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Argininosuccinic aciduria: clinical and biochemical findings in three children with the late onset form, with special emphasis on cerebrospinal fluid findings of amino acids and pyrimidines.

Three children with the late onset form of argininosuccinic aciduria are presented. The first two are sisters. The clinical features are characterized by mild retardation and ataxia, complicated by episodes of hyperammonemia. All patients showed elevated concentrations of argininosuccinic acid and its anhydrides in all body fluids, most pronounced in cerebrospinal fluid (CSF). Moreover, in Cases 1 and 2, we found elevated concentrations of pseudouridine and uridine limited to CSF, which was not reported before. In Case 3, with some residual activity of argininosuccinate lyase (ASL), we found normal values of these compounds. In urine we found elevated concentrations of uracil in Cases 1 and 2, and orotic acid in Case 2. Plasma showed an elevated concentration of orotic acid in all three patients, uracil was elevated in Case 2, cytidine was elevated in Cases 2 and 3. The results are being discussed and indicate that CSF values of pyrimidines reveal new biochemical abnormalities of brain tissue in urea cycle disorders.

Argininosuccinate Lyase↗

Tobramycin levels in cerebrospinal fluid of patients with slightly and severely impaired blood-cerebrospinal barrier.

Concentrations of tobramycin in cerebrospinal fluid (CSF) and serum of neurosurgical and of meningitis patients were determined following systemic application of 80-mg doses. Tobramycin was detectable only in 8 tests out of 17 in CSF of 11 neurosurgical patients. The highest drug amount in CSF in these tests was 0.47 microgram/ml. In CSF of 7 meningitis patients with CSF cell counts ranging from 173/3 to 20,500/3 cells, tobramycin was detectable in all 11 determinations. However, the tobramycin level was above 1.0 microgram/ml in only one CSF sample. These results also confirm the poor penetrability of aminoglycosides through the blood-CSF barrier for tobramycin. In proven gram-negative bacillary meningitis and tobramycin treatment, additional intraventricular aminoglycoside application is necessary to obtain effective levels in CSF. An exception may be treatment of neonatal meningitis.

Anti-Bacterial Agents↗

[Demonstration of postural asymmetry factors in a hypophyseal tissue culture as affected by the cerebrospinal fluid of cats with unilateral damage to the hemispheric cortex].

It has been experimentally established that special hypophysiotropic factors--precursors of pose asymmetry factors (PAF) are found in the cerebrospinal fluid of donor animals with cortical lesions during the first postoperative hours. These factors, being specific for the location of the cortical lesion in donor animals, appear to be hypophysiotropic peptide substances inducing the production of corresponding PAF by hypophysial cells. The production of certain PAF by the rat hypophysial tissue cultures under the influence of cerebrospinal fluid from cats gives evidence in favour of species nonspecificity of PAF inductors.

Animals↗

Age-related differences in volumes of subcortical nuclei, brain matter, and cerebrospinal fluid in healthy men as measured with magnetic resonance imaging.

Magnetic resonance imaging was used to determine the volumes of brain, subcortical gray matter nuclei, and the ventricular and sulcal cerebrospinal fluid in 27 healthy men. Subjects were divided into young (less than 35 years, n = 10) and old (greater than 60 years, n = 17) groups. Volumes were normalized as percent intracranial volume. Older subjects had significantly less brain mass and significantly larger ventricular and peripheral cerebrospinal fluid volumes than the younger men. The caudate and lenticular nuclei were significantly smaller in older than younger men. This significant difference remained when their volumes were expressed as a ratio of cerebral brain matter volume. This cross-sectional study demonstrates age-related atrophy and concurrent dilation of cerebrospinal fluid spaces in healthy subjects. Of brain regions affected, the caudate and lenticular nuclei are significantly more affected by healthy aging than is cerebral brain matter; this may account for some of the motor abnormalities in aging.

Adult↗

Effect of cerebrospinal fluid drainage and/or partial exsanguination on tolerance to prolonged aortic cross-clamping.

Paraplegia as a consequence of spinal cord ischemia associated with procedures on the thoracic and thoracoabdominal aorta has been linked to the interaction of proximal hypertension with elevated cerebrospinal fluid pressure (CSFP) during aortic cross-clamping (AXC). CSFP reduction via cerebrospinal fluid (CSF) drainage is thought to significantly prolong the cord's tolerance to AXC. Likewise, partial exsanguination is reported to effectively reduce ischemic injury by controlling proximal hypertension. To evaluate the individual and collective efficacy of both techniques, 18 mongrel dogs (25 to 35 kg), divided into three equal groups, underwent a fourth interspace left thoracotomy AXC. Baseline proximal arterial blood pressure (PABP), distal arterial blood pressure (DABP), and CSFP were established and monitored at 5-minute intervals during 120 minutes of AXC, and for 30 minutes thereafter. Group I animals were partially exsanguinated prior to AXC to maintain PABP at a mean of 115 to 120 mmHg. Group II animals had sufficient (16 +/- 5 cc) CSF withdrawn to maintain a DABP-CSFP gradient, i.e., spinal cord perfusion pressure (SCPP) of 20 mmHg. Group III animals were treated with both CSF drainage and partial exsanguination in the same manner as groups I and II, respectively. Perioperative somatosensory evoked potential (SEP) monitoring evaluated cord function. Postoperative neurological outcome was assessed with Tarlov's criteria. SEPs degenerated approximately 22 minutes following AXC for groups II and III. In contrast, group I exhibited rapid (10 +/- 7 min) SEP loss. All five surviving group I animals displayed paralysis 48 hours postoperatively.(ABSTRACT TRUNCATED AT 250 WORDS)

Animals↗