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Biomedical subjects

L T Ch'ien

Publications and source records attributed to L T Ch'ien.

At least 19 recordsLinked to original sources
PubMed1983

Herpes simplex virus encephalitis. Diagnostic and treatment considerations.

The patient with meningoencephalitis should be evaluated carefully for the presence of focal signs referable to involvement of the frontotemporal regions of the brain. A significant percentage of cases of encephalitis with discrete focal features are caused by infection with herpes simplex virus. If focal signs are not present, the patient should be managed conservatively but examined regularly because focal neurologic dysfunction may present at any time in the course of disease. If there is clinical and electrographic evidence of involvement of the frontotemporal lobes, radiographic imaging is indicated as well as studies of the serum and cerebrospinal fluid for antibodies indicative of a recent infection with herpes simplex virus. Assuming that the radiographic scans identify the characteristic changes of a focal encephalitis and that the antibody responses are indicative of a recent herpes simplex virus infection, brain biopsy should be done in order to confirm the diagnosis. With positive evidence for HSE, treatment with ara-A should be initiated and continued for 10 days. If the biopsy proves negative for virus, ara-A should be discontinued and the patient managed conservatively (Fig. 3). Although the NIAID study of ara-A treatment of HSE is encouraging, the numbers are small and the evidence is, at best, only suggestive. It is reasonable to use this drug until a better one becomes available for the treatment of known HSE, and treatment should be instituted early before cellular injury is extensive. In centers familiar with this problem, biopsy confirmation of the diagnosis is a simple and informative procedure and can be defended. If patients cannot be moved to a center of this type, physicians familiar with the many facets of this problem should be consulted, and a decision regarding biopsy and treatment should be individualized in light of the circumstances. Biopsy should only be undertaken when the procedure can be done with minimal risk to the patient and the assurance that the maximum amount of information can be gained.

Acyclovir↗
PubMed1983

Neuropsychological sequelae of postradiation somnolence syndrome.

Postirradiation somnolence syndrome in children with acute lymphocytic leukemia treated with cranial irradiation has been identified as a possible precursor of later cognitive dysfunction. To test this, the neuropsychological evaluation of 48 children who developed somnolence syndrome was compared with that of 31 children who did not have the syndrome at approximately 1 1/2 and 3 3/4 years after treatment. No differences in performance between the two groups were found on many measures of neuropsychological functioning with the exception of fine motor speed. Children without somnolence syndrome scored somewhat less than normal on measures of academic achievement. No other differences from normal performance were noted in either group. The results of the study indicated that if children with somnolence are at greater risk for the development of cognitive dysfunction than those not manifesting the syndrome, such risks occur at a time farther from treatment than 3 to 4 years.

Adolescent↗
PubMed1983

Language-related skills in children with acute lymphocytic leukemia.

The success of cranial irradiation and chemotherapy in the treatment of acute lymphocytic leukemia (ALL) has raised concerns about possible central nervous system damage in long-term survivors. The current study focused on language-related skill measures obtained as part of a neuropsychological test battery. Performance was analyzed as a function of age at diagnosis and age at evaluation in a sample of 110 children with ALL (mean age = 13.28 years). Results, although for the most part nonsignificant, do reveal significantly poorer performance on an Aphasia Screening Test rating by those diagnosed early (at or before five years of age) and assessed at or before 13 years of age when contrasted with those diagnosed early and assessed after their thirteenth year. Further analysis revealed a significant decline in spelling scores for those off therapy for more than two years. There was also a consistent trend for decline across most language-related skills measures as time off therapy increased. These results reflect either recovery from mild acute language dysfunction over time superimposed on a general trend for decline in language-related skills as time off therapy increases, or sampling error. Suggestions for future lines of investigation are discussed.

Achievement↗
PubMed1982

Metastatic epidural tumors in children.

