RESUMEN
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 < 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
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 Sjögrens 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
Asunto(s)
Humanos , Polineuropatía Paraneoplásica/diagnóstico , Diagnóstico Precoz , Enfermedades del Sistema Nervioso/patología , Polineuropatía Paraneoplásica/fisiopatología , Estudios Retrospectivos , Electrofisiología/métodos , Diagnóstico Diferencial , Electromiografía/instrumentaciónRESUMEN
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.
Asunto(s)
Ganglios Espinales , Polineuropatía Paraneoplásica/diagnóstico , Enfermedades del Sistema Nervioso Periférico/diagnóstico , Potenciales de Acción , Técnicas de Diagnóstico Neurológico , Diagnóstico Precoz , Electrodiagnóstico , Humanos , Polineuropatía Paraneoplásica/inmunología , Polineuropatía Paraneoplásica/fisiopatología , Enfermedades del Sistema Nervioso Periférico/inmunología , Enfermedades del Sistema Nervioso Periférico/fisiopatología , Estudios RetrospectivosRESUMEN
RATIONALE: Paraneoplastic syndrome is a very rare syndrome among prostate cancer patients. In particular, paraneoplastic sensorimotor neuropathy has never been reported as a complication of prostatic adenocarcinoma. PATIENT CONCERNS: A 75-year-old man who was diagnosed with prostatic adenocarcinoma with multiple metastases received cancer treatment. But, numbness and tingling sensations in both sides of the upper and lower limbs got progressively worse. DIAGNOSESE: He was diagnosed with positive anti-Hu antibodies paraneoplastic sensorimotor polyneuropathy caused by prostatic adenocarcinoma. INTERVENTIONS: The patient received physical therapy, occupational therapy, and opioid medication during 3 weeks at cancer rehabilitation department during 3 weeks. OUTCOMES: There was no improvement in functional outcome in this patient. But, the patient's neuropathic pain was improved by the use of opioid agents. LESSONS: This case report is the first to report anti-Hu antibody-positive paraneoplastic sensorimotor neuropathy in a patient with adenocarcinoma of the prostate.
Asunto(s)
Adenocarcinoma , Analgésicos Opioides/administración & dosificación , Autoanticuerpos/sangre , Proteínas ELAV/inmunología , Extremidades , Polineuropatía Paraneoplásica , Modalidades de Fisioterapia , Neoplasias de la Próstata , Adenocarcinoma/complicaciones , Adenocarcinoma/patología , Adenocarcinoma/terapia , Anciano , Protocolos Antineoplásicos , Extremidades/inervación , Extremidades/fisiopatología , Retroalimentación Sensorial , Humanos , Masculino , Estadificación de Neoplasias , Polineuropatía Paraneoplásica/sangre , Polineuropatía Paraneoplásica/etiología , Polineuropatía Paraneoplásica/fisiopatología , Polineuropatía Paraneoplásica/terapia , Neoplasias de la Próstata/complicaciones , Neoplasias de la Próstata/patología , Neoplasias de la Próstata/terapia , Resultado del TratamientoRESUMEN
BACKGROUND: Sensory ganglionopathy (SG) is characterised by asymmetrical sensory fibre degeneration, with the primary pathology occurring at the level of the dorsal root ganglion. It is seen in the context of autoimmune, paraneoplastic, and degenerative disorders. There is limited literature examining the electrophysiological correlate of the trigeminal ganglion and associated pathways, the blink reflex (BR), in cases of SG. Previous work has suggested that the BR is preserved in cases of SG associated with paraneoplasia. METHODS: The local clinical neurophysiology database was searched for patients diagnosed with SG from peripheral nerve conduction studies in whom the BR was performed. Twenty-six patients were included in the final analysis. RESULTS: Sjögren's syndrome constituted the most common SG aetiology (8/26), followed by idiopathic cases (7/26) and paraneoplasia (5/26). BR abnormalities were seen in 9 of the 26 patients (34.6%) across all aetiologies. No patients reported sensory disturbance in the distribution of the trigeminal nerve, indicating that the changes noted are subclinical. Three patients showed abnormality of the R1 response; in the remaining six patients, only R2 responses were affected. CONCLUSIONS: Subclinical abnormalities of both R1 and R2 can be seen in the context of SG of varying aetiologies, including paraneoplasia. Performing the BR in patients with suspected of having SG may be helpful in providing additional evidence of patchy sensory fibre involvement that is characteristic of the disease.
