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PubMed2006

Hot water epilepsy with cerebral lesion: a report of five cases with cranial MRI findings.

Hot water epilepsy (HWE) is included in the reflex epilepsies. Although, in general, not common, HWE is concentrated in certain regions of the world. Different bathing habits and genetic factors may be responsible for the high incidence of HWE in these regions. However, the exact pathogenesis of HWE is not known. The facts that complex partial seizures are the most common clinical presentation and EEG recordings show an epileptic focus in the temporal lobe suggested the presence of a structural lesion in the temporal lobe. To our knowledge, however, there were no demonstrable structural changes on MRI and CT scans except in a few case reports. Here, we describe an additional five cases of HWE having an intracranial pathology, for example, hippocampal sclerosis, dysplasia, and a huge cystic lesion. We believe that investigations with new detailed neuroimaging techniques, in addition to experimental and clinical studies, might help us to understand the mechanism of this reflex epilepsy.

Adolescent↗
PubMed2004

Telephone-induced seizures: a new type of reflex epilepsy.

PURPOSE: To report a new form of reflex epilepsy in which the seizures are repeatedly and exclusively triggered by answering the telephone. METHODS: Three patients with a history of telephone-induced seizures were studied in detail by means of clinical, EEG, and neuroradiologic investigations. Intensive video-EEG monitoring to record the reflex seizures also was performed in all cases. RESULTS: The patients (two men, one woman, aged 21 to 30 years) had the onset during early adulthood of complex partial and secondarily generalized seizures exclusively triggered by answering the telephone. The seizures were stereotyped, with subjective auditory or vertiginous auras and inability to speak or understand the spoken voices. In one patient, a telephone-induced seizure arising from the dominant temporal lobe was recorded by means of video-EEG technique. In the interictal EEGs, temporal abnormalities were detected in all cases. The patients had a normal neurologic examination and normal magnetic resonance imaging or computed tomography scans. CONCLUSIONS: We suggest that telephone epilepsy is a previously unrecognized form of reflex epilepsy induced by a complex auditory stimulus involving the lateral temporal areas.

Adult↗
PubMed1983

Absence status manifested by compulsive masturbation.

Absence status manifested by compulsive masturbation occurred in a 41-year-old man with generalized epilepsy. Ictal simple and complex sexual manifestations during partial seizures in focal epilepsy are rare, but, to our knowledge, our report of this sexual automatism during absence status in generalized epilepsy is unique.

Adolescent↗
PubMed2001

Intractable epilepsy following radiosurgery for arteriovenous malformation.

Radiosurgery is often used to treat arteriovenous malformations (AVMs) located in deep brain locations. Most of these procedures are successful not only in obliterating the AVM but also in decreasing the frequency and severity of associated seizures. Although radiosurgery is occasionally associated with the development of easy-to-control seizures immediately postoperatively, there have been no reports of intractable epilepsy developing after radiosurgery. In this report, however, a case is presented in which a patient underwent gamma knife surgery (GKS) for an AVM, after which intractable epilepsy and mesial temporal sclerosis (MTS) gradually developed. A 37-year-old right-handed woman underwent GKS for a right mesial parietotemporooccipital AVM. One year later, the AVM had reduced in size, but the patient began to experience complex partial seizures (CPSs). These CPSs initially occurred at a frequency of one per month, but 6 months later they were occurring every other week. She also started having secondarily generalized tonic-clonic seizures (GTCSs) once per month. Over the next year the frequency of her seizures gradually increased to several CPSs per day and two to three GTCSs per week, despite treatment with various combinations of antiepileptic drugs. By this time her AVM had decreased to one half of its original size. Video-electroencephalography monitoring demonstrated that both the CPSs and GTCSs were arising from the right posterior quadrant. Magnetic resonance imaging revealed not only the presence of the right-sided AVM, but also right-sided MTS. The patient underwent surgical resection of the AVM and right temporal lobectomy. She has been free from seizure for longer than 1 year. Radiosurgery may be associated with intractable epilepsy and MTS.

Adult↗
PubMed1996

Mesial temporal lobe epilepsy in childhood.

