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Biomedical subjects

N Telerman-Toppet

Publications and source records attributed to N Telerman-Toppet.

At least 37 records · Page 2Linked to original sources
PubMed1981

[Terminal innervation in 3 cases of leprous neuropathy (author's transl)].

Three cases of leprosy are reported. In all patients, the diagnosis was made by finding Hansen bacilli in skin lesions, nasal secretions and ears, and the immunological typing of leprosy was established. Conduction velocity of motor and sensory nerves was measured and muscle biopsies were obtained. Histochemical typing of muscle fibers and measurement of the terminal innervation ratio of motor axon vitally stained with methylene blue were performed. A subclinical involvement of peripheral motor nerves was demonstrated by these investigations.

Action Potentials↗
PubMed1980

Terminal motor innervation in myopathic hamsters.

Terminal motor innervation and the histochemical profile of muscle fibers were investigated, before and after sciatic nerve crushing, in 1- to 150-day-old myopathic hamsters. The observed morphologic abnormalities in terminal motor innervation were of varying degree prior to the nerve injury. These abnormalities were found in close proximity to lesions of the myofibers, and became more pronounced with age and the progression of the disease process. Collateral ramification of healthy axons occurred in hamsters of 120 days of age with reinnervation of damaged muscle fibers. When submitted to sciatic nerve crushing, myopathic hamsters showed an ability to regenerate and reinnervate the subneural apparatuses. The regenerative process in these animals was essentially the same as in normal, sciatic nerve-crushed hamsters. Thus, it was concluded that the morphologic changes in terminal motor innervation were secondary to muscle cell degeneration.

Aging↗
PubMed1979

Charcot-Marie-Tooth disease associated with retinal pigment dystrophy and protanopia. Neurological, ophthalmological and genetic study of a family.

Neurological, ophthalmological and genetic investigations were performed on a family, a member of which presented with a rare association of tapeto-retinal degeneration, protanopia and Charcot-Marie-Tooth disease (CMT), and asked for genetic counseling. The neurological enquiry was completed by measurement of motor nerve conduction velocity in several completed by measurement of motor nerve conduction velocity in several members of the family. The propositus was submitted to a muscle biopsy. The ophthalmological examination included ophthalmoscopy, fluorescein angiography, electroretinogram and electrooculogram. The propositus, a woman aged 40, had typical CMT disease and her father also had a mild form of it. She had protanopia as had her father, her son and her nephew. In addition she had large macular pigmented changes, described as retinal dystrophy, "flavus flavimaculatus." Her mother had only senile pigmented modification of the fundus and her three daughters had mild macular pigmented changes, like "salt and pepper." Two genes are probably involved: one for protanopia with X linked recessive inheritance, the other responsible of CMT and tapeto-retinal degeneration, with an autosomal dominant inheritance, giving a 50% risk of recurrence.

Adolescent↗
PubMed1979

Differential diagnosis of limb-girdle muscular dystrophy and spinal muscular atrophy.

Neuromuscular biopsies from 18 patients with proximal muscle weakness were classified electromyographically as myopathy (11 cases), denervation (3 cases), or inconclusive (4 cases). Myopathic changes of muscle fibers occurred in all biopsies. Small angular dark fibers were observed in nine biopsies, and small-group atrophy in four biopsies from the three above-mentioned groups. Two biopsies classified as denervation showed large-group atrophy. Terminal innervation ratio (TIR) was increased only in the three cases classified as denervation and in one inconclusive case. TIR, which is more closely correlated with electromyographic (EMG) results than are muscle fiber changes, may help differentiate spinal muscular atrophy from limb-girdle muscular dystrophy.

Adolescent↗
PubMed1977

Familial focal loss of cross striations.

