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In 15 patients we hypothesized the origin of epilepsies to be ‘extratemporal’ based on videotaped seizures and surface EEG. Neuropsychological tests and neuroimaging (CAT, MRI, and PET scans) were then compared to the hypothesized ictal sites. Neuropsychological tests were abnormal in 86.6% and FDG-PET scans were abnormal in 73%. The neuropsychological tests and PET localized or lateralized areas of dysfunction to the same sites as electroclinical characteristics did in 85% of patients ( P , Fisher exact test). No statistically significant correlation between lesion sites on CT and MRI and the ictal origin was observed due to the high proportion of normal or non-specific scans. These observations should be verified in a larger series of extratemporal seizures.

Hippocampal Cell Loss in Posttraumatic Human Epilepsy. Swartz BE, Houser CR, Tomiyasu U, Walsh GO, DeSalles A, Rich JR, Delgado-Escueta A. Epilepsia 2006;47(8): 1373–1382. Purpose: We performed this study to determine whether significant head trauma in human adults can result in hippocampal cell loss, particularly in hilar (polymorph) and CA3 neurons, similar to that observed in animal models of traumatic brain injury. We examined the incidence of hippocampal pathology and its relation to temporal neocortical pathology, neuronal reorganization, and other variables. Methods: Twenty-one of 200 sequential temporal lobectomies had only trauma as a risk factor for epilepsy. Tissue specimens from temporal neocortex and hippocampus were stained with glial fibrillary acidic protein (GFAP) and hematoxylin and eosin (H&E). Eleven hippocampal specimens had additional analysis of neuronal distributions by using cresyl violet and immunolabeling of a neuron-specific nuclear protein. Results: The median age at onset of trauma was 19 years, the median time between trauma and onset of seizures was 2 years, and the median epilepsy duration was 16 years. The length of the latent period was inversely related to the age at the time of trauma ( r = 0.75; Spearman). The neocortex showed gliosis in all specimens, with hemosiderosis ( n = 8) or heterotopias ( n = 6) in some, a distribution differing from chance ( p = 0.02; Fisher). Hippocampal neuronal loss was found in 94% of specimens, and all of these had cell loss in the polymorph (hilar) region of the dentate gyrus. Hilar cell loss ranged from mild, when cell loss was confined to the hilus, to severe, when cell loss extended into CA3 and CA1. Some degree of mossy fiber sprouting was found in the dentate gyrus of all 10 specimens in which it was evaluated. Granule cell dispersion ( n = 4) was seen only in specimens with moderate to severe neuronal loss. Conclusions: Neocortical pathology was universally present after trauma. Neuronal loss in the hilar region was the most consistent finding in the hippocampal formation, similar to that found in the fluid-percussion model of traumatic head injury. These findings support the idea that head trauma can induce hippocampal epilepsy in humans in the absence of other known risk factors.

BACKGROUND The popularity of subdural electrodes for the presurgical evaluation of patients with intractable seizures is increasing. However, few reports have prospectively dealt with their efficacy and safety. METHODS We conducted a 5-year prospective study of patients evaluated by the California Comprehensive Epilepsy Program, who subsequently underwent subdural electrode implantation at one of two institutions. Efficacy was examined by ultimate outcome with regards to postsurgery resection seizure frequency. Fifty-five patients underwent 58 implant procedures and postresection outcomes were available in 47 patients. Safety was defined by the incidence of expected and unexpected complications, and neuropathologic examination of tissue specimens. RESULTS The most common expected adverse effects during implant were fever ≤102° (41%), cerebrospinal fluid leakage (19%), headache (15%), and nausea (4%). There were no infections. Unexpected adverse events included fever > 102°F (5%), migraine (5%), iatrogenic electrode dysfunction (5%), and temporalis muscle fibrosis (5%). The incidence of pathologic findings suggestive of foreign body reaction was 10%. There were no permanent sequelae. Surgical outcomes were excellent in all (≥75% seizure reduction) with 50% seizure free regardless of the focus. CONCLUSIONS Subdural electrodes are a safe, easy, and efficacious tool for evaluating seizure foci prior to resective surgery. They should no longer be considered investigational devices.

Eight patients were diagnosed as having extratemporal seizures based on their ictal electroclinical manifestations. The correlation between the focus based on semiology and that based on intracranial electrographic recording and surgical excision was excellent in five, good in three. The electroclinical manifestations of seizures are important data to consider in the diagnosis and treatment of extratemporal seizure foci.