To clarify the clinical picture of mesial temporal lobe epilepsy (MTLE) in childhood, we carried out a clinical, electroencephalographic, and neuroradiologic study of 19 patients. MTLE was noted in 19 (0.82%) of 2,319 epileptic patients with childhood onset. Three types of initial seizure were recognized: febrile convulsion, afebrile generalized convulsion, and complex partial seizure (CPS). As presumed causes, various prolonged convulsions (persisting for > 30 min) were found in 12 (63.2%) cases. Regardless of the presence of preceding convulsions (febrile or afebrile), the clinical course was not uniform, with CPS in the early period temporarily controlled in some cases and intractable from the early period in others. Unilateral hippocampal abnormalities were confirmed on magnetic resonance imaging (MRI) before the age of 5 years in two cases, suggesting that mesial temporal sclerosis (MTS) is formed within a relatively short period in some cases. Seizures were controlled for > 6 months in only two (10.5%) cases and persisted in 17. In four (21.1%) cases, surgical treatment was considered to be available.

Age Factors↗
PubMedDate not supplied

[Febrile convulsions and epilepsy].

We can divide the epilepsies connected to febrile convulsions (F.C.) in five groups: Early epileptic syndromes with generalized febrile and non febrile seizures. Early epileptic syndromes with polymorphic febrile and non febrile seizures. Generalized epilepsies occurring after F.C. Partial epilepsies (whether with elementary or complex fits) occurring after F.C. H.H.E. syndromes. The first two types of epilepsies are rather uncommon and mostly with poor prognosis (especially type 2). The epilepsies occurring after F.C. start at different ages and are characterized from various clinical and prognostical features. The most frequent type are the generalized forms, which present mostly a good evolution, as well as the partial forms with elementary fits. The partial ones with complex fits occur rather frequently and often evolue badly: in some of these cases we can have cerebral damage, as occurs in H.H.E. syndromes.

Child, Preschool↗
PubMed1997

Proton magnetic resonance spectroscopic imaging for discrimination of absence and complex partial seizures.

We performed proton magnetic resonance spectroscopic imaging of the temporal lobes between, during, and soon after nonconvulsive seizures in 20 patients with documented temporal lobe epilepsy, 5 patients with primary generalized epilepsy, and 2 patients with secondary generalized epilepsy. Our objective was to determine whether there were metabolic changes observable by magnetic resonance spectroscopic imaging during seizures and whether these changes were specific for focal or generalized nonconvulsive seizures. We found a significant increase in lactate to creatine plus phosphocreatine (lactate/creatine) values, reflecting an imbalance in energy supply and demand or an adaptation in response to ictal neuronal discharges, during and soon after complex partial seizures, but not during or soon after absence seizures associated with generalized epilepsy. In patients with temporal lobe epilepsy, the N-acetylaspartate resonance relative to creatine plus phosphocreatine was low in one or both temporal lobes, indicating neuronal loss or damage. This was not observed in patients with primary generalized epilepsy. The regions with abnormal lactate/creatine and N-acetylaspartate/creatine values corresponded to the epileptogenic focus as defined by clinical-electroencephalographic investigation. There was no change in the N-acetylaspartate/creatine values in the temporal lobes between the interictal, ictal, or postictal states. We conclude that (1) partial seizures are associated with abnormally high lactate levels, but absence seizures are not, and (2) no short-term changes of N-acetylaspartate occur during or soon after complex partial seizures or absence seizures. These findings may be related to the lack of postictal confusion in patients with absence seizures, as well as with the more benign course of primary generalized epilepsy with nonconvulsive attacks.

Adolescent↗
PubMed2001

Antiepileptic drug withdrawal in patients with temporal lobe epilepsy undergoing presurgical video-EEG monitoring.

