Depression as a cerebral manifestation of scleroderma: Immunological findings in serum and cerebrospinal fluid

Abstract

The immunological hallmark of systemic scleroderma is the appearance of various autoantibodies in the serum (Meurer et al 1985; Chorzelski et al 1985; Tan 1988). In contrast to other collagenous vascular diseases, such as systemic lupus erythematodes (LE) and periarteritis nodosa, the nervous system is described as being rarely involved in systemic scleroderma (Gordon and Silverstein 1970). There exists a great amount of information about psychiatric symptoms in systemic LE (Feinglass et al 1976), but in patients with systemic scleroderma psychiatric manifestations have very rarely been noted; only one patient (Wise and Ginzler 1975) has been reported in the literature as suffering from an acute organic brain syndrome, resulting in hallucinations, paranoid delusions, disorientation, and hyperactivity. This case, however, showed positive antinuclear antibodies (ANA) but no cerebrospinal fluid (CSF) abnormalities and the psychiatric s ymptomatology responded to systemic corticos*,eroid therapy (Wise and Ginzler 1957). Two older case reports described depressive syndromes in patients suffering from systemic scleroderma (Sensemann 1957; Gulledge 1968), but no results of neuropsychiatric organic investigations such as CSF and electroencephalogram (EEG) have been noted. On the other hand, reports in the literature of pathological EEGs (Gottwald and Sturm 198 l) and an involvement of the autonomic nervous system (K|imiuk et al 1988) in scleroderma patients suggest that psychiatric manifestations in scleroderma are more common. In an extensive study, Gordon and Silverstein (1970) found neurological symptoms in about 18% ,~ 130 patients with systemic scleroderma. So far, however, neurological involvement has been mostly observed as cranial nerve lesions, mainly IV (Teasdall et al 1980) and V nerve (Mejias 1986; Berth-Jones et al 1990; Kabadi and Sinkoff 1977; Thompson and Robertson 1973) neuropathy. Until now there are few findings suggesting a direct central nervous system (CNS) involvement in scleroderma, possibly because less attention has been given to it. The case reported here shows scleroderma-typical autoantibodies and inflammatory signs in the CNS, presenting it as a chronic depressive syndrome.