Neuroleptic malignant syndrome.

The neuroleptic malignant syndrome is an uncommon, severe illness that consists of fever, muscular rigidity, and stupor. Various neuroleptics have been associated with the disease. A detailed neurological, medical, and neuropathological evaluation of this case was performed. Presumably, the syndrome is secondary to biochemical dysfunction of the basal ganglia and possible of the hypothalmus.

Postictal psychiatric events during prolonged video-electroencephalographic monitoring studies.

BACKGROUND: Postictal psychiatric events presenting as postictal psychotic events and postictal nonpsychotic events are known to occur following seizure clusters. Accordingly, patients undergoing prolonged video-electroencephalographic (EEG) monitoring studies may be at increased risk of experiencing postictal psychiatric event, as they often have flurries of seizures during these studies. OBJECTIVES: To determine the annual incidence and clinical characteristics of postictal psychotic events and postictal nonpsychotic events in video-EEG monitoring studies in patients with partial seizure disorders and to identify potential pathogenic factors. RESULTS: Thirteen patients met the criteria for a postictal psychiatric event during the 18-month study period, 10 presenting as postictal psychotic events and three as postictal nonpsychotic events. The annual incidence of postictal psychiatric events at our monitoring unit for 1988 was 7.8%, 6.4% presenting as postictal psychotic events and 1.4% as postictal nonpsychotic events. Seven patients had their first-ever postictal psychiatric event during the monitoring study. In 12 of the 13 patients, the postictal psychiatric events mimicked well-defined psychiatric entities of shorter duration (mean, 66.5 hours); they appeared 12 to 72 hours after the last seizure and remitted spontaneously or with the use of low-dose psychotropic medication. No significant differences in EEG, neuroradiologic, psychiatric, medical, or psychosocial data were found between the patients with postictal psychiatric events and a group of 13 age-matched control patients. Follow-up data of comparable duration were available in nine patients with postictal psychiatric events and nine controls. Psychiatric events were reported more frequently by patients with postictal psychiatric events than by control patients (P=.03). In three patients, postictal psychiatric events converted to interictal events. CONCLUSION: These findings suggest that monitoring studies increase the risk for postictal psychiatric events, which neurologists need to be familiar with, as they represent important morbidity associated with these studies.

Juvenile Niemann-Pick disease with vertical supranuclear ophthalmoplegia. Two cases reports and review of the literature.

Examination of two siblings who had histories of progressive decline in speech, intelligence, and coordination disclosed vertical supranuclear ophthalmoplegia, hepatosplenomegaly, and signs of diffuse CNS dysfunction. Niemann-Pick "foam cells" were found in the bone marrow of both patients. The features of these cases correlate in appearance and clinical findings with those of 21 other previously reported cases, which are reviewed in this article. Knowledge of the clinical manifestations of this particular variety of Niemann-Pick disease should aid in its earlier diagnosis.

The value of PET scan (and MRI and Wada test) in patients with bitemporal epileptiform abnormalities.

BACKGROUND: Lateralization remains difficult in patients with bitemporal epileptiform abnormalities. OBJECTIVE: To evaluate the reliability of the interictal fluorodeoxyglucose F18-positron emission tomographic (PET) scan in this setting. METHODS: We analyzed PET scan findings in 25 patients who required bilateral depth electrodes for lateralization of temporal lobe epilepsy. The contribution of magnetic resonance imaging and the intracarotid amobarbital procedure was also analyzed. RESULTS: Positron emission tomographic scan revealed lateralized hypometabolism in 15 patients. This was in agreement with the depth evaluation in 10 cases (67%). In five cases (33%), the PET and depth findings were in disagreement, with no predominant side of seizure origin by depth electroencephalography (EEG) in four cases and PET lateralization opposite to depth findings in one case. In 10 of the 25 patients, PET scans could not lateralize hypometabolism to one temporal lobe, of which six were confirmed by depth EEG not to have a predominant side of seizure onset. The overall sensitivity of PET for concordant lateralized seizure onset was 67%. Ten of 14 patients who underwent a lobectomy had a successful outcome, including three of four patients whose temporal lobe epilepsy was lateralized by depth EEG but not by PET. One patient had PET lateralization opposite to the depth EEG and side of surgery and failed to improve. CONCLUSIONS: In this highly selected group of patients, the lateralizing value of PET and magnetic resonance imaging was somewhat diminished, and the intracarotid amobarbital procedure was not found useful for lateralization. Positron emission tomography and magnetic resonance imaging, when pointing to the side opposite depth EEG, may indicate a poor prognosis.

