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Seven observations, having in common an accumulation of polyglucosan bodies in the nervous system, are reported. In 2 cases of adult polyglucosan body disease, characteristic polyglucosan bodies were found on the peripheral nerve biopsies, and also on the cerebral biopsy in one of these patients who was demented. The 5 other cases presented medically intractable temporal lobe epilepsy. Recovery was obtained by a temporal lobectomy in which a massive accumulation of polyglucosan bodies was discovered. These personal observations are discussed with regard to other conditions characterized by accumulation of polyglucosan bodies in the nervous system. The non specificity of these formations is emphasized.

Neuromyopathies developed in three patients with gluten-sensitive enteropathy, a long time after they had been cured of their digestive disease by following a gluten-free diet. These cases differed radically from typical deficiency neuropathies by the presence of microvascular inflammatory lesions in nerves and muscles. The semiological findings were similar in all 3 cases, and were distinguished by the association of signs eveking lesions of the largest myelinated nerves fibers to the posterior rami with lesions in the muscles. Corticotherapy improved the condition but did not affect its chronic course. Nerve and muscle biopsies revealed the presence of segmentary microrascularitis, mainly lymphohistiocytic. The probable mechanism of these histological changes is alterations in the circulating immune-complexes, usually found in gluten-sensitive enteropathy, producing various types of associated disorders. Some of these immune-complexes would not be related straight to digestive intolerance to gluten, but would persist during the gluten-free diet period, and could be responsible for the micro-angiitis.

23 patients with obstruction of one or both main iliac arteries underwent one or more transluminal angioplasties by catheterization via the obstructed vessel. 26 iliac arteries were thus reopened. The length of obstructions varied from 1 to 10 cm. The majority of the obstructions were of recent onset whilst in 6 cases the presumed date was more than 6 months previously. In all cases potency was obtained with normal follow-up angiograms in 20 cases, and a residual though not hemodynamically significant stenosis in 6 cases. All patients were followed up by Doppler and in the majority of cases by venous digital angiography, with a follow-up at the present time of 6 months to 2 years. 2 patients underwent surgery because of reobstruction occurring 2 and 13 months after dilatation. Cumulative patency is 79% in 2 years, similar to results obtained by surgery. These results show that iliac angioplasty, a simple procedure, may avoid or delay surgery, and that it should be widely used in this precise anatomical indication.

The first small bowel diseases have been known for three centuries, but essential breakthroughs have occured in the last twenty years. A new classification may be proposed according to, the physiopathology, the anatomo-pathology, the etiology. From the physiopathological point of view one may separate enterocytic, pre-enterocytic or post-enterocytic malabsorptions. Small bowel injuries described by the pathologist include small bowel resections, villous atrophy, lesion of the lamina propria, or transmural lesions, the vascular pathology and the ulceration of the small bowel. A new syndrome is proposed: the rupture of the cellular continuity or intercellular syndrome.

When oral or enteral feeding becomes impossible or inadequate, total parenteral nutrition makes it possible to reduce the occurrence of malnutrition states as complications of medicosurgical disorders. From a practical point of view it should provide a minimum fluid intake of 30 ml/kg with a nitrogen intake of the order of 150 to 300 mg/kg/day with sufficient quantities of all essential amino acids in a balanced ration. In most instances the calorie intake should be 40 to 60 Kcal/kg/day. Non-protein calories are obtained from carbohydrates or fats but the ideal percentage of each of these two nutrients is not known. In order to be effective and well tolerated, intravenous nutrition of this sort must be adapted to each patient and should be administered by a qualified medical and nursing team in order to minimise the metabolic and septic risks secondary to the techniques used.

Many opinions are expressed about the ontogenetical evolution of the basal angle of the skull. The authors present an analytical study with respect to age and sex and find that the basal angle decreases significatively with age, however the difference with the sex is probably not significative.

Until recently, to diagnose exsudative enteropathy required the use of radio-isotopes and a stay of several days in hospital. Alpha-1-antitrypsin can now be used as endogenous plasma marker and assayed in the serum and faeces by a simple immunochemical method, thus measuring its intestinal clearance. Fifteen healthy subjects and 13 patients with organic digestive diseases were investigated by this method. The mean clearance values were inferior to 10 ml/24 h in the control group and significantly higher in the patients' group. Digestive protein loss can therefore be diagnosed by the new method, which offers the additional advantages of being reliable, easy, relatively inexpensive and applicable to out-patients.

The authors present a retrospective study of 11 patients who had suffered from a severe attack of ulcerative colitis with extensive undermining of the mucosa on barium enema. All patients were given corticosteroids and antibodies. Oral feeding was suspended in 7, and 3 received exclusive parenteral nutrition. In 2 cases medical treatment resulted in complete remission, but 9 patients underwent surgery (total proctocolectomy in 4, total colectomy with ileo-rectal anastomosis in 3, colostomy in 1 and exploratory laparotomy in 1). Six of the operated patients were followed up for 1 to 7 years and are now in good health; the remaining three died early in the post-operative period. Extensive mucosal undermining in ulcerative colitis is a factor of poor prognosis.