Spinal cord compression is a rare but serious complication of malignant diseases in children. Epidural cord compression was noted in 81 patients within the past 17 years at this center. The complication developed at different times during the course of the primary disease. For 29 of our patients, cord dysfunction was one of the initial signs of cancer--Ewing sarcoma, neuroblastoma, Hodgkin disease, and malignant lymphoma. By contrast, for most of the patients with osteosarcoma and rhabdomyosarcoma, it appeared later in their clinical course. The treatment outcome of patients who were paraplegia with complete loss of sensory function for greater than or equal to 48 hours was poor. Only four of 22 in this group became ambulatory. Ten patients with osteosarcoma did not undergo laminectomies because they all had multiple metastases and terminal disease. Paraplegia developed in all ten. There was no difference in ambulatory rates among other patients, with or without laminectomies.

Child↗
PubMed1982

Neuromyelitis optica (Devic's syndrome) in two sisters.

Neuromyelitis optica (Devic's syndrome) typically occurs in a sporadic pattern. We studied the disorder in two sisters, ages 6 and 10 years, who presented with sudden loss of vision at age 3 years, followed 5 months later by a transverse myelopathy. The older girl had only light perception in either eye, persistent paraparesis and progressive scoliosis; her sister had a visual acuity of 20/200 in both eyes and mild spinal cord dysfunction. Results of electrodiagnostic tests correlated well with the severity of clinical neurologic deficits and provided objective estimates of the extent of tissue damage in both the optic nerves and the spinal cord. The presence of Devic's syndrome in these two sisters could be coincidental; however, the possibility of a shared genetic factor(s) should be considered.

Child↗
PubMed1981

Progression of methotrexate-induced leukoencephalopathy in children with leukemia.

From 1972-1974, 228 children began treatment for acute lymphocytic leukemia and were prospectively assessed for neurologic complications. After CNS irradiation (2,400 rad) and intrathecal methotrexate (MTX), they received weekly intravenous maintenance therapy with MTX alone (40-60 mg/m2; 20 patients) or MTX (10-30 mg/m2) with other drugs (208 patients). Signs of leukoencephalopathy appeared in 11 children (nine without CNS leukemia) after 4-15 months of IV MTX alone, and included lethargy, seizures, spasticity, paresis, drooling, and dementia. Before or during the clinical onset, EEG frequencies slowed (all ten patients tested). Radionuclide scans showed periventricular accumulation of 99mTc (9/11 patients) and remained abnormal for greater than or equal to six months in eight patients. Cranial computed tomograms or neuropathology findings (five patients each) demonstrated leukoencephalopathy (nine patients) and radiation-related microangiopathy (ten patients). Severe neurologic and neuropsychologic dysfunctions were present in four long-term survivors.

Brain↗
PubMed1980

Immunoreactive myelin basic protein in the cerebrospinal fluid in neurological disorders.

Cerebrospinal fluid from 582 persons was analyzed by a double-antibody radioimmunoassay for the presence of material cross-reactive with peptide 43-88 of human myelin basic protein (BP). In a group of 104 patients with multiple sclerosis (MS), 23 of 33 individuals clinically judged to have had an exacereation within two weeks prior to the time CSF was obtained had detectable material ranging from 2 to 200 ng/ml. In the remaining 71 MS patients who either were stable or had had an exacerbation more than two weeks before, only 1 patient had a marginally elevated level of immunoreactive material. CSF from 53 persons with cerebrovascular disease was studied, and 13 of 29 with recent infarctions had values of 2 to 540 ng/ml. The degree of elevation in strokes generally paralleled the predicted volume of the lesion, but the amounts detected did not correlate quite so closely temporally with onset as they did with the periods of active disease in MS. Of the remaining 425 patients, 29 had immunoreactive material of 2 to 400 ng/ml in their CSF. Most of these patients with detectable material had acute diseases known to affect the myelin sheath. Eight of 10 persons with acute disseminated encephalomyelitis had no detectable material. The presence in CSF of material cross-reactive with BP peptide 43-88 does not have diagnostic specificity for MS but can be used as a means for determining recent myelin injury. The type of BP peptide formed and mechanisms for clearance of BP and BP peptides may be important in determining the biological consequences following release of this potentially immunogenic material from the central nervous system.