Two patients, a brother and sister, both suffering from congenital generalized muscle weakness with a progressive course are reported. Muscle biopsy revealed areas with loss of cross striations in the muscle fibers, electronmicroscopically presenting complete disorganization of the myofibrils with streaming of the Z discs and absence of mitochondria. Vesicular nuclei with prominent nucleoli were present in these areas. There was a type I fiber predominance in both cases. The mean diameter of the type I muscle fibers in one of the cases was too small. Motor endplate alterations in this patient gave no evidence of denervation but were suggestive of a delayed development of motor nerves.

Adolescent↗
PubMed1977

Morphological changes of motor units in Duchenne's muscular dystrophy.

We studied the intramuscular motor innervation in 19 muscle biopsy specimens from boys with Duchenne's muscular dystrophy and the fiber-type pattern was analyzed in nine biopsy specimens. The main change in motor innervation was a longitudinal displacement of motor end plates that could reach 10 mm and the presence of numerous unemployed axons ending freely within connective tissue. There was no increased collateral ramification of subterminal axons and the terminal innervation ratio was normal. The fiber-type pattern was characterized by a random variation of fiber diameters, a slight predominance of type 1 fibers, and an impared type differentiation in many fibers. The morphological data do not support the possibility of denervation and collateral reinnervation that could be related to the late component potentials found in Duchenne's muscular dystrophy.

Child↗
PubMed1977

Oculocraniosomatic neuromuscular disease with hypoparathyroidism.

During a six-year period, an adolescent girl developed a polyglandular disease characterized by hypoparathyroidism, chemical diabetes, growth failure and pubertal delay, hypercholesterolemia, and hypomagnesemia. A slowly progressive neurological disorder occurred simultaneously, consisting of progressive external ophthalmoplegia, mitochondrial myopathy, ataxia, neural deafness, mental subnormality, atypical retinitis, corneal dystrophy, cataract, and increased protein level in the cerebrospinal fluid. An intracardiac conduction defect was also found. This disorder, the cause of which is uncertain, is termed oculocraniosomatic disease. Our patient is apparently unique in that there was an associated hypoparathyroidism.

Adolescent↗
PubMed1977

[Polymyositis].

Explore the source record for details and available documents.

Adolescent↗
PubMed1976

Morphological and histochemical changes of motor units in myasthenia.

Neuromuscular biopsies were obtained from 45 myasthenic patients. Motor innervation was studied in all specimens by vital staining with methylene blue. Quantitative data included the proportion of elongated motor endings, and the terminal innervation ratio (TIR) of motor axons. Quantitative histochemical data, obtained on 12 biopsies, included the atrophy factors of type I and II fibers, the I/II ratio, and the proportion of fibers strongly reacting to both ATPase and NADH diaphorase (type III fibers). Statistical analysis of the data led to the following conclusions: (1) elongated motor endings, found in 26 biopsies, were not related to denervation or to the severity of the disease, and were preferentially observed in younger patients; (2) increased TIR suggesting denervation was observed in 7 biopsies, only in patients over 50 years; and (3) various histochemical changes were found, mainly numeric reduction of type II fibers, having no demonstrable relationship with the incidence of elongated motor endings. The highest TIR was observed in a biopsy containing an increased proportion of type III and intermediate muscle fibers.

Adolescent↗
PubMed1976

Changes in motor innervation and histochemical pattern of muscle fibers in some congenital myopathies.

Changes in motor innervation were compared with histologic and histochemical pattern of muscle fibers in three biopsies of central core disease, four biopsies of nemaline myopathy, one biopsy of myotubular myopathy, and three biopsies of mitochondrial myopathy. Evidence of collateral reinnervation was obtained only in one biopsy from central core disease. In other biopsies, no structural or ultrastructural abnormality of axis cylinders, myelin, or myoneural junction suggesting denervation were observed. The only relevant change found in centronuclear myopathy and to a lesser extent in nemaline myopathy was an unusual smallness and simplication of motor endings, suggesting delayed or impaired maturation. Muscle fibers strongly reactive for both adenosinetriphosphatase and nicotinamide-adenine dinucleotide diaphorase, observed in central core disease and mitochondrial myopathy, were not associated with increased terminal innervation ratio.

Adolescent↗