Cases reported "Epilepsy, Tonic-Clonic"

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1/40. Integration of preoperative and intraoperative functional brain mapping in a frameless stereotactic environment for lesions near eloquent cortex. Technical note.

    The authors present a method of incorporating preoperative noninvasive functional brain mapping data into the frameless stereotactic magnetic resonance (MR) imaging dataset used for image-guided resection of brain lesions located near eloquent cortex. They report the use of functional (f)MR imaging and magnetic source (MS) imaging for preoperative mapping of eloquent cortex in difficult cases of brain tumor resection such as those in which there are large expansive masses or in which reoperations are required and the anatomy is distorted from prior treatments. To correlate methods of preoperative and intraoperative mapping localization directly, the authors have developed techniques of importing preoperative MS and fMR imaging data into an image-guided frameless stereotactic computer workstation. The data appear as a seamless overlay on the same preoperative volumetric MR imaging dataset used for stereotactic guidance during the operation. Intraoperatively identified functional locations mapped by cortical stimulation are recorded as digitally registered points. This approach should prove useful in assessing the accuracy and reliability of various preoperative functional brain mapping techniques.
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2/40. Northern epilepsy: a novel form of neuronal ceroid-lipofuscinosis.

    Northern epilepsy is an autosomal recessive childhood onset epilepsy syndrome, clinically characterized by generalized tonic-clonic seizures with onset at 5 to 10 years of age and subsequent slowly progressive mental deterioration. The patients may reach 50 or 60 years of age. A mutation responsible for the disease has recently been identified in a novel gene on chromosome 8p23, encoding a putative membrane protein with an unknown function. The present study, based on three autopsied patients, is the first neuropathological analysis of the disease, and showed intraneuronal accumulation of cytoplasmic autofluorescent granules. The granules were strongly stained by the Luxol fast blue, periodic acid-Schiff, and sudan black B methods in paraffin sections, and were immunoreactive for subunit c of the mitochondrial ATP synthase and sphingolipid activator proteins A and D. The intraneuronal storage was highly selective: the third layer of the isocortex and the hippocampal CA2, CA3, and CA4 sectors were severely affected, while other layers of the isocortex, the CA1 sector, and the cerebellar cortex were only minimally involved. The membrane-bound storage cytosomes showed a curvilinear ultrastructure with admixture of some granular components. Western blotting and N-terminal sequence analysis of purified storage material identified subunit c as the major component.These findings establish Northern epilepsy as a new form of neuronal ceroid-lipofuscinosis with an exceptionally protracted course.
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3/40. Detection of epileptogenic focal cortical dysplasia by depth, not subdural electrodes.

    Centers that perform presurgical epilepsy evaluations disagree on whether depth or subdural electrodes represent the optimal technique for invasive recording, especially in seizures originating outside the temporal lobe. A 13-year-old girl with a normal magnetic resonance imaging scan had unlocalized partial onset seizures, despite scalp and subdural grid ictal video/EEG recordings. Repeat video/EEG with depth electrodes showed a discrete site of continuous interictal spiking and seizure onset that was located 2-2.5 cm beneath the surface of the sensory cortex. The resected region showed focal cortical dysplasia and the patient had greater than 95% seizure frequency reduction at 3-year follow-up. We conclude that although subdural electrodes have many advantages when recording seizures outside the temporal lobes, depth electrodes may provide superior recordings when the epileptogenic region is beneath the cortical surface.
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keywords = cortex
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4/40. Clonic convulsion caused by epileptic discharges arising from the human supplementary motor area as studied by subdural recording.

    In order to help clarify the mechanism of tonic convulsion, which is commonly seen in supplementary motor area (SMA) seizures, we investigated the temporal relationship between ictal discharges arising from the SMA and the associated EMG discharges in the foot, in a patient with SMA seizures, prior to surgical treatment. The patient's intractable seizures consisted of tonic followed by clonic convulsion of the left foot also involving at times, the left hand. Ictal EEGs were investigated by subdural electrodes placed on the SMA-proper and pre-SMA, which were defined by cortical stimulation and by recording cortical-evoked potentials. Interictally, repetitive spike discharges were seen at the pre-SMA. Each seizure initially had a tonic phase associated with an electrodecremental EEG pattern. It was followed by clonic convulsion as shown by clonic EMG discharges of the left tibialis anterior (TA) muscle. It had a duration of 300 to 500 msec, and on each occasion a positive cortical activity at the pre-SMA preceded the EMG onset by 110 msec, and a negative spike at the SMA-proper preceded the EMG onset by 50 to 60 msec. Epileptic discharges originating from the pre-SMA spread to the SMA-proper and possibly also to the primary motor cortex (MI) in this patient. Since both SMA-proper and MI could elicit EMG discharges through the independent corticospinal tracts having different conduction velocities, even a single spike arising from the SMA could give rise to a long EMG burst, which may play some role in the tonic convulsion which characterizes SMA seizures.
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keywords = cortex
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5/40. Hippocampal sclerosis following brief generalized seizures in adulthood.