A new classification of the small bowel diseases is proposed according to - the physiopathology; - the anatomo-pathology; - the etiology. In this second part, the ethiological classification is developed. One may distinguish the post-surgical lesions, congenital defects, the side-effects of drugs and the vascular lesions. Allergy and infection are especially studied. The main problems which may be solved in a next future are: the mechanism of chronic infections, the biochemical pathology and especially the normal and pathological immunology.

It is commonly postulated that the enzymatic hydroxylation of phenylalanine, tyrosine and tryptophan involves the concomitant oxidation of a tetrahydropteridinic cofactor to an unstable quinonoid product, converted to the initial compound under the catalytic action of dihydropteridine reductase. We now report UV, NMR, mass spectrum and spectroscopic studies of 2-amino-4-hydroxy-6,7-dimethyl-5, 6, 7, 8-tetrahydropteridine oxidation process either by atmospheric O2 or by the H2O2-peroxidase system. No quinonoid form was visualized and, moreover, the spectral characteristics of UV absorbance spectra, initially reported as specific for the quinonoid form, are related to other oxidation products whose formation is explained here.

A radio-isotopic method was used to study the gastric emptying of an ordinary meal in 15 control subjects. The technique developed may be used for direct measurement of the early phase of gastric emptying and a simultaneous study of emptying of liquid and solid phases, respectively labelled with Tc99m and Cr51. Emptying of the liquid phase of the meal takes place in accordance with a mono-exponential curve in relation to time, the T 1/2 of which is 54 min +/- 6 (MSD). Emptying of the solid phase is significantly slower than that of the liquid phase. The reproductibility of the method was confirmed and the imprecision of each measurement does not exceed 5%.

Arteriovenous communications are a rare but specific complication of in situ saphenous vein arterial bypass. In this bypass procedure, some collaterals of the saphenous vein may remain patent. In this report, a characteristic example illustrates this complication. Local and general manifestations secondary to the development of these fistulas must be prevented by complementary surgical or radiological obliteration.

Metiamide an histamine H2-receptors antagonist has been used to treat a case of Zollinger-Ellison syndrome characterized by a long standing diarrhea, an important gastric hypersecretion and a moderatly elevated plasma gastrin but without digestive ulceration. At the dose of 600 mg per day, Metiamide induced a complete suppression of acid secretion, an effect which lasted for 15 days after stopping the drug. Accordingly and since the only finding at time of laparotomy was a small lymph node enlarged with endocrine metastatic tissue, the stomach was left intact and Metiamide pursued. During the first 4 months of chronic administration of Metiamide, acid secretion was maintained at levels below 25 p.cent of initial values. Ulteriorly however, although dosages of Metiamide were increased, acid hypersecretion resumed and a duodenal ulcer developed. Total gastrectomy was then performed 11 months after the beginning of Metiamide. In spite of the failure of Metiamide treatment, the long term follow up of this case of Zollinger-Ellison Syndrome, allowed us to get theoretical and practical informations.

The neurological and muscular complications seen in coeliac disease in adults are usually attributed to deficiency secondary to malabsorption. Amongst them, however, there exists a very rare cateogory, described by Cooke et al. (1966) taking the form of a chronic myeloneuropathy which cannot be explained in terms of the malabsorption syndrome. Our two cases of gluten intolerance enteropathy, confirmed by biopsy before and after diet, fell into this group of polyneuropathies. The patients, both women, suffered from an essentially sensory ataxic polyneuropathy with accessory motor component with pyramidal and posterior column signs. CSF findings showed a meningeal inflammatory reaction in one of the two cases. These neurological signs, appearing paradoxically during a digestive disease cured by diet, evolve chronically but become stabilised with corticosteroid therapy. Any vitamin deficiency may be excluded in the aetiology of these problems. Neuropathological study of neuromuscular biopsies in very fine serial sections confirmed the mild peripheral nervous involvement but revealed identical inflammatory lesions in the nerve and muscle which were remarkable by virtue of their very highly segmentally selective micro-vasculitis appearance. In these two cases, general, clinical and biological arguments, as well as the type of histological lesion, make it possible to exclude monoclonal gammapathies, malignant haemopathies, amyloidosis and the major collagen diseases. This micro-vasculitis, having transient forms with P.A.N. is no less distinctive, and may be integrated into the provisional group of "allergic angeitis", related to physiopathology of circulating immune complexes and very fashionable in theories as to the mechanism of gluten-sensitive enteropathies. The exact nature of the link between the latter and these types of polyneuropathy remains unknown.