RESUMO
We reported a 61-year-old man presented with 10-month progressing left sciatic neuropathy and 10-day right facial neuropathy. Serum amphiphysin-IgG was positive. 18F-FDG PET/CT of the whole body showed no signs of malignancy. Treatment with plasma exchange and oral prednisone relieved the symptoms. Nine months later, right hemiparesis and seizure of right limbs developed. 18F-FDG and 18F-PBR06 (18 kDa translocator protein, TSPO) radioligand PET/MRI of the whole body revealed intense uptake in the intracranial lesions. Intracranial lymphoma was diagnosed by stereotactic needle brain biopsy. Mononeuropathies could be paraneoplastic syndromes. TSPO shows high uptake in intracranial lymphoma on 18F-PBR06 PET images.
Assuntos
Neoplasias do Sistema Nervoso Central , Doenças do Nervo Facial , Linfoma , Neuropatia Ciática , Humanos , Masculino , Pessoa de Meia-Idade , Encéfalo/imunologia , Doenças do Nervo Facial/etiologia , Doenças do Nervo Facial/imunologia , Doenças do Nervo Facial/terapia , Fluordesoxiglucose F18 , Imunoglobulina G/imunologia , Tomografia por Emissão de Pósitrons combinada à Tomografia Computadorizada/métodos , Tomografia por Emissão de Pósitrons/métodos , Compostos Radiofarmacêuticos , Receptores de GABA/metabolismo , Neuropatia Ciática/etiologia , Neuropatia Ciática/imunologia , Neuropatia Ciática/terapia , Neoplasias do Sistema Nervoso Central/complicações , Neoplasias do Sistema Nervoso Central/diagnóstico por imagem , Neoplasias do Sistema Nervoso Central/imunologia , Doenças Autoimunes/etiologia , Doenças Autoimunes/imunologia , Linfoma/complicações , Linfoma/diagnóstico por imagem , Linfoma/imunologia , Polineuropatia Paraneoplásica/etiologia , Polineuropatia Paraneoplásica/imunologia , Prednisona/uso terapêutico , Glucocorticoides/uso terapêutico , Troca Plasmática , Proteínas do Tecido Nervoso/imunologiaRESUMO
BACKGROUND AND AIMS: SOX1 antibodies are generally associated with small cell lung cancer and anti-Hu antibody overlap is common. This case demonstrates isolated anti-SOX1 antibodies with an uncommon tumor type, and relapse of a paraneoplastic syndrome with recurrence of tumor. METHODS: We describe a case of a 65-year-old male with a paraneoplastic peripheral neuropathy and anti-SOX1 antibody positivity in the context of a prior male breast Grade 2 ductal carcinoma, in remission at the time of the initial neurological presentation. RESULTS: Treatment response to intravenous immunoglobulin (IVIg) was demonstrated. After period of clinical stability on IVIg in the context of remission of breast carcinoma, the patient experienced a relapse of his neuropathy. This was associated with tumor recurrence and again responded to tumor excision, radiotherapy and IVIg. INTERPRETATION: Male breast carcinoma has not previously been associated with anti-SOX1 antibody positive paraneoplastic neuropathy.
Assuntos
Neoplasias da Mama Masculina/complicações , Carcinoma Ductal de Mama/complicações , Polineuropatia Paraneoplásica/tratamento farmacológico , Polineuropatia Paraneoplásica/etiologia , Fatores de Transcrição SOXB1/imunologia , Idoso , Autoanticorpos/imunologia , Autoantígenos/imunologia , Humanos , Imunoglobulinas Intravenosas/uso terapêutico , Masculino , Polineuropatia Paraneoplásica/imunologiaRESUMO
INTRODUCTION: Sensory ganglionopathies or sensory neuronopathies are subacute acquired diseases of the dorsal root ganglion, frequently associated with disinmune, paraneoplastic and toxic agents. Patients present sensory alteration of asymmetric distribution and early ataxia. Early identification is essential, as they may announce an underlying neoplasia or autoimmune disease. AIM: To study asymmetries of the sensory nervous action potential (SNAP) of nerve pairs and the relationship amplitude of ulnar sensory/ulnar motor potential (USMAR) with serial electroneurophysiological studies for the early diagnosis of sensory ganglionopathies. PATIENTS AND METHODS: Six patients with sensory ganglionopathies were retrospectively studied with electroneurophysiological studies: four paraneoplastic cases with positivity for onconeuronal antibodies, one associated with Sjogren's syndrome and two idiopathic. RESULTS: Electroneurophysiological studies showed axonal sensory involvement in all cases, with asymmetry > 50% in SNAP amplitude in two pairs of nerves in four cases and normal motor with USMAR < 0.71 in five cases. Serial electroneurophysiological studies were essential in the diagnosis of two cases in the beginning of the disease with mild sensory symptoms. CONCLUSIONS: This work evidences the importance of the study of asymmetries in the amplitude of the SNAP of nerve pairs, the USMAR and the serial electroneurophysiological studies in the early diagnosis of sensory ganglionopathies, to further identification of the disinmune and onconeuronal associated antibodies with the nervous system affection to search for hidden neoplasia.
