The Island of the Colorblind

With Estella, specifically, there was the sense of calm, of her being in her own world, the sense of an achieved equilibrium both within her, and in relation to her family and community – and the fear that medication might ‘stir her up’ and imperil this.

But it was very different for Jose, her husband: physiologically different, as a start – for he had the most intense jamming, clenching, locking parkinsonism, where muscle groups, rigid, fought against each other and jammed each movement at its inception. If he wished to straighten his arm, activation of the triceps was at once opposed by an activation of its antagonist, the biceps (which normally relaxes to allow the arm’s extension), and vice versa – so that the arm got locked, perpetually, in strange positions, and he could neither bend nor straighten it. Similar jams, similar binds, affected all his muscle groups – the whole innervation of the body was perverse. He would go red in the face with the intensity of his effort to get through the block, and sometimes it would give way suddenly, and then the force of his effort would make him jerk violently or fall.

In this sort of parkinsonism, the ‘explosive-obstructive’ sort, the whole body, so to speak, is set against itself, locked in irre-soluble inner conflict. It is a state full of tension, effort, and frustration, a tormenting condition which one of my patients once called ‘the goad and halter.’ Jose’s state was wholly different from the strange muscular compliance, the waxy flexibility, which went with Estella’s catatonia. One could see, in this one couple, the extremes of furious resistance and total surrender – the antipodes of the subcortical will. After Jose and Estella, I examined Claudia and her two brothers briefly, but none of them, it was clear, had any sign of the disease. Nor did they seem to have any fear of getting it, despite the fact that both their parents, and many older relatives, were affected. John contrasted their confidence with the great anxiety felt by members of the older generation, who often feared – especially if they had relatives with the disease – that they might have it already latent in their bodies. These folk attitudes, John pointed out, were entirely appropriate, given the fact that no one born after 1952 had been known to contract the disease.

Jose’s sister, who lives with them, showed yet another form of the disease, one marked by a severe and progressive dementia. She was at first frightened by our presence – she had lunged at me, and tried to scratch me, when we first entered the house. She became angry, and perhaps jealous, as we talked to the others, and now she came across the room, pointing to herself, saying ‘Me, me, me – ME.’ She was also quite aphasic, and very restless, given to bursts of screaming and giggling – but music calmed and cohered her to an amazing extent. This too had been discovered by the family; the traditional knowledge of these disorders, and ways of dealing with them, is very considerable. To calm her, the family started to sing an old folk song, and the old lady, so demented, so fragmented, most of the time, joined in, singing fluently along with the others. She seemed to get all the words, all the feeling, of the song, and to be composed, restored to herself, as long as she sang. John and I slipped out quietly while they were singing, suddenly feeling, at this point, that neurology was irrelevant.

‘You can’t see a family like this,’ said John as we set out the next morning, ‘without wondering what causes so many of them to be affected. You see Jose and his sister, and you think, this must be hereditary. You see Estella and her husband, who are not blood relatives, though their lives are intertwined: Is the lytico-bodig due to something in the environment they share, or has one passed the disease to the other? You look at their children, born in the 1960s, free of the disease, like all their contemporaries, and infer that the cause of the disease, whatever it was, vanished or became inoperative in the late forties or fifties.’