Biomedical subjects

C W van Veelen

Publications and source records attributed to C W van Veelen.

At least 19 recordsLinked to original sources

PubMed2001

[Dutch Collaborative Epilepsy Surgery Program: reduction of seizures, operative complications and tapering of medication in 338 patients, 1973-1998].

OBJECTIVE: To determine the results of surgical treatment in patients with drug-resistant epilepsy, referred to the Dutch Epilepsy Surgery Program, who were treated in the University Medical Centre Utrecht, the Netherlands, in the period January 1973-December 1998. DESIGN: Retrospective descriptive. METHOD: A total of 338 patients were operated on; 269 underwent temporal lobe resection, 41 extratemporal resection, 12 a functional hemispherectomy and 10 callosotomy. Six patients were treated with vagus nerve stimulation. For seven of the patients no follow-up data was available. RESULTS: After a minimum follow-up of 1 year class I or class II results (in accordance with the University of California in Los Angeles classification (UCLA) where class I = seizure-free and class II < or = 3 seizures per year) were obtained in 91% of patients who underwent temporal lobe resections, 67% of patients who underwent extratemporal resections, 81% of patients who underwent functional hemispherectomy and 10% of patients who underwent anterior callosotomy. In five of these patients an improvement in their behaviour occurred. Of the 6 patients who underwent vagus nerve stimulation only I experienced a beneficial seizure reduction (UCLA class III). Transient physical complications occurred in 4% of the patients treated and permanent damage in 1%. Postoperative psychiatric complications occurred almost exclusively following temporal resections; in 11% of which 7% de novo. After 4 postoperative years this had decreased to 5%. In a group of 143 patients who were seizure-free for 2 or more years, post-surgery medication was tapered in 75 cases, stopped in 33 cases and remained unchanged in 35 cases. The relapse rate following a tapering or stopping of the medication was 30% and with unchanged medication 17%. Although the majority of patients were once again seizure-free upon restarting the medication, a significant number continued to experience seizures. CONCLUSION: For a number of carefully selected epilepsy patients with intractable seizures, surgery is a successful treatment with few serious complications.

Anticonvulsants↗

PubMed2001

Localization of implanted EEG electrodes in a virtual-reality environment.

In the planning of epilepsy surgery procedures, intracranial electrodes are implanted in a significant fraction of the patients. Accurate localization of the individual electrode contacts with respect to the brain cortex is imperative. Because the manual tracking of an EEG electrode in a CT scan in a slice-by-slice fashion is cumbersome and subjective, the goal of this study was to develop an easier and more accurate way to localize implanted EEG electrodes. In this paper, we present our solution in the form of a virtual-reality environment with interactive tools to assist the clinician with EEG localization. With the help of a high-quality and fast volume renderer, a view is created of the inside of the patient's skull to obtain an overview of the electrodes in relation to the cortical structures. Depth, grid, and reed electrodes are characterized semi-interactively using different methods. For depth electrodes, the contacts (which are not visible in the CT scan) are derived by measuring off the theoretical distance between the contact and the end of the electrode from the central axis produced by a three-dimensional (3D) line tracker. For grid electrodes, the contacts are visible in a CT, so the 3D view is merely used to find the contacts and to resolve the overlap of grids with other grids, tail wires, or bone ridges. For reed electrodes, the contacts, which are again not visible in this case, are calculated from a line model fitted to the positions of lead markers. After letting the user place artificial spheres on the lead markers and wire, a B-spline is fitted to the spheres' centers to estimate the positions of the contacts. The approach was evaluated by applying it to CT scans of seven patients. It appeared that the method is generally applicable (even crossing electrodes or electrodes with gaps were correctly characterized), and that the display via 3D views and slices is so good that manual placement of spheres performed as well as semi-automatic placement. From computer experiments, it appeared that the final localization error in the position of EEG contacts could be estimated to lie in the order of the dimensions of one voxel.