TITLE: Ganglionopatías o neuronopatías sensoriales paraneoplásicas y disinmunes. Importancia de una detección temprana.Introducción. Las ganglionopatías o neuronopatías sensoriales son enfermedades subagudas adquiridas del ganglio raquídeo dorsal, frecuentemente asociadas con trastornos disinmunes y paraneoplásicos, y agentes tóxicos. Los pacientes presentan alteración sensorial de distribución asimétrica y ataxia temprana. La identificación temprana es esencial, ya que pueden anunciar una neoplasia subyacente o una enfermedad autoinmune. Objetivo. Estudiar las asimetrías del potencial de acción nervioso sensitivo (SNAP) de pares de nervios y la relación de amplitud del potencial de acción sensitivomotor del nervio cubital (USMAR) con estudios electroneurofisiológicos seriados para el diagnóstico precoz de las ganglionopatías sensoriales. Pacientes y métodos. Se estudió retrospectivamente a siete pacientes con ganglionopatías sensoriales con estudios electroneurofisiológicos: cuatro casos paraneoplásicos con positividad para anticuerpos onconeuronales, uno asociado al síndrome de Sjögren y dos idiopáticos. Resultados. Los estudios electroneurofisiológicos mostraron afectación sensorial axonal en todos los casos, con asimetría mayor del 50% en la amplitud de SNAP en dos pares de nervios en cuatro casos y motor normal con USMAR menos de 0,71 en cinco casos. Los estudios electroneurofisiológicos seriados fueron esenciales en el diagnóstico de dos casos en el inicio de la enfermedad con síntomas sensoriales leves. Conclusiones. Este trabajo evidencia la importancia del estudio de asimetrías en la amplitud del SNAP de pares de nervios, la USMAR y los estudios electroneurofisiológicos seriados en el diagnóstico temprano de ganglionopatías sensoriales, para la consiguiente identificación de los anticuerpos disinmunes y onconeuronales con afectación del sistema nervioso periférico y la búsqueda de neoplasia oculta.
Assuntos
Gânglios Espinais , Polineuropatia Paraneoplásica/diagnóstico , Doenças do Sistema Nervoso Periférico/diagnóstico , Potenciais de Ação , Técnicas de Diagnóstico Neurológico , Diagnóstico Precoce , Eletrodiagnóstico , Humanos , Polineuropatia Paraneoplásica/imunologia , Polineuropatia Paraneoplásica/fisiopatologia , Doenças do Sistema Nervoso Periférico/imunologia , Doenças do Sistema Nervoso Periférico/fisiopatologia , Estudos RetrospectivosRESUMO
Antibodies to the ganglioside GD1b have been reported in various forms of immune-mediated neuropathy, but their clinical relevance for diagnosis and prognosis is unknown. We investigated the prevalence of anti-GD1b antibodies in acute and chronic immune-mediated neuropathies, and the clinical presentation and outcome in Guillain-Barré syndrome (GBS) and Miller Fisher-GBS overlap syndrome (MF-GBS). Anti-GD1b, anti-GM1 and anti-GQ1b antibodies were tested in serum of patients with GBS (N = 165), Miller Fisher syndrome (N = 10), MF-GBS (N = 28), monoclonal gammopathy of unknown significance neuropathy (MGUS; N = 101), chronic inflammatory demyelinating polyneuropathy (N = 29), paraneoplastic syndrome with anti-Hu-associated neuropathy (PNS; N = 11), other auto-immune diseases (AID; N = 60), and healthy controls (HC; N = 60). All samples were tested by enzyme-linked immunosorbent assay according to the Inflammatory Neuropathy Cause and Treatment protocol. IgM anti-GD1b antibodies were found in GBS (N = 4; 2.4%), MGUS (N = 3; 3.0%), and PNS patients (N = 1; 9.1%). IgG anti-GD1b antibodies were found in GBS (N = 20; 12.1%) and MF-GBS (N = 4; 14.3%) patients, but not in the AID and HC group. In the combined group of MF-GBS and GBS patients ((MF-)GBS), 14/36 (38.9%) patients with IgG anti-GD1b antibodies also had IgG anti-GM1 antibodies, and IgG anti-GD1b and IgG anti-GQ1b antibodies were found in 3/29 (10.3%) patients. Patients with (MF-)GBS and anti-GD1b without anti-GM1 antibodies did not differ regarding sensory disturbances or disease severity but recovered faster regarding the ability to walk independently compared with patients without anti-GD1b antibodies (P = 0.031) and with patients with both anti-GD1b and anti-GM1 antibodies (P = 0.034). In conclusion, testing for anti-GD1b antibodies may identify a specific group of immune-mediated neuropathies and (MF-)GBS patients with only anti-GD1b antibodies tend to recover faster.
