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Frontotemporal dementia-amyotrophic lateral sclerosis complex is simulated by neurodegeneration with brain iron accumulation.

Abstract
We describe a case of late onset neurodegeneration with brain iron accumulation (NBIA) presenting as frontotemporal dementia (FTD) with amyotrophic lateral sclerosis (ALS). A male patient presented at age 66 with change of personality: disinhibition, emotional blunting, and socially inappropriate behavior, coupled with dysarthria, dystonia, and corticospinal tract involvement. Magnetic resonance imaging showed general cortical atrophy, iron deposits in the globus pallidus, and the "eye of the tiger" sign. Neuropsychologic performance was globally reduced, especially executive functions. Fluorodeoxyglucose positron emission tomography showed hypometabolism predominantly in frontal and temporal areas. Repeated neurophysiologic examinations showed signs of chronic denervation. The patient was diagnosed with NBIA but fulfilled consensus criteria for FTD and had a clinical picture of ALS, without neurophysiologic confirmation. Our finding introduces NBIA as a possible cause of FTD and as a differential diagnosis of the FTD-ALS complex.
AuthorsAlexander Frizell Santillo, Lena Skoglund, Maria Lindau, Karin Edebol Eeg-Olofsson, Metin Tovi, Henry Engler, Rose-Marie Brundin, Sofie Ingvast, Lars Lannfelt, Anna Glaser, Lena Kilander
JournalAlzheimer disease and associated disorders (Alzheimer Dis Assoc Disord) 2009 Jul-Sep Vol. 23 Issue 3 Pg. 298-300 ISSN: 1546-4156 [Electronic] United States
PMID19568152 (Publication Type: Case Reports, Journal Article)
Chemical References
  • Iron
Topics
  • Aged
  • Amyotrophic Lateral Sclerosis (physiopathology)
  • Brain (metabolism, pathology)
  • Diagnosis, Differential
  • Electromyography
  • Frontotemporal Dementia (physiopathology)
  • Humans
  • Iron (metabolism)
  • Magnetic Resonance Imaging
  • Male
  • Neuropsychological Tests
  • Pantothenate Kinase-Associated Neurodegeneration (diagnosis, pathology, physiopathology)
  • Pedigree
  • Positron-Emission Tomography

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