Cases reported "Epilepsies, Partial"

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1/425. Cortical dysplasia with angiodysgenesis and chronic inflammation in multifocal partial epilepsy.

    A 25-year-old man with a long history of temporal lobe epilepsy developed right occipital lobe seizures and a progressive right homonymous hemianopia. MRI showed diffuse enhancement of the left temporoparieto-occipital white matter and cortical thickening of the left medial temporal lobe. The resected temporal lobe revealed cortical dysplasia and angiodysplasia with foci of more recent ischemic necrosis and chronic inflammation as an explanation for the clinical deterioration.
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ranking = 1
keywords = epilepsy
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2/425. Cognitive deterioration associated with focal cortical dysplasia.

    Surgery for an area of focal cortical dysplasia in a critical region is reported in a right-handed female manifesting intractable focal epilepsy and verbal cognitive deterioration. She developed the first seizure at 2 years of age and was treated with phenytoin and zonisamide, with good control until 10 years of age. Although seizures did not occur at 9 years of age, she manifested dyscalculia, right-left disorientation, and finger agnosia, and N-isopropyl-p-iodoamphetamine single-photon emission computed tomography (SPECT) revealed focal hypoperfusion in the left parietal lobe. At 11 years of age, she developed regular nocturnal seizures and gradually lost the ability to understand the meaning of sentences. Verbal IQ declined from 94 to 63, and the area of hypoperfusion detected by interictal N-isopropyl-p-iodoamphetamine SPECT spread over the left parietotemporal lobes. magnetic resonance imaging revealed focal cortical dysplasia mainly in the left parietal lobe, and ictal technetium-99m-ethyl cysteinate dimer SPECT images demonstrated an area of hyperperfusion around the focal cortical dysplasia, including the left precentral gyrus. Because of the overlap between the epileptogenic and functional cortex, the authors concluded that cortical resection, including focal cortical dysplasia, was inappropriate in this patient.
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ranking = 0.2
keywords = epilepsy
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3/425. Reactivation of herpes virus after surgery for epilepsy in a pediatric patient with mesial temporal sclerosis: case report.

    OBJECTIVE: This case report is presented to raise the awareness of the potential risk of reactivation of herpes simplex virus (HSV) encephalitis after intracranial surgery. CLINICAL PRESENTATION: The case of an 8-year-old male patient who suffered a reactivation of HSV encephalitis after undergoing amygdalohippocampectomy for complex partial seizures is reported. This patient had previously contracted HSV 1 meningoencephalitis at the age of 16 months. Six years later, a left amygdalohippocampectomy was proposed after the development of intractable partial epilepsy associated with left mesial temporal lesions. During the postoperative period, the patient suffered severe clinical deterioration with partial status epilepticus, aphasia, and hyperthermia, which resolved after intensive antiepileptic treatment supported by acyclovir. CONCLUSION: We advise prophylactic pre-, peri-, and postoperative treatment with acyclovir for patients with known histories of HSV encephalitis who undergo intracranial procedures.
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ranking = 1
keywords = epilepsy
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4/425. Partial seizures with focal epileptogenic electroencephalographic patterns in three related female patients with fragile-X syndrome.

    epilepsy and abnormal electroencephalographic (EEG) patterns have been reported in mentally retarded males with fragile-X syndrome, but the high incidence of epilepsy in such persons has been recognized only recently. These individuals have focal spikes in the EEG similar to the benign rolandic pattern. female carriers have very rarely been reported to have epilepsy or nonspecific abnormal EEG patterns. We report partial seizures with a focal epileptogenic EEG pattern in two sisters and their grandmother, who are all carriers of fragile-X syndrome. The sisters have mild developmental delay, but the grandmother is of normal intelligence. The mother of the two sisters is known to be a carrier of the fragile-x chromosome and is of normal intelligence, with no history of seizures. It is important for physicians to be aware of the possibility that females presenting with partial seizures of unknown cause may be fragile-X carriers, and enquiry for a family history of intellectual disability should be pursued.
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ranking = 0.4
keywords = epilepsy
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5/425. The role of early left-brain injury in determining lateralization of cerebral speech functions.

    Preparatory to craniotomy for the relief of medically refractory focal epilepsy, the lateralization of cerebral speech functions was determined by the Wada intracarotid Amytal test in 134 patients with clinical and radiologic evidence of an early left-hemisphere lesion. Their results were compared with those for 262 patients (140 right-handed, 122 left-handed), who were tested in a similar way. One-third of the patients with early lesions were still right-handed, and 81% of these right-handers were left-hemisphere dominant for speech. In the non-right-handers, speech was represented in the left cerebral hemisphere in nearly a third of the group, in the right hemisphere in half the group, and bilaterally in the remainder. Bilateral speech representation was demonstrated in 15% of the non-right-handers without early left-brain injury and in 19% of those with evidence of such early injury, whereas it was extremely rare in the right-handed groups. In addition, nearly half the patients with bilateral speech representation exhibited a complete or partial dissociation between errors of naming and errors in the repetition of verbal sequences after Amytal injection into left or right hemispheres. This points to the possibility of a functionally asymmetric participation of the two hemispheres in the language processes of some normal left-handers. The results of the Amytal speech tests in this series of patients point to locus of lesion as one of the critical determinants in the lateralization of cerebral speech processes after early left-brain injury. It is argued that in such cases the continuing dominance of the left hemisphere for speech in largely contingent upon the integrity of the frontal and parietal speech zones.
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ranking = 0.2
keywords = epilepsy
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6/425. Psychiatric presentation of epilepsia cursiva.

