Cushing and epilepsy surgery: Two successfully treated cases with long-term follow-up

Surg Neural 1989;32:241--5

Historical

Vignette

Cushing and Epilepsy Surgery: with Long-term Matthew

R. Moore,

Two Successfully

Treated

M.D., Eugene

Moore MR, Rossitch E, Shillito J. Cushing Neural

treated

Rossitch,

Jr., M.D., and John

cases with long-term

and epilepsy follow-up.

surgery: Surg

1989;32:241-5.

Two of Dr. Harvey Cushing’s patients who were successfully treated surgically for epilepsy are presented to illustrate his understanding of the problem. Both cases include his operative sketches and long-term follow-up, one includes the patient’s recent brain computed tomography scan and autopsy specimen. Cushing’s operative sketching habits and their importance are discussed. KEY WORDS: Craniotomy; Epilepsy; Harvey Cushing; Medical history; Medical illustration

Introduction Some of the earliest evidence of therapeutic craniotomies was found in the remains of trephined skulls of Neolithic and Incan man from more than 2000 years ago { 1,9]. Occasionally, these early trepanation points were located in the line of depressed skull fractures, which may have unwittingly decreased seizures and neurologic deficits by draining a subdural hematoma [4,7]. Even Hippocrates had noted and directed skull trepanation to be opposite the side of body convulsions [S]. However, the first documented brain operation where removal of an intracranial lesion successfully “cured” epileptic attacks was performed by Heyman in 183 1 181. He operated on a young girl with epilepsy and an old, healed, depressed left parietal skull fracture. After removal of a firm, yellow, gliotic abscess and capsule, the patient remained seizure-free. Later in the 19th century, Krause {5] reported on his experience with focal cortical excisions in 54 cases with Address Neurosurgery,

reprint

requem

Children’s

Cases

Follow-up Shillito, Jr., M.D.

Neurosurgery Service, Brigham & Women’s Hospital and The Children’s Hospital, and the Department Harvard Medical School, Boston, Massachusetts

two successfully

241

to: John Shillito, Jr., M.D., Department of Hospital, 300 Longwood Avenue, Boston,

Massachusetts 02 115. Received February 7, 1989; accepted April 12, 1989. 0 1989 by Elsevier Science Publishing Co., Inc.

of Surgery,

epilepsy. Of these 54 cases, 29 were attributed to traumatic or infectious etiology but with no tumor seen. In about the same period while at Johns Hopkins Hospital, Harvey Cushing developed an appreciation of cortical localization through faradic stimulation of motor and sensory regions in patients having surgery under local anesthesia. Even before Penfield’s electrocorticography, Cushing suggested in 1909, “. . . for if it will be possible in the future to pick out with an electrode areas of the brain from which the sensory aura of focal convulsion has originated, we shall have advanced a long way toward the possible operative localization of subcortical irritative lesions of the immediately postcentral field” 121. In this paper {2], Cushing reported on an exploratory craniotomy with cortical excision localized to where direct cerebral stimulation in a 15-year-old boy with epilepsy mimicked his preseizure sensory aura. The sophisticated multichannel direct electrocorticography for tailored excisions later developed by Penfield was not used in Cushing’s operative theater. Although Cushing is not primarily known for epilepsy surgery, he did occasionally perform operations to treat this disorder. We present two of Cushing’s successful operations to treat focal epilepsy. The first case includes a more than 50-year follow-up with original gross pathology, recent computed tomography (CT) scans, and, finally, the autopsy specimen. The second case includes a 23-year follow-up. Both cases have drawings of the operative exposure by Dr. Cushing.

Case Reports Case 1 CM was an otherwise healthy girl until 3 years of age, when she sustained “contusions of the head and a scalp hematoma” in the right occipital region as a passenger in a motor vehicle accident. She had no loss of consciousness, and neurological examinations at the Peter Bent 0090-3019/89/$3.50

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Figure 1. Cushingi- operative drawing (November 20, 1930) of right occipital gliosis in patient CM and approach to enbloc excision oft&~ epileptigenic form.

