RESUMEN
Hashimoto encephalopathy has a wide clinical spectrum with various neuropsychiatric features. The detection of anti-thyroid antibodies in patient sera is helpful, but alone not sufficient for the diagnosis of Hashimoto encephalopathy because of the high prevalence of antibodies in the normal population. Here, a patient with Hashimoto encephalopathy, who presented with progressive cerebellar ataxia with mild abnormality on electroencephalography (EEG) and who showed marked improvement after steroid administration, is described. This emphasises that Hashimoto encephalopathy can present with isolated cerebellar ataxia and is responsive to immunosupression.
RESUMEN
We report a 55-year-old man with a chief complaint of wasting and weakness of the left quadriceps muscle. At age 54, he noticed difficulty in running and weakness in the left thigh, which gradually progressed. On the first admission to our hospital, based on the nerve conduction studies (NCS), the muscle biopsy findings showing neurologenic changes, and no abnormality of spinal MRI, we diagnosed as unilateral quadriceps amyotrophy, which resulted from an atypical form of spinal progressive muscular atrophy. One year later, he showed the bilateral hand weakness, conduction blocks on the right median and ulnar nerves by NCS, and the presence of serum anti-GM 1 antibody. From these findings, Lewis-Sumner syndrome was diagnosed. The therapy of high-dose intravenous immunoglobulin moderately improved his symptoms. The clinical symptoms of quadriceps amyotrophy is produced by various disorders including spinal progressive muscular atrophy, spinal extradural arachnoid cyst, rimmed vacuole myopathy, Becker dystrophy, limb-girdle dystrophy, and focal myositis. However, there have been no reports of a case of Lewis-Sumner syndrome. It is important to consider Lewis-Sumner syndrome in the differential diagnosis of quadriceps amyotrophy.
Asunto(s)
Enfermedades Desmielinizantes/diagnóstico , Enfermedad de la Neurona Motora/diagnóstico , Trastornos Musculares Atróficos/etiología , Enfermedades Desmielinizantes/complicaciones , Diagnóstico Diferencial , Humanos , Inmunoglobulinas Intravenosas/administración & dosificación , Masculino , Persona de Mediana Edad , Enfermedad de la Neurona Motora/complicaciones , Enfermedades Musculares , Trastornos Musculares Atróficos/diagnóstico , Trastornos Musculares Atróficos/terapia , SíndromeRESUMEN
A 54-year-old female developed dysarthria, left limb ataxia, and crossed dissociated sensory impairment, followed by external ophthalmoplegia, severe bulbar palsy and bilateral pyramidal tract involvement. A high titer of anti-GQ1b IgG antibody was detected in her serum. Brain MRI (T2 weighted image, diffusion weighted image) and peripheral nerve conduction study were normal. We diagnosed her as having Bickerstaff's brainstem encephalitis. In the past literature, we could not find any case of Bickerstaff's brainstem encephalitis showing crossed dissociated sensory impairment.
Asunto(s)
Tronco Encefálico , Encefalitis/diagnóstico , Síndrome Medular Lateral , Autoanticuerpos/sangre , Biomarcadores/sangre , Diagnóstico Diferencial , Femenino , Gangliósidos/inmunología , Humanos , Inmunoglobulina G/sangre , Imagen por Resonancia Magnética , Persona de Mediana EdadRESUMEN
We treated a married couple who developed Mycoplasma pneumonia at the same time, and whose clinical courses and serum soluble interleukin-2 receptor (sIL-2R) levels were markedly different. The 30-year-old wife developed acute respiratory failure and her sIL-2R levels were extremely increased. After pulse therapy, her clinical state was improved, with a marked decrease in sIL-2R. In contrast, the 36-year-old husband, also suffered from the pneumonia with a moderate increase of sIL-2R, and recovered without any complications. The difference in their clinical states may be reflected in their serum sIL-2R levels, a marker of T cell activation in vivo.