[The fate of alpha and epsilon lysine amino groups in rat liver in vitro (author's transl)]. / La destinée des groupements aminés alpha et epsilon de la lysine dans le foie de rat in vitro

The fate of alpha and epsilon lysine amino groups has been explored in rat liver homogenate by means of L-lysine labelled selectively in the two positions. alpha-15NH2 and epsilon-15NH2 are rapidly incorporated into the amino group of glutamic acid and it seems at first that both transaminations occur simultaneously. But the reversible transfer of the amino group between alpha-aminoadipic acid and glutamic acid, determined by means of labelled alpha-aminoadipic acid, proceeds swiftly, and the incorporation of alpha-15NH2 from the corresponding labelled lysine in glutamic acid may be easily explained by epsilon-transamination and saccharopine formation. The direct transamination of the alpha-amino group of L-lysine is most improbable and might be limited to some microorganisms and to epsilon-N-substituted lysine derivatives.

Plasma proteins immunologically related to inter-alpha-trypsin inhibitor.

SDS-polyacrylamide gel electrophoresis and immunoblot were applied to analysis of plasma proteins immunologically related to inter-alpha-trypsin inhibitor (ITI). In this system, anti-ITI sera were able to identify ITI and other components with an Mr near 120 kDa which would be degradation products of ITI by limited proteolysis. An anti-UTI (urinary trypsin-inhibitor) serum could detect, beside these derivatives, two minor components (Mr values near 90 and 60 kDa). Analysis of perchloric acid supernatants of plasma samples, using the same technic, induced visualization of a new component, similar to urinary trypsin inhibitor which could not be detected by direct analysis. This one was also characterized in a higher content in pathological samples (renal failure and infectious diseases).

Purification and characterization of pig inter-alpha-inhibitor and its constitutive heavy chains.

With the view of investigating the metabolism of inter-alpha-inhibitor, a plasma serine-proteinase inhibitor, in an animal model of inflammatory syndrome, we isolated inter-alpha-inhibitor from pig plasma. A high yield was obtained (140 mg/liter) with a two-step procedure: anion-exchange chromatography followed by affinity chromatography on heparin-Sepharose. In contrast to bovine inter-alpha-inhibitor was highly similar to human inter-alpha-inhibitor: its heavy chains are homologous to the human H1 and H2 heavy chains, as shown by chromatographic and electrophoretic properties, cross-immunoreactivity and N-terminal sequencing. Pig may therefore represent a good animal model to study inter-alpha-inhibitor metabolism and elucidate its physiological role.

[Purification and characterization of protease inhibitors from urine during pregnancy (author's transl)]. / Purification et caracterisation des inhibiteurs de protéases de l'urine humaine.

Two forms of urinary trypsin inhibitor, A and B, were purified from the pooled urine from pregnant women using non-denaturing methods. The inhibitor B arose from the inhibitor A and was not present in native urine. Electrophoresis on polyacrylamide gel in the presence of sodium dodecyl sulfate indicated a new heterogeneity of the inhibitor B with molecular weights of 33 000 and 24 000; the molecular weight obtained for the inhibitor A was 50 000. Inhibitors A and B were acidic proteins with an isoelectric pH of about 2.6 for A and about 4.2 for B. Inhibitor A and inter-alpha-trypsin inhibitor formed a precipitate with an antiserum to purified inhibitor B. But neither inhibitor A nor inhibitor B formed a precipitate with anti whole human serum or anti-inter-alpha-trypsin inhibitor antiserum. Measurements of specific activity of inhibitor A were consistent with two active sites in the molecule.

Marchiafava-Bignami disease, syndrome of interhemispheric disconnection, and right-handed agraphia in a left-hander.

We present an original case of Marchiafava-Bignami disease in a 47-year-old left-handed alcoholic man. Computed tomography and magnetic resonance imaging demonstrated the typical lesion, a necrosis of the middle portion of the corpus callosum. Diagnosis may thus be established in the living. In our case, the course was not fatal, which, to our knowledge, has only been described in four other cases in the literature. Clinically, our patient demonstrated an interhemispheric disconnection syndrome. The striking feature is that some of the symptoms were on the side opposite of the one that has previously been described in the literature, eg, right-handed agraphia, while others were on the usual side, eg, left-handed anomia. We discuss cerebral dominance for speech and handedness in left-handers and come to the conclusion that our patient's clinical features can only be explained by right hemispheric dominance for handedness and bilateral hemispheric representation of speech.

Pig I alpha I appears unmodified in plasma in case of endotoxin-induced disseminated intravascular coagulation.

