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Complex partial status epilepticus in childhood.

We report 5 pediatric patients (2 male, 3 female; age range: 4-8 years) with complex partial status epilepticus (CPSE). Four patients had previous illnesses and mild motor or mental retardation. In 2 patients, CPSE was induced by inappropriate management or selection of antiepileptic drugs. Clinical features varied and automatisms were observed in 3 patients. In 1 patient, decreased physical tone with syncope and impaired consciousness with amaurosis were observed. The episodes of CPSE were continuous in 3 patients and recurrent in 2 patients. In 4 patients, ictal electroencephalographic (EEG) findings, including video-EEG analyses of 3 patients, demonstrated persistent focal epileptic features. Intravenous diazepam abolished CPSE in 3 patients with brief periods of definite EEG localizations remaining. In 4 patients, seizure prognoses were favorable after appropriate treatments; in 1 patient, seizures were intractable even after antiepileptic drug administration.

Anticonvulsants↗

Vigabatrin in the treatment of complex partial seizures.

We report the effect of vigabatrin on seizure frequency in 13 severely drug-resistant patients with intractable complex partial seizures (CPS) with or without secondary generalization. Patients were followed for a 3-month period before vigabatrin administration to establish a 'baseline'. Six patients became seizure free for 2-3 weeks immediately after starting vigabatrin. In seven patients a transient (4-6 weeks) increase in seizures above baseline occurred, which was attenuated by vigabatrin dose increments. After 3 months, the mean baseline CPS frequency was reduced from 7.75 +/- 1.18 (median 8, range 2.6-16) to 2.77 +/- 0.7 (median 1, range 0-7). At 6 months a > 50% improvement remained in seven patients. After 12 or more months CPS frequency returned to baseline in four patients, improved (by 25-62.5%) in four and deteriorated in three. One patient who was seizure free lost control at 16 months. Other effects were drowsiness (3), weight increase (3), diarrhoea (1), depression (2) and mood elevation (2). Four patients discontinued vigabatrin; one because of severe depression, three owing to lack of efficacy. Three patients have undergone and two are awaiting neurosurgery for their epilepsy. Thus, CPS frequency progressively deteriorated toward baseline in all patients, however, secondary generalizations were abolished in four and reduced in two.

Adolescent↗

Neuropsychiatric sequelae of cerebral malaria in Vietnam veterans.

Approximately 250,000 Vietnam veterans suffered cerebral malaria, an illness that often results in damage to subcortical white matter and fronto-temporal areas of neocortex. Case reports dating back 2500 years indicate that survivors of cerebral malaria show depression, poor memory, personality change, and irritability/violence. The purpose of the present study was to compare the neuropsychiatric status of Vietnam veterans who had suffered cerebral malaria in the remote past (i.e., 1966 to 1969) with that of Vietnam veterans wounded in combat who had not suffered malaria or other neurological conditions. Findings indicate that cerebral malaria results in multiple, major, substantially underappreciated neuropsychiatric symptoms in Vietnam veterans, including poor dichotic listening, "personality change," depression, and, in some cases, partial seizure-like symptoms. Findings strongly suggest that history of malaria should be considered in any medical, psychological, or psychiatric workup of a Vietnam War veteran because a positive response could result in substantial changes in diagnosis and treatment.

Adult↗

Unruptured aneurysm of the middle cerebral artery presenting with psychomotor seizures: case study and review of the literature.

An intracranial aneurysm would be low on the differential diagnosis of a patient presenting with behavioral or emotional changes. Nonetheless, complex partial seizures (CPS) may cause such symptoms and result from an unruptured intracranial aneurysm. Failure to diagnose and treat this condition in a timely manner increases the patient's risk of catastrophic aneurysmal rupture. This report describes a 55-year-old woman who presented following two CPS which began with the perception of a strange smell and culminated in a brief loss of consciousness. She had no history of seizure disorder or recent trauma. Magnetic resonance imaging (MRI) revealed a space-occupying lesion over the right temporal lobe near the amygdala. Magnetic resonance angiography (MRA) confirmed a 1.5-cm right middle cerebral artery aneurysm, with a dome projecting toward the amygdalohippocampal region. Following surgical ablation, the patient's CPS were well controlled. A review of the literature is performed on this unusual etiology and management strategies are discussed.

