Neuroscience For Kids

Eugene O'Neill - What Went Wrong?
April 22, 2000

The Man

Eugene O'Neill is perhaps the most well-known American playwright. He was awarded Pulitzer Prizes in 1920, 1922, 1928 and 1957 and a Nobel Prize for literature in 1936. Yet for all of his writing success, O'Neill led a life filled with tragedy and he was afflicted with a misdiagnosed neurological disorder that contributed to his death. Now, almost 47 years after O'Neill's death and with the permission of O'Neill's surviving grandchildren, the autopsy results of this amazing writer have been released. These results, published in The New England Journal of Medicine (NEJM, April 13, 2000), shed new light on O'Neill's health.

pen Eugene Gladstone O'Neill was born in New York City on October 16, 1888. He had a reputation for heavy alcohol drinking, and he attempted suicide in 1912. In addition to surviving battles with malaria and tuberculosis, O'Neill struggled with bouts of depression, some of which required a stay in the hospital. Many biographers believe that depression also affected O'Neill's mother, father, brother and two sons. Two of O'Neill's sons, Eugene Jr. and Shane, committed suicide.

Neurological Symptoms

pen According to the new NEJM report, O'Neill noticed slight shaking (tremor) of his hands when he was a freshman at Princeton University in 1906. These tremors became worse over the years and in 1941 he was diagnosed with Parkinson's disease.

Other symptoms displayed by O'Neill:

Difficulty writing: trembling hands made his writing difficult to read.
Unsteady gait: he had difficulty walking.
Problems with motor coordination: he had trouble using his arms to feed himself.
Speech problems: it became difficult to understand what he was saying.

O'Neill tried various drugs to help with these problems, but none of them had a beneficial effect.

The Brain

O'Neill's brain weighed 1.33 kg, well within the normal range. However, a significant abnormality was found in his cerebellum. The cerebellum is the part of the brain important for controlling posture, balance and motor coordination. In O'Neill's brain, certain areas of the cerebellar cortex had shrunk. In fact, over 90% of the "Purkinje cells" in the part of the cerebellum called the "vermis" was lost. Purkinje cells (see photo to the right) are neurons that send projections out of the cerebellar cortex to deep cerebellar nuclei; from these areas, messages go to other areas of the brain that influence movement. The number of another type of neuron called the granule cell was also reduced in the vermis. No abnormalities were found in the cerebral cortex or the substantia nigra. In cases of Parkinson's disease, neurons in the substantia nigra degenerate.

The Diagnosis

cerebellum The examination of O'Neill's brain clearly shows that he did not have Parkinson's disease. Parkinson's disease is characterized by severe degeneration of neurons in the substantia nigra. This abnormality was not seen in O'Neill's brain. Most likely, O'Neill suffered from a disorder called "late-onset cerebellar cortical atrophy." The authors of the NEJM paper argue that it was unlikely that this disorder was caused by alcohol consumption because O'Neill had a decent diet and did not abuse alcohol in the later years of his life. Rather, this disorder may have been inherited.

References

Price, B.H. and Richardson, E.P., The neurological illness of Eugene O'Neill - A clinicopathological report, The New England Journal of Medicine, April 13, 2000.
Black, S.A., Eugene O'Neill. Beyond Mourning and Tragedy. New Haven: Yale University Press, 1999.
Purkinje cell image courtesy of "The Slice of Life"

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