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The mesial-temporal lobe and autism: case report and review.

Human and non-human primate research has shown that pathological processes affecting the temporal lobe, particularly the amygdala and hippocampus, are related to the development of the autistic syndrome. This case report describes a young male child with left temporal oligodendroglioma, who demonstrated a constellation of autistic behaviors meeting DSM-III-R criteria for pervasive developmental disorder. Abnormalities in social interaction, affective expression and communication were particularly evident. Some of the symptoms improved after tumor resection, while other signs of qualitative abnormalities in development emerged or persisted. This case adds evidence to the hypothesis that damage to mesial-temporal structures at an early developmental period may lead to the autistic syndrome.

Autistic Disorder↗

Treatment-responsive limbic encephalitis identified by neuropil antibodies: MRI and PET correlates.

We report seven patients, six from a single institution, who developed subacute limbic encephalitis initially considered of uncertain aetiology. Four patients presented with symptoms of hippocampal dysfunction (i.e. severe short-term memory loss) and three with extensive limbic dysfunction (i.e. confusion, seizures and suspected psychosis). Brain MRI and [(18)F]fluorodeoxyglucose (FDG)-PET complemented each other but did not overlap in 50% of the patients. Combining both tests, all patients had temporal lobe abnormalities, five with additional areas involved. In one patient, FDG hyperactivity in the brainstem that was normal on MRI correlated with central hypoventilation; in another case, hyperactivity in the cerebellum anticipated ataxia. All patients had abnormal CSF: six pleocytosis, six had increased protein concentration, and three of five examined had oligoclonal bands. A tumour was identified and removed in four patients (mediastinal teratoma, thymoma, thymic carcinoma and thyroid cancer) and not treated in one (ovarian teratoma). An immunohistochemical technique that facilitates the detection of antibodies to cell surface or synaptic proteins demonstrated that six patients had antibodies to the neuropil of hippocampus or cerebellum, and one to intraneuronal antigens. Only one of the neuropil antibodies corresponded to voltage-gated potassium channel (VGKC) antibodies; the other five (two with identical specificity) reacted with antigens concentrated in areas of high dendritic density or synaptic-enriched regions of the hippocampus or cerebellum. Preliminary characterization of these antigens indicates that they are diverse and expressed on the neuronal cell membrane and dendrites; they do not co-localize with VGKCs, but partially co-localize with spinophilin. A target autoantigen in one of the patients co-localizes with a cell surface protein involved in hippocampal dendritic development. All patients except the one with antibodies to intracellular antigens had dramatic clinical and neuroimaging responses to immunotherapy or tumour resection; two patients had neurological relapse and improved with immunotherapy. Overall, the phenotype associated with the novel neuropil antibodies includes dominant behavioural and psychiatric symptoms and seizures that often interfere with the evaluation of cognition and memory, and brain MRI or FDG-PET abnormalities less frequently restricted to the medial temporal lobes than in patients with classical paraneoplastic or VGKC antibodies. When compared with patients with VGKC antibodies, patients with these novel antibodies are more likely to have CSF inflammatory abnormalities and systemic tumours (teratoma and thymoma), and they do not develop SIADH-like hyponatraemia. Although most autoantigens await characterization, all share intense expression by the neuropil of hippocampus, with patterns of immunolabelling characteristic enough to suggest the diagnosis of these disorders and predict response to treatment.

Adult↗

Occipitotemporal epilepsies: evaluation of selected patients requiring depth electrodes studies and rationale for surgical approaches.

In 8 patients in whom it was uncertain whether they had occipital or temporal lobe (TL) epilepsy, clinical, scalp EEG, and radiologic features were correlated with the sites of seizure onset as determined by depth EEG. The 8 patients were selected from > 40 with occipital epilepsy because they had (a) an aura considered to be of occipital lobe (OL) origin, (b) an occipital interictal epileptic focus, (c) an OL lesion, or (d) a combination of all of these. Scalp EEG and clinical patterns suggested temporal involvement in all, however. Extracranial EEG recordings were often misleading, showing multilobar interictal epileptic abnormalities, and seizure onset was of poor localizing value and did not clarify the problem sufficiently. Intracranial EEG recordings showed that seizure onset could be ordered along an occipitotemporal gradient. Consistent OL seizure onset was observed in patients who had only elementary visual auras. Those who had inconsistent aura or no aura, suggesting OL origin, had onset of most attacks in the TL. All patients had a seizure spread pattern suggesting early TL involvement. To prevent visual field defect, surgical approaches included temporal resection when temporal seizure origin or spread was demonstrated; although occasionally this produced excellent results, it was of limited benefit in most patients, even when some seizures were proven to originate in TL structures. In patients with malignant epilepsy and in those with an occipital lesion, occipital resection should be considered.

Adolescent↗

Epileptogenic foci on subdural recording in intractable epilepsy patients with temporal dysembryoplastic neuroepithelial tumor.

To investigate the epileptogenic foci in dysembryoplastic neuroepithelial tumor (DNT) in the temporal lobe, we studied extraoperative electrocorticography (ECoG) with subdural electrode arrays from nine patients with intractable epilepsy due to temporal DNT. Ictal onset zones and irritative zones were decided by the ECoG. The locations of these zones were compared to the location of the tumor. The number of ictal onset zone and irritative zone was 2.1+/-0.93 and 2.9+/-1.45 in a patient with a DNT. They were detected more frequently in the adjacent tissues of the tumor (88.9%) rather than within the tumor or in mesial temporal area (66.7%). Mesial temporal involvement was found in 6 patients (66.7%) as an ictal onset zone, and in 5 (55.6%) as an irritative zone. The 7 patients (77.8%) had ictal onset zone in areas different from active irritative zone. The surgical outcome was better, when ictal onset zone was completely resected rather than partially removed. Temporal DNT can make multiple ictal onset zones and irritative zones in different regions including the mesial temporal area. Deliberate resection of epileptogenic foci, including all ictal onset zones and irritative zones, ensures excellent seizure control.

Adolescent↗

[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.

Adult↗

The crocodile tear syndrome.

Gusto-lacrimation, or "crocodile tear syndrome," is a rare complication, with 95 cases reported in the literature. Two patients are presented here, one after a facial fracture which apparently extended into the temporal bone proximal to the optic ganglion, and one after a Bell's palsy. The mechanism appears to be a misdirection of regenerating gustatory fibers destined for the salivary glands, so that they become secretory fibers to the lacrimal gland and cause homolateral tearing while the patient is eating. A simple procedure, involving subtotal resection of the palpebral lobe of the involved lacrimal gland, proved to be an effective corrective measure in these cases. Although it was not done in these cases, it would perhaps be advisable to do a Schirmer's test to assist in determining the amount of gland to be removed.

Adult↗

Surgical treatment of intractable epilepsy attributable to multiple sclerosis.

Surgically removing a focus of epileptogenicity attributable to a multiple sclerosis (MS) plaque has not previously been considered a treatment option. Medically intractable partial epilepsy due to a chronically situated MS plaque is uncommon because most cases are self-limiting or managed with antiepileptic medication. We report a case of partial epilepsy resulting from such a plaque situated at the gray-white interface in the anterior parahippocampal gyrus. A favorable outcome was achieved by resection of the epileptogenic area.

Adult↗

The electrocorticographic status and the extent of the cortical resection in epileptic patients.

In three patients operated on for focal epilepsy small pathological lesions in the functionally important cortical regions were microsurgically extirpated. The neighbouring areas of cortex were preseved, although they showed epileptic activity on electrocorticography. The patients are seizure-free three years, two years, and nineteen months later, respectively. On the bases of these facts and experience with another 35 patients operated on for focal epilepsy the authors speculate on the relations between the pathological lesion, epileptogenic cortical area, and the relative dependence of the extent of cortical resection on the electrocorticographic status.

Adult↗

A retrospective study of the clinical outcomes and significant variables in the surgical treatment of temporal lobe tumor associated with intractable seizures.

A retrospective study was performed assessing the preoperative evaluation, and the postoperative outcomes in 35 patients with a temporal lobe tumor with intractable seizures who were treated by our neurosurgery service between October 1995 and December 1999. The mean age of the patients at the time of surgery was 27.9 years. The period of follow-up after surgery was a mean of 33.0 months. Of the study group of 35 patients, 27 (77.1%) became completely seizure free after surgery (class I), and 2 patients (5.7%) had no more than 2 seizures per year (class II). Worthwhile seizure control was achieved in 29 patients (82.8%). There was a statistical significance between the extent of tumor resection and favorable seizure outcome (p = 0.014). The most frequent histopathological diagnoses were classic epilepsy-associated ganglioglioma in 16 (45.7%) patients and low-grade astrocytoma in 10 (28.6%) patients. Complete resection of tumor was the most significant factor in obtaining a favorable seizure outcome.

Adolescent↗

MRI lesion and epileptogenic focus in temporal lobe epilepsy.

The spatial relationship between a circumscribed lesion in the temporal lobe detected by MRI and an epileptogenic focus identified by ictal depth EEG along with a correlation of the MRI lesion with neuropathological findings were investigated in patients with medically intractable temporal lobe epilepsy but without any focal lesion on CT. Four parameters (an areal ratio of the temporal lobe against the hemisphere, area and calculated T1, T2 values of the hippocampus) were used to determine the abnormal MRI side. An agreement was reached in 67-72% of 18 patients between the abnormal values of the hippocampal area and of calculated T1, T2 and the side of the epileptogenic focus. In 14 of 17 patients, typical hippocampal sclerosis was demonstrated in resected tissue in accordance with the MRI lesions (atrophy and/or prolonged T2 of hippocampus). These results imply: 1) MRI abnormality thus defined may, if not all, indicate the side of the epileptogenic focus, and 2) also the presence of hippocampal sclerosis. It was emphasized that the MRI lesion would be a usable instrument to explore the causal relationship of hippocampal sclerosis to a generation of epileptogenic lesions as well as for presurgical evaluation.

Adolescent↗

Microanatomy of medial temporal area and subtemporal amygdalohippocampectomy.

For seizure control in temporal lobe epilepsy, the head of the hippocampus to the choroidal point, parahippocampal gyrus, entorhinal area, uncus, and at least the basolateral nucleus of the amygdala should be completely removed. The subtemporal approach should be selected for removal of these structures, and it does not interrupt the temporal stem and optic radiation. Pre- and postoperative neuropsychological examinations revealed that there is no significant decline of scores of various examinations including WAIS, WMS, Randt memory, and verbal associates learning tests, even if the resection were performed on the language dominant side. Seizure control for the 20 non-lesional patients operated with this approach is 60% (Class I and II), without definite permanent complications.

Amygdala↗

The lateralizing and surgical prognostic value of a single 2-hour EEG in mesial TLE.

We investigated 109 patients who received anterior temporal lobectomy for intractable mTLE by post-operative follow-up for at least 11/2 years. We reviewed pre-operative 2-hour interictal EEGs, counted interictal epileptiform discharges (IEDs), and compared the lateralization of IEDs with the side of surgery and surgical outcome. Twenty of 22 patients who had no spikes and 44 of 51 who had unitemporal spikes became seizure free after surgical resection. The correct lateralization of the epileptogenic side was possible in 90. 9% of the patients with unitemporal IEDs. In 12 seizure-free patients of 15 patients with less than 70% predominance of IED in one temporal lobe, the positive predictive value of the lateralization was 41.7%. In 16 seizure-free patients of 21 with more than 70% preponderance of IED in one lobe, the positive predictive value was 81.3%. Surgical outcome of patients with unitemporal and bitemporal IEDs were not significantly different. Interictal scalp EEG can be used as a lateralizing tool in mTLE when the temporal IEDs appear with more than 70% preponderance in one side. Although the presence of bitemporal IEDs often causes confusion in terms of the correct lateralization, it does not affect the surgical outcome.

Adult↗

From the archives of the AFIP: superficial gliomas: radiologic-pathologic correlation. Armed Forces Institute of Pathology.

Glial neoplasms that are peripherally located and involve the cortical gray matter are noteworthy because of their predilection to serve as a seizure locus, their amenability to surgical resection, their generally favorable prognosis, and their characteristic imaging features, which facilitate diagnosis before surgery. The smaller lesions include ganglioglioma and dysembryoplastic neuroepithelial tumor. Gangliogliomas contain both neuronal and glial components and occur most commonly in the temporal lobe. Variant forms of gangliogliomas may occur and are related to the different compositions of the underlying cellular population. Gangliocytomas lack glial cells and are located both in the cerebral hemispheres and the cerebellum. Lhermitte-Duclos disease represents a specific type of cerebellar gangliocytoma with dysplastic features and is characterized by a laminar pattern at imaging. Dysembryoplastic neuroepithelial tumors occur predominantly in children and young adults with partial seizures and most commonly arise in the temporal lobe, frequently in combination with cortical dysplasia. Surrounding vasogenic edema is conspicuously absent in both gangliogliomas and dysembryoplastic neuroepithelial tumors. The larger masses in this group include desmoplastic infantile ganglioglioma and pleomorphic xanthoastrocytoma and tend to involve the leptomeninges and cortical territory. Both invoke an intense desmoplastic reaction, which appears as an enhancing soft-tissue component at imaging.

Adolescent↗

Stereotactic amygdalohippocampotomy for the treatment of medial temporal lobe epilepsy.

PURPOSE: This study was carried out to assess the safety and efficacy of stereotactic ablation of the amygdala and hippocampus for the treatment of medial temporal lobe epilepsy. METHODS: Twenty-two stereotactic amygdalohippocampotomies were performed in 19 patients with unilateral temporal lobe seizures by using magnetic resonance imaging (MRI) localization for target planning and radiofrequency techniques for lesion production. Seizure frequency was assessed at 3-monthly follow-up visits. Two lesion groups were defined. In group I, four to 11 (mean, 6.4) discrete lesions were made, encompassing the amygdala and anterior 13-21 mm (mean, 16.8 mm) of the hippocampus. In group II, a large number of confluent lesions were made (mean, 26.0; range, 12-54) encompassing the amygdala and anterior 15-34 mm (mean, 21.5 mm) of the hippocampus. MRI scanning was carried out 24 h and 6-9 months after surgery. RESULTS: In five group I patients, one (20%) experienced a favorable seizure outcome. Of 15 group II patients, one of whom had previously undergone limited lesioning and was also analyzed as part of group I, nine (60%) experienced a favorable seizure outcome, with two seizure free. MRI scans at 6- to 9-months' follow-up disclosed discrete areas of atrophy in the amygdala and hippocampus, interspersed with preserved brain in the group I patients. More uniform and complete destruction of amygdala and hippocampus was evident in group II patients. All lesions were confined to the amygdala and hippocampus, sparing the parahippocampal gyrus (PHG). CONCLUSIONS: The extensive amygdalohippocampal ablation in group II patients improved seizure outcome compared with more limited ablation in group I, but these results were not so good as those from temporal lobectomy in a similar patient group. When considered together with the results of selective amygdalohippocampectomy, and temporal resections that spare hippocampus or amygdala (all producing similar outcomes, and all involving resection of the entorhinal cortex), this study suggests a pivotal role of the entorhinal cortex in temporal epileptogenesis.

Adolescent↗

Characteristics of medial temporal lobe epilepsy: II. Interictal and ictal scalp electroencephalography, neuropsychological testing, neuroimaging, surgical results, and pathology.

