Dynamic cerebral autoregulation during sevoflurane anesthesia: a comparison with isoflurane

OBJECTIVE: To study the incidence and pattern of epilepsy in patients with periventricular leukomalacia (PVLM) in two specialty clinic settings. BACKGROUND: Motor and cognitive deficit as well as epilepsy are common in patients with PVLM. With modern imaging techniques, PVLM is now easily recognized. METHODS: Epileptic seizures and syndromes as well as motor and cognitive deficits were correlated with MRI findings. Two patient populations were studied: Group A-children with cerebral palsy and PVLM presenting to a center for children with motor disability (n = 19); and Group B-epileptic patients with PVLM presenting to a tertiary epilepsy center (n = 12). A single patient with PVLM and epilepsy who underwent extensive investigations, including intracranial EEG telemetry, is reported. RESULTS: In Group A, 47% of patients had epilepsy (9/19). PVLM was found in 1.27% of patients investigated for epilepsy at a tertiary epilepsy center (12/942). The majority of patients in both groups had multiple seizure types, with complex partial seizures being most common. Of patients with seizures (Groups A and B), 85.7% had intractable epilepsy (18/21). Intracranial EEG in the illustrative case demonstrated a multifocal epileptic process with occipitotemporal predominance. CONCLUSIONS: PVLM was an uncommon underlying cause in patients presenting with epilepsy (Group A); however, patients presenting with motor disability and PVLM (Group B) had a high incidence of seizures. PVLM in epileptic patients is associated with multiple seizure types and medically refractory disease.     

Congenital ocular motor apraxia: imaging findings

PURPOSE: To determine the frequency of cerebellar and cerebral abnormalities on brain imaging studies in children with congenital ocular motor apraxia. METHODS: Brain imaging studies were performed in 19 children with typical congenital ocular motor apraxia who were in the care of a visual impairment program at a children's hospital. Independent clinical review categorized the subjects as having partial (n = 10) or expanded (n = 9) congenital ocular motor apraxia on the basis of extent of associated speech or neurodevelopmental problems. Fifteen CT studies and 13 MR examinations of the brain performed in these children were reviewed independently by two pediatric neuroradiologists. Radiologic findings were agreed on by consensus. RESULTS: Cerebellar abnormalities were found in 12 of 19 cases. The cerebellar vermis was small in 10 children. A small cerebellar vermis was the only abnormality in five of 10 children with partial congenital ocular motor apraxia and in two of nine children with expanded congenital ocular motor apraxia. Among seven children with a small vermis examined with high-resolution MR imaging, the inferior portion of the vermis was preferentially involved in each case. Of these seven subjects, none of four with partial congenital ocular motor apraxia but two of three with expanded congenital ocular motor apraxia had an abnormality of the superior portion of the vermis. Miscellaneous supratentorial lesions affecting both gray and white matter were found in six subjects. Five of the 19 children had normal imaging findings. CONCLUSION: Inferior vermian hypoplasia is the most common abnormality in children with congenital ocular motor apraxia.     

Comparative assessment of treatment efficacy with lamotrigine (Lamictal) together with valproate and/or carbamazepine in patients with refractory epilepsy

For the study 17 patients aged 26-55 years were qualified after ineffective treatment with carbamazepine and/or valproate for simple partial, complex partial or secondary generalized seizures. The criterion of resistance to treatment was the occurrence of four or more seizures monthly. The treatment with lamotrigine was successful in 9 (52%) cases, with reduction of seizures by at least 50%. In 2 cases the seizure activity in EEG became normal. In 8 cases the addition of lamotrigine was ineffective and the drug was discontinued (in one case due to allergic rash and in one the cause was toxic hepatocellular damage).     

Hemodynamically-induced transitory ischemic attacks. A differential focal motor seizures diagnosis?

