (rinorrea hialina, tos productiva así como diarrea) una semana previa al inicio del padecimiento. A la exploración física presentaba ataxia a la bipedestación que imposibilitaba la marcha. Ante la ausencia de otra sintomatología es considerado inicialmente como una cerebelitis postinfecciosa, posteriormente se agregan al cuadro clínico polimioclonias y opsoclonos, con estos datos se hace el diagnóstico de síndrome de Kinsbourne. La Imagen de resonancia magnética cerebral, electroencefalograma, citoquímica y cultivo de líquido cefalorraquídeo no mostraron alteraciones. Se realizó tomografía axial abdominal y catecolaminas en orina en busca de neuroblastoma, ambos estudios normales. Se dio manejo con prednisolona a dosis de 2 mg/kg/día. Al mes de tratamiento el paciente estaba asintomático Conclusión: el síndrome opsoclonos mioclonos es una entidad rara que debe ser considerada como diagnóstico diferencial en los casos de ataxia aguda...

Síndrome neurológico paraneoplásico como manifestación de un carcinoma de colon / Paraneoplastic neurological syndrome as a colon cancer manifestation

Los síndromes paraneoplásicos neurológicos son poco comunes, más frecuentes en neoplasias ginecológica y pulmonar; muy rara en neoplasias intestinales. Más del 60% de los pacientes con Síndrome Paraneoplásico presentan el cuadro neurológico antes de ser diagnosticados como portadores de una malignidad. Por otro lado, solo 5% de los casos de cáncer de colon aparece en jóvenes. Una complicación poco usual es la perforación intestinal. El objetivo de este artículo es mostrar mediante un estudio descriptivo (caso clínico) las manifestaciones poco comunes del cáncer de colon en una paciente joven, quien debutó con un síndrome paraneoplásico. La patología maligna se expresó como una perforación. Se trata de paciente femenina de 32 años de edad, quien consulto por disminución progresiva de fuerza muscular predominio proximal (cintura escapular y pélvica) y episodios de dolor abdominal difuso de l mes de evolución, hospitalizada en otra Institución durante ese período, sin diagnóstico definitivo; por exacerbación de la sintomatología es remitida. Ingresa con deterioro neuropático motor que precisó ventilación mecánica en unidad de terapia intensiva, con signos de sepsis, irritación peritoneal. Fue sometida a exploración quirúrgica con impresión diagnóstica de peritonitís difusa asociada a tumor pélvico. En los hallazgos intraoperatorios se evidenciaron 3000 cc de pus en cavidad abdominal, perforación del ángulo hepático del colon. Se realizó hemicolectomía derecha, ileostomía terminal y fistula mucosa del transverso. Resultado anatomopatológico: Adenocarcinoma de colon (Dukes B2). La paciente evolucionó satisfactoriamente con restitución de fuerza muscular hasta IV/V. Recibió posteriormente quimioterapia coadyuvante. Diagnóstico de egreso: Adenocarcinoma de colon bien diferenciado.- Síndrome Paraneoplásico Neurológico: Polineuropatía motora desmielinizante crónica.

Neurologic Paraneoplastic Syndromes (NPS) are rare, most common in pulmonary and Gynecologic tumor and are very rare in intestinal neoplasms. Over 60% of patients with paraneoplastic syndrome begin with neurological manifestations before being diagnosed as having a malignancy. On the other hand, only 5% of cases of colon cancer appear in young people. An unusual complication is intestinal perforation. The aim of this paper is to show through a descriptive study (case report), rare manifestations of colon cancer in a young patient who presented with a NPS. The malignant disease was shown as a perforation. This female patient aged 32, who consulted with progressive decline of muscle strength predominantly proximal (shoulder and pelvic girdle), and episodes of diffuse abdominal pain of one month of evolution was hospitalized in another institution during that period with no definitive diagnosis. For exacerbation of symptoms she was referred to our hospital. She began neuropathic motor impairment needing mechanical ventilation, signs of sepsis and also signs of peritoneal irritation. She underwent surgery with clinical impression of diffuse peritonitis and pelvic tumour. Findings: 3000 cc of pus in the abdominal cavity and a perforation at the hepatic flexure of the colon. Right hemicolectomy and end ileostomy and mucous fistula of the transverse were performed. Anatomopathology: colon adenocarcinoma (Dukes B2). Patient recovered satisfactorily with restoration of muscle strength to IV/V. Subsequently, she received adjuvant chemotherapy. Discharge diagnosis: colon well differentiated adenocarcinoma - Paraneoplastic Neurological Syndrome: Chronic demyelinating motor polyneuropathy.