Assuntos
Autoanticorpos/imunologia , Autoantígenos/imunologia , Doenças Autoimunes do Sistema Nervoso/imunologia , Gangliosídeos/imunologia , Gamopatia Monoclonal de Significância Indeterminada/imunologia , Polineuropatia Paraneoplásica/imunologia , Adulto , Idoso , Autoanticorpos/sangue , Doenças Autoimunes do Sistema Nervoso/sangue , Gangliosídeo G(M1)/imunologia , Síndrome de Guillain-Barré/sangue , Síndrome de Guillain-Barré/imunologia , Humanos , Imunoglobulina G/sangue , Imunoglobulina G/imunologia , Imunoglobulina M/sangue , Imunoglobulina M/imunologia , Lúpus Eritematoso Sistêmico/sangue , Lúpus Eritematoso Sistêmico/imunologia , Pessoa de Meia-Idade , Síndrome de Miller Fisher/sangue , Síndrome de Miller Fisher/imunologia , Gamopatia Monoclonal de Significância Indeterminada/sangue , Polineuropatia Paraneoplásica/sangue , Polirradiculoneuropatia Desmielinizante Inflamatória Crônica/sangue , Polirradiculoneuropatia Desmielinizante Inflamatória Crônica/imunologia , Síndrome de Sjogren/sangue , Síndrome de Sjogren/imunologiaRESUMO
INTRODUCTION: Antitumor immune responses are postulated to initiate paraneoplastic neurological disorders when proteins that are normally restricted to neural cells are expressed as oncoproteins. Mutated oncopeptides could bypass self-tolerant T cells to activate cytotoxic effector T lymphocytes and requisite helper T lymphocytes to stimulate autoantibody production by B lymphocytes. METHODS: We investigated muscle-type nicotinic acetylcholine receptor (AChR) antigen expression at transcriptional and protein levels in a small-cell lung cancer line (SCLC) established from a patient with AChR-immunoglobulin G (IgG)-positive myasthenia gravis. RESULTS: We identified messenger RNA transcripts encoding the 2 AChR α1-subunit isoforms and 7 alternative-splicing products, 3 of which yielded premature stop codons. Despite detecting native muscle-type AChR pentamers in the tumor, we did not identify mutant α1-peptides. However, we found α1-subunit-derived peptides bound to tumor major histocompatibility complex (MHC)1-protein. In a control SCLC from an antineuronal nuclear autoantibody, type 1 (anti-Hu)-IgG-positive patient, we identified MHC1-complexed Hu protein-derived peptides but not AChR peptides. DISCUSSION: Our findings support onconeural protein products as pertinent immunogens initiating paraneoplastic neurological autoimmunity. Muscle Nerve 58: 600-604, 2018.
