6533b85bfe1ef96bd12babe5
RESEARCH PRODUCT
Sporadic Parkinson disease and amyotrophic lateral sclerosis complex (Brait-Fahn-Schwartz disease).
Vincenzo La BellaAlfonsa Claudia TaielloValeria BonoConcetta MannoAlessio Liparisubject
MalePathologymedicine.medical_specialtyExtrapyramidal signsbusiness.industryParkinsonismAmyotrophic Lateral SclerosisParkinson DiseaseDiseaseMiddle Agedmedicine.diseasenervous system diseasesPathogenesisNeurologyKii peninsulaClinical evidencemedicineHumansFemaleNeurology (clinical)Sporadic Parkinson diseaseAmyotrophic lateral sclerosisbusinessAgeddescription
Clinical evidence for parkinsonism may accompany Amyotrophic Lateral Sclerosis with a frequency ranging from 5% to 17%. The concurrence of Amyotrophic Lateral Sclerosis and Parkinson's disease, outside the known Guam and Kii Peninsula foci, is instead rare, but this raises the possibility of a common pathogenesis. Clinically this complex presents with a levodopa-responsive parkinsonism and Amyotrophic Lateral Sclerosis and has been termed Brait-Fahn-Schwartz disease. Here we describe two patients with this uncommon neurodegenerative complex. Both presented with Parkinson disease and progressed to a full blown Amyotrophic Lateral Sclerosis. We further suggest that the association of Parkinson disease and Amyotrophic Lateral Sclerosis represents a distinct nosological entity, which should be kept separated from extrapyramidal signs and symptoms that may occur in Amyotrophic Lateral Sclerosis.
| year | journal | country | edition | language |
|---|---|---|---|---|
| 2012-11-23 | Journal of the neurological sciences |