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

A patient with uncontrolled posttraumatic epilepsy and acute intermittent prophyria was subjected to successive therapeutic trials with phenytoin, carbamazepine, and clonazepam, while eating an adequate diet. Both phenytoin and carbamazepine treatments caused significant increases in porphobilinogen excretion and appeared to induce acute porphyric attacks. In contrast, treatment with clonazepam under rigid dietary control for 10 days caused no increase in porphilbinogen excretion. During the subsequent 7 months of treatment with clonazepam, neither seizures nor porphyric attacks recurred. These findings suggest that clonazepam may be a safe and effective treatment for chronic or severe generalized seizure disorders in patients with acute intermittent porphyria.
 ...
PMID:Posttraumatic epilepsy and acute intermittent porphyria: effects of phenytoin, carbamazepine, and clonazepam. 9 34

Sudden permanent blindness of cerebral origin, in addition to severe abdominal pain, hypertension, convulsions, and peripheral neuropathy developed in a 21-year-old woman, a victim of acute intermittent porphyria. Findings of the pathological examination of the brain showed extensive infarction in both occipital lobes. The pathological changes were consistent with anoxia. We discuss and review the literature of the possibility of "vasospasm" of both posterior cerebral arteries. Follow-up studies with serial EEG showed either focal epileptogenic activity or diffuse slow waves. The most consistent epileptic discharges were found in the occipital regions. The favorable response to the treatment of seizures with carbamazepine in this patient might encourage further clinical trials.
 ...
PMID:Blindness of cerebral origin in acute intermittent porphyria. Report of a case and postmortem examination. 19 74

A 36-year-old white man had both acute intermittent porphyria and long-standing idiopathic grand mal seizures. Diphenylhydantoin apparently adversely affected both the clinical and biochemical parameters of the acute intermittent porphyria. Comparison of urinary levels of the porphyrin precursors, delta aminolevulinic acid and porphobilinogen, under controlled diet conditions before and after withdrawal of diphenylhydantoin, showed that this drug accounted for approximately one-half of the porphyrin precursor excretion. Significant clinical improvement of the porphyria followed withdrawal of the diphenylhydantoin. Bromides appeared to be approximately as effective as diphenylhydantoin for seizure control in this patient.
 ...
PMID:Grand mal seizures and acute intermittent porphyria. The problem of differential diagnosis and treatment. 81 8

Report on clinical and electrophysiological findings in four members of a family with acute intermittent porphyria in the remission period. One patient had suffered from repeated epileptic seizures of the grand-mal type since the age of 24 years. Generalized and multifocal epileptic potentials were found in her EEG. Two other members of the family, a man and a woman, were found electromyographically and neurographically to have a florid neuropathy with damage to the axon and the myelin sheath. Only the female patient showed manifest clinical signs of the polyneuropathy. The 4th member, who years previously had had abdominal colics and suspect biochemical signs of acute intermettent porphyria, was not striking either neurologically or in electromyographic and neurographic polyneuropathy screening at the time of the examination. Epileptic seizures and the symptoms of the polyneuropathy had a close connection with the menstrual cycle (two cases). Before and at the beginning of the menstruation a deterioration of the disease was observed. Problems of antiepileptic therapy in acute intermittent porphyria are discussed.
 ...
PMID:[Polyneuropathy and epileptic seizures in a family with acute intermittent porphyria]. 91 35

A young woman with acute intermittent porphyria (AIP) and juvenile myoclonic epilepsy began to have generalized tonic-clonic seizures (GTCs) at age 13. Subsequently, she had myoclonic seizures, which were often precipitated by visual stimulation, tended to occur in the morning, and sometimes evolved into GTCs. Valproate (VPA) resulted in a worsening of latent AIP, and treatment with a combination of phenytoin (PHT), carbamazepine (CBZ), and clonazepam (CZP) led to severe neuropathy of AIP and an electrolyte imbalance. These conditions were improved by water restriction, infusion of high doses of carbohydrates, and discontinuation of all antiepileptic drugs (AEDs) except for CZP. CZP appeared to be effective both in improving GTCs and myoclonic seizures and did not induce any symptoms of AIP. CZP may be porphyrogenic but can be used safely at a low dose for treatment of epilepsy in patients with AIP.
 ...
PMID:Acute intermittent porphyria and epilepsy: safety of clonazepam. 173 41

