Periventricular nodular heterotopia and intractable temporal lobe epilepsy: poor outcome after temporal lobe resection

We describe 5 women and 5 men with periventricular nodular heterotopia and electroclinical features suggestive of temporal lobe epilepsy, who were surgically treated for control of medically refractory seizures. Magnetic resonance imaging revealed bilateral periventricular nodular heterotopia in 7 of the 10 patients. Because of the lack of clear localization, 6 patients were studied with intracranial depth electrode recordings. Seizures were of hippocampal onset (3 patients), regional temporal lobe onset (2 patients), or occipital-temporal onset (1 patient). Anterior temporal lobectomy was performed in 6 patients; selective amygdalohippocampectomy, in 1; and anterior temporal lobectomy plus resection of the heterotopic tissue, in 3. None of the 9 patients followed for more than 12 months postoperatively were seizure free. Two patients were initially seizure free for approximately 18 months, but then seizures recurred. One patient had a major reduction in seizure frequency at a 39-month follow-up after most of the unilateral heterotopic tissue was included in the temporal resection. Temporal resection did not lead to a long-term favorable outcome in this group of patients with periventricular nodular heterotopia and epileptogenic discharges involving the temporal lobe. This suggests a more widespread disorder with epileptogenic activity possibly originating in or near the heterotopic tissue. The clinical and electrographic features of periventricular nodular heterotopia pointing to temporal lobe origin are misleading and temporal resection does not result in long-term cessation of seizures.     

Outcome after temporal lobectomy in bilateral temporal lobe epilepsy

We determined how noninvasive presurgical data relate to prognosis after temporal lobectomy in patients with independent bilateral temporal lobe (IBTL) complex partial seizures on the intracranial electroencephalogram (EEG). Between 1986 and 1994, 28 patients had IBTL seizures on intracranial EEG. Fifteen of these 28 patients underwent temporal lobectomy and 13 were not offered surgery. Of the 15 patients who had surgery, 10 patients became seizure-free. Magnetic resonance imaging (MRI) and the Wada test were the only variables associated with a seizure-free outcome. Seven of 10 seizure-free patients had a lateralized Wada result or the presence of unilateral hippocampal sclerosis, whereas none of the patients with persistent seizures had either of these findings. Variables not found to be predictive of a seizure-free outcome included location of scalp interictal spikes, degree of seizure-onset laterality, presence of early epilepsy risk factor, duration of epilepsy, and full-scale intelligence quotient. We conclude that MRI and the Wada test provide information of prognostic value in patients with bilateral temporal seizures independent of intracranial EEG data.     

Bilateral temporal lobe volume reduction parallels cognitive impairment in progressive aphasia

BACKGROUND: Patients with isolated aphasia in the absence of other cognitive abnormalities have been the focus of several studies during the past decade. It has been called primary progressive aphasia (PPA), and the typical features of this syndrome are marked atrophy of the left temporal lobe according to the radiological examination and a language disorder as the initial symptom. In previous studies of PPA, the selection of the patients was based mainly on linguistic symptoms. Now, when computed tomography or magnetic resonance imaging scans are part of the routine investigation of cognitive impairment and suspected dementia, the patients with lobar atrophy will be found at an earlier stage. In the present study, we used a new approach and defined the study group by selecting patients with obvious left temporal lobe atrophy, assessed by MRI, and we referred to them as patients with temporal lobe atrophy (TLA). OBJECTIVE: To identify the features that distinguish TLA from other primary neurodegenerative disorders. PATIENTS: Six patients with TLA were compared with patients with Alzheimer disease (AD), patients with frontal lobe dementia (FLD), and healthy control subjects. METHODS: The investigations included magnetic resonance imaging volumetry, single photon emission computed tomography, and neuropsychologic and linguistic evaluations. RESULTS: In the TLA group, the mean volume of the left temporal lobe was 35% smaller than the right, while in the AD and FLD groups, the atrophy was symmetrical and bilateral. In the TLA group, the absolute volumes of the temporal lobes were significantly smaller on the left side compared with the AD and FLD groups, whereas there was no difference on the right side. The cerebral blood flow pattern in TLA was asymmetric and differed from that in the other study groups. All patients with TLA had a history of progressive Wernicke-type aphasia, ranging from 2 to 6 years. They showed primary verbal memory impairment but had preserved visuospatial functions. The clinical condition of all patients with TLA deteriorated during the study period; severe aphasia developed, and the patients exhibited signs of frontal lobe dysfunction. Serial volumetric measurements in 4 of 6 patients showed an annual 8% to 9% decrease of both left and right temporal lobes. CONCLUSIONS: The initial marked asymmetry in cognitive function found in patients with TLA contrasts with the general decline found in patients with AD. The bilateral degenerative process evident in patients with TLA paralleled the clinical deterioration, indicating TLA to be a non-AD lobar atrophy that develops into generalized cognitive dysfunction and dementia.     

