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NEUROLOGY 2009;73:606-611
© 2009 American Academy of Neurology

Motor cortex and thalamic atrophy in Unverricht–Lundborg disease

Voxel-based morphometric study

P. Koskenkorva, MD, J. Khyuppenen, MD, PhD, E. Niskanen, MSc, M. Könönen, MSc, P. Bendel, MD, E. Mervaala, MD, PhD, A. E. Lehesjoki, MD, PhD, R. Kälviäinen, MD, PhD and R. Vanninen, MD, PhD

From the Departments of Clinical Radiology (P.K., M.K., P.B., R.V.), Neurology (J.K., R.K.), Clinical Neurophysiology (E.N., M.K., E.M.), and Physics (E.N.), Kuopio University Hospital and University of Kuopio, and Folkhälsan Institute of Genetics, Department of Medical Genetics and Neuroscience Center (A.E.L.), University of Helsinki, Finland.

Address correspondence and reprint requests to Dr. Päivi Koskenkorva, Department of Clinical Radiology, Kuopio University Hospital, Puijonlaaksontie 2, FIN-70210 Kuopio, Finland paivi.koskenkorva{at}kuh.fi

Objective: To evaluate possible changes in the gray matter volume of patients with Unverricht–Lundborg disease (EPM1) compared with healthy controls.

Methods: Thirty-four genetically verified patients with EPM1 and 30 healthy controls matched for age and sex underwent MRI (T1-, T2-, fluid-attenuated inversion recovery-, and T1-weighted 3-dimensional images). T1-weighted 3-dimensional images were analyzed with voxel-based morphometry (VBM) to compare the regional differences in gray matter volumes between patients and controls. The patients with EPM1 were also clinically evaluated for myoclonus severity using the Unified Myoclonus Rating Scale.

Results: VBM analysis revealed atrophy in the bilateral primary, premotor, and supplementary motor cortex. The thalamus and precuneus were also bilaterally affected. No infratentorial changes were detected in the group analysis.

Conclusion: The cortical motor areas of the brain are particularly affected in EPM1, correlating with the motor symptoms of this disease. The combination of detailed imaging with neurophysiologic evaluation may help to reveal the pathogenesis of Unverricht–Lundborg disease.

Abbreviations: AED = antiepileptic drug; GM = gray matter; ICV = intracranial volume; JME = juvenile myoclonic epilepsy; MNI = Montreal Neurological Institute; NA = not available; PME = progressive myoclonus epilepsy; TMS = transcranial magnetic stimulation; UMRS = Unified Myoclonus Rating Scale; VBM = voxel-based morphometry; WM = white matter.

The study was funded by the Academy of Finland/the NEURO Research Programme, Vaajasalo Foundation, and UCB Pharma SA with unrestricted scientific grants. The sponsors had no role in design or conduct of the study; collection, management, analysis, or interpretation of the data; or preparation, review, or approval of the manuscript.

Disclosure: Author disclosures are provided at the end of the article.

Received November 11, 2008. Accepted in final form May 27, 2009.






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