This case report intends to focus attention on hemodynamic TIAs as cause of repetitive involuntary movements (RIMs) as differential diagnosis of simple partial motor seizures. We report two patients with episodic unilateral limb shaking lasting up to several minutes and which could be triggered by orthostasis, heat or physical exertion. Repeated EEG recordings revealed no epileptic discharges. In both patients, duplex ultrasonography revealed an occlusion of the internal carotid artery (ICA) contralateral to the side of the RIMs. Blood flow velocities in the middle cerebral artery ipsilateral to ICA occlusion were reduced and vasomotor reactivity to hypercapnia was absent. After elevation of blood pressure both patients became asymptomatic. We suggest that in patients with episodes of RIMs, Doppler sonography and tests of cerebral vascular reserve capacity should be performed to search for a hemodynamic origin of these symptoms.     

Problematic standards: improving organizational performance through the assess and improve phases

Since 1987, we have diagnosed 10 patients, 4 males and 6 females, aged 2-11 years at the last evaluation, who all met the following criteria of severe myoclonic epilepsy in infancy (SMEI): generalized or unilateral long-lasting febrile clonic seizures in the first year of life; the subsequent appearance of myoclonic seizures and other types of seizure (partial seizures, atypical absences and convulsive status epilepticus); and neuropsychological deterioration for a certain period. Family histories of epilepsy and febrile seizures could be traced in 1 and 3 cases, respectively. None of them had previous personal history of brain insult. Electroencephalographic (EEGic) recordings in febrile seizure stage were normal; and continuous prophylaxis with phenobarbital failed to prevent the recurrence of febrile seizures. EEG studies in myoclonic stage showed generalized spike-and-waves, polyspike-and-waves, focal abnormalities and/or photosensitivity. The seizures were highly resistant to antiepileptic drugs. Our experiences suggested that comedication of valproic acid, clonazepam and carbamazepine may be most effective in treatment of the diverse seizures including myoclonic seizures, myoclonic-tonic-clonic seizures, atypical absences and partial seizures. Myoclonic seizures and atypical absences diminished in parallel to a clear-cut decrease in generalized abnormalities on EEG in 4 cases aged more than 7 years. However, the partial seizures, secondarily generalized seizures and status epilepticus were still present. Further investigations should aim to identify the underlying etiology and to search more effective treatment.     

Semiological seizure classification

We propose an epileptic seizure classification based exclusively on ictal semiology. In this semiological seizure classification (SSC), seizures are classified as follows: a. Auras are ictal manifestations having sensory, psychosensory, and experiential symptoms. b. Autonomic seizures are seizures in which the main ictal manifestations are objectively documented autonomic alterations. c. "Dialeptic" seizures have as their main ictal manifestations an alteration of consciousness that is independent of ictal EEG manifestations. The new term "dialeptic" seizure has been coined to differentiate this concept from absence seizures (dialeptic seizures with a generalized ictal EEG) and complex partial seizures (dialeptic seizures with a focal ictal EEG). d. Motor seizures are characterized mainly by motor symptoms and are subclassified as simple or complex. Simple motor seizures are characterized by simple, unnatural movements that can be elicited by electrical stimulation of the primary and supplementary motor area (myoclonic, tonic, clonic and tonic-clonic, versive). Complex motor seizures are characterized by complex motor movements that resemble natural movements but that occur in an inappropriate setting ("automatisms"). e. Special seizures include seizures characterized by "negative" features (atonic, astatic, hypomotor, akinetic, and aphasic seizures). The SSC identifies in detail the somatotopic distribution of the ictal semiology as well as the seizure evolution. The advantages of a pure SSC, as opposed to the current classification of the International League Against Epilepsy (ILAE), which is actually a classification of electroclinical syndromes, are discussed.     

Adolescent onset of idiopathic photosensitive occipital epilepsy after remission of benign rolandic epilepsy

