RESUMEN
Bilateral facial palsy with paresthesia (FDP) is a rare variant of GBS, characterized by simultaneous bilateral facial palsy and paresthesia of the distal limbs. Mounting evidence indicates that the presence of anti-GT1a IgG has a pathogenic role as an effector molecule in the development of cranial nerve palsies in certain patients with GBS, whereas anti-GT1a antibody is rarely presented positive in FDP. Here, we report the case of a 33-year-old male diagnosed with FDP presented with acute onset of bilateral facial palsy and slight paresthesias at the feet as the only neurological manifestation. An antecedent infection with no identifiable reason for the fever or skin eruptions was noted in the patient. He also exhibited cerebrospinal fluid albuminocytologic dissociation and abnormal nerve conduction studies. Notably, the testing of specific serum anti-gangliosides showed positive anti-GT1a IgG/IgM Ab. The patient responded well to intravenous immunoglobulin therapy. This case brings awareness to a rare variant of GBS, and provides the first indication that anti-GT1a antibodies play a causative role in the development of FDP. The case also suggests that prompt management with IVIG should be implemented if FDP is diagnosed.
Asunto(s)
Autoanticuerpos , Parálisis Facial , Gangliósidos , Parestesia , Humanos , Masculino , Adulto , Parestesia/inmunología , Parestesia/diagnóstico , Parestesia/etiología , Parálisis Facial/diagnóstico , Parálisis Facial/etiología , Parálisis Facial/inmunología , Autoanticuerpos/inmunología , Autoanticuerpos/sangre , Gangliósidos/inmunología , Inmunoglobulinas Intravenosas/uso terapéutico , Inmunoglobulina G/inmunología , Inmunoglobulina G/sangre , Síndrome de Guillain-Barré/diagnóstico , Síndrome de Guillain-Barré/inmunologíaRESUMEN
RATIONALE: Systemic lupus erythematosus (SLE) is a complex autoimmune inflammatory disease that frequently affects various organs. Neuropsychiatric manifestations in SLE patients, known as neuropsychiatric SLE, are clinically common. However, the principal manifestation of cranial neuropathy in patients with SLE and comorbidities is relatively rare. PATIENT CONCERNS: In this report, we describe a 51-year-old Chinese woman who was admitted with a chief complaint of chronic-onset facial paresthesia, dysphagia, and choking cough when drinking water, accompanied by slurred speech, salivation, and limb weakness. The blood autoantibody test results showed that many SLE-associated antibodies were positive. Meanwhile, anti-nuclear matrix protein 2 (NXP2) antibody was strongly positive in the idiopathic inflammatory myopathy (IIM) spectrum test from the serum. Muscle biopsy indicated inflammatory infiltration of the muscle fiber stroma. DIAGNOSES: Taking into account the clinical manifestations and laboratory tests of the present case, the diagnosis of SLE and probable IIM was established. INTERVENTIONS: Corticosteroids and additional gamma globulin were administered and the clinical symptoms were relieved during the treatment process. OUTCOMES: Unfortunately, the patient experienced sudden cardiac and respiratory arrest. Multiple system dysfunctions exacerbated disease progression, but in the present case, we speculated that myocardial damage resulting from SLE could explain why she suddenly died. LESSONS: To our knowledge, multiple neurological manifestations in patients with SLE and anti-NXP2-positive myositis are rare. Note that SLE is still a life-threatening disease that causes multiple system dysfunctions, which requires increasing attention.