Brain Mapping↗

PubMed2001

Expression of brain-derived neurotrophic factor and tyrosine kinase B receptor proteins in glioneuronal tumors from patients with intractable epilepsy: colocalization with N-methyl-D-aspartic acid receptor.

Recent evidence suggests that brain-derived neurotrophic factor (BDNF) and its tyrosine kinase B (TrkB) receptor, in addition to promoting neuronal survival and differentiation, modulates synaptic transmission by increasing N-methyl-D-aspartic acid receptor (NMDAR) activity. Overexpression of BDNF may, then, interfere with normal brain function, causing increased excitability. We have examined the immunohistochemical expression of BDNF, full-length TrkB receptor and the NMDAR subunit 1 and subunit 2A/B proteins (NMDAR1 and NMDAR2A/B) in glioneuronal tumors (gangliogliomas, GG, n = 40; dysembryoplastic neuroepithelial tumors, DNT, n = 15), from patients with chronic intractable epilepsy. The great majority of tumors studied were positive for all markers examined, supporting the high level of neurochemical differentiation of these lesions. BDNF and TrkB immunoreactivity (ir) was mainly observed in the neuronal component of the tumors. In GG, more than 90% of tumors contained very intense BDNF-ir ganglion cells. Double labeling confirmed the presence of BDNF-ir and TrkB-ir in neurons which contained NMDAR1. NMDAR2A/B intensely labeled abnormal neurons in both GG and DNT and co-localized with NMDAR1. The presence of BDNF and its receptor in the neuronal component of GG and DNT may suggest a role for this neurotrophin in the development of these lesions, preventing the death of abnormal neuronal cells. In addition, since these neurons contain both NMDAR1 and NMDAR2A/B subunits, the BDNF-TrkB pathway may also contribute through a modulation of glutamatergic transmission to the intrinsic epileptogenicity of glioneuronal tumors.

Adolescent↗

PubMed2001

A grading system for hippocampal sclerosis based on the degree of hippocampal mossy fiber sprouting.

In patients suffering from temporal lobe epilepsy (TLE) a highly variable degree of hippocampal sclerosis (HS) can be observed. For standard neuropathological evaluation after hippocampal resection, neuronal cell loss in the hippocampal subareas is assessed (Wyler score 0-4) [Wyler et al. (1992) J Epilepsy 5: 220-225]. Other marked morphological changes in the sclerotic hippocampus are gliosis and loss of mossy fibers in the hilus and mossy fiber sprouting in the supragranular layer. In this study we quantified changes in mossy fiber density using Timm's stain in resected hippocampal tissue from patients with various degrees of sclerosis. We found that tissue specimens from patients without sclerosis (W0) show almost no mossy fiber sprouting. Patients with moderate sclerosis show sprouting without fiber loss in the hilus, whereas specimens from patients with severe sclerosis show sprouting as well as fiber loss in the hilus. Thus, analysis of mossy fiber abundance in hilus and supragranular layer by the rapid and simple Timm's stain is a sensitive measure for hippocampal sclerosis. It provides a reliable rapid tool for neuropathological evaluation, even if the tissue only contains dentate gyrus due to the sectioning procedure.

Axons↗

PubMed2001

Glioneuronal tumors and medically intractable epilepsy: a clinical study with long-term follow-up of seizure outcome after surgery.

The present study intends to identify factors that predict postoperative clinical outcome in patients with gangliogliomas (GG) and dysembryoplastic neuroepithelial tumors (DNT). We evaluated the medical records of 45 patients with GG and 13 patients with DNT, treated surgically between 1985 and 1995. We assessed several clinical and histopathological features and analyzed the data statistically. At 5 years postoperatively, 63% of patients with GG and 58% of patients with DNT were seizure-free (Engel's class I). Younger age at surgery (P<0.01 for GG and P<0.05 for DNT), total resection (P<0.01 for GG), shorter duration of epilepsy (P<0.01), absence of generalized seizures (P<0.01 for GG; P<0.05 for DNT) and absence of epileptiform discharge in the post-operative EEG (P<0.01 for GG; P=0.01 for DNT) predicted a better postoperative seizure outcome. Tumor recurrence with malignant progression occurred in eight histologically benign GG and two anaplastic GG and was associated which older age at surgery (P=0.01) and subtotal resection of the tumor (P<0.01). Our results indicate that a prompt diagnosis, relatively soon after seizure onset, followed by complete resection of glioneuronal tumors provides the best chance for curing epilepsy and preventing their malignant transformation.