PURPOSE: To investigate antiepileptic drug (AED) withdrawal during video-EEG monitoring in adult patients with temporal lobe epilepsy (TLE). METHODS: Between 1995 and 1997, 102 consecutive patients with refractory TLE were admitted to the epilepsy monitoring unit for presurgical evaluation. Patients were monitored with ongoing AEDs being rapidly decreased and discontinued in 4-6 days. The monitoring was continued until sufficient numbers of seizures were recorded. Serum AED levels were checked at admission and after the first complex partial seizure (CPS). RESULTS: In all, 89 patients had 429 CPSs (mean, 4.8 per patient), including 156 (36.4%) secondarily generalized. A mean of 153.8 h (16-451 h) was required for completing the monitoring in each patient. Forty-three (48.3%) patients experienced seizure clusters, and eight (9.0%) had generalized seizures that had never occurred or had been absent for years. However, none evolved to status epilepticus. Carbamazepine was the most commonly used AED in 71.9% of patients, followed by valproate and phenytoin. When the first CPS occurred, mean 77.2 h since the beginning of the monitoring, serum levels of these three AEDs were mostly subtherapeutic rather than minimal. CONCLUSIONS: Acute AED withdrawal effectively provoked seizures in TLE patients undergoing presurgical video-EEG monitoring. However, nearly 50% of patients had seizure clusters or secondarily generalized seizures. Serum AED levels were mostly subtherapeutic when the first CPS occurred.

Adolescent↗
PubMed1996

Significance of head turn sequences in temporal lobe onset seizures.

We studied head turning in 239 complex partial seizures with or without generalization, in 32 patients with unilateral temporal lobe epilepsy. Head turns occurred in 73% of seizures that did not evolve to focal jerking or secondary generalization, and in all 41 seizures that secondarily generalized. In seizures without focal jerking or secondary generalization the most common pattern was that of single head turns (70%) which were ipsilateral to the focus in 94%. The next most common pattern was that of two or more head turns, with the first two turns in the same direction (19%), always ipsilateral to the focus. In seizures with secondary generalization, the most common sequence was that of two head turns contralateral to each other (59% of seizures). The first was always ipsilateral to the focus, associated with dystonic posturing in 96%, and was not tonic in character. The second was always contralateral, was tonic in character, and was still present within five seconds of secondary generalization or focal jerking. Our results suggest different patterns and sequences of head turning temporal lobe complex partial seizures without, and those with focal jerking or secondary generalization. Some sequences have powerful lateralizing value that can complement other lateralizing features.

Behavior↗
PubMed2000

Surgical management of intractable epilepsy associated with cerebral neurocytoma.

Neuronal neoplasms of the CNS constitute a rarely encountered group of tumors. This report concerns the surgical management of seizures encountered in four cases (ranging from 2 to 10 years-of-age at onset; consisting of two males and two females) of a recently recognized morphologically unique tumor, called 'cerebral neurocytoma'. All patients were associated solely with intractable complex partial seizures. The tumor involved the temporal lobe in two cases, and the frontal in two. Magnetoencephalography (MEG) clearly demonstrated an accumulation of equivalent current dipoles originating from the interictal spikes on the cortex around the tumor. On intra-operative electrocorticography (ECoG), the epileptogenic zone was topographically distinct from the region of the tumor. No definite ECoG activities were observed at the tumor site, although this tumor did consist of small mature neuronal cells. Either a complete or a subtotal resection of the tumor and the epileptogenic cortex was performed and, post-operatively, universal freedom from seizures was demonstrated in all patients. A histological examination of the epileptogenic cortex revealed the presence of minute cortical dysplasia or tumor involvement in the hippocampus. A resection of the epileptogenic cortex along with the tumor was thus found to improve the seizure outcome in patients with neurocytoma-associated epilepsy without inducing any identifiable neurological deficits attributable to the incremental resection.

Adult↗
PubMed1999

Spitting automatism in complex partial seizures: a nondominant temporal localizing sign?

PURPOSE: Spitting as an ictal automatism has been rarely reported. We aimed to establish its potential lateralizing and localizing significance. METHODS: Review of patients undergoing surgery for intractable epilepsy at two comprehensive epilepsy centers. RESULTS: Five patients were found who had spitting as a stereotyped automatism of their complex partial seizures. All had evidence of right temporal ictal onset and underwent resective surgery. Two had tumors; one, a cavernous angioma; one, hippocampal gliosis, and one, hippocampal sclerosis. We found no instances of ictal spitting in patients with left hemisphere onset. CONCLUSIONS: Spitting as an automatism in complex partial seizures, although uncommon, may be a localizing sign to the nondominant temporal lobe.

