Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0036572 (seizures)
 80,221 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We have observed an increasing number of autopsies on patients with chemotherapy-related complications. One complication is toxic leukoencephalopathy, which is due to a direct toxic effect of chemotherapeutic agents on the central nervous system white matter. Autopsies of four cases of toxic leukoencephalopathy were performed following standard protocols. The brain and spinal cord were examined routinely, and histological sections were taken for evaluation. We report here three patients with hematologic malignancies and one patient with metastatic carcinoma with chemotherapy-induced leukoencephalopathy. The first was a 56-year-old male treated with multiple chemotherapeutics for multiple myeloma. He presented with confusion and focal seizures with a rapid progression to coma and decerebrate posturing. The second was a 36-year-old male who developed mental status changes, ataxia and dysarthria following treatment for lymphoma. The third was a 16-year-old male who developed a profound peripheral and central neuropathy after chemotherapy treatment for T-cell acute lymphoblastic leukemia. The fourth was a 49-year-old female patient who was treated with multiple chemotherapeutics for Stage II breast carcinoma and subsequently developed visual acuity and field defects. The neuropathologic findings in these cases, although similar, varied in severity and distribution. The white matter was affected by severe myelin pallor, edema, and a prominent macrophage infiltrate in each of the cases. The location and extent of the central nervous system pathology correlated with the type and severity of clinical symptoms. These four cases, with their varied presenting symptoms, clinical courses, and degree of pathology, emphasize the importance of considering toxic leukoencephalopathy as an etiology of acute neurologic deterioration following high-dose chemotherapy.
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PMID:Chemotherapy-induced toxic leukoencephalopathy causes a wide range of symptoms: a series of four autopsies. 1470 18

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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PMID:Postpartum blindness: two cases. 1474 15

Epileptic nystagmus (EN) is a rare form of rhythmic eye oscillations occurring during seizure activity. Not only convulsive states, but also nonconvulsive status may represent with EN and this phenomenon may be the only motor manifestation of seizure activity. Epileptic activation of a cortical saccade region may be distinguished from activation of a cortical pursuit region clinically as activation of pursuit regions results in nystagmus slow phases that bring the eyes across the midline. Horizontal EN results most commonly from seizure activity involving the occipital cortex. In this report, horizontal EN in a patient with nonconvulsive status epilepticus (NCSE) is described with clinical, radiological and electrophysiological findings that occur probably due to posterior leukoencephalopathy syndrome.
Seizure 2004 Apr
PMID:Epileptic nystagmus in a patient with nonconvulsive status epilepticus. 1501 57

Alexander disease is a leukoencephalopathy that usually presents during infancy with developmental delay, macrocephaly and seizures. Several sequencing analyses have identified mutations in the gene encoding glial fibrillary acidic protein (GFAP) of patients with Alexander disease. We described a girl who developed seizures in infancy with atypical CT findings and in whom a novel heterozygous mutation, L90P (283T --> C), was detected in exon 1 of the GFAP gene. The neurological deterioration was mild and appeared relatively late for infantile onset.
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PMID:A case of infantile Alexander disease with a milder phenotype and a novel GFAP mutation, L90P. 1503 Sep 11

Canavan disease (CD) is a rare autosomal recessive genetic disorder characterized by early onset progressive spongy degeneration of the brain involving the axon's myelin sheath. Patients with CD have leukoencephalopathy and megalencephaly; clinically they show a variable course ranging from slow neurodegenerative course to no neurological development or rapid regression. Current treatment is symptomatic including management of seizures and spasticity. Topiramate (TPM) is a novel antiepileptic drug for treatment of a broad spectrum of seizure types in adults and children. We used TPM in two of our patients diagnosed with CD at six months of age. At seven months and 15 months' follow-up, respectively, each patient showed a decrease in head growth velocity. We suggest that TPM can be used in patients with CD and possibly in other childhood neurodegenerative diseases with leukoencephalopathy and megalencephaly. Further studies are required to reveal the underlying mechanisms that lead to decreased head growth velocity, and to conclude whether this ameliorates the clinical course of CD.
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PMID:Effect of topiramate on enlargement of head in Canavan disease: a new option for treatment of megalencephaly. 1507 77

