Computed tomography of the brain in the Smith-Lemli-Opitz syndrome

Computed tomographic (CT) scans of the brain in a child with Smith-Lemli-Opitz syndrome revealed enlargement of the ventricular system, hypoplasia of the cerebellum, and abnormal thickening of the gray matter, consistent with pachygyria. These findings have been previously noted in autopsies performed on patients with this disorder. We conclude that CT scanning is a valuable tool in the evaluation of children suspected of having the Smith-Lemli-Opitz syndrome.     

Total-body protein turnover in parenterally fed neonates: effects of energy source studied by using [15N]glycine and [1-13C]leucine

The effects of nonprotein energy source (ie, glucose only vs glucose and lipid) on nitrogen retention and total-body protein turnover were studied in 20 parenterally fed newborn infants. All infants received approximately 3 g amino acids and 80-90 kcal.kg body wt.d. Total-body protein synthesis was estimated by using three constant-infusion, end-product methods: enrichment of urinary urea and ammonia in response to a [15N]glycine label and exhaled carbon dioxide enrichment in response to a [1-13C]leucine label. No differences were seen in nitrogen retention between the two energy sources. The estimate of total-body protein turnover obtained from the 13C label was similar to that obtained with the [15N]urea label. No differences in turnover rates were observed between the two diet groups. Use of the glucose-plus-lipid fuel system enhanced energy storage and the reutilization of amino acid for protein synthesis.     

Cervico-arthrotic myelopathies. Treatment and prognosis

The authors present 50 cases of cervical spondylotic myelopathy treated by anterior or posterior approach. To assess the severity of the pre- and postoperative neurological symptoms, they define an original ten-point classification scale taking into account gait and urinary disturbance, ability to manipulate objects and pain. With C2-C7 laminectomy, 60% of the patients are improved, essentially for walk. A few cases only (14%) are upgraded for upper limbs function. The authors conclude that this surgical treatment should be applied only when there is a multi-level narrowed canal and predominant lower limbs deficits. The efficacy of the anterior approach depends on complete removal of osteophytes. In cases with complete osteophytectomy, this surgical treatment can improve both the upper and lower limbs function and 77.8% good results are obtained. In a few cases it may be necessary to use both approach successively. Discriminant analysis applied to 12 factors shows that pre-operative scores of upper and lower limbs and sagittal diameter of spinal canal are the most important factors for neurological prognosis.     

C(3)-cyclopropyl cephems and carbacephems

A series of C(3)-cyclopropyl cephems and carbacephems has been prepared by palladium catalyzed addition of diazomethane to the corresponding C(3)-vinyl derivatives. The phenylglycyl cyclopropyl cephem derivatives exhibit better Gram-positive activity than cephalexin or cefaclor, while the aminothiazole oxime cyclopropyl cephem derivatives were not as active as the corresponding C(3)-vinyl cephems.     

HTLV-1 and tropical spastic paraparesis. 1. Clinical features, pathology and epidemiology

The clinical profile of tropical spastic paraparesis (TSP), described in scattered tropical and subtropical territories over the past 30 years, has been more clearly defined since the discovery of its direct association with human T lymphotropic virus type 1 (HTLV-1). A chronic disease of adults, commoner in women, it usually presents as a progressive spastic paraparesis with sphincter disturbance, sometimes with backache and lower limb sensory disorder. Most cases are chair-bound within 10 years. Histology reveals a chronic lymphocytic meningomyelopathy, predominantly in the spinal cord, together with long tract demyelination and hyalinoid thickening of the media and adventitia of small blood vessels. Geographical areas of high prevalence of TSP are known in the Caribbean, South America, South Africa, southern Japan, the Seychelles and probably in India, and it is sparsely endemic elsewhere. The virus appears to exist within lymphocytes for long periods. Vertical transmission occurs postnatally, and sexual and transfusion infection are also recognized, but much remains to be clarified regarding its pathogenesis and epidemiology.     

Effect of euphylline and no-shpa on cerebrovascular resistance and the survivability of animals after cerebral ischemia

In acute experiments on anesthetized cats with total ischemia of the brain (15-minute arrest of blood autoperfusion of the cerebral vessels by a stable blood volume) it was shown that euphylline and no-shpa administered before ischemia or in the early period after ischemia inhibit or prevent the development of the postischemic phenomenon of non-recovery of the cerebral blood flow. The two drugs contributed to survival of albino rats following the brain ischemia produced by ligation of both carotid arteries.     

The dissociation of visuospatial neglect and neglect dyslexia.

A right-handed man with a left hemisphere lesion extending into the right hemisphere, with evidence of both a left-sided neglect dyslexia and right-sided visuospatial neglect is reported. When copying simple geometric designs he omitted to copy figures on the right-hand side of the page, when bisecting lines he tended to bisect the line to the left of the line's actual centre. He had a neglect dyslexia which was characterised by paralexic errors affecting the beginning (that is, left) of words. The occurrence of these two phenomena provides evidence of a dissociation of these forms of neglect. The findings are discussed in relation to the possible mechanisms of unilateral neglect.