US location of the adductor canal hiatus: morphologic study

In the lower extremities, the adductor canal hiatus is a site of predilection for arterial stenoses and occlusions. The high incidence of vascular disease in this region is thought to be due to a local factor. To gain more insight into the mechanisms leading to such disease, the authors used ultrasound to locate the adductor canal hiatus in dissecting room preparations and in healthy volunteers.     

Congenital nemaline myopathy. I. Defective organization of alpha-actinin is restricted to muscle

This investigation was undertaken to find out whether the structural and biochemical changes seen in skeletal muscles in congenital nemaline myopathy (CNM) occurred also in nonmuscle cells. It was confirmed that nemaline bodies contain α-actinin. The distribution of actin and α-actinin in the examined nonmuscle cells was considered normal. The motility of the leucocytes and the fibroblasts was indistinguishable from that of those in normal controls. Slight changes in the peripheral nerve fibers of one patient were found, but these were not seen in another patient. The results indicate that congenital nemaline myopathy is not a generalized disorder but is restricted to skeletal muscle.     

Wirtschaftliche Vorteile aus Vertragsarztzulassung als nicht abnutzbares immaterielles Wirtschaftsgut

Abstrakt Der wirtschaftliche Vorteil aus einer Vertragsarztzulassung stellt ein nicht abnutzbares immaterielles Wirtschaftsgut dar.     

Estimation of the number of motor units based on macro-EMG.

The technique of the macro-EMG was used to estimate the number of motor units in the tibialis anterior muscles of healthy subjects in a wide range of ages, and of patients with myasthenia gravis and patients with amyotrophic lateral sclerosis or spinal muscular atrophy. The results obtained suggest a decrease in the number of motor units in the tibialis anterior muscle with increasing age in normal subjects. In myasthenic patients the motor unit count was within the normal range for their age group. Patients with motor neuron disorders on the average had a very low number of motor units.     

The heterogeneity of distal arthrogryposis

A study of distal arthrogryposis is described including neurology, electromyography, cerebral and muscular CT-scanning, and muscle and nerve biopsies. In four cases presenting with congenital distal contractures, various neuromuscular disorders were diagnosed. They were respectively, congenital myopathy with core-like structures, congenital hypertrophic neuropathy, axonal neuropathy and anterior horn cell disease. The role of cerebral disorders in the pathogenesis of distal contractures is also considered. The significance of abnormal dermatoglyphics in the determination of the prenatal time of onset of congenital myopathies and arthrogryposis is discussed. Our findings provide evidence for the hypothesis that distal arthrogryposis may not be a distinct clinical entity with an autosomal dominant inheritance pattern, but a symptom, indicating various cerebral, neuromuscular and connective tissue disorders, present in numerous congenital syndromes with different modes of inheritance. In addition the value of electromyography, nerve conduction velocity studies, muscle and cerebral CT-scanning, and histology of muscle and nerve biopsies in the differential diagnosis of (distal) arthrogryposis is stressed.     

Systemic carnitine deficiency: Benefit of oral carnitine supplements vs. persisting biochemical abnormalities

A patient is described who was admitted with a condition similar to the Reye syndrome at the age of 9 months. Hypoglycemia, hyperammonemia, hepatomegaly, and lethargy were present. The plasma concentrations of free and acylcarnitine were extremely low and the urine contained excessive amounts of dicarboxylic acids. Extensive biochemical and histological investigations of biopsied liver and muscle led to the diagnosis of systemic carnitine deficiency. The patient was put on oral carnitine treatment, upon which he remained clinically well. A prolonged fasting test during this treatment gave abnormal results: there was no ketonemia, but an increase of -oxidation of fatty acids. In spite of the treatment the liver and muscle carnitine content remained below normal.     

The inferior oblique as muscle of choice for biopsies of extraocular muscles.

Suitable biopsies can easily be taken from the inferior oblique muscle without unwanted side effects from the belly of this muscle, allowing complete examination by light and electron microscopy. The inferior oblique is the muscle of choice for biopsy studies of extraocular muscle.     

Proximal weakness of the extremities as main feature of amyloid myopathy.

Two patients with muscle weakness caused by amyloid myopathy are described. Characteristic features such as pseudohypertrophy and abnormal firmness, and tumours of muscles were absent. It is suggested that muscle weakness in amyloid myopathy is caused by layers of amyloid covering muscle fibres. In middle aged or elderly patients with proximal muscle weakness the diagnosis of amyloid myopathy should be considered.