Preictal SPECT in temporal lobe epilepsy: regional cerebral blood flow is increased prior to electroencephalography-seizure onset

Peri-ictal SPECT provides unique information on the dynamic changes in regional cerebral blood flow (rCBF) that occur during seizure evolution and, thus, could be useful in clarifying the poorly understood interplay of the interictal and ictal states in human focal epilepsy. The regional hyperperfusion observed on ictal SPECT is generally believed to be a consequence of electrical seizure activity. However, recent studies using invasive long-term cortical CBF monitoring have demonstrated that rCBF changes occur up to 20 min prior to ictal electroencephalography (EEG) onset. Because of apparent technical difficulties, no preictal SPECT studies have been reported so far. Therefore, we present our results on two patients with temporal lobe epilepsy in whom preictal SPECT scans were performed fortuitously under continuous video-EEG monitoring control. METHODS: Technetium-99m-hexamethyl propyleneamine oxime was injected 11 min (Patient 1) and 12 min (Patient 2) before clinical and EEG seizure onset, as documented from simultaneous video-EEG monitoring in two patients with temporal lobe epilepsy. We obtained accurate anatomical reference of CBF changes visible on SPECT by a special coregistration technique of MRI and SPECT. RESULTS: Whereas interictal SPECT showed a hypoperfusion of the temporal lobe ipsilateral to the seizure focus, on preictal SPECT, a significant increase in rCBF in the epileptic temporal lobe could be observed. These rCBF changes were not accompanied by any significant changes of the ongoing EEG. CONCLUSION: Our study provides evidence that rCBF is increased in the epileptic temporal lobe several minutes before EEG seizure onset. Thus, rCBF changes observed on peri-ictal SPECT scan cannot be considered a mere consequence of EEG seizure activity but may rather reflect a change in neuronal activity precipitating the transition from the interictal to the ictal state.     

Strategy of evaluation and surgical results in medial temporal lobe epilepsy]

To analyse the presurgical evaluation process of mediotemporal lobe epilepsy (MTLE) in relation to electroclinical characteristics of the patients, we reviewed the presurgical data (clinical, EEG, neuropsychological, MRI, FDG-PET and sometimes intracranial EEG) and the post-operative results of 53 consecutive cases treated by antero-medial temporal resection for MTLE (without lesion other than mediotemporal sclerosis). Forty one patients (77 p. 100) had a typical and well-lateralised presentation and were operated without invasive procedure. Twelve patients (23 p. 100) presented less pure data: unusual or absent auras, divergent scalp EEG features (bitemporal, widespread or extratemporal) or absence of MRI-based hippocampal sclerosis or FDG-PET hypometabolism. They were explored by invasive monitoring which confirmed the medial temporal origin of seizures. Outcome (follow-up: 30 months) was excellent in 88 p. 100 of the pure cases (Engel's class I) and less favorable in the more difficult ones (50 p. 100 of class I). In conclusion, the diagnosis and surgical treatment of MTLE can be performed without invasive investigations in the majority of cases owing to the strong contribution of brain imaging and video EEG monitoring. Some patients however present a less pure presentation suggesting more complex epileptogenic networks and associated to relatively less favorable postsurgical results.     

Amygdala atrophy and seizure outcome after temporal lobe epilepsy surgery

Surgical outcome in hippocampal atrophy (n = 44) and amygdalohippocampal atrophy (n = 14) were compared. Hippocampal atrophy had better seizure-free outcome than amygdalohippocampal atrophy (80% versus 50%, p = 0.043). Severity of hippocampal atrophy correlated with duration of epilepsy in patients with hippocampal atrophy (r = 0.4, p = 0.007), but not in those with amygdalohippocampal atrophy, suggesting that these two groups may have a different pathogenesis.     

