Cerebellum and brainstem vasculopathy in systemic lupus erythematosus: two clinico-pathological cases.

Abstract

The case histories are reported of two patients with systemic lupus erythematosus (SLE) who developed fatal neurological involvement, each presenting with cerebeliar and brainstem signs. Neuro-pathological changes were confined to the cerebellum and brainstem, with clear evidence of a small vessel vasculopathy. The predominant histological abnormalities were multiple perivascular haemorrhages in one case and classical vasculitic changes in the other.

Histopathological analysis of cerebral hemorrhage in systemic lupus erythematosus complicated with antiphospholipid syndrome

“…It should be pointed out, however, that, as reported by Smith et al, the clinicopathological features of 2 SLE patients with vasculopathy in the brain were found to be, in one case, vasculitis with fibrinoid necrosis in a cerebellar vessel, whereas in the other case there was perivascular hemorrhage in small vessels containing fibrin thrombi, without any features of acute vasculitis [16]. In the brain hematoma of our patient, there was inflammatory cell accumulation mostly on the walls of small arteries, inducing rupture of the lamina elastica interna.…”

Vasculitis in the central nervous system

“…In SLE, brain pathology most often reveals a vasculopathy, with small vessel thickening, hyalinization, intramural platelet deposition, and thrombus formation (73). Frank vasculitis is uncommon (<7%) but has been documented in patients with acute, dramatic, and often fatal CNS presentations (74)(75)(76). The pathology and pathogenesis of vascular injury in SLE have recently been reviewed (77).…”

Controversies in lupus: nervous system involvement.

“…Similar findings were reported in two subsequent clinicopathological studies-one performed between 1955 and 1977 on 57 patients,2 and the most recent between 1972 and 1989.3 Infrequently, the lesions may be restricted to the cerebellum and mid-brain. 4 The association of the microvascular lesions with microinfarcts suggested that occlusion of small vessels is the basis for the damage to the nervous system. While the histopathological features are straightforward, the clinical spectrum of CNS involvement is broad.…”

“…It is evident that the role of cytokines in lupus requires further study. 4 Diagnosis In reviewing the extensive range of immunoserological, electrophysiological and neuroimaging techniques which have been investigated as possible markers of CNS lupus, it is apparent that there is still no single test which is diagnostic. Diagnosis of neuropsychiatric SLE, therefore, is a matter of clinical acumen.…”