Cases reported "Epilepsy, Tonic-Clonic"

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1/74. Encephalopathy complicating high-dose melphalan.

    High-dose melphalan (HDM) with peripheral blood stem cell transplant (PBSCT) is a common treatment for patients with multiple myeloma (MM) and more recently also with AL amyloidosis (ALA). We report two female patients with severe renal failure who underwent treatment with HDM for MM (patient 1) and ALA (patient 2). Both patients developed severe encephalopathy with generalised tonic-clonic seizures and a glasgow coma scale (GCS) of 3/15. Causes for coma such as infections, metabolic disturbances, cerebral ischaemia or haemorrhage were excluded. Patient 1 died on day 25 post transplant while comatose. Patient 2 recovered from her comatose state 18 days after transplantation. To our knowledge this is the first report on a possible role of high-dose melphalan in the development of encephalopathy.
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2/74. Systemic lupus erythematosus with C1q deficiency.

    We report a case of systemic lupus erythematosus associated with C1q deficiency. Our patient presented at the age of 6 years with cutaneous lupus. She later developed Raynaud's phenomenon, non-scarring alopecia, oral ulceration and grand mal seizures due to cerebral vasculitis. complement c3 and C4 levels were consistently normal during flares of her lupus and haemolytic activity of her complement was absent, suggesting a deficiency of an early component of the complement cascade. No C1q could be detected.
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3/74. New-onset tic disorder following acute hemorrhage of an arteriovenous malformation.

    The etiology of tic disorder includes idiopathic, postencephalitic, head injury, carbon monoxide poisoning, stroke, and developmental syndromes. We report a case of new-onset complex motor and vocal tics that began after hemorrhage of an arteriovenous malformation located in the left frontal lobe. We have found no reported cases of new-onset tics related to arteriovenous malformations or hemorrhage into the frontal lobes. The patient is a 16-year-old right-hand-dominant boy who presented with generalized tonic-clonic seizures. Evaluation, including magnetic resonance imaging, revealed a left frontal arteriovenous malformation, confirmed by angiogram. Following resection, there was an intraparenchymal hemorrhage of the left frontal lobe with intraventricular hemorrhage, noted most prominently in the left lateral and IIIrd ventricles, and a subdural hematoma caudal to the craniotomy. The postoperative course was complicated by hemiparesis and global aphasia. During recovery, the patient developed what was thought to be a complex partial seizure evidenced by head turning to the right with vocalization and left upper extremity clonic jerks. These were brief and occurred multiple times per day. A trial of carbamazepine was given with no improvement. It was noted that the spells occurred more frequently under stress, as when the patient was frustrated with communication. The diagnosis was changed to complex motor tics and the therapy changed to clonidine. The tics subsequently improved by 80%, although they were still present. We believe the development of complex motor tics due to frontal hemorrhage represents a unique etiology and could complicate postsurgical recovery in similar cases.
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keywords = ventricle
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4/74. Disconnective hemispherectomy.

    hemispherectomy is a valuable procedure in the management of seizure disorders caused by unilateral hemispheric disease. Modifications to anatomical hemispherectomy have been proposed to reduce the incidence of superficial cerebral hemosiderosis and hydrocephalus while still achieving seizure control. We report on the modification of a previously described disconnective form of hemispherectomy. We used this procedure on 2 children, with the aid of stereotactic navigation in 1 of the 2 cases. This disconnection was achieved via a transventricular route with minimal cortical resection or disruption of the blood supply. Over the 20 months of follow-up, 1 patient achieved complete seizure control, and 1 patient achieved control of previously incapacitating seizures with few minor seizures persisting. Motor function and speech significantly improved in both patients. Blood loss during the two procedures was significantly less than that reported for anatomical hemispherectomy, and so far there have been no signs of postoperative complications. The hospital stay was limited to 7-14 days after surgery.
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5/74. Successful treatment of acute promyelocytic leukaemia during pregnancy.