Adult↗
PubMed2005

Depression and anxiety disorders in pediatric epilepsy.

PURPOSE: This study examined affective disorders, anxiety disorders, and suicidality in children with epilepsy and their association with seizure-related, cognitive, linguistic, family history, social competence, and demographic variables. METHODS: A structured psychiatric interview, mood self-report scales, as well as cognitive and language testing were administered to 100 children with complex partial seizures (CPSs), 71 children with childhood absence epilepsy (CAE), and 93 normal children, aged 5 to 16 years. Parents provided behavioral information on each child through a structured psychiatric interview and behavior checklist. RESULTS: Significantly more patients had affective and anxiety disorder diagnoses (33%) as well as suicidal ideation (20%) than did the normal group, but none had made a suicide attempt. Anxiety disorder was the most frequent diagnosis among the patients with a diagnosis of affective or anxiety disorders, and combined affective/anxiety and disruptive disorder diagnoses, in those with suicidal ideation. Only 33% received some form of mental health service. Age, verbal IQ, school problems, and seizure type were related to the presence of a diagnosis of affective or anxiety disorder, and duration of illness, to suicidal ideation. CONCLUSIONS: These findings together with the high rate of unmet mental health underscore the importance of early detection and treatment of anxiety disorders and suicidal ideation children with CPSs and CAE.

Adolescent↗
PubMed2003

Intractable temporal lobe epilepsy with rare spikes is less severe than with frequent spikes.

OBJECTIVE: To analyze clinical, electrophysiologic, and neuroradiologic characteristics of a group of patients with nonlesional intractable temporal lobe epilepsy (TLE) and rare or absent interictal epileptiform abnormalities (IEA). METHODS: Between 1990 and 2000, 31 patients (11 men; mean +/- SD age 34.3 +/- 11.7 years) with nonlesional intractable TLE were consecutively selected on the basis of the absence or paucity of IEA (<1/h) on serial scalp EEG recording; these were defined as "oligospikers." The clinical and laboratory characteristics of oligospikers were compared with those of a group of 27 age-matched control subjects (10 men; mean +/- SD age 38.5 +/- 11 years), randomly selected from a pool of patients with nonlesional TLE with frequent IEA. RESULTS: Oligospikers showed a later age at seizure onset (mean +/- SD 19.1 +/- 14.4 versus 10.2 +/- 7.4 years; p = 0.004), lower monthly frequency of complex partial seizures (median 6 versus 12; p = 0.035), lower incidence of secondarily generalized tonic-clonic seizures (10 versus 81%; p < 0.001), and no status epilepticus (0 versus 22%) than control subjects. Also, hippocampal atrophy (HA) was less commonly found in oligospikers (55 versus 96%; p = 0.001). However, there were no differences between the two groups in the frequency of family history of epilepsy, risk factors, febrile convulsions, and type of medication. Twenty-three (74%) oligospikers and 25 (93%) control patients underwent either a selective amygdalohippocampectomy or corticoamygdalohippocampectomy. Excellent surgical outcome (Engel's Class Ia) was found in 14 of 23 (61%) oligospikers and 17 of 25 (67%) control patients. CONCLUSIONS: This study identified a subgroup of patients with nonlesional intractable TLE with no or few IEA. Oligospikers have a later age at seizure onset, less frequent and less severe seizures, besides a lower incidence of HA. The similarity of etiologic factors compared with patients with frequent IEA suggests that the rarity of spikes could reflect a disease not really distinct but less severe, even though still intractable and incapacitating enough to consider surgery. In spite of the absence or paucity of IEA, oligospikers have excellent surgical outcome.

Adult↗
PubMed2000

Temporal lobe epilepsy with sensory aura: interictal glucose hypometabolism.

