Cases reported "Epilepsy, Generalized"

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1/22. Functional imaging in schizencephaly using [18F]fluoro-2-deoxy-D-glucose positron emission tomography (FDG-PET) and single photon emission computed tomography with technetium-99m-hexamethyl-propyleneamine oxime (HMPAO-SPECT).

    We analyzed interictal [18F]fluoro-2-deoxy-D-glucose positron emission tomography (FGD-PET) and single photon emission computed tomography with technetium-99m-hexamethyl-propyleneamine oxime (HMPAO-SPECT) in a 23-year-old female with schizencephaly. She had epilepsy and mild left hemiparesis, but was otherwise developmentally normal. We found the glucose metabolism and perfusion of the wall of the schizencephalic cleft to be identical to those of normal cerebral cortex. The wall of the transcerebral clefts, which were observed to be lined by abnormally organized gray matter as a result of a migration disorder, demonstrated gray matter metabolic activity and perfusion. FDG-PET and HMPAO-SPECT were thus found to be a useful complement to magnetic resonance imaging for evaluating schizencephaly.
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ranking = 1
keywords = cortex
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2/22. Intractable epilepsy after a functional hemispherectomy: important lessons from an unusual case. Case report.

    Residual seizures after functional hemispherectomy occur in approximately 20% of patients with catastrophic epilepsy. These episodes are traditionally attributed to incomplete disconnection, persistent epileptogenic activity in the ipsilateral insular cortex, or bilateral independent epileptogenic activity. The authors report on the case of an 8-year-old boy with an intractable seizure disorder who had classic frontal adversive seizures related to extensive unilateral left hemispheric cortical dysplasia. The initial intervention consisted of extensive removal of the epileptic frontal and precentral dysplastic tissue and multiple subpial transections of the dysplastic motor strip, guided by intraoperative electrocorticography, Subsequently, functional hemispherectomy including insular cortex resection was performed for persistent attacks. After a seizure-free period of 6 months, a new pattern ensued, consisting of an aura of fear, dystonic posturing of the right arm, and unusual postictal hyperphagia coupled with an interictal diencephalic-like syndrome. electroencephalography and ictal/interictal single-photon emission computerized tomography were used to localize the residual epileptic discharges to deep ipsilateral structures. Results of magnetic resonance imaging indicated a complete disconnection except for a strip of residual frontobasal tissue. Therefore, a volumetric resection of the epileptogenic frontal basal tissue up to the anterior commissure was completed. The child has remained free of seizures during 21 months of follow-up review. Standard hemispherectomy methods provide extensive disconnection, despite the presence of residual frontal basal cortex. However, rarely, and especially if it is dysplastic, this tissue can represent a focus for refractory seizures. This is an important consideration in determining the source of ongoing seizures posthemispherectomy in patients with extensive cortical dysplasia. It remains important to assess them fully before considering their disease refractory to surgical treatment.
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ranking = 3
keywords = cortex
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3/22. Ictal EEG patterns in band heterotopia.

    Band heterotopia (BH) or "double cortex" syndrome is a neuronal migration disorder resulting in a diffuse band of subcortical grey matter and variable abnormality of the overlying cortex. patients with BH have a spectrum of psychomotor delay and seizures. Associated epileptic syndromes and interictal EEG findings have been described, but ictal EEG patterns are lacking. methods: We describe the clinical, interictal, and ictal EEG findings in two girls with BH and intractable seizures. RESULTS: Ictal EEG patterns correlated well with clinical seizure types, and did not have features unique to BH. Similarly, seizure behaviors and interictal EEG findings were typical of those seen in symptomatic generalized epilepsies. CONCLUSIONS: Despite evidence implicating the ectopic grey matter in seizure discharges, we conclude that seizure semiology and associated ictal EEG patterns in BH are no different from those seen in other causes of symptomatic generalized epilepsies.
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ranking = 2
keywords = cortex
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4/22. ifosfamide encephalopathy and nonconvulsive status epilepticus.