Asunto(s)
Parpadeo/fisiología , Electromiografía , Polineuropatía Paraneoplásica/fisiopatología , Trastornos de la Sensación/patología , Trastornos de la Sensación/fisiopatología , Ganglio del Trigémino/patología , Adulto , Anciano , Bases de Datos Factuales/estadística & datos numéricos , Femenino , Humanos , Masculino , Persona de Mediana Edad , Adulto JovenAsunto(s)
Neoplasias de la Mama/complicaciones , Carcinoma Ductal de Mama/complicaciones , Enfermedad de la Neurona Motora/etiología , Polineuropatía Paraneoplásica/etiología , Neoplasias de la Mama/terapia , Carcinoma Ductal de Mama/terapia , Femenino , Humanos , Persona de Mediana Edad , Polineuropatía Paraneoplásica/fisiopatologíaRESUMEN
We report the development of hypothermia in a patient with Hodgkin lymphoma which resolved with chemotherapy administration. A review of the literature revealed 16 previous reports of hypothermia in patients with Hodgkin lymphoma. Overall prognosis seems to be unfavorable. To the best of our knowledge this is the first report of hypothermia in a patient with Hodgkin lymphoma transforming from chronic lymphocytic leukemia (Richter's syndrome). A possible pathophysiology could be paraneoplastic autonomic neuropathy. Physicians should be aware that Hodgkin lymphoma can present with hypothermia and should carefully monitor newly diagnosed patients with advanced disease for this complication. Likewise, patients with Hodgkin lymphoma who develop hypothermia should be screened for signs of autonomic neuropathy.
Asunto(s)
Enfermedad de Hodgkin/fisiopatología , Hipotermia/etiología , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapéutico , Enfermedades del Sistema Nervioso Autónomo/etiología , Enfermedades del Sistema Nervioso Autónomo/fisiopatología , Enfermedades del Sistema Nervioso Autónomo/prevención & control , Transformación Celular Neoplásica , Resultado Fatal , Enfermedad de Hodgkin/tratamiento farmacológico , Enfermedad de Hodgkin/etiología , Humanos , Hipotermia/prevención & control , Leucemia Linfocítica Crónica de Células B/fisiopatología , Masculino , Persona de Mediana Edad , Polineuropatía Paraneoplásica/etiología , Polineuropatía Paraneoplásica/fisiopatología , Polineuropatía Paraneoplásica/prevención & controlAsunto(s)
Humanos , Masculino , Persona de Mediana Edad , Polineuropatías/complicaciones , Polineuropatías/diagnóstico , Polineuropatía Paraneoplásica/complicaciones , Polineuropatía Paraneoplásica/diagnóstico , Polineuropatías/microbiología , Polineuropatías/fisiopatología , Polineuropatías , Polineuropatía Paraneoplásica/microbiología , Polineuropatía Paraneoplásica/fisiopatología , Polineuropatía ParaneoplásicaRESUMEN
Multiple nerve enlargements at non-entrapment sites are usually caused by hereditary or acquired immune-mediated neuropathies.We describe a case of multifocal hypertrophic mononeuropathies detected by nerve sonography with a clinical picture of progressive mononeuritis multiplex caused by a paraneoplastic syndrome associated with anti-Hu antibodies. This case illustrates an unusual but important paraneoplastic differential diagnosis of progressive multifocal hypertrophic neuropathies. It emphasizes the role of nerve ultrasound in the diagnostic work-up of peripheral nervous system disorders.