Etiologic factors for unitemporal vs bitemporal epileptiform discharges.

We compared the etiologic factors and clinical characteristics of 30 patients with unitemporal vs those of 30 patients with bitemporal independent (minimum 20% from one side) interictal epileptiform discharges on extracranial electroencephalograms. Febrile seizures occurred significantly more frequently in the unitemporal (40%) than in the bitemporal (17%) group. Mass lesions were more common in the bitemporal group, and seven of 10 patients with mass lesions showed bitemporal interictal epileptiform discharges. There were no statistically significant differences in age at onset, frequency of seizures, duration of epilepsy, and history of central nervous system infection or trauma between the two groups. A history of febrile seizures or central nervous system infection that may be expected to cause diffuse cerebral injury does not appear to be the major factor predisposing to the development of bitemporal interictal epileptiform discharges.

Comparison of scalp electroencephalogram with subdural electrocorticogram recordings and functional mapping in frontal lobe epilepsy.

We compared the findings of scalp electroencephalogram with subdural electrode array (SEA) recordings in 19 patients with refractory frontal lobe epilepsy. Prolonged scalp interictal recordings localized the epileptogenic zone in 12 patients; seven had no interictal sharp waves. The SEAs showed multifocal interictal sharp waves in all patients. Seven patients with localized seizure onset on scalp recording showed extensive ictal onset on the SEA recording. Five patients with lateralized seizure onset to one hemisphere on scalp recording were found to have ictal onset on SEA restricted to a smaller area. Because of the large epileptogenic zone found on SEA recordings, a complete resection was possible in only five (33%) of the 15 patients who had resections. Eight (53%) of the 15 patients benefited from surgery (mean follow-up, 4.6 years). The SEAs also allowed functional localization in most patients. From these data, we suggest that a localizing scalp electroencephalogram in patients with frontal lobe epilepsy may be misleading because SEA recordings show larger epileptogenic zones than anticipated. Furthermore, we postulate that the larger extensive epileptogenic zone may account for the poorer surgical outcome in patients with frontal lobe epilepsy compared with patients with temporal lobe epilepsy.

Electrophysiologic pathologic correlations in patients with complex partial seizures.

We studied interictal activity and site of ictal onset in 26 patients with complex partial seizures of temporal lobe origin. All patients had prolonged electrocorticographic recordings from subdural electrode arrays placed both over the convexity and beneath the temporal lobe. We found a significant correlation between the epileptogenic focus and the type of pathologic lesion found at time of surgery. Macroscopic lesions strongly tended to have an epileptogenic focus on the lateral surface of the temporal lobe; patients with only microscopic abnormalities tended to have an epileptogenic focus in the mesial/basal region of the temporal lobe.

Presurgical evaluation of temporal lobe epilepsy using interictal temporal spikes and positron emission tomography.

OBJECTIVE: Our goal was to determine the role of fludeoxyglucose F 18-positron emission tomography (18FDG-PET) and interictal temporal spikes in lateralizing the epileptogenic region in patients who (1) were diagnosed as having temporal lobe epilepsy based on clinical symptoms and exclusively temporal interictal spikes and (2) did not have a structural lesion on magnetic resonance imaging. DESIGN: This was a retrospective study of 40 consecutive patients fulfilling the above criteria who underwent 18FDG-PET scanning. A firm electrophysiologic diagnosis and 1 complete year of postsurgical follow-up, where applicable, were required. Outcome measures included surgical outcome and final electrophysiologic diagnosis. RESULTS: Unilateral, interictal temporal spikes (ITS) were present in 33 (82.5%) of 40 patients. Seven patients (17.5%) had bitemporal, independent spikes. Thirty-one (77.5%) of 40 patients had unilateral temporal hypometabolism (TH). Twenty-eight (70%) patients had concordant TH and ITS. One year after surgery, 31 of 33 patients with unilateral ITS were greatly improved; two of five who had bitemporal ITS showed similar improvement. In 28 patients, unilateral TH and unilateral ITS were concordant. The paired result always concurred with the final neurophysiologic assessment. Surgical outcome between patients with 18FDG-PET showing unilateral TH (26 of 30 greatly improved) and those not showing unilateral TH (six of eight greatly improved) was not significantly different. CONCLUSION: In temporal lobe epilepsy not associated with a mass lesion, unilateral ITS are reliable lateralizing features and suggest a good surgical outcome. Use of 18FDG-PET provides corroborative lateralizing information but 18FDG-PET that fails to show unilateral TH does not preclude a good surgical outcome.