Patients with mesial temporal lobe epilepsy (mTLE) exhibit marked depressions of the regional cerebral glucose metabolism (rCMRGlu) in the mesiotemporal region. We hypothesised that patients with temporal lobe epilepsy (TLE) who have a bilateral somatosensory or acoustic ( = temporolateral/SII-) aura can be differentiated from mTLE by rCMRGlu depressions primarily involving temporo-perisylvian locations. We therefore used this ictal semiology as a clinical criterion to define a subgroup of such patients and measured the rCMRGlu in 16 patients with TLE as evident from interictal and ictal EEG-video monitoring. Clinically, they presented with medically refractory complex partial seizures and were subjected to presurgical evaluation. The pattern of the interictal rCMRGlu in the TLE patients was different from that observed in patients with mTLE and showed significant depressions ipsilateral to the epileptic focus in mesial temporal and lateral temporal regions but spared the thalamus. The neocortical metabolic depressions were spatially more extended in right than in left TLE patients. Magnetic resonance images (MRI) were either normal (n = 5) or revealed unilateral or bilateral hippocampal atrophy/sclerosis (n = 7), or temporal or extratemporal focal cortical dysplasia (n = 4). The selected TLE patients presented here comprise a heterogeneous group showing most pronounced metabolic depressions in the lateral temporal cortex. Thus, our data suggest that non-invasive metabolic imaging can assist in identifying the neocortical symptomatogenic zone in putative temporo-perisylvian lobe epilepsy.

Abdomen↗
PubMed1997

A clinically recognizable neuronal migration disorder: congenital bilateral perisylvian syndrome. Case report with long-term clinical and EEG follow-up.

Congenital bilateral perisylvian syndrome (CBPS) is a recently described, neuronal migration disorder, characterized by pseudobulbar palsy, epilepsy and mental retardation and bilateral perisylvian dysplasia. A 15-year-old boy was diagnosed with CBPS according to the typical clinical, and magnetic resonance imaging (MRI) features. The patient was suffering from atypical absence seizures, repeating daily in spite of antiepileptic drug therapy, since age 7 years. He had also experienced rare generalized tonic-clonic seizures and complex partial seizures. Neurological examination showed severe restriction of tongue movements, severe dysarthria, dysphagia, facial diplegia, mild pyramidal signs and moderate mental retardation. A computed tomographic (CT) scan demonstrated bilateral perisylvian enlargement. The diagnosis was corrected with MRI after six years. Frequent irregular generalized spike and wave abnormalities and focal sharp and slow waves over the posterior regions of both hemispheres were shown by electroencephalograms (EEG). The patient was treated with Na-Valproate, carbamazepine and lamotrigine but did now show any significant change in seizure frequency in the eight-year follow-up period. Intractable seizures, mental retardation and particularly congenital pseudobulbar palsy suggest this congenital entity. Those patients who exhibit these typically clinical features, must have MRI.

Adolescent↗
PubMed2003

Anticonvulsant properties of acetone, a brain ketone elevated by the ketogenic diet.

The ketogenic diet (KD), a treatment for drug-resistant epilepsy, elevates brain acetone. Acetone has been shown to suppress experimental seizures. Whether elevation of acetone is the basis of the anticonvulsant effects of the KD and whether acetone, like the KD, antagonizes many different types of seizures, however, is unknown. This study investigated the spectrum of the anticonvulsant effects of acetone in animal seizure models. Rats were injected with acetone intraperitoneally. Dose-response effects were measured in four different models: (1) the maximal electroshock test, which models human tonic-clonic seizures; (2) the subcutaneous pentylenetetrazole test, which models human typical absence seizures; (3) the amygdala kindling test, which models human complex partial seizures with secondary generalization; and (4) the AY-9944 test, which models chronic atypical absence seizures, a component of the Lennox-Gastaut syndrome. Acetone suppressed seizures in all of the models, with the following ED(50)'s (expressed in mmol/kg): maximal electroshock, 6.6; pentylenetetrazole, 9.7; generalized kindled seizures, 13.1; focal kindled seizures, 26.5; AY-9944, 4.0. Acetone appears to have a broad spectrum of anticonvulsant effects. These effects parallel the effects of the KD. Elevation of brain acetone therefore may account for the efficacy of the KD in intractable epilepsy.

