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Query: UMLS:C0036572 (seizures)
 80,221 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The influence of ACTH on the prognosis of patients with infantile spasms remains controversial. We have examined retrospectively the long-term benefits of initially successful ACTH therapy in patients treated at this institution between 1961 and 1974. Individuals with equivocal or minimal improvement during ACTH therapy were excluded from this study. Eighteen affected infants showed a favorable early response consisting of cessation of seizures for at least 3 weeks during ACTH therapy and concurrent disappearance of the hypsarhythmic EEG pattern. Modal age at last follow-up was 5 years (range, 15 months to 16 years). Infantile spasms recurred in 7 patients (39%), and 8 patients subsequently had other seizure types. All epileptiform (spike) activity disappeared from the EEGs of 8 patients during ACTH therapy, but in 4 of these cases epileptiform activity was present in later tracings. In the remaining 10 patients the hypsarhythmic pattern disappeared in association with ACTH therapy, but the EEG remained epileptiform (often only in sleep). Later EEGs were free of epileptiform activity in 5 of the 10 patients whose tracings contained spike discharges in the early follow-up period. Four patients (22%) were seizure free and without intellectual impairment when last evaluated.
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PMID:Long-term prognosis of patients with infantile spasms following ACTH therapy. 22 Dec 12

The effects of the thyroid gland upon the nervous system with neurologic-psychiatric symptoms are discussed. With hyperthyreosis in younger patients hyperactive and psychotic signs occur, in older patients so-called apathic thyreotoxicosis. Neurologically there appear acute and chronic thyreotoxic myopathies, paroxysmal paralyses, the so-called hyperthyreotic chorea and a series of disturbances of eye muscles. In a thyreotoxic crisis psychotic preliminaries may proceed to a coma. With hyperthyreoses there occur intellectual impairment, showing but also psychoses as well as multiple neurological defect syndromes and epileptic seizures. Neurological defects as a consequence of thyreostatic therapy are rare, mostly these are disturbances of periphal nerves.
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PMID:[Psychiatric and neurologic disturbances in thyroid disorders (author's transl)]. 28 Oct 58

Tapetoretinal degeneration is described in two siblings in association with generalized major motor seizures and intellectual impairment. Neither of these patients have the characteristic dysmorphic features or biochemical abnormalities seen in previously described cases, which are reviewed. Inheritance was probably autosomal recessive.
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PMID:Familial tapetoretinal degeneration and epilepsy. 47 18

A retrospective study of the neurological problems arising in HIV-I seropositive patients in a single defined geographical area was undertaken. Ninety patients were referred for a neurological opinion from a total known HIV-I seropositive population of 436. Minor problems were frequently encountered early in the course of disease (20 at CDC stage II, 12 at CDC stage III), including seizures related to drug abuse in six. The most frequent neurological problem in those patients in CDC group IV (58 patients) were the AIDS dementia complex (14 patients), an axonal sensorimotor neuropathy (12), toxoplasmosis (nine) and cryptococcal meningitis (three). All patients with a structural lesion had appropriate focal signs on examination. The value and role of CT cranial scanning in the diagnosis of toxoplasmosis is discussed and the importance of recognizing potentially treatable causes of both intellectual impairment and cytomegalovirus-related neuropathies is stressed. This is the first report of an unselected series of patients at all stages of HIV-I related neurological disease from a single UK centre.
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PMID:The neurological features of HIV-positive patients in Glasgow--a retrospective study of 90 cases. 132 56

We describe eight patients from three families presenting with myoclonus, ataxia, infrequent seizures and minimal intellectual impairment. All were Arabs from different parts of the Arabian peninsula. The new consensus on terminology, genetic and clinical definition of Baltic myoclonus, Ramsay Hunt syndrome and Unverricht-Lundborg disease suggests that our group are best categorised under the term of progressive myoclonic ataxia of the Unverricht-Lundborg type. Moreover, this report reinforces the existence of this syndrome outside Scandinavia.
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PMID:Progressive myoclonic ataxia without ragged red fibres: Unverricht-Lundborg disease vs Ramsay Hunt syndrome. 133 90

