Craniofacial Neurofibromatosis: A Roentgen Profile

Multiple radiographic projections were employed to study osseous abnormalities of the skull in twenty cases of craniofacial neurofibromatosis. Calvarial defects in the lambdoid suture were observed in eight cases. Large midline defects produced a cranium bifidum appearance in one case. Mandibular changes consisted of hypoplasia with flattening of the external contour, thinning of the body and the ramus and widening of the medial and lateral coronoid spaces. Mandibular overgrowth with a convex outer contour and increase in the vertical height was seen in one case and a large cystic lesion around three unerrupted molar teeth was seen in another case. Abnormalities were also observed in the zygomatic arch and the mastoid process. The tympanic bone was absent in three cases. A 20 degree occipitomental projection best demonstrated these abnormalities. Orbital changes consisted of enlargement, outwardly curved innominate line, indistinct lower part of the innominate line, elevated lesser wing of the sphenoid, irregular enlargement or obliteration of the superior orbital fissure and intraorbital calcification. The pituitary fossa was enlarged in two cases without evidence of raised intracranial tension. Macrocranium was observed in one case. Functional disabilities observed in some cases were conductive deafness, loss of vision, cranial nerve palsies and mental retardation. Carotid angiograms done in three cases showed tortuous branches of the external carotid artery feeding the plexiform scalp lesions. A fourth ventricle tumour was seen on ventriculography in one case which presented with hemiplegia and cerebellar signs and symptoms. Skeletal abnormalities corresponded closely to the locations of soft tissue masses indicating developmental interdependance of neuroectodermal and mesodermal derivatives.     

TONGUE INVOLVEMENT IN LEPROMATOUS LEPROSY

Background. Involvement of the oral cavity in lepromatous leprosy is well-documented. The tongue may demonstrate multiple nodules, thickening, and scarring. Methods. Ten consecutive untreated patients with lepromatous leprosy with a bacteriologic index of 4+ or more were clinically and histopathologically studied for evidence of tongue involvement. Results. Three patients showed clinical tongue involvement, as a noduloplaque lesion in one patient and fissured tongue (lingua plicata) in two patients. Tongue was clinically normal or showed nonspecific changes in the remaining seven patients. Histologic evidence of tongue involvement by lepromatous process was seen in six patients, including three without clinical involvement. Conclusions. We conclude from this study that the tongue is as prone to involvement by lepromatous process as buccal and palatal mucosa.     

Effect of Low Doses of Alpha Chlorohydrin on the Dehydrogenases and Oxidases of Rat Testis. A Histochemical Study

Effekt einer low-dose Behandlung mit α-Chlorohydrin auf die Dehydrogenase und Oxidasen im Rattenhoden — Eine histochemische Untersuchung
In dieser histochemischen Untersuchung wurden folgende Enzyme nach low-dose Behandlung mit α-Chlorohydrin (6,5 mg/kg/9 Tage) in den verschiedenen Zellarten des Rattenhodens untersucht: Isocitratdehydrogenase, Succinatdehydrogenase, Malatdehydrogenase, Glutamatdehydrogenase, Δ⁵- 3α-Hydroxysteroiddehydrogenase, NAD- und NADP-Diaphorase und Monoaminoxidase. Die Anwendung von α-Chlorohydrin führt zu einer Verminderung oder dem Verschwinden all dieser Enzyme bis auf NADP-Diaphorase und Δ⁵ -3α-HSD. Diese Veränderungen der Enzymaktivitäten im Rattenhoden zeigen deutlich, daß nach einer low-dose Behandlung mit α-Chlorohydrin lange bevor histologische Defekte auftreten, der Zitronensäurezyklus und Aminosäurestoffwechsel im Hoden geschädigt werden. Die Steroidbiosynthese bleibt jedoch unbeeinträchtigt, wie die gleichbleibenden Aktivitäten der Δ⁵ -3α-HSD und NADP-Diaphorase bewiesen.     

Portage guide to early intervention: cross-cultural aspects and intra-cultural variability

The issues arising from implementing an early intervention service, developed in the rural United States in the late 1960s in a range of different cultural contexts over a period of a quarter of a century, are explained. Services from India, Bangladesh, Jamaica and the United Kingdom are compared. As well as considering cross-cultural aspects of Portage, variability within one country, the United Kingdom, is considered by comparing one service in an inner-city area and one in a rural area.     

Synthetic,immunological and structural studies on repeat unit peptides of Plasmodium falciparum antigens

Using solid phase methodology, we have synthesized five peptides (16-18 residues long) corresponding to repeat sequences of four antigens of a human malarial parasite, Plasmodium falciparum. Three of these antigens (RESA, FIRA, and ABRA) are found in the asexual blood-stages of the parasite, while the remaining one (CSP) is found in the sporozoites. The synthetic peptides, conjugated to bovine serum albumin, elicited high levels of antibodies in rabbits, and these antibodies were found to cross-react with the heterologous peptides. The degree of cross-reactivity, as estimated in an ELISA, was quite remarkable among all the peptides. The peptide corresponding to the RESA tetrapeptide repeat was found to be the most immunogenic and highly cross-reactive. For this reason this tetrapeptide repeat unit, peptide 1, may be a suitable candidate for inclusion in a multiple epitope polypeptide vaccine design. Conformational studies using circular dichroism spectroscopy show that these peptides have similar conformational characteristics with a common feature OF ～30% and ～50% helical content in water and TFE respectively. Theoretical predictions regarding conformation using the Chou-Fasman method have also been presented.     

PAPILLON-LEFEVRE SYNDROME

Two cases of Papillon-Lefevre Syndrome (PLS) in a family are reported. Association of recurrent infections, arachnodactyly, intracranial calcification and phalangeal abnormality have been observed. Because there is no history of consanguinity, and autosomal recessive pattern of inheritance is suggested.     

阿立哌唑成功治疗抽动秽语综合征1例报告(英文)

概述:抽动-秽语综合征(Tourette syndrome,TS)是一种儿童期发病的神经精神障碍,特征是多发性运动抽动和发声抽动,病程至少1年。本病例报告描述了一位16岁男孩患有抽动-秽语综合征6年,症状已使其功能严重受损。阿立哌唑10 mg/d治疗一个月左右后,该患者的症状完全消失,恢复了病前的生活。在这种情况下,需要权衡长期使用抗精神病药物潜在的不良反应与持续的抽动秽语症状对患者生活造成的破坏性影响。