Early stage vocal cord cancer: results after external beam radiotherapy

Much has been written about suck confusion due to a baby's oral experience with objects other than the breast--but how much difference is there in the relative impacts on a baby's ineffective suck if a teat is used instead of other currently accepted techniques such as cup and finger feeding? Mothers who seek the help of a lactation consultant will commonly already be using bottles and teats as part of their management regime for a difficult situation. Especially if the problems are well developed and complex, many mothers will not be able to accept unfamiliar alternatives such as cup and finger feeding and prefer to wean rather than do so. Current teat designs have evolved with no reference to Woolridge's landmark research published over ten years ago which clearly demonstrated how normal breastfeeding works. Rather, manufacturers offer consumers an array of teats which have been shortened in length and distorted in conformation when compared with teats available thirty years ago which coincidentally were a closer physiological match with the positioning of the nipple and breast tissues in the mouth of a breastfeeding baby than teats designed and produced since then. Fortunately one UK manufacturer still markets this thirty year old style of teat in a flow rate suitable for very young babies. Over a three year period, we at first hesitantly, but with increasing confidence asked mothers to use this teat when suck retraining and supplementation were indicated. We are able to report that use of these teats has resulted in a success rate in excess of 90% converting babies with significant sucking problems into fully effective breastfeeders.     

Long-term follow-up after temporal lobe resection for lesions associated with chronic seizures

A follow-up study was conducted on 60 patients who had standard en bloc anterior temporal lobe resection, including mesiotemporal structures, as treatment for temporal lobe lesions associated with chronic, medically intractable seizures. Lesions were identified as glial tumors, hamartomas, or vascular malformations. Long-term outcome was assessed in terms of seizure frequency and certain psychosocial sequelae. Seizure onset occurred at an average age of 15 years (median = 13.5 years), and patients experienced seizures for an average of 13 years prior to surgery. The mean time of follow-up was 8.4 years postsurgery (median = 6 years). The Kaplan-Meier curve at median follow-up showed a seizure-free rate of 80%. Late seizure recurrence was documented for three patients; two had been seizure-free for 10 years and one for 15 years after surgery, before re-onset of seizures in the absence of tumor recurrence. A prolonged history of seizures prior to surgery was associated with a poorer seizure outcome (p = 0.06), suggesting that secondary epileptogenesis at sites distant to the lesion may develop with years of uncontrolled seizures. There was a low tumor recurrence rate of 3.3% (two cases). The psychosocial outcome was generally good, with 67% working or engaged in educational studies, and improvement noted in 59% of cases for one or more of the psychosocial factors investigated. This study confirms that anterior temporal lobe resection for temporal lesions associated with chronic seizures is a successful treatment with a high seizure-free rate following surgery and good psychosocial outcome.     

Olfactory functioning before and after temporal lobe resection for intractable seizures.

An olfactory test battery was administered monorhinically to 21 epilepsy patients prior to and 6 mo after unilateral medial temporal lobe resection for treatment of intractable seizures. 33 matched control Ss were also tested. Presurgically, controls and patients exhibited normal sensitivity for 1-butanol. Patients performed at levels significantly below controls on tests for odor discrimination, odor memory, and odor naming. Left-resected patients did not show significant changes following surgery. Right-resected patients demonstrated significant right nostril decline. Postsurgical changes demonstrated by the right-resected group were not attributable to deficits in primary sensory function or to deficits in cognition brought on by surgery. These findings imply that right medial temporal lobe structures play a greater role in olfactory processing than do corresponding structures in the left hemisphere. (PsycINFO Database Record (c) 2010 APA, all rights reserved)     

Familial temporal lobe epilepsy: a clinically heterogeneous syndrome

We describe the clinical characteristics of a group of patients with familial temporal lobe epilepsy (TLE) in 11 kindreds with 36 affected individuals identified and investigated at the Montreal Neurological Hospital. Seizure types were simple partial (n = 20), complex partial (n = 29), and rare generalized tonic-clonic. Simple and complex partial seizures were infrequent or well controlled by anticonvulsant medication in 17 of 29 patients (59%) and without optimal response to medical therapy in 12 of 29 patients (41%). Pedigree analysis suggested autosomal dominant inheritance with incomplete penetrance. The syndrome of familial TLE has heterogeneous clinical manifestations and is not always benign.     

