Anti-fucosyl-GM1 ganglioside IgG and IgM autoantibodies in human serum: no link to pathology.

Anti-fucosyl-GM1 ganglioside antibodies were detected in sera of five persons: four patients with autoimmune neuropathies and more recently, IgG antibodies in one with Graves' disease (Adler et al., Autoimmunity 18, 149-152, 1994) [1]. In the latter case, we were unable to find any relation between the occurrence of antibodies and thyroid disease.

Fiber density in congenital muscle fiber type disproportion. II. Congenital muscle hypotonia and hip dislocation.

Our previous paper presenting electromyographic findings in patients with congenital fiber type disproportion myopathy, confirmed the myogenic character of the disease process. That group of patients was however fairly heterogenous regarding both the clinical features and the morphological changes in muscle fibers (e.g.

Fiber density in congenital muscle fiber type disproportion. I. Congenital myopathies.

In 15 cases with congenital muscle fiber type disproportion, concentric needle EMG (CNEMG) and single fiber EMG (SFEMG) with fiber density (FD) estimation was performed. The aim of the work was to establish the nature of congenital muscle fiber type disproportion. In 11 cases CNEMG revealed interference pattern on maximal effort and low, polyphasic motor unit action potentials (MUAPs) of short duration indicating a myopathic lesion.

Changes in respiratory muscle function after neostigmine injection in patients with myasthenia gravis.

In sixteen patients with myasthenia gravis, the weakness of respiratory muscles, especially of expiratory, caused some disturbances in lung function. Thirty minutes after injection of neostigmine, general improvement of muscle function was accompanied by an increase of strength and endurance of respiratory muscles. The mean value of maximal inspiratory mouth pressure (PImax) increased by 33%, maximal expiratory mouth pressure (PEmax) by 23.

[Spinal cord tumors simulating the clinical course of Charcot-Marie disease].

The authors describe two cases of extramedullary tumours in the vertebral canal with long (7 and 15 years) course without pains and with clinical manifestations simulating Charcot-Marie-Tooth disease. It is stressed in conclusion that sporadic cases of this disease with involvement of the lower extremities only, with normal conduction velocity in the peroneal nerves should be differentiated against spinal cord tumours situated in the vertebral canal.

Is Kugelberg-Welander spinal muscular atrophy a fetal defect?

Twelve children who had developed Kugelberg-Welander (K-W) spinal muscular atrophy (SMA) before the age of six years were investigated. Electrophysiological, histochemical, ultrastructural, and biochemical studies demonstrated features of immature muscle fibers suggesting a fetal defect as in the Werdnig-Hoffmann (W-H) form of SMA. Comparison with known patterns of human myogenesis and of experimental denervation of immature muscle suggested that the defect in K-W SMA probably takes place in fetal life at a stage later than in W-H SMA.

[Case of extraocular muscle paralysis with changes of the ragged-red fiber type].

The authors describe a case of progressive paresis of the extraocular muscles in a 57-year-old woman followed up since 1974. There were no abnormalities in the biochemical investigations, EEG, EMG, ECG, pneumoencephalography and tests for myasthenia. Only in fragments of the biceps brachii muscle histological and histochemical investigations showed mitochondrial changes of the type of "ragged-red fibres".

Evaluation of results of thymectomy in myasthenia gravis.

The results of thymectomy carried out in 150 cases of myasthenia gravis are discussed. In a group of 123 cases followed for 1 to 5 years after the operation, full remission was observed in 24.4% of cases, significant improvement in 36.

Development of chequerboard pattern in human foetal skeletal muscle.

The study was undertaken to race the histochemical and electron microscopic development patterns of human myogenesis from the 9th to the 26th week of foetal life. Particular attention was paid to the possibility of appearance of metabolic or structural differences between individual skeletal muscle fibres in early periods of myogenesis. The 9th week of foetal life is the period when primitive myotubes are formed.

Surgical treatment of myasthenia by thymectomy.

From 1967 to 1973 Thymectomies for Myasthenia gravis have been done at the Zakopane Chest Surgery Hospital. Preoperative duration of the disease was some 2 1/2 years. Histological types: Thymoma 6, Thymus persistens 61, malignant lymphatical growth 1.