Adolescent↗

PubMed2001

The value of electrophysiology results in patients with epilepsy and vigabatrin associated visual field loss.

PURPOSE: To determine the value of electrophysiological findings in patients with temporal lobe epilepsy and to relate these findings to the amount of concentric contraction of the visual field and the use of vigabatrin. METHODS: Electro-retinograms and electro-oculograms were done on 30 patients, operated for temporal lobe epilepsy. The patients were divided into three groups: (A) concentric contraction of the visual field associated with a history of vigabatrin medication (15 patients), (B) normal visual field with vigabatrin use (11 patients) and (C) normal visual field without vigabatrin medication (4 patients). RESULTS: Electrophysiological abnormalities were found in 50% of the patients in group A. The Arden ratio of the EOG was lowered in 57%. Abnormalities in the ERG were found: b-wave implicit time photopic F was prolonged (50%), b-wave amplitudes scotopic B (53%), C (73%) and G (50%) and photopic H (50%) were diminished. The amount of visual field loss and the total dose of vigabatrin used, showed only slight correlation with the ERG and EOG. The use of vigabatrin during the ERG and EOG recording in group A, gave a higher b-wave amplitude scotopic G in 64% of cases. The a-wave implicit times scotopic G (73%) and photopic G (59%) and H (73%) were shortened in group B. CONCLUSION: EOG was abnormal in 57% in group A. ERG abnormalities could only be found in 50% of group A, mainly in the inner retina. Since also the total dose of vigabatrin and the amount of visual field loss did not really show a correlation with the electrophysiological findings and results of literature are not unanimous, electrophysiology does not appear at present to be a good method to detect patients with, or at risk of, vigabatrin associated visual field loss. Regularly performed visual field examination remains the cornerstone in screening.

Adult↗

PubMed2001

Ionotropic and metabotropic glutamate receptor protein expression in glioneuronal tumours from patients with intractable epilepsy.

Glioneuronal tumours are an increasingly recognized cause of chronic pharmaco-resistant epilepsy. In the present study the immunocytochemical expression of various glutamate receptor (GluR) subtypes was investigated in 41 gangliogliomas (GG) and 16 dysembryoplastic neuroepithelial tumours (DNT) from patients with intractable epilepsy. Immunocytochemistry with antibodies specific for ionotropic NR1, NR2A/B (NMDA) GluR1, GluR2 (AMPA), GluR5-7 (kainate), and metabotropic mGluR1, mGluR2-3, mGluR5, mGluR7a subtypes demonstrated in both GG and DNT the presence of an highly differentiated neuronal population, containing subunits from each receptor class. More than 50% of tumours contained a high percentage of neuronal cells immunolabelled for NMDA, AMPA and kainate receptor subunits. A high percentage of neurones showed strong expression of NR2A-B, which co-localized with NR1. Group I mGluRs (mGluR1 and mGluR5) were highly represented in the neuronal component of the tumours. Immunolabelling for several GluRs was also present in the glial component. Increased expression of mGluR2-3, mGluR5 and GluR5-7 was observed in reactive astrocytes in the perilesional zone compared to normal cortex. The neurochemical profile of glioneuronal tumours, with high expression of specific GluR subtypes, supports the central role of glutamatergic transmission in the mechanisms underlying the intrinsic and high epileptogenicity of these lesions.

