Dataset Information

Nigral iron deposition in common tremor disorders.

ABSTRACT:

Objective

We investigated R2* relaxation rates as a marker of iron content in the substantia nigra in patients with common tremor disorders and explored their diagnostic properties.

Methods

Mean nigral R2* rates were measured in 40 patients with tremor-dominant Parkinson's disease (PD), 15 with tremor in dystonia, 25 with essential tremor, and 25 healthy controls.

Results

Tremor-dominant PD patients had significantly higher nigral R2* values (34.1?±?5.7) than those with tremor in dystonia (30.0?±?3.9), essential tremor (30.6?±?4.8), and controls (30.0?±?2.8). An R2* threshold of 31.15 separated tremor-dominant PD from controls with a sensitivity and specificity of 67.5% and 72%. The sensitivity and specificity for discrimination between PD and non-PD tremor patients was 67.5% and 60%.

Conclusion

Iron content in the substantia nigra is significantly higher in tremor-dominant PD than in tremor in dystonia, essential tremor, and controls. Because of the considerable overlap, nigral R2* cannot be suggested as a useful diagnostic tool. © 2018 The Authors. Movement Disorders published by Wiley Periodicals, Inc. on behalf of International Parkinson and Movement Disorder Society.

SUBMITTER: Homayoon N

PROVIDER: S-EPMC6590652 | biostudies-literature | 2019 Jan

REPOSITORIES: biostudies-literature

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Publications

Nigral iron deposition in common tremor disorders.

Homayoon Nina N Pirpamer Lukas L Franthal Sebastian S Katschnig-Winter Petra P Kögl Mariella M Seiler Stephan S Wenzel Karoline K Hofer Edith E Deutschmann Hannes H Fazekas Franz F Langkammer Christian C Ropele Stefan S Schmidt Reinhold R Schwingenschuh Petra P

Movement disorders : official journal of the Movement Disorder Society 20181210 1

<h4>Objective</h4>We investigated R2* relaxation rates as a marker of iron content in the substantia nigra in patients with common tremor disorders and explored their diagnostic properties.<h4>Methods</h4>Mean nigral R2* rates were measured in 40 patients with tremor-dominant Parkinson's disease (PD), 15 with tremor in dystonia, 25 with essential tremor, and 25 healthy controls.<h4>Results</h4>Tremor-dominant PD patients had significantly higher nigral R2* values (34.1 ± 5.7) than those with tre ...[more]

PMID: 30536988

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Project description:Disruption of cerebral iron regulation appears to have a role in aging and in the pathogenesis of various neurodegenerative disorders. Possible unfavorable impacts of iron accumulation include reactive oxygen species generation, induction of ferroptosis, and acceleration of inflammatory changes. Whole-brain iron-sensitive magnetic resonance imaging (MRI) techniques allow the examination of macroscopic patterns of brain iron deposits in vivo, while modern analytical methods ex vivo enable the determination of metal-specific content inside individual cell-types, sometimes also within specific cellular compartments. The present review summarizes the whole brain, cellular, and subcellular patterns of iron accumulation in neurodegenerative diseases of genetic and sporadic origin. We also provide an update on mechanisms, biomarkers, and effects of brain iron accumulation in these disorders, focusing on recent publications. In Parkinson's disease, Friedreich's disease, and several disorders within the neurodegeneration with brain iron accumulation group, there is a focal siderosis, typically in regions with the most pronounced neuropathological changes. The second group of disorders including multiple sclerosis, Alzheimer's disease, and amyotrophic lateral sclerosis shows iron accumulation in the globus pallidus, caudate, and putamen, and in specific cortical regions. Yet, other disorders such as aceruloplasminemia, neuroferritinopathy, or Wilson disease manifest with diffuse iron accumulation in the deep gray matter in a pattern comparable to or even more extensive than that observed during normal aging. On the microscopic level, brain iron deposits are present mostly in dystrophic microglia variably accompanied by iron-laden macrophages and in astrocytes, implicating a role of inflammatory changes and blood-brain barrier disturbance in iron accumulation. Options and potential benefits of iron reducing strategies in neurodegeneration are discussed. Future research investigating whether genetic predispositions play a role in brain Fe accumulation is necessary. If confirmed, the prevention of further brain Fe uptake in individuals at risk may be key for preventing neurodegenerative disorders.

Dataset Information

Nigral iron deposition in common tremor disorders.

Objective

Methods

Results

Conclusion

Publications

Nigral iron deposition in common tremor disorders.

Similar Datasets

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