Joumal of Intellectual Disability Research, 1993,37, 111-112

Letters to the Editor

Gaze avoiding and torso turning in the fragile-X syndrome

Sir,On first greeting and shaking the hand of a person with the fragile-X syndrome, it hasbeen noted that the head may be turned to one side with gaze avoidance, with thetorso turning in the same direction as the head as well. So far, this has felt to be arobust clinical finding in as much as it was thought to be consistently repeatable overtime (Wolff et al. 1989). However, my own clinical observation suggests that this maybe otherwise.

On a recent home visit, when accompanied for the first time by two medicalstudents, I proceeded to demonstrate the 'fragile-X handshake'. I had paid severalprevious solo visits to the house in preceding years, always being greeted by the 'fragile-X handshake'. Much to my surprise, the man in question faced me fairly and squarelywith good eye contact and shook my hand warmly. He was then introduced for thefirst time to the medical students to whom he presented the 'classical' gaze avoiding,torso turning handshake.

Thus, it would seem not unreasonable to suggest that the clinical handshake is seenin social situations which are new, presumably with associated anxiety, but with thepassage of time and familiarity, this clinical sign is replaced by an apparently ordinaryfiiendly handshake. This is a single case report (in a non-autistic, 60-year-old man withproven fragile-X) and should be judged as such. Perhaps a larger study is warrantedif colleagues are aware of similar experiences.

MIKE COOPER

REFERENCE

Wolff P.H., Gardner J., Paccia J. & Lappen J. (1989) The greeting behavior of fragile-X males.American Joumal of Mental Retardation 93, 406-11.

'Violence, death and associated factors on a mental handicapped ward' byA. K. Shah

Sir,It was with a great deal of interest that I read the excellent review by A. K. Shah in theJoumal of Intellectual Disability Research (Volume 36, Part 3, June 1992). I wasparticularly interested that two out of the four most violent patients had sudden,unexpected deaths. Although the causes of death that were given were physical innature, one would wonder if there may not have been yet other associated factors.

I recall at an informal pathological conference attempting to assist in determiningthe cause of death in another violent patient in a distant institution; the following

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factors were also involved. This was a mildly intellectually impaired individual witha history of violence. He was on high doses of major tranquillizers. During an episodeof strenuous physical restraint, he had a sudden cardiac arrest. Although the opinionof one pathologist was that the physical restraint, which was inept, had produced thecardiac arrest, a neuropathologist and psychopharmacologist both came to the firmconclusion that the individual had had a major adrenergic crisis which, in associationwith the presence of high levels of the major tranquillizer, had produced a cardio-toxicefifect. Thus, the combination of major or excessive physical excitement with high dosesof tranquillizers might also be considered as a contributing factor in such patients.

It would be of interest to compare the dosage equivalents of tranquillizers beingreceived by the violent versus the non-violent patients in Shah's groups.

I would strongly concur with Shah's suggestion that non-tranquillizing medicationshould be used if it is required to be given over an extended period of time.

BENJAMIN GOLDBERG