Search: onr:"swepub:oai:DiVA.org:uu-107562" >
Frontotemporal Deme...
Frontotemporal Dementia-amyotrophic Lateral Sclerosis Complex is Simulated by Neurodegeneration With Brain Iron Accumulation
-
- Santillo, Alexander (author)
- Uppsala universitet,Geriatrik,Uppsala Univ, Dept Publ Hlth Geriatr, Uppsala 75185, Sweden
-
- Skoglund, Lena (author)
- Uppsala universitet,Geriatrik,Uppsala Univ, Dept Publ Hlth Geriatr, Uppsala 75185, Sweden
-
- Lindau, Maria (author)
- Uppsala universitet,Geriatrik,Uppsala Univ, Dept Publ Hlth Geriatr, Uppsala 75185, Sweden
-
show more...
-
- Eeg-Olofsson, Karin (author)
- Uppsala universitet,Institutionen för neurovetenskap,Uppsala Univ, Dept Neurosci Clin Neurophysiol, Uppsala 75185, Sweden
-
- Tovi, Metin (author)
- Karolinska Univ Hosp, Dept Diagnost Radiol, Stockholm, Sweden
-
- Engler, Henry (author)
- Uppsala universitet,Institutionen för medicinska vetenskaper,Uppsala Univ, Dept Med Sci Clin Physiol, Uppsala 75185, Sweden
-
- Brundin, Rose-Marie (author)
- Uppsala universitet,Geriatrik,Uppsala Univ, Dept Publ Hlth Geriatr, Uppsala 75185, Sweden
-
- Ingvast, Sofie (author)
- Uppsala universitet,Geriatrik,Uppsala Univ, Dept Publ Hlth Geriatr, Uppsala 75185, Sweden
-
- Lannfelt, Lars (author)
- Uppsala universitet,Geriatrik,Uppsala Univ, Dept Publ Hlth Geriatr, Uppsala 75185, Sweden
-
- Glaser, Anna (author)
- Uppsala universitet,Geriatrik,Uppsala Univ, Dept Publ Hlth Geriatr, Uppsala 75185, Sweden
-
- Kilander, Lena (author)
- Uppsala universitet,Geriatrik,Uppsala Univ, Dept Publ Hlth Geriatr, Uppsala 75185, Sweden
-
show less...
-
(creator_code:org_t)
- 2009
- 2009
- English.
-
In: Alzheimer Disease and Associated Disorders. - 0893-0341 .- 1546-4156. ; 23:3, s. 298-300
- Related links:
-
https://urn.kb.se/re...
-
show more...
-
https://doi.org/10.1...
-
https://urn.kb.se/re...
-
show less...
Abstract
Subject headings
Close
- 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.
Subject headings
- MEDICIN OCH HÄLSOVETENSKAP -- Medicinska och farmaceutiska grundvetenskaper -- Neurovetenskaper (hsv//swe)
- MEDICAL AND HEALTH SCIENCES -- Basic Medicine -- Neurosciences (hsv//eng)
Keyword
- neurodegeneration with brain iron accumulation
- Hallervorden-Spatz disease
- frontotemporal dementia
- amyotrophic lateral sclerosis
- MEDICINE
- MEDICIN
Publication and Content Type
- ref (subject category)
- art (subject category)
Find in a library
To the university's database
- By the author/editor
-
Santillo, Alexan ...
-
Skoglund, Lena
-
Lindau, Maria
-
Eeg-Olofsson, Ka ...
-
Tovi, Metin
-
Engler, Henry
-
show more...
-
Brundin, Rose-Ma ...
-
Ingvast, Sofie
-
Lannfelt, Lars
-
Glaser, Anna
-
Kilander, Lena
-
show less...
- About the subject
-
- MEDICAL AND HEALTH SCIENCES
-
MEDICAL AND HEAL ...
-
and Basic Medicine
-
and Neurosciences
- Articles in the publication
-
Alzheimer Diseas ...
- By the university
-
Uppsala University
-
Högskolan Dalarna