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Query: UMLS:C0014547 (focal epilepsy)
 1,627 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Isolated infantile convulsions were noted in 206 patients (13%) of 1,572 patients who underwent cortical resection for medically refractory focal epilepsy at the Montreal Neurological Institute over the period 1928 through 1977. In 59 patients (29% of the 206), the isolated infantile convulsion was associated with an identifiable febrile systemic or neural illness. In 54 patients (26%), there was a definite history of cerebral birth injury in addition to the isolated infantile convulsions. Twenty patients (10%) had some other potential etiological factor for the later development of seizures, such as postnatal head injury, Sturge-Weber syndrome, etc., in addition to the isolated infantile convulsions. In 95 patients, 6% of the total 1,572 patient series and 41% of those with isolated infantile convulsions, the latter was the sole apparent cause for the later development of recurring seizures. Although this was the case in a small but significant percentage in this series of patients, more frequently, the isolated infantile convulsion represents a manifestation of invasion of the brain by an infectious agent of a systemic or brain disease, or is a harbinger of the later development of a seizure tendency due to some other preexisting etiological factor.
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PMID:Relative significance of isolated infantile convulsions as a primary cause of focal epilepsy. 11 7

We report 3 cases of epilepsy with bilateral occipital calcifications followed up for several years. These cases were compared with 21 published cases and were found to differ from the classical Sturge-Weber syndrome on several points: 1) the disease appeared around the age of 5 years and consisted of focal epilepsy without neurological or mental disorders; 2) the epilepsy was easy to control during 2 to 5 years. This was followed by a diffuse encephalopathy with severe, treatment-resistant epilepsy, Gerstmann's syndrome, optic ataxia, cerebellar syndrome and slow activity at EEG. It appears from these 3 cases that: 1) occipital calcifications may be unilateral at the onset of the disease; 2) visual evoked potentials are affected at a late stage, and 3) CT scans are of considerable value in the prognosis of benign epilepsy in childhood.
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PMID:[Epilepsy and bilateral occipital calcifications: 3 cases]. 219 37

We studied four patients with a focal epilepsy and bilateral occipital corticosubcortical calcifications without any sign of phakomatosis. The clinical course of the disease was similar in all the patients and evolved from a benign onset to a severe encephalopathy with progressive mental impairment. The question of whether these patients have an incomplete and atypical form of Sturge-Weber syndrome or a previously undescribed disorder is addressed.
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PMID:Epilepsy with bilateral occipital calcifications: a benign onset with progressive severity. 336 73

Two infants with severe drug refractory focal epilepsy caused by Sturge-Weber syndrome and extensive cerebral leptomeningeal angiomatosis were referred for preoperative video-electroencephalographic evaluation. Brain imaging with computed tomography and gadolinium-enhanced magnetic resonance imaging demonstrated bilateral disease in both children with a predominance of involvement of one hemisphere. Clinical examination and neurophysiology with ictal video recording demonstrated epileptogenesis from one hemisphere. Successful surgical treatment with functional hemispherectomy was followed by good long-term seizure control in both patients. The dramatic seizure control was accompanied by markedly improved quality of life for the family and children. These cases indicate that the spectrum of children that may benefit from epilepsy surgery should not be viewed too restrictively, and subsets of children with localization related epilepsy caused by extensive lesions may be resective surgical candidates with a good seizure outcome prognosis.
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PMID:Epilepsy surgery in bilateral Sturge-Weber syndrome. 1205 3

Parry-Romberg syndrome is a rare neurocutaneous syndrome characterized by progressive shrinkage and degeneration of the tissues beneath the skin, usually on one side of the face. It is associated with neurological disorders such as trigeminal neuralgia, facial paresthesia, headache, and focal epilepsy. Concomitant occurrence of discoid lupus erythematosus (DLE) and morphea in the same skin lesion is exceptional, defined as overlap syndrome with two or more different connective tissue disease concurrently or consecutively. A 32-year-old female developed DLE on a long-standing lesion of scleroderma over left temporal area with characteristics histopathological changes. She was treated with oral antimalarials and steroids which halted the progress of the disease.
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PMID:Parry-romberg syndrome: a linear variant of scleroderma with discoid lupus erythematosus on scalp - an association. 2519 Oct 42

This review updates the radiologist on current epilepsy surgery practice in children, with a specific focus on the role of imaging in pre-surgical work-up, current and novel surgical techniques, expected post-surgical imaging appearances and important post-operative complications. A comprehensive review of the current and emerging international practices in paediatric epilepsy surgical planning and post-operative imaging is provided with details on case-based radiological findings. A detailed discussion of the pathophysiology and imaging features of different epileptogenic lesions will not be discussed as this is not the objective of this paper. Epilepsy surgery can be an effective method to control seizures in certain children with drug-resistant focal epilepsy. Early surgery in selected appropriate cases can lead to improved cognitive and developmental outcome. Advances in neurosurgical techniques, imaging and neuroanaesthesia have driven a parallel expansion in the array of epilepsy conditions which are potentially treatable with surgery. The range of surgical options is now wide, including minimally invasive ablative procedures for small lesions such as hypothalamic hamartomata, resections for focal lesions like hippocampal sclerosis and complex disconnective surgeries for multilobar conditions like Sturge Weber Syndrome and diffuse cortical malformations. An awareness of the surgical thinking when planning epilepsy surgery in children, and the practical knowledge of the operative steps involved will promote more accurate radiology reporting of the post-operative scan.
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PMID:Epilepsy surgery in children: what the radiologist needs to know. 3243 87