Paraneoplastic ataxia and dystonia secondary to a malignant fibrous histiocytoma.
BMJ Case Rep
; 20102010 Nov 12.
Article
em En
| MEDLINE
| ID: mdl-22798441
ABSTRACT
Paraneoplastic movement disorders are part of the spectrum of paraneoplastic syndromes caused by the production of onconeural antibodies such as anti-Hu by underlying tumours. These attack specific neurons depending on receptor aetiology. We report the case of a 53-year-old man who presented 8 years previously with symptoms of upper limb weakness, light headedness, dizziness and falls. His condition followed a progressive course. Two years after onset he had right-sided weakness, diplopia and generalised dystonia. Initial investigations identified a positive anti-Hu antibody, but an extensive search for a primary tumour was negative. A malignant fibrous histiocytoma in his right gluteal fold was subsequently identified. At this stage he was bed bound with severe ataxia, dystonia and spasticity. Following surgical excision and treatment with high dose steroids and pulse immunoglobulin, further progression was arrested and minor improvements occurred. He can now ambulate with bilateral assistance but remains severely disabled.
Texto completo:
1
Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Ataxia
/
Neoplasias de Tecidos Moles
/
Síndromes Paraneoplásicas do Sistema Nervoso
/
Distonia
/
Histiocitoma Fibroso Maligno
Tipo de estudo:
Diagnostic_studies
/
Etiology_studies
/
Prognostic_studies
Limite:
Humans
/
Male
/
Middle aged
Idioma:
En
Revista:
BMJ Case Rep
Ano de publicação:
2010
Tipo de documento:
Article
País de afiliação:
Reino Unido