RESUMEN
Neuromuscular symptoms may develop or persist after resolution of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection. Besides residual sensorimotor symptoms associated with acute neuromuscular complications of coronavirus disease-2019 (COVID-19), such as Guillain-Barré syndrome, critical illness neuromyopathy, and rhabdomyolysis, patients may report persistent autonomic symptoms, sensory symptoms, and muscle symptoms in the absence of these acute complications, including palpitations, orthostatic dizziness and intolerance, paresthesia, myalgia, and fatigue. These symptoms may be associated with long COVID, also known as post-COVID-19 conditions or postacute sequelae of SARS-CoV-2 infection, which may significantly impact quality of life. Managing these symptoms represents a challenge for health-care providers. Recent advances have identified small-fiber neuropathy as a potential etiology that may underlie autonomic dysfunction and paresthesia in some long COVID patients. The pathogenic mechanisms underlying myalgia and fatigue remain elusive and need to be investigated. Herein we review the current state of knowledge regarding the evaluation and management of patients with persistent post-COVID-19 neuromuscular symptoms.
Asunto(s)
COVID-19 , Disautonomías Primarias , Humanos , COVID-19/complicaciones , SARS-CoV-2 , Síndrome Post Agudo de COVID-19 , Mialgia/etiología , Parestesia/etiología , Calidad de Vida , Fatiga/etiologíaRESUMEN
INTRODUCTION/AIMS: The development and persistence of neurological symptoms following severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection is referred to as "long-haul" syndrome. We aimed to determine whether small fiber neuropathy (SFN) was associated with SARS-CoV-2 infection. METHODS: We retrospectively studied the clinical features and outcomes of patients who were referred to us between May 2020 and May 2021 for painful paresthesia and numbness that developed during or after SARS-CoV-2 infection and who had nerve conduction studies showing no evidence of a large fiber polyneuropathy. RESULTS: We identified 13 patients, Eight women and five men with age ranging from 38-67 y. Follow-up duration ranged from 8 to 12 mo. All patients developed new-onset paresthesias within 2 mo following SARS-CoV-2 infection, with an acute onset in seven and co-existing autonomic symptoms in seven. Three patients had pre-existing but controlled neuropathy risk factors. Skin biopsy confirmed SFN in six, all of whom showed both neuropathy symptoms and signs, and two also showed autonomic dysfunction by autonomic function testing (AFT). Of the remaining seven patients who had normal skin biopsies, six showed no clinical neuropathy signs and one exhibited signs and had abnormal AFT. Two patients with markedly reduced intraepidermal nerve fiber densities and one with normal skin biopsy had severe and moderate coronavirus disease 2019 (COVID-19); the remainder experienced mild COVID-19 symptoms. Nine patients received symptomatic neuropathy treatment with paresthesias controlled in seven (77.8%). DISCUSSION: Our findings suggest that symptoms of SFN may develop during or shortly after COVID-19. SFN may underlie the paresthesias associated with long-haul post-COVID-19 symptoms.
Asunto(s)
COVID-19 , Enfermedades del Sistema Nervioso Periférico , Neuropatía de Fibras Pequeñas , COVID-19/complicaciones , Femenino , Humanos , Masculino , Enfermedades del Sistema Nervioso Periférico/etiología , Estudios Retrospectivos , SARS-CoV-2 , Neuropatía de Fibras Pequeñas/complicacionesRESUMEN
Since the onset of the COVID-19 pandemic, there have been rare reports of spinal cord pathology diagnosed as inflammatory myelopathy and suspected spinal cord ischemia after SARS-CoV-2 infection. Herein, we report five cases of clinical myelopathy and myeloradiculopathy in the setting of post-COVID-19 disease, which were all radiographically negative. Unlike prior reports which typically characterized hospitalized patients with severe COVID-19 disease and critical illness, these patients typically had asymptomatic or mild-moderate COVID-19 disease and lacked radiologic evidence of structural spinal cord abnormality. This case series highlights that COVID-19 associated myelopathy is not rare, requires a high degree of clinical suspicion as imaging markers may be negative, and raises several possible pathophysiologic mechanisms.