Magnetic resonance imaging (1.5 tesla) in patients with intractable focal seizures.

Magnetic resonance (MR) (1.5 tesla) studies were performed in ten patients with temporal lobe epilepsy and two with temporofrontal epilepsy. Two patients with temporal lobe epilepsy and one with temporofrontal epilepsy exhibited areas of increased signal intensity on T2-weighted images in the mesiobasal portion of the temporal lobe shown by electroencephalography to be the epileptogenic focus; no analogous abnormalities had been found in these patients on computed tomographic scans. Pathologic studies have not revealed a specific ultrastructural correlate for the MR findings in this group of patients. We found MR to be a useful, noninvasive diagnostic adjunct in the presurgical assessment of some patients with temporal lobe epilepsy. Where abnormalities were found, they corresponded with the epileptogenic focus as defined by electroencephalography.

Hair loss after prolonged EEG/video monitoring.

We report five patients who experienced delayed patchy hair loss after prolonged EEG monitoring. The location of the patches corresponded to the electrode sites in four. The hair loss was temporary and most probably was due to traumatic noncicatricial alopecia. The frequency of hair loss was approximately 2% of monitored patients.

Effect of cranial irradiation on seizure frequency in adults with low-grade astrocytoma and medically intractable epilepsy.

We report the effect of cranial irradiation on seizure frequency in five adults with unresected (biopsy-proven) cerebral hemisphere low-grade astrocytoma and medically intractable epilepsy. Seizures were refractory to standard antiepileptic drugs for 7 months to 27 years. Treatment with 5,400 cGy to 6,120 cGy focal radiation reduced seizure frequency by more than 90% in three patients (one of whom became seizure-free) and by more than 75% (but less than 90%) in one patient. One patient had no response. Brain CT or MRI showed a partial tumor response to radiation in three of the four patients with reduced seizure frequency. In three patients, the reduced seizure frequency continued to the most recent follow-up of 1 to 1.5 years. In the patient who became seizure-free, seizures recurred at 8.2 years and were associated with tumor progression. Irradiation can reduce seizure frequency in some patients with unresected cerebral hemisphere low-grade astrocytoma and medically intractable epilepsy.

Asterixis associated with sodium valproate.

Intoxication with most anticonvulsants can produce asterixis. Asterixis rarely occurs with therapeutic serum anticonvulsant levels. We report two patients with asterixis who were taking valproic acid and had serum levels within the therapeutic range. Neither patient had clinical or laboratory evidence of hepatotoxicity. Only one other patient has been reported with valproate-associated asterixis in the absence of toxic serum drug levels or hepatotoxicity. Asterixis seems to be due to a central effect of the drug unrelated to hepatotoxicity or sedation.

Memory for objects presented soon after intracarotid amobarbital sodium injections in patients with medically intractable complex partial seizures.

We evaluated the abilities of 36 patients with intractable temporal lobe epilepsy and left hemisphere dominance for language to later recognize objects presented in the confusional phase after left intracarotid amobarbital injection. Eighteen of 24 patients with left, but only 4/12 with right, temporal lobe epilepsy recognized at least two-thirds of objects during a post-test. These results demonstrate that the initial muteness and apparent confusion after amobarbital injection do not prohibit the formation of new memories; this gives further support to the idea that consciousness can be retained despite transient disruption of function of the language-dominant hemisphere.

Cortical generators of somatosensory evoked potentials to median nerve stimulation.

In 12 patients with intractable partial seizures, chronically implanted subdural electrodes were used to define the relationship of the epileptogenic focus to cortical functional areas. Cortical somatosensory evoked potentials (SEPs) to median nerve stimulation were recorded from these electrodes. The initial cortical positivity, postrolandic primary cortical potential (PCP), was recorded in all 12 patients with a mean latency of 22.3 +/- 1.6 msec. A potential of opposite polarity, prerolandic PCP, was defined in nine patients with a mean latency of 24.1 +/- 2.7 msec. The latency of the postrolandic PCP was 1.61 +/- 1.59 msec shorter than the prerolandic PCP (p less than 0.01, paired t test). The maximum amplitude postrolandic PCP was 2.1 times larger than the maximum prerolandic PCP (p less than 0.02, paired t test). The phase reversal of the SEPs was compared with the position of the rolandic fissure (RF) defined by electrical stimulation. This study shows that the latency and amplitude characteristics of post- and prerolandic PCPs are significantly different and give support to the concept that they are produced by different generators; and cortical SEPs are helpful in locating the RF.

