We describe an atypical case of pantothenate kinase-associated neurodegeneration (PKAN) in which slowly progressive arm tremor was the predominant symptom beginning at the age of 25, with late-onset dystonia and dysarthria developing at the age of 50. Compound heterozygous mutations resulting in mis
Regression of symptoms after selective iron chelation therapy in a case of neurodegeneration with brain iron accumulation
β Scribed by Gian Luca Forni; Manuela Balocco; Laura Cremonesi; Giovanni Abbruzzese; Roberto Carlo Parodi; Roberta Marchese
- Publisher
- John Wiley and Sons
- Year
- 2008
- Tongue
- English
- Weight
- 185 KB
- Volume
- 23
- Category
- Article
- ISSN
- 0885-3185
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β¦ Synopsis
Abstract
We report the results of iron chelating treatment with deferiprone in a 61βyearβold woman with signs and symptoms of neurodegeneration with brain iron accumulation (NBIA). After 6 months of therapy the patient's gait had improved and a reduction in the incidence of choreic dyskinesias was observed. Her gait returned to normal after an additional 2 months of therapy, at which time there was a further reduction in involuntary movements and a partial resolution of the blepharospasm. Β© 2008 Movement Disorder Society
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