The unrestricted activity of leukocyte proteinases is thought to contribute to the degradation of plasma proteins and thus amplify the coagulation disorders occurring in septic shock. Inter-alpha-inhibitor (I alpha I) is a plasma protein particularly susceptible to their action. Therefore we investigated its behavior in a porcine model of endotoxin shock which reproduces the coagulation changes observed in human sepsis. We did not detect any qualitative or quantitative modification of porcine I alpha I in plasmas collected from pigs after endotoxin infusion. To explain these data, I alpha I was incubated with polymorphonuclear neutrophils (PMN) stimulated by FMLP in the presence of cytochalasin B. We found that, unlike human PMN, porcine cells were unable to proteolyze I alpha I. Moreover, in the incubation medium of pig PMN, triggered either by FMLP or PMA, no measurable elastase activity was evidenced. Therefore, we urge to better take into account species differences in functional responses of PMN, to explain the experimental results obtained in animal models of septic shock.

[Properties of histones on hydrophobic chromatography]. / Le comportement des histones en chromatographie hydrophobe

The hydrophobic chromatography on alkylated Sepharose allows to separate the histones into three groups which exhibit an increasing affinity for the support HI less than H2A-H2B less than H3-H4. In this fractionation procedure, the behaviour of the histones taken separately or pair-associated, is discussed in relation with the ability of these proteins to complex each other in vitro and in vivo.

Development of an enzyme-linked immunosorbent assay for human plasma inter-alpha-trypsin inhibitor (ITI) using specific antibodies against each of the H1 and H2 heavy chains.

Inter-alpha-trypsin inhibitor (ITI) is a serine-proteinase inhibitor of human plasma enzymes. ITI is composed of three polypeptide chains covalently linked: bikunin, responsible for the antiprotease activity and two heavy chains H1 and H2. Human plasma also contains other components immunologically related to ITI such as pre-alpha-trypsin inhibitor (paI), inter-alpha-like inhibitor (IalphaLI) and free bikunin. The ELISA procedure we propose exclusively measures native ITI within the range 12.5-200 microgram/l. The intra- and interassay coefficients of variation were less than 5.6% and 8.7%, respectively. When ITI was added to plasma samples, full recovery was obtained. EDTA-plasma from 30 healthy individuals revealed a mean level of 241.5 mg/l (range 145.5-506). The high specificity, sensitivity, reproducibility and accuracy of the present assay should facilitate the specific measurement of native ITI in blood and thus might represent a useful tool for further physiopathological studies.

A simple method for the measurement of different forms of alpha 1 proteinase inhibitor in the faeces of patients with Crohn's disease.

Alpha-1 proteinase inhibitor (alpha 1PI), formerly named alpha 1-antitrypsin, is excreted in the faeces of patients with Crohn's disease as isoforms clearly separated by SDS-PAGE and immunoblot analysis. Relapses in Crohn's disease are generally associated with the appearance in faeces of M(rs) 51,000 and 45,000 glycosylated forms of alpha 1PI, as compared with normal subjects and most of the patients in quiescent phases of their disease who excrete an M(r) 38,000 unglycosylated form of alpha 1PI. We used their differential Concanavalin-A reactivity to design a specific test. The proposed assay is potentially helpful for the follow-up of patients under therapy and for early recognition of attacks of Crohn's disease.

Urinary trypsin inhibitory capacity determination: study in patients with disseminated cancers.

We present a method for automated analysis of urinary trypsin inhibitory capacity. The validity of the method has been established. The mean value of urinary antitryptic activity is higher in patients with disseminated cancers (70 IU/1, n = 243) than in healthy donors (14,4 IU/1, n = 117). However, frequency distribution of urinary trypsin inhibitor values shows a great overlap, so that an increased level of urinary trypsin inhibitor cannot be considered as a marker of neoplastic diseases but seems to be a non specific indicator of inflammatory syndromes.

[Decrease of fecal beta-galactosidase activity in Crohn disease]. / L'activité bêta-galactosidasique fécale est diminuée au cours de la maladie de Crohn.