Assuntos
Autoantígenos/genética , Miastenia Gravis/genética , Síndromes Paraneoplásicas do Sistema Nervoso/genética , RNA Mensageiro/metabolismo , Receptores Nicotínicos/genética , Idoso , Autoantígenos/imunologia , Estudos de Casos e Controles , Linhagem Celular Tumoral , Feminino , Antígenos de Histocompatibilidade Classe I/metabolismo , Humanos , Pessoa de Meia-Idade , Mutação , Miastenia Gravis/imunologia , Polineuropatia Paraneoplásica/genética , Polineuropatia Paraneoplásica/imunologia , Síndromes Paraneoplásicas do Sistema Nervoso/imunologia , Receptores Nicotínicos/imunologia , Receptores Nicotínicos/metabolismoAssuntos
Anticorpos Monoclonais Humanizados/uso terapêutico , Imunossupressores/uso terapêutico , Polineuropatia Paraneoplásica/tratamento farmacológico , Síndromes Paraneoplásicas do Sistema Nervoso/tratamento farmacológico , Humanos , Polineuropatia Paraneoplásica/complicações , Polineuropatia Paraneoplásica/imunologia , Síndromes Paraneoplásicas do Sistema Nervoso/complicações , Síndromes Paraneoplásicas do Sistema Nervoso/imunologia , Resultado do TratamentoRESUMO
PURPOSE OF REVIEW: To review recent advances in paraneoplastic neuropathies with emphasis on their definition, different forms and therapeutic development. RECENT FINDINGS: A strict definition of definite paraneoplastic neuropathies is necessary to avoid confusion. With carcinoma, seronegative sensory neuronopathies and neuronopathies and anti-Hu and anti-CV2/Contactin Response Mediator Protein 5 antibodies are the most frequent. With lymphomas, most neuropathies occur with monoclonal gammopathy including AL amyloidosis, Polyneuropathy-Organomegaly-Endocrinopathy-M component-Skin changes (POEMS) syndrome, type I cryoglobulinemia and antimyelin-associated glycoprotein (MAG) neuropathies and Waldenström's disease. Neuropathies improving with tumor treatment are occasional, occur with a variety of cancer and include motor neuron disease, chronic inflammatory demyelinating neuropathy and nerve vasculitis. If antibodies toward intracellular antigens are well characterized, it is not the case for antibodies toward cell membrane proteins. Contactin-associated protein-2 antibodies occur with neuromyotonia and thymoma with the Morvan's syndrome in addition to Netrin 1 receptor antibodies but may not be responsible for peripheral nerve hyperexcitability. The treatment of AL amyloidosis, POEMS syndrome, anti-MAG neuropathy and cryoglobulinemia is now relatively well established. It is not the case with onconeural antibodies for which the rarity of the disorders and a short therapeutic window are limiting factors for the development of clinical trials. SUMMARY: A strict definition of paraneoplastic neuropathies helps their identification and is necessary to allow an early diagnosis of the underlying tumor.
Assuntos
Polineuropatia Paraneoplásica/terapia , Autoanticorpos/imunologia , Humanos , Neoplasias/complicações , Neoplasias/terapia , Polineuropatia Paraneoplásica/classificação , Polineuropatia Paraneoplásica/imunologia , Paraproteinemias/etiologia , Paraproteinemias/terapiaRESUMO
Acute confusion is a common symptom of physical illness in the older patient. In the majority, it is transient and resolves on treatment of precipitants. In a subset of patients, however, neurological decline is progressive, raising concern about a serious underlying cause. We describe the case of a 71-year-old woman who developed progressive cognitive impairment following insertion of a permanent pacemaker for sinoatrial arrests. An initial diagnosis of delirium secondary to a urinary tract infection was suspected; however, the patient became increasingly confused despite treatment. Laboratory tests revealed serum anti-Hu paraneoplastic antibodies, and CT chest identified an occult lung tumour. Cervical lymph node histopathology confirmed a diagnosis of small cell carcinoma of the lung. Although a rare cause of confusion, paraneoplastic encephalomyelitis should be recognised early to allow timely identification and treatment of the associated cancer.
