Purpose. To report the outcomes of the 58 hemispherectomies performed at Johns Hopkins between 1968 and January 1996. Methods. Charts were reviewed of the 58 hemispherectomies performed at Johns Hopkins Medical Institutions by the Pediatric Epilepsy Group during the years 1968 to 1996. Twenty-seven operations were done for Rasmussen's syndrome, 24 operations for cortical dysplasias/hemimegalencephalies, and 7 for Sturge-Weber syndrome or other congenital vascular problems. Seizure control alone did not seem to adequately describe the outcomes of the procedure. Therefore, a score was constructed that included seizure frequency, motor disability, and intellectual handicap. This burden of illness score better described the child's handicap before and after surgery. Results. Perioperative death occurred in 4 out of 58 children. Of the 54 surviving children, 54% (29/54) are seizure-free, 24% (13/54) have nonhandicapping seizures, and 23% (12/54) have residual seizures that interfere to some extent with function. Reduction in seizures was related to the etiology of the unilateral epilepsy. Eighty-nine percent of children with Rasmussen's, 67% of those with dysplasias, and 67% of the vascular group are seizure-free, or have occasional, nonhandicapping seizures. All operations were considered by the parents and the physicians to have been successful in decreasing the burden of illness. In 44 the procedure was very successful, in 7 it was moderately successful, and in 3 it was minimally successful. Success was related to the etiology, and early surgery was preferable. Conclusion. Hemispherectomy can be a valuable procedure for relieving the burden of seizures, the burden of medication, and the general dysfunction in children with severe or progressive unilateral cortical disease. Early hemispherectomy, although increasing the hemiparesis in children with Rasmussen's syndrome, relieves the burden of constant seizures and allows the child to return to a more normal life. In children with dysplasias, early surgery can allow the resumption of more normal development.

To clarify the exact anatomic relationship of electrically identified hand areas to the central sulcus, we constructed cortical surface renderings of magnetic resonance images (MRI) to locate the central sulcus accurately and measured the distances of stimulated points from the central sulcus and the Sylvian fissure. We obtained hand responses in 33 patients who underwent implantation of subdural grid electrodes for evaluation and surgical treatment of intractable epilepsy and analyzed these responses according to the presence of motor, sensory, mixed motor and sensory, and arrest responses. Hand motor responses occurred not only in the precentral gyrus but also in the postcentral gyrus, with great variability in superior-to-inferior distribution. Sensory responses also occurred in both the precentral and postcentral gyri with a distribution more ventral than that of motor responses. Mixed motor and sensory responses tended to be limited to the middle part of the central sulcus. Sites where electrical stimulation arrested simple hand repetitive voluntary movements occurred widely throughout the premotor and primary sensorimotor cortices. These data indicate a marked variability in the location of the human cortical hand area, and suggest that motor and sensory hand cortices overlap and are not divided in a simple manner by the central sulcus.

Electrical stimulation of the basal temporal region of the dominant hemisphere before partial temporal lobectomy for epilepsy sometimes produces temporary interruption of language function, but the significance of removal of this area is unknown. We evaluated the correlation between resection of the basal temporal language areas (BTLA) and certain types of postoperative language deficits. In a population of 25 patients, we mapped the inferolateral temporal lobe with cortical electrical stimulation, verifying the positions of the stimulating electrodes with three-dimensional computed tomography (CT). Eighty percent of the patients developed transient language deficits with stimulation, particularly with tests of confrontation naming and comprehension. BTLA was primarily located in the fusiform gyrus, from 1 to 9 cm from the temporal tip. At testing 6-12 months after operation, patients with BTLA resection performed worse (mean 9% decrease) than those with no BTLA resection (mean 4% improvement) on tests of confrontation naming (p = 0.03). Resection size accounted for less of the variance in decrease in confrontation naming than did resection of the BTLA. Tests of performance I.Q. (PIQ), verbal I.Q. (VIQ), or recognition memory for word and verbal learning showed no significant difference between these groups. Most patients do not have language decrease with removal of basal temporal lobe 5-6 cm from the tip, even with removal of BTLA. Some patients, however, have persistent decrease in naming.

