Bioavailability of phenobarbital by rectal administration.

Phenobarbital was administered to five patients, eight to 20 years of age, by four different administration routes: Intravenous, intramuscular, oral, and rectal. At each administration, extent of bioavailability, elimination rate constant, absorption rate constant, and volume of distribution of phenobarbital were calculated using a one-compartment open model. The results obtained suggest that rectal administration of phenobarbital is more reliable than intramuscular or oral administration of the drug for rapid treatment in children.

An acute variant of subacute sclerosing panencephalitis: an autopsy case report.

An acute variant of subacute sclerosing panencephalitis (SSPE) was described in a 5-year-old boy who showed rapid progression of coma within 14 days of right hemiplegia. He had measles at 3 years of age. The diagnosis of SSPE was based on the following findings: high anti-measles antibody titer in the serum and in the spinal fluid, periodic complex of EEG, and typical pathological changes of the brain.

A new variant of Lowe oculocerebrorenal syndrome.

A case is reported of a 5-year-old boy with Lowe syndrome. The patient was unusual in that he had only mild and transient acidosis with no rickets. The corneal opacities in the parents suggest that this disease might be autosomal recessive.

Ornithine transcarbamylase (OTC) in white blood cells.

A radiochemical assay method of ornithine transcarbamylase (OTC) was developed using labeled carbamyl phosphate as a substrate. The enzyme activities determined by this method in peripheral white blood cells from ten normal subjects were 1.32 +/- 0.

Facial nerve palsy following therapeutic embolization.

Ischemic neuropathy is a previously described but rarely encountered complication of therapeutic embolization. Two cases of peripheral facial nerve palsy following embolization of branches of the external carotid artery are reported, and the possible mechanism is discussed.

[Multiple cerebral tuberculomata involving brainstem and cerebellum--report of a case (author's transl)].

A case of multiple cerebral tuberculomata involving the pons and cerebellum was presented. The lesions were demonstrated by CT as isodense to slightly dense foci. All four intra-axial lesions showed homogeneous enhancement following an intravenous injection of the contrast medium, and one of them was surrounded by a small area of low density, probably representing the perifocal edema.

Agenesis of an internal carotid artery: angiographic, tomographic and computed tomographic correlation.

Congenital absence of one internal carotid artery was found by angiography in a woman of 52 years who had subarachnoid hemorrhage. The absence of the bony carotid canal on the affected side substantiated the congenital nature of this rare vascular anomaly. We believe this to be the first report of absence of the bony carotid canal and the intracavernous portion of the internal carotid artery confirmed by computed tomography and cavernous sinography.

Serum vitamin E concentration in patients with severe multiple handicaps treated with anticonvulsants.

Serum vitamin E concentrations were measured in 47 severely handicapped patients, aged from 4 to 23 years, and in 22 controls. Thirty-three of the handicapped patients with seizures were treated with phenytoin and phenobarbital; the remaining 14 patients were not treated. The serum vitamin E levels were lower in the handicapped than in controls.

Balloon embolization of a giant aneurysm using a newly developed catheter.

A new catheter with a releasable balloon has been developed for the controllable embolization of intracranial vascular lesions located beyond many arterial curves such as the carotid siphon. Embolization is accomplished by introduction of the balloon to the lesion, followed by inflating the balloon with a polymerizable liquid. After this liquid sets to a gel, the catheter is twisted off to detach the balloon at a specially designed joint.

Metabolic acidosis in patients receiving anticonvulsants.

Blood pH, bicarbonate, PCO2, serum calcium, alkaline phosphatase and red cell carbonic anhydrase were measured in 37 selected patients receiving anticonvulsants. Patients with metabolic acidosis showed a high incidence of hypocalcemia with increased alkaline phosphatase and a significant reduction of carbonic anhydrase-B activity. High iPTH levels were found in 13 patients, but this was not correlated with acid-base balance status.

Lysine intolerance in a variant form of citrullinemia.

An oral loading of lysine (100 mg of lysine-HCL/kg was performed in two patients, 18-and 23-yr-old, with a variant form of citrullinemia. Serum citrulline levels were approximately 10 times higher than control level and lysine levels were within the normal range, in contrast to the classical form of the disease in which serum citrulline is approximately 100 times normal levels and hyperlysinemia is usually present. After lysine loading, lysine levels rose sharply and clearance was decreased.

Iatrogenic and transient hyperglycinemia in patients with phenylketonuria.

Two patients with phenylketonuria detected by newborn screening for inborn errors of metabolism were treated with low phenylanine formulae. Serum phenylalanine levels were well controlled, but serum glycine levels were elevated until 4 or 5 months of age. This was probable due to the high content of glycine in the formulae.

Intracavernous giant aneurysm associated with Marfan's syndrome.

A case of intracavernous giant aneurysm associated with Marfan's syndrome is reported. The patient was treated with a superficial temporal artery-middle cerebral artery bypass and a ligation of the internal carotid artery in the neck. Although they are common in aorta and pulmonary artery, the vascular abnormalities in Marfan's syndrome are very rarely reported in intracranial arteries.

Embolization and superselective angiography by means of balloon catheters.

Three types of thin flexible balloon catheters were made. One of them is a releasable balloon catheter, which is prepared for embolization of intracranial aneurysms, arteriovenous malformations and fistulas. The other two are nonreleasable balloon catheters that are utilized for temporary occlusion, catheterization, and superselective angiography of the intracranial arteries.