Vagal nerve stimulation increases right ventricular contraction and relaxation and heart rate.

Objective: To determine the effects of vasoactive intestinal peptide, released from the right and left vagal nerves, on ventricular contraction, relaxation, and heart rate.

Methods: The muscarinic and beta-adrenergic receptors were blocked with atropine and propranolol, and afterload was controlled in 48 anesthetized, open-chest mongrel dogs. Experiments were performed in the absence (Series 1, 10 dogs) and in the presence (Series 2, 22 dogs) of a controlled heart rate and prior to and after the administration of a sensitive and selective vasoactive intestinal peptide antagonist (Series 3, 16 dogs).

Type 2 fiber predominance in muscle cramp and exertional myalgia.

In patients complaining of muscle cramps and exertional myalgia, we found a significant decrease of type 1 muscle fiber proportion in comparison with a control group. The possible mechanism of this change is discussed.

Familial myopathies with restricted distribution, facial weakness and inflammatory changes in affected muscles.

A myopathy characterized by restricted involvement of few muscles and inflammatory cell infiltration was observed in three families. In the first family, clinical features, hereditary transmission and biopsy findings were consistent with the diagnosis of facioscapulohumeral dystrophy. However in three of the four affected members, the occurrence of atrophies was specifically initiated by severe muscular pain.

Unclassified familial myopathy resembling Steinert disease, without myotonia.

A familial myopathy with predominantly proximal muscle atrophy is described. Although several clinical features such as sternomastoid and brachioradialis muscle involvement suggested Steinert disease, myotonia was not demonstrated by clinical examination or EMG. Histological data were consistent with myotonic dystrophy.

[Changes in the motor units in patients with traumatic paraplegia].

Neuromuscular biopsies were obtained in 10 patients with traumatic spinal cord lesions at various levels and in one patient with cerebral lesions due to a long lasting coma. In all cases, there was a rapid onset of both fibers types atrophy, with numerous transitional type III and intermediate fibers. Only the two patients with cervical lesions had an increased terminal innervation ratio suggesting denervation.

Neurogenic benign fasciculations, pseudomyotonia, and pseudotetany. A disease in search of a name.

We studied two patients with abnormal spontaneous muscular activity. The first had widespread fasciculations, painful spasms, delayed muscular relaxation, and hyperhidrosis. Improvement occurred after several years.

Adult form of basal cell naevus syndrome: a family study.

A 32-year-old patient had marked reduction of visual acuity due to falciform folds of the retina and retinal detachment, and severe neurological abnormality: bilateral pyramidal involvement, fasciculation in all limbs and gait ataxia. Skull radiographs showed internal frontal hyperostosis; CT scan showed calcification of the falx cerebri, and multiple arachnoid cysts were shown by myelography. A naevoid lesion had previously been removed from the left forearm.

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.

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.

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.

[Familial periodic paralysis with hypokaliemia, hyperaldosteronism and extracellular vacuolization (author's transl)].

The observation of a 20 years old patient with a periodic paralysis is reported. During a crisis induced by 20 U of insulin and 150 g of glucose, transient hyperaldosteronism was found and the muscular biopsy performed in the left biceps brachii, disclosed vacuoles with a double membrane. The significance of these findings is discussed.

Motor innervation and fiber type pattern in amyotrophic lateral sclerosis and in Charcot-Marie-Tooth disease.

A quantitive study of the terminal innervation ratio (TIR) was conducted using 18 amyotrophic lateral sclerosis (ALS) and 12 Charcot-Marie-Tooth disease (CMT) muscle biopsies. Morphometric and histochemical analyses of muscle fibers were performed in 9 ALS and 6 CMT biopsies. The results revealed that TIR and type grouping were significantly greater in CMT than in ALS.

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.

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.

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.

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.

[Changes of motor innervation in myasthenic muscles in relation to age (author's transl)].

The motor innervation in muscle biopsies from 45 myasthenic patients was studied by intravital staining with methylene blue. The enzymatic pattern and fibre type distribution was analysed in 12 cases. Quantitative data including the proportion of elongated motor endings and the terminal innervation ratio (TIR) of motor axons, were compared to histochemical data and to clinical data including age of patients.

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.