Altered power spectral density in the resting-state sensorimotor network in patients with myotonic dystrophy type 1

Abstract

Myotonic dystrophy type 1 (DM1) is a multisystemic disease that involves the brain with several neurological symptoms. Although there were few imaging studies on DM1, no studies have investigated functional alterations in the sensorimotor network at rest in patients with DM1. In the current study, a power spectral density (PSD) analysis of resting-state fMRI data was performed to assess possible alteration in spontaneous neural activity of the sensorimotor network in patients with DM1. Compared to healthy controls, patients with DM1 showed higher PSD responses in the orbitofrontal cortex, parahippocampus and basal ganglia (corrected P < 0.05). Patients with DM1 showed higher PSD responses in white matter structures associated with motor function (corrected P < 0.05). Furthermore, correlation analysis indicated that the brain regions showing PSD differences were correlated with measures of motor performance (P < 0.05). In gray matter, our findings suggest that motor disability in DM1 is not an isolated deterioration of the motor power but a multimodal dysfunction that also involves the visual system. In addition, the widespread PSD alteration in white matter structures suggest that motor deficits in DM1 involve motor movement structures as well as structures important for its coordination and regulation.

Current Progress in CNS Imaging of Myotonic Dystrophy

“…It seems more convincing that disturbed networks build the functional and structural substrate of clinical symptoms observed in DM1 as already seen in other neuropsychiatric diseases. Consecutively, structural and functional network analyses revealed altered functional connectivity within the default mode network in relation to personality traits/disorders in DM1, altered Theory of Mind network in relation to social cognition and detection of connectivity patterns observed in autism spectrum disorders and impaired sensorimotor networks associated with motor performance, CTG repeat length and disease duration ( 36 , 75 , 80 , 82 ).…”

“…The most recent functional MRI study by Park et al investigated power spectral density (PSD) in the resting-state sensorimotor network in DM1 patients ( 75 ). In contrast to functional connectivity analysis based on the correlation analysis between different brain regions, they applied power spectral density (PSD) analysis.…”

Functional anomaly mapping reveals local and distant dysfunction caused by brain lesions

“…This may help to clarify the nature of the signal, and may also provide critical evidence regarding the brain basis of stroke recovery, the basis of day-to-day fluctuations in behavioral performance, and also the basis of behavioral response to neurorehabilitation, medications, and neuromodulation. Finally, efforts have been made to use resting BOLD data to identify functional changes in several clinical populations, often examining changes in resting state connectivity (He et al, 2007;Liu et al, 2018;Nair et al, 2015;Park et al, 2018;Ranasinghe et al, 2017); likewise, the technique described here may be applied to other populations without gross anatomical damage to identify functional anomalies in individuals with neurodegenerative disorders, psychiatric disorders, developmental disorders, or other clinical populations. Beyond clinical applications, this technique could also potentially be used to measure development or skill acquisition (e.g., literacy) or examine any source of individual difference in brain function, such as sensory experience (e.g., blind or deaf populations), multilingualism, or socioeconomic status.…”

Advances in imaging of brain abnormalities in neuromuscular disease

“…These findings suggest that motor disability in DM1 is not an isolated deterioration of structures involved in motor coordination and regulation but also a multimodal dysfunction that includes the visual system. 94…”