The Victim Is Always the Same
by I. S. Cooper MD.
Harper & Row, 160 pp., $6.95
In a passage in “Bread and Freedom” Camus expresses his revulsion at the way in which, in political arguments, one atrocity may be bartered for another: if one protests at some enormity of the communists, three American negroes are “thrown in one’s face.” In any such disgusting attempt at outbidding, Camus says, one thing does not change—the victim, freedom. It is this reflection that gives Irving Cooper’s book its title: the victim is the patient, and the aptness of the Camus quotation becomes very clear as the book goes on.
The subject of Dr. Cooper’s book is the treatment of a rare and unqualifiedly dreadful neuromuscular disease of stealthy onset known as dystonia musculorum deformans (DMD), in which the limbs and extremities of the victims are locked firmly into grotesquely functionless attitudes by the simultaneous contraction of antagonistic muscles, and in addition victims may suffer mad-looking tremors. It is a disease of the kind that makes even the most devout question the existence of a benevolent deity. Although Lewis Carroll himself thought “Anglo-Saxon attitudes” a sufficient diagnosis, I put it to Mr. Martin Gardner that the White King’s looking-glass messenger Haigha, whose extraordinary movements and postures caused Alice so much surprise, was in reality the victim of DMD.
Janet, the patient whose treatment Dr. Cooper describes in the greatest detail, contracted the disease at the age of six when she was a reasonably bright girl who weighed about fifty pounds. Five years later on admission to Dr. Cooper’s clinic, she weighed only thirty-seven pounds. Her back was arched in such a way as to force her rib cage and stomach forward; the right leg stuck out like a ramrod, with the toes coiled under the sole of the foot, and her left leg was doubled back so that the foot pressed against the buttock. Attempts to move her arms produced clonic contractions, i.e., contractions not sustained but compulsively repeated. Clinical examination was made almost impossible by the fact that Janet screamed with pain at any attempt to move her limbs and her back arched so much that her head almost touched her buttocks. Her undernourishment was fairly typical and when, as with Janet, the disease follows a chronic and progressive course patients become bedfast and die eventually from inanition or from bedsores.
Although conventional wisdom had long had it that neurosurgery could only substitute paralysis for involuntary muscular contraction Cooper had assembled enough circumstantial evidence to justify the audacious hypothesis that DMD could be meliorated without paralysis by the inactivation through freezing of a group of cells deep in the thalamus, the part of the brain through which sensory impulses pass on their way to the cerebral cortex and which has important motor connections with the cortex.
The operation requires that the head should be kept absolutely still so that a freezing probe, mounted on a stereotactic apparatus, should be guided into the appropriate region of the thalamus. The operation has to …
Outbidding April 17, 1975