Acetone↗
PubMed1986

Excitotoxic mechanisms of epileptic brain damage.

It is well established that the putative excitatory neurotransmitters, glutamate (Glu) and aspartate (Asp), are neurotoxins that have the potential of destroying central neurons by an excitatory mechanism. Kainic acid (KA), a rigid structural analog of Glu, powerfully reproduces the excitatory neurotoxic (excitotoxic) action of Glu on central neurons and, in addition, causes sustained limbic seizures and a pattern of seizure-linked brain damage in rats that closely resembles that observed in human epilepsy. In the course of studying the seizure-related brain damage syndrome induced by KA, we observed that a similar type of brain damage occurs as a consequence of sustained seizure activity induced by any of a variety of methods. These included intraamygdaloid or supradural administration of known convulsants such as bicuculline, picrotoxin and folic acid, or systemic administration of lithium and cholinergic agonists or cholinesterase inhibitors that have not commonly been viewed as convulsants. We have further observed that this type of brain damage can be reproduced in the hippocampus by persistent electrical stimulation of the perforant path, a major excitatory input to the hippocampus that is thought to use Glu as transmitter. It is a common feature of all such neurotoxic processes that the acute cytopathology resembles the excitotoxic type of damage induced by Glu or Asp, which is acute swelling of dendrites and vacuolar degeneration of neuronal soma, without acute changes in axons or axon terminals. We have found that the seizure-brain damage syndrome induced by cholinergic agents can be prevented by pretreatment with atropine and that the syndrome induced by any of the above methods, cholinergic or noncholinergic, can be either prevented or aborted respectively by either pre-or posttreatment with diazepam. Our findings in experimental animals may be summarized in terms of their potential relevance to human epilepsy as follows. Sustained complex partial seizure activity consistently results in cellular damage if allowed to continue for longer than 1 hr. Hippocampal, or Ammon's horn, sclerosis is the primary pathological result. It may be a priority goal, therefore, in the management of human epilepsy to control such seizure activity within very narrow limits. This proposal is discussed in terms of three major transmitter systems that may be involved; cholinergic, GABAergic, and glutamergic/aspartergic. The cholinergic system may play a role in generating or maintaining this type of seizure activity, and anticholinergics may protect against it provided they are given prior to commencement of behavioral seizures.(ABSTRACT TRUNCATED AT 400 WORDS)

Administration, Topical↗
PubMed1997

Stereotactic amygdalohippocampotomy and mesial temporal spikes.

PURPOSE: To investigate the mechanism of mesial temporal spike generation, we sought to determine whether amygdalohippocampotomy by radiofrequency lesions altered spike rates in patients with medically intractable temporal lobe epilepsy. METHODS: The subjects were 14 patients whose ictal semiology, ictal and interictal EEGs, and neuropsychological profile were compatible with seizure origin from MRI-demonstrated unilateral mesial temporal sclerosis. Mesial temporal spikes were recorded by a multicontact electrode line stereotactically placed in the temporal horn of the lateral ventricle. A recording and lesioning electrode was also stereotactically advanced to multiple amygdala and hippocampal targets. Several confluent (9 patients) or discrete (5 patients) radiofrequency lesions were made in the amygdala and anterior hippocampus. Scalp and invasive recordings were performed before lesioning, between each lesioning process, and for 48 postoperative h. RESULTS: As compared to prelesion recordings, no consistent change in anterior and total mesial temporal spike rates occurred intraoperatively or postoperatively. Postlesion: prelesion spike ratios failed to correlate with effectiveness of lesionectomy for seizure control. In contrast, complex partial seizures improved in 13 of 14 patients. CONCLUSIONS: Amygdala and hippocampal destructive lesions had no consistent effect on meisal temporal spike quantity, but reduced temporal lobe CPS in 13 of 14 patients. This dichotomy suggests that CPS may result from concerted epileptogenesis of the hippocampus, entorhinal cortex, and possibly the amygdala, while the entorhinal cortex alone can produce interictal spikes.

Adolescent↗