PURPOSE: We describe 2 girls, aged 19 years, who experienced a rolandic seizure at ages 4 and 5, respectively, together with the interictal EEG features of benign rolandic epilepsy (BRE). In adolescence both patients developed photosensitive occipital seizures accompanied by spontaneous and photic-induced occipital EEG paroxysms. METHODS: We have been following 33 patients with a history of BRE, between ages 12 and 28 years (mean 17 years). Twenty-one of these patients had experienced their last rolandic seizure before the age of 10 years and 9 of them had been without treatment since age 11 or earlier. In 2 of these 9 patients, other types of seizures recurred after remission of BRE. Clinical, EEG, and evoked potential findings on these 2 patients are presented. RESULTS: After having experienced BRE, both patients suffered partial seizures from age 12, with elementary visual hallucinations, visual blurring, slow head turning, cephalic pain, epigastric discomfort, unresponsiveness, and vomiting. Seizure onset was related to watching TV or exposure to bright light. EEG showed interictal occipital spikes, and a photoparoxysmal response limited to the occipital lobes. Visual evoked potentials were greatly increased in amplitude. One patient had two visual attacks only and remained seizure free after 4 years of follow-up, while the other had seizures controlled by an association of valproate and carbamazepine. CONCLUSIONS: Clinical and neurophysiological characteristics suggest that these two patients may have presented different age-related expressions within the spectrum of a benign seizure susceptibility syndrome rather than sharply distinct epilepsy syndromes.     

Multilobar polymicrogyria, intractable drop attack seizures, and sleep-related electrical status epilepticus

BACKGROUND AND OBJECTIVE: Patients with cortical malformations often have intractable seizures and are candidates for epilepsy surgery. Within an unselected series of patients with various forms of cortical malformation, nine patients with multilobar polymicrogyria had electrical status epilepticus during sleep (ESES) accompanied by infrequent focal motor seizures. Eight patients also had intractable atonic drop attack seizures. Because ESES usually is accompanied by a good long-term seizure prognosis, the objective of this study was to examine ESES outcome among patients with a structural lesion that is usually highly epileptogenic and has a low seizure remission trend. METHODS: The nine patients had follow-up periods lasting 4 to 19 years. All underwent brain MRI, serial sleep EEG recordings, and cognitive testing during and after ESES. RESULTS: ESES and drop attack seizures appeared between the ages of 2 and 5 years (mean, 4 years) and ceased between the ages of 5 and 12 years (mean, 8 years). At the last visit patients were 8 to 23 years of age (mean, 14.5 years) and were either seizure free or had very infrequent focal motor seizures during sleep. Three patients were free from antiepileptic drugs. In no patient was definite cognitive deterioration apparent after ESES in comparison with earlier evaluations. CONCLUSIONS: Age-related secondary bilateral synchrony underlying ESES may be facilitated in multilobar polymicrogyria. The good seizure outcome contrasts with that usually found in the presence of cortical malformations. For children with polymicrogyria and drop attack seizures, surgical treatment of the epilepsy should be considered cautiously, and sleep EEG recordings should be performed systematically.     

Clinical examination on localization-related epilepsies with elementary visual seizures--clinical, electroencephalographic and imaging diagnostic studies

The clinical, electroencephalographic and Imaging diagnostic features of the 45 patients of Localization-related Epilepsy who had elementary visual symptoms at seizure onset were investigated. There were 24 males and 21 females aged 19 to 78. Their ages at seizure onset ranged from 1 to 55 with a mean of 15.4. The patients were divided into 3 groups based on the pattern of elementary visual symptoms: Group 1; 23 patients (51%) with only elementary positive visual symptoms, Group 2; 9 patients (20%) with only elementary negative visual symptoms, Group 3; 13 patients (29%) with others. It came to our notice that 8 (18%) patients in Group 3 had positive + negative (P*N) visual seizures such as scintillation scotoma. It was quite difficult to draw a distinction between the P*N seizures and scintillation scotoma in migrainous patients in quality. Therefore, a question arises whether similarities between them are ascribed to the same underlying mechanism. It is possible that the mechanism of the P*N seizures is different from that of the scintillation scotoma, but the both produce the same condition. However, we are not competent to discuss this hypothesis. Further work along this line is necessary. In addition to the elementary visual symptoms, autonomic (69%), focal motor (29%), illusion (29%), vertiginous (22%) manifestations, etc. were also observed. These manifestations suggest that epileptic ictal discharges spread into many different brain areas. Regarding Group 1-3, occurrence of illusional seizures was more common in Group 2. In this group, in only one patient, occipital interictal discharges were observed. The subjects were subclassified into two groups depending on whether the seizures were well controlled (good outcome) or poorly controlled (poor outcome group). On these groups, comparative studies were performed. The poportion of the patients with a family history of convulsive disorder was higher in the latter than that in the former. To the contrary, the poportion of the patients with a etiologic episode was higher in the former than that in the latter. With respect to the imaging study, regardless of localizations, abnormal findings were detected in 25% on CT scan, in 29% on MRI, and in 71% on SPECT. Whether or not these abnormal findings completely or partially agreed with presumed epileptogenic brain region (occipital lobes) was investigated. CT scan was positive (correspondent) in 8%, MRI was positive in 13%, and SPECT was positive in 54%. The incidence of SPECT abnormalities was higher in the poor outcome group (70 %) than that in the good outcome one (43%). We may, therefore, conclude that SPECT seems to be useful for the detection of epileptogenic region, especially in intractable cases. But the incidence of these imaging studies' abnormalities located in occipital lobe was not so high, therefore, there seems to be no doubt that clinical symptoms and EEG findings are most important for diagnosis.     

