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Adult-onset atypical form of Hallervorden-Spatz disease [Eastern J Med]
Eastern J Med. 2013; 18(4): 221-223

Adult-onset atypical form of Hallervorden-Spatz disease

Aysel Milanlıoğlu1, Mehmet Nuri Aydın1, Alper Gökgül1, Mehmet Hamamcı1, Temel Tombul1

Hallervorden-Spatz syndrome is a rare neurodegenerative disease, resulting from mutation in the Pantothenate kinase-2 gene, and characterized by progressive pyramidal and extrapyramidal dysfunction, dementia, retinal degeneration and optic nerve atrophy. Clinical symptoms are related to abnormal iron deposition in the globus pallidus and substantia nigra. We present a case report of a 50-year old woman that was diagnosed as atypical Hallervorden-Spatz disease with dominant extrapyramidal symptoms and the lack of typical eye-of-the-tiger sign in brain MRI.

Keywords: Hallervorden-Spatz disease, iron deposition, eye-of-the-tiger sign, neurodegeneration related to pantothenate kinase, basal ganglia


Aysel Milanlıoğlu, Mehmet Nuri Aydın, Alper Gökgül, Mehmet Hamamcı, Temel Tombul. Adult-onset atypical form of Hallervorden-Spatz disease. Eastern J Med. 2013; 18(4): 221-223


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