At the heart of primary progressive multiple sclerosis: three cases with diffuse MRI abnormalities only

Abstract

We present three patients with a clinical course and cerebrospinal fluid findings consistent with a diagnosis of primary progressive multiple sclerosis (PPMS). Extensive and repeated magnetic resonance imaging (MRI) examinations showed only diffuse abnormality in brain and spinal cord, but no focal lesions. We propose that these cases represent the most pure form of PPMS, even though according to currently applied criteria this diagnosis can not be made in the absence of focal lesions on MRI. Multiple Sclerosis 2008; 14: 428—430. http://msj.sagepub.com

Keywords

MRI primary progressive multiple sclerosis

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References

Poser CM , Paty DW , Scheinberg L. , McDonald WI , Davis FA , Ebers GC et al. New diagnostic criteria for multiple sclerosis: guidelines for research protocols . Ann Neurol 1983; 13: 227-31.

McDonald WI , Compston A. , Edan G. , Goodkin D. , Hartung HP , Lublin FD et al. Recommended diagnostic criteria for multiple sclerosis: guidelines from the international panel on the diagnosis of multiple sclerosis . Ann Neurol 2001; 50: 121-7.

Polman CH , Reingold SC , Edan G. , Filippi M. , Hartung HP , Kappos L. Diagnostic criteria for multiple sclerosis: 2005 revisions to the `McDonald criteria' . Ann Neurol 2005; 58: 840-6.

Lycklama à Nijeholt GJ , Barkhof F. , Scheltens P. , Castelijns JA , Ader H. et al. MR of the spinal cord in multiple sclerosis: relation to clinical subtype and disability . AJNR Am J Neuroradiol 1997; 18: 1041-8.

Lycklama à Nijeholt GJ , Castelijns JA , Lazeron RHC , van Waesberghe JH , Polman CH , Uitdehaag BM et al. Magnetization transfer ratio of the spinal cord in multiple sclerosis: relationship to atrophy and neurologic disability . J Neuroimaging 2000; 10: 67-72.

Bot JCJ , Barkhof F. , Polman CH , Lycklama a Nijeholt GJ , de Groot V. , Bergers E. et al. Spinal cord abnormalities in recently diagnosed MS patients . Neurology 2004; 62: 226-33.

Fu L. , Matthews PM , De Stefano N. , Worsley KJ , Narayanan S. , Francis GS et al. Imaging axonal damage of normal-appearing white matter in multiple sclerosis . Brain 1998; 121: 103-13.

Filippi M. , Rovaris M. , Rocca MA Imaging primary progressive multiple sclerosis: the contribution of structural, metabolic, and functional MRI techniques . Mult Scler 2004; 10: S36-44.

Thompson AJ , Montalban X. , Barkhof F. , Brochet B. , Filippi M. , Miller DH et al. Diagnostic criteria for primary progressive multiple sclerosis: a position paper . Ann Neurol 2000; 47: 831-5.

10.

Thompson AJ , Polman CH , Miller DH , McDonald WI , Brochet B. , Filippi M. et al. Primary progressive multiple sclerosis . Brain 1997 ; 120: 1085-96.

11.

Lycklama à Nijeholt GJ , Bergers E. , Kamphorst W. , Bot J. , Nicolay K. , Castelijns JA et al. Post-mortem high-resolution MRI of the spinal cord in multiple sclerosis - a correlative study with conventional MRI, histopathology and clinical phenotype . Brain 2001; 124: 154-66.

12.

Bergers E. , Bot JCJ , van der Valk P. , Castelijns JA , Lycklama a Nijeholt GJ , Kamphorst W. et al. Diffuse signal abnormalities in the spinal cord in multiple sclerosis: direct postmortem in situ magnetic resonance imaging correlated with in vitro high-resolution magnetic resonance imaging and histopathology . Ann Neurol 2002; 51: 652-6.

13.

Kutzelnigg A. , Lucchinetti CF , Stadelmann C. , Bruck W. , Rauschka H. , Bergmann M. et al. Cortical demyelination and diffuse white matter injury in multiple sclerosis . Brain 2005; 128: 2705-12.

14.

De Stefano N. , Narayanan S. , Francis SJ , Smith S. , Mortilla M. , Tartaglia MC et al. Diffuse axonal and tissue injury in patients with multiple sclerosis with low cerebral lesion load and no disability . Arch Neurol 2002; 59: 1565-71.

15.

Filippi M. , Bozzali M. , Rovaris M. , Gonen O. , Kesavadas C. , Ghezzi A. et al. Evidence for widespread axonal damage at the earliest clinical stage of multiple sclerosis . Brain 2003; 126: 433-7.

16.

Rocca MA , Iannucci G. , Rovaris M. , Comi G. , Filippi M. Occult tissue damage in patients with primary progressive multiple sclerosis is independent of T2-visible lesions - a diffusion tensor MR study . J Neurol 2003; 250: 456-60.