Adolescent↗

PubMed2000

[Vigabatrin: balancing effectiveness vs. (irreversible) visual field loss as a side effect].

The antiepileptic drug vigabatrin causes an asymptotic concentric contraction of the visual field in 30 to 40% of the patients. The visual field loss seems to be correlated with the cumulative dose of vigabatrin and is not reversible. The cause of this field loss is located in the inner retina (the horizontal and/or amacrine cells). The exact mechanism is still unknown. Though abnormalities in electrophysiology and colour vision are found, regular visual field examination remains the cornerstone in detecting visual field loss. A careful judgement has to be made in individual patients before starting or continuing vigabatrin medication.

4-Aminobutyrate Transaminase↗

PubMed2000

Glutamate and gamma-aminobutyric acid content and release of synaptosomes from temporal lobe epilepsy patients.

During surgical intervention in medically refractory temporal lobe epilepsy (TLE) patients, diagnosed with either mesial temporal lobe sclerosis (MTS)- or tumor (T)-associated TLE, biopsies were taken from the anterior temporal neocortex and the hippocampal region. Synaptosomes, isolated from these biopsies were used to study intrasynaptosomal Ca(2+) levels ([Ca(2+)](i)), and glutamate and gamma-aminobutyric acid (GABA) contents and release. All synaptosomal preparations demonstrated a basal [Ca(2+)](i) of about 200 nM, except neocortical synaptosomes from MTS-associated TLE patients (420 nM). K(+)-induced depolarization resulted in a robust increase of the basal [Ca(2+)](i) in all preparations. Neocortical synaptosomes from TLE patients contained 22.9 +/- 3.0 nmol glutamate and 4.6 +/- 0.5 nmol GABA per milligram synaptosomal protein, whereas rat cortical synaptosomes contained twice as much glutamate and four times as much GABA. Hippocampal synaptosomes from MTS-associated TLE patients, unlike those from T-associated TLE patients, contained about 70% less glutamate and 55% less GABA than neocortical synaptosomes. Expressed as percentage of total synaptosomal content, synaptosomes from MTS-associated TLE patients exhibited an increased basal and a reduced K(+)-induced glutamate and GABA release compared to rat cortical synaptosomes. In MTS-associated TLE patients, only GABA release from neocortical synaptosomes was partially Ca(2+)-dependent. Control experiments in rat synaptosomes demonstrated that at least part of the reduction in K(+)-induced release can be ascribed to resection-induced hypoxia in biopsies. Thus, synaptosomes from MTS-associated TLE patients exhibit a significant K(+)-induced increase in [Ca(2+)](i), but the consequent release of glutamate and GABA is severely impaired. Our data show that at least part of the differences in glutamate and GABA content and release between human biopsy material and fresh rat tissue is due to the resection time.

Animals↗

PubMed2000

The wada test: prediction of focus lateralization by asymmetric and symmetric recall.

PURPOSE: The intracarotid amytal test is commonly used as a predictor of memory dysfunctioning after anterior temporal lobe resection (ATL) for intractable epilepsy. Asymmetry in memory scores can provide focus lateralizing information. In this study the predictive value of a set of Wada test parameters was analyzed, including patients with symmetrical memory scores. METHODS: The Wada test was carried out in 226 patients undergoing ATL (94 L, 132 R). Data were collected on item recognition (five items), story recall, amytal dose, presentation time, EEG and arterial filling of amytal. A logistic regression analysis was performed on these data in order to find a set of variables which could best predict the side of seizure onset. RESULTS: The analysis yielded four variables, i.e. both memory scores, story recall after right-sided injection and presentation time of stimuli after left-sided injection which could correctly predict seizure lateralization in 85% of the cases. Misclassification was lower for right foci than for left foci. Seizure outcome was four times more favourable in the correctly classified patients. CONCLUSIONS: The results suggest that prediction of focus lateralization in temporal lobe epilepsy can be fairly exact even if left/right memory scores are equal. Performing the Wada test with only five memory items keeps the time window of active amytal short enough and gives accurate information about contralateral temporal lobe functioning. The classification scheme can be useful in predicting a lowered chance of seizure freedom.