Brigham Hospital was normal. Skull x-rays showed a “questionable shadow in the occipital region,” thought not to be a fracture. The patient was discharged home in stable condition without change in neurological examination or seizure activity. At 10 years of age, the patient began experiencing “fainting spells,” usually preceded by vertigo, left-sided weakness, and head and eye rotation to the left, after which she would cry out and lose consciousness for several minutes. She frequently bit her tongue and passed urine during these attacks, which occurred every 3 to 5 days. The diagnosis of epilepsy was made and the patient was started on Luminal. The patient was readmitted to Dr. Cushing’s service at age 14 with a s-year history of worsening “epileptiform seizures” and frequent left frontal headaches. Physical examination was remarkable for a “small, round depression” in the right occipital region, and neurological examination showed only a left homonomous hemianopia. Repeat skull x-rays were read as “irregular thinning on the right side posteriorly along the lambdoid suture . . . no general signs of pressure . . . no other localizing signs of tumor.”

While under observation prior to ventriculogram and while off Luminal, the patient had a “series of several convulsive seizures with the intervals between of complete mental obscuration.” Dr. Cushing then wrote that, “she was entitled to an exploration with the hope of finding an occipital growth and if one was not disclosed in the hope of blocking out an area.” The patient underwent a right occipital osteoplastic exploration with block amputation of the occipital pole down to the ventricle on November 20, 1930. Cushing’s operative drawing (Figure 1) illustrates his description. On starting to reflect the dura from the mesial edge of

the incision downward I found a multitude of adhesions between the dura and brain and finally on uncovering the region I found the posterior pole to be partly cystic and otherwise tough and greyish.

The gross specimen (Figure 2) was submitted with Cushing’s mention of “possible porencephalic nature that harks back to an accident.” The pathologist reported cortical tissue with gliosis surrounding a cystic

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men (Figure 4) show the right occipital amputation 6 cm from the pole. There are metallic surgical clip artifacts at the borders of the resection in the CT scan and based along the tentorium in the skull x-ray. Cushing’s previously made surgical cavity communicated freely with the ventricular system at the atrium, and the ventricles are seen slightly dilated after removal of the septic VA shunt.

Case 2

Figure 2. Growspathological specimen of right occipital pole gliosis cystic change from operation on patient

and

CM.

structure and no tumor seen. Postoperatively, the patient had only “minor” attacks occurring only every 2 to 3 months without the preceding auras or loss of consciousness. She remained on Luminal treatment. She was living independently and doing well until suffering a fall down stairs in 1973, when she sustained cerebral contusions and a subarachnoid hemorrhage resulting in hydrocephalus. After placement of a ventriculoatrial (VA) shunt, she again did well for 15 years until developing fevers and staphylococcal sepsis. After removal of the shunt, her terminal events were multiple: septic endocarditis, superior vena cava syndrome, thrombocytopenia, and hepatic and renal failure. Computed tomography scans (Figure 3) and autopsy speci-

MR was an otherwise healthy 4%year-old white woman until 9 years prior to surgery. At that time, she had a focal seizure that began with twitching of the right arm and face. The seizure then generalized and she lost consciousness. She was left with a mild aphasia that lasted 2 months. Over the next 7 years, the patient continued to have infrequent focal seizures, but they did not interfere with her daily activities. However, by 1920, 2 years prior to surgery, she began having more frequent attacks. On one day, for example, she had eight seizures. After these episodes, she was again partially aphasic for a period of time. In November 1920, she was examined by Dr. Gilbert Horrax at the Peter Bent Brigham Hospital, who noted only slightly increased reflexes in her right arm and an otherwise nonfocal examination. His impression at that time was a “possible left paracentral endothelioma” (i.e., left parasagittal meningioma). Over the following year, despite the Luminal regimen, the attacks became more frequent, with long periods of aphasia and right facial weakness, particularly occurring during times of fatigue. The patient was then readmitted to Dr. Cushing’s service to undergo a left frontotemporal craniotomy. Skull x-rays prior to surgery showed a normal sella turcica, with no evidence of thickening of the skull or increased intracranial pressure. At surgery on this case of focal epilepsy on January 31, 1922, Dr. Cushing noted, a “curious” bony anomaly, well illustrated by his drawing of the operative field (Figure 5). He describes the operation as follows. This was a somewhat difficult osteoplastic resection owing to the adherence of the dura and the definitely thick bone. On reflecting the flap a curious nodule of bone elevated possibly 5 mm within the surface of the skull, just to the lower corner of the field, was disclosed. The dura in correspondence with this was greatly thinned and there was a very marked indentation of the brain.

scan without contrast of patient CM (September 1988) after removal of infected VA shunt. Note the large anterior extent of Gushing’s resection communicating with the right lateral ventricle.