Amygdala↗

Valproate-associated carnitine deficiency and malignant cerebral edema in the absence of hepatic failure.

We describe a 27-year-old woman who developed encephalopathy and cerebral edema during treatment of refractory complex partial seizures that included acute administration of valproate (VPA) at a dosage of 35 mg/kg per day. Multiple random VPA levels were within therapeutic range, and results of liver function studies did not show evidence of hepatic failure. Cerebral computerized tomography (CT) showed evidence of massive cerebral edema with central herniation. Just prior to death, plasma levels of free and acyl carnitines were markedly decreased. Analysis of urinary organic acids showed increased excretion of lactate, but a normal distribution of VPA metabolites. Carnitine deficiency may predispose patients to the development of coma and life-threatening cerebral edema associated with acute administration of VPA, even in the absence of concomitant hepatic failure. We suggest specific guidelines for the evaluation and management of altered consciousness in patients with seizures receiving VPA.

Adult↗

Developmental plasticity after right hemispherectomy in an epileptic adolescent with early brain injury.

OBJECTIVES: The authors present the case of an adolescent affected with refractory epilepsy due to a neonatal ischemic infarction of the right medial cerebral artery. Hemiplegic since the first months of life, she began presenting motor partial seizures associated with drop attacks at 4.5 years; these were initially well controlled by antiepileptic drugs, but at 10 years seizures appeared again and became refractory. Thus, at 14 years and 10 months, she was submitted to a right hemispherectomy that made her rapidly seizure free. In the post-surgical follow-up lasting 5 years, neuropsychological serial assessments showed an impressive progressive improvement of cognitive skills, namely, visuospatial abilities. This case seems to challenge the widely spread feeling that functional catch-up in brain-injured children could only occur early in life. In effect, the astonishing recovery especially of visuospatial skills in our case occurred in adolescence after a late surgical intervention of right hemispherectomy. METHODS: Different neuropsychological aspects are discussed. The reorganisation process recovered the spatial and linguistic abilities as well as the verbal and visuospatial memory; however, there was a persistent impairment of complex spatial and perceptual skills as well as recall abilities. Despite the deficit of complex visual stimuli processing, the patient showed a good performance in the recognition of unknown faces. CONCLUSIONS: Probably, the absence of seizures in the first 4 years of life could have allowed a generally adequate compensatory reorganisation, successively masked by the persistent and diffuse epileptic disorder. The seizure control produced by surgery eventually made evident the effectiveness of the brain reorganisation.

Adolescent↗

Low levels of somatostatin-like immunoreactivity in neocortex resected from presumed seizure foci in epileptic patients.

The concentration of somatostatin-like immunoreactivity (SS-LI) was determined by radioimmunoassay in neocortical tissue resected from 20 patients with pharmacologically intractable complex partial seizures. Most resections included either the anterior temporal pole neocortex (15 cases) or cingulate gyrus neocortex (3 cases). The concentration of SS-LI was lowest in cortical tissue immediately adjacent to cortical tumors. Preoperative electrical recordings suggested that this tissue was the seizure focus. In vitro recordings showed that this tissue also exhibited abnormal hyperexcitable synaptic responses. Higher levels of SS-LI, similar to normal values previously reported in human cortex, were present in non-focal temporal neocortical tissue (resected from patients in whom the seizure focus was in the ipsilateral hippocampus) in which no hyperexcitable synaptic activity was present in vitro. The functional loss of inhibitory transmitters suggested by the low SS-LI levels might provide a theoretical basis for the hyperexcitability observed in vivo and in vitro.