Anterior temporal abnormality in temporal lobe epilepsy: a quantitative MRI and histopathologic study

OBJECTIVE: To examine the nature and frequency of anterior temporal lobe (AT) abnormalities that occur in intractable temporal lobe epilepsy (TLE). METHODS: We reviewed the MR scans and clinical histories of 50 consecutive patients with intractable TLE. Histopathology was available in 42 surgically treated cases. RESULTS: MRI demonstrated loss of the gray-white matter differentiation and decreased T1- and increased T2-weighted signal in the ipsilateral AT in 58% of the 50 patients. This appearance was observed in 64% of the 36 patients with hippocampal sclerosis (HS) but was also seen in patients without HS. These changes were associated with temporal lobe atrophy, a higher hippocampal T2 relaxation time, and a history of febrile convulsions. Pathologic examination showed that the MRI appearances were not caused by dysplasia, degenerative abnormalities, or inflammatory change. Histologic quantitation showed increased glial cell nuclei counts in the intractable TLE cases compared with controls. There was no difference in glial cell numbers between cases with AT abnormality and those without this appearance. Presence or absence of changes was not predictive of preoperative neuropsychology, postoperative change in neuropsychology, or seizure outcome after surgery. CONCLUSIONS: These frequently seen ipsilateral changes are not caused by gliosis and may reflect a nonspecific increase in water content in the temporal lobe. This may be due to myelin abnormalities or some other as yet unidentified pathologic factor.     

The anterior and posterior selective temporal lobe amobarbital tests: angiographic, clinical, electroencephalographic, PET, SPECT findings, and memory performance

The techniques, results, and problems of three types of selective temporal lobe (TL) amobarbital procedures (balloon technique with temporary occlusion of the internal carotid artery distal to the origin of the anterior choroidal artery (acha) [n = 19]; selective anterior catheterization of the acha [n = 20]; and selective catheterization of the peduncular P2-segment of the posterior cerebral artery [n = 5]) are described in a group of 40 patients with medically refractory complex partial seizures of mesial TL origin. Selective amobarbital tests were carried out before surgery to predict the memory deficit after an intended selective amygdalohippocampectomy. The effects of selective anaesthetization of TL were correlated with clinical data, pattern and duration of amobarbital induced EEG changes, and performance on verbal and nonverbal memory tasks measured during the test. In 4 patients the effect of selective amobarbital injection on regional and global metabolism was studied with 18F-FDG-PET, with the PET tracer being injected intravenously immediately after amobarbital. More recently in 2 patients the vascular territory perfused by amobarbital in the acha test was studied with SPECT using 99m Tc ECD injected immediately prior to the amobarbital into the acha. Whereas the PET studies showed a rather widespread and bilateral amobarbital-induced decrease of metabolism, the SPECT studies confirmed the selective distribution of the tracer in the vascular territory of the acha, i.e., in amygdala and hippocampus. The comparison of selective TL amobarbital test performance with postoperative neuropsychological performance showed that the predictive value of this test is rather good for the postoperative verbal memory but underestimates postoperative nonverbal ("figural") memory performance.     

Microdysgenesis in mesial temporal lobe epilepsy: a clinicopathological study

The interrelationship of mesial temporal lobe epilepsy (MTLE), hippocampal sclerosis, and febrile convulsions still remains an enigma. Additional microscopical cortical dysplasia or microdysgenesis has been suggested as pre-existent susceptibility factor rendering the affected brain vulnerable to the development of MTLE after initial precipitating injuries such as febrile convulsions. Twenty-four MTLE cases with histopathologically definite hippocampal sclerosis were examined for clearly defined features of microdysgenesis and further signs of neocortical dysplasia. Although unequivocal signs of dysplasia were absent, 29.2% of cases showed cortical neuronal clustering, 25.0% showed perivascular clustering, and 20.8% showed increased white matter neurons. The features of microdysgenesis studied here were not linked with each other and were not related to initial precipitating injuries, positive family history, or any other clinical parameter. Their suggested fundamental role as dysplastic factor within development of hippocampal sclerosis and MTLE is not confirmed.     

Late results after pulmonary middle lobe resections in childhood (author's transl)

15 of 19 children with typical middle-lobe syndrome were reinvestigated 4 to 10 years after surgical treatment. Morphological and functional alterations as a result of middle-lobe resection are of minor importance. The treatment of choice in childhood is the pulmonary lobe resection. Late effects of bronchiolitis follicularis are important because multifocal spread of infection seems to be possible.     

