Magnetization transfer measurements of cerebral white matter in patients with myotonic dystrophy

To determine whether patients with myotonic dystrophy (MyD) have structural changes in the cerebral white matter, we performed magnetization transfer (MT) imaging of the cerebral white matter in 14 MyD patients and 11 age-matched normal controls. We calculated MT ratios in both the white matter lesions (WMLs) and the normal-appearing white matter (NAWM) of MyD patients using region of interest (ROI) analysis. MT ratios in WMLs were markedly decreased, and all ROIs in NAWM also showed significantly lower MT ratios in MyD patients than in normal controls. The average MT ratio of all ROIs in WMLs and NAWM in each patient showed a significant negative correlation with duration of illness, but not with the patient's age or age at onset. The results of the present study indicate not only the presence of pathological changes in WMLs but also the widespread involvement of NAWM in MyD patients. The results also suggest that structural changes in the white matter may be progressive during the clinical course of MyD.

[1]  K. Abe,et al.  The fluid-attenuated inversion-recovery pulse sequence in assessment of central nervous system involvement in myotonic dystrophy , 1998, Neuroradiology.

[2]  R I Grossman,et al.  Quantitative volumetric magnetization transfer analysis in multiple sclerosis: Estimation of macroscopic and microscopic disease burden , 1996, Magnetic resonance in medicine.

[3]  R. B. Wright,et al.  Central nervous system magnetic resonance imaging findings in myotonic dystrophy. , 1988, Archives of neurology.

[4]  C E Metz,et al.  The magnetization transfer effect in cerebral infarction. , 1994, AJNR. American journal of neuroradiology.

[5]  N. Martin-Duverneuil,et al.  Cranial MRI findings in myotonic dystrophy , 1997, Neuroradiology.

[6]  N. Otsuka,et al.  Presenile appearance of abundant Alzheimer's neurofibrillary tangles without senile plaques in the brain in myotonic dystrophy , 2004, Acta Neuropathologica.

[7]  S. Terae,et al.  Anterior temporal white matter lesions in myotonic dystrophy with intellectual impairment: an MRI and neuropathological study , 1998, Neuroradiology.

[8]  J. Mathieu,et al.  Myotonic dystrophy , 1992, Neurology.

[9]  M. Koch,et al.  The clinical and genetic correlates of MRI findings in myotonic dystrophy , 1996, Neuroradiology.

[10]  Kiyoharu Inoue,et al.  Neuropathological changes of the brain in myotonic dystrophy - some new observations , 1987, Journal of the Neurological Sciences.

[11]  K. Mizukami,et al.  An autopsy case of myotonic dystrophy with mental disorders and various neuropathologic features , 1999, Psychiatry and clinical neurosciences.

[12]  K. Boone,et al.  Cerebral abnormalities in myotonic dystrophy. Cerebral blood flow, magnetic resonance imaging, and neuropsychological tests. , 1993, Archives of neurology.

[13]  G B Pike,et al.  Measure of magnetization transfer in multiple sclerosis demyelinating plaques, white matter ischemic lesions, and edema. , 1996, AJNR. American journal of neuroradiology.

[14]  R S Balaban,et al.  Quantitative 1H magnetization transfer imaging in vivo , 1991, Magnetic resonance in medicine.

[15]  M Filippi,et al.  Normal-appearing white matter changes in multiple sclerosis: the contribution of magnetic resonance techniques , 1999, Multiple sclerosis.

[16]  K. Nagao,et al.  Intracytoplasmic inclusion bodies of the substantia nigra in myotonic dystrophy Immunohistochemical observations , 1997, Journal of the Neurological Sciences.

[17]  J. C. Vuletin,et al.  A Light and Electron Microscopic Study , 1976 .

[18]  K. Abe,et al.  Involvement of the central nervous system in myotonic dystrophy , 1994, Journal of the Neurological Sciences.

[19]  A. Culebras,et al.  Cytoplasmic inclusion bodies within neurons of the thalamus in myotonic dystrophy. A light and electron microscope study. , 1973, Journal of the neurological sciences.

[20]  Hirofumi Sakurai,et al.  Magnetization transfer ratio in cerebral white matter lesions of Binswanger’s disease , 1999, Journal of the Neurological Sciences.

[21]  J. Kissel,et al.  Magnetic resonance imaging and clinical correlates of intellectual impairment in myotonic dystrophy. , 1990, Archives of neurology.

[22]  M Takasaki,et al.  Diffusion-weighted magnetic resonance and magnetization transfer imaging in the assessment of ischemic human stroke. , 1998, Internal medicine.

[23]  Kiyoharu Inoue,et al.  Intracytoplasmic inclusion bodies of the thalamus and the substantia nigra, and Marinesco bodies in myotonic dystrophy: a quantitative morphological study , 2004, Acta Neuropathologica.

[24]  U. Salvolini,et al.  Brain involvement in myotonic dystrophy: MRI features and their relationship to clinical and cognitive conditions , 1994, Acta neurologica Scandinavica.

[25]  D. S. Wood,et al.  Criteria for establishing the validity of genetic recombination in myotonic dystrophy , 1989, Neurology.

[26]  M W Weiner,et al.  Magnetization transfer ratio of white matter hyperintensities in subcortical ischemic vascular dementia. , 1999, AJNR. American journal of neuroradiology.

[27]  M Filippi,et al.  A Magnetization Transfer Imaging Study of Normal-Appearing White Matter in Multiple Sclerosis , 1995, Neurology.

[28]  R I Grossman,et al.  Microscopic disease in normal-appearing white matter on conventional MR images in patients with multiple sclerosis: assessment with magnetization-transfer measurements. , 1995, Radiology.

[29]  R S Balaban,et al.  Magnetization transfer contrast in magnetic resonance imaging. , 1992, Magnetic resonance quarterly.

[30]  A. Delacourte,et al.  Specific tau variants in the brains of patients with myotonic dystrophy , 1996, Neurology.

[31]  R. Balaban,et al.  Magnetization transfer contrast (MTC) and tissue water proton relaxation in vivo , 1989, Magnetic resonance in medicine.

[32]  G S Francis,et al.  Combined magnetization transfer and proton spectroscopic imaging in the assessment of pathologic brain lesions in multiple sclerosis. , 1999, AJNR. American journal of neuroradiology.

[33]  W. Dorndorf,et al.  Magnetic resonance imaging of muscle and brain in myotonic dystrophy , 2004, Journal of Neurology.