The Island of the Colorblind

But it was not a wholly simple ‘awakening’ for Euphrasia, any more than it had been for hyperbolic Hester. For along with the motor animation, the liveliness, the playfulness, which suddenly came on her, there came a tendency to wisecrack, to tic, to sudden lookings and touchings, to tossing and darting, to jabbing and lunging – a dozen strange impulsions, a drivenness of body and mind. There was this tremendous rush of life, of extravagant activation, both healthy and pathological, and then, twenty minutes later, a re-descent into her original state, coupled with repeated yawning, a sudden complete lethargy.

‘What do you think of that, eh?’ asked John, at my side, eagerly. ‘Remind you of anything?’

When he is not seeing patients, John teaches at the Guam Memorial Hospital in Tamuning and does research in his laboratory there. He has lobbied hard for more research funding to be put into local facilities, and would like to establish a complete center on the island for investigating the lytico-bodig, with sophisticated neuropathology equipment and facilities for MRI scans and other brain imaging. At present, many of these studies have to be done on the mainland, while much of the epidemiological work – interviewing patients and piecing together extensive family trees – as well as basic clinical and lab work of various kinds, is done here on the island.

He showed me into his lab; he had something special he wanted me to see. ‘Let me show you these slides, Oliver,’ said John, waving me over to a microscope. I looked through the eyepiece, under low power first, and saw pigmented cells, symmetrically arranged in a V.

‘Substantia nigra,’ I said. ‘Many of the cells are pale and depigmented. There’s a lot of glial reaction, and bits of loose pigment.’ I shifted to a higher power, and saw a huge number of neurofibrillary tangles, densely staining, convoluted masses, harshly evident within the destroyed nerve cells. ‘Do you have samples of cortex, hypothalamus, spinal cord?’ John handed these to me, I looked at them one after another – all were full of neurofibrillary tangles.

‘So this is what lytico-bodig looks like,’ I said, ‘neurofibrillary degeneration everywhere!’

‘Yes,’ said John, ‘that’s very typical. Here’s another case – have a look at this.’ I went over it as before; the findings were very similar, and there was much the same distribution of tangles.

‘All the lytico-bodig cases look like this?’ I asked.

‘Actually, Oliver’ – John smiled broadly – ’what you’re looking at now isn’t lytico-bodig at all. It’s your disease, it’s postencephalitic parkinsonism – these slides were sent to me by Sue Daniel in London.’

‘I haven’t done much pathology since I was a resident,’ I said, ‘and I’m no expert – but I can’t tell them apart.’

John grinned, pleased. ‘Here, I have some more slides for you.’ I looked at this new series, starting with the substantia nigra, the midbrain, moving up and down from there.

‘I give up,’ I said, ‘I can’t tell whether it’s lytico-bodig or post-encephalitic parkinsonism.’

‘Neither,’ said John. ‘This is my disease, progressive supranuclear palsy. In fact, it’s from one of the original cases we described in 1963 – even then we wondered about its similarity to post-encephalitic parkinsonism. And now we look at the Guam disease…and all three look virtually the same.

‘Sue Daniel and Andrew Lees and their colleagues at the Parkinson’s Brain Bank have wondered whether these diseases are, in fact, related – perhaps even the same disease, a viral one, which could take three different forms.

‘These are very similar to the neurofibrillary tangles to be found in Alzheimer’s disease,’ John went on, ‘though in Alzheimer’s there are not as many, and they occur in a different distribution. So we have tangles – like little tombstones in the nervous system – in four major neurodegenerative diseases. Perhaps the tangles contain vital clues to the process of neu-rodegeneration, or perhaps they are relatively nonspecific neural reactions to disease – we don’t know.’

As we got back in his car to return to Umatac, John continued to sketch the history of the lytico-bodig. Another dimension was added to the problem as the 1960s advanced, and a curious change was observed in the natural history of the disease: cases of bodig, which had been much rarer than cases of lytico in the 1940s and early ‘50s, now came more and more to outnumber them. And the age of onset was also increasing – there were no more teenage cases (like the nineteen-year-old youth with lytico whom Kurland had seen), and almost no cases in their twenties.