Asunto(s)
Enfermedades de los Nervios Craneales/inmunología , Trastornos de Deglución/inmunología , Lupus Eritematoso Sistémico/diagnóstico , Parestesia/inmunología , Polimiositis/diagnóstico , Adenosina Trifosfatasas/inmunología , Autoanticuerpos/sangre , Autoanticuerpos/inmunología , Biopsia , Enfermedades de los Nervios Craneales/diagnóstico , Enfermedades de los Nervios Craneales/tratamiento farmacológico , Proteínas de Unión al ADN/inmunología , Trastornos de Deglución/diagnóstico , Trastornos de Deglución/tratamiento farmacológico , Quimioterapia Combinada/métodos , Resultado Fatal , Femenino , Humanos , Factores Inmunológicos/administración & dosificación , Lupus Eritematoso Sistémico/complicaciones , Lupus Eritematoso Sistémico/tratamiento farmacológico , Lupus Eritematoso Sistémico/inmunología , Persona de Mediana Edad , Músculo Esquelético/inmunología , Músculo Esquelético/patología , Parestesia/diagnóstico , Parestesia/tratamiento farmacológico , Polimiositis/complicaciones , Polimiositis/tratamiento farmacológico , Polimiositis/inmunología , Quimioterapia por PulsoRESUMEN
BACKGROUND: Human immunodeficiency virus (HIV)-associated distal symmetric peripheral neuropathy (DSPN) is one of the most frequent neurological complications of HIV infection, and causes pain and dysaesthesias in millions globally. Many individuals with this infection report using acupuncture to manage their symptoms, but evidence supporting the use of acupuncture is limited. This systematic review will assess the effectiveness and safety of acupuncture for patients with HIV-associated DSPN. METHODS: Databases including MEDLINE, EMBASE, the Cochrane Central Register of Controlled Trials, Scopus, Web of science, AMED (Allied and Complementary Medicine), the Chinese Biomedical Literature Database, the China National Knowledge Infrastructure, Wanfang Database, VIP Database and clinical trials registers (the WHO International Clinical Trials Registry Platform portal and www.ClinicalTrials.gov) will be electronically searched from inception to December 1, 2020. All randomized controlled trials in English or Chinese without restriction on publication status will be included. Selection of studies, extraction of data, and assessment of studies quality will be independently performed by 2 reviewers. The primary outcome measure will be the change in pain intensity assessed by validated scales. Secondary outcomes include change in neurologic summary scores, quality of life, physical function evaluated by admitted tools, and adverse events related to acupuncture reported in the included trials. If possible, a meta-analysis will be conducted to provide an estimate of the pooled treatment effect using Review Manager 5.3 statistical software. Otherwise, qualitative descriptive analysis will be given. The results will be presented as the risk ratio for binary data and the mean difference (MD) or standardized MD for continuous data. RESULTS: The results of the systematic review will be disseminated via publication in a peer-reviewed journal and presented at a relevant conference. CONCLUSION: This review will be the first review entirely focused on assessing the effectiveness and safety of acupuncture for HIV-associated DSPN. PROSPERO REGISTRATION NUMBER: CRD42020210994.
Asunto(s)
Terapia por Acupuntura/efectos adversos , Infecciones por VIH/complicaciones , Neuralgia/terapia , Parestesia/terapia , Polineuropatías/terapia , Infecciones por VIH/inmunología , Infecciones por VIH/virología , Humanos , Metaanálisis como Asunto , Neuralgia/diagnóstico , Neuralgia/inmunología , Neuralgia/virología , Dimensión del Dolor , Parestesia/diagnóstico , Parestesia/inmunología , Parestesia/virología , Polineuropatías/diagnóstico , Polineuropatías/inmunología , Polineuropatías/virología , Calidad de Vida , Ensayos Clínicos Controlados Aleatorios como Asunto , Índice de Severidad de la Enfermedad , Revisiones Sistemáticas como Asunto , Resultado del TratamientoAsunto(s)
Neoplasias del Mediastino/complicaciones , Neurilemoma/complicaciones , Síndromes Paraneoplásicos/etiología , Vasculitis Sistémica/etiología , Anciano , Anemia Macrocítica/tratamiento farmacológico , Anemia Macrocítica/etiología , Anemia Macrocítica/inmunología , Anticuerpos Anticardiolipina/inmunología , Complejo Antígeno-Anticuerpo/inmunología , Crioglobulinas/inmunología , Mareo/etiología , Mareo/inmunología , Fiebre/etiología , Fiebre/inmunología , Glucocorticoides/uso terapéutico , Humanos , Inmunosupresores/uso terapéutico , Inhibidor de Coagulación del Lupus/inmunología , Masculino , Neoplasias del Mediastino/cirugía , Neurilemoma/cirugía , Síndromes Paraneoplásicos/tratamiento farmacológico , Síndromes Paraneoplásicos/inmunología , Síndromes Paraneoplásicos del Sistema Nervioso/tratamiento farmacológico , Síndromes Paraneoplásicos del Sistema Nervioso/etiología , Síndromes Paraneoplásicos del Sistema Nervioso/inmunología , Parestesia/etiología , Parestesia/inmunología , Neuropatías Peroneas/etiología , Neuropatías Peroneas/inmunología , Fibrosis Pulmonar/etiología , Fibrosis Pulmonar/inmunología , Factor Reumatoide/inmunología , Vasculitis Sistémica/tratamiento farmacológico , Vasculitis Sistémica/inmunologíaRESUMEN
OBJECTIVE: To explore the diversity and clinical features of anti-glutamate decarboxylase (GAD) antibody-associated neurological diseases. METHODS: Clinical data of a series of 5 patients positive for anti-GAD antibodies were retrospectively analyzed. RESULTS: All 5 patients were female, with a median age of 41.5 years (range 19-60 years). Their neurological symptoms included stiff-person syndrome (SPS), encephalitis, myelitis, cramp, visual loss, and paresthesia. Three patients (60%) were diagnosed with tumors, 2 cases of thymic tumor and 1 of breast cancer. On immunohistochemistry for tumor pathology, expression of GAD65 was found only in 1 patient. Four patients (80%) had abnormal brain MRI findings. All patients received immunotherapy and improved significantly after treatment, but 4 (80%) then experienced a relapse. CONCLUSIONS: Neurological manifestations in anti-GAD-positive patients are diverse and include SPS, encephalitis, myelitis, cramp, visual loss, and paresthesia.
