[Westphal-Strumpell disease. Description of a clinical case].
A case of hepatolenticular degeneration with the clinical pattern of Westphal-Strumpell disease is described. Diagnosis was based on clinical pattern, absence of hepatic impairment, typical biochemical abnormalities of copper metabolism and response (clinical and biochemical) to D-penicillamine.
Nuccetelli
F
F
Università degli Studi di Chieti, Clinica Neurologica.
Assetta
M
M
Colangelo
U
U
Gambi
D
D
D000328:Adult; D003300:Copper / Q000378:metabolism; D005260:Female; D006527:Hepatolenticular Degeneration / Q000175:diagnosis* / Q000188:drug therapy / Q000378:metabolism; D006801:Humans; D010396:Penicillamine / Q000627:therapeutic use
D003300:Copper; D010396:Penicillamine
La malattia di Westphal Strumpell. Descrizione di un caso clinico.