The Eyes Give Warning

The patient's perspective can teach us so much. When the patient is a gifted artist, the lessons, even from the past, can be especially compelling.

Historical Note The Eyes Give Warning Amadeo Raul Rodriguez, MD, Felix J. Tyndel, MD, MA, FRCPC T he patient's perspective can teach us so much. When the patient is a gifted artist, the lessons, even from the past, can be especially compelling. Frigyes Karinthy (1887-1938) was an already wellknown Hungarian writer when, while sitting at a café in Budapest, he heard the "distinct rumbling noise" of a passing train. When it recurred several times and nobody else seemed to notice it, he realized he was having a hallucination. In retrospect, it was the first symptom of a brain tumor, and the profound human emotions of facing such a diagnosis are intimately described in his autobiographical book "A Journey Round My Skull" (1), in which he shares his uncertainty, fear, denial, and hope, which are not different from what patients still experience. Karinthy uses his characteristically clever and sardonic prose to describe his symptoms and interactions with the medical professionals of his time. As one would expect, the diagnosis and treatment of brain tumors nowadays differ significantly because of the tremendous advances in medicine, yet, as he takes us along on his journey, the diagnostic difficulties he faced were strikingly similar to what we continue to encounter. As his initial symptom was the sound of "the trains," the first physician he consulted was "a well-known ear specialist" who thought his "duct was inflamed." Shortly thereafter, a psychoanalyst explained his symptoms by "bringing the noises and headaches into organic associations with my character, desires, and disappointments." Often regarded as a consequence of the hyperspecialization of modern medicine, fragmentation of care was already present at that time. Karinthy would later experience a disturbing episode: "[T]he mirror opposite me seemed to move. Not more than an inch or two, then it hung still." He felt that "everything, myself included, seemed to have lost its grip on reality. I did not feel certain I was there myself, or the man sitting there was I." He was extremely frightened by this experience. We can only imagine his disappointment when he Department of Surgery (Ophthalmology), and Medicine (Neurology) (ARR), McMaster University, Hamilton, Canada; and Departments of Medicine (Neurology), and Ophthalmology (FJT), University of Toronto, Toronto, Canada. The authors report no conflicts of interest. Address correspondence to Amadeo Raul Rodriguez, MD, Department of Surgery (Ophthalmology), and Medicine (Neurology), McMaster University, 2757 King St East, Hamilton, ON L8G 5E4, Canada; E-mail: [e-mail] Rodriguez and Tyndel: J Neuro-Ophthalmol 2020; 40: 281-282 then saw a new doctor who diagnosed nicotine poisoning "before I could describe half my symptoms." "He did not even examine me," he said, unfortunately a sentence we still hear too often. As we would expect, his symptoms continued despite giving up smoking. To "the trains" and the headaches, "fainting fits, retching, and attacks of giddiness" were soon added. Other people noticed changes as well; his son commented that he was veering to the left, and his wife complained of his distorted handwriting. Karinthy, who had briefly studied medicine and was married to a physician, suspected there was something really wrong with him and became fearful of "a brain tumor." This idea was met with derision by his friends. Like in a story in which the detectives seem to dismiss any clue that points at the murderer right in front of them, doctors were more often interested in him as the famous writer he already was than as a patient and his complaints. When he confessed his fears to his wife, she thought he was acting "like a first-year medical student with professional hypochondria." But things were about to change. When his vision started deteriorating, he consulted an ophthalmologist who provided the missing piece of the puzzle. "Choked disks, in the left eye one and a half, in the right two and a half diopters" read the report he was given to take to a hospital as soon as possible. We can readily relate to those patients we still encounter with headaches, among other symptoms, who consult several doctors until someone finally performs ophthalmoscopy and sends them to the emergency department with a note reading "papilledema." It is as if patients must prove their complaints are genuine. The presence of this clinical sign was as powerfully confirming then as it remains today. Indeed, Karinthy dedicated an entire chapter of his book to his experience in the eye clinic, which he