Asunto(s)
Autoanticuerpos/inmunología , Enfermedades Autoinmunes del Sistema Nervioso/fisiopatología , Glutamato Descarboxilasa/inmunología , Síndromes Paraneoplásicos del Sistema Nervioso/fisiopatología , Adulto , Enfermedades Autoinmunes del Sistema Nervioso/diagnóstico por imagen , Enfermedades Autoinmunes del Sistema Nervioso/inmunología , Encéfalo/diagnóstico por imagen , Neoplasias de la Mama/metabolismo , Encefalitis/diagnóstico por imagen , Encefalitis/inmunología , Encefalitis/fisiopatología , Femenino , Glutamato Descarboxilasa/metabolismo , Humanos , Imagen por Resonancia Magnética , Persona de Mediana Edad , Calambre Muscular/inmunología , Calambre Muscular/fisiopatología , Mielitis/inmunología , Mielitis/fisiopatología , Síndromes Paraneoplásicos del Sistema Nervioso/diagnóstico por imagen , Síndromes Paraneoplásicos del Sistema Nervioso/inmunología , Parestesia/inmunología , Parestesia/fisiopatología , Recurrencia , Estudios Retrospectivos , Síndrome de la Persona Rígida/inmunología , Síndrome de la Persona Rígida/fisiopatología , Neoplasias del Timo/metabolismo , Trastornos de la Visión/inmunología , Trastornos de la Visión/fisiopatología , Adulto JovenAsunto(s)
Hemorragia Cerebral/inmunología , Hipersensibilidad Tardía/inmunología , Níquel/inmunología , Parestesia/inmunología , Stents/efectos adversos , Biopsia , Enfermedades de las Arterias Carótidas/cirugía , Arteria Carótida Interna/diagnóstico por imagen , Arteria Carótida Interna/cirugía , Hemorragia Cerebral/diagnóstico , Femenino , Humanos , Hipersensibilidad Tardía/diagnóstico , Aneurisma Intracraneal/cirugía , Imagen por Resonancia Magnética , Persona de Mediana Edad , Parestesia/diagnóstico , Pruebas del Parche , Piel/inmunología , Piel/patologíaRESUMEN
We herein report the case of a 19-year-old woman with facial diplegia and paresthesias (FDP) preceded by flu-like symptoms. We diagnosed the patient with a regional variant of Guillain-Barré syndrome due to decreased tendon reflexes, albuminocytological dissociation in the cerebrospinal fluid and demyelinating features on nerve conduction studies. The patient also had IgM anti-GalNAc-GD1a antibodies, and treatment with glucocorticoids was effective for treating the facial diplegia, but not paresthesia. Therefore, facial palsy may have a different pathophysiology from paresthesia or other symptoms of FDP, which responds to glucocorticoid therapy.
