Faculty Bibliography

The prolonged QT syndrome is associated with ventricular tachyarrhythmias and sudden death. We report two patients and review eight previously reported cases of this syndrome, presenting as epilepsy. The average age at the time of the first convulsion was 4.7 years. Episodes were often infrequent, and the time to correct diagnosis ranged from 1 to 28 years. Only one-half the patients had histories suggestive of a familial syndrome. Presyncopal complaints and 'lifelessness' prior to seizure activity were common findings in retrospect. Beta-blockade was effective in preventing recurrences in all patients who received treatment

We reviewed the seizure frequencies of 38 patients with medically refractory epilepsy 6 weeks before (baseline) and 3 weeks after withdrawing their antiepileptic drugs (AEDs) for video-EEG monitoring. Seizure frequency during the first 3 weeks after restarting AEDs was diminished compared with baseline (P < 0.05). We found no correlation between seizure frequency and patient age, specific antiepileptic drugs, number of seizures during the video-EEG monitoring, number of days without AEDs, or partial vs total withdrawal of AEDs. Medication tachyphylaxis, functional tolerance, or long term post-ictal depression of the seizure threshold is hypothesized. The diminished seizure frequency after reinstitution of medications in four of five patients who did not have seizures during their hospitalization suggests that drug tachyphylaxis is a relevant mechanism

We examined material-specific memory in 45 left hemisphere language dominant patients with temporal complex partial seizures (24 right, 21 left) during the intracarotid amobarbital procedure (IAP) by showing eight cards displaying two line drawings of common objects, two printed words, one colored shape, one math expression, one face, and one abstract shape following amobarbital injection (mean = 109.9 mg). We assessed delayed recall and recognition following clearing. Patients with right foci recognized significantly fewer verbally mediated stimuli (words, object drawings, colored shape) with left than with right injection. Patients with left foci recognized a nonverbal stimulus (abstract shape) more poorly following right versus left injection. Discriminant function analysis lateralized 85% of the sample from memory predictors, upheld to 81% on crossvalidation. Material-specific memory remains intact in the hemisphere contralateral to a seizure focus, but wider representation may occur for stimuli normally dominant for the hemisphere with the seizure focus. The IAP significantly lateralizes a seizure focus with use of both types of stimuli

We studied 92 secondary generalized tonic-clonic seizures (SGTCS) in 29 patients with a clearly lateralized seizure focus using video-EEG telemetry. An examiner, blind to the EEG, reviewed direction and type (forced versus nonforced) of head/eye deviation (HD). Forced HD consisted of sustained, unnatural tonic or clonic movements. Nonforced HD consisted of sustained deviations that were neither tonic nor clonic and were similar to volitional head movements. Eighty-three of the 92 SGTCS (26 of 29 patients) had lateralized and sustained HD. The direction of HD was contralateral in over 90% of seizures when the movement either (1) continued as the seizure generalized, or (2) occurred in the 10 seconds prior to generalization. The direction of HD was ipsilateral in over 90% of seizures if the movement ended before the seizure began to generalize (in all cases, the movement ended more than 10 seconds before generalization). HD occurring within the first 10 seconds after seizure onset was not of lateralizing significance. Forced HD was 89% contralateral, while nonforced HD was not of lateralizing significance. HD in SGTCS provides information on seizure focus lateralization

The review of previous research and the studies undertaken at our center suggest an important role for mapping of nonlinguistic cognitive functions with cortical stimulation. Such mapping is usually restricted to language functions, which is understandable given the importance of language to daily activities and the reports of postoperative declines in naming. However, memory functions are also critical to adaptive daily living, and these studies show that memory may be effectively addressed through mapping procedures. Both Ojemann's studies and those at our center suggest an important role for much of the temporal convexity in memory processing, and we believe that postoperative memory declines can be minimized with mapping of the temporal lobe prior to resection. Patients presenting with unique abilities that may be susceptible to postoperative declines can also be effectively mapped with stimulation studies tailored to their unique abilities. We encourage the expansion of mapping paradigms beyond just the language domain and believe that such studies will both improve the quality of our patients' functioning and further our knowledge of brain-behavior relationships

Presents the cases of 2 right-handed patients (a 62-yr-old man and a 24-yr-old woman) with ictal and postictal apraxia. The Ss were unable to perform previously learned motor tasks either during or after a partial seizure arising in the left temporal region with rapid spread to the left frontoparietal areas. The Ss were able to recall and clearly describe the apraxic nature of their deficit. These cases are consistent with an apraxia resulting from inhibition of dominant hemisphere association cortex subserving complex motor acts.

Identified 37 patients (aged 11-57 yrs) with juvenile myoclonic epilepsy (JME) out of 740 consecutive epilepsy patients. Only 3 were initially diagnosed with JME. Factors leading to misdiagnosis included warnings (auras) suggesting partial seizures before generalized tonic-clonic seizures (GTCS), confusion between absence and complex partial seizures, failure of patients to spontaneously report or for physicians to obtain a history of myoclonic jerks, and failure to report myoclonic jerks to medical personnel despite specific questioning. Psychiatric disorders were common: depression occurred in 9 Ss and panic disorder in 7 Ss. Following diagnosis, 86% of Ss were seizure-free on antiepileptic drugs. To improve diagnostic sensitivity, Ss with epilepsy should be routinely questioned about the occurrence of myoclonic seizures, and JME should not be excluded because patients report nonspecific warnings before GTCS.

We report seven patients with crying during video-EEG-documented simple or complex partial seizures. During simple partial seizures, crying occurred with or without appropriate affect. Crying occurred postictally in two patients and was associated with persistent spiking in one of them. Six patients had ictal activity in the nondominant hemisphere, maximal in the anteromesial temporal region in five and in the mesial frontal region in another. These cases support theories proposing a lateralization of emotion, with right hemisphere dominance for negative affective states

In 5 cases suffering from intractable seizures and ictal onset in exquisite (primary somatosensory or language related) cortex, surgical therapy has been done consisting wholly or in part of multiple subpial transections. In two cases with involvement of the primary somatosensory cortex, good seizure control without detectable neurological deficit was achieved. In the other three cases with involvement of the language cortex, deficits were minimal and cleared with time. Patients became seizure-free