MRI of cortical dysplasia--correlation with pathological findings.

Cortical dysplasia (CD) is the most epileptogenic structural lesion associated with epilepsy and patients with intractable seizures caused by this condition are good surgical candidates. MRI plays an important role in detecting the abnormalities of CD. We clarified the MRI characteristics of CD by comparing imaging and histological findings in 20 patients with intractable seizures who underwent surgical resection. There were 12 males and eight females, mean age at operation was 15 years. MRI was performed at 1.5 tesla; T1-weighted, T2- and proton density-weighted spin-echo and fluid-attenuated inversion-recovery (FLAIR) images were obtained. The lesions were in the frontal lobe in nine cases, temporal in two, occipital in another two, insular in one and multilobar in six. Blurring of the grey/white matter junction was seen in all patients, and T2 prolongation in white matter and/or at the grey/white matter junction in 19. Abnormal signal intensity was more frequent in the white matter or at the grey/white matter junction than in the grey matter. FLAIR images made this abnormal high signal easier to appreciate, and we thought them very useful in this context. In areas of T2 prolongation, we saw dysplastic neurones and/or balloon cells, dysmyelination, and ectopic neuronal clustering histologically; glial proliferation played an important role in prolonging T2.

Acute psychosis during intracranial EEG monitoring: close relationship between psychotic symptoms and discharges in amygdala.

PURPOSE: This report examined the underlying mechanism of psychosis associated with epilepsy. METHODS: An adult patient with epilepsy manifesting acute psychosis during long-term EEG monitoring is presented, together with a literature review on this subject. RESULTS: A 25-year-old woman with intractable temporal lobe epilepsy developed acute psychosis while she underwent long-term intracranial EEG monitoring. After a clustering of seizures, she manifested psychotic symptoms including hallucination, stupor, and repeated fear. The transition of psychotic symptoms corresponded to the changes in frequency and morphology of seizure discharges restricted to the left amygdala. Improvement of psychosis coincided with disappearance of seizure discharges. CONCLUSIONS: This case confirmed a close relationship between psychotic symptoms and seizure discharges in the left amygdala. It is suggested that paroxysmal bombardment of the medial temporal lobe structure may be a pathogenetic factor of acute psychosis associated with epilepsy.

[Neuroimaging and electrophysiological study in epilepsy].

There exist various morphological and biochemical changes closely associated with electrophysiological phenomena which cause epileptic seizures in the brains of epilepsy patients. Recent developments in investigation methods, not only electrophysiological(EEG and MEG), but also neuroimaging involving morphological imaging(CT and conventional MRI) and functional imaging(SPECT, PET, functional MRI and MRS) is able to demonstrate these changes. SPECT and PET can particularly clarify the changes of cerebral blood flow and glucose metabolism between interictal and ictal periods. In our experience of 423 patients who underwent epilepsy surgery for intractable seizures, these interventions provide important information to identify the epileptogenic foci. However, in practice, discordance in the results of these presurgical evaluations is recognized, and invasive intracranial recordings are needed in such cases. These problems in diagnosis were shown especially in patients with mesial temporal sclerosis and focal cortical dysplasia. To detect an epileptogenic focus more clearly, a combination of morphological and functional findings, new functional imaging such as neurotransmitter receptor imaging, EEG-triggered or neuropharmacological functional MRI, as well as, statistical parametric analysis may be needed.

Neuroradiologic findings in focal cortical dysplasia: histologic correlation with surgically resected specimens.

