DR DELBANCO: Mr C is a 48-year-old man with a long history of cognitive and psychological problems, marked by mood swings, memory loss, and seizures involving the frontal and temporal lobes of the brain. A primary physician, a neurologist with special interest in seizures (Dr J), and a psychiatrist (Dr N) manage Mr C’s care. He has private insurance, works part-time, and lives near Boston with his wife. Mr C’s problems likely date to birth. He was the product of an uncomplicated pregnancy but during labor there may have been some anoxia. He recalls a “foggy feeling” in his head from the first time he can remember. As a child, he fell out of a tree and hit his head without notable sequelae. In elementary school, he did well but had difficulty with his memory. He continued a successful academic experience in high school, during which time he had intermittent abdominal pains, and then was graduated from a top university with extraordinarily high academic achievement. In college, he describes having had difficulty understanding lectures and remembering details. He read avidly and worked endless hours but felt his ability to learn was hampered by a poor memory. He had difficulty handling several different tasks at once. In graduate school, depression because of his struggles with his cognitive limitations led to a suicide attempt, which caused a contusion to the right side of the head. Thereafter, Mr C was hospitalized; lithium followed by carbamazepine and phenytoin gave him more, but not complete, control of his symptoms. Neurologic evaluation at that time showed no persistent physical findings or lateralization. He was ambidextrous, and his right hand was noted to be a bit smaller than his left. Reflexes were diminished but bilaterally symmetrical. There were no motor or sensory findings. Multiple electroencephalogram (EEG), magnetic resonance imaging (MRI), computed tomography (CT), and positron emission tomography (PET) evaluations showed inconsistent findings. There were no clear ictal patterns on EEG. Over the next years, he was treated with multiple medications. Multiple MRI, CT, and PET scans, along with serial EEGs, continued to show inconsistent findings, with frequent abnormalities identified in the left frontal and temporal lobes. Mr C fared increasingly better, working at various jobs and getting married, until serious cognitive and affective disruption again supervened. Multiple high-dose antidepressants, augmentation strategies, neuroleptics, and atypical neuroleptics were tried without effect. However, over a period of several years, his mood and functionality slowly improved. In recent years, Mr C has worked part-time in jobs far less demanding than his superior intellectual attainment might otherwise anticipate. In 2000, Mr C contemplated surgery for possible focal epileptiform lesions in the brain, but depth electrode investigation revealed abnormalities that were multifocal and involved the anterior cingulate and supplementary motor areas. The abnormalities were more active on the left than on the right. He also had a left temporal lobe slow wave focus. Electrical stimulation through the left supplementary motor area electrode at subseizure threshold levels elicited his characteristic paroxysmal, seizurelike cognitive and affective symptoms. In addition to seizures, Mr C notes very poor memory, which has led to great difficulty in recognizing people and has put him in awkward social circumstances on many occasions. A talented musician, Mr C plays an instrument with considerable competence but has great difficulty remembering pieces even after extensive practice. He feels worse during the winter months and notes that lack of sleep exacerbates the symptoms that are likely partial seizures. Sexual activity at times exacerbates his symptoms. He notes also long-standing tinnitus in the right ear, with a sense of white noise coming from his left ear. During seizure activity, he feels he is spinning backward in space and that his limbs become very long. For decades, Mr C developed an intermittent rash on his right arm, and recent evaluation demonstrated herpes simplex virus 2 infection. Appearance of the eruption appears to coincide with exacerbations of his seizures, and, in the
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Orrin Devinsky (2003) studied this question.
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