Adult↗

PubMed2000

Concentric contraction of the visual field in patients with temporal lobe epilepsy and its association with the use of vigabatrin medication.

PURPOSE: To describe concentric visual field loss found in the presurgical evaluation of patients with drug-resistant temporal lobe epilepsy and relate the findings to potential causative factors. METHODS: A series of 157 consecutive patients with drug-resistant temporal lobe epilepsy, who had been selected for neurosurgical treatment, was examined in a study set up as a prospective investigation of their visual fields, to document the loss of visual field resulting from surgery. Pre-as well as postoperative visual field examinations were performed following a standard protocol using static and kinetic perimetry. As a number of patients appeared to have an unexplained concentric visual field contraction in the presurgical examination, a relation with potentially causative factors was analyzed in a cross-sectional study of all these patients. Correlations were sought with duration and severity of the seizure disorder, underlying pathology as indicated by magnetic resonance imaging (MRI) and demonstrated by pathology, any type of antiepileptic drug (AED) ever prescribed, and gender. RESULTS: In this cross-sectional analysis of 157 consecutive patients who were candidates for surgery for temporal lobe epilepsy, absolute concentric contraction of the visual field of 10 to 30 degrees was found in the presurgical examination in 20 (17%) of 118 patients who had ever used vigabatrin (VGB) and in none of 39 who had not had this medication. This difference was significant (p = 0.004). In addition, men [15 (21%) of 72] were significantly more often affected (p = 0.007) than women [five (6%) of 85]. The degree of visual field loss, as indicated by the Esterman grid, showed a positive correlation with the duration of VGB medication. There was no correlation of visual field contraction with a history of meningitis as potential cause of the epilepsy, duration of the epilepsy, status epilepticus in the medical history, or histologic abnormality of the brain tissue removed. Ophthalmologic examination of the patients with concentric contraction revealed no abnormalities. None of the patients with concentric contraction complained spontaneously of their visual field loss. CONCLUSIONS: VGB medication is a causative factor in concentric visual field loss. Visual field examination of patients using VGB should be seriously considered.

Anticonvulsants↗

PubMed2000

Long term changes in the visual fields of patients with temporal lobe epilepsy using vigabatrin.

AIM: To study the long term changes in the concentric contraction of the visual field in patients with temporal lobe epilepsy on vigabatrin medication. METHODS: Repeated Goldmann visual field examinations were compared in 27 patients with drug resistant temporal lobe epilepsy and concentric contraction of the visual field. Two groups were studied: 16 patients who had already stopped vigabatrin medication before surgery and 11 patients who continued vigabatrin medication. RESULTS: Concentric contraction of the visual field did not change in 16 patients who stopped vigabatrin before the first examination; there was slight but significant progress in visual field loss in 11 patients who continued the use of vigabatrin. CONCLUSION: Long term follow up of concentric contraction in this selected group of patients indicates that vigabatrin associated visual field loss is not reversible and that progression is possible when vigabatrin is continued.

Anticonvulsants↗

PubMed1999

Characterization of neocortical and hippocampal synaptosomes from temporal lobe epilepsy patients.

