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C Mhiri

Publications and source records attributed to C Mhiri.

46 records · Page 3Linked to original sources
PubMed1989

Focal myelin thickenings in a peripheral neuropathy associated with IgM monoclonal gammopathy.

Nerve biopsy in peripheral neuropathies associated with an IgM monoclonal gammopathy may occasionally display focal myelin thickenings. In a patient with such an IgM neuropathy, in whom an anti-myelin-associated glycoprotein (MAG) antibody activity was present in the serum, single-fiber preparations revealed 34% of internodes bearing myelin swellings. The morphometric, morphological and ultrastructural findings were reminiscent but not identical to those of the hereditary tomaculous neuropathy with liability to pressure palsies. Atypical features for tomacula included lack of spiralization of the redundant loops of myelin around the axons and their predominant external situation with regard to the myelin sheath. The frequent colocalization of myelin thickenings and the widening of myelin lamellae typical of IgM neuropathies, are highly suggestive of some pathogenetic link between the two abnormalities. The redundant loops of myelin in IgM neuropathies possibly result from a defect in the axon-myelin adhesion secondary to the binding of IgM on an epitope of MAG directly involved in cell-cell adhesion.

Aged↗
PubMed1989

[Peri-nephritic phlegmon in children. Apropos of 13 cases].

The authors present their experience on perinephric abscess in children based upon a series of 13 cases in 12 children. After review of the classical data given by the clinical inquiry and radiology, a special regard is given to new imaging investigations which have considerably improved diagnosis. Treatment also has been ameliorated by advent of percutaneous drainage under echography or tomodensitometry control.

Adolescent↗
PubMed1988

[Intracerebral schwannoma. Apropos of a case].

The authors report a case of intracerebral Schwannoma discovered in a 13 year old girl who had shown symptoms of increased intracranial pressure since four years and half evolving progressively to aggravation. CT scan images and operating appearance were uncharacteristic. Histological diagnosis of the tumor was only established at pathological examination. The tumor recurred 15 months after in spite of its complete extirpation. The characteristics of this extremely rare localization of Schwannoma (14 cases published in the literature) are pin-pointed by the authors.

Adolescent↗
PubMed1988

[Chronic proximal spinal amyotrophies in Tunisia. Clinical, genetic, epidemiologic and histopathologic study].

The results of a clinical, pathological, genetic and epidemiological study of 101 cases of chronic proximal spinal muscular atrophy are reported. Ages at onset allow to distinguish clearly an infantile, a juvenile and an adult groups. The infantile and juvenile groups comprising 93 p. 100 of the patients, were characterized by onset before the age of 20 (54 p. 100 before age 5) and by a large intra- and interfamilial clinical variability. The severity of the disease was unrelated either to sex or the sporadic or familial nature of the disease. On the other hand it was closely related to age at onset and to the pathology of the muscles. There seems to be no genetic heterogeneity in this group. Inheritance is of the autosomal recessive type in the great majority of cases. Certain sporadic cases may be due to a dominant mutation or to a phenocopy, as suggested by segregation analysis. The adult group, comprising 6 patients, included 4 cases of autosomal dominant inheritance without consanguinity of the parents, and one case of dominant inheritance.

Adolescent↗
PubMedDate not supplied

The slim disease in African patients with AIDS.

Ten unselected African patients infected with human immunodeficiency virus (HIV) and with slim disease were evaluated using physical examination, anthropometric measurements, Karnovsky performance score, and muscle biopsy. All had marked weight loss (36.8 +/- 10.8%) with extreme fatigue, marked diffuse wasting with significantly decreased circumferences of arms, thighs and calves (P < or = 0.002), and a low Karnovsky performance score (range 30-70). Mild to moderate motor deficit (in 9/10 patients) contrasted with the major amyotrophy. Chronic diarrhoea (in 7/10) and/or prolonged fever (in 7/10) were always associated with the amyotrophy. Atrophy of muscle fibers was the main finding of muscle biopsy. Only 5 patients met the CDC criteria for the 'HIV wasting syndrome'. We conclude that slim disease, which is highly suggestive of the acquired immune deficiency syndrome (AIDS) in Africa, is a condition associated with chronic diarrhoea and/or prolonged fever, that encompasses the 'HIV wasting syndrome' sensu stricto and probably other debilitating diseases associated with AIDS, such as tuberculosis.

Acquired Immunodeficiency Syndrome↗
PubMedDate not supplied

Polysaccharide storage myopathy--case report and literature review.

Polysaccharide myopathy is a rare form of storage muscular disorder. The clinical picture of this particular form of myopathy is unspecific. We report a 62-year-old woman with late-onset progressive weakness and wasting, affecting proximal muscles of the four limbs and the girdles. No myalgia, dysphagia nor symptoms of cardiac failure were observed. Muscle biopsy revealed a vacuolar myopathy with accumulation of amylopectin-like polysaccharide. This material was strongly PAS-positive and diastase-resistant. At electron microscopy, the deposits were composed of non-membrane-bound filamentous and granular material surrounded by numerous mitochondria. No enzyme deficiency was found. Clinical presentation of our patient was similar to the 16 cases reported in the literature. She did not have myocardiopathy and her survival is much longer. Hypothetic mechanisms of polysaccharide accumulation are reviewed.

1,4-alpha-Glucan Branching Enzyme↗