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At least 19 recordsLinked to original sources
PubMed1992

The epidemiology of progressive encephalopathies in childhood. I. Live birth prevalence in west Sweden.

Progressive encephalopathies in the west Swedish region were investigated in a population-based study. Cases were allocated to one of five main groups: 1) disorders covered by impairments in subcellular organelles, 2) disorders covered by deficiencies in the intermediate metabolism, 3) biochemically undefined neurometabolic disorders, 4) neuroimmunological disorders and 5) a symptom-orientated miscellaneous group. Progressive encephalopathies were identified in 76 of 132,138 children born alive in the study area during the 16-year-period 1970-85. This gives a live birth prevalence of 0.58 per 1000. In Sweden the size of the group corresponds to that of neural tube defects and that of congenital hydrocephalus. However, a vast number of different disorders are represented.

Adolescent↗
PubMed1978

3-methylglutaconic aciduria: report on a sibship with infantile progressive encephalopathy.

Choreoathetosis, spastic parapareses, dementia and optic atrophy were the main clinical features in a sibship with progressive encephalopathy of late onset. The urine contained constantly elevated amounts of 3-methylglutaric and 3-methylglutaconic acids. The identity of these metabolites was confirmed by synthesis and mass spectrometry. On leucine loading, the excretion of the metabolites was elevated.

Amino Acid Metabolism, Inborn Errors↗
PubMed1978

Acute and chronic progressive encephalopathy due to gasoline sniffing.

Acute encephalopathy caused by gasoline sniffing is well recognized, but has been thought to be completely reversible. We report a patient who developed a progressive encephalopathy characterized by ataxia, tremor and dementia following repeated, deliberate gasoline inhalation. Blood and urine lead levels were consistently elevated and at autopsy, the formalin-fixed brain lead content was between 5200 and 6500 micrograms/100 gm of tissue. This case shows that repeated gasoline sniffing can result in irreversible encephalopathy and that both the acute and chronic encephalopathy probably result from organic lead intoxication and not from the gasoline itself.

Adult↗
PubMed1977

A light and electron microscopic study of experimental portal-systemic (ammonia) encephalopathy. Progression and reversal of the disorder.

A sequential light and electron microscopic study of cerebral cortex was performed in a rat model of portal-systemic encephalopathy produced by creating a portacaval shunt and followed by ammoniate resin feedings. Prior to coma, astrocytes were characterized ultrastructurally by marked cytoplasmic enlargement, proliferation of mitochondria and endoplasmic reticulum, and an accumulation of cytoplasmic glycogen. The Alzheimer type II astrocyte change was seen only in coma and was characterized ultrastructurally by additional hydropic and degenerative mitochondrial and nuclear changes. Attempts at reversal of the encephalopathy were successful only if ammoniated resin feedings were discontinued prior to coma. Results suggest (1) that the astrocyte response initially reflects an ammonia-induced increased metabolic activity in that cell; (2) that subsequently a gliopathy develops having the light microscopic appearance of the Alzheimer type II change; and (3) that the Alzheimer type II astrocyte change may be responsible for an irreversible clinical course in this experimental condition.

Alzheimer Disease↗
PubMed1992

Hartnup syndrome, progressive encephalopathy and allo-albuminaemia. A clinico-pathological case study.

Clinical, biochemical, neuropathological and neurochemical findings in a case of Hartnup syndrome are reported. After initially normal development, the affected girl suffered progressive neuropsychiatric decline with statomotor and mental retardation and intractable seizures and died at the age of 2 years. Postmortem neuropathological and neurochemical investigations showed a combination of extensive neuronal degeneration and cerebral dysmyelination. Pathogenetic hypotheses and the relationship between neuropsychiatric disease and Hartnup syndrome are discussed. Additionally, a fast type bisalbuminaemia present in the girl and her mother is described.

Amino Acids↗
PubMed1976

Progressive dialytic encephalopathy.

A subacutely progressive dialytic encephalopathy lasting for three to 15 months in 11 patients who had been on haemodialysis for 14 to 36 months was characterized by dementia, language disorder, myoclonic jerks, behavioural disturbance, distinctive EEG abnormalities, and normal or nonspecific neuropathological findings.

Adult↗
PubMed1978

Progressive dialysis encephalopathy from dialysate aluminum.

Progressive dialysis encephalopathy was observed as a frequent complication (eight of 34 patients) in a small hemodialysis unit. After the water used for dialysis was deionized, no further cases of encephalopathy developed in 29 patients. In the absence of other apparent changes in therapy, this significant difference in incidence (P less than .005) is considered to be the result of reduction in unusually high water aluminum levels from 0.637 mg/liter to less than 0.001 mg/liter by use of a deionizer.

Adult↗
PubMed1978

Clinical and electroencephalographic changes in progressive uremic encephalopathy.