Sensitivity and specificity of asymmetric recall on WADA test to predict outcome after temporal lobectomy.

Some reports suggest that the intracarotid amobarbital test (IAT) is useful for predicting good seizure outcome after temporal lobectomy. The sensitivity, specificity, and predictive value of the IAT in this condition has not been previously studied. We designed this study to establish the value of memory recall asymmetry on the IAT as a predictor of outcome after temporal lobectomy. We studied memory recall on the IAT in 108 consecutive patients with intractable epilepsy who underwent presurgical evaluation for temporal lobectomy and had at least 1 year follow up after surgery. At a level of 30% asymmetry of recall, specificity for favorable outcome (Engel Class I and II) was 100% (95% confidence interval [CI], 85 to 100), sensitivity 51% (95% CI, 40 to 62), positive predictive value 100% (95% CI, 92 to 100), and negative predictive value 34% (95% CI, 23 to 47). At the same level of asymmetry, specificity for seizure-free outcome (Engel Class I) was 88% (95% CI, 68-95), sensitivity 37% (95% CI, 40 to 64), positive predictive value 87% (95% CI, 71 to 96), and negative predictive value 38% (95% CI, 27 to 50). Asymmetric recall on the IAT is highly specific but not very sensitive in predicting outcome after temporal lobectomy.

Supplementary motor seizures: clinical and electroencephalographic findings.

The clinical and EEG features of 11 patients with seizures arising in the supplementary motor area (SMA) were reviewed. All patients underwent prolonged EEG with simultaneous video recording. Three patients had recordings and electrical stimulation of the SMA using subdural electrode arrays. All patients had preservation of consciousness during the seizure unless it became secondarily generalized. Tonic posturing of the extremities was present in all patients, and in seven it was present bilaterally. Adversive movements were not seen unless the seizure became secondarily generalized. Interictal and/or ictal abnormalities were present at or adjacent to the midline in ten patients. Seizures arising from the supplementary motor region are clinically distinct, and the diagnosis can almost always be verified with prolonged EEG/video recording.

Transient neuropsychological abnormalities (including Gerstmann's syndrome) during cortical stimulation.

A patient with intractable partial seizures was intensively studied before surgical removal of the epileptogenic focus. A subdural electrode array was surgically placed over the left temporoparietal cortex to better localize the epileptogenic focus and localize cortical function. In addition to speech and sensory findings, acalculia, agraphia, right-left confusion, and finger agnosia were transiently produced by electrical stimulation in the perisylvian area. These findings and their relationship to the controversy surrounding Gerstmann's syndrome are discussed.

Versive eye movements elicited by cortical stimulation of the human brain.

We studied the eye movements (EM) elicited by electrical stimulation of the frontal lobe in 19 awake patients evaluated with subdural electrodes for epilepsy surgery. All patients had only contralateral conjugated EM. They were saccadic in 16 patients (84%). Head version, always following the eye deviation, occurred in 11 patients (58%). We also determined the eye field somatotopic distribution analyzing the responses obtained from the electrodes adjacent to the eye fields. All patients had motor cortex contiguous to the eye fields. In 17 patients (90%) the eye fields were located in front or at the level of the motor representation. There was no silent cortex between the motor strip and the eye fields.

Ipsilateral trigeminal sensory responses to cortical stimulation by subdural electrodes.

Twelve patients with medically intractable epilepsy had plates of chronic subdural electrodes placed over the lateral and basal cortical hemispheres during evaluations for surgical therapy. During cortical stimulation, ipsilateral sensations involving any of the branches of the trigeminal nerve were noted in the eye, face, and mouth. Some responses could have been due to dural or direct trigeminal nerve trunk stimulation, but others were probably due to electrical stimulation of trigeminal fibers accompanying the pial-arachnoidal vessels. These fibers had been demonstrated in animals, but not in humans.

The lateralizing significance of versive head and eye movements during epileptic seizures.

We studied 37 patients who had head and eye turning during 74 spontaneous epileptic seizures. Videotapes and EEGs were analyzed independently. Turning movements were classified without knowledge of EEG or clinical data as either versive (unquestionably forced and involuntary, resulting in sustained unnatural positioning) or nonversive (mild, unsustained, wandering, or seemingly voluntary). Videotape observations were then correlated with the EEG location of seizure onset. Contralateral versive head and eye movements occurred during 61 seizures in 27 patients, but ipsilateral versive movements did not occur. Nonversive lateral head and eye movements occurred ipsilaterally and contralaterally with equal frequency and were nonlocalizing, but versive movement was a reliable lateralizing sign.