    BACKGROUND: ifosfamide (IFX), an alkylating agent and isomer of cyclophosphamide, is used as a single agent or a component of multi-agent chemotherapy in the treatment of ovarian, testicular, head and neck cancers, sarcomas and lymphomas. Encephalopathy is manifested by cerebellar ataxia, confusional state, complex visual hallucinations, extrapyramidal signs, seizures, and mutism. case reports: We report two patients with non-Hodgkin's lymphoma presenting with mutism and confusional state after IFX infusion. Nonconvulsive status epilepticus (NCSE) as the cause of confusion was diagnosed on the basis of EEG pattern and the apparent improvement following intravenous administration of diazepam. CONCLUSIONS: Electroencephalogram abnormalities during IFX treatment have been described but recordings are only available in six cases. In three of them, paroxysmal alterations warranted the diagnosis of NCSE; however, most cases of IFX encephalopathy might have associated NCSE.
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ranking = 0.0061463194077728
keywords = visual
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5/22. Magnetoencephalographic studies of two cases of diffuse subcortical laminar heterotopia or so-called double cortex.

    Two cases (a young male and a girl, suffering intractable epilepsy) of diffuse subcortical laminar heterotopia, or so-called double cortex (DC) have been investigated using magnetoencephalography (MEG). MEG confirmed involvement of both cortices (hetero- and normocortex) in the genesis of interictal spikes, and, according to the heterogeneity of DC syndrome, some differences were observed: spike initiation in the normocortex and latter involvement of the heterotopic cortex in the man, and rather a cancellation in both cortices in the girl. In addition, participation of heterotopic cortex in physiological activities could be demonstrated in the man.
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ranking = 9
keywords = cortex
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6/22. 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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ranking = 4
keywords = cortex
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7/22. 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 = 1
keywords = cortex
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8/22. A long-term observation on a case of epilepsy with occipital continuous spikes.

    We have reported a 38-year-old female who did not show clinical seizures during continuous spikes in the left occipital area over a period of 18 years. As to the neurological findings, visual disturbance, optic nerve atrophy and right hemianopsia were almost always present. The low density area distributing from the left occipital area to the left temporal one on the CT revealed to be porencephaly. From the clinical findings obtained in this case, the lesion responsible for the occipital continuous spikes could be ascribed to be functional abnormality of the visual pathway connecting the left lateral geniculate body with the left occipital cortex.
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ranking = 1.0122926388155
keywords = cortex, visual
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9/22. Are absences truly generalized seizures or partial seizures originating from or predominantly involving the pre-motor areas? Some clinical and theoretical observations and their implications for seizure classification.

    In both the current (1981) ILAE classification of Epileptic seizures and the recently Proposed Diagnostic Scheme for People with epilepsy and Epileptic seizures, typical absence seizures are defined as generalized seizures, implying widespread subcortical and cortical neuronal involvement from onset with impairment of consciousness as the clinical hallmark. Clinical observations from three patients and clinical and experimental data from the literature suggest, however, that: (1) consciousness is retained in many typical absences; (2) the true hallmark of these seizures is arrest of motor initiation due to disturbance of pre-motor area frontal-lobe function; (3) typical absences and partial seizures from these areas may show similar clinical and EEG features and involve the same neuronal circuits. The neuronal system primarily involved in these seizures consists of a relatively limited cortico-thalamo-cortical circuit, including the reticular thalamic nucleus, the thalamocortical relay and the predominantly anterior and mesial frontal cerebral cortex, with the cortex probably acting as the primary driving site. Typical absences thus should not be classified or defined as generalized seizures, particularly since neuropathological and imaging studies increasingly argue for localized structural abnormalities, even in idiopathic or primary generalized epilepsy. These observations further highlight the intrinsic weaknesses of the current classification system for seizures and support further adaptations of the diagnostic system currently under development.
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ranking = 2
keywords = cortex
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10/22. NARP syndrome and adult-onset generalised seizures.

    The neurogenic muscle weakness, ataxia and retinitis pigmentosa (NARP) syndrome is a maternally inherited disorder attributable to a heteroplasmic mtDNA point mutation. Catastrophic epilepsy may accompany severe, early onset forms of NARP, but seizures seem to be rare in cases with adolescent and adult onset. We describe a patient who developed clumsiness and visual problems in her teens. She had no clinical seizures but an EEG showed generalized spike and wave discharges. At this time the patient remained without a specific diagnosis. At the age of 21, the patient developed progressive ataxia and she also experienced a tonic-clonic status epilepticus. Further examinations revealed NARP syndrome. EEG abnormalities may precede adult onset seizures in the NARP syndrome.
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ranking = 0.0061463194077728
keywords = visual
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