Case Of Central Pontine Myelinolysis Research Articles

Central pontine myelinolysis

Summary This appears to be the first report of a case of central pontine myelinolysis associated with chronic alcoholism and liver cirrhosis in the United Kingdom. The pathological features and theories of aetiology are briefly discussed.

Central pontine myelinolysis: An ultrastructural and elemental study

Central pontine myelinolysis (CPM) has been classified as a unique disease of myelin with a peculiar localization in the central pons. Although its etiology and pathogenesis are unknown, some have compared its histopathology to that of multiple sclerosis. Ultrastructural studies of suitably preserved tissue have been lacking. We have recently studied 3 cases of CPM, selectively immunostaining 2 cases and examining the fine structure and elemental composition of the third case obtained shortly after death. IgG could not be demonstrated within or around the lesions. The findings of an increased Na K ratio and of intramyelinic vacuoles at the periphery of the lesion suggest that the pathogenesis of CPM might include a phase of intramyelinic edema with subsequent rupture of the distended myelin sheaths. An increase in the permeability of the blood-brain barrier might represent a complicating factor. The spheroids in our case are primarily reactive in type and do not support prior light-microscopic interpretations of concomitant neuroaxonal dystrophy. The unexplained presence of tin within the lesion indicates a need for further study of this element in CPM.

CENTRAL PONTINE MYELINOLYSIS:

The first case of central pontine myelinolysis in Scandinavia is presented. A premortem diagnosis was established on the basis of the history of chronic alcoholism, the picture of severe electrolyte disturbance and the development of the neurological syndrome. The postmortem findings verified the clinical diagnosis. A possible aetiological mechanism is postulated. The cause is believed to be a damage to the energy dependent electrolyte transport across the cell membrane, resulting in a cytotoxic oedema and death of the oligodendroglial cells of the brainstem which are particularly vulnerable.

Central pontine myelinolysis. Pathogenic aspects with reference to morphometric studies of pontine basis (author's transl)

23 cases of central pontine myelinolysis (CPM) were investigated histopathologically. According to the degree of glial reaction, two groups could be distinguished—those of early (8 cases) and late (15 cases) phases.

Central pontine myelinolysis in childhood and adolescence.

Central pontine myelinolysis, a pathological entity first described by Adams, Victor, and Mancall in 1959, is characterized by destruction of myelin sheaths in the central portion of the basis pontis, with relative sparing of both axis cylinders and neurones. Oligodendroglia are decreased in number or absent, and foamy macrophages accumulate in the involved area. Though the disorder may be suspected clinically, a diagnosis can be made only at necropsy. About 70 cases of central pontine myelinolysis have been reported (Adams, 1962; Adams et al., 1959; Aleu and Terry, 1963; Chason, Landers, and Gonzalez, 1964; Ito, Hasegawa, and Uchida, 1965; Kepes, Reece, and Oxley, 1965; Lopez and Collins, 1968; Mathieson and Olszewski, 1960; Matsuoka et al., 1965; McCormick and Danneel, 1967; Nakamura et al., 1966; Nishiyama, 1965; Rosman, Kakulas, and Richardson, 1966; Schneck, 1966; Shiraki, Ilzuka, and Seitelberger, 1963), of which 11 have occurred in childhood or adolescence (Table). The disease, however, may be more common in children than these reports suggest, since, in the past 3 years, we have observed the following 4 additional cases in patients aged 3, 31, 4i, and 15 years.

CENTRAL PONTINE MYELINOLYSIS. (A CASE REPORT).

A case of central pontine myelinolysis, the 19th so far published or communicated, is described in a Jewish woman of Yemenite extraction who had suffered for 12 years prior to her death from a mental condition diagnosed as chronic schizophrenia. Anatomo-pathologically the pontine lesion was similar to those described in previous cases, except that in this case there were “pools” of mucoid material present in it. The lesion developed in the absence of alcoholism and in association with a state of general dehydration and electrolyte imbalance in the patient.