Aberrant iron metabolism in the brain is typified by Hallervorden-Spatz syndrome. In this disorder, large amounts of iron are deposited in the globus pallidus and the pars reticulata of the substantia nigra. It is characterized by extrapyramidal dysfunction, as demonstrated by dystonia, rigidity, and choreoathetosis; onset during the first two decades of life; and progression of signs and symptoms. Corroborative findings include corticospinal tract involvement, ie, spasticity and extensor toe signs, progressive intellectual impairment, retinitis pigmentosa and optic atrophy (usually associated visual evoked response and electroretinogram abnormalities), seizures, familial occurrence, hypointense areas in the basal ganglia on magnetic resonance imaging scans (particularly in the substantia nigra), abnormal cytosomes in circulating lymphocytes, and sea-blue histiocytes in bone marrow. Iron function in normal brain metabolism is manifold, but high concentrations of iron in the basal ganglia area may signal a unique relationship. Data support the likelihood that iron plays a role in the modulation of dopamine binding to postsynaptic receptors. In addition, transferrin receptors and iron are also concentrated in oligodendrocytes in normal brain and, thus, may have a function in myelination. A role of iron also seems likely in oxidation and peroxidation reactions involving membranes and DNA, a capability that becomes uncontrolled when protective biologic mechanisms become inadequate.
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PMID:Hallervorden-Spatz syndrome and brain iron metabolism. 184 35

A young boy, aged 5 years 7 months, presented with generalized hypotonia and proximal muscle weakness, and had exhibited delayed motor milestones since birth, He showed talipes planovalgus, a myopathic face, nasal tone vocalization, positive Gowers' sign and decreased tendon reflexes, but there was no intellectual impairment or seizure. The serum creatine kinase level and peripheral nerve conduction velocity, as well as the electromyogram and electrocardiogram, were within normal limits. A biopsy specimen from the left biceps brachii muscle revealed minimal nonspecific changes and mild variations in fiber size with an increased number of undifferentiated type 2C fibers, but no subcellular abnormalities were found on either the histochemical or electron microscopic examinations. The patient was diagnosed as having minimal change myopathy and improved clinically in muscle strength after one year of follow-up.
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PMID:Minimal change myopathy: report of a case. 198 82

Overall, children with epilepsy have poorer concentration and mental processing and are less alert than age-matched controls. The relationship between cognitive functioning and epilepsy is complex, however, with widely differing degrees of intellectual impairment--ranging from minimal to severe and progressive--related to diverse types of epileptic seizures, syndromes, and etiological factors. Prolonged and frequently repeated seizures are typically associated with more severe effects on cognitive functioning, particularly if epilepsy is symptomatic, i.e., secondary to a demonstrable brain lesion. A combination of such factors may contribute to the mental deterioration seen in many children suffering from severe epilepsy.
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PMID:Children with epilepsy: the effect of seizures, syndromes, and etiological factors on cognitive functioning. 212 34

Approximately 10% of children treated with contemporary therapy for acute lymphoblastic leukemia (ALL) will experience an isolated relapse in the central nervous system (CNS). From 5 to 25% of this group will become long-term survivors, but only after additional, more aggressive therapy. A review of the limited number of studies of children surviving treatment for CNS relapse disclosed a strikingly higher incidence of intellectual impairment than was found in similarly treated patients who remain in complete remission. Specific risk factors for this complication included the number of courses of cranial irradiation, a young age at treatment, increasing time since treatment, neuropathological changes apparent on abnormal computed tomography scans of the brain, and seizures. The methodological problems inherent in neurobehavioral research in childhood acute lymphoblastic leukemia are critically reviewed and suggestions for future studies are offered. Children with a CNS relapse should be serially evaluated for psychoeducational performance to facilitate early intervention in cases of learning difficulties.
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PMID:Neuropsychological status of children with acute lymphoblastic leukemia treated for central nervous system relapse. 265 77

In order to characterize neurological, intellectual and behavioral sequelae better after surgical treatment of brain abscess in childhood, 32 children were studied over a 10-year time span after treatment of a brain abscess. Depending on the clinical condition, surgical treatment consisted of simple aspiration through a burr hole or of total excision after craniotomy. There were only 3 postoperative deaths (9%). The scheduled time for long-term follow-up was 3 years after surgery. The patient assessment included information concerning the presence of seizures, accurate neurological examination, an EEG, and a battery of intellectual tests to evaluate the presence of behavioral-mental deficits. Late seizures developed in 7 of the 29 survivors. Five patients presented permanent hemiparesis after surgical treatment. It is noteworthy that the intellectual impairment observed was more severe in younger patients, while the behavioral disturbances were more marked in school-age children.
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PMID:Neurological and behavioral sequelae in children operated on for brain abscess. 275 27


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