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Cortical and Subcortical Grey and White Matter Atrophy in Myotonic Dystrophies Type 1 and 2 Is Associated with Cognitive Impairment, Depression and Daytime Sleepiness.

Schneider-Gold C, Bellenberg B, Prehn C, Krogias C, Schneider R, Klein J, Gold R, Lukas C - PLoS ONE (2015)

Bottom Line: Volumes of brain, ventricles, cerebellum, brainstem, cervical cord, lesion load and VBM results of the patient groups were compared to 33 matched healthy subjects.In DM2, depression was associated with brainstem atrophy, Daytime sleepiness correlated with volume decrease in the middle cerebellar peduncles, pons/midbrain and the right medio-frontal cortex.GM and WM atrophy was significant in DM1 and DM2.

View Article: PubMed Central - PubMed

Affiliation: Department of Neurology, St. Josef-Hospital, Ruhr-University Bochum, Bochum, Germany.

ABSTRACT

Objectives: Central nervous system involvement is one important clinical aspect of myotonic dystrophy type 1 and 2 (DM1 and DM2). We assessed CNS involvement DM1 and DM2 by 3T MRI and correlated clinical and neuocognitive symptoms with brain volumetry and voxel-based morphometry (VBM).

Methods: 12 patients with juvenile or classical DM1 and 16 adult DM2 patients underwent 3T MRI, a thorough neurological and neuropsychological examination and scoring of depression and daytime sleepiness. Volumes of brain, ventricles, cerebellum, brainstem, cervical cord, lesion load and VBM results of the patient groups were compared to 33 matched healthy subjects.

Results: Clinical symptoms were depression (more pronounced in DM2), excessive daytime sleepiness (more pronounced in DM1), reduced attention and flexibility of thinking, and deficits of short-term memory and visuo-spatial abilities in both patient groups. Both groups showed ventricular enlargement and supratentorial GM and WM atrophy, with prevalence for more GM atrophy and involvement of the motor system in DM1 and more WM reduction and affection of limbic structures in DM2. White matter was reduced in DM1 in the splenium of the corpus callosum and in left-hemispheric WM adjacent to the pre- and post-central gyrus. In DM2, the bilateral cingulate gyrus and subgyral medio-frontal and primary somato-sensory WM was affected. Significant structural-functional correlations of morphological MRI findings (global volumetry and VBM) with clinical findings were found for reduced flexibility of thinking and atrophy of the left secondary visual cortex in DM1 and of distinct subcortical brain structures in DM2. In DM2, depression was associated with brainstem atrophy, Daytime sleepiness correlated with volume decrease in the middle cerebellar peduncles, pons/midbrain and the right medio-frontal cortex.

Conclusion: GM and WM atrophy was significant in DM1 and DM2. Specific functional-structural associations related morphological changes to cognitive impairment, depression and daytime sleepiness, partly indicating involvement of complex neuronal networks.

No MeSH data available.


Related in: MedlinePlus

ESS and brain grey and white matter in DM2.a) Significant negative correlations between GM and WM and Epworth sleepiness score in 15 DM2 patients detected by voxelwise multiple regression analysis. Age was used as a covariate of no interest. Clusters with FWE corrected cluster p-values < 0.07 are depicted; MNI coordinates: negative X-values reflect left side and positive X-values right sided location. b) Scatterplot of global brain GM and WM in dependency on ESS; the lines and annotations reflect the results of the linear regression analysis.
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pone.0130352.g004: ESS and brain grey and white matter in DM2.a) Significant negative correlations between GM and WM and Epworth sleepiness score in 15 DM2 patients detected by voxelwise multiple regression analysis. Age was used as a covariate of no interest. Clusters with FWE corrected cluster p-values < 0.07 are depicted; MNI coordinates: negative X-values reflect left side and positive X-values right sided location. b) Scatterplot of global brain GM and WM in dependency on ESS; the lines and annotations reflect the results of the linear regression analysis.

Mentions: DM patients in both disease groups suffered from distinctive daytime sleepiness (Table 1). Although DM1 patients were more affected, correlation analyses between the global CNS volumes and the ESS in both groups revealed weak negative associations between global GM and WM and the ESS only in the DM2 group (Spearman correlation: GM ρ = -0.392, P = 0.148; WM ρ = -0.460, P = 0.084, and Fig 4b).