Effect of chronic electrical stimulation of the centromedian thalamic nuclei on various intractable seizure patterns: I. Clinical seizures and paroxysmal EEG activity

Twenty-three patients with various intractable seizure patterns were divided into four groups based on their most frequent seizure type and their clinical and EEG response to chronic electrical stimulation of the centromedian thalamic nuclei (ESCM): group A, generalized tonic-clonic (GTC, n = 9); group B, partial motor (Rasmussen type) (n = 3); group C, complex partial seizures (CPS, n = 5); and group D, generalized tonic seizures (Lennox-Gastaut type) (n = 6). CM were radiologically and electrophysiologically localized by means of stereotaxic landmarks and by thalamically induced scalp recruiting-like responses and desynchronization. ESCM consisted of daily 2-h stimulation sessions for 3 months. Each stimulus consisted of a 1-min train of square pulses with a 4-min interstimulus interval, alternating right and left CM. Each pulse was 1.0 ms in duration at 60/s frequency and 8-15 V (400-1,250 microA) amplitude. Voltage (V), current flow (microA) and impedance (k omega) at the electrode tips were kept constant. A significant decrease in the number of seizures per month and paroxysmal EEG waves per 10-s spochs occurred in group A patients between the baseline period (BL) and the ESCM period. These changes persisted for > 3 months after discontinuation of ESCM (poststimulation period, Post). Post was accompanied by a significant decrease in the number of paroxysmal EEG discharges. A substantial decrease in seizures and paroxysmal discharges was also observed in patients of group B. In contrast, patients of groups C and D showed no significant changes from BL to ESCM and Post periods, except for a significant decrease in the number of seizures in group D patients from BL to Post periods.     

Need for electroencephalogram video confirmation of atypical absence seizures in children with Lennox-Gastaut syndrome

Children with childhood epileptic encephalopathy (Lennox-Gastaut syndrome) frequently have both multiple seizure types and nonepileptic stereotyped events that are difficult to differentiate. We hypothesize that electroencephalogram (EEG) video monitoring is essential for correct identification of atypical absence seizures in this population. All video/EEG monitoring records on patients with confirmed Lennox-Gastaut syndrome between September 1992 and December 1996 were reviewed for clinical events and EEG changes. A subset of patients with suspected atypical absence seizures during the video/EEG formed the cohort for analysis. Thirty-eight patients had 48 monitoring periods ranging from 1 to 4 days (mean, 2.2 days). Twenty-six monitoring periods captured suspected atypical absence seizures and formed the study cohort. Suspected atypical absence seizures were epileptic seizures in only 27% (7 of 26) of the study cohort. By contrast, parents reliably and correctly identified tonic, atonic, and tonic-clonic seizures in the study cohort. Reliable diagnosis and subsequent counting of atypical absence seizures in patients with Lennox-Gastaut syndrome cannot be made on the basis of observation and/or history alone. Future outpatient studies of investigational anticonvulsant medications for patients with Lennox-Gastaut syndrome should consider parental counts of atypical absence seizures unreliable. We recommend that video/EEG monitoring be done on all Lennox-Gastaut syndrome patients with suspected atypical absence seizures not controlled by medication.     

