The Island of the Colorblind

A vast epidemic of viral sleepy-sickness, encephalitis lethargica, starting in Europe in the winter of 1916-17, swept through the world in the following years, coming to an end in the mid-1920s. Many patients seemed to recover from the acute illness entirely, only to fall victim, years or decades later, to strange (and sometimes progressive) post-encephalitic syndromes. There were thousands of such patients before the 1940s, and every neurologist at the time had a vivid idea of these syndromes. But by the 1960s, there were only a few hundred of these patients left – most very disabled and forgotten in chronic hospitals; and neurologists training at this time were scarcely aware of them. In 1967, when L-DOPA became available for treating parkinsonism, there were only, to my knowledge, two ‘colonies’ or communities of post-encephalitic patients left in the world (at Beth Abraham Hospital in the Bronx and the Highlands Hospital in London).

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Zimmerman’s brief report, in fact, was written up for the U.S. Navy, but not available generally; its existence was virtually unknown for almost a decade. It was not until the late 1950s that his paper was recognized as the first to report on the Guam disease.

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Hirano’s visit to Guam is still vivid for him thirty-five years later – the long and complex journey there, his delight in the island, the patients he saw, the autopsies he performed, the microscopic sections he prepared. He presented his findings at the 1961 annual meeting of the American Association of Neuropathologists – the same meeting at which, three years later, Steele, Olszewski, and Richardson presented their findings on progressive supranuclear palsy, another equally strange ‘new’ disease. Hirano was struck at the time by the fact that ‘the histological and cytological features were essentially similar in the two,’ and concluded, in his remarks as a discussant of their paper, that:

The striking similarity of tissue response in these two disorders, occurring at two different geographical locations, certainly deserves attention, not only in the clinical and pathological sense, but also from the standpoint of their familial and epidemiological features.

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It was Freycinet’s impression that though the cycads had always been common on Guam, they had not been eaten ‘until the Spanish taught the natives how to separate its substance from the poisonous juice it contained.’ But this is a matter which has to be questioned, for in many other cultures the use of cycads and the knowledge of how to prepare and detoxify them go back to prehistoric times, as David Jones remarks in Cycads of the World:

Studies suggest that Australian aborigines had developed the technology for the preparation of edible foods from cycads at least 13,000 years ago…Perhaps toxic cycads were one of the first dangerous plants to be tamed by humans…Nevertheless, in view of the presence of virulent toxins, the use of cycad parts by humans as food is quite extraordinary.…Although the techniques of preparation are relatively simple…there is room for error. It is tempting to speculate on the hit or miss learning procedure which must have preceded the successful development of such a methodology.

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Cycads, properly speaking, do not have fruits, for fruits come from flowers, and cycads have no flowers. But it is natural to speak of ‘fruits,’ for the seeds are enclosed in a brightly colored, luscious outer tunic (or sarcotesta), which resembles a greengage or plum.

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Raymond Fosberg spent his entire professional life studying tropical plants and islands. ‘From a childhood fascination with islands,’ he remarked in a 1985 commencement address at the University of Guam: