RESUMEN
Osteosclerotic myeloma is a plasma-cell dyscrasia characterized by osteosclerotic bone lesions, which may be associated with progressive demyelinating polyneuropathy. We describe a 49-year-old patient with rapidly deteriorating polyneuropathy associated with osteosclerotic myeloma, who responded favorably to a combination of intravenous immunoglobulin and radiotherapy.
Asunto(s)
Enfermedades Desmielinizantes/etiología , Cadenas lambda de Inmunoglobulina/análisis , Inmunoglobulinas Intravenosas/uso terapéutico , Vértebras Lumbares , Proteínas de Mieloma/análisis , Osteosclerosis/etiología , Síndrome POEMS/complicaciones , Enfermedades del Sistema Nervioso Periférico/etiología , Plasmacitoma/complicaciones , Neoplasias de la Columna Vertebral/complicaciones , Terapia Combinada , Enfermedades Desmielinizantes/fisiopatología , Enfermedades Desmielinizantes/radioterapia , Enfermedades Desmielinizantes/terapia , Progresión de la Enfermedad , Humanos , Vértebras Lumbares/patología , Masculino , Persona de Mediana Edad , Células Madre Neoplásicas/química , Conducción Nerviosa , Síndrome POEMS/diagnóstico , Enfermedades del Sistema Nervioso Periférico/fisiopatología , Enfermedades del Sistema Nervioso Periférico/radioterapia , Enfermedades del Sistema Nervioso Periférico/terapia , Células Plasmáticas/química , Plasmacitoma/diagnóstico , Neoplasias de la Columna Vertebral/diagnósticoRESUMEN
The effects of X-irradiation on the course of chronic demyelinating disease were examined in mice with experimental allergic encephalitis (EAE), mouse hepatitis virus (MHV) or Theiler's virus (DAV) infection. One month after the induction of EAE or 2-16 months after inoculation of DAV, exposure of the cervical spinal cord to 20 Gy X-rays caused local exacerbation of disease activity but spinal irradiation did not affect MHV-induced demyelination. In EAE, there was a significant increase in the number of inflammatory cells in the irradiated part of the cord. Mice infected with DAV showed locally increased demyelination and axonal degeneration but no change in the titer of infectious virus within the cord. Thus in DAV infection, as in EAE, the exacerbation of disease seemed to be due to vascular or immunological factors rather than viral reactivation.
Asunto(s)
Enfermedades Desmielinizantes/radioterapia , Encefalomielitis Autoinmune Experimental/complicaciones , Infecciones por Enterovirus/complicaciones , Hepatitis Viral Animal/complicaciones , Médula Espinal/efectos de la radiación , Animales , Enfermedades Desmielinizantes/etiología , Enfermedades Desmielinizantes/microbiología , Femenino , Masculino , Virus Maus Elberfeld/efectos de la radiación , Ratones , Ratones Endogámicos BALB C , Ratones Desnudos , Virus de la Hepatitis Murina/efectos de la radiación , Médula Espinal/microbiologíaRESUMEN
Four patients with refractory or poorly responsive chronic progressive demyelinating polyneuropathy (CPDP) were treated with total lymphoid irradiation (total dose, 2000 rad) in an uncontrolled feasibility study. All patients had previously failed conventional therapy for CPDP, as well as other unconventional treatments. During a follow-up period of 7 to 12 months after total lymphoid irradiation, there was a profound and sustained suppression of the absolute lymphocyte count and in vitro lymphocyte function, as well as an increase in the ratio of Leu-2 (suppressor/cytotoxic subset) to Leu-3 (helper/inducer subset) T cells in the blood. Three of the four patients demonstrated improvement in distal muscle strength, and this was associated with increased functional capabilities in two patients. In contrast, no clinical improvement in sensation was noted in any patient. Nerve conduction studies showed patchy improvement in three patients. The results of this preliminary uncontrolled study indicate that radiotherapy deserves further study in the treatment of CPDP.