To investigate epilepsy-associated changes in the presynaptic terminal, we isolated and characterized synaptosomes from biopsies resected during surgical treatment of drug-resistant temporal lobe epilepsy (TLE) patients. Our main findings are: (1) The yield of synaptosomal protein from biopsies of epilepsy patients was about 25% of that from rat brain. Synaptosomal preparations were essentially free of glial contaminations. (2) Synaptosomes from TLE patients and naive rat brain, quickly responded to K(+)-depolarization with a 70% increase in intrasynaptosomal Ca(2+) ([Ca(2+)](i)), and a 40% increase in B-50/GAP-43 phosphorylation. (3) Neocortical and hippocampal synaptosomes from TLE patients contained 20-50% of the glutamate and gamma-aminobutyric acid (GABA) contents of rat cortical synaptosomes. (4) Although the absolute amount of glutamate and GABA released under basal conditions from neocortical synaptosomes of TLE patients was lower than from rat synaptosomes, basal release expressed as percentage of total content was higher (16.4% and 17.3%, respectively) than in rat (11.5% and 9. 9%, respectively). (5) Depolarization-induced glutamate and GABA release from neocortical synaptosomes from TLE patients was smaller than from rat synaptosomes (3.9% and 13.0% vs. 21.9% and 25.0%, respectively). (6) Analysis of breakdown of glial fibrillary acid protein (GFAP) indicates that resection time (anoxic period during the operation) is a critical parameter for the quality of the synaptosomes. We conclude that highly pure and viable synaptosomes can be isolated even from highly sclerotic human epileptic tissue. Our data show that in studies on human synaptosomes it is of critical importance to distinguish methodological (i.e., resection time) from pathology-related abnormalities.

Adult↗

PubMed1999

[No adverse effects on intelligence 6 years after surgery for epilepsy].

OBJECTIVE: To evaluate the long-term effects of epilepsy surgery on intelligence. DESIGN: Prospective descriptive. METHOD: The group included 20 patients of the Instituut voor Epilepsiebestrijding, Heemstede, and the Epilepsiecentrum Kempenhaeghe, Heeze, the Netherlands, who suffered from medication-resistant epilepsy originating from the temporal lobe, in 9 patients from the left lobe (mean age: 33.1 years), in 11 from the right one (34.6 years). The epilepsy focus was removed surgically (Academic Hospital, Utrecht). The scores on the 'Wechsler adult intelligence scale' (WAIS) were determined before the operation and 6 months, 2 years and 6 years after operation. RESULTS: The mean WAIS IQs before operation were: verbal IQ (VIQ): 111.8 and performance IQ (PIQ): 117.7 in the group treated on the left and VIQ: 113.5 and PIQ: 112.4 in the group treated on the right. The mean gains 6 years after operation were 0.8 and 2.6 VIQ-points and 8.7 and 8.5 PIQ-points respectively. In the patients operated on the left the difference between VIQ and PIQ was significant 2 and 6 years after operation. The increase in IQs remained within what could be expected in retests. Only for the VIQ of patients treated on the left was a smaller retest effect found. CONCLUSION: These figures show that in the long term epilepsy surgery does not have an adverse outcome on the intelligence.

Adult↗

PubMed1999

Language area localization with three-dimensional functional magnetic resonance imaging matches intrasulcal electrostimulation in Broca's area.

In this study, intraoperative electrocortical stimulation mapping (ioESM), the current gold standard for the localization of critical language areas, is compared with functional magnetic resonance imaging (fMRI) in a 14-year-old girl with medically intractable epilepsy caused by a tumor in the region of Broca's area. Prior to the operation, four different fMRI tasks that target inferior frontal language areas were applied. Prior to the resection, ioESM as well as fMRI detected no language areas at the exposed cortical area. After removal of the tumor, a unique opportunity presented itself, where ioESM could be performed in the depth of a now exposed and intact gyrus. One specific locus that was indicated to be a critical language area by multiple-task fMRI was targeted. IoESM selectively confirmed the location of this language area to within an estimated 3 mm. We propose that the combined use of different fMRI tasks increases the sensitivity and specificity for the detection of essential language areas.

Adolescent↗

PubMed1999

MR contribution in surgery of epilepsy.

The contribution of MR imaging in patients with drug-resistant epilepsy considered for surgical therapy is discussed. In this review we focus on: (a) focal abnormalities (mesial temporal sclerosis, focal migration disorders, hamartomatous lesions and low-grade tumours, phakomatosis and vascular malformations) associated with therapy-resistant partial epilepsy, requiring resective surgery; (b) abnormalities leading to generalized seizures that require more drastic surgical procedures, such as callosotomy and functional hemispherectomy; and (c) localisation of implanted depth-electrodes.