Asunto(s)
Polineuropatía Paraneoplásica/diagnóstico por imagen , Polineuropatía Paraneoplásica/fisiopatología , Humanos , Masculino , Persona de Mediana Edad , Polineuropatía Paraneoplásica/diagnóstico , UltrasonografíaRESUMEN
OBJECTIVE: We studied a case series of peripheral nerve hyperexcitability (PNH) aiming to describe clinical characteristics, immunologic and cancer associations, antibodies against neuronal antigens (voltage-gated potassium channel antibodies [VGKC-Abs] and other), and muscle biopsy findings. METHODS: Patients presenting with clinical and electrophysiologic signs of PNH were selected. We studied clinical and electrophysiologic features; a panel of non-neuronal organ-specific antibodies, immunofluorescence on rat nervous tissues, and radioimmunoprecipitation for VGKC-Abs; and muscle biopsies. RESULTS: Thirty-eight patients were included. After the exclusion of 6 cases with axonopathy of known origin, patients were subdivided according to the presence of electrophysiologic findings of motor axonopathy and association with cancer: axonopathic-PNH (group A: 12 patients), isolated nonparaneoplastic PNH (group B: 16 patients), and isolated paraneoplastic PNH (3 with thymoma and myasthenia gravis, 1 with thyroid carcinoma). PNH clinical features were similar in groups A and B. We found an overall high prevalence of clinical autoimmunity (33% of group A and 63% of group B) and systemic non-neuronal autoantibodies (42% of group A and 75% of group B). However, VGKC-Abs were only positive in 2 patients of group B. Ten patients underwent muscle biopsy, which showed inflammatory changes in 2 cases and nonspecific myopathic features in 8. CONCLUSIONS: PNH is a heterogeneous disorder involving the peripheral nerves in patients with a high propensity for developing autoimmunity. Associated muscle diseases are frequent in the form of myositis, myasthenia gravis, or nonspecific myopathic pathologic findings. VGKC-Abs were uncommon in this series.
Asunto(s)
Anticuerpos Antinucleares/metabolismo , Músculo Esquelético/patología , Enfermedades del Sistema Nervioso Periférico/inmunología , Enfermedades del Sistema Nervioso Periférico/fisiopatología , Canales de Potasio con Entrada de Voltaje/metabolismo , Adolescente , Adulto , Anciano , Biopsia , Electrofisiología , Femenino , Técnica del Anticuerpo Fluorescente , Humanos , Inflamación , Masculino , Persona de Mediana Edad , Miastenia Gravis/inmunología , Polineuropatía Paraneoplásica/inmunología , Polineuropatía Paraneoplásica/fisiopatología , Enfermedades del Sistema Nervioso Periférico/patología , Ensayo de Radioinmunoprecipitación , Factores de Riesgo , Timoma/inmunología , Neoplasias de la Tiroides/inmunología , Adulto JovenRESUMEN
Acquired sensory neuronopathies encompass a group of paraneoplastic, dysimmune, toxic or idiopathic disorders characterized by degeneration of peripheral sensory neurons in dorsal root ganglia. As dorsal root ganglia cannot easily be explored, the clinical diagnosis of these disorders may be difficult. The question as to whether there exists a common clinical pattern of sensory neuronopathies, allowing the establishment of validated and easy-to-use diagnostic criteria, has not yet been addressed. In this study, logistic regression was used to construct diagnostic criteria on a retrospective study population of 78 patients with sensory neuronopathies and 56 with other sensory neuropathies. For this, sensory neuronopathy was provisionally considered as unambiguous in 44 patients with paraneoplastic disorder or cisplatin treatment and likely in 34 with a dysimmune or idiopathic setting who may theoretically have another form of neuropathy. To test the homogeneity of the sensory neuronopathy population, likely candidates were compared with unambiguous cases and then the whole population was compared with the other sensory neuropathies population. Criteria accuracy was checked on 37 prospective patients referred for diagnosis of sensory neuropathy. In the study population, sensory neuronopathy showed a common clinical and electrophysiological pattern that was independent of the underlying cause, including unusual forms with only patchy sensory loss, mild electrical motor nerve abnormalities and predominant small fibre or isolated lower limb involvement. Logistic regression allowed the construction of a set of criteria that gave fair results with the following combination: ataxia in the lower or upper limbs + asymmetrical distribution + sensory loss not restricted to the lower limbs + at least one sensory action potential absent or three sensory action potentials <30% of the lower limit of normal in the upper limbs + less than two nerves with abnormal motor nerve conduction study in the lower limbs.
