Search PubMed⌕ Search

Biomedical subjects

T L Perry

Publications and source records attributed to T L Perry.

At least 109 records · Page 6Linked to original sources
PubMed1980

Failure of aminooxyacetic acid therapy in Huntington disease.

Seven patients with Huntington disease were treated with aminooxyacetic acid (AOAA), an inhibitor of gamma-aminobutyric acid aminotransferase (GABA-T), in an effort to alleviate symptoms by increasing brain GABA content. AOAA was given orally in a placebo-controlled crossover trial in which patients, relatives, and three of the evaluating physicians remained blind. Toxic symptoms occurred in all seven patients when AOAA dosage was increased beyond 2 mg per kilogram per day, and included drowsiness, ataxia, seizures, and psychotic behavior. In five patients who took AOAA for 4 months, no clinical improvement was observed. Biochemical monitoring showed that less inhibition of hepatic GABA-T enzyme activity was achieved than in patients treated with large doses of isoniazid. Results of this trial neither support nor exclude the possible therapeutic usefulness of increasing brain GABA content in Huntington disease.

Acetates↗
PubMed1979

Gamma-aminobutyric-acid deficiency in brain of schizophrenic patients.

Gamma-aminobutyric acid (G.A.B.A.) was measured in the nucleus accumbens and thalamus of brains from patients who had died with schizophrenia or Huntington's chorea (H.C.) and from control subjects. Mean G.A.B.A. content was significantly reduced in both brain areas in schizophrenia and in H.C. Extraneous factors, such as age, interval from death to necropsy, cause of death, and drug use, did not readily explain the observed reduction in brain G.A.B.A. G.A.B.A. deficiency may be a biochemical characteristic of some forms of schizophrenia.

Brain Chemistry↗
PubMed1979

Familial fatal Parkinsonism with alveolar hypoventilation and mental depression.

The clinical, pathological, and neurochemical characteristics of a newly recognized inherited neurological disorder are reported. Lethargy and mental depression are early symptoms, followed by mild parkinsonism and progressive weight loss. Failure of automatic respiratory control develops and may result in sudden death. Advanced degeneration of the substantia nigra, cell loss and gliosis of the basal ganglia, and focal gliosis in the medulla are seen on pathological study. Degeneration of the nigrostriatal dopaminergic system is evidenced by low levels of tyrosine hydroxylase, dopamine, homovanillic acid, and L-dopa decarboxylase in postmortem brain samples. Taurine concentrations in fasting plasma and CSF are somewhat depressed; brain contents of taurine are within normal limits.

Brain↗
PubMed1979

Isoniazid therapy of Huntington disease.

We describe clinical and biochemical changes in seven patients with Huntington disease given isoniazid (INH) in dosages three to five greater than normally used in tuberculosis. Because INH inhibits the enzyme gamma-aminobutyric acid aminotransferase (GABA-T), and increases GABA content in the brains of experimental animals, it might correct the brain GABA deficiency characteristic of Huntington disease. Of six patients treated long enough to be clinically evaluated, one showed marked and two others showed signifciant improvement. High-dose INH therapy carries serious toxic risks, which are influenced by patients' acetylator phenotypes. Nevertheless, results are sufficiently promising to warrant further controlled trials of INH or other GABA-T inhibitors in Huntington disease.

4-Aminobutyrate Transaminase↗
PubMed1978

Amino compounds and organic acids in CSF, plasma, and urine of autistic children.

Amino compounds were measured with an amino acid analyzer in the fasting plasma of 34 patients with childhood psychoses (28 having infantile autism) and 40 control children, and in the cerebrospinal fluid (CSF) of 19 of the psychotic children and 23 control children. Organic acids were determined by gas chromatography in urine, plasma, and CSF of the psychotic patients. The mean concentration of ethanolamine in CSF was significantly higher in psychotic children than in control subjects. A subgroup of autistic children may possibly have a brain disorder involving ethanolamine metabolism. None of the known inherited diseases of organic acid metabolism was found in any of the psychotic children, but future studies utilizing sophisticated gas chromatography--mass spectrometry--computer techniques might disclose abnormal organic acid content in the CSF of such patients.

Adolescent↗
PubMed1977

Antimicrobial drug use in three Canadian general hospitals.

Total amounts of antimicrobial drugs used to treat inpatients during 1975 were calculated for three Canadian general hospitals, one of them the principal teaching hospital of a medical school. Use of drugs was compared with that reported for Boston City Hospital during periods when antimicrobial therapy was and was not supervised by infectious disease consultants. Ampicillin, tetracyclines, cephalosporins, erythromycin and aminoglycosides for prophylactic oral administration were used excessively in the three hospitals. The degree of overuse was comparable to that at Boston City Hospital during years when drug use was uncontrolled. Overuse or improper choice of antimicrobial drug decreases the quality of patient care and increases its cost. More rigorous education is needed for both medical students and practising physicians in the rational use of antimicrobial drugs. Informal consultation with an infectious disease unit should be required before certain overly popular or toxic antibiotics are administered to hospitalized patients.

Anti-Bacterial Agents↗
PubMed1977

Studies of the glycine cleavage enzyme system in brain from infants with glycine encephalopathy.

Glycine content and enzyme activity of the glycine cleavage system were compared in autopsied brain from five infants dying with glycine encephalopathy and four control infants, including two with other types of hyperglycinemia. Glycine content was elevated 2- to 8-fold and glycine cleavage enzyme activity was undetectable in the brains of the glycine encephalopathy patients. Glycine content and enzyme activity were normal in the brains of the control patients, including one with ketotic hyperglycinemia secondary to methylmalonic acidemia. Prolonged dialysis failed to restore glycine cleavage enzyme activity in brain homogenates of glycine encephalopathy patients, and these homogenates failed to inhibit enzyme activity when added to homogenates of control brain. Radioactive bicarbonate was converted to radioactive glycine by control brain, but not by glycine encephalopathy brain. This finding, together with the results of recombination experiments between solubilized human brain enzymes and purified protein components of the bacterial glycine cleavage system of Arthrobacter globiformis, indicates that the enzyme defect in glycine encephalopathy involves at least the second or H protein of the 4-protein glycine cleavage enzyme system.

Amino Acid Metabolism, Inborn Errors↗
PubMed1977

Aspartate-taurine imbalance in dominantly inherited olivopontocerebellar atrophy.

Amino acids were measured in autopsied brain from two patients who died with a dominantly inherited form of olivopontocerebellar atrophy. Neuropathologic changes found in the brain of these patients suggested a loss of cerebellar climbing fibers. The contents of aspartic acid, gamma-aminobutyric acid, and homocarnosine were reduced in the cerebellar cortex and the dentate nucleus, while taurine content was markedly elevated in the same brain regions. These findings are compatible with the possibility that aspartic acid is the excitatory synaptic transmitter of the climbing fibers and taurine is the inhibitory neurotransmitter of one or more types of interneurons in the cerebellum.

Adult↗