Asunto(s)
Autoanticuerpos/sangre , Parálisis Facial/etiología , Gangliósidos/inmunología , Síndrome de Guillain-Barré/diagnóstico , Síndrome de Guillain-Barré/tratamiento farmacológico , Inmunoglobulina M/sangre , Parestesia/etiología , Aciclovir/administración & dosificación , Aciclovir/análogos & derivados , Adulto , Antivirales/administración & dosificación , Parálisis Facial/tratamiento farmacológico , Parálisis Facial/inmunología , Femenino , Glucocorticoides/administración & dosificación , Síndrome de Guillain-Barré/inmunología , Síndrome de Guillain-Barré/fisiopatología , Humanos , Metilprednisolona/administración & dosificación , Parestesia/tratamiento farmacológico , Parestesia/inmunología , Recuperación de la Función , Resultado del Tratamiento , Valaciclovir , Valina/administración & dosificación , Valina/análogos & derivadosRESUMEN
Previous studies showed comorbidity of some ocular, enteral, and affective symptoms comprising irritable eye syndrome. Aims of the present study were to learn more about the pathogenic mechanisms of this syndrome and to evaluate benefits of food supplements on these disorders. In in vitro assay, Lactobacillus acidophilus lysate inhibited interleukin (IL)-1ß and tumor necrosis factor (TNF)-α generation of lipopolysaccharide (LPS)-stimulated macrophages in dose- and size-dependent manner. For a prospective, open-label phase I/II controlled clinical trial, 40 subjects affected by ocular dysesthesia and hyperesthesia and comorbid enteral and anxiety-depression symptoms were randomly assigned either into the treated group, which received a composition containing probiotic lysate, vitamins A, B, and D and omega 3 fatty acids, or into the control group, which received vitamins and omega 3 fatty acids. For reference, 20 age- and sex-matched healthy subjects were also selected. White blood count (WBC) and lymphocyte and monocyte counts, as well as IL-6 and TNF-α levels, were significantly above the reference levels in both treated and control groups. After 8 weeks, WBC and lymphocyte and monocyte counts, and cytokine levels significantly decreased, and ocular, enteral, and anxiety-depression symptoms significantly improved in the treated group as compared to the control group. This proof-of-concept study suggested that subclinical inflammation may be a common mechanism connecting ocular, enteral, and anxiety/depression symptoms, and supplements affecting dysbiosis may be a new approach to treating this syndrome.
Asunto(s)
Ácidos Grasos Omega-3/administración & dosificación , Queratitis/inmunología , Queratitis/terapia , Probióticos/uso terapéutico , Vitaminas/administración & dosificación , Adulto , Animales , Aceite de Hígado de Bacalao/administración & dosificación , Estreñimiento/complicaciones , Depresión/complicaciones , Diarrea/complicaciones , Femenino , Humanos , Interleucina-1beta/inmunología , Interleucina-6/inmunología , Queratitis/complicaciones , Lactobacillus acidophilus , Macrófagos/inmunología , Masculino , Ratones Endogámicos , Persona de Mediana Edad , Neuroinmunomodulación/inmunología , Parestesia/inmunología , Parestesia/terapia , Estudios Prospectivos , Factor de Necrosis Tumoral alfa/inmunologíaRESUMEN
A 52-year-old man with chronic hepatitis C presented with painless, bilateral, simultaneous nonarteritic anterior ischemic optic neuropathy (NAION) and peripheral neuropathy. Symptoms began 19 weeks after starting peginterferon alpha-2a. The peripheral neuropathy and vision of the right eye improved, but the vision of the left eye worsened after stopping interferon. We identified 23 additional cases of NAION during interferon alpha therapy. At least 12 of these patients suffered bilateral NAION. Patients lost vision 1-40 weeks after initiating therapy. Of 21 eyes that had documented initial and follow-up acuities, 8 improved, 1 worsened, and the rest remained stable. One patient had a painful peripheral neuropathy. Treatment with interferon alpha may result in NAION. Discontinuation of therapy deserves consideration after weighing individual risks and benefits.
