Published online July 1, 2005
PEDIATRICS Vol. 116 No. 1 July 2005, pp. 242-245 (doi:10.1542/peds.2004-0425)

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EXPERIENCE AND REASON

Living a Normal Life With the Nondominant Hemisphere: Magnetic Resonance Imaging Findings and Clinical Outcome for a Patient With Left-Hemispheric Hydranencephaly

Stephan Ulmer, MD^*, Friederike Moeller, MD^*, Marc A. Brockmann, MD, Johann P. Kuhtz-Buschbeck, MD, PhD, Ulrich Stephani, MD, PhD^|| and Olav Jansen, MD, PhD^*

^* Section of Neuroradiology, Department of Neurosurgery
^|| Department of Neuropediatrics, University Hospital of Schleswig-Holstein, Kiel, Germany
Institute of Radiology, University Hospital of Schleswig-Holstein, Luebeck, Germany
Institute of Physiology, Christian Albrechts University, Kiel, Germany

In hemihydranencephaly, the human brain lacks 1 complete hemisphere. An occlusion of the carotid artery, affecting all supplied territories, is thought to be the underlying mechanism. This extremely rare disorder, of which only 7 cases have been reported to date, is thought to occur before the last trimester of gestation (20th to 27th week), after neural migration but before synaptogenesis. We report on a 36-year-old man born at term, with no complications, from nonconsanguineous healthy parents. Cranial computed tomography had been performed because of left-sided headaches. Because of the imaging findings, the patient presented at our institution for additional MRI and clinical testing (including the Motor Activity Log, Wolf motor function test, 2-point discrimination test, Purdue pegboard test, gross motor function test, Physician Rating Scale, and Aachener aphasia test, including patterns for spontaneous speech, repetition, naming, comprehension, written language, and the token test). The patient's disabilities were related to deficits in fine motor control and reduced precision. Therefore, the patient was unable to perform the Purdue pegboard test with his affected hand. According to the Aachener aphasia test, no aphasia could be demonstrated for this strongly left-handed patient. Strong mirror movements were found. Cortical reorganization is possible if damage occurs in very early childhood. Motor function and speech were controlled by the remaining, nonaffected hemisphere, with a remarkable outcome. Because the damage is thought to occur before synaptogenesis, existing or prepared cortical areas and pathways have the potential to execute the lacking functions of the destroyed hemisphere.

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Key Words: adult outcomes • brain development • brain imaging • cerebral infarction • functional assessment • children

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Accepted Oct 14, 2004.

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This article has been cited by other articles:

				E. L. Altschuler, B. Matsumura, J. Capoor, K. Weinshelbaum, and H. Ghuznavi Left-Hemispheric Hydranencephaly With Less Favorable Findings Pediatrics, December 1, 2005; 116(6): 1603 - 1604. [Full Text] [PDF]

				S. Ulmer and O. Jansen Left-Hemispheric Hydranencephaly With Less Favorable Findings: In Reply Pediatrics, December 1, 2005; 116(6): 1604 - 1604. [Full Text] [PDF]