Cases reported "Epilepsy, Generalized"

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1/50. Epileptic falling spells after epidural haematoma in adult Down's syndrome.

    A 35-year-old man with Down's syndrome showed epileptic falling spells. He had suffered from a traumatic right-sided epidural haematoma 3 years before. It had been neurosurgically treated, but MRI taken 5 days later had revealed a small contracoup contusion at the left temporal lobe. His falling spell was a brief tonic seizure without disturbance of consciousness. Background activities of EEG consisted of slow alpha waves interspersed with sporadic theta waves and the amplitude at the left temporal area was lower than the opposite one. Interictal EEG showed sharp waves or sharp and slow wave complexes predominantly at the right temporo-centro-parietal area as well as diffuse, though predominantly at frontal areas, bursts of slow waves with high amplitude. The EEG suggested focal epileptic activities evolving into secondary generalization. SPECT of the brain showed the hypoperfusion at the left temporal area and at the right posterotemporo-parietal area, where the hypoperfusion was somewhat reduced after the improvement of seizures. seizures were well controlled with phenytoin combined with phenobarbital. The incidence of epilepsy in the Down's syndrome has been reported to increase after the middle age in association with the development of Alzheimer's neuropathology. When those people would sustain head injuries, it was necessary to follow carefully using SPECT and EEG.
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2/50. Tiagabine-induced absence status in idiopathic generalized epilepsy.

    Several medications such as baclofen, amitriptyline and even antiepileptic drugs such as carbamazepine or vigabatrin are known to induce absence status epilepticus in patients with generalized epilepsies. Tiagabine (TGB) is effective in patients with focal epilepsies. However, TGB has also been reported to induce non-convulsive status epilepticus in several patients with focal epilepsies and in one patient with juvenile myoclonic epilepsy. In animal models of generalized epilepsy, TGB induces absence status with 3-5 Hz spike-wave complexes. We describe a 32-year-old patient with absence epilepsy and primary generalized tonic-clonic seizures since 11 years of age, who developed her first absence status epilepticus while treated with 45 mg of TGB daily. Administration of lorazepam and immediate reduction in TGB dosage was followed by complete clinical and electroencephalographic remission. This case demonstrates that TGB can induce typical absence status epilepticus in a patient with primary generalized epilepsy.
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3/50. Postictal mania following primary generalized seizures.

    A 29 year old male with primary generalized seizures for 13 years stopped his anticonvulsants leading to an increase in seizure frequency. Five months later he developed a severe manic episode postictally which responded well to a combination of neuroleptics and anticonvulsants. Postictal psychoses usually follow complex partial seizures. Manic episodes are uncommon. This case had some similarities with other cases of postictal psychoses reported previously. It underlines the need for further investigation of several facets of this complex relationship between mood disorders and epilepsy.
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4/50. Gamma knife radiosurgery for hypothalamic hamartomas in patients with medically intractable epilepsy and precocious puberty. Report of two cases.

    hamartoma of the hypothalamus represents a well-known but rare cause of central precocious puberty and gelastic epilepsy. Due to the delicate site in which the tumor is located, surgery is often unsuccessful and associated with considerable risks. In the two cases presented, gamma knife radiosurgery was applied as a safe and noninvasive alternative to obtain seizure control. Two patients, a 13-year-old boy and a 6-year-old girl, presented with medically intractable gelastic epilepsy and increasing episodes of secondary generalized seizures. Abnormal behavior and precocious puberty were also evident. Magnetic resonance (MR) imaging revealed hypothalamic hamartomas measuring 13 and 11 mm, respectively. After general anesthesia had been induced in the patients, radiosurgical treatment was performed with margin doses of 12 Gy to 90% and 60% of isodose areas, covering volumes of 700 and 500 mm3, respectively. After follow-up periods of 54 months in the boy and 36 months in the girl, progressive decrease in both seizure frequency and intensity was noted (Engel outcome scores IIa and IIIa, respectively). Both patients are currently able to attend public school. Follow-up MR imaging has not revealed significant changes in the sizes of the lesions. Gamma knife radiosurgery can be an effective and safe treatment modality for achieving good seizure control in patients with hypothalamic hamartomas.
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5/50. Seizure with prominent tonic initial signs followed by psychomotor features: a case report clinically manifesting an unusual ictal evolution.

    A clinically tonic seizure phase, immediately followed by psychomotor features (right hand dystonic posture, left hand and oral automatisms), was recorded by video and EEG, in a patient who had gliosis of the left temporal lobe and left hippocampal atrophy. Interictal epileptiform discharges were frequently seen in the left temporal area, and at the time of the tonic seizure phase, ictal spike discharges were continuously observed at the left posterior temporal area, which was recognized only by applying a high frequency filter (HFF) of 15 Hz to the digitally recorded EEG because EMG artifacts totally obscured the EEG with a HFF of 60 Hz. It is most likely that tonic seizure can occur in an adult patient with temporal lobe epilepsy, and it is speculated that an epileptogenic focus might activate a certain brain area which is regarded as a symptomatogenic zone for tonic seizures. If the tonic seizure phase is immediately followed by psychomotor features as seen in the present patient, the former could be due to focal epilepsy.
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6/50. Idiopathic generalized epilepsy presenting with hemiconvulsive seizures.

