Andreas Hufnagel

Surgical treatment of extratemporal epilepsy: clinical, radiologic, and histopathologic findings in 60 patients.

Summary: Purpose and Methods: The aim of this study was to analyze clinical, radiologic, and histopathologic findings in 60 consecutive patients with medically intractable extratemporal epilepsy who were operated on between November 1987 and May 1993.

Surgical Pathology of Temporal Lobe Epilepsy. Experience with 216 Cases

The surgical treatment of chronic epilepsies is increasing rapidly. Here we report the histopathologic findings in 216 consecutive surgical specimens of patients with chronic pharmacoresistant temporal lobe epilepsy. In 75 cases (34.7%) there were tumors, all but two of which were of low histopathological grade (WHO grade I or II). The most common tumors were gangliogliomas (34 cases), pilocytic astrocytomas (17 cases), oligodendrogliomas (9 cases), fibrillary astrocytomas (6 cases), and dysembryoplastic neuroepithelial tumors (6 cases). There were 51 cases with non-neoplastic focal lesions and an additional 13 cases with tumors and non-neoplastic focal lesions within the same specimen. The most frequent non-neoplastic focal lesions were microscopic glioncuronal hamartias (32 cases), glioneuronal hamartomas (7 cases), and vascular malformations (13 cases). The hippocampal formation was structurally well preserved in 71 specimens. In 51 of these (71.8%) there was Ammons horn sclerosis. Presurgical placement of depth electrodes was invariably associated with circumscribed defects of the brain parenchyma. The implantation of subdural electrodes was sometimes followed by chronic inflammatory changes of the leptomeninges. Our findings indicate that in the majority of patients with medically intractable temporal lobe epilepsy there are significant histopathologic findings, many of which are only rarely encountered otherwise.

Surgical treatment of neoplasms associated with medically intractable epilepsy.

OBJECTIVE Surgical treatment in patients with brain tumors and medically intractable epilepsy is aimed at the removal of the neoplasm and complete seizure control. However, an adequate surgical approach is still controversial. This study was designed to analyze the factors for the optimum surgical treatment of these patients. METHODS The clinical, electrophysiological, operative, and histopathological data of 146 consecutive patients who underwent surgery between November 1987 and May 1995 for intrinsic brain tumors and pharmacoresistant epilepsy were evaluated. RESULTS The majority of the tumors were located in the temporal lobe (n = 116) and involved the cortical gray matter. The most frequent tumors were gangliogliomas (n = 65), pilocytic astrocytomas (n = 21), and dysembryoplastic neuroepithelial tumors (n = 19). All but three tumors (98%) were of low histopathological grade (World Health Organization Grades I or II). The biological behavior of the tumors was strikingly indolent, as indicated by a long preoperative history of chronic seizures (mean, 14 yr). In all cases, complete resection of the tumor, including the epileptogenic area (as determined by noninvasive and/or invasive recordings of the zone of seizure onset and persistent interictal activity), was intended. Complications were encountered in 11 cases (8%). However, no patient died and there was no permanent morbidity. Of the 124 patients who had postoperative follow-up examinations more than 6 months after resection, 71% were seizure-free, 11% had no more than two seizures per year, 13% showed a reduction of seizure frequency of at least 75%, and 5% had no appreciable reduction in seizure frequency. CONCLUSION The data indicate that neoplasms associated with medically intractable epilepsy constitute a distinct clinicopathological group of tumors that arise in young hosts, involve the cortex, and exhibit indolent biological behavior for many years. Complete surgical removal of these tumors, including the epileptogenic area, can achieve excellent seizure control.

Subdural and depth electrodes in the presurgical evaluation of epilepsy

SummaryFrom 1987 to 1992, invasive EEG studies using subdural strips, grids or depth electrodes were performed in a total of 160 patients with medically intractable epilepsy, in whom scalp EEG was insufficient to localize the epileptogenic focus. Dependent on the individual requirements, these different electrode types were used alone or in combination. Multiple strip electrodes with 4 to 16 contacts were implanted in 157 cases through burrholes, grids with up to 64 contacts in 15 cases via boneflaps, and intrahippocampal depth electrodes in 36 cases using stereotactic procedures. In every case, localization of the electrodes with respect to brain structures was controlled by CT scan and MRI.Visual and computerized analysis of extra-operative recordings allowed the localization of a resectable epileptogenic focus in 143 patients (89%), who subsequently were referred for surgery, whereas surgery had to be denied to 17 patients (11%). We did not encounter any permanent morbidity or mortality in our series.In our experience, EEG-monitoring with chronically implanted electrodes is a feasible technique which contributes essentially to the exact localization of the epileptogenic focus, since it allows nearly artefact-free recording of the ictal and interictal activity. Moreover, grid electrodes can be used for extra-operative functional topographic mapping of eloquent brain areas.

Clinical Relevance of Quantified Intracranial Interictal Spike Activity in Presurgical Evaluation of Epilepsy

Summary: Purpose: Electrocorticograms of 32 patients with medically intractable seizures, recorded through intracranial electrodes, were retrospectively analyzed. The objective was to assess whether quantitative analysis of interictal spikes may be used for delineation of the epileptogenic zone.