Cerebral Cortex↗

Fast improvement of verbal memory function after left temporal tumour resection.

CASE REPORT: A 7-y-old, right-handed girl presented at our clinic with complex partial seizures, behavioural problems and word-finding difficulties. Clinical examination was normal, but electroencephalogram revealed bilateral epileptic discharges in the temporal and paracentral leads. Magnetic resonance imaging showed an intracerebral mass in the left mesial temporal lobe in the areas of the gyrus temporooccipitalis medialis and gyrus parahippocampalis. Neuropsychological testing brought to light an above-average general intelligence, but the girl performed below average in verbal memory tests and average in figural memory tests. RESULTS: The tumour was completely removed by surgery. Neuropsychological testing 3 and 11 wk after tumour resection demonstrated above-average verbal learning and memory abilities and improved visual memory. The emotional disturbances, seizures and electroencephalogram abnormalities disappeared completely during the postoperative follow-up. CONCLUSION: After resection of a left temporal brain tumour, the patient was free of seizures, and neuropsychological functions recovered completely within weeks.

Brain Neoplasms↗

[Complicated benign partial epilepsy].

OBJECTIVE: We review the complicated forms of different types of idiopathic benign partial epilepsy pointing out their relation to continuous point and wave tracings in slow sleep (POCS), the epileptic opercular syndrome and the Landau Kleffner syndrome. We insist on the role of some anti epileptic drugs in triggering the problem and withdrawal of these drugs as the most important aspect of the treatment of these complications replacing them by CLB in all cases. DEVELOPMENT: We describe various clinical cases which illustrate the problem

Anticonvulsants↗

Trimethylaminuria associated with seizures and behavioural disturbance: a case report.

A 16-year-old left-handed male is presented with a history of seizures associated with a fish-like odour and behavioural disturbances thought to be related to trimethylaminuria. His seizures were complex-partial (cursive) seizures and started at the age of 18 months. They occurred in the context of discrete episodes several times per year. The episodes would start with a fish-like odour, followed by seizures occurring in clusters and behavioural disturbance consisting of agitation, mixed affective symptoms, auditory hallucinations and delusions. A urinary assay of trimethylamine (TMA) was elevated, confirming the diagnosis of trimethylaminuria in this patient. He was treated with a choline-restricted diet with resolution of his symptoms. The occurrence of seizures and psychiatric disturbance in this patient was thought secondary to his trimethylaminuria due to the temporal relationship of his seizures and psychiatric disturbance with the odour and his response to treatment. The possible relationship of trimethylaminuria to seizures and to psychiatric disturbance are discussed and a review of the literature presented.

Adolescent↗

Single photon imaging computed tomography (SPECT) for localization of epileptogenic focus in patients with intractable complex partial seizures.

Interictal and ictal HIPDM-SPECT brain scans were obtained in 38 patients who eventually underwent temporal lobectomy for treatment of medically intractable complex partial seizures (CPS). Interictal studies revealed decreased regional cerebral perfusion (rCP) in the temporal lobe corresponding to the eventual site of surgery in 76% of the patients. Similarly, ictal studies demonstrated increased rCP in 91% of the patients. Of the 33 patients who had both interictal and ictal SPECT studies, 23 (70%) showed increased rCP in the ictal state and decreased rCP in the interictal state in the same temporal lobe which was subsequently removed. The SPECT scans were particularly helpful in providing confirmatory evidence for localization and in deciding to undertake temporal lobectomy in 16 patients whose EEG studies had provided less secure localization. Our experience suggests that interictal and ictal SPECT brain imaging can be easily obtained and provides reliable localizing information in the epileptic patients being considered for temporal lobectomy.

Adolescent↗

Partial seizures associated with cisplatin administration: a case report.