Multiple subpial transection (MST) is a new surgical technique for treating seizures that arise from functionally critical cortical areas. It has a reported efficacy comparable to that of standard temporal lobe resections. Although the mechanism through which MST works is unknown, the carefully controlled lesions, placed 5 mm apart at the midlevel of the cortical gyri, could produce fiber damage that would prevent horizontal synchronization and spread of epileptic discharges while allowing normal cortical functions such as those related to movement or speech to be preserved. We studied the acute neuropathological features associated with MST in 8 patients with intractable temporal lobe epilepsy. Transections were made along major temporal gyri just before standard lobectomy was performed. After resection, tissue was processed by conventional histological and immunocytochemical techniques. Macroscopically, subpial transections (STs) were perpendicular to the main gyral axis and had an appropriate spacing. Microscopically, most of the lesions were perpendicular and at midlevel. However, many transections involved the lateral aspects of the small gyri, resulting in oblique or deep STs, some of which reached the gray-white matter junction due to the complex microscopic neocortical architecture, in which small gyri are superimposed on major lobar gyri, and to the variable cortical thickness. Extensive acute pyknosis and tissue edema were also evident adjacent to the transections. These changes were variable and extenDed 1-3 mm laterally as irregular columnar blocks. In the deep lesions, myelin pallor and decreased neurofilament immunoreactivity were observed in the white matter. Based on the distribution of STs and their adjacent parenchymal injury, we conclude that this technique produces block-type lesions that probably disrupt propagation of epileptogenic activity. In most instances, midlevel horizontal fibers are damaged; one third of the cases showed additional deep injury that would sever afferent and efferent axons. Therefore, in addition to horizontal desynchronization, a deafferentation mechanism involving different fiber systems may contribute to the anti-seizure effects of MST. We hypothesize that preservation of cortical function is mediated by cortex remaining in the sulcus and gyral crown and possibly by reorganization of tissue adjacent to transections.

This report describes the diagnosis of progressive multifocal leukoencephalopathy (PML) in nine patients using cytopathologic and histopathologic examination of computed tomographically guided stereotactic brain biopsies in combination with immunostaining for SV-40-related antigen and the polymerase chain reaction (PCR) for the JC virus. In four patients the diagnosis of PML was based on the microscopic appearance of the biopsies and immunostaining for SV-40-related antigen. In one of these patients the diagnosis was also supported by PCR for the JC virus. In two patients whose biopsies were only suggestive of PML, a definitive diagnosis was possible utilizing immunohistochemistry and PCR. In another case the histopathologic features were atypical of PML, and the diagnosis was established with immunostaining and PCR. The diagnosis of PML was established by PCR alone in two patients whose biopsies showed only suggestive or nonspecific findings. We conclude that the accuracy of stereotactic biopsy in the diagnosis of PML is enhanced by using a combination of light microscopy, immunohistochemistry and PCR.

The intracranial spatiotemporal distributions of brainstem auditory evoked potentials were analyzed in two patients with epilepsy to study the dipole directions of waves III and V, previously found to have a horizontal and a vertical dipole, respectively. Depth electrodes were implanted bilaterally into the frontal and temporal lobes of these patients, the targets including the amygdala and hippocampus which are close to the upper pons and midbrain. Recordings around the upper pons and from the subcortical area downwards along the dorsal aspect of the upper pons indicated that waves III and V are constructed of mixed dipoles, wave III also having a vertical dipole segment and wave V also a horizontal one. These results suggest that several neural activities from the upper pons to midbrain contribute to the generation of waves III and V.

