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Ohtahara syndrome (OS), also known as Early Infantile Developmental & Epileptic Encephalopathy (EIDEE) [2] is a progressive epileptic encephalopathy.The syndrome is outwardly characterized by tonic spasms and partial seizures within the first few months of life, [3] and receives its more elaborate name from the pattern of burst activity on an electroencephalogram (EEG).
In a prior study, 20 of 31 patients became seizure-free with anti-seizure medication, and valproic acid was the most effective drug. Lamotrigine and ethosuximide also showed success. [3] There are recognised "rescue therapies" for seizures, medications given quickly while a seizure occurs. [5] Such treatment may reduce or prevent serial ...
KCNQ2 encephalopathy typically presents with tonic seizures from the first week of life. The seizures can be frequent and often difficult to treat. Seizures can resolve within months or years but can impair the development of several domains such as motor, social, cognitive and language. [1]
Vitamin B12 deficiency was reported to be the cause of seizures for adults [9] [10] and for infants. [11] [12] Folic acid in large amounts was considered to potentially counteract the antiseizure effects of antiepileptic drugs and increase the seizure frequency in some children, although that concern is no longer held by epileptologists. [13]
Syndromes are characterized into 4 groups based on epilepsy type: [1] a. Generalized onset epilepsy syndromes. These epilepsy syndromes have only generalized-onset seizures and include both the idiopathic generalized epilepsies (specifically childhood absence epilepsy, juvenile absence epilepsy, juvenile myoclonic epilepsy and epilepsy with generalized tonic- clonic seizures alone), as well as ...
Anti-gamma-aminobutyric acid A receptor (anti-GABA-AR) encephalitis was first reported in 2014 in six patients (two male children, one female teenager and three male adults). They developed a rapidly progressive encephalopathy with early behavioral or cognitive changes that evolved with refractory seizures and multifocal lesions as seen on ...
Rasmussen syndrome or Rasmussen's encephalitis is a rare inflammatory neurological disease, characterized by frequent and severe seizures, loss of motor skills and speech, hemiparesis (weakness on one side of the body), encephalitis (inflammation of the brain), and dementia.
Epilepsy-intellectual disability in females also known as PCDH19 gene-related epilepsy or epileptic encephalopathy, early infantile, 9 (EIEE9), is a rare type of epilepsy that affects predominantly females and is characterized by clusters of brief seizures, which start in infancy or early childhood, and is occasionally accompanied by varying degrees of cognitive impairment.