Two Fatal Cases due to Porphyric Peripheral Neurophathy
Journal of the Korean Neurological Association
;
: 599-606, 1993.
Artigo
em Coreano
| WPRIM
| ID: wpr-89036
ABSTRACT
We report two cases of porphyric peripheral neuropathy in a 19-year-old male with variegate porphyria and in a 39 year-old male with intermittent acute prophyria. Clinically, there were sensory, motor disturbance and autonomic symptoms including decreased sweating, urinary and sphinctor distrubances. Variegate porphyria showed facial diplegia and positive family history inherited by autosomal dominent trait. Intermittent acute porphyria was combined-with SIADH. Both cases were expired due to respiratory failure. Nerve conduction studies were carried out in two cases and both cases showed slow motor, sensory nerve conductlon velocity ,and significant low CMAPs(Compound Muscle Action Potentials). Sural nerve biopsy was carried out in a variegate prophyria compared with one normal control. Decreased large myelinated fibers was found. In nerve fiber teased study. 8.5% of nerve fibers showed axonal degenration and only 2.3% of the segmental demyelination. There findings are suggesting that the porphyric neuro might be the axonal type.and severe neuropathy in a sign of poor prognosis.
Texto completo:
DisponíveL
Índice:
WPRIM (Pacífico Ocidental)
Assunto principal:
Prognóstico
/
Insuficiência Respiratória
/
Axônios
/
Nervo Sural
/
Suor
/
Sudorese
/
Biópsia
/
Doenças Desmielinizantes
/
Porfiria Aguda Intermitente
/
Doenças do Sistema Nervoso Periférico
Tipo de estudo:
Estudo prognóstico
Limite:
Adulto
/
Humanos
/
Masculino
Idioma:
Coreano
Revista:
Journal of the Korean Neurological Association
Ano de publicação:
1993
Tipo de documento:
Artigo
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