Paediatric pain management--the next step?

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.     

Can the age limit for endoscopy be increased in dyspepsia patients who do not have alarm symptoms?

We studied the interictal EEG of 50 epileptic patients (28 males, 22 females) who had parenchymal neurocysticercosis, diagnosed by CAT/MRI of the brain, positive immunological reaction for cysticercosis in cerebral spinal fluid or both. Age ranged from 5 to 61 years old; the mean age of onset was 24.2 +/- 12.2 years. Thirty-six patients had generalized seizures, 13 partial seizures with secondarily generalized seizures, and 1 had complex partial seizures. Twenty-two patients had parenchymal calcifications (inactive form); 21 had parenchymal cysts (active form) and 7 had both. EEG was abnormal in 14 patients (28%): 8 had focal slowing, 3 had focal sharp or spike activity, and 3 had both. The EEG was normal in patients with inactive forms of neurocysticercosis. The EEG was abnormal in 50% of patients with active and mixed forms of neurocystercosis and in 48% of patients with active form only. We conclude that the active forms of neurocysticercosis should be suspected when the EEG is found to be abnormal. In additional, EEG abnormality does not depend on the number of lesions, but rather on location and viability of the cysts, and on host response.     

Familial resemblances in disgust sensitivity and animal phobias

We present 100 children diagnosed with epilepsy who were seizure-free for more than 1 year and still on monotherapy of antiepileptic drugs (AEDs). We matched each child with a healthy classmate and performed neuropsychological testing and EEG before and after complete withdrawal of the AEDs. The withdrawal phase lasted 3 months, but the dose decrease was individualized for each patient. Three to 4 months after complete withdrawal of the drug all patients were reassessed. Patients with seizure relapse are excluded from the study. Seventeen patients are regarded as dropout, 11 because of seizure relapse and six because of protocol violation. The remaining 83 patients were treated with carbamazepine (n = 56), valproic acid (n = 17), or phenytoin (n = 10). Serum concentrations of the AEDs were measured using peak plasma levels that were taken immediately before or after psychological testing. We used neuropsychological tests to assess psychomotor function and "central" cognitive processing such as information processing or memory function. We found significant improvement attributable to drug withdrawal on only one of the cognitive tests, namely, psychomotor speed, suggesting that the impact of AED treatment on higher-order cognitive function is rather limited. In addition, we found group differences between the epilepsy group and the control group at baseline that persisted after drug withdrawal. Subsequent analysis showed some factors that may have contributed to these group differences. First, patients with a former diagnosis of absence seizures show lower scores both at baseline and after drug withdrawal. We may assume that the seizure propensity has not disappeared completely in these patients. Some evidence is found that phenytoin may have a different cognitive profile than carbamazepine, with more impairment on tests that measure motor and mental speed. Again, this impairment persists after drug withdrawal.     

Fluorometric assessments of acrosomal integrity and viability in cryopreserved bovine spermatozoa

It is widely agreed that after two or more seizures patients should be given antiepileptic treatment, but there is still controversy about the treatment of patients after a first unprovoked seizure. In a multicenter, randomized, open trial, patients with a first tonic-clonic seizure were randomized to immediate treatment (carbamazepine, phenytoin, phenobarbital, or sodium valproate) or to treatment only after another seizure. Fifty-two (24%) of the 215 patients randomized to immediate treatment and 85 (42%) of the 204 randomized to delayed treatment experienced seizure recurrence during follow-up. Age, acute treatment of the seizure with benzodiazepines, remote etiologic factors, and EEG abnormalities were significant predictors of relapse. Of the immediately treated patients, 87% had no seizures for a year and 68% had no seizures for 2 years, whereas only slightly fewer initially untreated patients (83% and 60%) achieved these endpoints. Patients treated after the first seizure and those treated after seizure relapse had the same time-dependent probability of achieving 1 and 2 seizure-free years. None of the variables that were prognostic predictors of relapse was significantly associated with the probability of having 1 or 2 years of seizure control. Anticonvulsants in patients presenting a first tonic-clonic seizure reduce the risk of relapse; however, 50% of patients who are not treated will never experience a second seizure. Moreover, the probability of long-term remission is not influenced by treatment of the first seizure.     

