Clinical presentation and electrophysiological findings of porphyric neuropathies: a follow-up study.
Muscle Nerve
; 51(3): 363-9, 2015 Mar.
Article
em En
| MEDLINE
| ID: mdl-24985076
ABSTRACT
INTRODUCTION:
A case series of acute intermittent porphyria (AIP) is described that focuses on the clinical course of the disease with regard to neurological manifestations of the peripheral nervous system.METHODS:
Eight patients were diagnosed with AIP on the basis of characteristic clinical findings, erythrocyte porphobilinogendeaminase activity, neuropathic patterns, serial changes in nerve conduction studies (NCS), and temporal relationship of central nervous system involvement.RESULTS:
Six patients diagnosed with AIP<2 months after symptom onset had neuropathy that was predominantly upper extremity, motor, and proximal. NCS recovery rates were slower in the lower than the upper limbs. Two patients diagnosed >2 months after symptom onset had distal sensorimotor polyneuropathy.CONCLUSIONS:
The findings from this case series suggest that the peripheral nerves may be differentially and selectively involved in different diagnostic stages of porphyric neuropathy.Palavras-chave
Texto completo:
1
Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Polineuropatias
/
Porfiria Aguda Intermitente
/
Eletromiografia
/
Condução Nervosa
Tipo de estudo:
Diagnostic_studies
/
Observational_studies
/
Prognostic_studies
Limite:
Adult
/
Female
/
Humans
/
Male
Idioma:
En
Revista:
Muscle Nerve
Ano de publicação:
2015
Tipo de documento:
Article
País de afiliação:
Taiwan