RESUMO
Leber hereditary optic neuropathy (LHON) is rarely associated with multiple sclerosis-like features. We present a case of a 65-year-old African American woman with LHON masquerading as neuromyelitis optica (NMO). We highlight the features of the clinical examination and MRI that were suggestive of an alternative diagnosis and review the literature regarding LHON and multiple sclerosis. The diagnosis of LHON should be considered in all cases of acute or subacute bilateral optic neuropathy, including presumed seronegative NMO.
Assuntos
Neuromielite Óptica/diagnóstico , Atrofia Óptica Hereditária de Leber/diagnóstico , Atrofia Óptica Hereditária de Leber/genética , Idoso , Diagnóstico Diferencial , Feminino , Humanos , Esclerose Múltipla/diagnóstico , Esclerose Múltipla/genética , Neuromielite Óptica/genética , Compressão da Medula Espinal/diagnóstico , Compressão da Medula Espinal/genéticaAssuntos
Sistema Nervoso Central/patologia , Sistema Nervoso Central/fisiopatologia , Doenças Desmielinizantes/patologia , Doenças Desmielinizantes/fisiopatologia , Paralisia de Bell/patologia , Paralisia de Bell/fisiopatologia , Diagnóstico Diferencial , Esclerose Cerebral Difusa de Schilder/patologia , Esclerose Cerebral Difusa de Schilder/fisiopatologia , Humanos , Imageamento por Ressonância Magnética , Esclerose Múltipla/patologia , Esclerose Múltipla/fisiopatologia , Neuromielite Óptica/patologia , Neuromielite Óptica/fisiopatologiaRESUMO
The neuromuscular aspects of West Nile virus (WNV) infection have not been characterized in detail. We have studied a group of six patients with proven WNV infection. All cases presented with acute, severe, asymmetric, or monolimb weakness, with minimal or no sensory disturbance after a mild flu-like prodrome. Four cases also had facial weakness. Three of our cases had no encephalitic signs or symptoms despite cerebrospinal fluid pleocytosis. Electrophysiological studies showed severe denervation in paralyzed limb muscles, suggesting either motor neuron or multiple ventral nerve root damage. This localization is supported further by the finding of abnormal signal intensity confined to the anterior horns on a lumbar spine magnetic resonance imaging. Muscle biopsies from three patients showed scattered necrotic fibers, implicating mild direct or indirect muscle damage from the WNV infection. In summary, we describe a group of patients with acute segmental flaccid paralysis with minimal or no encephalitic or sensory signs. We have localized the abnormality to either the spinal motor neurons or their ventral nerve roots. It will be important for physicians to consider WNV infection in patients with acute asymmetric paralysis with or without encephalitic symptoms.