Theodor Wanko

Studies in familial periodic paralysis

Abstract This is a report on a study of a family with familial periodic paralysis in an attempt to clarify the underlying pathogenesis of the disorder. It was found in this particular family that no marked cationic ingress into muscle fibers could be demonstrated. Large dilatations containing granules of material staining with a positive periodic Schiff reaction were demonstrated with the paralysis. That such cisterns contained also water was confirmed. Intracellular recordings demonstrated no hyperpolarization of the membrane potential. During paralysis no propagated impulse could be recorded from intracellular electrodes by indirect or direct stimulation of the muscle fiber. This was indirectly confirmed by the presence of large quiescent areas in the electromyograph. Direct measurement of aldosterone excretion showed no increase of this hormone preceding or during the paralysis. Injection of aldosterone did not precipitate paralysis. No change in blood volume was noted during paralysis. Pitressin would not precipitate an attack. Thus aldosterone seems unlikely to be an etiological agent in this disorder. The action of thyroidal and thyrotropic hormones in the familial form of this disorder appears to be opposite to that described in the sporadic form. Electron microscopic studies suggest that fluid is accumulated in the endoplasmic reticulum and that there is a characteristic change in the mitochrondria during the paralysis. Such changes are reversible. Limited studies in carbohydrate metabolism with these patients suggest an abnormality in which intermediaty metabolites in the formation of glycogen may be responsible for the intracellular shift of water into the fiber to maintain osmotic, electric, and Donnans equilibriums. Enzymatic studies of muscle homogenates would be the next logical study. In these patients as in others reported, paralysis may be precipitated in the presence of normal serum potassium values.

An electron microscopic study of T2 bacteriophage in thin sections

Thin sections of osmium-fixed T2L bacteriophage were examined in the electron microscope. A DNA-free region near the center of the phage head was resolved. The dimensions of this region were about 150 × 70 A, with the largest diameter parallel to the long axis of the phage head.

The muscle fiber in central core disease

SummaryFrom correlated histochemical and electron microscope investigations, of a biopsy specimen of central core disease, several pathological characteristics are recognized in the muscle cell. The central core area consists of amassed myofilaments without distinct separation into fibrils. This region contains a pathological, filament-like opaque substance which is thought to be derived from the Z band. The interfibrillar spaces in the core region are greatly reduced or absent. Concomitantly, neither mitochondria nor an elaborate reticulum is present: this is reflected histochemically in the absence of lipoid and basophilic elements. The periphery of the muscle fiber outside the core appears normal.ZusammenfassungHistochemische und elektronenmikroskopische Untersuchungen an Biopsiematerial eines Falles von Zentralfibrillenerkrankung der Muskelfasern (central core disease) führen zur Feststellung verschiedener pathologischer Merkmale. So besteht die Zone der zentralen Fibrillen aus einer Anhäufung von Myofilamenten ohne Unterteilung in Fibrillen. Außerdem enthalten sie eine krankhafte, opake Formation in filamentartiger Anordnung, die vermutlich mit dem Z-Streifen in Zusammenhang steht. Der interfibrilläre Zellraum in der Zentralregion ist entweder nicht oder nur in geringstem Ausmaße vorhanden. Gleichzeitig fehlen an dieser Stelle sowohl Mitochondrien als auch ein voll ausgebildetes sarkoplasmisches Reticulum, ein Befund, dem auch die histochemisch erhobene Abwesenheit von lipoiden und basophilen Gewebselementen entspricht. Der periphere Anteil der Muskelfaser entspricht der Norm.

Central Core Disease: A Myofibrillary and Mitochondrial Abnormality of Muscle: Combined Clinical Staff Conference of the National Institutes of Health

Excerpt Dr. G. Milton Shy: We should like to take this opportunity to present what appears to be a new disease of muscle. This disorder was first described from these Institutes in 1956 (1). At tha...