Technetium-99m-HMPAO brain SPECT in systemic lupus erythematosus with CNS involvement

Functional brain SPECT is playing an increasingly important role in evaluating CNS conditions in patients with systemic lupus erythematosus (SLE). However, SPECT findings varied in different studies because of their small population. Furthermore, earlier researchers, being restricted by the resolution of the camera, might not have been able to evaluate deep-seated nuclei such as the basal ganglia and thalamus. In this study, we describe the different patterns of SPECT findings in SLE patients with CNS involvement. METHODS: Seventy-two SLE patients (aged 14-67 yr; mean 33.2 yr) were divided into three groups: Group 1 with definite neuro-psychiatric disorder (including stroke, seizures and psychosis); Group 2 with minor neuropsychiatric disorders (headache, dizziness and recent memory impairment); and Group 3 without any neuropsychiatric symptoms or signs. Ninety minutes after injection of 1110 MBq 99mTc-HMPAO, brain SPECT was performed using a dual-head camera and fan-beam collimator. In addition, MRI and an electroencephalography (EEG) were also performed. RESULTS: SPECT findings were normal in 87% of the Group 3 patients and abnormal in all Group 1 patients; 84.6% of the Group 2 patients had abnormal SPECT findings. The parietal, frontal and temporal lobes were the most common areas of CNS involvement. Parietal lobes were involved in 95.6% of Group 1 patients and 80.7% in Group 2 patients. Frontal lobes were involved in 56.5% of Group 1 patients and 65.3% of Group 2 patients. Temporal lobes were involved in 56.5% of Group 1 patients and 46.1% of Group 2 patients. The basal ganglion was involved in about 30% of Group 1 patients and 11.5% of Group 2 patients, while the thalamus and cerebellum were less involved in neuropsychiatric SLE. MR images showed less sensitivity in the detection of CNS involvement than the SPECT and were normal in 27.3% of patients with definite neuropsychiatric disorders. The EEG and anticardiolipin antibody did not correlate well to the clinical diagnosis. CONCLUSION: HMPAO brain SPECT had the best correlation with the clinical diagnosis and may provide additional and objective information on SLE patients with potential CNS involvement.     

Large colon resection

Early motor manifestations are the main components of focal seizures involving the frontal lobe. We examined the relationship between the initial ictal motor manifestations and interictal abnormalities of cerebral glucose consumption (rCMRGlc) as assessed by PET in 48 consecutive patients with focal seizures of neocortical origin. Group data analysis revealed that patients with predominantly unilateral clonic seizures had a significant contralateral perirolandic hypometabolism and to a lesser degree a contralateral frontomesial hypometabolism. Patients with predominantly focal tonic manifestations showed a hypometabolism within the frontomesial and perirolandic regions that was unilateral in all patients with lateralized tonic seizures. Patients with versive seizures had mainly contralateral metabolic depressions without a consistent regional pattern. Patients with hypermotor seizures had metabolic depressions involving frontomesial, anterior cingulate, perirolandic, and anterior insular/frontal operculum areas. In all patient groups, bilateral and symmetric hypometabolism of the thalamus and cerebellum was observed. We propose that this pattern of distinctly abnormal metabolic brain regions demonstrates not only possible epileptogenic zones but also symptomatogenic brain regions as shown by the associations between clinical manifestations and sets of abnormal brain regions, particularly if epileptogenic zones are in a clinically silent neocortical brain region. The detection and possible differentiation of symptomatogenic and epileptogenic zones might improve the effectiveness of presurgical noninvasive studies.     

