Celiac Disease, Bilateral Occipital Calcifications and Intractable Epilepsy: Mechanisms of Seizure Origin

Summary: Purpose: To elucidate the mechanisms of seizure origin in patients with celiac disease and bilateral occipital calcifications (CEBOC). Individuals with CEBOC frequently present with occipital lobe seizures, but additional lesions and additional attack patterns may occur.
Methods: We studied two men and one woman who had CEBOC. Villous atrophy was revealed in the two patients who underwent duodenal biopsy. All had a comprehensive presurgical evaluation, including prolonged video-EEG recordings. Two had magnetic resonance imaging (MRI) with volumetric study of mesial temporal structures (MRIV). One patient had undergone stereotactic intracranial depth electrode studies (SEEG).
Results: All patients presented with intractable complex partial seizures. Two had partial simple seizures with visual aura. Neurologic examination was normal; one was of normal intelligence, and two were mildly retarded. Neuroimaging studies showed that each had bilateral occipital calcifications as well as epileptiform abnormalities over temporal lobes. In one, MRI showed an additional right frontal lesion, but SEEG demonstrated right occipital lobe seizure origin with anterior spread; this male patient later underwent a right occipital lobe resection. Another with a history of prolonged febrile convulsions had bilateral hippocampal and amygdalar atrophy demonstrated by MRIV.
Conclusions: In one patient, SEEG confirmed that seizures originated in the occipital lobe. The presence of dual pathology was demonstrated in another, raising the possibility of both occipital and temporal seizure onset. The presence of extraoccipital lesions or of mesial temporal atrophy requires SEEG for clarification of seizure onset. In the absence of confounding factors and when laterality can be demonstrated, surgical treatment may be considered.     

A case of right mesial temporal lobe epilepsy accompanied with ictal polyopsia]

A 59-year-old, right-handed woman had a paroxysmal polyoptic visual illusion, in which multiple copies of the object she saw spread horizontally in the left hemi-visual field. Polyopsia appeared for a few seconds. Neurological examination was normal. Magnetic resonance imaging (MRI) showed a tumor-like lesion involving the cortical and subcortical matters in the right mesial temporal regions. An interictal EEG showed frequent spikes in the right mesial temporal area and intermittent theta waves in the right fronto-temporal area. Video-EEG monitoring using the sphenoidal electrodes showed the seizure discharges originating in the right sphenoidal lead accompanying the polyoptic visual illusion. The seizure discharges were restricted within the right mesial temporal lobe. Paroxysmal visual illusion disappeared after administration of anti-epileptic drugs. EEG showed rare spikes in the right mesial temporal area. Polyopsia in this patient presumed to be associated with right mesial temporal lobe epilepsy because polyopsia and seizure activities on the ictal EEG were coupled and polyopsia ceased after administration of anti-epileptic drugs. Polyopsia is recognized as visual perseveration in space and a rare visual illusion. The lesion causing polyopsia has been reported to be mostly within posterior cerebral areas including occipital, parietal and temporal regions. This patient whose ictal polyopsia associated with mesial temporal lobe epilepsy is a very rare case because most reported cases presenting ictal polyopsia are neocortical temporal lobe epilepsy. The precise mechanism of polyopsia remains unknown. The mesial temporal lobe includes the hippocampus and parahippocampal formation that have been reported to receive information from the diverse association cortex and work as memory controllers. Ictal polyopsia may result from dysfunction of the visual association cortex or visual memory systems induced by the epileptic activities in the mesial temporal lobe. Polyopsia is a rare ictal semeiology of mesial temporal lobe epilepsy and may be one of the important ictal symptoms.     

Ictal Oroalimentary Automatisms with Preserved Consciousness: Implications for the Pathophysiology of Automatisms and Relevance to the International Classification of Seizures

Summary: A patient showing seizures presenting ictal automatisms with preserved consciousness is reported. A 30-year-old, right-handed man with normal development and without family history of epilepsy was referred for surgical treatment of epilepsy. At 15 he began to have seizures, starting with an epigastric aura, occasionally developing automatisms (lip-smacking, chewing), sometimes followed by tonicxAonic convulsions. At the time of referral, he averaged six convulsive seizures per year and one nonconvulsive per week. His sleep EEG showed sharpened slow activity over the right anterior quadrant magnetic resonance imaging (MRI) showed a benign lesion in the mesial aspect of the right occipital lobe. Simultaneous video monitoring and intracranial EEG with subdural strips recording from the right temporal and occipital lobes was undertaken. During one seizure, he had pronounced oroalimentary automatisms while holding a conversation with a technician, answering her questions, and explaining details of his seizures. Memory of this event was preserved. At seizure onset, spike activity was seen at the mesial occipital strips. At mid-seizure, high-voltage sharpened delta was seen throughout the right hemisphere. Left-sided scalp electrodes remained relatively uninvolved. The lesion, a dysembryoplastic neuroepithelial tumour was removed. Surgery was followed by abolition of seizures described. Because it is agreed that complex partial seizures require impaired consciousness, a history of automatisms with retained consciousness usually suggests nonepileptic attacks. This case suggests that automatisms in epileptic seizures can take place with minimal loss of consciousness, particularly if there is widespread but unilateral involvement. The need for a revision of the International Classification is suggested.     

Pilomotor seizures in mesial temporal lobe epilepsy: a case confirmed by intracranial EEG.

We report the first case in which intracranial EEG was used to confirm pilomotor seizures of mesial temporal origin. A 41-year-old man who had pilomotor seizures in the left arm in addition to complex partial seizures was admitted for presurgical evaluation. Intracranial EEG revealed ictal discharges in the left mesial temporal area during left pilomotor seizures. Moreover, electrical stimulation of the left mesial temporal area induced a habitual pilomotor seizure. After anteromesial temporal lobectomy, the patient became seizure-free. The localizing and lateralizing value of pilomotor seizures are discussed.     

Ictal vomiting in association with left temporal lobe seizures in a left hemisphere language-dominant patient

Ictal vomiting is considered a localizing sign indicating nondominant lateralization in patients with partial seizures of temporal lobe origin. We report a case of ictal vomiting associated with left temporal seizure activity in a left hemisphere language-dominant patient with a left mesial temporal glioma. Bilateral mesial temporal depth electrodes helped verify seizure lateralization. Surgery consisting of tumor resection and a left anterior temporal lobectomy and amygdalohippocampectomy resulted in freedom from seizures and episodes of vomiting. This case indicates that ictal vomiting can occur as a manifestation of left temporal onset seizures in left hemisphere-dominant patients.     

Ictal visual hallucination intermittent photic stimulation: using evaluation of the clinical findings, ictal EEG, ictal SPECT, and rCBF]

A 43-year-old, right-handed woman experienced right hand paresthesias and a visual field abnormality. We attributed her symptoms to psychiatric abnormalities, due to the presence of delusions and auditory hallucinations. Upon photostimulation, she experienced left visual field hallucinations and demonstrated slow waves on the right parieto-occipital regions. The clinical and electro-encephalographic findings suggested that these episodes were epileptic seizures originating from the right occipital region. Ictal fear appeared at the end of the seizure, reflecting the spread of seizure activity to the mesial temporal region. Ictal SPECT images showed hyper-perfusion in the right occipital region and left cerebellar cortex. rCBF in the occipital lobe was significantly asymmetrical. When we encounter an epileptic patient with psychosis who has a visual hallucination, we should consider the possibility of epileptic seizure originating from the occipital lobe.     

An unusual case of epilepsy exhibiting gelastic seizure, simple visual hallucination, and transient swelling of the left parieto-occipital region]

We report a 74-year-old man with gelastic seizure, simple visual hallucination, and adversive seizure. The patient described his visual hallucinations as "rotating light like a firefly" and "mimicking a stream". Brain CT scan showed a transient swelling as well as low density of a left parieto-occipital region. Electroencephalographic study revealed spikes and fast waves beginning at left occipital region. Although temporal lobe and hypothalamic lesions (especially hypothalamic hamartomas) are well known as origins of gelastic seizures, we could not find any report that described a series of occurrence of gelastic seizure and simple visual hallucination. Usually, simple visual hallucination is thought to occur in occipital lesion. In our case, it is possible that gelastic seizure and simple visual hallucination are related to the epileptic discharge from occipital lesion directly or indirectly. The reversible brain swelling with low density seen in the present case might be caused by cytotoxic edema due to status epilepticus.     

New-onset mesial temporal lobe epilepsy in a 90-year-old: clinical and EEG features

Although there is a peak in the incidence of epilepsy in the elderly compared with the general population, complex partial seizures represent less than 15% of the seizure types reported. We report on a 92-year-old woman with a 2-year history of daily complex partial seizures. Prolonged video/EEG recording showed bilateral anterior mesial temporal interictal spikes, which predominated on the left, and two typical seizures arising from the left temporal area. Cranial MRI scanning showed multiple lacunar infarcts without temporal lobe involvement or mesial temporal atrophy. Our case appears to be oldest patient in the literature with newly diagnosed mesial temporal lobe epilepsy confirmed by video/EEG recording.     

枕叶癫痫的术前定位

目的:通过回顾分析9例枕叶癫痫患者的临床资料,讨论枕叶癫痫的临床特征和致痫灶的术前定位。方法:应用临床发作症状评估、CT/MRI扫描、SPECT/PET检查、长程视频脑电图监测、颅内电极记录以及皮层脑电监测等方法综合定位枕叶癫痫患者的致痫灶。结果:视觉先兆及头或眼向一侧偏转是最常见的发作早期改变;发作形式以复杂部分性发作为主,也可以继发全面性发作。致痫灶位于枕叶内侧面2例,背外侧面7例,其中致痫灶位于枕叶与顶叶的临界区1例,枕叶与颞叶的临界区或累及颞叶后部4例。术后随访1年以上,按照Engel's术后效果分级:Ⅰ级5例,Ⅱ级2例,Ⅲ级1例,Ⅳ级1例。结论:视觉先兆的出现常常提示致痫灶位于枕叶,MRI检查以及发作初始期头和眼向一侧偏转等症状学特点均有较高的定侧定位价值。当其他方法定位致痫灶较困难时,应用颅内电极记录有助于定位。     

Analysis of the initial ictal phenomenon in patients with temporal lobe epilepsy

We aimed to assess the localizing value of the initial semiological element in temporal lobe epilepsy (TLE). Video-EEG-documented seizures of 97 adult TLE patients were studied in relation to seizure origin (left versus right; mesial versus extra-mesial). Strikingly, seizures with mesial onset started with very few ictal phenomena, while seizures of extra-mesial origin began with a larger variety of ictal elements. Furthermore, following noticeable distributions were observed for the mesial group: (i) aura was the most common initial ictal phenomenon in the total patient collective, occurring significantly more frequently in mesial than in extra-mesial seizure onset. Aura appeared most often in seizures of left mesial origin. (ii) Vocalization presented a trend towards mesial left seizure origin. (iii) Oral automatisms showed a trend towards mesial seizure origin. Following noticeable distribution was observed for the extra-mesial group: In patients without aura, restlessness as initial ictal phenomenon appeared exclusively in seizures of extra-mesial right origin. Finally, behavioral arrest showed a trend towards left-sided seizure origin. In conclusion, the initial ictal element may add useful information concerning differentiation of seizure onset in TLE.     

Epidermoid tumors of the temporal lobe as epileptogenic foci

Intracerebral epidermoid tumors of the temporal lobe are exceedingly rare. The vast majority of these slow-growing tumors remain clinically silent while only a select few are associated with overt symptomatology. We report two patients with epidermoid tumors whose unique location in the temporal lobe resulted in secondarily generalized seizures. Our first patient, a 19-year-old female, presented with a secondarily generalized tonic–clonic seizure and MRI revealed a superior temporal lobe lesion that was subsequently treated with an awake craniotomy and gross total resection. Our second patient was a 71-year-old male with a recent seizure history and known left temporal lobe lesion. The patient underwent craniotomy for gross total resection of the mass. In these two patients, we found that MRI proved diagnostic and surgical resection was curative. Our clinical experience and review of the literature indicate that gross total resection of these lesions confers control of localization-related epilepsy.     

Human "memories" can be evoked by stimulation of the lateral temporal cortex after ipsilateral medial temporal lobe resection

A patient with medically intractable seizures and mesial temporal sclerosis underwent a left anterior temporal lobectomy and amygdalohippocampectomy. After 4 months, his seizures recurred and a left temporal, subdural grid of electrodes was placed to localise his seizure focus. Stimulation through the grid evoked four distinct "memories", or experiential phenomena, despite absence of the ipsilateral medial temporal lobe. To our knowledge, this is the first documented case of experiential phenomena evoked by cortical stimulation in the absence of the ipsilateral medial temporal lobe.     

Concomitant bihemispheric cerebral ganglioglioma and hemangioma in an 18-month-old child: case report

This is a rare case of concomitant bihemispheric cerebral ganglioglioma and hemangioma in an 18-month-old child. There were two identical processes; a combination of ganglioglioma and hemangioma was located in the parieto-occipital region of both cerebral hemispheres. The child underwent diagnostic computed tomographic and magnetic resonance imaging examinations for seizures and right-sided facial palsy, which showed the bilateral presence of parieto-occipital, oval, well-demarcated, subcortically located processes. A biparieto-occipital craniotomy was carried out and two well-demarcated abnormalities were observed and both were removed in toto. Two years after the operation, the boy had normal psychomotor development and remained free from seizures with no therapy required. Repeat magnetic resonance imaging showed no tumor recurrence. Gangliogliomas are relatively uncommon tumors in childhood, located in the supratentorial compartment and usually associated with seizures. Histopathologic and immunohistochemical analysis of the two processes found in our patient showed them to be a ganglioglioma and a hemangioma, confirming the dysontogenetic origin of these alterations and the association between malformations and tumor growth. The combination of ganglioglioma and hemangioma, especially bilateral presentation of two such identical processes in a young child, is very rare and was not found in the recent literature.     

Magnetoencephalography and diffusion tensor imaging in gelastic seizures secondary to a cingulate gyrus lesion

Gelastic seizures are relatively uncommon and rarely observed secondary to frontal lobe lesions. This report presents magnetoencephalography (MEG) and diffusion tensor imaging (DTI) findings in an adolescent with gelastic seizures secondary to a left anterior cingulate gyrus lesion. Ictal scalp video EEG showed bilateral frontal 4 Hz theta discharges. Interictal EEG showed left fronto-temporal spikes or sharp waves. Interictal MEG showed spike sources over bilateral temporal regions. DTI and tractography delineated slightly shifted corpus callosum posterior to the lesion, unaffected uncinate and inferior longitudinal fasciculi. The patient became seizure free for 12 months after surgical excision of a pleomorphic xanthoastrocytoma in the left anterior cingulate region. In our patient, MEG and EEG did not localize the deep-seated epileptogenic zone. The combination of DTI and neurophysiologic studies, however, possibly disclosed neuronal connections within the epileptic network and indicated that epileptic discharges propagated via the uncinate fibers from the primary epileptogenic zone in the anterior cingulate region to the mesial temporal region in this case with gelastic seizures secondary to a cingulate lesion.     

Interictal and ictal activity recorded with subdural electrodes during preoperative evaluation for surgical treatment of epilepsy

A total of 94 subdural strip electrodes were implanted in 22 patients during preoperative EEG evaluation for surgery of epilepsy. Eighteen patients had temporal lobe seizure onset, three had frontal lobe seizure onset, and one had occipital lobe seizure onset. Most electrodes (total, 83) were localized over the temporal lobe cortex, but in four cases additional strip electrodes (total, 11) covered the frontal, parietal, and occipital lobe cortexes. The electrodes were left in place for up to 28 days. No complications occurred. Interictally, focal spiking was recorded subtemporally, mostly without being seen in electrodes recording from the lateral temporal cortex. In three patients studied with simultaneous subdural and sphenoidal wire electrodes, spiking recorded from subdural electrodes was often not seen in the sphenoidal recording. There were 151 seizures recorded (with or without simultaneous video monitoring). The mean number of seizures per patient was 6.7 (range, 0–21). The seizures were classified as having focal (80 seizures) or local (71 seizures) onset. It is concluded that subdural electrodes are safe and have a sufficient selectivity with regard to localization of interictal spiking and seizure onset in patients with mesial temporal epileptic lesions. In such cases, electrodes have to be placed subtemporally. Other cortical areas may also be explored with these electrodes.     

Occipital ganglioglioma in an older adult

Gangliogliomas are rare benign tumors of the central nervous system that typically involve the temporal lobe in younger patients. We present a 63-year-old man with an unusual occipital ganglioma with new seizures resolving after resection. A search of the literature revealed only three reports of occipital ganglioma in adults over 30 years old. Therefore, ganglioglioma of the occipital lobe in older patients is rare, but is a diagnostic consideration.     

