RESUMO
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.
Assuntos
Doenças Desmielinizantes/etiologia , Cadeias lambda de Imunoglobulina/análise , Imunoglobulinas Intravenosas/uso terapêutico , Vértebras Lombares , Proteínas do Mieloma/análise , Osteosclerose/etiologia , Síndrome POEMS/complicações , Doenças do Sistema Nervoso Periférico/etiologia , Plasmocitoma/complicações , Neoplasias da Coluna Vertebral/complicações , Terapia Combinada , Doenças Desmielinizantes/fisiopatologia , Doenças Desmielinizantes/radioterapia , Doenças Desmielinizantes/terapia , Progressão da Doença , Humanos , Vértebras Lombares/patologia , Masculino , Pessoa de Meia-Idade , Células-Tronco Neoplásicas/química , Condução Nervosa , Síndrome POEMS/diagnóstico , Doenças do Sistema Nervoso Periférico/fisiopatologia , Doenças do Sistema Nervoso Periférico/radioterapia , Doenças do Sistema Nervoso Periférico/terapia , Plasmócitos/química , Plasmocitoma/diagnóstico , Neoplasias da Coluna Vertebral/diagnósticoRESUMO
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.
Assuntos
Doenças Desmielinizantes/radioterapia , Encefalomielite Autoimune Experimental/complicações , Infecções por Enterovirus/complicações , Hepatite Viral Animal/complicações , Medula Espinal/efeitos da radiação , Animais , Doenças Desmielinizantes/etiologia , Doenças Desmielinizantes/microbiologia , Feminino , Masculino , Vírus Elberfeld do Camundongo/efeitos da radiação , Camundongos , Camundongos Endogâmicos BALB C , Camundongos Nus , Vírus da Hepatite Murina/efeitos da radiação , Medula Espinal/microbiologiaRESUMO
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.