Asunto(s)
Enfermedades del Sistema Nervioso Periférico/diagnóstico , Células Receptoras Sensoriales/fisiología , Potenciales de Acción , Antineoplásicos/efectos adversos , Estudios de Casos y Controles , Cisplatino/efectos adversos , Diagnóstico Diferencial , Femenino , Humanos , Masculino , Persona de Mediana Edad , Conducción Nerviosa , Polineuropatía Paraneoplásica/diagnóstico , Polineuropatía Paraneoplásica/fisiopatología , Enfermedades del Sistema Nervioso Periférico/inducido químicamente , Enfermedades del Sistema Nervioso Periférico/fisiopatología , Estudios ProspectivosRESUMEN
A 53-year-old man was referred to our hospital for dry cough and a mass in the hilum of the right lung on chest CT which was diagnosed as small cell lung cancer by bronchofiberscopy (T3N2M0, stage IIIB). Also, he was aware of progressive muscle weakness in the lower extremities on the first consultation. An electromyogram showed neuropathic changes and did not show waxing phenomenon in response to high frequency repetitive stimulation. Sensory nerve conduction velocity was low, so we diagnosed small cell cancer associated with paraneoplastic sensory neuropathy. Serum antineuronal antibodies were negative. His neurological symptoms improved dramatically after chemoradiotherapy for small cell lung cancer. A complete response was obtained by concurrent chemoradiotherapy and prophylactic cranial irradiation was administrated. He is alive without recurrence at 11 months after the treatment.
Asunto(s)
Neoplasias Pulmonares/complicaciones , Neoplasias Pulmonares/terapia , Polineuropatía Paraneoplásica/fisiopatología , Carcinoma Pulmonar de Células Pequeñas/complicaciones , Carcinoma Pulmonar de Células Pequeñas/terapia , Terapia Combinada , Humanos , Masculino , Persona de Mediana EdadRESUMEN
BACKGROUND: Paraneoplastic neurological syndromes (PNS) are mainly associated with small-cell lung cancer, gynaecological tumours and lymphomas. Few studies report the association of neurological syndromes with a carcinoid, the majority being a serotonin-related myopathy. We report four patients with a PNS associated with carcinoid. PATIENTS AND RESULTS: The clinical syndromes were sensory neuropathy, limbic encephalitis, myelopathy and brain stem encephalitis. Two patients had antineuronal autoantibodies (one anti-Hu, one anti-Yo), one patient had antinuclear antibodies, and one patient had no autoantibodies. For two of the carcinoids, expression of HuD in the tumour could be demonstrated. CONCLUSION: This study demonstrates that carcinoids can also be associated with classical antineuronal antibody-associated PNS.
Asunto(s)
Tumor Carcinoide/complicaciones , Síndromes Paraneoplásicos/etiología , Anciano , Autoanticuerpos/inmunología , Biomarcadores/análisis , Biomarcadores/metabolismo , Tumor Carcinoide/patología , Tumor Carcinoide/fisiopatología , Proteínas ELAV/inmunología , Encefalitis/etiología , Encefalitis/patología , Encefalitis/fisiopatología , Femenino , Humanos , Encefalitis Límbica/patología , Encefalitis Límbica/fisiopatología , Masculino , Persona de Mediana Edad , Proteínas del Tejido Nervioso/inmunología , Polineuropatía Paraneoplásica/patología , Polineuropatía Paraneoplásica/fisiopatología , Síndromes Paraneoplásicos/patología , Síndromes Paraneoplásicos/fisiopatología , Enfermedades de la Médula Espinal/etiología , Enfermedades de la Médula Espinal/patología , Enfermedades de la Médula Espinal/fisiopatología , Adulto JovenRESUMEN
Paraneoplastic myeloneuropathy has rarely been reported with breast cancer. We report the case of a 59-year-old woman who presented with a peripheral neuropathy and cranial involvement and later developed a myelopathy. The neuropathy was found to be electrophysiologically and histologically demyelinating in nature. Magnetic resonance imaging studies failed to identify any structural brain or spinal cord abnormalities. The patient was diagnosed with breast carcinoma 4 months after initial presentation and underwent resective surgery, radiotherapy, and hormonotherapy. Paraneoplastic antibodies (anti-Hu, anti-Yo, anti-Ri, anti-CV2, anti-Ma, and anti-amphiphysin) were all negative. Her condition did not progress further after cancer treatment. Partial neurologic improvement occurred with oral steroid therapy, with subsequent deterioration on treatment withdrawal.