    PURPOSE: Unilateral seizures, or hemiconvulsive attacks, are motor seizures with tonic and/or clonic phenomena that involve only one side of the body. methods: We describe three adolescents who presented with hemiconvulsive seizures and were found to have 3-cps generalized spike-and-wave discharges on ictal and/or interictal EEG. All had normal neuroimaging studies. Two patients had been previously treated with carbamazepine, which led to a partial response in one patient. RESULTS: All three patients, however, are now seizure free on either sodium valproate or a combination of sodium valproate and lamotrigine. We believe the electroclinical diagnosis is that of idiopathic generalized epilepsy. CONCLUSIONS: Idiopathic generalized epilepsy presenting with hemiconvulsive seizures has not, to our knowledge, been previously described. However, the correct diagnosis of an idiopathic generalized seizure disorder, as opposed to a partial seizure disorder, has important treatment implications. The possible mechanism of hemiconvulsive seizures in idiopathic generalized epilepsy is discussed.
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7/50. time-related configuration change in benign focal epileptiform discharges of childhood: from a dipole to a negative monopole in a patient with generalized epilepsy.

    The case of a 6-year-old child with generalized epilepsy and benign focal epileptiform discharges (BFEDs) of childhood is presented. During his first EEG, performed when he was 6 years old, the patient had three staring episodes accompanied by bursts of 3-Hz spike-and-wave complexes. Interictally, frequent BFEDs were seen, configured as dipoles with a right temporal negativity and a right frontal positivity. The patient was administered ethosuximide and the staring spells disappeared. On follow-up EEG performed 2 years later, no generalized discharges were seen. However, the EEG again showed frequent spikes with a characteristic morphology of BFEDs. This time they were not configured as dipoles, but as monopoles with a maximum negativity over the right frontal region. The change in the generator's orientation over time (from a horizontal to a vertical dipole) is discussed, as well as the prognostic implications of the morphology and configuration of focal spikes. In addition, the authors review the coexistence of BFEDs and 3-Hz spike-and-wave complexes in their patient database.
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8/50. The clinical value of electroencephalogram/magnetic resonance imaging co-registration and three-dimensional reconstruction in the surgical treatment of epileptogenic lesions.

    With the rapid developments in image processing, new clinical applications of manipulation and three-dimensional (3-D) reconstruction of neuro-imaging are evolving. Combination with other non-invasive techniques aimed at localising electric sources in the brain is of particular interest. These techniques rely on the recording of brain electrical activity and/or the associated magnetic fields from multiple areas on the scalp. Data obtained from an electroencephalogram (EEG) or from magnetoencephalography (MEG) can be fused in 3-D arrangement with anatomical [magnetic resonance imaging/computerised tomography (MRI/CT)] and/or metabolic [positron emission tomography (PET)] data. Such techniques highlight information on the functional correlates of anatomical or space-occupying lesions and their role in the localisation of related symptomatic epilepsy. In the present study we report on methodological issues and preliminary clinical data on spectral EEG/MRI co-registration procedures, offering two examples of children presenting with hemispheric lesions, one frontal tumour and one temporal arterio-venous malformation. The EEG was acquired from 32/64 electrode location. The electrode position and that of four reference points were measured with a dual sensor Polhemus 3D Isotrak digitiser. Sources of EEG activity were determined in 3-D space with the inverse solution method low resolution electromagnetic tomography (LORETA), providing for each frequency component, the topographic distribution of active electrical sources. The positions of the reference points were also measured on MRI, and co-registration of EEG and MRI was achieved using a transformation algorithm. The reconstructed 3-D images of co-registered EEG/MRI clearly demonstrate the relationship between the space-occupying lesion and the epileptic activity. Preliminary results show that in all the patients it was possible to identify with a remarkable accuracy the 3-D topographic relationship between lesion and cortical areas showing localised abnormalities on the EEG. The present method could further enhance the understanding of the effect of resective treatment of structural lesions on brain functioning. The new combined images can be used in combination with image-guided surgery equipment to modify effective surgical resection.
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9/50. Idiopathic generalised epilepsy with phantom absences and absence status in a child.

    A syndrome of idiopathic generalised epilepsy with phantom absences of undetermined onset has been recently described. This syndrome clinically becomes apparent in adulthood with generalised tonic clonic seizures and frequently absence status epilepticus. We report an 11 year-old normal girl with frequent episodes of absence status and no other overt clinical manifestations. However, appropriate video-EEG recordings documented that she had frequent absence seizures that were so mild as to escape recognition by her and the parents. These consisted of mild impairment of cognition and eyelid fluttering during brief generalised discharges of spike/multiple spike and slow waves. No further seizures occurred and the EEG normalised after appropriate drug treatment. Thus, it appears that this syndrome of phantom absences and absence status may start much earlier, in late childhood. Appropriate video-EEG documentation is needed for the recognition of these patients that may be more common than it appears from the few published cases (with Video).
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10/50. Simultaneous EEG and functional MRI of epileptic activity: a case report.

    OBJECTIVES: Attempts to localize the source of epileptic activity by linking electroencephalographic (EEG) abnormalities to blood oxygenation level-dependent (BOLD) magnetic resonance imaging (MRI) signal alterations are hampered mainly by EEG distortions during MRI, subject motion, and unknown hemodynamic response characteristics. methods: Using T2*-weighted echo-planar imaging at 2.0 T (2 s temporal resolution, 2 x 2 x 4 mm(3) spatial resolution), this work demonstrates strategies to alleviate some of these problems while studying a patient who had ideopathic generalized epilepsy with poly-spike and slow-wave complexes. RESULTS: Continuous EEG recordings during dynamic MRI (500 ms scanning, 1500 ms delay) and post-examination derivation of an EEG reference function for MRI analysis revealed positive BOLD MRI responses with temporal characteristics similar to those obtained for functional challenges. CONCLUSIONS: The ability to map focal epileptic activity and/or associated cognitive processing provides new potential for both epilepsy research and clinical patient management.
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