The Island of the Colorblind

When Yoshiro Yase, an ardent sport-fisherman as well as a neurologist, went to study the newly identified disease focus on the Kii Peninsula, he was told there were scarcely any fish in the local rivers, and this prompted him – memories of the Minamata tragedy still being vivid – to analyze their waters. Though these were free of infectious agents or toxins, they were oddly low in calcium and magnesium. Could this, he wondered, be the cause of the disease?

Gajdusek was fascinated by Yase’s findings, the more so as he had been struck by the red soil, rich in iron and bauxite, in the swamp lands around the Auyu and Jakai villages. When he was able to return in 1974 – Western New Guinea having become Irian Jaya in the intervening upheavals – he now tested the water from the shallow wells which the villagers dug in the red soil, and found unusually low levels of calcium and magnesium, as well as elevated levels of iron, aluminum, and other metals.

At this point, Kurland moved to the Mayo Clinic to pursue other research, feeling that the cycad hypothesis, though valid, could not be proven. His place at the NIH was taken by Gajdusek, who was now intrigued and excited at the notion of a mineral etiology of the Western Pacific disease. Gajdusek enlisted Yase, and together they examined well water from Guam and found that this too was low in calcium and magnesium. This triple coincidence seemed definitive:

Comparison of the Western New Guinea focus with the foci of ALS and Parkinson’s disease on Guam and the Kii Peninsula of Japan is inescapable [Gajdusek wrote], and the close association of parkinsonism and motor neuron symptoms in yet another non-Chamorro population group should not only dispel most doubts about the probably close relationship between the two syndromes, but also point to an aetiologic role of some unknown environmental factor.

The unknown environmental factor, it seemed likely, had to do with low calcium and magnesium levels in the drinking water, and the consequences of these on the nervous system. Such low levels, he speculated, might trigger a compensatory reaction in the parathyroid glands, leading in turn to excessive absorption of calcium, aluminum, and manganese ions. The deposition of these in the nervous system, he felt, might result in the premature neuronal aging and death seen with the lytico-bodig.

It was John’s hope, in 1983, that he might join Gajdusek’s team and help crack the disease at last. But Gajdusek told him he was too late – the cause of the lytico-bodig had now been established, and in any case the disease had almost vanished, because of the shift to a Western diet, which was high in calcium – there was not much left to do, and his team would be pulling out of there soon. John was surprised to hear Gajdusek express himself so forcefully, he told me, and disappointed, because he had hoped to work with him. But he decided to come to Guam nonetheless, if only to take care of patients as a physician, and not as an investigator.

But the very day after John arrived on Guam, he had an experience comparable to Zimmerman’s nearly forty years before: working in the naval hospital in Agana, he saw a dozen patients with the lytico-bodig in his first clinic. And one of them also had a supranuclear palsy – a complex disturbance of gaze, in which the patient can look sideways, but not up or down. This had never previously been reported in lytico-bodig, but it was the hallmark of the syndrome John and his Toronto colleagues had delineated nearly twenty years before. This convinced him that lytico-bodig was neither extinct nor comprehensively described, and that there was still time and opportunity for its further investigation.

Guam had superb medical facilities on the naval base, but in the outlying villages, basic medical care was very inadequate, and neurological care scarce – there was only one overworked neurologist, Dr. Kwang-Ming Chen, to care for 50,000 Chamor-ros, and 100,000 other residents of the island as well. Not only were there still many hundreds of Chamorro people with lytico-bodig, Chen told John, but new cases kept appearing – several dozen a year, he thought, and these new cases sometimes took forms different from either the classic lytico or bodig; the man with supranuclear palsy was a case in point.