A case with left mesial temporal lobe epilepsy characterized by abnormal massive salivation]

A 26-year-old woman was admitted because of a 12-year history of intractable epileptic seizures. Every seizure began with an upper abdominal discomfort, and often followed by massive salivation. Whenever the epileptic salivation happened, the patient lost consciousness, and sometimes she developed generalized convulsions. In terictal sphenoidal EEG recordings revealed abnormal discharges in the left mesial temporal region. MRI demonstrated left hippocampal atrophy. Since her seizures were medically intractable, left temporal lobectomy was performed. Neuropathologic examination revealed hippocampal sclerosis. After the operation, she became completely seizure-free with no episode of massive salivation. From the literature, epileptic salivation can originates from the rolandic area; fronto-orbital cortex & cingulate gyrus; insula & operculum; and mesial temporal structures. The abnormal massive salivation in our patient might be attributable to the activation of frontal limbic system triggered by hippocampal abnormal firing during the ictal period of TLE.     

Medically refractory epilepsy associated with temporal lobe ganglioglioma: characteristics and postoperative outcome

OBJECTIVES: To define the postoperative seizure outcome and its predictors in patients with ganglioglioma-related temporal lobe epilepsy (TLE). PATIENTS AND METHODS: We reviewed the pre- and post-surgical evaluation data of 23 patients with temporal lobe ganglioglioma, who had completed >or=1 year of postoperative follow-up. They comprised 4.9% of the patients with TLE and 67.6% of the tumoral TLE operated in a developing country epilepsy center during an 8-year period. RESULTS: Median age at surgery was 20 years; median duration of epilepsy prior to surgery was 9 years. Magnetic resonance imaging (MRI) revealed tumor in mesial temporal location in 18 patients (78.3%) and in the lateral location in 2; in the remaining 3, involved both mesial and lateral regions. EEG abnormalities were localized to the side of lesion in the majority. Mesial temporal lobe structures were included in the resection, if they were involved by the tumor; otherwise, lesionectomy alone was performed. During a median follow-up of 4 years, 19 (82.6%) patients were completely seizure-free. Epileptiform abnormalities persisting in the 1-year postoperative EEG predicted unfavorable seizure outcome. CONCLUSION: We emphasize that, in patients with temporal lobe ganglioglioma, when the seizures are medically refractory, surgery offers potential for cure of epilepsy in the majority.     

Suprasellar ganglioglioma presenting with diabetes insipidus in a young boy: a rare clinical presentation

Objective  

Gangliogliomas are rare tumors composed of an admixture of glial and neuronal components. These usually occur in young patients, who present with therapy-resistant seizures. Clinical presentation of ganglioglioma with diabetes insipidus is extremely rare with only one case reported earlier in the available literature. Due to this rarity, ganglioglioma is not considered in the differential diagnosis in a patient with diabetes insipidus.     

Intractable epilepsy secondary to cyclosporine toxicity in children undergoing allogeneic hematopoietic bone marrow transplantation

The long-term evolution to intractable epilepsy in children treated with cyclosporine administered for graft-versus-host-disease after hematopoietic stem cell transplantation was evaluated. In a group of 185 children treated with cyclosporine after bone marrow transplantation, 15 (8%) presented with acute seizures that were generalized in 7 and focal in 7 and had absence status in 1. Electroencephalography (EEG) and neuroimaging showed predominant abnormalities in the occipital regions. One patient died shortly after the seizure; in seven cases, seizures remitted, whereas relapses were observed in seven others. After the first year, seizures persisted chronically in four cases and evolved to intractable epilepsy. Focal temporal epilepsy was diagnosed in three cases, whereas in the fourth case, a multifocal epilepsy was observed. Magnetic resonance imaging (MRI) detected mesial temporal sclerosis in all of these cases. The risk factors associated with evolution to epilepsy included lower age at transplantation (3-5 years), more than one relapsing seizure in the first year after transplantation, and longer treatment with cyclosporine. Not only can cyclosporine cause acute central nervous system toxicity, it can also determine intractable epilepsy associated with mesial temporal sclerosis.