A 20-year-old woman had an attack of acute intermittent porphyria (AIP) with seizures and hallucinations. MRI revealed multiple lesions in both hemispheres. Both the cerebral clinical abnormalities and the MRI lesions resolved following treatment. These findings suggest that a vascular mechanism may underlie the pathogenesis of cerebral dysfunction in AIP.
 ...
PMID:MRI reveals multiple reversible cerebral lesions in an attack of acute intermittent porphyria. 805 73

We report a 16-year-old girl with acute intermittent porphyria who had abdominal pain, generalized tonic-clonic and simple partial seizures, and inappropriate antidiuretic hormone secretion. Because most antiepileptic drugs are contraindicated in porphyria, she was treated with magnesium sulfate i.v. Soon after starting treatment, seizures stopped, returned, and then again responded in several trials with discontinuation and reinstitution of i.v. magnesium sulfate. Our experience encourages the use of magnesium sulfate for treatment of seizures in patients with porphyria.
 ...
PMID:Treatment of porphyric convulsions with magnesium sulfate. 191 81

A case of acute intermittent porphyria in a 10-year-old boy with seizures and hypercholesterolemia is presented. The problems of management when seizures and porphyria coincide and discussion of hypercholesterolemia are included. A comprehensive review of the world literature reveals that prepubertal patients with acute intermittent porphyria are predominantly male and show an increased incidence of seizures when compared to older age groups. The principal clinical features in all age groups include abdominal pain, vomiting, fever, and tachycardia in addition to mental changes, limb paresis, and hyporeflexia.
 ...
PMID:Juvenile acute intermittent porphyria with hypercholesterolemia and epilepsy: a case report and review of the literature. 359 6

delta-Aminolevulinic acid, an intermediate in heme formation, is elevated in certain human disorders including acute intermittent porphyria, tyrosinemia, and lead poisoning. It has been implicated in the central nervous system manifestations of these disorders via interactions with the GABAergic system. This potential interaction was examined by testing whether or not delta-aminolevulinic acid could alter the latency to seizure in mice. Seizures were induced in a variety of inbred strains of mice including C57BL, C3H, DBA mice and in a heterogeneous stock of mice. Flurothyl and 3-mercaptopropionic acid were used to induce seizures in the presence and absence of delta-aminolevulinic acid administered either i.p. (0.5 and 1.5 mmol/kg), or i.c.v. (4.5 and 450 nmol). delta-Aminolevulinic acid increased the latency to myoclonic and clonic seizures induced by flurothyl when administered i.p.; i.c.v. injections also delayed clonic seizures induced by flurothyl, and increased the latency to tonic seizures induced by 3-mercaptopropionic acid. The degree to which delta-aminolevulinic acid altered seizure latency in all tests was dependent on strain of mouse tested. These data support the conclusion that delta-aminolevulinic acid can act as an anticonvulsant agent, and mimic the effects of GABA. Moreover, there is genetic variation in the sensitivity of the various strains of mice to delta-aminolevulinic acid.
 ...
PMID:Genetic differences in the effects of delta-aminolevulinic acid on seizure latency in mice. 377 Jan 19

Seizures may occur in acute intermittent porphyria or other hepatic porphyrias. Management is difficult, because barbiturates and hydantoins exacerbate the porphyric state. We studied one patient with major motor seizures and acute intermittent porphyria. The seizure disorder was exacerbated by phenytoin and did not respond to a high-carbohydrate diet or to intravenous hematin. Clonazepam was ineffective in treating the seizures and, in high doses, seemed to exacerbate the porphyria. Both clonazepam and valproate were porphyrinogenic in experimental test systems. Because both drugs may exacerbate the acute hepatic porphyrias, bromide remains the drug of choice to treat these seizures.
 ...
PMID:Seizure management in acute hepatic porphyria: risks of valproate and clonazepam. 677 Feb 87


1 2 3 4 5 6 7 Next >>