A 49-year-old man was treated with cisplatin and pirarubicin for tongue cancer. After the second course of chemotherapy, partial seizures including transient motor aphasia, tonic finger movement, and loss of consciousness were observed. The EEG showed frequent diffuse (multiple) spike and slow wave discharges. Following the administration of carbamazepine and diazepam, no seizures occurred and no paroxysmal discharges were observed or EEGs. We conclude that carbamazepine and diazepam administration was effective.

Antibiotics, Antineoplastic↗

Serum prolactin levels after seizure and syncopal attacks.

Loss of consciousness and falling are the key features of syncope. Common accompaniments include tonic and myoclonic muscle activity, eye deviations, automatisms, vocalizations and hallucinations that may render the distinction from epileptic seizures difficult. The frequently increased levels of serum prolactin (SPRL) were observed immediately after generalized and complex partial seizures. Presumably, the hormone release is caused by the propagation of epileptic activity, usually from the temporal lobe to the hypothalamic pituitary axis. Numerous reports have demonstrated that the post-ictal SPRL level may be used to differentiate between epileptic and syncopal, non-epileptic attacks. In order to confirm the hypothesis, the SPRL levels were measured in patients with complex partial seizures (CPS) and patients with vaso-vagal syncopal attacks (VVS). The SPRL levels were prospectively measured for each patient as soon as possible after the event (within 1 hour), then 1 hour after the first determination and finally blood was sampled 24 hours later. During the study period (18 months), 18 patients with CPS and 15 patients with VVS were investigated in total. The mean values of SPRL levels in both groups were increased immediately after the event (CPS group: 1142 +/- 305 mIU/l; VVS group: 874 +/- 208 mIU/l). The elevated SPRL levels were found in 14 (78%) patients immediately after CPS and in 9 (60%) patients immediately after VVS. After examining the results of the present study we conclude that the elevated serum prolactin level after an epileptic attack is of no significant value in differential diagnosis between epileptic and vaso-vagal syncopal attacks.

Adult↗

MRI evidence for the involvement of basal ganglia in epileptic seizures: an hypothesis.

Recent clinical and experimental studies have suggested that the basal ganglia are involved in epileptic seizures as a propagation pathway or as a remote inhibitory control circuit. The present case report may provide additional evidence from post-ictal magnetic resonance imaging (MRI) supporting this hypothesis. A healthy 13 year-old boy was admitted for a complex partial status epilepticus. MRI, performed one week later, revealed bilateral T2 hyperintense signals in the striata and a left temporal arachnoid cyst. Left temporal slow waves were noted on EEG recording. No obvious metabolic alterations were identified. During the next six years of follow-up, no seizure occurred and striatal alterations progressively disappeared. The clinical characteristics of the seizures, EEG slow waves, and probably the presence of an arachnoid cyst suggest that seizures originated from the left temporal lobe. The long-lasting MRI changes suggest that bilateral striatal alterations may have been secondary to an inflammatory process, which in turn could have disrupted a striatal inhibitory control over seizures. On the basis of these arguments, we speculate involvement of basal ganglia in epileptic seizures, as a part of a modulatory control system over seizures rather than a propagation pathway. Future reports will support or invalidate our hypothesis.

Adolescent↗

Level and contents of consciousness in connection with partial epileptic seizures.

The present study introduces concepts and methods that can be used in the systematic psychological study of a seizure, to gain more insight into the seizure as it is experienced by the patient and the significant other. Fourteen patients reported 40 descriptions of their subjective experiences during complex partial seizures. We analyzed the descriptions with respect to the temporal progression of the seizure and the level and contents of consciousness. There were three main findings: (1). We identified an impairment of the voluntary control of attention ("forced attention") that seems to characterize the early stages of the seizure in all patients. (2). Although most patients reported the total absence of consciousness, we identified a subgroup of patients with a fluctuating level of consciousness during the seizure. (3). The patients who reported some contents of consciousness during the seizure were found to usually experience internal mental images rather than other contents of consciousness (e.g., sensations or perceptions). We propose that use of a qualitative methodology for the psychological assessment of seizures could lead to a better understanding of seizures as experienced from the patient's perspective and thereby to improvements in the treatment of seizures.