Asunto(s)
Interferón-alfa/efectos adversos , Nervio Óptico/efectos de los fármacos , Neuropatía Óptica Isquémica/inducido químicamente , Nervios Periféricos/efectos de los fármacos , Enfermedades del Sistema Nervioso Periférico/inducido químicamente , Polietilenglicoles/efectos adversos , Antivirales/efectos adversos , Atrofia/inducido químicamente , Atrofia/inmunología , Atrofia/fisiopatología , Ceguera/inducido químicamente , Hepatitis C/tratamiento farmacológico , Hepatitis C/inmunología , Hepatitis C/virología , Humanos , Enfermedad Iatrogénica/prevención & control , Interferón alfa-2 , Imagen por Resonancia Magnética , Masculino , Persona de Mediana Edad , Nervio Óptico/inmunología , Nervio Óptico/fisiopatología , Neuropatía Óptica Isquémica/inmunología , Neuropatía Óptica Isquémica/fisiopatología , Parestesia/inducido químicamente , Parestesia/inmunología , Parestesia/fisiopatología , Nervios Periféricos/inmunología , Nervios Periféricos/fisiopatología , Enfermedades del Sistema Nervioso Periférico/inmunología , Enfermedades del Sistema Nervioso Periférico/fisiopatología , Proteínas Recombinantes , Ribavirina/uso terapéutico , Campos Visuales/efectos de los fármacos , Campos Visuales/fisiologíaRESUMEN
Hereditary angioedema (HAE) is a rare disease caused by deficiency in the production or function of C1 inhibitor. It predisposes individuals to paroxysmal acute attacks causing painful, debilitating, disfiguring, and life-threatening angioedema. Prodromes occurring hours to days before attacks have been described in the literature; however, their significance as predictive signals of impending attacks is uncertain. Given the morbidity and mortality associated with HAE attacks and the increasing availability of therapeutic products for their treatment, identifying prodromes that accurately predict the onset of attacks could provide the basis for the development of a validated instrument to identify the onset of such attacks requiring abortive therapeutic intervention before the development of clinically significant angioedema. The aim of this study was to review the literature to identify the prodromes reported to occur with HAE attacks. A literature review of English language journal articles was performed using search terms hereditary angioedema, HAE, angioneurotic edema, prodrome, signs, and symptoms. Nineteen original English language articles that included both case reports and studies describing prodromes associated with HAE attacks were obtained. Our review indicates that there is significant variability in the expression, manifestation, prevalence, timing, and predictive reliability of the prodromes that have been described. There is considerable variability of the prodromal manifestations that may occur before or during HAE attacks. We have not found any evidence that their sensitivity and specificity for accurately predicting such attacks has been studied.
Asunto(s)
Angioedemas Hereditarios/diagnóstico , Angioedemas Hereditarios/fisiopatología , Proteínas Inactivadoras del Complemento 1/metabolismo , Angioedemas Hereditarios/genética , Angioedemas Hereditarios/inmunología , Bradiquinina/inmunología , Bradiquinina/metabolismo , Proteínas Inactivadoras del Complemento 1/genética , Proteínas Inactivadoras del Complemento 1/inmunología , Proteína Inhibidora del Complemento C1 , Tracto Gastrointestinal/inmunología , Tracto Gastrointestinal/patología , Humanos , Parestesia/etiología , Parestesia/inmunología , Parestesia/patología , Piel/inmunología , Piel/patologíaAsunto(s)
Neuropatías del Plexo Braquial/diagnóstico , Plexo Braquial/patología , Hipoestesia/diagnóstico , Procesamiento de Imagen Asistido por Computador , Imagen por Resonancia Magnética , Enfermedad de la Neurona Motora/diagnóstico , Debilidad Muscular/diagnóstico , Parestesia/diagnóstico , Polirradiculoneuropatía Crónica Inflamatoria Desmielinizante/diagnóstico , Neuropatías del Plexo Braquial/inmunología , Diagnóstico Diferencial , Femenino , Gangliósidos/inmunología , Humanos , Hipertrofia , Hipoestesia/inmunología , Masculino , Persona de Mediana Edad , Enfermedad de la Neurona Motora/inmunología , Debilidad Muscular/inmunología , Parestesia/inmunología , Polirradiculoneuropatía Crónica Inflamatoria Desmielinizante/inmunologíaAsunto(s)
Autoanticuerpos/sangre , Enfermedades Autoinmunes/inmunología , Gangliósidos/inmunología , Inmunoglobulina M/sangre , Enfermedad de la Neurona Motora/inmunología , Conducción Nerviosa/fisiología , Neuritis/inmunología , Oftalmoplejía/inmunología , Parestesia/inmunología , Adulto , Anciano , Enfermedades Autoinmunes/diagnóstico , Progresión de la Enfermedad , Femenino , Estudios de Seguimiento , Ataxia de la Marcha/diagnóstico , Ataxia de la Marcha/inmunología , Humanos , Masculino , Persona de Mediana Edad , Enfermedad de la Neurona Motora/diagnóstico , Neuritis/diagnóstico , Examen Neurológico , Oftalmoplejía/diagnóstico , Parestesia/diagnósticoRESUMEN
Undiagnosed chronic Lyme disease caused by Borrelia burgdorferi is considered a differential diagnoses in medically unexplained symptoms like arthralgias, distal paresthesias, depressive symptoms, lack of concentration and fatigue. The aims of the study were to assess the association of mental and physical complaints with seropositivity for anti-Borrelia IgG in a general population sample. Seropositivity indicated an infection with Borrelia in the past. The Study of Health in Pomerania was conducted in a community living in a region with endemic Lyme disease. Mental and physical complaints were assessed on 38 items with the von Zerssen's complaint scale. IgG antibodies to Borrelia were determined by ELISA in 4264 individuals. Seropositivity was analyzed applying two cut-off scores (>5 and >10 IU/ml). IgG antibodies to Borrelia were found positive in 388 subjects (9.1%) applying the >5 IU/ml cut-off and in 130 subjects (3.0%) applying the >10 IU/ml cut-off. In multivariate analyses (MANCOVA), both definitions of seropositivity were not associated with increased mental or physical complaints while adjusting for gender, age, employment status, rural residency, physical activity, diabetes mellitus and number of chronic diseases. In the general population, seropositivity for anti-Borrelia IgG antibodies was not associated with an increase of self-rated mental or physical complaints or impairments. Therefore, clinicians should not overvalue seropositivity for anti-Borrelia IgG as a medical cause for unexplained mental or physical complaints.
