Angiitis of the Central Nervous System After Allogeneic Bone Marrow Transplantation?

Abstract

Background and Purpose-There is only limited information about late neurological complications after bone marrow transplantation (BMT). The purpose of this study is to describe a cerebral angiitis-like syndrome after allogeneic BMT. Methods-Clinical and diagnostic findings of 5 BMT patients with chronic graft versus host disease and neuropathological data of 1 patient were reported. Results-In the described patients, focal neurological signs and neuropsychological abnormalities occurred years after BMT. MRI revealed periventricular white matter lesions, lacunar or territorial infarctions, leukoencephalopathy, and hemorrhages. Angiitis of the central nervous system was confirmed in 1 patient at autopsy, and an angiitis-like syndrome was suspected in the other patients because of the clinical course and response to treatment. Three patients received cyclophosphamide and steroids (2 improved, 1 died), 1 patient improved after steroids alone, and 1 patient without immunosuppressive therapy deteriorated further. Conclusions--We propose that an angiitis-like syndrome of the central nervous system can be a neurological manifestation of graft versus host disease, which should be considered a possible cause of cerebral ischemic episodes and pathological MRI scans in BMT patients with graft versus host disease. (Stroke. 1999;30:1651-1656.)

Cerebral angiitis in four patients with chronic GVHD

“…In contrast, cerebral infiltration has been reported in eight long-term survivors with chronic GVHD. [8][9][10][11][12][13] The histological pattern of inflammation was consistent with our findings concerning perivascular localization of lymphomononuclear infiltrates and glial activation. Involvement of the vessel wall was present in all of our patients, whereas transmural granulomas and occurrence of giant cells, as described earlier, 10,11 could not be detected.…”

“…Cerebral angiitis in association with chronic GVHD may involve both small and larger vessels, 13 consistent with a wide spectrum of observed MRI findings. We did not perform angiography because of its low sensitivity in the diagnosis of cerebral angiitis.…”

“…Owing to the short follow-up period, symptoms were mild and confined to the peripheral nervous system. 3 Severe angiitis-like syndromes were reported casuistically to occur in patients with chronic GVHD, but diagnosis was based either on angiography 7 or on histological analysis, [8][9][10][11][12][13] with a heterogenous spectrum of immunohistological methods used.…”

Central nervous system graft-versus-host disease presenting with granulomatous encephalitis

“…Of the previously reported cases, there are limited histological data available and variable pathological findings. 1,[3][4][5][6] Figure 1 MRI of the brain demonstrating right parietal lobe lesions.…”

Mesial temporal sclerosis – a late complication in four allogeneic pediatric recipients with persistent seizures after an acute episode of cyclosporine-A neurotoxicity

“…Viral pathogens (HHV6, EBV, CMV, Human papovavirus JC) were also ruled out by negative PCR on CSF. This picture, associated with an angiitis-like MRI pattern, 5,6 suggested an immunomediated neurological complication that led to treatment with CSA, steroids, and ECP. The EEG showed slow bilateral occipital and paroxysmal activity prevailing on the left side.…”