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Biomedical subjects

T Lerman-Sagie

Publications and source records attributed to T Lerman-Sagie.

84 records · Page 5Linked to original sources
PubMed1989

Light and electron microscopic retinal findings in Leigh's disease.

Funduscopic and retinal light- and electron-microscopic findings are described in an infant with progressive neurologic deterioration leading to death. Brain autopsy findings were consistent with Leigh's disease. The retinal mitochondria showed marked degenerative changes, the cristae were almost completely destroyed and electron-dense material filled a major part of the cavity. These changes are typically described in the late stages of mitochondrial diseases but have not been described before in retinal mitochondria in a patient with Leigh's disease.

Autopsy↗
PubMed1989

[Leigh's syndrome].

Leigh's syndrome is a degenerative nervous system disorder with well-characterized neuropathology. The clinical picture shows progressive neurologic deterioration in infancy leading to death from respiratory arrest. Mitochondrial enzymatic deficiencies are implicated in the pathogenesis of the disease. A 6-month-old male infant with progressive neurologic deterioration and brain findings at autopsy consistent with Leigh's syndrome is described.

Autopsy↗
PubMed1988

CSF glucose levels in febrile infants.

Simultaneous blood and CSF glucose levels were investigated in 143 febrile children without cerebromeningeal illness, who were evaluated due to fever in the first 2 months of life or febrile convulsions. There was a significant decrease (P less than 0.001) in the mean CSF-blood glucose ratio from 0.67 +/- 0.13 in the first 2 weeks of life to 0.56 +/- 0.11, 0.57 +/- 0.8 and 0.58 +/- 0.11 at the ages 2-4, 5-6 and 6-8 weeks, respectively. The mean CSF and blood glucose levels did not change significantly in this period. After the 2nd month of life there was a significant rise P less than 0.01 in the mean CSF-blood glucose ratio to 0.72 +/- 0.11, the customary normal value in children. This was associated with a significant rise in CSF glucose levels as compared to the first 8 weeks as a whole. Our study suggests age-related changes in CSF blood-glucose ratios during the first weeks of life which are important when evaluating infants for the possibility of meningitis.

Blood Glucose↗
PubMed1987

Syndrome of osteopetrosis and muscular degeneration associated with cerebro-oculo-facio-skeletal changes.

We describe an infant with cerebro-oculo-facio-skeletal manifestations, radiologic and pathologic findings of osteopetrosis, and severe myopathic degeneration proven on histopathologic study of muscles. The muscle changes appear to be part of the pathogenetic process in this syndrome and the cause of the flexion contractures present at birth. Real-time ultrasonography may prove a useful tool in prenatal diagnosis of this syndrome.

Arthrogryposis↗
PubMed1987

Effect of valproic acid therapy on zinc metabolism in children with primary epilepsy.

The effect of long-term treatment with valproic acid (VPA) on zinc (Zn) metabolism was studied in 15 children with absence seizures. During treatment with VPA the erythrocyte Zn content was significantly lower than that found in controls matched for sex and age. Plasma and urine values of Zn and of copper were within normal limits. It is suggested that the anticonvulsive action of VPA may be mediated through its effect on the metabolism of Zn in the brain and the concomitant changes in the activity of the enzymes glutamic acid decarboxylase and carbonic anhydrase.

Adolescent↗
PubMed1987

Low erythrocyte zinc content in acquired aphasia with convulsive disorder (Landau-Kleffner syndrome).

Abnormal zinc metabolism in a 6-year-old male patient with Landau-Kleffner syndrome (acquired aphasia and convulsive disorder) is the subject of our report. We describe a significant decrease of erythrocyte and plasma zinc levels in the patient as compared with normal. Red blood cell zinc content is normally 38.5 +/- 2.7 micrograms/g hemoglobin v 16.5 micrograms/g hemoglobin found in the patient. Plasma zinc level is 94.6 +/- 10.4 micrograms/dL in normal in comparison with 50.4 micrograms/dL in the patient. Normal oral zinc tolerance test performed on the patient precludes altered zinc absorption or increased urinary loss. The possible connection between abnormal zinc metabolism and the pathophysiology of Landau-Kleffner syndrome is discussed.

Aphasia↗
PubMedDate not supplied

Cerebrospinal fluid findings in infants with nonpolio enteroviral meningitis.

We studied 25 infants aged less than 9 months who were diagnosed as having nonpolio enteroviral meningitis (EM) confirmed by virus isolation from cerebrospinal fluid (CSF). CSF protein, glucose and cellular content in these infants was compared with that of 125 age-matched febrile infants without cerebromeningeal illness. In order to control for changes occurring in the CSF during the first 2 months of life, the studied infants and the control subjects were stratified into 2-week age-groups and were compared. All but three of the infants with EM had increased CSF leukocyte count, and 10 infants showed domination of the polymorphonuclear leukocytes in the CSF. Only two had increased CSF protein levels, and low CSF/blood glucose ratios were found in all but two cases. None of the infants with EM had increased values of CSF protein concomitant with decreased CSF/blood glucose ratio. CSF in EM is characterized by increased white blood cell count in contrast with normal or near-normal CSF protein and glucose levels. In some infants, enterovirus isolation may be the only evidence of EM.

Age Factors↗