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A familial syndrome of unilateral polymicrogyria affecting the right hemisphere

From the Department of Neurology (B.S.C., K.A.A.), Beth Israel Deaconess Medical Center and Harvard Medical School, Boston, MA; Department of Neurology (R.C.), Pediatric Hospital "Prof. Juan P. Garrahan," Buenos Aires, Argentina; Paediatric Neurology (J.H.C., A.M.), Institute of Child Health and Great Ormond Street Hospital, University College London, London, United Kingdom; Neurology and Pediatrics (R.D.J.), Medical College of Wisconsin, Milwaukee, WI; Departments of Radiology and Psychiatry and Laboratory of Clinical Neurophysiology (K.D.V.), University of São Paulo School of Medicine, São Paulo, Brazil; Pediatric Neuroradiology (A.J.B.), Department of Radiology, University of California San Francisco, San Francisco, CA; Howard Hughes Medical Institute and Department of Neurology (C.A.W.), Beth Israel Deaconess Medical Center and Harvard Medical School, Boston, MA.

Address correspondence and reprint requests to Dr. Bernard S. Chang, New Research Building 268B, 77 Avenue Louis Pasteur, Boston, MA 02115; e-mail: bchang{at}bidmc.harvard.edu

A number of familial syndromes of bilateral polymicrogyria (PMG) have been described, but reported unilateral PMG cases have generally been sporadic. The authors identified four families in which unilateral right-sided PMG on MRI was present in more than one individual, with pathologic confirmation in one. Core clinical features included contralateral hemiparesis, developmental delay, and focal seizures. The authors' findings suggest that unilateral PMG exists in a familial syndrome of probable germline genetic origin.

B.S.C. is supported by the National Institute of Neurologic Disorders and Stroke (K23 NS049159) and the Clinical Investigator Training Program (Beth Israel Deaconess Medical Center and Harvard-MIT Division of Health Science and Technology, in collaboration with Pfizer Inc.). C.A.W. is supported by the National Institute of Neurologic Disorders and Stroke (R37 NS35129) and is an Investigator of the Howard Hughes Medical Institute.

Disclosure: The authors report no conflicts of interest.

Received June 1, 2005. Accepted in final form September 28, 2005.

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