An increased degradation of colonic mucus by bacterial enzymes might participate in the development of mucosal lesions in inflammatory bowel disease. The biodisponibility of drugs used in the treatment of such disease relies upon the metabolic activity of colonic bacterial flora. This activity can be indirectly assessed by measuring fecal enzymatic activities. The aim of this study was to compare fecal beta-galactosidase (beta-gal) activity in controls, in patients suffering from extradigestive inflammatory disease and in patients with Crohn's disease (CD). Three groups were studied including 11 healthy volunteers (6 F, 5 M) mean age 29 years (21-37), 20 patients with rheumatoid arthritis (RA) (17 F, 3 M), mean age 61.5 years, and 34 patients with non operated CD (21 F, 13 M) mean age: 27 years (13-50). The Crohn disease activity index (CDAI) was > 150 in 24 and < 150 in 10. beta-gal activity was measured in fecal extracts by its ability to hydrolyze paranitrophenyl beta-D-galactopyranoside and expressed as units of enzymatic activity/gram of fecal proteins. beta-gal activity was significantly decreased in patients with CD (16 +/- 4.5 U/g) (m +/- sem) as compared with patients with RA (353 +/- 64 U/g) (P < 0.0001) and to controls (263 +/- 40 U/g) (P = 0.002). beta-gal activity was not significantly different in controls and in patients with RA. Patients with active CD had a significantly lower beta-gal activity than patients with quiescent CD (9.5 +/- 3.7 U/g vs 31.4 +/- 11.5 U/g) (P = 0.006).(ABSTRACT TRUNCATED AT 250 WORDS)

[Qualitative study of fecal alpha-1-antitrypsin in patients with inflammatory digestive disease and patients with ileostomy]. / Etude qualitative de l'alpha-1-antitrypsine fécale dans les maladies inflammatoires du tube digestif et chez des patients avec iléostomie.

Alpha-1-antitrypsin is a glycoprotein which is excreted in feces under three different forms of molecular weight 38, 45 and 51 kDa. The 45 and 38 kDa forms are the result of a partial or total removal of the carbohydrate moiety, respectively. We determined the molecular forms of fecal alpha-1-antitrypsin in 10 controls, 13 patients with protein-losing enteropathy other than inflammatory bowel disease, 70 patients with active (n = 55) (CDAI greater than 150) and inactive (n = 15) (CDAI less than 150) Crohn's disease, 14 patients with active (n = 12) and inactive (n = 2) ulcerative colitis, and 17 patients with ileostomy. Fecal 38 kDa alpha-1-antitrypsin was found in all controls, all patients with protein-losing enteropathy, in 82 percent of patients with inactive inflammatory bowel disease, and in 20 percent of patients with active inflammatory bowel disease. In contrast, the 51 kDa and 45 kDa forms were present in feces of 80 percent of patients with active inflammatory bowel disease, and in only 17 percent of patients with inactive inflammatory bowel disease. Patients with Crohn's disease and the 51 kDa form (n = 39) had significantly higher values of activity index (CDAI) and orosomucoid than patients with Crohn's disease and the 38 kDa form (n = 26) (P less than 0.01). Deglycosylated 38 kDa alpha-1-antitrypsin was never recovered in ileostomy samples. This suggests that deglycosylation of alpha-1-antitrypsin occurred in the colon and is impaired in patients with active inflammatory bowel disease.

[Crural hemiparesis with ipsilateral ataxia]. / Hémiparésie crurale avec ataxie ipsilatérale.

A 61 year-old man with severe hypertension present with a crural paresis and ipsilateral ataxia. CT scan showed a low density area suggestive of a lacuna in the posterior limb of the internal capsule.

[Predominantly crural hemiparesis and ipsilateral ataxia caused by meningioma of the falx cerebri]. / Hémiparésie à prédominance crurale et ataxie ipsilatérale par méningiome de la faux du cerveau.

In a 61 year-old woman with a right crural hemiparesis, and a history of progressive development of ipsilateral ataxia, CT scan showed a meningioma of falx cerebri confirmed by neurosurgery. Neurologic signs regressed within weeks. Compression of corticopontine and corticospinal tracts at their origin could account for the clinical disorders.

[Non-traumatic spinal epidural hematomas (2 cases): contribution of the CT scanner]. / Hématomes épiduraux rachidiens non traumatiques (2 cas): apports du scanner X.

Two cases of non-traumatic epidural hematoma are reported. The first case, a 76 year old woman receiving anticoagulant treatment, presented with paralysis of both lower limbs and right upper limb. Myelography and CT scan showed a widespread hematoma from C4 to T2. The second patient, a 63 year old hypertensive woman, developed flaccid paraparesis with urinary retention, myelography and CT scan showed an epidural hematoma extending from T12 to L2. In both cases, surgical evacuation was followed by a fair motor recovery. The 190 published cases of non-traumatic spinal epidural hematoma include 14 of precise etiology, 69 idiopathic and 107 associated with or 2 predisposing factors. Three mechanisms are proposed to explain the spontaneous cases: alteration of peridural veins; rupture of dural vascular clusters; minimal arterial injury. CT scan imaging shows a biconvex, hyperdense epidural image and is the investigation of choice for acute, hyperalgesic myelo-radicular compression syndromes.

[Focal motor crises: a transitory ischemic attack?]. / Crises motrices focales: un accident ischémique transitoire?