titled "The Eyes Give Warning." Today, a scan would have shown the presence of a mass lesion, but at that time, the diagnosis was not as simple. He underwent numerous examinations in Budapest and subsequently went to the prestigious Wagner-Jauregg Clinic in Vienna, where his wife, a psychiatrist, worked at that time. There, he was extensively investigated under the supervision of Dr. Otto Pötzl. In the meantime, he tried to remain hopeful, although it was not always possible. He could not avoid thinking of one of his friends who had a terrible death due to a brain tumor. That was the first time he had heard of "the fundus of the eye being tested," and he now underwent the same. Just as our patients consult the 281 Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited. Historical Note Internet, he went to the library in search of information. His symptoms continued and eventually his headaches became so intense that he even considered suicide. His vision got to a point where he "could not recognize faces unless they came close to mine." He even had difficulty reading his own writing. His fundi worsened; "the papilla has again deteriorated by two and a half diopters, and there are signs of atrophy now," said the ophthalmologist, who also informed him that if the tumor was not removed, he would go completely blind. Finally, Professor Pötzl rendered the verdict: "[A] cyst, now approaching a hen's egg size, is growing within a strictly circumscribed area on the right side of the brain and at the back of the cerebellum." It was decided that he would go to Sweden to see the neurosurgeon Dr. Herbert Olivecrona, who had studied in the United States with Dr. Harvey Cushing. Karinthy and his wife embarked on the journey. He was admitted to Stockholm's Serafimer Hospital where investigations continued until in May 1936, he was finally taken to the operating room. Karinthy's description of his experience is richly detailed. He could feel how his head was shaved, and he subsequently heard "an infernal scream" as the electric trephine penetrated his skull. He was then transported to the X-ray room in Dr. Lysholm's department where he underwent pneumoencephalography: "[T]hey had drained the fluid from my brain cavities and had filled them with air." From there, he was taken back to the operating room. Karinthy had watched an amateur film of one of Cushing's operations, and as he lay prone, strapped to the table, he recalled it to understand his own experience: the incision, trephination, removal of a part of his skull, and draining of the fluid. Although he did not experience pain, he found terrifying the apparently impossible idea of "lying with his head open and his brain exposed to the outer world, not merely alive but conscious and in his right mind." 282 The operation lasted several hours, and it took several days before he regained full consciousness. It was a success, and Karinthy was undoubtedly grateful to Olivecrona who, however, was afraid that he might not have been able to save his patient's sight. But not without pleasure, Karinthy realized that "the Professor" was wrong. To prove it, he picked Thomas Mann's story of Joseph, which he had been reading until his vision deteriorated and he could no longer continue, and read it out loud to his wife. Bishop (2) has pointed out the extraordinary parallel between the paragraph he read about the rescue of Joseph by wandering merchants from the well into which he was thrown by his brothers and the restoration of Karinthy's health by Olivecrona. The raising of Joseph and the raising of Karinthy! Yes, he was still alive and was also able to see. "It's a miracle!," commented the ophthalmologist who examined him. From this remark, we can infer that his optic nerves must have been markedly pale. Three weeks after his surgery, he was able to stand up and walk, so he was allowed to leave the hospital to return to his beloved Hungary. Karinthy's book is remarkable in giving us a look back at the diagnostic and therapeutic aspects of medicine and the doctor-patient relationship of the time. It is also a poignant example of the fundamental role of the fundus examination (3). It was this finding that led to the suspicion of and ultimate diagnosis of Karinthy's tumor. Then, just as now, "the eyes give warning." REFERENCES 1. Karinthy F. A Journey Round My Skull. New York, NY: New York Review of Books, 2006. 2. Bishop MG. The genius of disease. 3. A journey round my skullFrigyes Karinthy. J R Soc Med. 1994;87:2-4. 3. Kattah JC, Halmagyi GM. A cautionary tale about a global problem. Neurol Clin Pract. 2015;5:97-98. Rodriguez and Tyndel: J Neuro-Ophthalmol 2020; 40: 281-282 Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited.