[Idiopathic and secondary capillary leak syndromes: A systematic review of the literature]. / Syndrome de fuite capillaire idiopathique et formes secondaires : une revue systématique de la littérature.

PURPOSE: The capillary leak syndrome (CLS) is a rare condition characterized by the onset of hypotension, edema, hemoconcentration and hypoalbuminemia. CLS can be idiopathic (Clarkson's disease) or secondary to various conditions and treatments. Here, we review the clinical and biological features, pathophysiology, causes and treatment of this rare condition. METHODS: We performed a systematic review of the literature (Medline database through February 2014) to identify all articles about CLS. The relevant references were selected by two independent authors. RESULTS: Secondary CLSs are mostly due to malignant hematological diseases, viral infections, and treatments such as chemotherapies and therapeutic growth factors. Diagnosis of idiopathic CLS is made by exclusion of secondary diseases, especially as a serum monoclonal immunoglobulin is present, or when there is a relapsing disease, no initial lung involvement or preserved consciousness despite low blood pressure. Acute episodes are treated with vasopressor therapy and judicious fluid replacement. Between episodes, patients with Clarkson's disease may be treated with intravenous immunoglobulins. CLS is a severe disease with significantly impaired prognosis. CONCLUSION: Clarification of the pathophysiological mechanisms of CLS is essential to improve the prognosis of this rare disease with more targeted treatments.

[Phytosterols and atherosclerosis]. / Phytostérols et athérosclérose.

Phytosterols/stanols (PS) enriched food products have been shown to consistently lower plasma cholesterol levels. The intake of 2g/d of PS decreases LDL-cholesterol by about 10%. With respect to the association of LDL-cholesterol lowering with reduction in the cardiovascular (CV) risk, it is likely that supplementation in PS reduces the incidence of CV disease. In addition, the vast majority of animal studies have shown that oral administration of PS reduces the progression atherosclerosis. However, it has been recently suggested that an increase in PS plasma concentrations may increase CV risk. Evidence to support this hypothesis come mainly from observations in sitosterolemic patients who hyperabsorb PS and cholesterol and display very high levels of PS, which may be associated with a premature atherosclerosis. Some epidemiological studies in non-sitosterolemic subjects have shown a positive correlation between PS plasma levels and coronary heart disease. However, these are observational studies and some of them present major methodological bias. In addition, recent studies with a larger number of subjects have indicated, either an absence or a negative relationship between PS and the incidence of CV disease. The guidelines of several French and international institutions recommend the use of PS enriched food in association with other classical recommendations in hypercholesterolemic subjects. However, further studies are highly encouraged to examine the CV benefit of PS enriched food.

Intravascular malignant lymphomatosis (so-called malignant angioendotheliomatosis): a case confined to the lumbosacral spinal cord and nerve roots.

Intravascular malignant lymphomatosis (IML), so-called malignant angioendotheliomatosis, was found in lumbosacral spinal cord and nerve roots of a 78-year-old women who died one month after the onset of symptoms. With regard to the majority of the 37 reviewed neurological cases in the literature, this report is unusual in that the disease was exclusively localized in the spinal cord and systemic involvement was absent. The usual clinical hallmark of the disease is a subacute dementia or encephalopathy, often associated with focal neurological signs, culminating in death within several months. The pathological features of IML characteristically include multiple small foci of necrosis of the whole brain, caused by occlusion of small vessels by noncohesive neoplastic cells and secondary changes of the vascular wall. All organs may be involved, especially the skin and the adrenals, sometimes with tumoral formations. Despite the fact that lymphoid tissues are usually spared, recent reports and the present case strongly suggest a lymphoid rather than endothelial origin of the malignant cells. The pathogenesis of this mainly intravascular lymphoma remains obscure.

[Sensory nerve involvement in X-linked bulbospinal amyotrophy (Kennedy syndrome). Contributions of electrophysiologic and histologic data]. / Atteinte du nerf sensitif dans l'amyotrophie bulbo-spinale liée a l'X (syndrome de Kennedy). Apport des données électrophysiologiques et histologiques.

Two new cases of X-chromosome linked bulbospinal muscular atrophy associated with gynecomastia (described by Kennedy in 1968) are reported. In one patient, an electrophysiological study and a muscle nerve biopsy were performed. Motor nerve conduction studies were normal but sensory action potentials were small or unrecordable in the absence of clinical sensory loss. Superficial peroneal nerve biopsy showed axonal lesions. These findings suggest that this disease is not purely due to degeneration of spinal motor neurons and is also associated with an axonopathy.

[Polyneuropathies with IgM monoclonal gammopathy. 12 cases]. / Polyneuropathies avec gammapathie monoclonale IgM. 12 cas.

12 cases of polyneuropathy with IgM monoclonal gammapathy are reported. An analysis of the clinical, electrophysiological, histological and immunological features of these cases and of those reported in the literature allows to distinguish 2 groups. In the first group (8./12 cases), the neuropathy showed clinical and electrophysiological features of a mainly demyelinating mechanism involving large fibers. Electromicroscopy disclosed a widening of the spaces between the lamellae of the myelin in half of these cases. A monoclonal deposit of IgM was demonstrated by direct immunofluorescence, on the remaining myelinated fibers in most cases. In this first group, the M-component always reacted with the myelin sheaths of a monkey's peripheral nerve. The results of indirect immunofluorescence were closely correlated with those of immunoblotting, which revealed an anti-M.A.G. (Myelin Associated Glycoprotein) activity. The second group is more heterogeneous: there was an predominantly motor neuropathy (1 case), an asymmetrical and painful neuropathy with an endoneural deposit of IgM (1 case). In 2 other cases which in no other ways differed from those of the first group, the M-component seemed devoid of antimyelinic activity. Nevertheless, the presence of IgM on the myelin sheaths of these 2 cases suggested a relationship between the neuropathy and the gammapathy. In both groups, results from the association of apheresis and chlorambucil were difficult to assess and vary greatly. Therapy appeared beneficent in half of the cases, but only one patient was markedly improved.

[Familial distal progressive spinal amyotrophy with asymmetry of the lower limbs]. / Amyotrophie spinale progressive distale et asymétrique des membres inférieurs à caractère familial.

Two cases of spinal muscular atrophy, localized on the lower limbs and strictly unilateral, in two half brothers, are reported. Such a distribution of clinical changes and such a hereditary transmission appear to be unusual in hereditary motor-neuronopathies. A recessive heterotopic transmission may be suspected. To our knowledge, it has been reported in Kennedy's disease only.

Neural generator of P14 far-field somatosensory evoked potential studied in a patient with a pontine lesion.

Somatosensory evoked potentials (SEPs) to electrical stimulation of the right and the left median nerves were studied in a patient with a pontine lesion. At first there was mainly right medial lemniscus involvement. Four months later the left medial lemniscus was found to be also involved. SEPs to stimulation of the right median nerve had normal wave forms and latencies while N20 was lacking and P14 was abnormal after stimulation of the left median nerve in the first SEP record. N20 and P14 were absent with preservation of P9 and P11 after stimulation of both left and right median nerves in the second SEP record. Therefore the P14 component has been found abnormal, then absent, in a patient with a pontine lesion.