Asunto(s)
COVID-19/complicaciones , Enfermedades de la Médula Espinal/etiología , Enfermedades de la Médula Espinal/patología , Anciano , Femenino , Humanos , Imagen por Resonancia Magnética , Masculino , Persona de Mediana Edad , SARS-CoV-2RESUMEN
INTRODUCTION: A conduction block at a noncompressible site warrants further investigation. METHODS AND MATERIALS: A 36-year-old woman with a history of Hodgkin lymphoma and chemotherapy-induced polyneuropathy developed bilateral hand numbness and paresthesias. Workup revealed bilateral carpal tunnel syndrome and an apparent superimposed conduction block of the median nerve in the forearm. Given the history of cancer, there was concern for an infiltrative or an immune-mediated process. RESULTS: Neuromuscular ultrasound demonstrated that the median nerve descended the upper extremity along an atypical path, deep along the posteromedial aspect of the upper arm, and relatively medially in the forearm. Ultrasound-directed nerve stimulation revealed there was no conduction block. This anatomical variant has been rarely described and has not been reported previously to mimic conduction block or been documented via ultrasound. CONCLUSIONS: This case demonstrates that neuromuscular ultrasound may supplement the electrodiagnostic study and limit confounding technical factors because of rare anatomic variation.
Asunto(s)
Síndrome del Túnel Carpiano/diagnóstico por imagen , Nervio Mediano/diagnóstico por imagen , Adulto , Electromiografía , Femenino , Humanos , Conducción Nerviosa , Parestesia/diagnóstico por imagen , UltrasonografíaRESUMEN
ABSTRACT: Elsberg syndrome is a rare cause of lumbosacral radiculitis with concomitant thoracic and lumbosacral myelitis that can be seen after an acute or reactivated viral infection. After the initial coronavirus surge in New York City, a 68-year-old man developed progressive lower extremity weakness and a defined sensory level at the lower abdomen. He had highly elevated SARS-CoV-2 IgG antibodies despite an absence of preceding COVID-19 symptoms. Serial electrodiagnostic testing revealed absent lower extremity late responses, with otherwise normal distal sensorimotor conductions. Electromyography revealed active neurogenic changes and reduced motor unit recruitment in the L3-L4 myotomes. Treatment with methylprednisolone and intravenous immunoglobulin was followed by minimal clinical improvement but re-emergence of the lower extremity late responses on electrodiagnostic testing. We report here, to the best of our knowledge, the first case of suspected COVID-19-associated Elsberg syndrome, which expands the spectrum of neuromuscular manifestations associated with SARS-CoV-2 infection and sheds light on ways to approach diagnostic and treatment options for these patients.
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COVID-19/complicaciones , Mielitis/etiología , Radiculopatía/etiología , Anciano , Antiinflamatorios/uso terapéutico , Electrodiagnóstico , Electromiografía , Humanos , Inmunoglobulina G/análisis , Imagen por Resonancia Magnética , Masculino , Metilprednisolona/uso terapéutico , Debilidad Muscular/etiología , Mielitis/diagnóstico , Conducción Nerviosa , Radiculopatía/diagnóstico , Columna Vertebral/diagnóstico por imagen , Síndrome , Resultado del TratamientoRESUMEN
This article provides an overview of the clinical features, diagnosis, and treatment of the major paraprotein-related peripheral neuropathies, including monoclonal gammopathy of undetermined significance, Waldenström macroglobulinemia, POEMS syndrome, multiple myeloma, transthyretin amyloidosis, and light chain amyloidosis. For each paraprotein neuropathy, the epidemiology, demographics, systemic findings, and electrophysiologic features are presented. Pharmacologic treatment of transthyretin amyloid polyneuropathy also is reviewed.
Asunto(s)
Gammopatía Monoclonal de Relevancia Indeterminada , Paraproteinemias , Enfermedades del Sistema Nervioso Periférico , Anciano , Humanos , Paraproteinemias/diagnóstico , Paraproteinemias/terapia , Enfermedades del Sistema Nervioso Periférico/diagnóstico , Macroglobulinemia de WaldenströmRESUMEN
INTRODUCTION: Our aim in this study was to determine whether intravenous immunoglobulin (IVIg) or plasma exchange (PLEx) for treatment of neurologic disease is a trigger for thrombotic events. METHODS: Using administrative data from 2005 to 2014, we identified index admissions for thrombotic events. We performed case-crossover analyses for these admissions with previous admissions for neurologic disease with IVIg or PLEx using exposure periods of between 7 and 120 days. RESULTS: We identified 1.9 million admissions for venous thrombosis embolism, myocardial infarction, or acute ischemic stroke. The odds ratio for venous thrombosis embolism within a 30-day window after exposure to IVIg was 3.33 (1.34-8.30, P = .0097) and for PLEx was 4.29 (1.88-9.76, P = .0005). Myocardial infarction and acute ischemic stroke admissions were not associated with exposure to either therapy. DISCUSSION: Patients admitted for venous thrombosis embolism (but not acute ischemic stroke or myocardial infarction) were more likely exposed to either IVIg or PLEx during previous admission for neurologic disease.
