Clonality of cold agglutinins in patients with hemolytic anemia: an analysis by high-resolution two-dimensional gel electrophoresis.

High-resolution two-dimensional gel electrophoresis (2-DGE) was used to analyse plasma samples and partially purified cold agglutinins (CA) obtained from two selected patients. Both presented an acute hemolytic anemia with CA of high thermal amplitude, normal immunoglobulin levels, no detectable paraproteinemia, and no clinical evidence of a malignant B-cell disorder. The electrophoretograms of their plasma showed evident alternations of the "normal" protein profile, which were directly related to hemolysis (absence of the spots of haptoglobin and in one case of those of hemopexin), but no monoclonal gammopathy. The electrophoretograms of their purified CA revealed two clearly different spot patterns respectively corresponding to a monoclonal IgM and to polyclonal IgM. These results show that the clonality of CA associated with hemolytic anemia can be easily determined by 2-DGE. This technique may be very useful to discriminate chronic cold agglutinin disease in the early phase from "parainfectious" CA.     

Hippocratic concept of hysteria]

The Greek physicians of the fourth century B.C. painstakingly described a number of symptoms that they thought were caused by migrations of a restless uterus, said "wandering womb". According to this concept, we can use the term "hysteria", despite the fact that the noun itself never appears in any of the Hippocratic texts. We can determine its origin as being in ancient Egypt from the medical writings of the Egyptian papyri. In the same way, the therapy is mainly of Egyptian origin, using a combination of recipes to make the moving womb return to its proper place in the body cavity. However, we can also identify the rationality of the Greeks, in particular as regards mechanical physiopathology. Hippocratic authors are closely connected with presocratical physics, and manifest a distinct break with magic and religion. Furthermore, they claim that hysteria may result from prolonged sexual continence, an opinion which was to influence medical history for over two thousand years.     

Foot and eye preferences in adults: relationship with handedness, sex and age.

Age, sex, and handedness effects in foot and eye preferences were studied by questionnaire in large samples of normal adult populations from five different countries (total sample, n = 5064). Foot and eye preference were significantly associated with handedness category (right or left) in all the 10 sex by country samples for foot, and in 9/10 samples for eye. The overall frequencies of crossed preferences were 5% between hand and foot and 19.5% between hand and eye. In right-handers, a gradual shift toward the "right" with increasing age was systematically observed, both for footedness and eyedness. The proportion of crossed hand-foot preference was higher in men than women (7.4% vs 2.5%), and higher in left-handers than right-handers (16.3% vs 4.1%). Sex differences in the proportion of crossed hand-eye preference were variable from one country to the other.     

Maintenance of targeting errors by isthmo-optic axons following the intraocular injection of tetrodotoxin in chick embryos.

We have studied the role of electrical activity in the elimination of axonal targeting errors, which is a normal process in brain development. The experiments were focused on the isthmo-optic nucleus (ION), which, in adults, projects in topographical order on the contralateral retina. During embryogenesis, however, a few isthmo-optic neurons project to the ipsilateral retina, and many project to topographically inappropriate parts of the contralateral one; both kinds of targeting error are known to be eliminated by the deaths of the parent neurons. We injected tetrodotoxin (TTX) intraocularly at embryonic days 13 and 15 and, on the latter, applied a retrograde label to the retina of the same eye. Embryos were fixed at embryonic day 17. In some embryos, the label was a peripherally placed fleck of the carbocyanine dye "diI"; the resulting retrogradely labeled neurons in the contralateral ION were much more widely scattered in the TTX-injected embryos than in controls (errors in topography). In other embryos, the label was a solution of rhodamine-B-isothiocyanate (RITC) injected into the vitreous body; this yielded several ipsilaterally labeled isthmo-optic neurons in the TTX-injected embryos, but virtually none in the controls. The numbers of both kinds of aberrantly projecting neuron approached those previously reported near the beginning of the ION's period of neuronal death. We conclude that electrical activity plays an important role in the elimination of axonal targeting errors in the chick embryo's isthmo-optic system.     

