Faculty Bibliography

Norman Geschwind (1926-1984) was a critical force in the emergence of behavioral neurology and neuropsychology as important disciplines in the 1960s and 1970s. Factors that influenced his early career are described, with extensive quotes from a document that Geschwind wrote in 1982, supplemented by interviews with his childhood friends, colleagues from his early career, and his daughter. He entered Harvard College as a math major, but became interested in psychology when he served in the Army infantry in World War II and observed soldiers who acted irrationally in combat. Returning to undergraduate studies, he was taught that knowledge of the brain would shed little light on behavior. His interest in behavioral neurology began with his medical student courses in physiology and neuroanatomy, where he first learned about epilepsy and aphasia. He then trained for 2 years at Queen Square, where his research focused on periodic paralysis, but was greatly influenced by the writings of Hughlings Jackson and Charles Bastian and the teachings of Sir Charles Symonds. Returning to Boston, Geschwind became Dr. Denny-Brown's Chief Resident at the Neurologic Unit of Boston City Hospital. Another unrecognized source of Norman Geschwind's intellectual legacy is the unpublished course he taught on The Neurology of Behavior at Harvard Medical School in the 1970s and 1980s. These lectures were an opportunity for Geschwind to formulate, share, and refine his ideas on behavioral neurology to an eclectic mixture of Boston academics, from linguists, philosophers, and anthropologists to physiologists and psychiatrists. From tape recordings of the Spring 1974 course, 10 lectures were transcribed. One of these slightly edited lectures, 'Personality Changes in Temporal Lobe Epilepsy,' is reproduced in this issue of Epilepsy & Behavior

We present our technique for extraoperative functional mapping in awake children with supratentorial tumors near eloquent cortex. This technique may help optimize tumor resection and/or the functional outcome, especially when an awake craniotomy is not possible or in the setting of coincident seizures. After subdural electrode implantation, extraoperative functional mapping and seizure focus mapping were performed, followed by staged tumor resection. Gross total resection was achieved in 6 patients and subtotal resection in 2. No complications due to electrode placement or invasive monitoring were observed. Staged resection of pediatric brain tumors near the eloquent cortex appears safe, allows functional mapping in awake patients, and may assist in decisions between maximal tumor resection and optimal functional outcome

OBJECTIVE: The goal of the work described here was to assess the efficacy and safety of vagus nerve stimulation in a cohort of patients with tuberous sclerosis complex with refractory epilepsy. Furthermore, we examined the impact of vagus nerve stimulation failure on the ultimate outcome following subsequent intracranial epilepsy surgery. METHODS: A retrospective review was performed on 19 patients with refractory epilepsy and TSC who underwent vagus nerve stimulator (VNS) implantation. There were 11 (58%) females and 8 (42%) males aged 2 to 44 years when the VNS was implanted (mean: 14.7+/-12 years). Twelve patients underwent primary VNS implantation after having failed a mean of 7.1 antiepileptic drugs. Two patients (17%) had generalized epilepsy, one had a single seizure focus, three (25%) had multifocal epilepsy, and six (50%) had multifocal and generalized epilepsy. Seven patients were referred for device removal and evaluation for intracranial procedures. One patient in the primary implantation group was lost to follow-up and excluded from outcome analysis. RESULTS: All implantations and removals were performed without permanent complications. The duration of treatment for primary VNS implants varied from 8.5 months to 9.6 years (mean: 4.9 years). Mean seizure frequency significantly improved following VNS implantation (mean reduction: 72%, P<0.002). Two patients had Engel Class I (18%), one had Class II (9%), seven had Class III (64%), and one had Class IV (9%) outcome. Three patients with poor response to vagus nerve stimulation therapy at our center underwent resection of one or more seizure foci (Engel Class I, two patients; Engel Class III, one patient). Seven patients referred to our center for VNS removal and craniotomy underwent seizure focus resection (6) or corpus callosotomy (1) (Engel Class II: 2, Engel III: 2; Engel IV: 3). In total, 8 of 10 (80%) patients experienced improved seizure control following intracranial surgery (mean reduction: 65%, range: 0-100%, P<0.05). CONCLUSIONS: VNS is a safe and effective treatment option for medically refractory epilepsy in patients with tuberous sclerosis complex. Nine of 11 patients (82%) experienced at least a 67% reduction in seizure burden. Lack of response to vagus nerve stimulation does not preclude subsequent improvement in seizure burden with intracranial epilepsy surgery

Hypersexuality, a feature of Kluver-Bucy syndrome, is a rare but dramatic postictal symptom of temporal lobe epilepsy. We describe a 39-year-old patient with uncontrolled partial epilepsy who developed postictal periods of hypersexual aggression toward his wife after nocturnal convulsions. Following these seizures, he would have forced intercourse with his wife, followed by tremendous guilt and remorse when informed of his actions in the morning. Nocturnal convulsions and postictal hypersexuality resolved with a combination of lamotrigine and levetiracetam, more sleep, and stress management. The temporal features of the hypersexual behavior with respect to convulsions and the resolution of the behavior with seizure control support that aggressive hypersexuality can occur as a transient postictal behavioral change. The more intense nature of his symptoms compared with previously reported cases may reflect the occurrence of symptoms after tonic-clonic rather than partial seizures