Antigen-Antibody Reactions↗
PubMed1980

Long-term neurological implications of somnolence syndrome in children with acute lymphocytic leukemia.

A longitudinal study of 49 children with acute lymphocytic leukemia (ALL) assessed the long-term effects of central nervous system (CNS) prophylaxis on brain function. From 10 to 12 electroencephalograms (EEGs) were done before and at intervals during after 30 months of treatment that included 2,400 rads of CNS irradiation plus intrathecal methotrexate therapy. None of the children had CNS leukemia, and all remained in first complete remissions. All 49 had abnormally slow EEG background frequencies during the four-year study, and 29 (60%) developed somnolence syndrome six to eight weeks after CNS prophylaxis. During this syndrome, EEG background frequencies decreased more than 3 standard deviations below the expected mean values for normal children. Thereafter, 7 of the 29 began to show signs of learning disabilities and 7 developed recurrent seizures. Of the 20 children who did not have the syndrome, none showed later evidence of CNS dysfunction. Somnolence may be an early indicator of long-term neurological sequelae after cranial irradiation.

Brain↗
PubMed1980

Neonatal herpes simplex virus infection introduced by fetal-monitor scalp electrodes.

An infant was severely infected with herpes simplex virus as a result of fetal monitoring with scalp electrodes on the buttocks. The first vesicles appeared at the site where the electrodes had been placed and, by day 9 after birth, additional lesions covered the entire perineum and lower extremities. The child developed acute meningoencephalitis and pneumatosis intestinalis. Herpes simplex virus was isolated from cultures of skin vesicle fluid, cornea, saliva, blood, and spinal fluid. The infection resolved after treatment with adenosine arabinoside, administered intravenously for 11 days and applied topically for another 15 days. A follow-up examination when the child was 2 years old disclosed no physical or neurologic abnormalities. This case illustrates the risk of introducing herpes simplex infection by internal fetal monitoring.

Electrodes↗
PubMed1979

Strychnine therapy in nonketotic hyperglycinemia.

Nonketotic hyperglycinemia is an inborn error of metabolism resulting from a defect in the glycine cleavage enzyme system. It is characterized biochemically by elevated concentrations of glycine in blood, spinal fluid, and urine. Previous therapies which have been directed toward reducing the glycine concentration in plasma and CSF have not been successful in preventing neurological deterioration, which may be the result of the role of glycine as an inhibitory neurotransmitter. Strychnine treatment was initiated because it is a specific antagonist of glycine at postsynaptic membranes. The patient reported here has shown clinical and EEG improvement while taking strychnine in conjunction with sodium benzoate.

Benzoates↗
PubMed1978

Sydenham's chorea and seizures. Clinical and electroencephalographic studies.

The hospital records of 28 children (mean age, 9.4 years) with typical Sydenham's chorea were reviewed. Nineteen of 28 patients had antistreptolysin O titers of greater than or equal to 200 Todd units. Other causes of chorea were excluded by appropriate laboratory and clinical follow-up studies. At the onset of the movement disorder, 17 of 28 patients had abnormal EEGs consisting of irregular posterior slowing in 15, sharp epileptic spikes in 5, and high-voltage sharp waves in 2. Two patients with spikes predominantly in the temporal lobe regions developed complex partial seizures. On follow-up evaluation, the EEGs returned to normal within one to four weeks. Seizures did not recur after therapy with anticonvulsants. Seizures have been reported only rarely in association with Sydenham's chorea. Our observation suggests that seizures may occur during chorea but may often be masked by frequent choreic movements and thus not recognized. The EEG changes and seizures were transient in our patients studied so far.

Adolescent↗