Asunto(s)
Anticuerpos Antibacterianos/sangre , Borrelia burgdorferi/inmunología , Estado de Salud , Inmunoglobulina G/sangre , Enfermedad de Lyme/epidemiología , Enfermedad de Lyme/inmunología , Trastornos Mentales/epidemiología , Adulto , Anciano , Causalidad , Trastornos del Conocimiento/epidemiología , Trastornos del Conocimiento/inmunología , Trastornos del Conocimiento/psicología , Comorbilidad , Estudios Transversales , Ensayo de Inmunoadsorción Enzimática , Fatiga/epidemiología , Fatiga/inmunología , Fatiga/psicología , Femenino , Alemania/epidemiología , Humanos , Enfermedad de Lyme/diagnóstico , Masculino , Trastornos Mentales/inmunología , Trastornos Mentales/psicología , Persona de Mediana Edad , Trastornos del Humor/epidemiología , Trastornos del Humor/inmunología , Trastornos del Humor/psicología , Dolor/epidemiología , Dolor/inmunología , Dolor/psicología , Parestesia/epidemiología , Parestesia/inmunología , Parestesia/psicología , Psicometría/métodos , Psicometría/estadística & datos numéricos , Pruebas Serológicas , Trastornos del Sueño-Vigilia/epidemiología , Trastornos del Sueño-Vigilia/inmunología , Trastornos del Sueño-Vigilia/psicología , Adulto JovenRESUMEN
Sensory neuronopathy in association with connective tissue disease is a disabling disorder for which there is no well-established therapy. Various immunosuppressive agents, plasmapheresis, and intravenous immunoglobulin have shown only anecdotal or modest beneficial effects. Tumor necrosis factor alpha is a proinflammatory cytokine that mediates TH1-cell inflammatory responses and is a plausible contributor to dorsal root ganglion injury in sensory neuronopathy. We describe a patient with severe painful and ataxic sensory neuronopathy in association with systemic lupus erythematosus, who showed marked and sustained improvement on etanercept, a tumor necrosis factor alpha inhibitor, despite a chronic and progressive course that was refractory to several immunomodulatory interventions. We review the therapeutic potential of tumor necrosis factor alpha blockade in immune-mediated neuropathies and the reported neurologic complications from its use, most notably central and peripheral demyelination.
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Ataxia/tratamiento farmacológico , Atetosis/tratamiento farmacológico , Inmunoglobulina G/uso terapéutico , Factores Inmunológicos/uso terapéutico , Lupus Eritematoso Sistémico/complicaciones , Parestesia/tratamiento farmacológico , Receptores del Factor de Necrosis Tumoral/uso terapéutico , Adulto , Ataxia/etiología , Ataxia/inmunología , Ataxia/patología , Atetosis/complicaciones , Atetosis/inmunología , Atetosis/patología , Enfermedad Crónica , Electromiografía , Etanercept , Femenino , Humanos , Lupus Eritematoso Sistémico/inmunología , Conducción Nerviosa , Neuronas Aferentes/inmunología , Neuronas Aferentes/patología , Parestesia/etiología , Parestesia/inmunología , Parestesia/patología , Enfermedades del Sistema Nervioso Periférico/complicaciones , Enfermedades del Sistema Nervioso Periférico/tratamiento farmacológico , Enfermedades del Sistema Nervioso Periférico/inmunología , Enfermedades del Sistema Nervioso Periférico/patología , Proteínas Recombinantes de Fusión/uso terapéutico , Resultado del TratamientoRESUMEN
A case of Graves' disease with white matter abnormalities is presented here. The diagnosis as Graves' disease was made when the patient was 5 years old, and a subtotal thyroidectomy was performed when she was 10. Her neurological symptoms began at age 19 with paresthesia of her legs and lower body. Cranial magnetic resonance imaging was normal; thoracic magnetic resonance imaging revealed demyelinating lesions. Intravenous pulse steroid therapy improved her symptoms. Ten months later she described dizziness, lower body paresthesia, and ataxia. Both her cranial and thoracic magnetic resonance imagings revealed demyelinating lesions. After pulse steroid therapy, glatiramer acetate therapy was initiated with diagnosis of an autoimmune multiphasic demyelinating syndrome. Five months later, she presented with right-sided mild optic neuritis followed by rapid spontaneous remission. Antithyroglobulin antibody levels remained normal; antithyroid peroxidase antibody level was high. This presents a rare case of Graves' disease associated with multiphasic demyelinating autoimmune syndrome.