Paraneoplastic neurological syndrome in 12 children / 中华儿科杂志

<p><b>OBJECTIVE</b>To investigate the basic clinical characteristics of paraneoplastic neurological syndrome (PNS) in children.</p><p><b>METHOD</b>To retrospectively analyze the clinical data of 12 PNS children who were hospitalized in neurology department in Beijing Children's Hospital from 2010 to 2011. Some patients were followed up after surgery.</p><p><b>RESULT</b>In 12 patients with PNS, 11 were male and 1 was female. The mean onset age were (30.5 ± 15.3) months. The mean duration from neurological symptom onset to finding out of tumor was (112.7 ± 154.4) days. The onset of the disease in 2 patients was acute, in 3 was subacute and in the other 7 was chronic (2 of 7 had 2 to 3 relapses). Of 12 patients, 11 had symptoms of ataxia (3 patients also had opsoclonus and myoclonus, OMS), 1 had weakness of limbs at onset and then had ataxia. Nine of 12 patients had surgery, and pathologic diagnosis was neuroblastoma and ganglioneuroma. Six patients were followed-up for 8 to 21 months. One patient had a little improvement and 5 almost recovered.</p><p><b>CONCLUSION</b>The PNS children can have neurological symptoms only at the onset and there were no particular evidence of tumor. It is prone to misdiagnosis. The prognosis of PNS in children was poor.</p>

A Case of Cranial Nerve Palsy as a Paraneoplastic Syndrome in Non-Small Cell Lung Cancer / 결핵및호흡기질환

Paraneoplastic neurologic syndrome is a group of assorted disorders resulting from damage to the nervous system in cancer, remote from primary site, and not related to metastasis, infection, or metabolic disorder associated with cancer. Patient with small cell lung cancer sometimes shows various neurological syndromes, but patient with non-small cell lung cancer rarely shows neurologic syndromes and few antineuronal antibodies have been found. Here, we report a case of 53-year-old male patient who developed ptosis and extraocular muscular limitation of left eye due to third and forth cranial nerve palsy in non-small cell lung cancer without brain metastasis. These neurologic symptoms improved after lobectomy without any other treatment immunotherapy.

Síndrome de opsoclonus-mioclonus-ataxia: relato de caso / Opsoclonus-myoclonus-ataxia syndrome: case report

Objetivo: descrever um caso da síndrome de opsoclonia-mioclonia-ataxia. Relato de caso: uma menina de 2 anos de idade passou a apresentar, há 1 ano, distúrbio da marcha, cefaléia e dificuldade visual. Ao exame físico tinha movimentos...

Objective: to report a case of opsoclonus-myoclonus-ataxia syndrome. Case report: a two years old girl was taken to hospital with a complaint of gait difficulty, headache and visual impairment lasting one year. At physical exam fast eyes movement...

Paraneoplastic papilloedema in a child with neuroblastoma.

Non-metastatic neurological disease complicating neuroblastoma is well recognized. Gross papilloedema in the absence of intracranial disease as initial manifestation of neuroblastoma is reported in adults. We report for the first time a case of bilateral papilloedema in a child with neuroblastoma in the absence of intracranial disease and hypertension.

study of paraneoplastic syndrome patterns in patients with bronchogenic carcinoma

