The goals of this project are to understand how spinal circuits are used to coordinate normal movement in humans and how malfunctioning of spinal circuits contributes to abnormal movements in neurological disorders. We have particularly focused on disorders of voluntary movement with excessive muscle contraction, such as Stiffperson syndrome and spasticity. During FY2001, most of our efforts focused on defining clinical subtypes of primary lateral sclerosis, a degenerative disorder of corticospinal neurons that produces profound spasticity and slowness of movement. In a subset of these patients physiological measures showed that only the corticospinal tract is involved, while primitive motor pathways are preserved. The motor cortex is inexcitable relatively early in the course of the disease, when strength and muscle bulk are preserved. In contrast to amyotrophic lateral sclerosis, early hyperexcitability was not seen. The long term effects of corticospinal dysfunction on spinal excitability and motor neuron function are presently being assessed.
| 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 |
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