Asunto(s)
Encéfalo/patología , Enfermedades Autoinmunes Desmielinizantes SNC/patología , Enfermedad de Graves/complicaciones , Fibras Nerviosas Mielínicas/patología , Adulto , Encéfalo/inmunología , Encéfalo/fisiopatología , Enfermedades Autoinmunes Desmielinizantes SNC/inmunología , Enfermedades Autoinmunes Desmielinizantes SNC/fisiopatología , Progresión de la Enfermedad , Femenino , Acetato de Glatiramer , Humanos , Inmunosupresores , Imagen por Resonancia Magnética , Fibras Nerviosas Mielínicas/inmunología , Neuritis Óptica/inmunología , Neuritis Óptica/fisiopatología , Parestesia/inmunología , Parestesia/patología , Parestesia/fisiopatología , Péptidos/uso terapéutico , Médula Espinal/inmunología , Médula Espinal/patología , Médula Espinal/fisiopatología , Esteroides/uso terapéutico , Glándula Tiroides/inmunología , Glándula Tiroides/fisiopatología , Tiroidectomía , Resultado del TratamientoRESUMEN
OBJECTIVE: To quantify the accumulation of inflammatory cells in traumatic neuromas of the human lingual nerve, and to establish any correlation with the patients' reported symptoms of dysaesthesia. DESIGN: Using fluorescence immunohistochemistry, the extent of any chronic inflammatory infiltrate was quantified in human lingual neuroma specimens removed from 24 patients at the time of microsurgical nerve repair. A pan-leucocyte marker (CD45) and a specific macrophage marker (CD68) were used, and comparisons made between neuromas-in-continuity (NICs) and nerve-end neuromas (NENs) in patients with or without symptoms of dysaesthesia. RESULTS: CD68 and CD45 labelling was significantly associated with areas of viable nerve tissue in neuromas and the CD68 labelling was significantly higher in NICs than NENs. CD68 labelling density tended to decrease with increasing time after the initial nerve injury, but this correlation was only significant for labelling associated with viable nerve tissue in NENs. No significant difference was found between the level of CD68 or CD45 labelling in patients with or without symptoms of dysaesthesia. CONCLUSION: This study has demonstrated the presence of inflammatory cells within traumatic neuromas of the human lingual nerve. These cells were found to be closely associated with regions of viable nerve tissue, but there was no correlation with the patients' clinical symptoms.
Asunto(s)
Neoplasias de los Nervios Craneales/patología , Nervio Lingual/patología , Neuroma/patología , Adulto , Antígenos CD/inmunología , Antígenos de Diferenciación Mielomonocítica/inmunología , Neoplasias de los Nervios Craneales/complicaciones , Neoplasias de los Nervios Craneales/inmunología , Femenino , Técnica del Anticuerpo Fluorescente/métodos , Humanos , Antígenos Comunes de Leucocito/inmunología , Leucocitos/inmunología , Nervio Lingual/inmunología , Traumatismos del Nervio Lingual , Macrófagos/inmunología , Masculino , Microscopía Fluorescente/métodos , Neuroma/complicaciones , Neuroma/inmunología , Parestesia/complicaciones , Parestesia/inmunología , Parestesia/patologíaRESUMEN
BACKGROUND: Celiac disease (CD) is a chronic inflammatory enteropathy resulting from sensitivity to ingested gluten. Neurologic complications are estimated to occur in 10% of affected patients, with ataxia and peripheral neuropathy being the most common problems. The incidence and clinical presentation of patients with CD-associated peripheral neuropathy have not previously been investigated. OBJECTIVE: To determine the incidence of CD in patients with neuropathy and to characterize the clinical presentation. METHODS: The records of 20 patients with neuropathy and biopsy-confirmed CD were reviewed. RESULTS: Six of the 20 patients had neuropathic symptoms alone without gastrointestinal involvement, and neuropathic symptoms preceded other CD symptoms in another 3 patients. All patients had burning, tingling, and numbness in their hands and feet, with distal sensory loss, and nine had diffuse paresthesias involving the face, trunk, or lumbosacral region. Only two had weakness. Results of electrophysiologic studies were normal or mildly abnormal in 18 (90%) of the patients. Sural nerve biopsies, obtained from three patients, revealed mild to severe axonopathy. Using the agglutination assay, 13 (65%) of the patients were positive for ganglioside antibodies. Excluding patients who were referred with the diagnosis of celiac neuropathy, CD was seen in approximately 2.5% of all neuropathy patients and in 8% of patients with neuropathy and normal electrophysiologic studies seen at our center. CONCLUSION: CD is commonly associated with sensory neuropathy and should be considered even in the absence of gastrointestinal symptoms.