A 65-year-old man with hypertension and coronary artery disease presented with a history of 6 tonic fits of right upper limb and 2 clonic fits of the right side of the face with language suspension, each lasting for only 2 to 3 minutes. CT with contrast showed a right occipital low density area, a probable old infarct, and left carotid angiography showed an ulcerated cervical stenosis. Based on reported experimental data, it is suggested that these paroxysmal manifestations are related to transient ischemic attacks, are of epileptic nature and due to an ischemic mechanism.

[Acute neurologic complications of rubella]. / Complications neurologiques aiguës de la rubéole.

Two cases of rubella with neurological complications are reported. In one case an extensive atypical polyradiculoneuritis was associated with a late albumino-cytologic dissociation. The other presented as a transverse myelitis with meningitis and radiculitis. In neither case was an history of contagion or recent vaccination elicited, and no eruption appeared in either patient. Clinical onset was sudden, with an acute meningitis followed several days later by neurological deficits. The course was marked by successive phases of early rapid deterioration, stabilization and slow regression over several months. Sequelae were minimal. Diagnosis was based on a sharp fall in the ratio of serum/CSF levels of specific antibodies to the rubeola virus. This was sure evidence of the presence of intrathecal synthesis of specific antibodies.

[Benign intracranial hypertension (pseudotumor cerebri). 12 cases]. / Hypertension intracrânienne bénigne (pseudo tumor cerebri). Douze cas.

Twelve cases of benign intracranial hypertension (BIH) are reported, there being a higher proportion of women patients and a greater frequency of those in the 3rd decade of life. Symptoms were principally headache (11 patients) and/or transient or permanent visual disorders (10 patients). Bilateral papillary edema was often associated with diminished visual acuity (5 cases) and/or amputation of the visual field (5 cases). Cerebral venous occlusion was detected in 3 patients and an infective focus in 7 cases. Three patients were receiving some treatment but none of the women was taking oral contraceptives. Two were pregnant. The CSF was abnormal in 5 of the 7 patients (mixed cell reaction and red blood cells: 1 case; pleiocytosis: 3 cases; raised protein levels: 1 case), and CT scans were normal except for reduced ventricular system size in 9 patients. Follow-up for a mean of 1 year showed total disappearance of symptoms and signs in 4 cases. Papillary edema had not totally regressed at the last ophthalmological examinations performed on 5 patients, and visual sequelae (visual acuity and/or visual field anomalies) were present in 5 cases. Two patients relapsed. The conventional acceptance of the benign nature of BIH must be tempered by the possible onset of possible serious visual disorders. Two factors appear to be determinant: the presence of severe visual disorders initially and/or the existence of arterial hypertension. Various physiopathogenic hypotheses have been evoked to explain BIH. The essential mechanism appears to be an anomaly of absorption of CSF, either from increased pressure in the dural venous sinuses or lesions of the arachnoid villi, but the role of cerebral edema and/or increased cerebral blood volume cannot be excluded. Incomplete understanding of physiopathogenic mechanisms account for the numerous treatments proposed and for some confusion in their indications.

[Giant vertebro-basilar aneurysm. Frontal syndrome]. / Anévrisme géant vertebro-basilaire. Syndrome frontal.

A 72-year-old man presented with an apparent frontal syndrome. He also had bilateral trigeminal neuralgia, a pyramidal syndrome of all 4 limbs, balance disturbances, a horizontal nystagmus when looking to the left and a right velopalatine paralysis. CT scan with contrast showed a hyperdense rounded lesion in the left cerebello-pontine angle. Cerebral angiography showed this to be a large aneurysm of the end of the vertebral arteries. The patient died suddenly. Autopsy confirmed the site and presence of the aneurysm. Balance disturbances, the pyramidal syndrome and velopalatine paralysis could all be explained by brain stem compression and the bilateral nature of the trigeminal neuralgia by compression of the trigemino-thalamic tract. The apparent frontal syndrome, the authors suggest could have resulted from subacute raised intracranial pressure.

[Marchiafava-Bignami disease. Interhemispheric disconnection, favourable outcome, neurological aspect]. / Maladie de Marchiafava-Bignami: disconnexion interhémisphérique, évolution favorable. aspect neuroradiologique.

We report two cases of Marchiafava-Bignami disease studied by CT and MR imaging. Both had hemispheric disconnection syndrome and favorable outcome. The only residual signs were agraphia with the left hand in the two cases and mild dementia in one. In one case, a control CT scan performed 3 weeks after the first one was normal whereas T2-weighted MR images showed high intensity signal in the corpus callosum. We conclude that: 1. there are benign forms of MB disease; 2. neuroimaging has diagnostic and prognostic value; 3. vitamin B deficiency may play a role in the pathogenesis of the disease.