Cortical and Subcortical Grey and White Matter Atrophy in Myotonic Dystrophies Type 1 and 2 Is Associated with Cognitive Impairment, Depression and Daytime Sleepiness.

Schneider-Gold C, Bellenberg B, Prehn C, Krogias C, Schneider R, Klein J, Gold R, Lukas C - PLoS ONE (2015)

ESS and brain grey and white matter in DM2.a) Significant negative correlations between GM and WM and Epworth sleepiness score in 15 DM2 patients detected by voxelwise multiple regression analysis. Age was used as a covariate of no interest. Clusters with FWE corrected cluster p-values < 0.07 are depicted; MNI coordinates: negative X-values reflect left side and positive X-values right sided location. b) Scatterplot of global brain GM and WM in dependency on ESS; the lines and annotations reflect the results of the linear regression analysis.
© Copyright Policy
Related In: Results  -  Collection

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getmorefigures.php?uid=PMC4482602&req=5

pone.0130352.g004: ESS and brain grey and white matter in DM2.a) Significant negative correlations between GM and WM and Epworth sleepiness score in 15 DM2 patients detected by voxelwise multiple regression analysis. Age was used as a covariate of no interest. Clusters with FWE corrected cluster p-values < 0.07 are depicted; MNI coordinates: negative X-values reflect left side and positive X-values right sided location. b) Scatterplot of global brain GM and WM in dependency on ESS; the lines and annotations reflect the results of the linear regression analysis.
Mentions: DM patients in both disease groups suffered from distinctive daytime sleepiness (Table 1). Although DM1 patients were more affected, correlation analyses between the global CNS volumes and the ESS in both groups revealed weak negative associations between global GM and WM and the ESS only in the DM2 group (Spearman correlation: GM ρ = -0.392, P = 0.148; WM ρ = -0.460, P = 0.084, and Fig 4b).

Bottom Line: Volumes of brain, ventricles, cerebellum, brainstem, cervical cord, lesion load and VBM results of the patient groups were compared to 33 matched healthy subjects.In DM2, depression was associated with brainstem atrophy, Daytime sleepiness correlated with volume decrease in the middle cerebellar peduncles, pons/midbrain and the right medio-frontal cortex.GM and WM atrophy was significant in DM1 and DM2.

View Article: PubMed Central - PubMed

Affiliation: Department of Neurology, St. Josef-Hospital, Ruhr-University Bochum, Bochum, Germany.

ABSTRACT

Objectives: Central nervous system involvement is one important clinical aspect of myotonic dystrophy type 1 and 2 (DM1 and DM2). We assessed CNS involvement DM1 and DM2 by 3T MRI and correlated clinical and neuocognitive symptoms with brain volumetry and voxel-based morphometry (VBM).

Methods: 12 patients with juvenile or classical DM1 and 16 adult DM2 patients underwent 3T MRI, a thorough neurological and neuropsychological examination and scoring of depression and daytime sleepiness. Volumes of brain, ventricles, cerebellum, brainstem, cervical cord, lesion load and VBM results of the patient groups were compared to 33 matched healthy subjects.

Results: Clinical symptoms were depression (more pronounced in DM2), excessive daytime sleepiness (more pronounced in DM1), reduced attention and flexibility of thinking, and deficits of short-term memory and visuo-spatial abilities in both patient groups. Both groups showed ventricular enlargement and supratentorial GM and WM atrophy, with prevalence for more GM atrophy and involvement of the motor system in DM1 and more WM reduction and affection of limbic structures in DM2. White matter was reduced in DM1 in the splenium of the corpus callosum and in left-hemispheric WM adjacent to the pre- and post-central gyrus. In DM2, the bilateral cingulate gyrus and subgyral medio-frontal and primary somato-sensory WM was affected. Significant structural-functional correlations of morphological MRI findings (global volumetry and VBM) with clinical findings were found for reduced flexibility of thinking and atrophy of the left secondary visual cortex in DM1 and of distinct subcortical brain structures in DM2. In DM2, depression was associated with brainstem atrophy, Daytime sleepiness correlated with volume decrease in the middle cerebellar peduncles, pons/midbrain and the right medio-frontal cortex.

Conclusion: GM and WM atrophy was significant in DM1 and DM2. Specific functional-structural associations related morphological changes to cognitive impairment, depression and daytime sleepiness, partly indicating involvement of complex neuronal networks.

No MeSH data available.


Related in: MedlinePlus