Validity and reliability of the infant Behavioral Summarized Evaluation (IBSE): A rating scale for the assessment of young children with autism and developmental disorders

The Infant Behavioral Summarized Evaluation (IBSE) is a rating scale adapted from the Behavioral Summarized Evaluation (BSE) and specifically related to the assessment of behaviors of young children having autistic disorders. Content validity and reliability studies described in the paper were made from behavior ratings of videotapes for 89 children aged from 6 to 48 months. Results show a significant group of 19 items including some characteristic early autistic behaviors (communicative and social abnormalities) and some that are less commonly described in the syndrome (attentional, perceptive, and adaptive disorders). The value of the use of this scale for clinicians and professionals involved in behavioral evaluations and treatment of young children with developmental disorders and the necessity for further psychometric investigations are discussed.This study was supported by INSERM U.316 Nervous System from the Foetus to the Child. Development, Circulation, Metabolism, L. Pourcelot, INSERM, Network INSERM No. 489001, Grant INSERM No. 911182 (D. Savrage) No. 911008 (L. Hameury) MRT. No. 9906 Fondation Langlois. Gratitude is expressed to Professor G. Lelord who initiated and supervised this study. The authors thank Professor J. Fermanian, Department of Biostatistics, CHU Necker, Paris, for his helpful suggestions and assistance. They also thank Dr. Larmande, Dr. Leddet, Dr. Glorion, and Dr. Garreau for their medical participation in the study and C. Richard, C. Fillatre, J. Rabant, and F. Etourneau for the psychological assessments of children. Special thanks to G. Calzas, M. Barré, A. Lardeux, and D. Lioret for their technical assistance.     

Asymptomatic nerve hypertrophy in lepromatous leprosy: a clinical,electrophysiological and morphological study

Summary In order to learn more about early nerve lesions observed in leprosy, we performed a clinical, electrophysiological and morphological study in seven patients with untreated lepromatous leprosy, palpably enlarged radial cutaneous nerve and preserved sensation in the corresponding territory. The conduction velocity of the cutaneous radial nerve, which was decreased in all patients, did not significantly differ from that of a group of patients with lepromatous leprosy, hypertrophy of the radial cutaneous nerve and sensory loss. In contrast, the sensory action potential was significantly lower in patients with sensory loss, which demonstrates that axon loss is more important than demyelination in producing sensory loss. In all patients nerve enlargement was due to thickening of the epineurium and of the perineurium subsequent to inflammatory infiltrates and proliferation of fibroblasts and perineurial cells. In several fascicles, the inflammatory infiltrates and the infected cells infiltrated endoneurial connective tissue septa and blood vessels.Mycobacteria leprae were abundant in peri neurial cells, fibroblasts, macrophages, Schwarm cells and endothelial cells, and lymphocytic vasculitis present in all cases. The average density of myelinated fibres was 2600 SD 880 fibres/mm² (control: 7700 fibres/mm²), with marked differences between individual fascicles, versus 420 fibres/mm² in patients with nerve hypertrophy and sensory loss (range 0–2080 fibres/mm²). Single fibre preparations showed that segmental demyelination pre dominated in two patients, axonal degeneration in one, while inflammatory infiltrates and proliferation of connective tissue adhering to individual fibres were prominent in the others. Both infection of Schwann cells and secretory products released by mononuclear cells involved in the inflammatory process are likely to play a role in the lesions of nerve fibres observed in early stages of lepromatous leprosy.Presented in part at the Second Meeting of the European Neurological Society, Brighton (UK), June 1990     

Anaerobes and short-chain fatty acids in crevicular fluid from adults with chronic periodontitis

Pathogeny of adult chronic periodontitis is still unclear. Bacteriological and chemical analysis of crevicular fluid have shown, in active sites of the disease, a simultaneous presence of anaerobes and their major by-product: short-chain fatty acids. The last can decrease "in vitro" the neutrophil intracellular pH, whenever these cells are incubated in an acid medium. Clinical investigations are scarce which hold out data useful to attempt verifying this possible physiopathological mechanism. This work shows the presence of anaerobes in the active periodontal pockets, together with the presence of short-chain fatty acids likely to reach a concentration level comparable to that used for inhibiting neutrophils "in vitro". Forthcoming studies should investigate about a possible intracellular pH drop in the neutrophils and other cells of the inflamed periodontium.     

