Late Onset Neurodegeneration with Brain-Iron Accumulation Presenting as Parkinsonism. (18th December 2012)

• Record Type: Journal Article
• Authors: Fekete, Robert
• Other Names: Ateş Ö. Academic Editor.; Gontkovsky S. T. Academic Editor.; Rajajee V. Academic Editor.; Sandroni P. Academic Editor.; Swash M. Academic Editor.; Wakabayashi Y. Academic Editor.
• Is Part Of: Case reports in neurological medicine. Volume 2012(2012)
• Publication Date: 2012-12-18
• Subjects: Neurology -- Periodicals; Nervous system -- Diseases -- Periodicals; Neurology; Nervous System Diseases; Nervous system -- Diseases; Neurology; Electronic journals; Periodicals; Periodicals; 616.8
• DOI: 10.1155/2012/387095
• Languages: English
• ISSNs: 2090-6676

Abstract

Abstract : Neurodegeneration with brain-iron accumulation (NBIA) encompasses a family of neurodegenerative disorders connected by evidence of abnormal brain iron deposition. Advances in imaging and genetic testing expanded the clinical spectrum of these disorders. Here, a case of parkinsonism and dystonia with orofacial stereotypies is presented. While the patient was initially diagnosed with Parkinson's disease and placed on levodopa therapy, dopamine transporter imaging via (123)I-FP-CIT SPECT (DaTSCAN) was normal. MRI brain showed "eye of the tiger" sign on T2 weighted imaging. NBIA should be considered in the differential diagnosis of atypical parkinsonism.