Asunto(s)
Enfermedad Celíaca/complicaciones , Trastornos Neurológicos de la Marcha/etiología , Parestesia/etiología , Adulto , Anciano , Anciano de 80 o más Años , Especificidad de Anticuerpos , Biopsia , Enfermedad Celíaca/dietoterapia , Enfermedad Celíaca/inmunología , Femenino , Trastornos Neurológicos de la Marcha/inmunología , Gangliósidos/inmunología , Gliadina/inmunología , Glútenes/efectos adversos , Humanos , Inmunoglobulina A/inmunología , Masculino , Persona de Mediana Edad , Parestesia/inmunología , Estudios Retrospectivos , Nervio Sural/patología , Transglutaminasas/inmunologíaAsunto(s)
Carcinoma de Células Pequeñas/complicaciones , Ataxia Cerebelosa/etiología , Neoplasias Pulmonares/complicaciones , Mioclonía/etiología , Síndromes Paraneoplásicos/etiología , Parestesia/etiología , Autoanticuerpos/sangre , Carcinoma de Células Pequeñas/inmunología , Carcinoma de Células Pequeñas/patología , Ataxia Cerebelosa/inmunología , Cerebelo/inmunología , Femenino , Humanos , Neoplasias Pulmonares/inmunología , Neoplasias Pulmonares/patología , Persona de Mediana Edad , Mioclonía/inmunología , Estadificación de Neoplasias , Degeneración Nerviosa/inmunología , Neuronas/inmunología , Síndromes Paraneoplásicos/inmunología , Parestesia/inmunologíaRESUMEN
A patient who had a polyneuropathy compatible with a chronic inflammatory demyelinating polyneuropathy and was initially negative for anti-myelin-associated glycoprotein (MAG) antibodies developed a double monoclonal gammopathy, IgM kappa and IgM lambda, two years after the diagnosis. The IgM kappa, but not the IgM lambda, exhibited strong anti-MAG antibody activity. The late appearance of the anti-MAG immunoreactivity suggests that in patients with an initial diagnosis of chronic inflammatory demyelinating polyneuropathy, the search for anti-MAG antibodies should be repeated during the course of the neuropathy.
Asunto(s)
Autoanticuerpos/sangre , Enfermedades Desmielinizantes/inmunología , Proteínas de la Mielina/inmunología , Paraproteinemias/inmunología , Parestesia/inmunología , Enfermedades Desmielinizantes/sangre , Enfermedades Desmielinizantes/fisiopatología , Ensayo de Inmunoadsorción Enzimática , Dedos/inervación , Humanos , Inmunoglobulina M/sangre , Cadenas kappa de Inmunoglobulina/sangre , Cadenas lambda de Inmunoglobulina/sangre , Masculino , Persona de Mediana Edad , Glicoproteína Asociada a Mielina , Paraproteinemias/sangre , Paraproteinemias/complicaciones , Parestesia/sangre , Parestesia/fisiopatologíaRESUMEN
A 67-year-old woman with a sensory polyneuropathy was shown to have a serum monoclonal immunoglobulin M lambda antibody with a titer of 1:10,000 toward GD1b ganglioside. The immunoglobulin M also reacted with some other gangliosides containing disialosyl groups such as GD2, GD3, and GQ1b, but it did not react with GM1, LM1, or GD1a. The principal reactive ganglioside in human cauda equina was GD1b.