A severe neurological sequela in acute intermittent porphyria: presentation of a case from encephalopathy to quadriparesis.
Br J Radiol
; 81(965): e135-40, 2008 May.
Article
em En
| MEDLINE
| ID: mdl-18440935
ABSTRACT
Porphyrias present themselves with autonomic or peripheral neuropathy or central nervous system dysfunction. They are a varied group of inborn errors of metabolism that are characterized by specific inherited enzyme defects in haem biosynthesis. A patient whose mother was diagnosed as having porphyria was admitted to hospital because of her abdominal pains and dysuria. She had three generalized convulsions and, in a few hours, she lost the vision in both of her eyes. As the seizures continued, she became quadriparetic and fell into a coma after gradually losing consciousness. She improved but with sequelae; her serial MRIs, including apparent diffusion coefficient map imaging, diffusion-weighted imaging and angiography, showed ischaemic lesions that were both unlike and more severe than the ones reported in the literature.
Texto completo:
1
Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Quadriplegia
/
Encefalopatias
/
Porfiria Aguda Intermitente
Tipo de estudo:
Diagnostic_studies
Limite:
Adult
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Female
/
Humans
Idioma:
En
Revista:
Br J Radiol
Ano de publicação:
2008
Tipo de documento:
Article