Blood Flow Velocity↗

PubMed1999

Non-linear analysis of intracranial human EEG in temporal lobe epilepsy.

OBJECTIVE: Intracranial EEG recordings from patients suffering from medically intractable temporal lobe epilepsy were analyzed with the aim of characterizing the dynamics of EEG epochs recorded before and during a seizure and comparing the classification of the EEG epochs on the basis of visual inspection to the results of the numerical analysis. METHODS: The stationarity of the selected EEGs was assessed qualitatively. The coarse-grained correlation dimension and coarse-grained correlation entropy were used for the non-linear characterization of the EEG epochs. RESULTS: High-pass filtering was necessary in order to make the majority of the epochs appear stationarity beyond a time scale of about 2 s. It was found that the dimension of the ictal EEGs decreased with respect to the epochs containing ongoing (interictal) activity. The entropy of the ictal recordings however increased. A scaling of the entropy was applied and it was found that the scaled entropy of the ictal EEG decreased, consistent with the increased regularity of the ictal EEG. The coarse-grained quantities discriminated well between EEG epochs recorded prior to and during seizures at locations displaying ictal activity and classification improved by including the linear autocorrelation time in the analysis. CONCLUSIONS: It is concluded that ictal and non-ictal EEG can be well distinguished on the basis of non-linear analysis. The results are in good agreement with the visual analysis.

Discriminant Analysis↗

PubMed1999

Relevance of temporal lobe white matter changes in hippocampal sclerosis. Magnetic resonance imaging and histology.

RATIONALE AND OBJECTIVES: To evaluate the diagnostic relevance of ipsilateral atrophy of the collateral white matter in the parahippocampal gyrus (ACWMp) and temporal lobe gray/white matter demarcation loss (GWDL) on magnetic resonance imaging in patients with histologically confirmed hippocampal sclerosis. In the second part of this investigation, histologic specimens were analyzed to find an explanation for GWDL. METHODS: Retrospective visual assessment of hippocampal signal intensity and size and of ACWMp and GWDL was performed using 4- to 5-mm coronal T2-weighted spin-echo magnetic resonance images of 80 patients with histologically proven hippocampal sclerosis and of 30 age-matched controls without epilepsy. Frequency of occurrence and likelihood ratios of ACWMp and GWDL were calculated and their contribution to the diagnosis of hippocampal sclerosis was assessed, particularly in patients with no or restricted hippocampal abnormalities (either high signal or smaller size) on magnetic resonance imaging. The second part of the study involved the morphologic histologic assessment of neocortical temporal lobe specimens of all patients. Myelin density was evaluated in specimens of a subgroup of six patients with hippocampal sclerosis and GWDL on MRI and six patients with hippocampal sclerosis without GWDL. RESULTS: ACWMp was found in 68% and GWDL in 65% of patients with hippocampal sclerosis on magnetic resonance imaging. Both features had an infinite positive likelihood ratio. Sixty-two patients (77.5%) had concomitant hippocampal signal increase and smaller size. Eighteen patients (22.5%) had no or restricted hippocampal abnormalities on magnetic resonance imaging. When using ACWMp and GWDL as additional diagnostic parameters, 13 of these 18 patients were more unambiguously diagnosed as having hippocampal sclerosis. No significant morphologic differences were found between GWDL-positive and GWDL-negative specimens. A significantly lower average myelin stain was found in the white matter of the GWDL-positive group compared to the GWDL-negative group. CONCLUSIONS: ACWMp and GWDL can improve the visual diagnosis of hippocampal sclerosis, particularly in patients with no or restricted hippocampal abnormalities. These results suggest that loss of myelin may be the underlying cause of GWDL in association with hippocampal sclerosis.

Adolescent↗