    A case is reported of a pregnant 16-year-old-woman diagnosed with Acute promyelocytic leukaemia (APL) at 25 weeks gestation and treated with all-trans retinoic acid (ATRA) (45 mg/m2) for 25 days in combination with chemotherapy. She achieved a complete cytogenetic and molecular remission. Clinical course was complicated, with an intracerebral bleed, respiratory failure requiring ventilation and prolonged pancytopenia following initial chemotherapy. A live female infant was born at 28 weeks gestation who survived to discharge with significant pulmonary complications. She remains oxygen dependent at 6 months of age. ATRA has been used from the 3rd week of gestation, but fetal malformations are common during the first trimester. In contrast it seems to be safe in the second and third trimesters with regard to teratogenesis but can cause other side-effects. Most successful outcomes in treatment of APL during pregnancy are seen after treatment with ATRA and delivery of the baby at as late a stage as possible. Pregnancies terminated before remission has been obtained or those treated in the first trimester have a poor maternal outcome.
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keywords = cerebral
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6/74. Cerebral lupus in patients whilst on treatment for lupus nephritis with cyclosporine.

    Two young female patients who were treated for severe lupus nephritis with cyclosporine, developed, while the renal parameters remained stable, signs of cerebral lupus (bilateral papiloedema and cranial nerve palsy in one patient and grand mal seizures in the second). The first patient responded to plasmapheresis and i.v. cyclophosphamide, and the second to temporary increase in prednisone with anti-convulsant medication. Both case histories suggest that cyclosporine, whilst controlling lupus nephritis, was not able to prevent serious attacks of cerebral lupus. This discrepancy might be related to relative inability of the drug to reduce antibody production or to the existence of heterogeneous pathogenetic mechanisms of lupus in different organ systems.
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ranking = 2
keywords = cerebral
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7/74. Linear scleroderma en coup de sabre and brain calcification: is there a pathogenic relationship?

    Extracutaneous calcifications are rare in scleroderma and related conditions. We describe a female patient with linear scleroderma en coup de sabre and a longstanding clinical history of tonic and clonic convulsions. Radiographic study showed extensive cerebral calcifications in the right occipital hemisphere, homolateral to the involved side of her face. This report further suggests a relationship between localized scleroderma and neurological manifestations. Brain imaging studies should be routinely performed in scleroderma patients exhibiting neurological manifestations, especially seizure disorder.
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keywords = cerebral
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8/74. Long lasting impaired cerebral blood flow after ecstasy intoxication.

    Four hours after having taken 10 ecstasy tablets a Grand Mal seizure occurred in a 19-year-old woman followed by coma, hyperthermia, tachycardia, tachypnea, and renal failure. After awakening she was oriented but presented with helplessness, disconcertion, hallucinations, panic attacks, and amnesic syndrome. Computed tomography and magnetic resonance imaging scans of the brain were normal. [99Tc]-hexamethylpropyleneamine oxime (HMPAO)-single photon emission computed tomography (SPECT), 20 days after intoxication, showed reduced, inhomogeneous, supratentorial tracer uptake bilaterally. electroencephalography (EEG) disclosed diffuse slowing and occasionally generalized sharp waves. valproic acid was begun. Except for slight amnesia, neuropsychological deficits had disappeared and [99Tc]-HMPAO-SPECT normalized, 29 days later. Decreased cortical blood flow was explained by vasoconstriction following ecstasy-induced depletion of serotonin.
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ranking = 4
keywords = cerebral
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9/74. Repeated hypoglycemia and cognitive decline. A case report.

    OBJECTIVE: diabetes mellitus has a high incidence in general population and goes by high morbidity by specific micro vascular pathology in the retina, renal glomerul and peripheral nerves. In type 1 DM, intensive therapy can prevent or delay the development of long-term complications associated with DM but hypoglycaemia especially severe hypoglycaemia defined, as a low blood glucose resulting in stupor, seizure, or unconsciousness that precludes self-treatment is a serious threat. Hypoglycaemia that may preferentially harm neurons in the medial temporal region, specifically the hippocampus, is a potential danger for the brain cognitive function which several studies failed to detect any significant effects, whereas others indicated an influence on it. A young diabetic case presented here with severe cognitive defect. Great number of severe hypoglycaemic or hyperglycaemic attacks and convulsion episodes were described in his medical history. RESULTS and CONCLUSION: Neuroradiologic findings on CT and MRI, pointed that global cerebral atrophy that is incompatible with his age. Brain perfusion studies (SPECT, (99m)Tc-labeled HMPAO) also showed that there were severe perfusion defects at superior temporal region and less perfusion defects at gyrus cingulum in frontal region. These regions are related with memory processing. Severe cognitive defect in this patient seems to be closely related these changes and no another reason was found to explain except the repeated severe hypoglycaemic episodes.
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keywords = cerebral
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10/74. 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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