Assuntos
Carcinoma de Células Pequenas/complicações , Disfunção Cognitiva/diagnóstico , Confusão/etiologia , Delírio/complicações , Síndromes Paraneoplásicas do Sistema Nervoso/complicações , Carcinoma de Pequenas Células do Pulmão/complicações , Infecções Urinárias/complicações , Idoso , Autoanticorpos/sangue , Carcinoma de Células Pequenas/diagnóstico por imagem , Carcinoma de Células Pequenas/patologia , Disfunção Cognitiva/complicações , Confusão/diagnóstico , Proteínas ELAV/imunologia , Evolução Fatal , Feminino , Humanos , Neoplasias Pulmonares/diagnóstico por imagem , Neoplasias Pulmonares/patologia , Linfonodos/diagnóstico por imagem , Linfonodos/patologia , Marca-Passo Artificial/efeitos adversos , Polineuropatia Paraneoplásica/imunologia , Síndromes Paraneoplásicas do Sistema Nervoso/etiologia , Síndromes Paraneoplásicas do Sistema Nervoso/imunologia , Carcinoma de Pequenas Células do Pulmão/diagnóstico por imagem , Carcinoma de Pequenas Células do Pulmão/patologia , Tomografia Computadorizada por Raios X/métodos , Infecções Urinárias/diagnósticoRESUMO
Anti-titin antibodies indicate a paraneoplastic etiology pointing towards a thymoma in myasthenia gravis (MG), but their seroprevalence and potential diagnostic value in patients with other paraneoplastic neurological syndromes (PNS) is unknown. Therefore, we screened the sera of 44 PNS patients with well-characterized onconeural antibodies (anti-Hu, Yo, Ri, CV2/CRMP5, Ma1, Ma2/Ta, or amphiphysin) for anti-titin reactivity. Two patients (4.5%) were positive for anti-titin antibodies: both patients differed regarding the PNS (sensorimotor neuropathy and subacute cerebellar degeneration vs. chorea), well-characterized onconeural antibodies (CV2/CRMP5 vs. Ri), and malignoma (small cell lung cancer vs. breast cancer). However, retrospectively, the patients neither showed any symptoms of MG nor a thymoma on a computed tomographic (CT) scan. The results of this study indicate that anti-titin antibodies without a predictive relevance for MG or thymoma may be present in a small proportion of patients with PNS.
Assuntos
Autoanticorpos/sangue , Conectina/imunologia , Polineuropatia Paraneoplásica , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Feminino , Humanos , Masculino , Programas de Rastreamento/métodos , Pessoa de Meia-Idade , Polineuropatia Paraneoplásica/diagnóstico , Polineuropatia Paraneoplásica/imunologia , Polineuropatia Paraneoplásica/metabolismo , Timoma/sangueRESUMO
INTRODUCTION: Voltage-gated calcium-channel autoimmunity (VGCC-P/Q and VGCC-N types) occurs beyond Lambert-Eaton syndrome and lung cancer. METHODS: We reviewed records for 236 Mayo Clinic patients with VGCC antibodies found in evaluation for paraneoplastic neurological autoimmunity (generally without myasthenic syndromes). RESULTS: VGCC autoantibodies were detected in 3.4% of neurological patients, 1.7% of healthy controls, and 4% of neurologically asymptomatic lung cancer controls. Fifty neurological patients (21%) had ≥ 1 neoplasm, historically (46) or detected prospectively [small-cell lung carcinoma (2), breast adenocarcinoma (2), lymphoma (1), and suspected tonsillar carcinoma (1)]. Autoimmune neurological diagnosis frequencies (encephalopathy, ataxia, myelopathy, neuropathy, neuromuscular junction disorder, and myopathy) among patients with medium values (24%; 0.10-0.99 nmol/L) or low values (19%; 0.03-0.10 nmol/L) were fewer than among patients with antibody values exceeding 1.00 nmol/L (71%; P = 0.02 and 0.004, respectively). CONCLUSIONS: Among neuronal VGCC-autoantibody-seropositive patients, autoimmune neurological phenotypes and cancer types are diverse. Cautious interpretation of results (particularly medium and low values) is advised. Muscle Nerve, 2016 Muscle Nerve 54: 220-227, 2016.