Psychiatry and Clinical NeurosciencesVolume 49, Issue 3 p. S223-S225 Manual and digitizer measurements of amygdalohippocampal volume: Reliability in comparison to computer-based measurement TOHRU HOSHIDA MD, Corresponding Author TOHRU HOSHIDA MD Department of Neurosurgery, Nara Medical UniversityDepartment of Neurosurgery, Nara Medical University, Shijyo-cho, Kashihara 634, Japan.Search for more papers by this authorTOSHISUKE SAKAKI MD, TOSHISUKE SAKAKI MD Department of Neurosurgery, Nara Medical UniversitySearch for more papers by this authorTETSUYA MORIMOTO MD, TETSUYA MORIMOTO MD Department of Neurosurgery, Nara Medical UniversitySearch for more papers by this authorHIROSHI HASHIMOTO MD, HIROSHI HASHIMOTO MD Department of Neurosurgery, Nara Medical UniversitySearch for more papers by this authorSHIN-ICHIRO KUROKAWA MD, SHIN-ICHIRO KUROKAWA MD Department of Neurosurgery, Nara Medical UniversitySearch for more papers by this authorHIROYUKI NAKASE MD, HIROYUKI NAKASE MD Department of Neurosurgery, Nara Medical UniversitySearch for more papers by this authorHIDEHIRO HIRABAYASHI MD, HIDEHIRO HIRABAYASHI MD Department of Neurosurgery, Nara Medical UniversitySearch for more papers by this authorSHOICHIRO KAWAGUCHI MD, SHOICHIRO KAWAGUCHI MD Department of Neurosurgery, Nara Medical UniversitySearch for more papers by this authorSHIGERU TSUNODA MD, SHIGERU TSUNODA MD Department of Neurosurgery, Nara Medical UniversitySearch for more papers by this authorSUMIO UEMATSU MD, SUMIO UEMATSU MD Department of Neurosurgery, Johns Hopkins Medical InstitutionsSearch for more papers by this authorRONALD P. LESSER MD, RONALD P. LESSER MD Department of Neurosurgery, Johns Hopkins Medical InstitutionsSearch for more papers by this author TOHRU HOSHIDA MD, Corresponding Author TOHRU HOSHIDA MD Department of Neurosurgery, Nara Medical UniversityDepartment of Neurosurgery, Nara Medical University, Shijyo-cho, Kashihara 634, Japan.Search for more papers by this authorTOSHISUKE SAKAKI MD, TOSHISUKE SAKAKI MD Department of Neurosurgery, Nara Medical UniversitySearch for more papers by this authorTETSUYA MORIMOTO MD, TETSUYA MORIMOTO MD Department of Neurosurgery, Nara Medical UniversitySearch for more papers by this authorHIROSHI HASHIMOTO MD, HIROSHI HASHIMOTO MD Department of Neurosurgery, Nara Medical UniversitySearch for more papers by this authorSHIN-ICHIRO KUROKAWA MD, SHIN-ICHIRO KUROKAWA MD Department of Neurosurgery, Nara Medical UniversitySearch for more papers by this authorHIROYUKI NAKASE MD, HIROYUKI NAKASE MD Department of Neurosurgery, Nara Medical UniversitySearch for more papers by this authorHIDEHIRO HIRABAYASHI MD, HIDEHIRO HIRABAYASHI MD Department of Neurosurgery, Nara Medical UniversitySearch for more papers by this authorSHOICHIRO KAWAGUCHI MD, SHOICHIRO KAWAGUCHI MD Department of Neurosurgery, Nara Medical UniversitySearch for more papers by this authorSHIGERU TSUNODA MD, SHIGERU TSUNODA MD Department of Neurosurgery, Nara Medical UniversitySearch for more papers by this authorSUMIO UEMATSU MD, SUMIO UEMATSU MD Department of Neurosurgery, Johns Hopkins Medical InstitutionsSearch for more papers by this authorRONALD P. LESSER MD, RONALD P. LESSER MD Department of Neurosurgery, Johns Hopkins Medical InstitutionsSearch for more papers by this author First published: June 1995 https://doi.org/10.1111/j.1440-1819.1995.tb02181.xCitations: 3AboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL Share a linkShare onFacebookTwitterLinked InRedditWechat Citing Literature Volume49, Issue3June 1995Pages S223-S225 RelatedInformation

てんかん焦点の側方性同定のために, MR画像からsoftwareを用いたcomputer-assisted measurement (CAM) による扁桃核や海馬の体積測定が行われ, その再現性と信頼性について報告されている。われわれは29名の部分てんかん患者を対象とし, CAM法と1mm方眼紙上に直接トレースしたdirect volume assessment (DVA) とイメージアナライザーを用いて測定したimage analyzing assessment (IAA) の3つの方法による再現性について比較検討した。1.5mmスライスのCAM法による複数回測定の変動係数は4.8%であった。一方, 1.5mmスライスでのDVA法と3mmスライスでのIAA法では3.1%と3.6%であった。同一患者では, 撮像時期が異なっていても, 体積の左右比を求めるとよく相関しており, DVA法でr=0.843, IAA法ではr=0.877であった。これらの結果から, CAM法より簡便であるイメージアナライザーを用いた扁桃核海馬の体積測定は, CAM法と同様の再現性であり, その左右比で検討することは臨床的に有用である。

Vasoconstrictive peptides and prostanoids have been implicated in the pathogenesis of hypertension and vasospasm. Recently, we have shown that human cerebromicrovascular endothelium [human brain endothelial cells (HBEC)] constitutively produces both endothelin-1 (ET-1) and prostanoids. The vasoactive peptides, arginine vasopressin (AVP) or angiotensin II (ANG II), stimulated secretion of both immunoreactive ET-1 and prostanoids from HBEC by a receptor-mediated induction of phospholipase C (PLC) and PLA2. The release of constitutive or AVP- or ANG II-induced ET-1 occurred at different rates during the 24-h incubation of HBEC in serum-free medium. The temporal profile of AVP-stimulated production of prostanoids differed from that of ANG II. AVP-induced release of prostaglandin D2 (PGD2) persisted for 24 h, whereas ANG II-stimulated PGD2 was only seen during the first 4 h of incubation. ANG II maximally stimulated PGI2 secretion during the 4- to 8-h interval, whereas AVP did not stimulate PGI2 secretion. Dexamethasone (Dxm), indomethacin (Indo), and nordihydroguaiaretic acid, the respective inhibitors of PLA2-cyclooxygenase II, cyclooxygenase, and lipoxygenase, increased both constitutive and AVP- or ANG II-stimulated secretion of ET-1. Dxm also decreased AVP- or ANG II-stimulated production of PGD2 and PGF2 alpha. These results indicate an interrelationship between HBEC production of ET-1 and prostanoids, which may play a role in regulating cerebral microcirculation.