    Coarse brain disease can first present as a behavioural or psychiatric disorder. Partial seizures with complex symptomatology (psychomotor or temporal lobe epilepsy) may offer particular difficulties in differential diagnosis from the "functional psychoses". The authors report a case of "running epilepsy" (epilepsia cursiva), that first presented as a behavioural problem, and review the literature on this rare form of psychomotor epilepsy.
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ranking = 0.6
keywords = epilepsy
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7/425. Ictal 99mTc HM-PAO brain single-photon emission computed tomography in electroencephalographic nonlocalizable partial seizures.

    A 9-year-old child with intractable focal epilepsy was studied for possible surgical treatment. Multiple electroencephalographic studies did not localize the epileptic focus. An ictal single-photon emission computed tomography (SPECT) study with technetium 99m-hexamethylpropyleneamineoxime demonstrated a focal area of hyperperfusion. Through three-dimensional, functional to anatomical image-matching techniques, the focus was overlaid on the magnetic resonance image localizing the cortical convolution responsible for the epileptogenic focus. Subdural electroencephalographic studies performed for seizure localization and functional mapping confirmed this location. This case emphasized the usefulness of ictal SPECT scans in patients with seizures nonlocalizable by electroencephalography being evaluated for epilepsy surgery.
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ranking = 0.4
keywords = epilepsy
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8/425. Central pain as a manifestation of partial epileptic seizures.

    Central pain of epileptic etiology is very rare. The frequency of painful sensations in epileptic seizures varies between 0.3 and 2.8%. We report a patient with short-lasting painful attacks in the right arm. Changes in the electroencephalography (EEG) and the effective treatment with anticonvulsants in contrast to the therapeutic failure of analgesics, lead to the diagnosis of partial epilepsy with painful seizures. magnetic resonance imaging (MRI) of the brain was normal, whereas a postcentral parietal site of seizure origin involving the secondary somatosensory area was suggested by electroencephalographic findings. The literature is reviewed for cases with pain as the sole or predominant symptom of epileptic seizures.
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ranking = 0.2
keywords = epilepsy
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9/425. plasma exchange in Rasmussen's encephalitis.

    The authors observed a 4-year-old girl who has Rasmussen's encephalitis. She started with frequent localized and generalized seizures. Standard antiepileptic treatment was almost ineffective. The frequency of the generalized seizures decreased, but the myoclonic jerks of the left part of the body persisted. An EEG showed partial status epilepticus. The results of the CT scan were normal. antibodies to viruses were absent from the blood and cerebrospinal fluid. An MR scan showed a T2-weighted hypersignal zone in the right frontal region. Intravenous bolus injections of corticosteroids and drips of immunoglobulins were inefficient, and we started plasma exchanges which have continued for 9 months. The clinical state stabilized, and the images on the MR scan improved, but the results of the EEG did not improve. The authors discuss the effect of the plasma exchange, the use of which is questionable in this disease.
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ranking = 0.0015732090563277
keywords = myoclonic
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10/425. A novel mutation in the human voltage-gated potassium channel gene (Kv1.1) associates with episodic ataxia type 1 and sometimes with partial epilepsy.

    Episodic ataxia type 1 (EA1) is a rare autosomal dominant disorder characterized by brief episodes of ataxia associated with continuous interattack myokymia. Point mutations in the human voltage-gated potassium channel (Kv1.1) gene on chromosome 12p13 have recently been shown to associate with EA1. A Scottish family with EA1 harbouring a novel mutation in this gene is reported. Of the five affected individuals over three generations, two had partial epilepsy in addition to EA1. The detailed clinical, electrophysiological and molecular genetic findings are presented. The heterozygous point mutation is located at nucleotide position 677 and results in a radical amino acid substitution at a highly conserved position in the second transmembrane domain of the potassium channel. Functional studies indicated that mutant subunits exhibited a dominant negative effect on potassium channel function and would be predicted to impair neuronal repolarization. potassium channels determine the excitability of neurons and blocking drugs are proconvulsant. A critical review of previously reported EA1 families shows an over-representation of epilepsy in family members with EA1 compared with unaffected members. These observations indicate that this mutation is pathogenic and suggest that the epilepsy in EA1 may be caused by the dysfunctional potassium channel. It is possible that such dysfunction may be relevant to other epilepsies in man.
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ranking = 1.4
keywords = epilepsy
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