A study of nine patients, aged 23 to 67 years, showed a remarkable sequence of EEG findings in progressive uremic encephalopathy. The initial characteristics suggested a disorder of subcortical gray matter, followed by involvement of cortical gray matter and finally white matter. The same EEG findings tended to persist in the early stages of the disease and were present throughout the night and during dialysis. During dialysis, the EEG background and clinical picture between paroxysms sometimes showed deterioration. Seizures indicated a grave prognosis. Five of six patients with seizures died. In some patients, progressive uremic encephalopathy may develop without hemodialysis. Routine EEGs in dialysis units or in patients prior to admission can help uncover progressive uremic encephalopathy before the clinical emergence of this disorder.

Brain Diseases, Metabolic↗
PubMed1978

Progressive dialysis encephalopathy.

The clinical features of 42 patients with the only recently recognized and generally fatal neurological syndrome of progressive dialysis encephalopathy are reviewed and the electroencephalographic and neuropathological findings are summarized. Despite apparently successful hemodialysis, these patients develop a wide spectrum of neurological abnormalities. Of these, sudden onset of hesitant, nonfluent speech is the most characteristic and usually the earliest sign. Both dysphasic and dysarthritic elements are found, though the former predominate. Myoclonus, dementia, seizures, and gait difficulty are also seen in the majority of these patients. EEGs are more abnormal than would be expected for the clinical severity, with some type of high-voltage spike-wave pattern intermixed with abundant slow activity. The combination of clinical and EEG features in the appropriate setting is virtually diagnostic. Transient episodes with variable periods of complete or partial remission have been recognized. Neuropathological changes are surprisingly mild and nonspecific. The cause is uncertain; current speculation focuses on aluminum as the offending neurotoxin. Treatment remains unsatisfactory.

Adult↗
PubMed1978

[Progressive myoclonic encephalopathy in dialysis patients. Clinical, electroencephalographic and neuropathological study. Pathogenetic discussion].

Clinical and Neuropathological data on sixteen cases of progressive myoclonic encephalopathy are reported. This neurological syndrome appears after an average duration of thirty two months of haemodialysis and leads to death in four and a half months, and is characterized by myoclonus, speech disorder, epileptic seizures, and mental-status changes. At first, clinical signs and symptoms are related to haemodialysis, later they become permanent. An early diagnosis is based on EEG which is the only useful laboratory test, demonstrating bisynchronous slow-wave bursts. The caracteristic histopathologic findings are neuronal depopulation, lipofuscin accumulation, and appearance of Neurofibrillary degeneration, especially in Motor cortex, red nucleus and dentato-olivary systems. It seems to be justified to attribute P.M.D.E. to aluminium chronic poisonning; the source of the aluminium intoxication is not aluminium containing phosphate-binding gels but intravenously administreted tape-water. The intracellular binding of aluminium is shown from a histochemical study employing fluorescent stain Morin.

Adult↗
PubMedDate not supplied

Effect of aluminum upon conditioned avoidance response acquisition in the absence of neurofibrillary degeneration.

Aluminum induces neurofibrillary degeneration in cats but not rats. Cats develop a progressive encephalopathy in which an early manifestation is impaired learning-memory performance. At brain aluminum concentrations of 5 to 6 times that found in cat, rats demonstrate an initial transient weight loss and acquisition deficit immediately following intracranial injection. However, rats do not develop a progressive encephalopathy or a chronic learning deficit.

Aluminum↗
PubMed1992

Tumor necrosis factor-alpha in pediatric HIV-1 infection.

OBJECTIVE: To evaluate the diagnostic and prognostic value of serum tumor necrosis factor-alpha (TNF-alpha) levels in HIV-1-infected children. DESIGN: Serum levels of TNF-alpha were evaluated in 57 HIV-1-infected symptomatic children aged between 7 months and 8 years. METHODS: TNF-alpha levels were determined by enzyme immunoassay. The sensitivity of the assay was 10 pg/ml. RESULTS: TNF-alpha levels (mean +/- s.d.) were significantly elevated in HIV-1-infected patients (285 +/- 390 pg/ml), compared with HIV-1-uninfected age-matched controls (22.7 +/- 4.9 pg/ml). Among HIV-1-infected children the highest levels of TNF-alpha were noted in those with Mycobacterium avium intracellulare (MAI) infection and those with interstitial lymphoid pneumonitis (LIP). In contrast, patients with Pneumocystis carinii pneumonia, progressive encephalopathy or cachexia did not have markedly elevated TNF-alpha levels. CONCLUSIONS: Serum TNF-alpha is increased in symptomatic HIV-1-infected children, with higher levels in children with LIP or MAI. Serum TNF-alpha levels are not diagnostic for cachexia or progressive encephalopathy.

AIDS Dementia Complex↗
PubMed1975

Diverticulation of the cerebral ventricles: a cause of progressive focal encephalopathy.

Diverticulation of the lateral ventricles of the brain has received little attention in the literature on hydrocephalus. From clinical observations on one child and one adult with diverticulation, it is apparent that force of CSF within the diverticulum is greater than that against the normal parts of the ventricular wall. In both cases, regression of focal symptoms occurred after insertion of a shunt.

Brain Damage, Chronic↗