Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0038220 (status epilepticus)
 7,272 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Three types of clinical features at the onset are well known to be characteristic of acute hemiplegia syndrome (AHS). Type 1 comprises status epilepticus of hemiconvulsions with fever. Representative diseases of this type are the infectious diseases of the central nervous system, acute encephalopathy and cerebral vascular diseases. Type 2 comprises status epilepticus of hemiconvulsions without fever. Cerebral vascular diseases and epilepsy are the major ones of this type. Type 3 comprises hemiplegia or hemiparesis of sudden onset without fever or convulsions. Most patients with this type had cerebral vascular diseases, about half of which were moyamoya disease in Japan. Recent progress in neuroimaging studies has allowed considerable elucidation of the etiology of AHS. Gadolinium-enhanced MRI showed minimal lesions such as capsular infarction more clearly than plain MRI. Acetazolamide test 99mTc-HMPAO SPECT imaging is one of the useful assisted diagnostic techniques for moyamoya disease, because it reveals the reserve capacity of the collaterals. [123I]IMP SPECT is useful for the diagnosis and follow-up of acute disseminated encephalomyelitis (ADEM), as the images of the lesions coincide well with the MRI ones. 99mTc-HMPAO SPECT in a case with alternating hemiplegia revealed normoperfusion in the ictal periods. Four cases of AHS are reported here.
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PMID:Acute hemiplegia syndrome in childhood. 805 23

We report a case of a 2-year-old boy with Down syndrome complicated by Moyamoya syndrome who developed extensive multiple cerebral infarction after status epilepticus on the third day of a measles infection. MR angiography revealed the occlusion of the terminal portion of the bilateral internal carotid artery and the basilar artery. Fever, dehydration, activation of coagulation associated with the infection, relatively decreased cerebral blood flow during status epilepticus, and central nervous invasion of the measles virus may have played a role in the development of the infarction. Moyamoya like cerebrovascular lesions occur more often and is more aggressive in children with Down syndrome than in the general pediatric population, and their clinical presentation is always the infarction type. In patients with Down syndrome complicated by Moyamoya syndrome, an early diagnosis and prevention of infarction are important.
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PMID:[A case of Down syndrome with moyamoya syndrome presenting extensive multiple cerebral infarction during measles infection]. 1065 50

We describe the case of a young Hispanic female who presented with thyrotoxicosis with seizures and ischemic stroke. She was diagnosed with a rare vasculopathy - moyamoya syndrome. After starting antithyroid therapy, her neurologic symptoms did not improve. Acute neurosurgical intervention had relieved her symptoms in the immediate post-operative period after re-anastomosis surgery. However, 2 post-operative days later, she was found to be in status epilepticus and in hyperthyroid state. She quickly deteriorated clinically and had expired a few days afterward. This is the second case in literature of a fatality in a patient with moyamoya syndrome and Graves' disease. However, unlike the other case report, our patient had undergone successful revascularization surgery. We believe her underlying non-euthyroid state had potentiated her clinical deterioration. Case studies have shown positive correlation between uncontrolled hyperthyroidism and stroke-like symptoms in moyamoya syndrome. Mostly all patients with these two disease processes become symptomatic in marked hyperthyroid states. Thus, it may be either fluctuations in baseline thyroid function or thyrotoxicosis that potentiate otherwise asymptomatic moyamoya vasculopathy.
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PMID:Fatal outcome in a Hispanic woman with moyamoya syndrome and Graves' disease. 2785 39