    This report describes a patient who had a first generalized tonic clonic seizure (GTCS) at the age of 18 years. The initial MRI scan was normal on visual and quantitative examination. After a further six GTCS, a second MRI scan showed reduced hippocampal volume and increased hippocampal T2 signal, which was confirmed in a third scan. Therefore, hippocampal sclerosis may be acquired in adulthood after brief GTCS.
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ranking = 0.0054098959335227
keywords = visual
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6/40. Cortical blindness after contrast-enhanced CT: complication in a patient with diabetes insipidus.

    Transient cortical blindness is an uncommon but well-known complication following cerebral angiography. One possible cause of this complication is an adverse reaction to contrast agent, resulting in an osmotic disruption of the blood-brain barrier that seems to be selective for the occipital cortex. We report the case of a 16-year-old male patient with cortical blindness after intravenous application of nonionic contrast agent during CT angiography performed because of seizure that was attributed to thrombosis of the basilar artery on the basis of clinical findings. To our knowledge, the development of cortical blindness after CT angiography has not been described in the literature. The patient's symptoms were triggered by hyponatriemia and diabetes insipidus.
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keywords = cortex
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7/40. Laminar heterotopic grey matter (double cortex) in a patient with late onset Lennox-Gastaut syndrome.

    The Lennox-Gastaut syndrome (LGS) is a severe epileptic disorder, characterized by developmental delay, typical electroencephalographic pattern and poor control of seizures. This epileptic syndrome generally presents in early childhood but exceptionally starts at the age of 5 years or later. We describe a patient who was classified as late-onset LGS, with evidence of mild hydrocephalus on CT. MRI added new diagnostic information by demonstrating laminar heterotopic grey matter.
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keywords = cortex
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8/40. Late postpartum eclampsia coincident with postdural puncture headache: a case report.

    Preeclampsia developed in this patient 4 days' postpartum. Visual changes, headache, and elevated blood pressures were present on arrival to the emergency department. Coincident with the preeclampsia was a postdural puncture headache complicating the diagnosis of late postpartum preeclampsia. pregnancy-induced hypertension laboratory values were normal, and relief of the postdural headache and visual disturbances was obtained after placement of an epidural blood patch. Blood pressures continued to increase, however, and late postpartum eclampsia developed. We conclude that symptoms of a postdural puncture headache can mimic those of postpartum preeclampsia. Early recognition and treatment of postpartum preeclampsia may decrease patient mortality and morbidity by preventing late postpartum eclampsia.
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ranking = 0.0054098959335227
keywords = visual
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9/40. Bilateral Todd's paralysis after focal seizures.

    Transient postictal hemiparesis or monoparesis is not uncommon after partial (focal) seizures. We report 2 patients who complained of severe bilateral limb weakness after generalized tonic-clonic seizures (GTCS) beginning focally. Bilateral Todd's paralysis was verified and documented in both patients. EEG and clinical evidence indicate the supplementary motor cortex as the most likely source of the seizures in both cases.
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ranking = 0.16666666666667
keywords = cortex
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10/40. Postpartum blindness: two cases.

    We present 2 cases, one eclamptic patient and one noneclamptic patient, of headache, cortical blindness, and seizures. Both patients demonstrated findings consistent with posterior leukoencephalopathy syndrome. posterior leukoencephalopathy syndrome is a rapidly evolving neurologic condition that is characterized by headache, nausea and vomiting, seizures, visual disturbances, altered sensorium, and occasionally focal neurologic deficits. posterior leukoencephalopathy syndrome can be triggered by numerous conditions, including preeclampsia-eclampsia, and can be seen in the postpartum period. It is characterized predominately by white matter vasogenic edema of the occipital and posterior parietal lobes. This condition can be difficult to differentiate clinically from cerebral ischemia, and magnetic resonance imaging with diffusion-weighted imaging and apparent diffusion coefficient are needed to do so. In most cases of posterior leukoencephalopathy syndrome, the prognosis is excellent, with full resolution of symptoms.
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ranking = 0.0054098959335227
keywords = visual
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