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Query: UMLS:C0085584 (encephalopathy)
 18,178 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A brief description is given of neurofibrillary changes of the paired helical filament type in a variety of chronic neurological diseases. These include subacute sclerosing panencephalitis, lead encephalopathy, tuberous sclerosis, Down syndrome, Hallervorden-Spatz disease, and lipofuscinosis. In these conditions, with the exception of Hallervorden-Spatz disease neurofibrillary changes were previously recognized but paired helical filaments were identified only in some cases. Moreover, in the present series, the age of patients at death was often younger than in previously recorded cases.
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PMID:Alzheimer neurofibrillary tangles in diseases other than senile and presenile dementia. 15

In a series of examinations of more than 1000 psychiatricneurological patients we tried to state the effects of diabetes mellitus on the nervous system. We attempted to diagnose the diabetic metabolic error by means of using the oral glucose tolerance test (100 g glucose), the plasma insulin levels and many other laboratory-technical examinations (altogether about 23 000 individual tests). It was found that 21,6% of the patients had a pathological glucose tolerance, 29.6% of them had a pathological insulin response and 5,5% of them showed a known and manifest diabetes mellitus. The comparison between the number of patients with a metabolic disturbance and the frequency of sick persons to be expected epidemiologically shows that an influence exercised by the diabetic metabolism may be stated in neurological and psychic disturbances of cerebral sclerosis and polyneuropathy. The central syndromes are more frequent than the peripheric ones. Mainly low plasma insulin is present. As a rule, pathological results may be seen more often during pathological glucose tolerance in electroencephalography, pneumencephalography, CSF-examinations, personality and intelligence states. This suggests that a diffuse disturbance upon the nervous system comes from the diabetic metabolism, without any acute metabolic disturbance which we did not find among our patients. This disturbance is evident clinically as so-called diabetic polyneuropathy and also encephalopathy. In opposition to that very often pathological changes can be seen by technical research methods in other neurological and psychiatric diagnoses without influencing essentially the autonomous progress of the psychiatricneurological illness.
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PMID:[Diseases of the central nervous system in diabetes mellitus]. 61 60

A 5-year-old boy with the epidermal nevus syndrome and hemimegalencephaly is reported. He had pigmented nevi on the forehead and neck, and hemihypertrophy of the body from the birth. He developed intractable seizures, mental retardation, and right hemiparesis. His seizure pattern changed from early infantile epileptic encephalopathy to infantile spasms at 2 months of age. Electroencephalograms showed a suppression-burst pattern in the neonatal period, subsequently changing to hypsarrhythmia. Computerized tomography of the brain disclosed slight dilatation of the posterior horn of the lateral ventricle at the age of 2 months. Later, hemimegalencephaly with calcification on the left side of the brain was noted. Histological examination of the pigmented nevus on the neck showed it to be an acanthosis nigricans-like lesion. Clinical differences between tuberous sclerosis and epidermal nevus syndrome with hemimegalencephaly are discussed.
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PMID:Epidermal nevus syndrome with hemimegalencephaly: a clinical report of a case with acanthosis nigricans-like nevi on the face and neck, hemimegalencephaly, and hemihypertrophy of the body. 275 Oct 68

The possible roles of pertussis immunisation and of other factors in the aetiology of infantile spasms were investigated by analysis of 269 cases reported to the National Childhood Encephalopathy Study. In 34% of the cases an antecedent factor which may have caused infantile spasms was identified; the commonest of these were perinatal hypoxia (38 cases) and tuberous sclerosis (16 cases). Case-control analyses showed no significant association between infantile spasms and pertussis immunisation in the 28 days before onset. There was, however, some clustering of cases immunised with either diphtheria-tetanuspertussis or diphtheria-tetanus vaccines in the 7 days before onset. The excess compared with controls was compensated for by a corresponding deficit over the remaining period up to 28 days. It is suggested that these vaccines do not cause infantile spasms but may trigger their onset in those children in whom the disorder is destined to develop.
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PMID:Infantile spasms and pertussis immunisation. 613 70

The neurodegenerative diseases of infancy and childhood include disorders in which there is progressive loss of neurological function due to structural abnormalities of the central nervous system. Well over six hundred disorders, many of which are rarely seen, can be included in this category. Yet, the conditions represent collectively over one-fourth of all admissions to pediatric neurology services. Five-year samples of admission characteristics of 1218 patients from two medical centers over twenty-two years permit an estimate of the frequency of the neurodegenerative diseases. The six most-encountered diagnoses, in declining order, were: subacute sclerosing panencephalitis; neuronal ceroid lipofuscinosis; tuberous sclerosis with degeneration; West disease, or idiopathic degenerative encephalopathy associated with infantile spasms; Werdnig-Hoffmann disease, and hereditary spastic paraplegia. A classification is offered grouping the neurodegenerative disorders into five major categories: polioencephalopathies, leukoencephalopathies, corencephalopathies, spinocerebellopathies, and diffuse encephalopathies. Disorders in each subgroup may be either genetic or nongenetic. Neurodegenerative diseases have multiple causes, including metabolic, viral, immunopathic, environmental, and epileptogenic. The cause of many remains unknown.
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PMID:Neurodegenerative diseases of infancy and childhood. 630 58