Asunto(s)
Neoplasias de la Mama/complicaciones , Carcinoma/complicaciones , Polineuropatía Paraneoplásica/diagnóstico , Polirradiculoneuropatía/diagnóstico , Enfermedades de la Médula Espinal/diagnóstico , Esteroides/uso terapéutico , Antiinflamatorios/uso terapéutico , Neoplasias de la Mama/inmunología , Carcinoma/inmunología , Progresión de la Enfermedad , Femenino , Humanos , Inmunoglobulinas Intravenosas/uso terapéutico , Persona de Mediana Edad , Debilidad Muscular/inmunología , Debilidad Muscular/fisiopatología , Polineuropatía Paraneoplásica/tratamiento farmacológico , Polineuropatía Paraneoplásica/fisiopatología , Paresia/inmunología , Paresia/fisiopatología , Nervios Periféricos/patología , Nervios Periféricos/fisiopatología , Polirradiculoneuropatía/tratamiento farmacológico , Polirradiculoneuropatía/fisiopatología , Prednisolona/uso terapéutico , Trastornos de la Sensación/inmunología , Trastornos de la Sensación/fisiopatología , Enfermedades de la Médula Espinal/tratamiento farmacológico , Enfermedades de la Médula Espinal/fisiopatología , Resultado del TratamientoAsunto(s)
Anticuerpos/metabolismo , Proteínas ELAV/inmunología , Polineuropatía Paraneoplásica/diagnóstico , Polineuropatía Paraneoplásica/inmunología , Diagnóstico Diferencial , Electromiografía/métodos , Femenino , Humanos , Persona de Mediana Edad , Polineuropatía Paraneoplásica/fisiopatología , Polineuropatía Paraneoplásica/terapiaRESUMEN
OBJECTIVE: To characterize the clinicopathologic features of ataxic and painful forms of paraneoplastic neuropathy. METHODS: Clinical, electrophysiologic, and histopathologic findings were assessed in 17 patients with paraneoplastic neuropathy. RESULTS: Clinical features can be categorized into two groups: one group (13 patients) with predominantly deep sensory disturbance and a second group (4 patients) with predominantly superficial sensory disturbance. The former group showed severe sensory ataxia and predominantly large myelinated fiber loss in the sural nerve. The latter group showed marked pain, in particular, severe mechanical hyperalgesia, and predominantly small myelinated and unmyelinated fiber loss. Nerve conduction assessment indicated an axonal neuropathy pattern in both groups, while sensory action potentials were more markedly diminished in the sensory ataxic form. Anti-Hu antibodies were detected in half of the patients in both groups. Treatment for cancer was effective to improve or stabilize neuropathic symptoms in some cases from both groups. Immunotherapy was effective only for a short time. CONCLUSIONS: Paraneoplastic neuropathy can be characterized into two groups by the presence of sensory ataxia or severe spontaneous pain and severe mechanical hyperalgesia. Preferential small myelinated and unmyelinated fiber loss correlated to the cases of severe pain.
Asunto(s)
Ataxia de la Marcha/etiología , Neuralgia/etiología , Degeneración Cerebelosa Paraneoplásica/etiología , Polineuropatía Paraneoplásica/clasificación , Potenciales de Acción , Anciano , Anticuerpos Antineoplásicos/inmunología , Antineoplásicos/uso terapéutico , Autoanticuerpos/inmunología , Autoantígenos/inmunología , Biopsia , Femenino , Humanos , Hipoestesia/etiología , Hipoestesia/patología , Neoplasias Pulmonares/complicaciones , Masculino , Persona de Mediana Edad , Proteínas de Neoplasias/inmunología , Neoplasias/complicaciones , Neoplasias/diagnóstico , Neoplasias/tratamiento farmacológico , Degeneración Nerviosa/etiología , Degeneración Nerviosa/patología , Fibras Nerviosas Mielínicas/patología , Fibras Nerviosas Amielínicas/patología , Proteínas del Tejido Nervioso/inmunología , Conducción Nerviosa , Degeneración Cerebelosa Paraneoplásica/inmunología , Degeneración Cerebelosa Paraneoplásica/fisiopatología , Polineuropatía Paraneoplásica/complicaciones , Polineuropatía Paraneoplásica/inmunología , Polineuropatía Paraneoplásica/fisiopatología , Reflejo Anormal , Trastornos de la Sensación/etiología , Trastornos de la Sensación/patología , Nervio Sural/patología , Factores de TiempoRESUMEN
Sensory ganglionopathies have a frequent association with neoplastic disorders (paraneoplastic subacute sensory neuronopathy, or SSN) or dysimmune disorders, with drugs, such as cisplatin or pyridoxine, and with inherited disorders with degeneration of dorsal root ganglion cells. Unsteady gait and pseudoathetoid movements of the hand are the distinctive signs encountered in these disorders. The chronic disorders are characterized by non-length-dependent abnormalities of sensory nerve action potentials (SNAPs) and differ from other sensory neuropathies in showing a global, rather than distal, decrease in SNAP amplitudes. This review focuses on recent advances in defining the mechanisms involved in sensory ganglionopathies, and describes the differential diagnosis including the rarely encountered hereditary neuronopathies and the infectious causes.