Extreme hypogammaglobulinemia disclosing large granular lymphocytes with hepatosplenorenal infiltration

We report a case of large granular lymphocytosis, or chronic "natural killer" lymphocytosis, a newly described entity. We were able to demonstrate the proliferative character of the disease by the finding of karyotype abnormalities. This case was remarkable for the pre-existence, for at least three years, of severe hypogammaglobulinaemia, for the very slow course of the proliferative process and for the progressive and tumoral infiltration of the spleen and liver, then kidney.     

Alcoholic foamy degeneration in Spain. Prevalence and clinico-pathological features

Among 389 consecutive chronic alcoholics in whom a liver biopsy was performed for diagnostic purposes, nine patients (2.3%) had histological changes of "alcoholic foamy degeneration" (AFD), characterized by numerous small fat droplets in the swollen hepatocytes of the centrilobular area. In five cases, there were non-specific clinical features, while the other four cases presented acute hepatic decompensation with jaundice and a reduced prothrombin activity. Seven patients had high serum lipid concentrations including hypertriglyceridemia and hypercholesterolemia, which improved after withdrawal of alcohol intake. In conclusion, AFD has a broad clinicopathological spectrum including asymptomatic patients and other cases with severe liver decompensation manifested by jaundice and reduced prothrombin activity. AFD is usually associated with hyperlipemia.     

Melanomatous meningitis. Apropos of 3 cases

The meninges are frequently involved during the dissemination of malignant melanoma. This "meningeal melanoma" ranks fairly high on the list of metastatic meningites, side by side with meningeal carcinomatosis proper (i.e. related to a malignant epithelial tumour). Meningeal melanoma may be associated with a cerebral metastasis or isolated, as in the three cases reported here. Although its prognosis is sombre, its diagnosis is important since the possibilities of treatment are limited but exist. CASE-REPORTS: Case 1. This was a 68-year old woman who had initially presented with malignant melanoma in the maxillary region (SSM level IV, thickness 2.9 mm). Two years after the primary tumor was excised, secondary lesions developed in the lymph nodes and bones. A few weeks later, the patient fell into mental confusion progressing towards delusion of persecution. Neurological examination and CT scans were normal, but numerous melanoma cells were found in the CSF. This woman died one month after the first neurological signs had appeared. Case 2. This 63-year old man presented with bilateral axillary adenopathy and inflammatory thoracic plaques which at histology had proved to be metastases from a malignant melanoma of the shoulder surgically treated 10 years previously. After 14 months of almost complete remission under multiple chemotherapy (CPDD, ACTD, VDS), headaches and lumbosacral pain developed. Standard radiography and CT of the spine gave normal results, and it was only at the third lumbar puncture that the CSF was found to contain malignant cells. The patient died 2 months after the first neurological manifestations. Case 3. A 42-year old woman developed headaches and dysesthaesia in the arms and head. This was followed by oppositional semi-mutism. All exploratory examinations were normal, except for that of the CSF which showed 18 cells/mm3, 50 p. 100 of which were melanoma cells. The patient was transferred to the Dermatology department where he underwent excision of a left scapular melanoma difficult to classify (malignant blue naevus?). Combined treatment with Fotemustine* and DTIC produced an incomplete but relatively prolonged response. Death occurred after 8 months. DISCUSSION: A. Meningeal melanoma accounts for about 10 p. 100 of all metastatic meningites. The most frequent primary tumours in meningeal carcinomas are breast cancer and lung cancer. Malignant melanoma ranks third or fourth, but when the relative frequency of each of these three malignant diseases is taken into account, it appears that the risk of malignant melanoma is higher than that of any other solid tumour. B. In 410 cases extracted from the literature, the meninges were involved in 30 p. 100 of malignant metastatic melanomas found at autopsy. There is no publication that makes it possible to evaluate the relative frequency of melanomas affecting only the meninges or coexisting with cerebral metastases. This also applies to meningeal melanoma associated with non-neuro