The results obtained in 151 children who underwent C.T. scan at the Pediatric Department of the University of Padova are reported. The high incidence of negative or aspecific results in the cases of static encephalopathy, in primary generalized epilepsy and in acute diffuse cerebral infectious process is emphasized. C.T. confirmed its high diagnostic value in tuberous sclerosis and cerebral abscess. While non providing the precise diagnosis it may be helpful in some demyelinating diseases. In secondary generalized epilepsy and in partial epilepsy C.T. scan may suggest the prognosis and a possible surgical approach. On the basis of these results and of other published reports a greater selectivity in the use of C.T. is strongly recommended.
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PMID:[Value and limits of C.T. scan in neurologics diseases in infancy and childhood (author's transl)]. 734 53

We investigated the etiology of West syndrome (WS) with special reference to prenatal factors in 180 cases. Prenatal cause was the most frequent diagnosis (77 cases, 42.8%), followed by perinatal (25 cases, 13.9%) and postnatal factors (12 cases, 6.7%); 48 cases (26.7%) were of uncertain etiology; eighteen cases (10.0%) were idiopathic. Of the three forms of age-dependent epileptic encephalopathy, prenatal cause was present in 12 of 15 cases (80.0%) of early-infantile epileptic encephalopathy with suppression-burst, 77 of 180 cases (42.8%) of WS, and 31 of 123 cases (25.2%) of Lennox-Gastaut syndrome (LGS). Prenatal factors of WS included tuberous sclerosis (23), chromosome abnormalities (10), cerebral dysgenesis (10), porencephaly (7), hydrocephalus (5), Aicardi syndrome (3), Aicardi syndrome associated with chromosome abnormality (1), and other causes (18). Chromosome abnormalities with WS consisted of 6 cases with 21 trisomy and one case each with 18q duplication, t(1;y) translocation, 7q duplication, and partial 2p trisomy. One patient with Aicardi syndrome also had a t(12;21) translocation. No significant difference was observed in the age of onset of WS among the five etiologic groups. The evolution from WS to LGS was not influenced by etiology, except for the idiopathic group. In patients followed for over 3 years, seizure remission occurred in 46.8% (22 of 47 cases) of the prenatal group. This was lower than the other four groups. Intellectual prognosis was also relatively poor in those with prenatal onset. Pyridoxal phosphate (PAL-P) treatment was effective in 9 of 70 (12.9%) prenatal cases and 5 of 18 (27.8%) idiopathic cases.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Prenatal etiologies of West syndrome. 833 May 83

To determine the recurrence risk of West syndrome (WS), we studied the familial antecedents of consecutively referred patients. Among siblings, there was an increased incidence of WS but not of febrile convulsions. Familial incidence of epilepsy was intermediate between the epileptic and nonepileptic control groups. When cases resulting from a genetically determined disease were excluded, incidence of epilepsy among siblings was similar to that in normal controls. Five of the 11 familial cases of WS were due to an identifiable cause: twin pregnancy, tuberous sclerosis, and recurrent maternal toxemia. In 4 of the remaining families, the clinical picture included spasms, erratic myoclonus, and postnatal microcephaly, suggestive of a previously unidentifiable progressive encephalopathy. Therefore, when identifiable familial diseases were excluded, the recurrence risk was < 1%.
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PMID:Genetic predisposition to West syndrome. 833 May 85

Eleven newborns with suspected cerebral disease were evaluated using 123I-IMP SPECT. In a normal subject, high uptake was shown in the sensorimotor cortex, thalamus, midbrain-brainstem, and cerebellar vermis. Decreased perfusion was also noted in the frontal lobe. In hypoxic ischemic encephalopathy (HIE), diffuse decreased uptake which showed no redistribution in the white matter was seen in two patients. These two patients had a poor prognosis. In one of the other 4 patients with HIE, persistent defect in parasagittal area was recognized and the patient also had a poor prognosis. In one of two patients with tuberous sclerosis, 123I-IMP SPECT showed high uptake in the area of increased density shown in CT. Thus, 123I-IMP SPECT of newborn has characteristic findings different from the adult. This tracer also might have a prognostic value of clinical improvement following HIE.
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PMID:[Evaluation of newborns with brain disease using 123I-IMP SPECT]. 837 95

Using magnetic resonance imaging (MRI), we studied the myelination of the brains of 8 patients with West syndrome. All cases were symptomatic, 2 having severe asphyxia, 1 lissencephaly, 1 Leigh encephalopathy, 2 tuberous sclerosis, 1 multiple anomalies and 1 microcephaly. Myelination of the pons, cerebellum, thalamus, internal capsules, optic radiation, centrum semiovale and cerebral white matter was separately assessed. The 2 cases with tuberous sclerosis exhibited normal myelination patterns, although asymmetry of the cerebral white matter was noted. These cases had a moderate degree of mental retardation and persistent seizures. The other 6 cases exhibited a marked delay of myelination throughout the central nervous system except for the midbrain. These cases had severe psychomotor retardation and persistent seizures. Although the difference in the outcome may simply reflect the different etiological disorders, these results suggest that the myelination pattern is related to the psychomotor retardation but not to the severity of the seizures in West syndrome. Atrophy of the corpus callosum developed during ACTH therapy and disappeared after the therapy. It was thus suggested that the callosal atrophy caused by the ACTH therapy was reversible.
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PMID:An MRI study of the myelination pattern in West syndrome. 883 97


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