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Original Articles

Bilateral limbic system destruction in man

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Pages 88-106 | Received 19 Mar 2009, Accepted 22 May 2009, Published online: 01 Feb 2010
 

Abstract

We report here a case study of a rare neurological patient with bilateral brain damage encompassing a substantial portion of the so-called “limbic system.” The patient, Roger, has been studied in our laboratory for over 14 years, and the current article presents his complete neuroanatomical and neuropsychological profiles. The brain damage occurred in 1980 following an episode of herpes simplex encephalitis. The amount of destroyed neural tissue is extensive and includes bilateral damage to core limbic and paralimbic regions, including the hippocampus, amygdala, parahippocampal gyrus, temporal poles, orbitofrontal cortex, basal forebrain, anterior cingulate cortex, and insular cortex. The right hemisphere is more extensively affected than the left, although the lesions are largely bilateral. Despite the magnitude of his brain damage, Roger has a normal IQ, average to above-average attention, working memory, and executive functioning skills, and very good speech and language abilities. In fact, his only obvious presenting deficits are a dense global amnesia and a severe anosmia and ageusia. Roger's case presents a rare opportunity to advance our understanding of the critical functions underlying the human limbic system, and the neuropsychological and neuroanatomical data presented here provide a critical foundation for such investigations.

We are greatly indebted to Roger and his family for their unwavering support and continued commitment to brain research. We would also like to thank Ned N. Tranel, Melissa Duff, Ken Manzel, Kathy Jones, Ruth Henson, Nick Jones, Jocelyn Cole, Tim Salomons, Laura Frey Law, Jennifer Lee, Carol Vance, India Morrison, Satish Rao, and the many other collaborators who played an integral part in testing Roger.

Supported by National Institute of Neurological Disorders and Stroke (NINDS) P01 NS19632, National Institute on Drug Abuse (NIDA) R01 DA022549, National Science Foundation (NSF) Graduate Research Fellowship, and the Kiwanis Foundation.

Notes

1Hemoglobin A1c, a measure of glycosylated hemoglobin, gives an accurate estimate of blood glucose levels during the previous three to four months. Hemoglobin A1c values of around 7% or lower in Type 1 diabetics are indicative of good glucose control and have been associated with a lower occurrence of peripheral neuropathy (CitationDiabetes Control and Complications Trial Research Group, 1993).

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