Figure

3.

Axial

CT

The dura was then reflected disclosing a wet hemisphere, and so far as I could tell this bony projection had caused an indentation of what seemed to me to be

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Figure 4. Posterior view of4rutopsy specimr ‘n of patient CM showing extensive decomjWessionand ventricular communic,ation.

the superior temporal convolution. It was in short some distance away from Broca’s convolution and from the face area, and in this respect does not account for the definitely focal attacks unless they had occurred by some sort of unusual “spread.” The brain was quite wet as is often the case with patients with epilepsy, and on palpation it seemed to me to be unusually firm, particularly in the region of the operculum (Broca’s region) there seemed to be a very tense

area, although the surface appeared normal. Through removal of this endostosis alone, the patient had marked decrease in seizure frequency. In a followup letter 23 years later, the patient’s physician stated that she was in remarkably good health and that seizures were extremely rare.

Discussion We have presented a rare view of Cushing’s operative result using modern tools (CT scanning) and autopsy in this first case. Many of Cushing’s patients, as in contemporary neurosurgery, presented with seizures as the major symptom of an intracranial neoplasm. Despite the absence of preoperative CT scanning, Cushing’s understanding of neuropathology, clinical history, and the neurological examination rarely misdirected an operative approach. His knowledge of functional anatomy led him, in the first case, to enbloc removal of gliotic, epileptigenic brain and, in the second case, only conservative osteoplastic remodeling. Both patients had significantly improved quality of life and decreased seizure frequency postoperatively. Although he was familiar with functional localization

based on direct cortical stimulation, Cushing was hesitant to use this method to map seizure foci. He even wrote in one report that, physiologically speaking, he did not understand how direct cortical stimulation could reproduce the perception of a limb sensation. Further, he felt uncomfortable basing location of resection on stimulation with such variables as stimulus spread, disproportionate “especially wide cortical representations,” and varied “unduly excitable” susceptibility of epileptigenic versus normal brain [2]. Cushing’s drawings were an integral part of his operative report. He frequently used an “X” at particular areas or made multiple views (e.g., “I,” “II”) on his sketches, which were referred to within the text. In 1929, Miss Mildred Codding joined Cushing’s team directly from her study of medical illustration under Max BrBdel at the Johns Hopkins Medical School. She was largely responsible for elaborating and clarifying surgical illustrations for publication. Cushing preferred this method rather than relying solely on the artist’s [3,6]. conception At the close of surgery, Cushing remained in the operating room, quickly sketching in pencil with the thick black rubber gloves still on his hands. Using a fresh legal-sized blue operative report sheet for each drawing, his sketches were drawn from the operator’s viewpoint; therefore, the lowest portion of a sketch was that closest to the surgeon, and the page’s upper margin was the far operative field. As seen in the sketches of Figures 1 and 5, he initially penciled the field starting above the hospital letterhead while still in the operating room. Later, India ink was added over the penciled lines, and the sheet was submitted for typing of the

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Figure 5. Gushing’s

operative drawing (January rraniotomy in patient MR. Note the bony exo.uosis on the flap and impression on superjcial temporal gyros.

31, 1922) of left frontotempora(

operative report directly below the sketch. Cushing infrequently signed these many drawings, yet his style was distinctive. According to one Cushing biographer [lo], his early medical sketches of patients in clinic were “small and hastily drawn.” They did, however, remarkably capture a clinical condition that thus helped him remember the patient later. While a resident at Johns Hopkins, Cushing was a good friend of Max Br6del. In fact, Cushing informally studied drawing with him, leading ultimately to the technique demonstrated in his later operative sketches.

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