PURPOSE: We investigated the neuroradiologic characteristics of focal findings of surgically resected specimens obtained from 47 patients with focal cortical dysplasia (FCD). METHODS: Forty cases were detected by magnetic resonance imaging (MRI), and two cases were detected only by single-photon emission computed tomography (SPECT), but five cases could not be detected before operation. RESULTS: MRI revealed abnormal gyri and sulci in 34 patients (pachygyric in 18, polymicrogyric in 10, both in six), and blurring of the gray matter-white matter junction in 29 (72%) patients. Signal abnormalities were found in 36 (90%) patients, in the gray matter in 32, with white matter in 30, and at the gray matter-white matter junction in 13. Moreover, peculiar patterns of abnormal signals in the white matter were recognized, including remarkably abnormal subcortical signals of T2 hyperintensity and T1 hypointensity adjacent to the dysplastic cortex in 15 cases, high radiated T2 signals extending from the ependymal surface of the lateral ventricle to the overlying cortex in 11 cases, and widespread abnormal signals in the white matter with gray matter involvement in four cases. Histologically, these abnormal signals corresponded to various degrees of dyslamination and morphologic abnormalities of neurons and glial cells in the gray matter, and to dysmyelination, ectopic clustering of dysplastic neurons, glial proliferation, and necrotic change in the white matter. Regional cerebral blood flow SPECT showed interictal hypoperfusion in 29 (62%) of the 47 patients, interictal hyperperfusion in two, and ictal hyperperfusion in 28 of the 34 patients associated with FCD. [123I]iomazenil SPECT demonstrating the distribution of central benzodiazepine receptors showed low accumulations localized spatially corresponding to the epileptogenic foci associated with FCD in seven of eight patients. CONCLUSIONS: These results demonstrate that neuroimaging reflects various structural and functional changes closely related to epileptogenesis in FCD.

New type of starch-binding domain: the direct repeat motif in the C-terminal region of Bacillus sp. no. 195 alpha-amylase contributes to starch binding and raw starch degrading.

The alpha-amylase from Bacillus sp. no. 195 (BAA) consists of two domains: one is the catalytic domain similar to alpha-amylases from animals and Streptomyces in the N-terminal region; the other is the functionally unknown domain composed of an approx. 90-residue direct repeat in the C-terminal region. The gene coding for BAA was expressed in Streptomyces lividans TK24. Three active forms of the gene products were found. The pH and thermal profiles of BAAs, and their catalytic activities for p-nitrophenyl maltopentaoside and soluble starch, showed almost the same behaviours. The largest, 69 kDa, form (BAA-alpha) was of the same molecular mass as that of the mature protein estimated from the nucleotide sequence, and had raw-starch-binding and -degrading abilities. The second largest, 60 kDa, form (BAA-beta), whose molecular mass was the same as that of the natural enzyme from Bacillus sp. no. 195, was generated by proteolytic processing between the two repeat sequences in the C-terminal region, and had lower activities for raw starch binding and degrading than those of BAA-alpha. The smallest, 50 kDa, form (BAA-gamma) contained only the N-terminal catalytic domain as a result of removal of the C-terminal repeat sequence, which led to loss of binding and degradation of insoluble starches. Thus the starch adsorption capacity and raw-starch-degrading activity of BAAs depends on the existence of the repeat sequence in the C-terminal region. BAA-alpha was specifically adsorbed on starch or dextran (alpha-1,4 or alpha-1,6 glucan), and specifically desorbed with maltose or beta-cyclodextrin. These observations indicated that the repeat sequence of the enzyme was functional in the starch-binding domain (SBD). We propose the designation of the homologues to the SBD of glucoamylase from Aspergillus niger as family I SBDs, the homologues to that of glucoamylase from Rhizopus oryzae as family II, and the homologues of this repeat sequence of BAA as family III.

Absence of simple partial seizure in temporal lobe epilepsy: its diagnostic and prognostic significance.

The diagnostic and prognostic significance of the absence of simple partial seizures (SPS) immediately preceding complex partial seizures (CPS) was examined in patients with temporal lobe epilepsy. The status of self-reported SPS in 193 patients with temporal lobe epilepsy who had surgical therapy more than 2 years ago was reviewed. Before surgery, 37 patients never experienced SPS before CPS (Group A), 156 patients either always or occasionally had SPS before CPS (Group B). The frequency of mesial temporal sclerosis (MTS) was lower and the age at onset of epilepsy was higher in Group A. The seizure focus was in the language-dominant temporal lobe in 73% of the cases in Group A, compared with 40% in Group B. The surgical outcome did not differ between the two groups. The findings suggest that temporal lobe seizures without preceding SPS tend to originate in the language-dominant temporal lobe that contains a pathologic etiology other than MTS, especially in the lateral temporal lobe. The surgical outcome in patients without SPS is similar to that in patients with SPS.