Asunto(s)
Ganglios Sensoriales/fisiopatología , Polineuropatía Paraneoplásica/fisiopatología , Enfermedades del Sistema Nervioso Periférico/fisiopatología , Potenciales de Acción , Encéfalo/patología , Diagnóstico Diferencial , Encefalomielitis/diagnóstico , Encefalomielitis/patología , Encefalomielitis/fisiopatología , Humanos , Enfermedad Iatrogénica , Imagen por Resonancia Magnética , Neuronas Aferentes/fisiología , Polineuropatía Paraneoplásica/diagnóstico , Enfermedades del Sistema Nervioso Periférico/terapiaAsunto(s)
Autoanticuerpos/metabolismo , Mejilla/fisiopatología , Proteínas ELAV/inmunología , Enfermedades Musculares/fisiopatología , Polineuropatía Paraneoplásica , Humanos , Masculino , Persona de Mediana Edad , Polineuropatía Paraneoplásica/inmunología , Polineuropatía Paraneoplásica/metabolismo , Polineuropatía Paraneoplásica/fisiopatología , SíndromeRESUMEN
BACKGROUND: Paraneoplastic peripheral neuropathy is sometimes associated with unidentified neuronal autoantibodies. OBJECTIVE: To examine the effects of serum from a patient with subacute sensory axonopathy on the function of the Trk high-affinity nerve growth factor receptor. PATIENT: An 86-year-old man with sensory neuropathy exhibiting an autoantibody to Trk. METHODS: Immunoblot analyses of the brain homogenates and immunoprecipitation were performed with human sera. We further examined the effect of sera on nerve growth factor-induced neurite outgrowth and Trk autophosphorylation. RESULTS: The patient showed sensory nerve axonopathy without well-known paraneoplastic autoantibodies. His serum inhibited nerve growth factor-induced neurite outgrowth and Trk autophosphorylation in PCtrk cells. Moreover, the patient's serum, but not control serum, immunoprecipitated Trk and recognized Trk in brain homogenates as well as in Trk immunoprecipitates. CONCLUSION: These data strongly suggest that an anti-Trk autoantibody might cause subacute sensory neuropathy.
Asunto(s)
Autoanticuerpos/sangre , Polineuropatía Paraneoplásica/inmunología , Receptor trkA/inmunología , Anciano , Anciano de 80 o más Años , Autoanticuerpos/inmunología , Western Blotting , Encéfalo/metabolismo , Células Cultivadas , Humanos , Linfoma no Hodgkin/complicaciones , Masculino , Neuronas/metabolismo , Polineuropatía Paraneoplásica/fisiopatologíaRESUMEN
We report a paraneoplastic neuropathy with severe motor involvement following sensory-ataxic disturbance. Anti-disialosyl immunoglobulin M (IgM) antibody was detected in the course of malignant lymphoma of diffuse large B-cell type, which usually spares the motor system. Onset was subacute, with relapsing and remitting sensory ataxia, muscle weakness, bulbar palsy, respiratory paralysis, and ophthalmoplegia; only neck rotation was retained in the terminal stage. Autopsy showed no lymphoma cells infiltrating the nervous system. Motor neurons survived in the spinal cord, but mean diameter of the ventral spinal nerve roots was reduced considerably. The gracile fasciculus and the sural nerve were more markedly degenerated than proximal portions. Morphometric study showed that most of the proximal motor and sensory axons did not extend distally. This autopsy report provides further definition of a neuropathy associated with malignant lymphoma and IgM antibodies against disialosyl residues.