Sixty-seven patients with temporal lobe epilepsy without circumscribed, potentially epileptogenic lesions, who were studied with intracranial electrodes and who became seizure free following temporal lobectomy were retrospectively evaluated with regard to preoperative scalp electroencephalographic (EEG) findings, neuropsychological test results, neuroimaging findings, results of surgery, and pathology of resected tissue. Interictal scalp EEG showed paroxysmal abnormalities during prolonged monitoring in 64 patients (96%). These were localized in the anterior temporal region in 60 (94%) of these 64 patients. Bilateral independent paroxysmal activity occurred in 42% of the patients and was preponderant over the side of seizure origin in half. Ictal EEG changes were rarely detected at the time of clinical seizure onset, but lateralized buildup of rhythmic seizure activity during the seizure occurred in 80% of patients. In 13%, the scalp EEG seizure buildup was, however, contralateral to the side of seizure origin as subsequently determined by depth EEG and curative surgery. Lateralized postictal slowing, when present, was a very reliable lateralizing finding. Neuropsychological testing provided lateralizing findings concordant with the side of seizure origin in 73% of patients. When neuropsychological testing produced discordant results or nonlateralizing findings, those patients were usually found to have right temporal seizure origin. Intracarotid amobarbital (Amytal) testing demonstrated absent or marginal memory functions on the side of seizure onset in 63% of patients, but 26 patients (37%) had bilaterally intact memory. In those patients who had magnetic resonance imaging, it was very sensitive in detecting subtle medial temporal abnormalities. These abnormalities were present in 23 of 28 magnetic resonance images, and corresponded with mesial temporal sclerosis on pathological examination in all but 2 patients.(ABSTRACT TRUNCATED AT 250 WORDS)

Brain↗

[Image-guided microsurgery in resection of intracranial cavernous hemangioma].

OBJECTIVE: To study the methods and effectiveness of image-guided microsurgery in resection of intracranial cavernous hemangioma. METHODS: Between July 1997 and January 2001, 44 patients with intracranial cavernous hemangioma, 27 males and 17 females with a mean age of 35 years, among which 5 cases had multiple lesions, underwent image-guided microsurgery. The locations of lesions included frontal lobe (n = 14), temporal lobe (n = 12), parietal lobe (n = 6), cerebellum (n = 6), thalamus (n = 5), pons (n = 5), and orbital lobe (n = 1). A small silicon catheter, used as a guider, was implanted to the deep-seated lesion (except the brain stem lesions) before excision of the lesion in order to prevent brain shift. RESULTS: Total removal of the lesions was achieved in all patients without operational death. Follow-up revealed marked improvement of symptoms in 26 case and no change of symptom(s) in 13 cases. 5 cases suffered from additional neurological deficits, mainly exacerbation of hemiparalysis and aphasia, the condition of two of which, however, gradually improved within the period of follow-up. No residue of lesion and relapse were found during follow up. CONCLUSION: With the assistance of the image-guided surgical system, functional area can be effectively avoided, and surgical injury can be decreased. It is well suited for accurate localization and safe resection of small, deep-seated cavernous malformations.

Adolescent↗

Extranodal sinus histiocytosis (Rosai-Dorfman disease) of the brain parenchyma.

Rosai-Dorfman Disease (RDD) is an idiopathic histiocytic proliferation affecting lymph nodes. Although extranodal involvement has been reported in diverse sites, manifestation in the central nervous system (CNS) is extremely rare, particularly in the brain parenchyma. A 39-year-old male presented with an isolated well-circumscribed brain mass in the right temporal lobe, preoperatively thought to be a meningioma. Histology and immunohistochemistry confirmed that the lesion was RDD. The intraparenchymal brain location of RDD appears to have a benign course. Although the adjuvant therapy is a treatment of choice, surgical resection seems to be the appropriate treatment modality. From the clinical point of view RDD might be an important intracerebral entity because it may mimic other lesions, particularly other histiocytic disorders.

Adult↗

[Surgical treatment of cerebral vascular pathologies in epileptic patients].

Surgery of arteriovenous malformations (AVM) and of cavernous angiomas (cavernoma) in the majority of cases is indicated subsequently to episodes of bleeding. With the development of techniques for diagnosis and surgery for epilepsy of difficult control, indication for surgery of these vascular lesions has become greater. We present nine patients with cerebral vascular lesions and very frequent crises in spite of adequate clinical treatment. Ages ranged from 12 to 42 years with an average of 25 years; there was a prevalence of the male sex (2:1). Surgery consisted of exercises of the lesion in all cases and in four there was also resection of the perilesional irritative area shown by electrocorticography. The pathologic study of lesions showed five cases of cavernoma, three cases of AVM, and one case of venous angioma. As to localization, we observed three lesions in the temporal lobe, four in the frontal, and two in the parietal region. Outpatient follow-up showed a reduction in crises in all of the patients, and seven evolved seizure free following surgery.

Adolescent↗