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 Volume 59, Number 12, December 24, 2002
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Neurology 2002;59:1860-1865
© 2002 American Academy of Neurology

Middle interhemispheric variant of holoprosencephaly

A distinct cliniconeuroradiologic subtype

A.J. Lewis, MD PhD, E.M. Simon, MD, A.J. Barkovich, MD, N.J. Clegg, PhD, M.R. Delgado, MD, E. Levey, MD and J.S. Hahn, MD

From Stanford University School of Medicine and Lucile Packard Children’s Hospital (Drs. Lewis and Hahn), CA; Children’s Hospital of Philadelphia (Dr. Simon), PA; University of California at San Francisco (Dr. Barkovich); Texas Scottish Rite Hospital (Drs. Clegg and Delgado), Dallas; and Kennedy Krieger Institute (Dr. Levey), Baltimore, MD.

Address correspondence and reprint requests to Dr. Jin S. Hahn, Department of Neurology, A343, 300 Pasteur Drive, Stanford, CA 94305-5235; e-mail: jhahn{at}stanford.edu

Background: The middle interhemispheric variant (MIH) is a subtype of holoprosencephaly (HPE) in which the posterior frontal and parietal areas lack midline separation, whereas more polar areas of the cerebrum are fully cleaved. While the neuroradiologic features of this subtype have been recently detailed, the clinical features are largely unknown.

Objective: To present the clinical manifestations of MIH and to compare them with classic subtypes (alobar, semilobar, and lobar) of HPE.

Methods: The authors evaluated 15 patients with MIH in a multicenter study. Neuroimaging and clinical data were collected and correlated. They compared the data with those of 68 patients who had classic HPE.

Results: The frequency of endocrinopathy in MIH (0%) was lower compared with the classic subtypes (72%) (p < 0.0001). This correlated with the lack of hypothalamic abnormalities. The percentage of patients with seizures (40%) did not significantly differ from classic HPE. Spasticity was the most common motor abnormality, seen in 86% of MIH patients, similar to other subtypes. The frequency of choreoathetosis in MIH (0%) was lower than that for semilobar HPE (41%) (p < 0.0039). This correlated with the lack of caudate and lentiform nuclei abnormalities. Developmental functions, including mobility, upper-extremity function, and language, of the MIH group were similar to the least severe classic type, lobar HPE.

Conclusion: MIH is a recognizable variant of HPE with differing clinical prognosis. Similar to the lobar subtype by functional measures, MIH differs from classic HPE by the absence of endocrine dysfunction and choreoathetosis.




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