Assuntos
Autoanticorpos/sangue , Canais de Cálcio Tipo N/imunologia , Neoplasias Pulmonares/sangue , Doenças do Sistema Nervoso/sangue , Polineuropatia Paraneoplásica/sangue , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Autoanticorpos/líquido cefalorraquidiano , Transtornos Cognitivos/sangue , Transtornos Cognitivos/imunologia , Feminino , Humanos , Neoplasias Pulmonares/líquido cefalorraquidiano , Neoplasias Pulmonares/imunologia , Masculino , Pessoa de Meia-Idade , Doenças do Sistema Nervoso/líquido cefalorraquidiano , Doenças do Sistema Nervoso/imunologia , Polineuropatia Paraneoplásica/líquido cefalorraquidiano , Polineuropatia Paraneoplásica/imunologia , Adulto JovemRESUMO
INTRODUCTION: Sural nerve biopsy is an important means of establishing the diagnosis of inflammatory neuropathies. We investigated the diagnostic value of endoneurial edema. METHODS: Diagnostic sural nerve biopsies from 42 patients with inflammatory and 28 patients with noninflammatory neuropathies were re-evaluated for the presence of endoneurial edema. Edema was assessed on hematoxylin-eosin stained paraffin and frozen sections and on azure II-methylene blue stained semithin sections. We determined the area of endoneurial edema on digitized images in relation to the entire endoneurial area of each fascicle. RESULTS: Edema was more extensive in neuropathies with short disease duration (≤12 months) as compared to long duration (>12 months; P < 0.01). Edema in inflammatory neuropathies of ≤12 months duration covered a larger area than in noninflammatory neuropathies (P < 0.01), and the extent of edema correlated negatively with disease duration (P < 0.05). CONCLUSIONS: Endoneurial edema may be a useful additional disease marker in inflammatory neuropathies of recent onset.
Assuntos
Edema/patologia , Doenças do Sistema Nervoso Periférico/patologia , Nervo Sural/patologia , Adulto , Idoso , Esclerose Lateral Amiotrófica/complicações , Esclerose Lateral Amiotrófica/imunologia , Esclerose Lateral Amiotrófica/patologia , Biópsia , Estudos de Casos e Controles , Neuropatias Diabéticas/complicações , Neuropatias Diabéticas/imunologia , Neuropatias Diabéticas/patologia , Edema/etiologia , Edema/imunologia , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Atrofia Muscular Espinal/complicações , Atrofia Muscular Espinal/imunologia , Atrofia Muscular Espinal/patologia , Polineuropatia Paraneoplásica/complicações , Polineuropatia Paraneoplásica/imunologia , Polineuropatia Paraneoplásica/patologia , Nervos Periféricos/imunologia , Nervos Periféricos/patologia , Doenças do Sistema Nervoso Periférico/complicações , Doenças do Sistema Nervoso Periférico/imunologia , Polirradiculoneuropatia Desmielinizante Inflamatória Crônica/complicações , Polirradiculoneuropatia Desmielinizante Inflamatória Crônica/imunologia , Polirradiculoneuropatia Desmielinizante Inflamatória Crônica/patologia , Nervo Sural/imunologiaRESUMO
Tonsillar metastatic small cell lung cancer (SCLC) is rare, while anti-Hu antibodies are frequently found in SCLC. A 66-year-old man was admitted to our hospital with painful dysesthesia and muscle weakness in the distal extremities for over 1 year, progressive dysphagia for over 1 month, and severe cough and dyspnea for over 1 week. He was diagnosed with SCLC accompanied by tonsillar metastasis and anti-Hu antibody-associated paraneoplastic sensory neuropathy (PSN). The patient tolerated 6 cycles of sequential chemoradiotherapy and gradually recovered. The patient's disease remained in remission 2 years after the diagnosis with a remarkable reduction of tumor burden and a persisting high titer of anti-Hu antibodies. To our knowledge, this is the first case of tonsillar metastatic SCLC accompanied by anti-Hu antibody-associated PSN, whereby the anticancer immune response was presumed to play a vital role in disease control. Unilateral tonsillar metastasis of SCLC accompanied by anti-Hu antibody-associated PSN can occur and in certain circumstances, may have a favorable prognosis.
