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F X Bergouignan

Publications and source records attributed to F X Bergouignan.

12 recordsLinked to original sources

Involvement of respiratory muscles in adult-onset dystonia: a clinical and electrophysiological study.

Involvement of respiratory muscles is unusual in dystonia, but its occurrence may be underestimated either because it is not conspicuous or because it is improperly imputed to another cause. Three patients who had adult-onset dystonia and who were exhibiting respiratory problems were examined clinically and electrophysiologically. In the three patients the onset was focal-cervical in two and blepharospasm in one. The respiratory problems appeared later. The first patient had involuntary deep and loud inspirations combined with spasms of axial dystonia, the second complained of breathing arrests, and the third had deep inspirations mainly on speaking or reading aloud, thus causing broken speech. Electromyographic findings, including of the diaphragm, were quite consistent with a respiratory involvement in these three cases of dystonia. Assuming that respiratory troubles could be in the first sign of a focal dystonia, electrophysiological studies of respiratory muscles could be used to confirm this.

Administration, Oral↗

A comparative study of the effects of chronic axotomy, crush lesion and re-anastomosis of the rat sural nerve on horseradish peroxidase labelling of primary sensory neurons.

Chronic axotomy is detrimental to the incorporation of horseradish peroxidase (HRP) by neurons of the central and peripheral nervous system. Using the rat sural nerve as a model, this study aimed to determine the effects of other types of nerve injury on the peroxidase labelling of dorsal root ganglion (DRG) cells. Compared to the decreased labelling occurring shortly after permanent transection of the sural axons at the ankle, crush injury of the nerve had no effect on the number and size distribution of peroxidase-stained cells. Re-anastomosing the sural nerve to its own distal segment or to the tibial nerve delayed the changes in HRP neuronal labelling, which subsequently were less severe in neurons allowed to reinnervate their own nerve. It also sustained the incorporation of HRP by many large DRG neurons, a function which is lost shortly after these cells are chronically axotomized. Nerve re-anastomosis also prevented the retrograde atrophy of myelinated and unmyelinated nerve fibers which is triggered by permanent transection. Based on the preservation of fiber counts in the sural nerves proximal to the site of surgery, with no evidence of degeneration, our observations possibly reflect alterations in the peroxidase metabolism of DRG neurons depending on the type of axonal injury they sustained and the possibility they had upon regeneration to contact endoneurial tubes and ultimately their original end-organs.

Animals↗

Disulfiram neuropathy.

A case of sensorimotor polyneuropathy during disulfiram administration is described. This complication partially resolved after the medication was stopped. Pathological observations revealed axonal changes and neurofilament accumulation was found in a few myelinated and unmyelinated axons.

Alcoholism↗

Peripheral neuropathy with essential mixed cryoglobulinemia: biopsies from 5 cases.

Essential mixed cryoglobulinemia, which can cause hypersensitivity vasculitis, was observed in five patients with peripheral neuropathy. Three cases presented with multifocal neuropathies and two cases with symmetrical polyneuropathy. One had cryoglobulinemia with IgM monoclonal gammopathy IgG polyclonal gammopathy, and the other four had cryoglobulinemia with polyclonal gammopathy. Biopsies showed perivascular infiltration by mononuclear cells around medium, and mainly small-sized blood vessels. This was observed in the epineurium (five cases) and muscular fragments (three cases). At ultrastructural examination two cases showed severe damage of most myelinated fibers, which presented acute stages of Wallerian-like degeneration, and the three other cases showed a less widespread destruction of myelinated fibers. Most endoneurial capillaries showed swollen endoneurial cells. Myelino-axonal degeneration of myelinated fibers is probably due mainly to the vasculitis always present in the epineurium. This damage was probably worsened by the modifications of endoneurial capillaries. These lesions and their mechanisms are quite different from those observed in cases of cryoglobulinemia with an isolated monoclonal gammopathy.

Aged↗

Uncompacted lamellae in three patients with POEMS syndrome.

Three patients with POEMS syndrome were studied: two of them had myeloma, whereas the third had M protein without myeloma. Peripheral nerve biopsies showed no deposits of anti-Ig sera at direct immunopathological examination. Ultrastructural study revealed myelino-axonal degeneration in all three cases. This was associated in the first case with a dramatic loss of myelinated fibers and there were lesions consistent with acute degeneration in the second case. In addition, all three had some fibers showing uncompacted myelin lamellae. This peculiar modification has previously been reported only in cases of dysglobulinemia, lymphoma and inflammatory demyelinating polyneuropathy, but its mode of formation remains unclear.