Adult↗

Kynurenine pathway metabolites in cerebrospinal fluid and serum in complex partial seizures.

The kynurenine pathway metabolites, quinolinic acid (QUIN) and L-kynurenine are convulsants, whereas kynurenic acid (KYNA) is an antagonist of excitatory amino acid receptors. Imbalances in the concentrations of these metabolites have been implicated in the etiology of human seizure disorders. In the present study, L-kynurenine and QUIN concentrations in both cerebrospinal fluid (CSF) and serum were reduced in patients with intractable complex partial seizures (CPS) in both the postictal period (15-75 min after a seizure) and the interictal period (absence of seizure for > 24 h) as compared with neurologically normal control subjects. Linear regression analyses and analysis of covariance showed that the reductions in serum QUIN and L-kynurenine were correlated to blood antiepileptic medication. L-Tryptophan (L-TRP) levels also tended to be lower in both CSF and serum of the seizure patients. CSF KYNA and serum 3-hydroxykynurenine concentrations were not affected in seizure patients, whereas serum levels of KYNA were reduced. 3-Hydroxykynurenine was not detected in the CSF of either control or seizure patients. The results do not support a role for a generalized reduction in KYNA concentrations or an increased ratio of QUIN:KYNA, or increases in CSF L-kynurenine in initiation and maintenance of intractable CPS humans.

Adolescent↗

Decreased neuronal burst discharge near site of seizure onset in epileptic human temporal lobes.

We examined auto-correlation and interval distribution characteristics of neuronal discharge from patients with complex partial seizures. The objective was to compare the interictal firing patterns of neurons in mesial temporal structures ipsilateral to the site of seizure onset with firing patterns of neurons in homologous contralateral structures. Spontaneous interictal recordings of 258 single neurons were acquired from 23 patients. A "burst area" measure was derived from the neuronal auto-correlation to assess the likelihood of grouped action potential discharge (burst discharge). Large burst area measures indicate a tendency for single neuronal burst discharge, but do not disclose information about interspike intervals within bursts. Although several measures based on single neuronal interspike interval distributions showed no overall difference between hemispheres, burst area was significantly reduced in mesial temporal structures ipsilateral to the site of seizure onset. Possible mechanisms of decreased burst discharge in epileptogenic regions include selective loss of burst-discharging neurons and increased recurrent inhibition.

Action Potentials↗

Assessment of colour vision in epileptic patients exposed to single-drug therapy.

Diplopia, blurred vision and colour disturbances are well-known side effects associated with anti-epileptic drugs (AEDs). Farnsworth-Munsell 100-hue colour test (F-100) is an accepted and sensitive tool to detect changes in colour perception. To determine the impact of AEDs upon colour vision, we evaluated 37 consecutive patients with complex partial seizures exposed to monotherapy with phenytoin (PHT, carbamazepine (CBZ) or valproic acid (VPA). All had normal IQ and no congenital disturbances in colour vision or ocular diseases. Twenty normal controls were used for statistical analysis. Thirteen patients were exposed to PHT, 12 to CBZ and 12 to VPA. Visual colour perception was impaired in 30/37 (82%) of the study group. The most significant abnormality was detected in the blue-yellow axis in 10/13 patients exposed to PHT (p < 0.02) and in 8/12 treated with CBZ (p < 0.009). In 8/12 patients taking VPA, no significant abnormality was observed (p < 0.06). None of the studied patients complained of colour vision disturbances. Our findings strongly support the negative effect of AEDs upon colour vision discrimination, most likely due to changes at the retinal processing level. F-100 proved to be very useful to assess early toxicity due to AEDs.

Adolescent↗