Assuntos
Anticorpos/metabolismo , Proteínas ELAV/imunologia , Neoplasias Pulmonares/patologia , Polineuropatia Paraneoplásica/complicações , Carcinoma de Pequenas Células do Pulmão/secundário , Neoplasias Tonsilares/secundário , Idoso , Humanos , Neoplasias Pulmonares/imunologia , Neoplasias Pulmonares/metabolismo , Masculino , Polineuropatia Paraneoplásica/imunologia , Polineuropatia Paraneoplásica/metabolismo , Carcinoma de Pequenas Células do Pulmão/imunologia , Carcinoma de Pequenas Células do Pulmão/metabolismo , Neoplasias Tonsilares/imunologia , Neoplasias Tonsilares/metabolismoAssuntos
Polineuropatia Paraneoplásica , Autoanticorpos/imunologia , Síndrome de Guillain-Barré/imunologia , Humanos , Imagem Multimodal , Polineuropatia Paraneoplásica/diagnóstico , Polineuropatia Paraneoplásica/imunologia , Polineuropatia Paraneoplásica/terapia , Polirradiculoneuropatia Desmielinizante Inflamatória Crônica/imunologiaRESUMO
OBJECTIVES: To study possibilities of immunological and electrophysiological methods for the diagnosis of paraneoplastic polyneuropathy in cancer. METHODS: We studied 88 cancer patients using electromyography and immunological assay (serum neuronal antibodies). RESULTS: A symmetrical, distal, sensory-motor, axonal-demyelinating form of polyneuropathy can develop in breast cancer and small cell lung cancer. Onconeural antibodies were detected in the serum of more than half of study participants as well as in some healthy donors. Symptoms of polyneuropathy appeared earlier than the diagnosed tumor. CONCLUSION: The diagnostic value of the methods used for the early diagnosis of breast cancer and small cell lung cancer is emphasized.
Assuntos
Anticorpos Antineoplásicos/sangue , Autoanticorpos/sangue , Neoplasias da Mama/sangue , Neoplasias Pulmonares/sangue , Neurônios/imunologia , Polineuropatia Paraneoplásica/diagnóstico , Carcinoma de Pequenas Células do Pulmão/sangue , Idoso , Neoplasias da Mama/complicações , Neoplasias da Mama/imunologia , Feminino , Humanos , Neoplasias Pulmonares/complicações , Neoplasias Pulmonares/imunologia , Masculino , Pessoa de Meia-Idade , Polineuropatia Paraneoplásica/sangue , Polineuropatia Paraneoplásica/imunologia , Carcinoma de Pequenas Células do Pulmão/complicações , Carcinoma de Pequenas Células do Pulmão/imunologiaRESUMO
Paraneoplastic syndromes are indirect manifestations of cancer due to functional peptides/hormones produced by a tumour, or due to cross reactivity between tumour and host antigens. Here the case of a 58-year-old woman presenting with ataxia, paraesthesia and subacute and progressive loss of vision is reported. The patient exhibited strong serum positivity for anti-Hu and anti-CV2 antibodies, and a chest CT scan showed a hypodense nodule in proximity of the right upper lobe bronchus and an enlarged ipsilateral paratracheal lymph node that was not visible on a lung x-ray. Histopathological examination of a biopsy specimen from this lymph node showed that small cell carcinoma of the lung was present. The patient's deficits were subsequently diagnosed as three coexisting paraneoplastic neurological syndromes (PNSs): subacute cerebellar ataxia, sensory neuropathy and retinopathy, respectively. Although rare, PNSs can be the first manifestations of cancer, and their rapid recognition facilitates an early treatment.
Assuntos
Neoplasias Pulmonares/complicações , Síndromes Paraneoplásicas do Sistema Nervoso/etiologia , Síndromes Paraneoplásicas Oculares/etiologia , Carcinoma de Pequenas Células do Pulmão/complicações , Feminino , Humanos , Neoplasias Pulmonares/imunologia , Pessoa de Meia-Idade , Degeneração Paraneoplásica Cerebelar/etiologia , Degeneração Paraneoplásica Cerebelar/imunologia , Polineuropatia Paraneoplásica/etiologia , Polineuropatia Paraneoplásica/imunologia , Síndromes Paraneoplásicas do Sistema Nervoso/imunologia , Síndromes Paraneoplásicas Oculares/imunologia , Carcinoma de Pequenas Células do Pulmão/imunologiaAssuntos
Carcinoma Pulmonar de Células não Pequenas/imunologia , Neoplasias Pulmonares/imunologia , Glicoproteína Associada a Mielina/imunologia , Polineuropatia Paraneoplásica/imunologia , Idoso , Autoanticorpos/imunologia , Autoantígenos/imunologia , Carcinoma Pulmonar de Células não Pequenas/complicações , Humanos , Neoplasias Pulmonares/complicações , MasculinoRESUMO
Thymoma, a common anterior mediastinal tumour, may present with paraneoplastic neurological symptoms. The presence of neuronal anti-Hu paraneoplastic antibodies in thymoma patients is very rare. Here, we describe a patient who presented with symptoms of a sensory peripheral neuropathy in the presence of onconeural antibodies cross-reactive with Hu antigen, in whom an underlying thymoma was diagnosed. Subsequent minimally invasive thymomectomy improved her neurological symptoms significantly.
