Small fiber dysfunction in patients with Wilson's disease / Disfunção de fibras finas em pacientes com doença de Wilson
Arq. neuropsiquiatr
;
72(8): 592-595, 08/2014.
Article
in English
| LILACS
| ID: lil-718130
ABSTRACT
Objective:
Patients with Wilson’s disease (WD) may develop a wide variety of neuropsychiatric symptoms, but there are few reports of autonomic dysfunction. Here, we described evidence of small fiber and/or autonomic dysfunction in 4 patients with WD and levodopa-responsive parkinsonism.Method:
We reviewed the charts of 4 patients with WD who underwent evaluation for the presence of neuromuscular dysfunction and water-induced skin wrinkling test (SWT).Results:
Two men and 2 women (33±3.5 years) with WD were evaluated. They all had parkinsonism at some point during their disease course. Parkinsonism on patient 4 almost completely subsided with treatment of WD. Two patients had significant sensory and 2 significant autonomic complaints, including syncopal spells. NCS/EMG was normal in all but SWT was abnormal in half of them (mean 4-digit wrinkling of 0.25 and 1).Discussion:
A subset of patients with WD exhibit evidence of abnormal skin wrinkling test (small fiber neuropathy). .RESUMO
Objetivo:
Pacientes com doença de Wilson (DW) podem desenvolver uma ampla variedade de sintomas neuropsiquiátricos, mas existem poucos relatos de disfunção autonômica. Aqui, nós descrevemos evidência de disfunção de fibras finas/autonômica em 4 pacientes com DW e parkinsonismo responsivo à levodopa.Método:
Nós revisamos os prontuários de 4 pacientes com DW que foram submetidos a avaliação neuromuscular e ao teste de quantificação do enrugamento cutâneo (TEC).Resultados:
Dois homens e 2 mulheres (33±3,5 anos) com DW foram avaliados. Todos apresentaram parkinsonismo durante o curso de sua doença. Parkinsonismo no paciente 4 quase completamente desapareceu com tratamento da DW. Dois pacientes apresentaram queixas sensitivas e 2 apresentaram queixas autonômicas significativas incluindo episódios de síncope. Eletroneuromiografia foi normal em todos e TEC foi anormal em metade deles (score do TEC nos 4 dedos de 0,25 e 1). Discussão Um subgrupo de pacientes com DW apresenta evidência de TEC anormal (neuropatia de fibras finas). .
Full text:
Available
Index:
LILACS (Americas)
Main subject:
Peripheral Nerves
/
Hepatolenticular Degeneration
/
Nerve Fibers
/
Neural Conduction
Limits:
Adult
/
Female
/
Humans
/
Male
Language:
English
Journal:
Arq. neuropsiquiatr
Journal subject:
Neurology
/
Psychiatry
Year:
2014
Type:
Article
Affiliation country:
Brazil
Institution/Affiliation country:
Universidade Federal do Ceará/BR
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