Electrical stimulation of the contralateral mesial temporal structure induces an ipsilateral seizure pattern in mesial temporal lobe epilepsy.

PURPOSE: We examined the mode of seizure development induced by electrical stimulation in patients with mesial temporal lobe epilepsy. METHODS: Of 25 patients undergoing intracranial EEG evaluation and electrical stimulation ipsilateral to the presumed site of habitual seizure origin, 17 patients had additional stimulation studies on the contralateral temporal lobe. RESULTS: Fourteen of the 25 patients had seizures induced with ipsilateral stimulation, and two of the 17 patients had seizures with contralateral stimulation. Seizures induced by ipsilateral stimulation started in the ipsilateral temporal lobe, whereas those induced by contralateral stimulation originated from the ipsilateral temporal lobe structure and were identical to the habitual seizures. CONCLUSIONS: Electrical stimulation of the temporal lobe structure opposite the site of habitual seizure origin is said to induce a seizure rarely. However, according to our preliminary results, if contralateral stimulation elicits a habitual seizure in the ipsilateral temporal lobe, it might be considered additional confirmatory evidence of seizure lateralization.

[Focal cortical dysplasia and epilepsy surgery].

We conducted corticectomy in twenty-five patients with intractable partial epilepsy due to focal cortical dysplasia (FCD). MRI could not detect FCDs in three patients, interictal SPECT, however, revealed hypoperfusion corresponding to FCDs in two of these patients, while the FCD in one remaining patient was histologically identified in a resected specimen. The location of FCDs was as follows: the frontal lobe in sixteen patients, the temporal in five, the occipital in two, fronto-parietal in one, and the temporo-parietal in one. Prior to the surgery, twenty-one patients underwent invasive long-term intracranial EEG/CCTV monitoring. Of the fourteen patients who were tracked for longer than 2 years following surgery, eleven belonged to Class I according to Engel's criteria, two to Class II, and one to Class III. We compared intracranial EEG findings (interictal and ictal) between these twenty-one patients and eight patients with frontal lobe epilepsy resulting from different lesions. The results of this comparison, together with the seizure outcome following surgery, indicated that FCD is intrinsically epileptogenic. The invasive long-term monitoring should, as a rule, be performed in all patients with FCDs prior to the corticectomy.

Recommendation of early surgery from the viewpoint of daily quality of life.

We surveyed pre- and postoperative levels of satisfaction with a range of the daily quality-of-life (QOL) domains in 132 sets of epilepsy surgery patients and their families. All patients underwent resective surgery for temporal lobe epilepsy and were monitored for > 2 years. Patient and family assessments showed patients' overall QOL markedly improves after surgery, depending on freedom from seizures. However, factors such as social contacts, family relations, or financial status improved little. Some families and patients were not satisfied with the postsurgical status, despite freedom from seizures. Patients who had surgery at a later age were not so satisfied with their postsurgical status as were patients who had surgery at a younger age, particularly on the QOL domains of role activities, memory function, leisure activities, or emotional well-being. This lower satisfaction level in older patients likely results from a variety of problems affecting patients during the long-lasting epileptic process; social handicaps, psychologic conflicts, and deterioration of cognitive/behavioral functions. Based on each case, we recommend that investigations start at an early stage of the illness, so that surgical intervention may be considered as early as possible.

Improvement of quality-of-life following resective surgery for temporal lobe epilepsy: results of patient and family assessments.