Asunto(s)
Isquemia Encefálica/etiología , Inmunoglobulinas Intravenosas/efectos adversos , Infarto del Miocardio/etiología , Intercambio Plasmático/efectos adversos , Accidente Cerebrovascular/etiología , Tromboembolia Venosa/etiología , Anciano , Anciano de 80 o más Años , Estudios Cruzados , Bases de Datos Factuales , Dermatomiositis/terapia , Femenino , Síndrome de Guillain-Barré/terapia , Humanos , Masculino , Persona de Mediana Edad , Esclerosis Múltiple/terapia , Miastenia Gravis/terapia , Polimiositis/terapia , Factores de RiesgoRESUMEN
Peripheral neuropathy is associated with numerous systemic diseases. It is often the heralding finding, which can lead to earlier diagnoses and better outcomes. An understanding of the epidemiology and clinical features of these diseases is paramount to their diagnosis and management. This article will focus on neuropathy associated with connective tissue diseases, monoclonal gammopathies, paraneoplastic disorders, medications including chemotherapeutic agents, nutritional deficiencies, alcohol, and toxins.
Asunto(s)
Antineoplásicos/uso terapéutico , Enfermedades del Tejido Conjuntivo/terapia , Quimioterapia , Enfermedades del Sistema Nervioso Periférico/terapia , Enfermedades del Tejido Conjuntivo/complicaciones , Quimioterapia/métodos , Humanos , Trastornos Nutricionales/terapia , Enfermedades del Sistema Nervioso Periférico/complicacionesRESUMEN
OBJECTIVES: We report a case of immune myopathy with perimysial pathology associated with anti-glycyl-transfer RNA synthetase (anti-EJ) antibody and an excellent treatment response. METHODS: Chart review. RESULTS: A 36-year-old woman presented with 3 months of fatigue, weight loss, progressive weakness in a scapuloperoneal distribution, and dysphagia. Nerve conduction studies, electromyography, and ultrasound suggested an irritable myopathy. She had marked elevations of creatine kinase and positive anti-glycyl-transfer RNA synthetase (anti-EJ) antibodies. A left biceps muscle biopsy revealed inflammation of the perimysium and surrounding perimysial blood vessels with focal fragmentation of the perimysium. Further evaluation revealed interstitial lung disease. Treatment with prednisone and mycophenolate mofetil led to marked clinical improvement of her symptoms. CONCLUSIONS: Our case adds to the growing spectrum of inflammatory myopathies and highlights the importance of performing a comprehensive, multisystem workup.
Asunto(s)
Autoanticuerpos/sangre , Glicina-ARNt Ligasa/inmunología , Enfermedades Pulmonares Intersticiales/complicaciones , Miositis/complicaciones , Adulto , Femenino , Humanos , Enfermedades Pulmonares Intersticiales/sangre , Enfermedades Pulmonares Intersticiales/diagnóstico por imagen , Músculo Esquelético/diagnóstico por imagen , Músculo Esquelético/patología , Músculo Esquelético/ultraestructura , Miositis/sangre , Miositis/diagnóstico por imagen , Tomógrafos Computarizados por Rayos X , UltrasonografíaRESUMEN
Peripheral neuropathy is a common complication of many of the systemic amyloidoses. Although the cause of neuropathy is not entirely clear, it is likely related to amyloid deposition within the nerve. This may lead to focal, multifocal, or diffuse neuropathies involving sensory, motor and/or autonomic fibers. The presenting symptoms depend on the distribution of nerves affected. One of the most common phenotypes is sensorimotor polyneuropathy, which is characterized by symptoms of neuropathic pain, numbness, and in advanced cases weakness. Symptoms begin in the feet and ultimately progress to the proximal legs and hands. The most common focal neuropathy is a median neuropathy at the wrist, clinically known as carpal tunnel syndrome. Carpal tunnel symptoms may include pain and sensory disturbances in the lateral palm and fingers; hand weakness may ensue if the focal neuropathy is severe. Autonomic neuropathy may affect a variety of organ systems such as the cardiovascular, gastrointestinal, and genitourinary systems. Symptoms may be non-specific making the diagnosis of autonomic neuropathy more difficult to identify. However, it is important to recognize and distinguish autonomic neuropathy from diseases of the end-organs themselves. This article reviews the inherited and acquired amyloidoses that affect the peripheral nervous system including familial amyloid polyneuropathy, and primary, secondary and senile amyloidosis. We emphasize the clinical presentation of the neurologic aspects of these diseases, physical examination findings, appropriate diagnostic evaluation, treatment and prognosis.