RESUMO
Neuromyelitis optica (NMO) has been attributed to different underlying pathological events. The aim of this paper is to present the first case report of a patient with Down's syndrome (DS) who died of a fulminant NMO. A 29-year-old woman with DS developed acute transverse myelitis, with complete visual loss and swollen optic discs. Two days later, she developed quadriplegia, respiratory arrest and died. The anatomical study demonstrated typical findings of DS in the brain without demyelinating lesions. A severe destruction of medulla and cervical cord with a very high degree of demyelination of the optic nerves was typical of monophasic NMO (Devic's disease). Most of the cases of NMO in Cuba are of the relapsing form, but this case report is the first one with monophasic NMO and DS with a very aggressive course. The link of the pathogenetic relationship between DS and NMO remains unclear; it may well be coincidence but the fact that the patient died very shortly after the onset suggests, at least on clinical grounds, that the presence of DS could have accelerated the fatal evolution of NMO.
Assuntos
Síndrome de Down/complicações , Neuromielite Óptica/complicações , Adulto , Autopsia , Síndrome de Down/patologia , Feminino , Humanos , Bulbo/patologia , Neuromielite Óptica/patologia , Nervo Óptico/patologia , Medula Espinal/patologiaRESUMO
INTRODUCTION: Although the nervous system is highly resistant to radiations, some cases have been reported about adverse effects of radiotherapy on patients with brain tumors. The following delayed effects have been observed: coagulation necrosis of all the cellular elements, fibrinoid and hyaline changes on the walls of the neoformed blood vessels, together with endothelial proliferation and perivascular fibrosis, reactive astrocytes, some of them with bizarre nuclei, as well as telangiectasia, hemorrhage and hemorrhagic infarcts. Telangiectasia and associated hemorrhage may occur in any of the irradiated zones of the brain. CLINICAL CASE: The present case is an example in which the indication of radiotherapy was due to an anatomopathological overdiagnosis. The first biopsy was diagnosed as a grade II cerebellar astrocytoma, after which 20 radiotherapy sessions were indicated. Nine years after that, a second operation was performed. This second biopsy revealed areas of fibrosis, hemorrhages, calcifications, necrosis, and thick hyaline-walled blood vessels. No tumor infiltration was observed. After an unfavorable evolution, the patient died. The microscopical study of the autopsy material revealed an area of neoplastic infiltration which was well defined as a pylocitic astrocytoma of the cerebellum. The rest of the cerebellum sections evidenced damage corresponding to an adverse effect of radiotherapy, particularly large zones of telangiectasia. CONCLUSIONS: It is stated that the patient s death was not because of the tumor. The adverse effects of radiotherapy, interpreted as tumor remains, motivated the second operation that generated complications causing the patient s death.