Merosin-negative congenital muscular dystrophy, occipital epilepsy with periodic spasms and focal cortical dysplasia. Report of three Italian cases in two families

We report clinical, EEG and neuroimaging findings of three patients in two Italian families with merosin-negative congenital muscular dystrophy (CMD), drug-resistant occipital epilepsy, diffuse persistent cerebral white matter changes and focal cortical dysplasia. Clinical and epilepsy histories, EEG and neuroimaging findings were very similar in all patients. Seizures started in childhood and mainly consisted of periodic spasms, a particular type of partial seizure characterized by clusters of epileptic spasms. The motor expression of the spasms was very mild so that they had been frequently missed or misinterpreted as non-convulsive generalized absence seizures. Interictal EEG showed occipital spike-waves and bilateral synchronous slow spike-wave discharges. Ictal EEG showed prolonged periodic sequences of slow waves with associated fast rhythm complexes, characteristic of periodic spasms. Two patients had normal intelligence, one patient presented moderate mental retardation. Focal cortical dysplasia in the posterior areas of the brain, in addition to marked diffuse white matter alterations, was detected in the magnetic resonance images of all patients. Findings in these patients indicate that in merosin-negative CMD brain involvement can include cortical dysplasia, in addition to white matter changes. In such cases the brain damage can lead to a childhood-onset localization-related symptomatic occipital epilepsy. Epileptic seizures and cortical dysplasia can be, however, difficult to detect in CMD. The clinical semiology of epileptic seizures may in fact be modified because of muscular weakness. This implies that epilepsy may be misdiagnosed or even missed and EEG-polymyographic recordings may be necessary to identify it. Similarly, cortical dysplasia may be very localized and visible by neuroimaging only if it is carefully investigated on the basis of epileptological and EEG-polymyographic findings.     

Idiopathic generalised epilepsy in adults manifested by phantom absences, generalised tonic-clonic seizures, and frequent absence status

OBJECTIVES: To describe the clinical and EEG features of adult patients with very mild absences, late onset generalised tonic clonic seizures, and frequent absence status. METHODS: Patients were referrals to a clinic for epilepsies. They all had clinical assessment and EEG, video EEG, or both for documentation of absences. RESULTS: Of 86 adults with idiopathic generalised epilepsies and EEG/video-EEG documented absences, 13 patients showed similar clinico-EEG features with: (a) "phantom absences" consisting of mild ictal impairment of cognition associated with brief (3-4 s), generalised 3-4 Hz spike/multiple spike and slow wave discharges; (b) infrequent, mainly late onset, generalised tonic clonic seizures, and (c), absence status which occurred in six of them either in isolation or terminating with generalised tonic clonic seizures. None of the patients had myoclonic jerks or photosensitivity. Two patients were father and daughter and another patient had a family history of infrequent generalised tonic clonic seizures. CONCLUSION: It seems that this is an idiopathic generalised epilepsy syndrome in adults which has not been previously recognised.     

Spectral EEG during short-term discontinuation of antiepileptic medication in partial epilepsy

The effect of short-term discontinuation of antiepileptic drugs (AEDs) on spectral analysis of EEG background activity (spectral EEG) was studied in patients undergoing preoperative evaluation for epilepsy surgery. We also wished to clarify whether AED discontinuation would provide lateralizing evidence in spectral EEGs of patients with temporal lobe epilepsy (TLE). Spectral EEGs were performed in 15 patients receiving their regular medication regimen and again after a 1-week period during AED withdrawal. A subgroup of 9 patients who had previously undergone temporal lobectomies (TLE group) was studied separately. In this group, we evaluated the effect of preceding seizures on spectral EEG derived from temporal neocortical areas. In all patients, spectral EEG changes were detected even after a short-term AED withdrawal. The total amount of absolute delta activity was reduced and occipital peak frequency and relative alpha activity were increased as compared with baseline values. In TLE patients with habitual seizures occurring < 20 h before the spectral EEG recording, lateralization was correctly identified by the greater amount of absolute delta activity ipsilateral to the epileptogenic focus. Epileptic seizures are accompanied by subtle focal slowing in spectral EEG for a much longer period than has been assumed. In addition, postictal spectral EEG over the temporal lobes may have lateralizing value. Further studies in larger numbers of patients are needed to evaluate the role of spectral EEG in the preoperative evaluation of patients for temporal lobe surgery.