Paraneoplastic syndromes, which are the distant effect of underlying carcinoma, which can present early, well before the primary lung lesion produces local symptoms and even when the tumor is undetected or very small. Paraneoplastic syndromes are common in patients with bronchogenic carcinoma. They may be the presenting finding or the first sign of recurrence. To assess the incidence and pattern of paraneoplastic syndrome [PNS] in patients with bronchogenic carcinoma. Peripheral Hospital in Kuwait. Retrospective study. A retrospective study of 52 confirmed bronchogenic carcinoma patients occurring from January 2000 to June 2004 in our hospital was carried out. The Medical records of all the confirmed cases of bronchogenic carcinoma admitted between January 2000 and June 2004 were reviewed. The clinical and biochemical parameters recorded were: age, sex, underlying medical illness, presentation complete blood count, blood urea, electrolytes and serum calcium levels, and appropriate hormone levels where indicated. The localization of the tumors was done by chest x-ray and CT scan. Tissue biopsy was obtained by bronchoscopy or under CT scan guidance and histopathological examination of the specimen was carried out. The type and pattern of PNS if any was noted. These parameters were entered into a database and later analyzed. The data management and statistical package used was Epi Info version 6 provided readily by the CDC [Centre for Disease Control] Bethesda USA. Forty-nine patients were male and 3 were females. All were heavy smokers or exsmokers. The histological types were 12 [22.4%] small cell carcinoma and 40 [67.6%] nonsmall carcinoma. The mean age 67.46 +/- 12.15 years. Associated illness found was diabetes mellitus [DM] ' 24 [46%], hypertension - 33 [63.6%], and chronic obstructive pulmonary disease [COPD] - 14 [27%]. Almost all the patients presented with cough - 52 [100%], shortness of breath [SOB] - 51 [99%], haemoptysis - 22 [42%], pleural pain - 16 [31%] and fever - 22 [42%] and weight loss - 39 [75%]. The PNS found were: Hypercalcemia - 22 [42%], thrombocytosis - 5 [9.6%], limbic encephalitis - 3 [5.8%], SIADH - 3 [5.8%], peripheral neuropathy - 2 [3.8%], GBS - 1 [1.9%], pancytopenia - 3 [5.8%]. There was no case with ACTH secretion or Lambert-Eaton syndrome. Hypercalcemia, thrombocytosis, SIADH, pancytopenia, limbic encephalitis and other unexplained neurological problems occurring in the middle aged or elderly smokers should arouse the suspicion of an occult bronchogenic carcinoma

A case of opsoclonus-myoclonus-ataxia with neuroblastoma.

A 2 year old boy presented with features of opsoclonus, myoclonus and ataxia. Routine investigations of blood, urine, x-ray chest, bone scan, EEG and MRI of brain, were normal. Urine for VMA was negative. A right suprarenal mass was detected at MRI of abdomen. The mass was resected completely and was found histologically to be of differentiating type of neuroblastoma. The child was treated initially with prednisolone for 6 weeks along with sodium valproate. He is still on sodium valproate for his neurological symptoms. His symptoms still persist though they have decreased in intensity.

Progressive encephalomyelitis with rigidity: a paraneoplastic presentation of oat cell carcinoma of the lung. Case report

A encefalomielite progressiva com rigidez e mioclonia (PEWR) é doença neurológica rara, caracterizada por rigidez muscular, espasmos dolorosos, mioclonia e evidência de envolvimento de tronco cerebral e medula espinhal. Um paciente branco de 73 anos foi admitido com história de 10 dias de espasmos musculares dolorosos e rigidez muscular contínua no membro inferior esquerdo. Apresentava espasmos involuntários em membros inferiores e evoluiu com encefalopatia associada a sinais de nervos cranianos e sintomatologia de trato longo de medula espinhal. A tomografia computadorizada de crânio e a ressonância magnética de coluna foram normais. O LCR evidenciou pleocitose linfocítica, sem outras alterações. A EMG mostrou atividade muscular involuntária, de duração de 2-6 segundos, intervalo de 30-50 ms e uma freqüência de 2/segundo no membro inferior esquerdo. Foram detectados anticorpos anti-GAD no sangue. Na evolução, foram observados sinais radiográficos de neoplasia pulmonar, sendo posteriormente diagnosticado carcinoma de pequenas células de pulmão. O paciente faleceu duas semanas após o diagnóstico de câncer.

Opsoclonus myoclonus syndrome: response to plasmapheresis.

An 18-month-old with idiopathic opsomyoclonus, refractory to therapy with ACTH, corticosteroids, and clonazepam received plasmapheresis along with oral corticosteroids and azathioprine. The subject improved dramatically following this treatment. Anticerebellar antibodies were detected from the plasma in this patient and in a two and a half year-old girl with cerebellar ataxia secondary to an adrenal ganglioneuroma.