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NEUROLOGY 1992;42:179
© 1992 American Academy of Neurology

Olfactory dysgenesis or hypoplasia

A variant in the arhinencephaly spectrum?

David N. Louis, MD, Paulina V. Arriagada, MD, Bradley T. Hyman, MD, PhD and E. Tessa Hedley-Whyte, MD

From the Departments of Pathology (Neuropathology) and Neurology, Massachusetts General Hospital and Harvard Medical School, Boston, MA.

Postmortem examination of a 65-year-old anosmic woman revealed rudimentary olfactory bulbs, an imperforate cribriform plate, and sulcal abnormalities of the orbitofrontal surface of the brain. The remainder of the brain, skull, and body was normal. This abnormality most likely resulted from a local insult to the area of the cribriform plate during early fetal life, occurring after the induction of olfactory bulb differentiation, but before migration and complete differentiation of the olfactory bulbs. Such a lesion can be dated to approximately 11 to 12 weeks gestational age. The malformation may represent another variant in the arhinencephaly spectrum, but is termed "olfactory dysgenesis" or "olfactory hypoplasia" to separate it from true olfactory aplasia and arhinencephaly.

Address correspondence and reprint requests to Dr. David N. Louis, Department of Pathology (Neuropathology), Warren 3, Massachusetts General Hospital, Boston, MA 02114.

Received May 7, 1991. Accepted for publication in final form June 14, 1991.




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[Abstract] [PDF]


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