Assuntos
Anticorpos Antineoplásicos/imunologia , Proteínas do Tecido Nervoso/imunologia , Polineuropatia Paraneoplásica/imunologia , Timoma/complicações , Neoplasias do Timo/complicações , Idoso de 80 Anos ou mais , Diagnóstico Diferencial , Feminino , Humanos , Polineuropatia Paraneoplásica/diagnóstico , Polineuropatia Paraneoplásica/etiologia , Timoma/diagnóstico , Timoma/imunologia , Neoplasias do Timo/diagnóstico , Neoplasias do Timo/imunologia , Tomografia Computadorizada por Raios XRESUMO
IgG4-RSD should be suspected in any patient presenting with lacrimal or salivary gland enlargement, particularly if male and manifesting mild glandular dysfunction. A serum IgG4 level, if increased, may be helpful, although a gland biopsy staining for IgG4-positive plasma cells is the definitive test. Primary low-grade B cell lymphomas of the glandular tissue, specifically MALT lymphoma and other glandular malignancy, should be considered, particularly in patients with asymmetric glandular enlargement. Patients with idiopathic uveitis should have a thorough evaluation to exclude malignancy, in particular PIOL and melanoma in adults, and diffuse retinoblastoma and ALL in children. RF remains a diagnostic challenge and atypical features such as outward displacement of the retroperitoneal structures should raise the suspicion for a malignant infiltrative process. CRPS rarely may be the first presentation of an occult malignancy and requires a thorough review of age-appropriate cancer screening. Carpal tunnel syndrome, if bilateral or associated with other systemic features, should prompt a search for amyloidosis.
Assuntos
Síndromes da Dor Regional Complexa/diagnóstico , Neoplasias Primárias Desconhecidas/diagnóstico , Polineuropatia Paraneoplásica/diagnóstico , Fibrose Retroperitoneal/diagnóstico , Sialadenite/diagnóstico , Uveíte/diagnóstico , Síndromes da Dor Regional Complexa/imunologia , Diagnóstico Diferencial , Humanos , Imunoglobulina G/sangue , Neoplasias Primárias Desconhecidas/imunologia , Polineuropatia Paraneoplásica/imunologia , Paraproteinemias/diagnóstico , Paraproteinemias/imunologia , Fibrose Retroperitoneal/imunologia , Sialadenite/imunologia , Uveíte/imunologiaAssuntos
Anticorpos/sangue , Gânglios Autônomos , Polineuropatia Paraneoplásica/diagnóstico , Polineuropatia Paraneoplásica/imunologia , Receptores Colinérgicos/imunologia , Biomarcadores Tumorais/sangue , Biomarcadores Tumorais/imunologia , Progressão da Doença , Evolução Fatal , Feminino , Gânglios Autônomos/metabolismo , Humanos , Neoplasias Pulmonares/complicações , Pessoa de Meia-Idade , Polineuropatia Paraneoplásica/etiologia , Receptores Colinérgicos/metabolismo , Carcinoma de Pequenas Células do Pulmão/complicaçõesRESUMO
OBJECTIVE: To describe a patient with diencephalic and mesencephalic presentation of a Ma1 and Ma2 antibody-associated paraneoplastic neurological disorder. DESIGN: Case report. SETTING: The Colorado Neurological Institute Movement Disorders Center in Englewood, Colorado, and the Mayo Clinic in Rochester, Minnesota. PATIENT: A 55-year-old man with a paraneoplastic neurological disorder characterized by rapid eye movement sleep behavior disorder, narcolepsy, and a progressive supranuclear palsy-like syndrome in the setting of tonsillar carcinoma. INTERVENTION: Immunotherapy for paraneoplastic neurological disorder, surgery and radiotherapy for cancer, and symptomatic treatment for parkinsonism and sleep disorders. MAIN OUTCOME MEASURES: Polysomnography, multiple sleep latency test, and neurological examination. RESULTS: The cancer was detected at a limited stage and treatable. After oncological therapy and immunotherapy, symptoms stabilized. Treatment with modafinil improved daytime somnolence. CONCLUSIONS: Rapid onset and progression of multifocal deficits may be a clue to paraneoplastic etiology. Early treatment of a limited stage cancer (with or without immunotherapy) may possibly slow progression of neurological symptoms. Symptomatic treatment may be beneficial.