To determine whether herpes simplex virus causes monofocal epilepsy and to assess the presence of herpes simplex virus 1 (HSV-1) and HSV-2 in surgical specimens from patients with epilepsy by using polymerase chain reaction and Southern blot analysis.Herpes simplex virus is a common neurotropic virus capable of latency within the central nervous system; it has a predilection for the temporal lobe. Central nervous system infection with HSV has been associated with seizure activity.Surgical specimens were removed from 50 patients as part of a treatment protocol for monofocal epilepsy. Neuropathological classification was done, and adjacent sections were screened for HSV by using polymerase chain reaction. Tissues obtained post mortem from the temporal lobe cortex of persons with Alzheimer disease (n=17), Parkinson disease (n=14), or nonneurological disease (n=17) served as controls.Twenty (40%) of the 50 epilepsy cases and 2 (4%) of the 48 control cases had at least one sample that tested positive for HSV (P.001). Sixty-seven percent (8/12) of the epilepsy cases with heterotopia were positive for HSV.There was a statistically significant difference in the frequency of HSV-positive surgical specimens from monofocal seizure epicenters compared with nonepilepsy control specimens. These data suggest an association of the virus with seizure activity. All specimens positive for HSV (surgical specimens and control specimens) should be examined to determine the activity or latency state of the virus and cellular localization.

Antiepileptic Drug Treatment The Start of Therapy Antiepileptic drug treatment is obligatory when a diagnosis of generalized or partial epilepsy has been made.31 The aim is to control epileptic seizures in the absence of systemic or neurologic toxicity. Treatment should be started with one antiepileptic agent, preferably a first-choice drug. Table 3 summarizes some of the pharmacologic properties of the antiepileptic drugs most commonly used as single agents (monotherapy). The practitioner should try to achieve plasma concentrations that are known to stop seizures (the therapeutic range). In general, because plasma clearance is initially lower in "naive" patients — i.e., those who have . . .

We studied the first clinical manifestations of 72 complex partial seizures (CPS) in 17 drug-resistant patients. CPS were indicated to be of hippocampal-amygdalar origin by scalp and depth EEG. We asked: (a) Do all CPS of hippocampal-amygdalar origin start with an initial motionless stare and/or oroalimentary automatisms? (b) If not, what other clinical manifestations appear at onset of the CPS? Results showed that approximately 39% of CPS begin with motionless staring, 25% with nonfocal discrete movements, 21% with oroalimentary automatisms, 10% with perseverative stereotyped automatisms, and 6% with vocalizations. Nonfocal discrete movements and oroalimentary automatisms were identified as the most common second and third clinical sequential manifestations during a CPS. We conclude that although approximately 60% of CPS of hippocampal-amygdalar origin start with motionless staring or oroalimentary automatisms, 40% do not.

As early as 400years B.C., Hippocrates [26] recognized the warning signs or symptoms (aura) of epilepsy. In 1677, Thomas Willis [59] gave the first description of a psychomotor attack, and in 1822 Prichard [42] first used the term “partial epilepsy.” However, it was John Hughlings Jackson who developed the conceptual framework and meaning of epileptic symptomatology and signs. Jackson, a true scholar, completely refounded epileptology and collated bedside observations with anatomical facts. By 1889, he [24, 25] had linked the symptoms and signs of psychomotor seizures to the “sphenoidal lobe” or the medial temporal areas of the uncus. Since then, modern studies, notably by Ajmone-Marsan and Abraham [1–3], Bancaud, Talairach and collaborators in Paris [4–12,20,21, 31–33,48, 53], Wieser and Ya§argil [52, 54, 55] in Zurich, the Spencers et al. [45, 46] and Williamson et al. [56–58] in New Haven, Rasmussen [44], Gloor [22, 23], and Quesney et al. [43] in Montreal, and our Comprehensive Epilepsy Program in Los Angeles [12–15, 34], have characterized a wide range of psychomotor automatisms on cine film or closed-circuit television videotape (CCTV) from simple lip smacking, chewing, pursing lips, and, eye blinking, to running, mimicry, gesticulations, and sometimes bizarre complex bilateral hand and leg movements. These clinical signs have been related to epileptiform discharges recorded by scalp and sphenoidal electroencephalography (EEG) and stereoelectroencephalography (SEEG).

• Hemispheric language dominance, as determined by intracarotid amobarbital sodium injections, and handedness, as reflected by writing and drawing preference, were evaluated in a select group of patients with intractable seizures who had documented focal epilepsy originating from one temporal lobe. Of the patients with left temporal lobe seizure focus, an unusually high percentage of right-handed patients (4/26 [15%]) had right hemisphere language dominance. Pathologic findings of the resected temporal lobe in these patients revealed microscopic damage (hippocampal sclerosis) of the hippocampus in three of three cases; one patient also had a small hamartoma in the midtemporal gyrus. We hypothesize that crossed dominance resulted from disruption by epileptiform activity during early development of selective areas of the left hemisphere.

