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Biomedical subjects

F Letaief

Publications and source records attributed to F Letaief.

8 recordsLinked to original sources
PubMed1987

Hypertrophic neuropathy in spinocerebellar degeneration. Morphological study of the superficial peroneal nerve in fourteen cases.

Fourteen patients belonging to eight families were studied. At least one member of each family presented a clinical picture of spinocerebellar degeneration (SCD) and lowered motor nerve conduction velocity (MNCV). Muscular atrophy of the Charcot-Marie-Tooth type was found in 11 cases. The average MNCV of the median nerve was less than half the value in the controls (P less than 0.001). Morphometric analysis of the superficial peroneal nerve showed a considerable reduction (P less than 0.001) in myelinated fibres, primarily those of large diameter (P less than 0.001), a high average density of onion bulb formations, and a large percentage (P less than 0.001) of teased fibres showing aspects of segmental demyelination, with or without remyelination. These results indicate the presence of a hypertrophic neuropathy (HN) associated with the SCD. In most cases, this HN bore the electrophysiological and morphological features of type I hereditary motor and sensory neuropathy. In certain cases, however, there was an individual and intra-familial discordance of the electrophysiological and histological aspects, which may correspond to a difference in phenotypic expression, or to mutant genes. It is possible that a single disease is involved, assuming the clinical appearance of both SCD and HN, the biochemical support of which remains to be determined.

Adolescent↗
PubMed1987

Morphometric study of the sensory nerve in classical (or Charcot disease) and juvenile amyotrophic lateral sclerosis.

Analysis of the superficial peroneal nerve sampled in 9 cases of classical amyotrophic lateral sclerosis (classical ALS, Charcot disease) and compared with 8 age-matched controls showed a very significant reduction of all myelinated fibres (P less than 0.001), affecting small-diameter (P less than 0.01) and large-diameter (P less than 0.02) fibres. Moreover, the small-diameter unmyelinated fibres were very significantly reduced (P less than 0.001) and the large-diameter fibres were highly increased (P less than 0.01). These results suggest a phenomenon of chronic axonal degeneration. Analysis of the same nerve in 7 patients suffering from juvenile ALS and compared with 4 age-matched controls showed a significant reduction (P less than 0.05) of myelinated fibres. The small-diameter and overall unmyelinated fibres were not significantly reduced while the large-diameter fibres, were significantly increased (P less than 0.01). The same analysis of 4 patients presenting an early-onset ALS compared with 3 controls showed lesions of a severity half-way between that of the classical and the juvenile form. Our study showed that the lesions of the sensory nerve are of the same type in classical ALS and in juvenile ALS, but of differing severity. The nosologic place of juvenile ALS compared with classical ALS and with heredodegenerative diseases of the nervous system is discussed.

Adolescent↗
PubMed1986

[Genetic study of spinocerebellar hereditary degenerations in Tunisia. Role of consanguinity in their occurrence].

The genetic analysis of 101 genealogical trees of families with spinocerebellar heredo-degeneration enabled the authors to specify the transmission inheritance for each clinical type. Autosomic recessive transmission has been observed for Friedreich's ataxia (68 out of 69 families), Pierre-Marie's heredo-ataxia (15 families) and familial spastic paraplegia (2 families). A dominant mode of transmission has been observed in 13 families affected by familial spastic paraplegia (Strumpell-Lorrain) and in only one family with Friedreich's ataxia (an intermediate or incomplete form). It has also been observed that the consanguinity rate among this group of families is very high compared with that of the general tunisian population (25%). Marriage between cousins occurs in 75% of the cases of Friedreich's ataxia, in 78% of the cases of Pierre-Marie's heredo-ataxia and in only 61% of familial spastic paraplegia of Strumpell-Lorrain. The authors have come to the conclusion that the recessive autosomic transmission of the spino-cerebellar heredo-degenerative diseases are closely related to a high consanguinity rate.

Cerebellar Diseases↗
PubMed1981

[Peroneal atrophy in Tunisia. Study of 70 cases, pure or associated with other heredodegenerative diseases].

Seventy cases of hereditary peripheral neuropathy of Charcot-Marie-Tooth type have been studied. One group of 40 cases from 30 families had a pure peripheral neuropathy, the other 30 from 20 families having other associated inherited nervous defects. The classification of Dyck and Lambert (1968) modified by Dyck (1975) was used, but it proved difficult to distinguish pure types and transitional forms were common. Histological criteria appeared more reliable than clinical features and were the most constant finding within a given family. In forms associated with other abnormalities a hypertrophic and a neuronal form could be distinguished but similar difficulties in classification were encountered as the mode of genetic transmission, age of onset, clinical features and nerve conduction velocity were comparable in the two groups. Discrepancies between electrophysiological and histological findings may result from examining motor nerves with the former technique and sensory with the latter. Despite subdivision there is still a sharp distinction between the various forms of Charcot-Marie-Tooth disease and the hypertrophic neuropathy of Déjerine-Sottas. The genetic pattern is complicated by the frequent association with other inherited abnormalities.

Charcot-Marie-Tooth Disease↗
PubMedDate not supplied

Histomorphometric study of the superficial peroneal nerve in leprosy.

A histomorphometric analysis of the superficial peroneal nerve was made in 15 cases of leprosy. Thirteen patients presented clinical signs of leprous neuropathy, while the other two showed only cutaneous signs of leprosy. The presence of M. leprae in all the nerves sampled, and the appearance of the histologic lesions, made it possible to confirm the diagnosis of leprosy and to specify the type of leprosy in each case, even in the absence of clinical signs of leprous neuropathy. Correlation of the histomorphometric results with the duration of development of the disease and with the time elapsing before treatment showed, in the beginning stage, a considerable reduction in myelinated and unmyelinated never fibres and a proliferation of Schwann cells, as well as segmental demyelination and axonal degeneration of the teased fibres. When treated early, the evolution of the lepromatous type (LL) appears favourable, with apparent regeneration of the nerve fibres. When treatment is not instituted early, gradual loss of nerve fibres, axonal degeneration of all the teased fibres, proliferation of the Schwann cell processes devoid of axons, and increase in endoneural connective tissue lead to a severe degeneration of the nerve. This unfavorable development appears to progress faster in the absence of treatment or when treatment is irregular. In the borderline lepromatous (BL) and borderline tuberculoid (BT) types, nerve degeneration appears to be more rapid than in the type LL.

Adolescent↗