Aged↗

Carbonic anhydrase activity in the normal and injured peripheral nervous system of the rat.

The carbonic anhydrase reactivity of primary neurons and axons of the L4 and L5 lumbar levels was studied in rats before and after various surgical procedures including transection of the spinal cord, removal of dorsal root ganglia, and transection of ventral or dorsal roots or spinal nerves. In normal animals, carbonic anhydrase reactivity was confined to large and medium size neurons of the dorsal root ganglia, and was also present in a sizeable percentage of cells scattered throughout the thoracolumbar sympathetic chain and in the celiac ganglion. At root level, enzymatic staining could be detected in 48.7% of the dorsal root myelinated axons of most sizes, whereas in ventral roots, it was restricted to small myelinated axons, in a proportion much higher at the L4 than in the L5 level. Spinal motoneurons remained unlabeled, despite procedures aimed at increasing the somal concentration of carbonic anhydrase, such as ventral root ligation and blocking of the fast or slow axoplasmic transport using colchicine or iminodiproprionitrile. However, it is likely that reactive ventral root axons originate from neurons situated segmentally in the spinal cord, and do not constitute aberrant sensory fibers, as carbonic anhydrase activity remained unchanged in the L4 and L5 ventral roots after removal of the corresponding spinal ganglia, whereas it disappeared after damage to the spinal cord at the lumbar level, or at a site distal to a ventral root section. Enzymatic staining of neurons of the dorsal root ganglia was not modified by a dorsal rhizotomy, but showed a marked decrease after transection of the spinal nerve.(ABSTRACT TRUNCATED AT 250 WORDS)

Animals↗

[Hypermyelination in a case of peripheral neuropathy with benign IgM monoclonal gammopathy].

A 65 year-old man presented with a 8 months history of ataxia and weakness of the lower limbs. Acroparesthesias had been present for ten years in all four limbs. Serum protein electrophoresis showed a monoclonal IgM kappa spike. Bone marrow showed no increase in plasma cells. Motor conduction velocities were reduced. A biopsy from the right superficial peroneal nerve revealed several enlarged myelin sheaths in each fascicle on semi-thin sections. Under electron microscopy these large myelin sheaths were either regularly arrayed around the axon or formed several large additional loops. The outer lamellae of these abnormal myelin sheaths and of the additional loops were frequently widened. Other fibers showed a widening of some myelin lamellae (W.M.L.) with a myelin sheath of varying thickness, and these widened lamellae were frequently joined together. A direct immunopathological study could not be made. The widening of some myelin lamellae is present in most cases of peripheral neuropathy with IgM monoclonal gammopathy and a fixation of anti IgM serum on some myelin sheaths may be demonstrated by direct immunopathology. Hypermyelination has been reported in a few cases. In this patient hypermyelination was as developed as in typical cases of tomaculous neuropathy. An association in some myelin sheaths of hypermyelination and W.M.L. has been noted in serum induced myelin aberrations in peripheral nervous system cultures.

Aged↗

[Prevention of poliomyelitis: live or killed vaccine? Remarks apropos of 2 cases of poliomyelitis].

The attenuated poliovirus vaccine is remarkably effective in preventing the disease but its innocuity has always been questioned. It seems to be responsible for occasional cases of spinal paralysis occurring in developed countries where poliomyelitis has virtually been eradicated. The authors report two cases of acute anterior poliomyelitis in young adults, in which the poliovirus recovered from the faeces might have been of vaccinal origin. Virological techniques do not always characterize the strains isolated, nor distinguish between a vaccinal mutant and a wild poliovirus. These data, together with previously published cases, have prompted the authors to discuss whether the use of a live poliovaccine is justified.

Adult↗

Occurrence of Renaut's bodies in a peripheral nerve.

We reviewed 1,012 biopsy specimens from the superficial peroneal nerve to assess the occurrence of Renaut's bodies. These structures were first described about 100 years ago, progressively forgotten, and only reexamined ten years ago. They appear as rounded, amorphous structures of the endoneurium and can be mistaken for small infarcts or amyloid deposits. In our biopsy samples, examined during a period of eight years, Renaut's bodies were encountered in 21 cases. Thus, their frequency in that nerve was estimated to be about 2%. The nerve fibers were normal in five cases and variously damaged in the remaining 16. No clinicopathologic correlation could be established. We concluded that Renaut's bodies are probably a normal structure of this peripheral nerve.

Fibroblasts↗