THE SPECIFIC effects of glucocorticoids on muscle histology have attracted much attention since the recognition that these compounds could induce muscle weakness. Although there have been numerous reports of the light microscopic and ultrastructural changes in muscle under these circumstances, 1-10 they have not been correlated with the histochemical alterations. 11 Furthermore, the type of muscle fiber most severely affected has not been clarified, with considerable variation in these observations amongst various reports. 6,11,12 Part of this inconsistency can be attributed to the species variation of muscle, as measured by the morphological differences and by the pathological response to glucocorticoids. In this report, the enzyme histochemical and ultrastructural changes in rat muscle following adrenal corticotrophic hormone (ACTH), glucocorticoids, and starvation will be described, in addition to changes in average muscle fiber diameters. Materials and Methods Forty-eight Osborn-Mendel rats, weighing 237 to 494 gm, were used. The food containers and

SUMMARYA patient has been described with Moebius syndrome in whom there was clinical paralysis of the muscles supplied by the third, fourth, sixth, and seventh cranial nerves. Limb muscle biopsy studies and electromyography of the limb muscle suggest denervation of an “anterior horn cell” type of abnormality in the spinal cord. Moebius was also of the opinion that the syndrome was secondary to lower motor neuron abnormality and that the cord could be involved. The hypogonadism of our patient was secondary to a deficiency of gonadotropic hormone.

The recommendation for vesicourethropexy is often made without adequate preoperative evaluation. The necessity to demonstrate urinary leakage during an increase in intra-abdominal pressure is fundamental to recommending this particular operative procedure. This report is of a woman complaining of continuing incontinence who had been advised to undergo vesicourethropexy by several urologists. Preliminary investigation did not reveal the typical findings of stress incontinence, and neurologic evaluation confirmed the presence of a brain neoplasm. The circumstances which led to the correct diagnosis and to incontinence caused by neoplasms of the central nervous system are discussed.

The effectiveness of long term EEG monitoring in the localization of the epileptic focus was studied in 37 patients with temporal lobe epilepsy comprising 190 recorded seizures, in 19 frontal lobe epileptic patients with 172 recorded seizures and in 12 additional patients which were classified as fronto-temporal. In the temporal lobe group, 49/190 seizures began focally (26%) and 20/190 seizures exhibited a regional onset (10%). In the frontal lobe group, only 21 out of 172 seizures (12%) had a focal ictal onset. 41/172 seizures began regionally (24%). In the fronto-temporal group, 31/55 seizures disclosed a focal EEG onset (57%). This study demonstrates that there is a two-fold increase in seizures beginning focally in the temporal lobe epilepsy group versus the frontal lobe group.

• We describe a patient who had a violent automatism that occurred during a partial complex seizure. The initial spike wave activity was recorded from nasopharyngeal leads that were lost as the patient began his vigorous, violent activity. This activity included nondirected, automatic, stereotyped behavior with physical assaults on objects in his path.

Seventeen patients had type I complex partial seizures (CPS) with three consecutive phases: initial motionless staring, oral-alimentary automatisms, and reactive quasipurposeful movements during impaired consciousness. Fifteen patients had stereoelectroencephalography. Focal or regional 8- to 20-Hz low-voltage epileptiform paroxysms in either hippocampus (10 patients), amygdala (1 patient), or both (1 patient) preceded initial motionless staring. Focal sphenoidal or nasopharyngeal ictal paroxysms preceded seizures in three other patients who underwent lobectomy. All 15 patients are seizure-free 2 to 11 years after temporal lobectomy. Type I CPS are most commonly of hippocampal origin.

In seven cases of idiopathic progressive ophthalmoplegia with abnormal biopsies of clinically normal or only minimally weak somatic muscles, histochemical and ultrastructural examination showed the muscle fiber abnormality to consist of clusters of normal and abnormal mitochondria and excessive lipid droplets ("ragged-red" fiber). That change was considered myopathic. The occasional small angular fibers, excessively dark with reduced diphosphopyridine nucleotide-tetrazolium reductase resembling those present in denervation, suggested an additional minor element of neuropathy in these cases. The described morphologic abnormalities, in combination with idiopathic progressive ophthalmoplegia, may represent a distinct clinicopathologic syndrome, "oculocraniosomatic neuromuscular disease with ragged-red fibers." Additional features usually include elevated cerebrospinal fluid protein levels and sometimes cardiac conduction abnormalities, retinal pigmentation, impaired growth, and sensorineural hearing defect, abnormal electroencephalogram, and ataxia.