Personal reflections on the neuropathology of kuru

Abstract

My involvement with kuru began towards the end of 1963 while I was a Fellow at the Massachusetts General Hospital (MGH). During the autumn of that year I was contacted by Dr Michael Alpers, who was on his way to Boston from the National Institutes of Health (NIH) in Bethesda, Maryland with a carload of New Guinean artefacts to be delivered to the Peabody Museum in Salem, Massachusetts on behalf of Dr Carleton Gajdusek. We had been students together at Adelaide University and St Mark's College in the 1950s and our friendship was thus rekindled.Sometime after this I was approached by representatives of NIH to investigate the Parkinsonism-dementia (PD) and amyotrophic lateral sclerosis (ALS) neurological complex prevalent on Guam, where it was the main cause of death among the Chamorro people. At that time, the cause of this devastating neurological complex was subject to a great deal of conjecture. These discussions led to visits to Bethesda, where I met Carleton and Joe Gibbs. This was at the exciting time when the first chimpanzees developed symptoms of kuru. Lifetime friendships were thus established with Carleton and Joe and with Jake Brody, as well as with Dr Asao Hirano in New York, who was the first to describe the neuropathology of the Guam disorders.As I was committed to the University of Western Australia, being on leave from an academic post in the medical school and feeling that I had a mission to set up neuropathology in my home country, I was not able to pursue the very attractive Guam opportunity and returned to Perth in 1965.However, earlier in that year, while deeply involved in a very busy schedule at Harvard Medical School working with my mentor in muscle diseases Dr Raymond D. Adams, Chief of Neurology at MGH, I was approached by an enthusiastic French-Canadian neurologist André-Roche Lecours. He told me about two kuru brains that had been cut in serial section at the Warren Museum of Harvard Medical School under the watchful eye of the distinguished developmental neuroscientist Dr Paul Yakovlev. Roche had concluded that I had the expertise to study and report on the two brains in association with him.At this time I had a large number of research projects going as well as being Chief Resident in Neuropathology under Dr Pierson Richardson at MGH, so I had no time at all for such painstaking work. Nevertheless, Roche prevailed and we both gave up our Sunday mornings for

Unilateral Arhinencephaly in Goldenhar‐Gorlin Syndrome

“…Some authors have claimed that arhinencephaly is specific for 13-1 5 trisomy syndrome (Miller er al. 1963) and that it may be associated with an increased number of nuclear abnormalities-'small clubs'-of the polymorphonuclear leukocytes (Kakulas andRosman 1965, Frutiger 1968), a finding which we were not able to confirm in our case. Subsequently it was recognized that arhinencephaly was not associated with specific chromosomal aberrations and that it was more likely to be due to various etiological factors (Miller andSelden 1967, Cohen et al 1971~).…”

Introduction

“…We were fortunate to have other neuropathologists at the meeting, including Byron Kakulas and Catriona McLean. Byron's work was done during the second wave of the neuropathological study of kuru ( Kakulas 2008 ) and Catriona's work was published during the last decade, from the examination of archival material held in Melbourne, which she has re-examined for these proceedings ( McLean 2008 ). In 1967, Gabriele Zu Rhein produced the first electron micrographs of kuru.…”