Assuntos
Encefalite Antirreceptor de N-Metil-D-Aspartato/terapia , Fatores Imunológicos/uso terapêutico , Imunoterapia/métodos , Adulto , Encefalite Antirreceptor de N-Metil-D-Aspartato/diagnóstico por imagem , Encefalite Antirreceptor de N-Metil-D-Aspartato/fisiopatologia , Eletroencefalografia , Feminino , Humanos , Imageamento por Ressonância Magnética , RecidivaAssuntos
Hemorragia Cerebral/etiologia , Síndrome de Churg-Strauss/complicações , Hemorragia Cerebral/diagnóstico , Síndrome de Churg-Strauss/diagnóstico , Humanos , Imageamento por Ressonância Magnética , Masculino , Pessoa de Meia-Idade , Lobo Occipital/diagnóstico por imagem , Lobo Occipital/patologia , Tomografia Computadorizada por Raios XRESUMO
OBJECTIVES: The involvement of the peripheral nervous system by anti-CV2/CRMP5 paraneoplastic antibodies is typically encountered as a mixed sensorimotor polyneuropathy. We report a fatal case of severe chronic progressive axonal polyradiculoneuropathy in association with this antibody. METHODS: Review of the patient's chart, nerve conduction/electromyographic studies, and nerve biopsy. RESULTS: A 51-year-old man presented with a progressive quadriparesis over a 4-month period. Extensive evaluation for potential etiologies was significant only for positive anti-CV2/CRMP5 antibodies without detection of an underlying neoplasm. Despite multiple immunomodulatory therapies, the patient progressed and demonstrated electrodiagnostic evidence for a chronic axonal polyradiculoneuropathy with ongoing denervation. The patient eventually died of respiratory failure. CONCLUSIONS: This case adds to the clinical spectrum of the peripheral nervous system involvement in patients with paraneoplastic anti-CV2/CRMP5 antibodies.
Assuntos
Autoanticorpos/imunologia , Proteínas do Tecido Nervoso/imunologia , Polirradiculoneuropatia/imunologia , Polirradiculoneuropatia/patologia , Autoantígenos/imunologia , Evolução Fatal , Humanos , Hidrolases , Masculino , Proteínas Associadas aos Microtúbulos , Pessoa de Meia-IdadeRESUMO
Systemic sclerosis (SSc) or scleroderma is a connective tissue disease with a diverse array of clinical manifestations secondary to underlying fibrosis and autoimmunity. Central nervous system (CNS) impairment is uncommon in SSc. Here we report the fourth known patient with CNS vasculitis caused by SSc. In each previous report, the patient was a middle-aged to elderly female. Our patient was 24 years old at the time of presentation, significantly younger than the other reported patients. Importantly, our patient's rapidly progressive clinical course and poor response to immunosuppression have not been reported in patients with CNS vasculitis secondary to scleroderma. Although CNS vasculitis is extremely rare in SSc, our report suggests that clinicians should consider this diagnosis in the differential of SSc patients with neurologic impairment.