G.P.14.15 Brain involvement in myotonic dystrophy type 2

Romeo, Vincenzo; Palmieri, Arianna; Squarzanti, F.; Ferrati, C.; Zucchetta, Pietro; Manara, Renzo; Trevisan, Caterina; Pegoraro, Elena; Angelini, Corrado

doi:10.1016/j.nmd.2007.06.324

Cited by 1 publication

(4 citation statements)

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“…By demonstrating changes in brainstem and corpus callosum our results extend the previously described cerebral involvement in DM2 [9–11, 15–17, 19, 24]. Earlier findings of global atrophy in DM2 patients, as estimated with the BPF, were confirmed by our data [10].…”

Section: Discussionsupporting

confidence: 91%

“…White matter alterations, mostly depicted on T2- or FLAIR-weighted images, are well known in DM2 patients [9, 11, 19]. The reductions, seen in the corpus callosum on T1-weighted images, further extend the spectrum of white matter involvement in DM2.…”

Section: Discussionmentioning

confidence: 99%

“…It is conceivable that white matter changes and reduced callosal size represent a morphological substrate of fatigue, cognitive dysfunction, and neuropsychological abnormalities in DM2 as well [16, 19, 21]. …”

Section: Discussionmentioning

confidence: 99%

“…Although neuromuscular symptoms predominate, there is clinical, neuropathological, and imaging evidence of cerebral involvement in DM2 [9–11, 17, 19, 24]. …”

Section: Introductionmentioning

confidence: 99%

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Grey and white matter loss along cerebral midline structures in myotonic dystrophy type 2

Minnerop

Lüders²,

Specht

et al. 2008

J Neurol

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Myotonic dystrophy type 2 (DM2) is an autosomal dominantly inherited multisystemic disorder and a common cause of muscular dystrophy in adults. Although neuromuscular symptoms predominate, there is clinical and imaging evidence of cerebral involvement. We used voxel-based morphometry (VBM) based on T1-weighted magnetic resonance images to investigate brain morphology in 13 DM2 patients in comparison to 13 sex-and age-matched controls. Further, we employed novel computational surface-based methods that specifically assess callosal thickness. We found grey and white matter loss along cerebral midline structures in our patient group. Grey matter reductions were present in brainstem and adjacent hypothalamic and thalamic regions, while white matter was mainly reduced in corpus callosum. The reduced callosal size was highly significant and independently confirmed by different methods. Our data provide first evidence for grey and white matter loss along brain midline structures in DM2 patients. The reduced size of the corpus callosum further extends the spectrum of white matter changes in DM2 and may represent the morphological substrate of neuropsychological abnormalities previously described in this disorder.

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Section: Discussionsupporting

confidence: 91%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

“…Although neuromuscular symptoms predominate, there is clinical, neuropathological, and imaging evidence of cerebral involvement in DM2 [9–11, 17, 19, 24]. …”

Section: Introductionmentioning

confidence: 99%

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