Maurice Victor

A STUDY OF THE NUTRITIONAL DEFECT IN WERNICKE'S SYNDROME: THE EFFECT OF A PURIFIED DIET, THIAMINE, AND OTHER VITAMINS ON THE CLINICAL MANIFESTATIONS

The salient clinical features of Wernickes syndrome (1) are partial to complete paralysis of extra-ocular muscles (most commonly the external recti), nystagmus, ataxia and mental disturbances. Postmortem examination reveals changes in the nervous structures adjacent to the third and fourth ventricles and the aqueduct. The lesions are characterized by varying degrees of necrosis of both nerve cells and nerve fibers with appropriate reactions of microglia and astrocytes, alteration of the small blood vessels, and in some cases, petechial hemorrhages (2-5). The syndrome is usually associated with chronic alcoholism and for that reason has sometimes been attributed to a neurotoxic effect of the alcohol; but the occurrence of the same clinical sequence in non-alcoholic patients with malnutrition (1, 2, 4-8) has directed attention to nutritional deficiency as the etiological basis. Moreover, similar pathological lesions have been produced in the rat (9, 10), fox (11, 12), and pigeon (3, 9, 13, 14), by maintaining these animals on thiamine-deficient diets.

THE ULTRASTRUCTURE OF MYELIN AND AXONAL ALTERATIONS IN SCIATIC NERVES OF THIAMINE DEFICIENT AND CHRONICALLY STARVED RATS.

SummaryPhase and electron microscopic study of sciatic and peroneal nerves of thiamine deficient and chronically starved rats demonstrated an increase in the incidence of normal variations in myelin sheath contour throughout the internode, focal alterations in the lamellar pattern of myelin adjacent to sites of demyelination, and numerous, complex, redundant loops of axon-Schwann cell membrane which projected into axons of myelinated fibers. The paranodal axoplasm of a few fibers from thiamine deficient rats contained collections of vesicles, mitochondria and dense bodies. The significance of these observations is briefly discussed.ZusammenfassungDie Wirkung des chronischen Thiaminmangels auf das periphere Nervensystem wurde bei Ratten mit Hilfe des Phasenkontrast- und Elektronenmikroskops untersucht. Zu diesem Zweck wurden die Tiere einer Mangeldiät von 15 bis 35 Wochen Dauer unterworfen. Sie zeigen in dieser Zeit typische Veränderungen, nämlich einen Gewichtsverlust, eine auffällige Langsamkeit der Bewegungen, sowie Schwäche, Ataxie und Tod. Für die morphologischen Untersuchungen wurden die Tiere in verschiedenen Zeitabschnitten getötet und die Nervi ischiadici et peronaei in Osmiumtetroxyd fixiert und in Epon eingebettet. Phasenkontrast-und elektronenmikroskopische Untersuchungen zeigen einen Zuwachs der schon normalerweise zu beobachtenden Variationen der Markscheidenform über das ganze Internodium hinweg. Außerdem treten fokale Änderungen im lamellären Muster des Myelins in der unmittelbaren Nachbarschaft von Demyelinisationen auf. Dazu kommen zahlreiche komplexe Schlingen und Schleifen der Schwannschen Zellmembran, die in den Achsencylinder myeliniserter Fasern vorspringen. Das paranodale Axoplasma einzelner Nervenfasern von durch Thiaminmangel geschädigten Ratten läßt auffällige Ansammlungen von Bläschen, Mitochondrien und sogenannten „dense bodies” erkennen.

Circulatory Studies in Wernicke's Encephalopathy With Special Reference to the Occurrence of a State of High Cardiac Output and Postural Hypotension

Although Wernickes disease is clearly related to thiamine deficiency, it is rarely accompanied by the overt signs of congestive failure. There are symptoms of disordered cardiovascular function in many patients with this disease, but the relation of these symptoms to thiamine deficiency has remained undefined. In the present study, an attempt is made to define these symptoms, particularly, in relation to specific treatment with thiamine. Part 1 of this study is concerned mainly with the determination of the cardiac output in 7 patients, as determined by the Evans blue dye-dilution technic; part 2 describes the postural response to tilting in 12 patients.

Case 26-1967

Presentation of Case First admission. A fifty-six-year-old man was admitted to the hospital because of the radiologic demonstration of an abnormality in the lung field. Three months previously he entered the Massachusetts Eye and Ear Infirmary because of retinal detachment. At that time the white-cell count was 6000, with 61 per cent neutrophils, 33 per cent lymphocytes, 5 per cent monocytes and 1 per cent eosinophils. A week before admission he returned for repair of leakage at the site of the detachment. The white-cell count was 5100, with 65 per cent neutrophils, 26 per cent lymphocytes, 1 per cent .xa0.xa0.