Chronic Rheumatic Encephalitis, Torsion Dystonia and Hallervorden-Spatz Disease

Abstract

IT IS GENERALLY accepted that Sydenham's chorea, or chorea minor, is a manifestation of rheumatic fever. It was only logical, therefore, that McCulloch1 (1938) should use the term "encephalitis rheumatica" for Sydenham's chorea, as had been done before him by Poynton and Paine 2 and by Greenfield.8 Greenfield studied the pathologic features of chorea minor in patients who died during the acute stage of the illness. He observed round cell infiltration, consisting of lymphocytes and plasma cells, within the perivascular spaces. More impressive, however, than the infiltration of elements of the blood was the glial reaction of the brain tissue around the perivascular spaces. Sometimes there was present only this spotty glial reaction, which resembles that seen in poliomyelitis and von Economo's (lethargic) encephalitis. There was a great increase in vascularity, with congestion of the capillaries and even subpial hemorrhages. Greenfield expressed the belief that the picture resembled the acute stage of encephalitis lethargica. In less severe cases the involvement of the brain is not so damaging, and McCulloch pointed out that acute rheumatic encephalitis is one of the exudative manifestations of rheumatic fever, rather than a proliferative one. In less severe cases no structural change of the type of rheumatic nodules or scars were observed in the brain. This is prob¬ ably the reason that chorea is commonly not followed by permanent paralysis, weakness or other neurologic alteration. McCulloch sug¬ gested, therefore, that the encephalitis of rheumatic fever is similar in its anatomic process to the arthritis, the acute pericarditis and the myocardial changes of an acute attack of rheumatic fever. The first changes

Hallervorden‐Spatz disease

“…They represent the specific response to several pathogenetic processes that have been found associated with different clinical and etiological situations. Yakolev (1942) described them associated in myoclonic epilepsy, Netsky et al (1951) in amyotrophic lateral sclerosis, Benda (1949) in chronic rheumatic encephalitis, and Van Bogaert (1939) in postencephalitic Parkinsonism and (1947) in familial neurofibromatosis. We think the emphasis should be on the presence of the association of clinical and histological changes, allowing a range of variation for both components.…”

“…Information on the ethnic and national background of the different families was not found in all the cases, and in most of them we assigned nationality according to the country from which the report originated. The familial cases have been reported from: Germany, 2 (Hallervorden & Spatz 1922, Kalinowsky 1927USA, 3 (Winkelman 1932, Herman & Schilder 1933, Benda 1949; Italy, 2 (Bini & Papetti 1943, Dooling et al 1974; Belgium, 2 (Myle & Fadilogu 1967, Radermecker & Rizzuto 1967; and one each from the following countries: Brazil (Borges-Fortes 1947), Romania (Urechia et al 1950), Switzerland (Rabinowicz & Wildi 1957), Hungary (Kornyey 1964), Japan (Yanagisawa et al 1966), Yugoslavia (GrEeviE 1966), Canada (Rozdilsky et al 1968, and the Columbian family, subject of this report.…”

“…This was an excellent opportunity to study inter-and intrafamilial variation of a condition which appears to be very well defined. It is the purpose of this paper to present our observations in this family, including the anatomo-pathological findings in one of them, and to compare the variation of the condition within this family with other familial cases and with the sporadic cases reported in the literature (Seitelberger et al 1963/64, Helfand 1935, Meyer & Earl 1936, Benda 1949, Bornstein et al 1966, Rademecker & Rizzuto 1967, Wigboldus & Bruyn 1968, and Roth et al 1971.…”

“…They were selected according to criteria of Wigboldus & Bruyn (1968) because the clinical and pathological findings were within the range of the original description and they were familial. Other cases in the literature were clinically and pathologically typical but sporadic (Seitelberger et al 19631 64, Helfand 1935, Meyer & Earl 1936, Benda 1949, Bornstein et al 1966, Rademecker & Rizzuto 1967, Wigboldus & Bruyn 1968, and Roth 1971, and they are also included in this analysis. A timetable of the appearance of the different signs and symptoms reported by Yanagisawa et al (1966) unfortunately could not be obtained since most reports do not include timing and evolution.…”