Glioneuronal Malformative Lesions and Dysembryoplastic Neuroepithelial Tumors in Patients with Chronic Pharmacoresistant Epilepsies

Abstract. Malformative glioneuronal lesions were examined in surgical specimens from 43 patients with chronic focal epilepsies in order to determine the scope of histopathological changes and to better understand their pathogenesis. The most common lesions were hamartias composed of randomly orinted neurons and astrocytes (24 cases). Most of these lesions also contained clustered oligodendrocyte-like cells which were often strongly immunoreactive for the developmentally regulated embryonal form of the neural cell adhesion molecule (E-NCAM). These hamaritias were typically minute, multifocal, and arranged in a pattern suggestive of a migration disorder. There were eight cases with aggregates of large disfigured neurons, oversized atypical astrocytes and ballooned multinucleated giant cells reminiscent of tuberous sclerosis-associated changes. Finally, there were 11 dysembryoplastic neuroepithelial tumors (DNT), an entity which has been proposed to be malformative rather than neoplastic. The oligodendroglia-like cells in DNT were negative for E-NCAM. However, strong E-NCAM expression was present in many dysplastic neurons of tuberous sclerosis-like lesions, hamartias and DNT and in reactive astrocytes. Significant immunoreactivity for the proliferation associated Ki-67 antigen was not observed. No similar lesions were observed in 500 consecutive autopsies from patients without epilepsy. Malformative glioneuronal lesions appear to be highly epileptogenic and most likely result from a disordered cell migration and differentiation.

Brain diffusion after single seizures.

Summary:  Purpose: Diffusion‐weighted magnetic resonance imaging (DWI) after focal status epilepticus has demonstrated focal alterations of the apparent diffusion coefficient (ADC) in the epileptogenic zone. We hypothesized that localized dynamic alterations of brain diffusion during the immediate postictal state will be detectable by serial DWI and correlate with the epileptogenic zone.

Clinical outcome and neuropsychological deficits after right decompressive hemicraniectomy in MCA infarction

Abstract.Background and Purpose: The purpose of this study was to analyse in detail the functional outcome and the neuropsychological deficits in patients with space-occupying infarction of the non-dominant hemisphere one year after surgery. Methods: Postoperative complications and retrospective consent to surgery were assessed in a semi-structured interview in 26 patients. Functional outcome was measured with the Barthel-Index (BI) and Rankin-Scale. Neuropsychological tests in 14 patients focused on visuo-spatial and visuo-constructive abilities, attention, spatial span and self-rated mood. Results: The one-year survival rate was 69 % (18 of 26). The functional outcome was good (BI ≥ 90) in 3 patients, fairly good (BI 75–85) in 6, moderate (BI 30–70) in 6, and poor (BI 0–25) in 3 patients. Age was an independent predictor of outcome, patients above 52 years had a BI of 50 or below. Neuropsychological tests (14 of 18) showed profound attention deficits in all patients, and visuo-spatial and visuo-constructive deficits in patients with lower formal education. Retrospectively, 4 of 18 patients would not give consent to surgery again, mostly because of the bad quality of life postoperatively. Conclusion: Older patients do not seem to benefit from decompressive hemicraniectomy; more than half of the surviving younger patients have a good outcome and live independently. Attention deficits are prominent in all patients; visuo-spatial and constructive deficits are less pronounced in patients with higher formal education. Retrospective agreement to decompressive hemicraniectomy is high in patients with good functional outcome.

Multiple Subpial Transection for Control of Epileptic Seizures: Effectiveness and Safety

Summary: Purpose: To assess the efficacy and safety of multiple subpial transection (MST), a new technique in epilepsy surgery, alone and in combination with resection.

Quantitative and qualitative evaluation of patterns of cerebral language dominance: An amobarbital study.

As a part of presurgical evaluation, 173 patients received bilateral intracarotid amobarbital tests for determination of cerebral language dominance. Language testing during intracarotid amobarbital procedures (IAP) consisted of the following tasks: automatic speech, sentence comprehension, body commands, naming, repetition, reading, and spontaneous speech. Patterns of cerebral language dominance were evaluated and discussed on five levels of analysis: (1) quantification of language dominance on the basis of a lateralization index derived from the total language scores in each IAP; (2) determination of five dominance subpatterns (left or right dominant, strongly bilateral, and incomplete left or right dominant) according to quantification performed on level (1) and clinical judgement; (3) qualitative differentiation of three kinds of bilaterality (positive, negative, and general) according to total language performance in left and right IAP; (4) analysis of grouped linguistic subfunctions extracted from performance in specific IAP subtests; (5) extraordinary individual case histories. The distribution of lateralization indices revealed only partially continuous degrees of lateralization, especially between the left-dominant and bilateral subgroups. As for the clinically oriented classification, incomplete left dominance is frequent (16.2%), while incomplete right dominance does not occur at all. Atypical dominance patterns are mostly correlated to bilateral and/or extratemporal foci. Concerning grouped subfunctions, a rotated factor matrix statistic yields an analysis of clusters of IAP subtests, where functions involving expressive language capacities are separated from those that are purely receptive. Further analyses of bilaterality subpatterns suggest that there are mainly four bilaterality phenomena, namely interhemispheric dissociation, double representation, unilateral representation of subfunctions, and partial representation of subfunctions in either hemisphere. Application of these differentiations to individual cases yields additional evidence that can be used in patient selection for operation in order to avoid postoperative neuropsychological deficits, especially in candidates for extratemporal surgery. In conclusion, a multilevel analysis of IAP language data is recommended since it permits a detailed account of varieties of language dominance patterns and contributes to more adequate presurgical decision-making in planned operations in cognitively relevant brain areas.