In order to evaluate the quality-of-life (QOL) of epilepsy surgery patients, we surveyed patients' degree of life satisfaction and their families' degree of satisfaction with patient's status in a range of domains both pre- and post-operatively. Of 100 patient-family sets of surveys that were mailed out, 93 were completed and returned from patients and 91 from their families. All patients surveyed had temporal lobe epilepsy and had been followed for longer than 2 years after resective surgery. Patients and their families rated overall QOL as having markedly improved following surgery. However, they rated social domains of QOL, including role activities, financial status, and social and family relationships as having improved relatively little. Despite freedom from seizures, a few patients' families were dissatisfied with the patients' post-operative status, primarily for psychosocial reasons. Patients operated on at a later age reported little gains in life satisfaction following surgery. This study supports the conclusion that surgical intervention should occur before patients are subjected to the psychological conflicts and social handicaps associated with chronic intractable epilepsy.

Localizing value of seizure manifestations of temporal lobe epilepsies and the consequence of analyzing their sequential appearance.

We investigated the localizing and lateralizing value of principal seizure manifestations in temporal lobe epilepsies (signal symptoms, oroalimentary automatisms, somatomotor manifestations, unilateral dystonic posturing, ictal speech, motionless stare) of 223 complex partial seizures in 50 patients. All the patients had invasive long-term monitoring with the combined implantation of intracerebral electrodes in and subdural electrodes on the bilateral temporal lobes. Postoperative freedom from seizures was ascertained for longer than one year. We found that 35 patients had amygdalohippocampal seizures and 15 had lateral temporal seizures. The value of the manifestations was established in relation to the site and side of seizure origin and to the progression of seizure discharges within the unilateral temporal lobe or to the contralateral cerebral hemisphere. Several signs among the manifestations were found to be reliable in predicting the site or side of the temporal lobe seizure focus. We emphasized the importance of investigating sequential changes of seizure manifestations in relation to ictal EEG findings by means of simultaneous recording.

Conditions for omitting invasive long-term monitoring before surgical resection in patients with temporal lobe epilepsy.

The omission of invasive long-term monitoring before surgical resection in patients with epilepsy should be permitted only for those in whom the epileptogenic focus is presumed to localize unilaterally in the mesial aspect of the temporal lobe. The localization may well be confirmed through noninvasive measures. Retrospective analyses of data obtained from noninvasive investigations (scalp-recorded and sphenoidal EEGs, neuroimages, and electroclinical seizure manifestations) were carried out in 58 patients. The localization of their epileptogenic focus was subsequently confirmed by the implantation of both intracerebral and subdural electrodes; the focus had an amygdalohippocampal origin in 41 patients and a lateral temporal origin in 17 patients. From the comparison of noninvasive findings between these two groups, we propose the following indispensable conditions for omitting an invasive evaluation: 1. Appearance of focal epileptic discharges unilaterally in the sphenoidal lead observed during the simple phase of partial seizures, or unilateral discharges with predominancy in the sphenoidal lead during the early phase of complex partial seizures. 2. Interictal spikes on scalp-recorded EEGs localizing unilaterally in the anterior region of the temporal lobe, and if bilaterally independent, presenting with unilateral predominancy in a ratio of greater than 4:1. 3. Presence of autonomic signs in the initial phase of signal symptoms. 4. Neuroimaging findings in the mesial temporal region: elongated T2 on MRI and hippocampal atrophy, or a tumorous lesion. The lateralization conforms to interictal and ictal paroxysmal EEG findings. There were 8 patients with seizure of amygdalohippocampal origin who satisfied all the indispensable condition, but not a single patient with seizures of lateral temporal origin.(ABSTRACT TRUNCATED AT 250 WORDS)

Neuropsychological evaluation before and after surgical treatment of temporal lobe epilepsy.

We present the results of pre- and postoperative neuropsychological evaluations of 58 patients with temporal lobe epilepsy who underwent a chronic intracranial EEG monitoring and a subsequent standard anterior temporal lobectomy. Wada's test provided valuable information on the speech dominant side and on the focus localization. Some warning signs as well as verbal automatisms indicated the effect for focus localization and lateralization. The results of interictal neuropsychological tests suggested that each subgroup of TLE performed differently. A postoperative neuropsychological performance has improved in many tests that may be explained by the diminished epileptic bombardment resulting from the resection.