The goal of this project is to determine the control and organization of human spinal circuits that help coordinate movement and to understand possible malfunctioning of these circuits in movement disorders, particularly those with excessive muscle contraction: Stiffperson syndrome, dystonia, and spasticity. Progress was made in three areas during FY2000. First, we found that the motor cortex is hyperexcitable in patients with Stiffperson syndrome, using the technique of paired pulse transcranial magnetic stimulation. This may reflect loss of inhibitory cortical interneurons in this disorder. We found that vibration, a stimulus that induces low frequency depression of the Ia stretch receptor afferents, produces increased cortical potentials in normal subjects only when it is sufficient to produce an increase in spinal excitability. This lays the groundwork to study why patients with focal dystonia are particularly sensitivity to vibration: is it caused by alterations in processing sensory inputs at the cortical or spinal level? Lastly, we have begun assessing whether patients with spasticity exhibit changes in low frequency depression of stretch reflexes, with a special emphasis on disorders with corticospinal degeneration.

Agency
National Institute of Health (NIH)
Institute
National Institute of Neurological Disorders and Stroke (NINDS)
Type
Intramural Research (Z01)
Project #
1Z01NS002976-02
Application #
6432941
Study Section
(OCD)
Project Start
Project End
Budget Start
Budget End
Support Year
2
Fiscal Year
2000
Total Cost
Indirect Cost
City
State
Country
United States
Zip Code
Cardenas, Agustin M; Sarlls, Joelle E; Kwan, Justin Y et al. (2017) Pathology of callosal damage in ALS: An ex-vivo, 7 T diffusion tensor MRI study. Neuroimage Clin 15:200-208
Schanz, Olivia; Bageac, Devin; Braun, Laura et al. (2016) Cortical hyperexcitability in patients with C9ORF72 mutations: Relationship to phenotype. Muscle Nerve 54:264-9
Meoded, Avner; Morrissette, Arthur E; Katipally, Rohan et al. (2015) Cerebro-cerebellar connectivity is increased in primary lateral sclerosis. Neuroimage Clin 7:288-96
Statland, Jeffrey M; Barohn, Richard J; Dimachkie, Mazen M et al. (2015) Primary Lateral Sclerosis. Neurol Clin 33:749-60
Floeter, Mary Kay; Katipally, Rohan; Kim, Meredith P et al. (2014) Impaired corticopontocerebellar tracts underlie pseudobulbar affect in motor neuron disorders. Neurology 83:620-7
Su, Zhaoming; Zhang, Yongjie; Gendron, Tania F et al. (2014) Discovery of a biomarker and lead small molecules to target r(GGGGCC)-associated defects in c9FTD/ALS. Neuron 83:1043-50
Flynn, Lauren; Stephen, Matthew; Floeter, Mary Kay (2014) Disease spread through contiguity and axonal tracts in primary lateral sclerosis. Muscle Nerve 49:439-41
Peters, Tracy L; Floeter, Mary Kay (2009) Usage of support services in primary lateral sclerosis. Amyotroph Lateral Scler 10:187-91
Floeter, Mary Kay; Mills, Reversa (2009) Progression in primary lateral sclerosis: a prospective analysis. Amyotroph Lateral Scler 10:339-46
Lupu, Vitalie D; Danielian, Laura; Johnsen, Jacqueline A et al. (2008) Physiology of the motor cortex in polio survivors. Muscle Nerve 37:177-82

Showing the most recent 10 out of 23 publications