Nigrostriatal dopaminergic function in familial amyotrophic lateral sclerosis patients with and without copper/zinc superoxide dismutase mutations

Neurology
S PrzedborskiD Eidelberg

Abstract

Some cases of familial amyotrophic lateral sclerosis (FALS) are associated with copper/zinc superoxide dismutase (Cu/Zn-SOD) mutations, which are implicated in the death of motor neurons. Because Cu/Zn-SOD is present in high amounts in nigrostriatal dopaminergic neurons, we considered the possibility that FALS may be associated with subclinical nigrostriatal dopaminergic dysfunction. We used [18F]fluorodopa (FDOPA) and PET to study 14 FALS patients (50 +/- 11 years [mean +/- SD]): seven with (FALS-1) and seven without (FALS-0) Cu/Zn-SOD mutations. Fourteen age-matched normal volunteers (48 +/- 18 years) served as controls. Striato-occipital ratios (SORs) for the caudate and the putamen were calculated. Five of the 14 FALS patients had reduced striatal FDOPA uptake in the caudate nucleus, putamen, or both. Mean caudate SOR did not differ among FALS-1, FALS-0, and control subjects. Mean putamen SOR was significantly abnormal in FALS-0 but not in FALS-1 patients. These findings indicate that subclinical nigrostriatal dopaminergic dysfunction is present in some FALS patients and that FDOPA/PET abnormalities are more likely to be associated with FALS-0 status. This suggests that SOD mutations are less cytotoxic to dopaminergic than ...Continue Reading

Citations

Mar 29, 2002·Movement Disorders : Official Journal of the Movement Disorder Society·Pierre-François PradatVincent Meininger
Jun 29, 2012·Neurological Sciences : Official Journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology·Young-Min LimKwang-Kuk Kim
Apr 27, 1999·Brain Research. Cognitive Brain Research·P ViereggeR Verleger
Jun 21, 2014·BioMed Research International·Pierre-François Pradat, Mohamed-Mounir El Mendili
Dec 6, 2008·Amyotrophic Lateral Sclerosis : Official Publication of the World Federation of Neurology Research Group on Motor Neuron Diseases·Federica AgostaMassimo Filippi
Dec 2, 2005·Amyotrophic Lateral Sclerosis and Other Motor Neuron Disorders : Official Publication of the World Federation of Neurology, Research Group on Motor Neuron Diseases·L SalauzeM Geffard
Sep 8, 2009·Movement Disorders : Official Journal of the Movement Disorder Society·Pierre-François PradatVincent Meininger
Mar 16, 2011·NMR in Biomedicine·Khema R SharmaVaran Govind
Jan 26, 2012·Journal of Neuroimaging : Official Journal of the American Society of Neuroimaging·Khema R SharmaVaran Govind
Mar 13, 2003·Clinical Genetics·D Majoor-KrakauerA Hofman
Dec 10, 2016·European Journal of Nuclear Medicine and Molecular Imaging·Stefanie M A WillekensKoen Van Laere
Mar 19, 2019·Frontiers in Neurology·Sheena Chew, Nazem Atassi

❮ Previous
Next ❯

Related Concepts

Related Feeds

ALS: Genetics

Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disorder characterized by muscle weakness. Here is the latest research investigating genetic alterations in this genetically heterogeneous disorder.

ALS: Genetics

Amyotrophic Lateral Sclerosis (ALS) is a progressive neurodegenerative disorder characterized by muscle weakness. ALS is a genetically heterogeneous disorder with several causative genes. Here are the latest discoveries pertaining to the genetics of this disease.

Basal Ganglia

Basal Ganglia are a group of subcortical nuclei in the brain associated with control of voluntary motor movements, procedural and habit learning, emotion, and cognition. Here is the latest research.

Amyloid Lateral Sclerosis

Amyotrophic Lateral Sclerosis (ALS) is a progressive nervous system disease associated with the death of neurons that control voluntary muscles. Discover the latest research on ALS here.