A diffuse electrodecremental ictal pattern (DEP) has been associated with tonic seizures and, less often, with other forms of epilepsy and has been considered to reflect a generalized seizure disorder of diffuse cortical or subcortical (brainstem) origin. In some seizures associated with DEP, however, focal ictal manifestations have been observed. We reviewed the records of all patients admitted to our seizure monitoring unit for 3 years and detected 39 patients with seizures associated with DEP. In 23 of 39 patients, clinical ictal behaviors resembled seizures of unilateral supero/mesiofrontal lobe origin and interictal EEG showed a prominent unilateral frontal component. Nine of 39 had complex absences (CA)/complex partial seizures (CPS); 4 of them were of unilateral frontal lobe origin. Seven of 39 patients had tonic or atonic seizures. Seven patients were studied further with subdural electrodes. Ictal onsets showed a high-frequency frontal lobe discharge. We conclude that in a subgroup of patients a generalized electrodecremental pattern on scalp EEG results from a regional cortical high-frequency ictal discharge originating in a single frontal lobe.

The vasoactive peptide endothelin-1 (ET-1) dose-dependently increased release of 51Cr from human cerebromicrovascular endothelial cells (HBEC), without affecting cell viability as assessed by lactate dehydrogenase release. ET-1 also induced transient accumulation of inositol triphosphate (IP3) and release of [3H] arachidonic acid (AA) from HBEC. The ET-1-induced 51Cr release, formation of IP3, and AA release from HBEC were competitively inhibited by selective ETA subtype receptor antagonist BQ-123. ET-1-stimulated 51Cr- and AA release from HBEC were potentiated by proteinkinase C (PKC) activator phorbol-myristate ester, and abolished by H7, an inhibitor of PKC. Dexamethasone, indomethacin, acetylsalicylic acid, imidazole, as well as the inhibitor of protein kinase A, H8, had no effect on 51Cr release. The results suggest that ETA-receptor mediated activation of PKC and increase in the HBEC 'permeability' for low molecular weight molecules in response to excessive release of endothelins from either HBEC or surrounding tissues during pathologic conditions may contribute to the formation of cerebral edema.

Motor and sensory cortical tongue representations were examined in 40 patients with intractable seizures who underwent chronic subdural electrode grid implantation. Tongue responses were observed in a wide area 4.5 cm anterior and 3 cm posterior to the central sulcus. The distribution of the responses was not influenced by whether the responses were unilateral or bilateral. In patients with fronto-parietal lesions, the tongue motor area was located significantly more superior to the Sylvian fissure and more anterior to the central sulcus than was the tongue motor area of patients without organic lesion. Both motor and sensory responses were found outside of the classic precentral or postcentral area on the lateral surface of the cortex. Motor responses ('parietal motor responses') could occur posterior to the central sulcus and, rarely, sensory responses ('frontal sensory responses') were identified anterior to the central sulcus. These paradoxical parietal motor and frontal sensory responses were seen in 17 out of 40 (42.5 %) patients. Nine of these 17 patients had no organic brain lesion on MRI. Clinical factors, such as patient's age, duration of seizures and cognitive functions (IQ, word fluency score), did not influence the frequency of the paradoxical responses. However, patients with brain lesions showed a tendency to have associated paradoxical responses (P < 0.05). In conclusion, paradoxical responses are not uncommon in epilepsy patients, particularly in those with organic lesions. The physiological and clinical implications of the paradoxical responses are discussed.

Prostaglandin D2 (PGD2) is the major prostanoid formed among other prostanoids in cultured microvascular endothelium derived from human brain (HBEC). Angiotensin II, arginine vasopressin and endothelium-1 stimulated the production of PGD2 and PGF2 alpha in a concentration-dependent manner, and this effect was inhibited by their specific receptor antagonists or dexamethasone (inhibitor of phospholipase A2/cyclooxygenase II). Both the peptidergic-induced PGD2 and the exogenously added PGD2 were converted in HBEC to 9 alpha, 11 beta-PGF2, a potent vasoconstrictor. Exogenous PGD2 also dose-dependently enhanced the production of vasoconstrictive PGF2 alpha, thromboxane B2, vasodilatory prostaglandin PGE2, and cAMP in these cells. The PGD2 stimulated formation of the prostanoids was inhibited by acetylsalicylic acid or indomethacin (inhibitors of cyclooxygenase I) but not dexamethasone, demonstrating for the first time that PGD2 may contribute to the production of prostanoids in HBEC. These findings strongly suggest that PGD2 may play a pivotal role in the regulation of cerebromicrovascular function.