The terms posterior reversible leukoencephalopathy, reversible posterior cerebral edema syndrome, and posterior reversible encephalopathy syndrome (PRES) all refer to a clinicoradiologic entity characterized by headaches, confusion, visual disturbances, seizures, and posterior transient changes on neuroimaging. Clinical findings are not sufficiently specific to readily establish the diagnosis; in contrast, magnetic resonance imaging pattern is often characteristic and represents an essential component of the diagnosis of PRES. Typical lesions predominate in the posterior white matter, with some involvement of the overlying cortex; are hyperintense on T2-weighted images; and are usually hypointense or isointense on diffusion-weighted images, with an increase of the apparent diffusion coefficient, indicating vasogenic edema. The pathogenesis is incompletely understood, although it seems to be related to the breakthrough of autoregulation and endothelial dysfunction. Since its initial description, this syndrome has been subsequently described in an increasing number of medical conditions, including hypertensive encephalopathy, eclampsia, and the use of cytotoxic and immunosuppressive drugs. The diagnosis has important therapeutic and prognostic implications because the reversibility of the clinical and radiologic abnormalities is contingent on the prompt control of blood pressure and/or discontinuing the offending drug. On the contrary, when unrecognized, conversion to irreversible cytotoxic edema may occur.
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PMID:Neuroimaging in posterior reversible encephalopathy syndrome. 1509 52

We report two hypertensive females, one suffering from chronic renal failure and on regular dialysis, the other with eclampsia. Both developed new onset seizures. The patients' MRI of brain showed signals consistent with the reversible posterior leukoencephalopathy syndrome (RPLES), but with the signals seen only in the hemisphere where the seizures predominated. The anatomic correlation of the clinical and imaging findings supports the notion that seizures have a major role in the genesis and evolution of RPLES.
Seizure 2004 Jun
PMID:The role of seizures in reversible posterior leukoencephalopathy. 1512 Nov 39

The leukoencephalopathy induced by tacrolimus is increasingly recognised as an important cause of neurological complications after transplantation. Magnetic resonance imaging (MRI) is of major help in the differential diagnosis of infection or vascular injury. We describe two children with coma and seizures after transplantation, in whom the results of MRI with FLAIR (fluid-attenuated inversion-recovery) and DWI (diffusion-weighted images) were the main positive elements for the diagnosis of drug-induced toxicity. The results of DWI favoured the role of oedema and/or demyelination in the pathophysiology of this disorder. Unlike other reported patients, in whom all symptoms resolved, lesions persisted, albeit improved, on the control MRI, and both children demonstrated learning disabilities after several years of follow-up.
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PMID:Magnetic resonance imaging for the diagnosis of acute leukoencephalopathy in children treated with tacrolimus. 1512 13

We report a pair of brothers who have leukoencephalopathy, arthritis, colitis, and hypogammaglobulinemia. Both presented initially with seizures in the early postnatal period. They have significant developmental delay, and brain MRIs demonstrate leukoencephalopathy, characterized by profound hypomyelination. They have developed arthritis, which in one brother has required chronic treatment; and persistent intermittent diarrhea, necessitating treatment for inflammatory bowel disease. Finally, they have had multiple hospitalizations, including several for sepsis; an immunological analysis revealed that they have IgG1-subclass hypogammaglobulinemia and low B cell levels. There is no family history of similar problems, and these brothers have an unaffected brother. We believe this constellation of symptoms represents a novel syndrome: LACH syndrome (leukoencephalopathy, arthritis, colitis, and hypogammaglobulinemia). The etiology of this syndrome remains unknown despite extensive investigations.
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PMID:Leukoencephalopathy, arthritis, colitis, and hypogammaglobulinemia (LACH) in two brothers: a novel syndrome? 1521 57

A cerebral arteriopathy with subcortical infarcts and leukoencephalopathy is described with a pedigree suggestive for an autosomal dominant condition. In contrast to the vasculopathy designated with the acronym CADASIL, no deposits of granular osmiophilic material were detected in the vasculature and no point mutations in the NOTCH 3 gene were found. The disease occurred in a family living near Hamburg, Germany, and affected 11 women and 11 men over the last six generations. Onset of the disease was between the age of 12 and 50. Clinical symptoms included gait disturbances, dysarthria, sensomotoric deficits and a progressive dementia. Migraine-like complaints and epileptic seizures were observed in one case each. Cranial computer tomography and magnetic resonance imaging scans showed large confluent areas with decreased density in the white matter and small necroses in the brain stem, the basal ganglia and the white matter. A correlation with factors predisposing for vascular diseases could not be demonstrated. In five cases an autopsy was performed which disclosed an angiopathy affecting predominantly the penetrating arteries with consecutive lacunar infarcts, diffuse demyelination and rarefication of the subcortical white matter and degeneration of the pyramidal tracts. Histologically, the vessels showed concentric and excentric intimal proliferation, an elastosis and hyalinosis, splitting of the lamina elastica interna and a degeneration of the tunica muscularis. Electron microscopy revealed fragmentation and thickening of the basal lamina but electron-dense granules characteristic for CADASIL were not detected.
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PMID:Subcortical angiopathic encephalopathy in a German kindred suggests an autosomal dominant disorder distinct from CADASIL. 1522 37


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