Medial temporal lobe epilepsy: videotape analysis of objective clinical seizure characteristics

PURPOSE: The syndrome of temporal lobe epilepsy has been described in great detail. Here we focus specifically on the clinical manifestations of seizures originating in the hippocampus and surrounding mesial temporal structures. METHODS: Seizure origin was confirmed in 67 cases by depth EEG recording and surgical cure after mesial temporal resection. RESULTS: Among nonlateralized manifestations, we commonly found oral automatisms, pupillary dilatation, impaired consciousness, and generalized rigidity. Appendicular automatisms were often ipsilateral to the seizure focus, whereas dystonia and postictal hemiparesis were usually contralateral. Head deviation, when it occurred early in the seizure, was an ipsilateral finding, but was contralateral to the seizure focus when it occurred late. Clear ictal speech and quick recovery were found when seizures originated in the non-language-dominant hemisphere, but postictal aphasia and prolonged recovery time were characteristic of seizure origin in the language-dominant hemisphere. CONCLUSIONS: These signs help to define the mesial temporal lobe epilepsy (MTLE) syndrome and often provide information as to the side of seizure origin.     

Human medial temporal lobe in aging: anatomical basis of memory preservation

Nucleotide sequence analyses of polymerase chain reaction-amplified genes were performed to determine whether adaptation of herpes simplex virus type 2 to replication in cultured cells or in internal organs during neonatal disseminated disease results in selection of variants with altered forms of three glycoproteins (gB, gC, or gD) that influence virus entry into cells. No variations in sequence were noted as a consequence of in vitro passage or replication in different organs. Five viruses from different subjects differed with respect to gB, gC, and gD gene sequences, expressing four distinct forms of gB, three of gC, and two of gD. These differences did not confer resistance to neutralization by guinea pig or human antisera from subjects immunized with recombinant gB or gD vaccines and may not be consequential for vaccine development.     

Medial temporal lobe atrophy in stroke patients: relation to pre-existing dementia

BACKGROUND: To investigate the role and impact of multiplane transesophageal echocardiography during thrombectomy in the inferior vena cava or the right atrium. EXPERIMENTAL DESIGN: Retrospective. SETTING: A university hospital. PARTICIPANTS: Four patients who underwent removal of tumor thrombus in the inferior vena cava (IVC) or the right atrium. INTERVENTIONS: The medical records of 4 patients and videotapes of these intraoperative transesophageal echocardiography examinations were reviewed. RESULTS: Before thrombectomy, multiplane transesophageal echocardiography (MTEE) provided excellent IVC long axis view, which offered precise recognition of the cephalic extent of tumor, extent of caval occlusion, characterization of the tumor head. During surgery, MTEE could provide continuous monitoring of cardiac function, cardiac volume, and pulmonary embolism. Moreover, MTEE could provide the useful images of a cannula or the caval occlusion balloon catheter, which facilitated removal of neoplasm extending into the IVC. CONCLUSIONS: We presented four surgical cases, in which the removal of the tumor extended into the inferior vena cava or the right atrium using MTEE. MTEE could provide valuable information such as excellent images of the tumor, cardiac function, the position of a cannula or the caval occlusion balloon catheter. These findings could improve the anesthetic management of the patients, as well as the surgical approach and technical maneuvers, and facilitate removal of neoplasm into the IVC.     

Distinctive forms of partial retrograde amnesia after asymmetric temporal lobe lesions: possible role of the occipitotemporal gyri in memory

We tested the hypothesis that partial forms of retrograde amnesia were associated with highly asymmetric lesions to the inferior and anterior-medial temporal lobe. Postencephalitic subjects EK and DR were both impaired on standardized retrograde memory tests, but showed strikingly different profiles in cognitive tasks of name stem completion, name:face matching, temporal ordering, forced choice recognition, and occupational judgments of famous names and faces from the past 3 decades. EK sustained left inferior and anterior-medial temporal lobe lesion with a small right temporal polar lesion, and showed near-complete loss of retrieval, knowledge, and familiarity associated with famous names but minimal deficiencies with famous faces. DR sustained right inferior and anterior-medial temporal lobe lesion and showed a milder retrograde loss limited to utilizing famous face prompts in name stem completion, name:face matching, occupational judgments, and forced choice recognition. These impairments were also different from the memory retrieval deficit, but intact recognition shown by a case of ruptured anterior communicating artery aneurysm with presumed basal forebrain damage. We hypothesize that EK's extensive loss of famous name knowledge was related to left inferior temporal lobe damage, particularly in the lateral and medial occipitotemporal gyri. This region in the left temporal lobe may serve as a critical processing area for retrograde memory that permits activation of established semantic, temporal, and visual (i.e., facial) associations biographically dependent on the category of proper names. On the basis of connectional anatomy patterns in the nonhuman primate, this region receives extensive hippocampal output and is interconnected with the temporal polar region and cortical association areas, which have been implicated in retrieval and storage aspects of retrograde memory. In the right hemisphere, the occipitotemporal gyri may serve an important role in famous face processing as part of a bilateral neural network.