There is growing evidence that GVHD affects the central nervous system (CNS). In this study, we describe the longterm follow-up of four allogeneic BM recipients who developed cerebral angiitis-like disease probably due to GVHD. The patients developed focal neurological signs, cognitive deficits and/or coma in association with GVHD, 2-18 years after transplantation, following reduction of immunosuppressive therapy. Magnetic resonance imaging was variable, showing generalized brain atrophy, ischemic lesions or leukoencephalopathy. Diagnosis of cerebral angiitis was confirmed by histopathological analysis of bioptic brain tissue and response to immunosuppressive therapy. By means of immunohistochemistry and immunofluorescence, perivascular lymphomononuclear cerebral infiltrates were shown to express the adhesion receptor, CD11a, and the chemokine receptor, CCR5. Our findings imply that GVHD should be considered in the differential diagnosis of noninfectious angiitis-like disease of the CNS in long-term survivors after allogeneic BMT. Infiltrating cells, in analogy to typical target organs of GVHD such as skin or liver, expressed CD11a and CCR5. These findings could be of etiopathological, diagnostic and therapeutic relevance.
infectious polyradiculitis, which, to our knowledge, has not yet been While receiving intravenous antibiotic therapy (ampicillin, 2 g six reported in association with L. monocytogenes infection, best explains times daily, and gentamicin, 240 mg/day), the patient developed distal our findings. flaccid peripheral pareses within the first 4 days. Foot flexion andThe patient developed a nephrotic syndrome with hypoalbumiextension were not possible on the left and were impaired on the right. nemia, pleural effusions, and proteinuria, which resolved with Arm muscle weakness was moderate on the left and severe on the symptomatic treatment. Infection of the pleural cavity with L. moright. Deep tendon reflexes had diminished, and the ankle reflexes were nocytogenes [6,7] is unlikely because of regressive inflammatory absent. The patient complained of tingling in the hands and feet, and parameters at the time of onset. Therefore, parainfectious etiology vibratory sensation was diminished distally (3/8). Cranial and spinal of nephrotic syndrome was assumed, although, to our knowledge, MRIs were normal. Somatosensory evoked responses showed no pean association with L. monocytogenes infection has not yet been ripheral waves. Brainstem auditory evoked responses were normal.described. Infectious pericarditis and myocarditis due to L. monoNerve conduction studies revealed absent (left median, right ulnar, and cytogenes have been reported as serious complications [8, 9]. In right peroneal nerves) or delayed (left tibial nerve) F-wave responses the patient we described, perimyocarditis resolved rapidly. Thus, consistent with polyradiculitis. Motor nerve conduction velocities were a parainfectious etiology appears to be possible. No underlying normal or slightly reduced; distal motor latency was delayed in one autoimmune disease was found. Lymphoma or hypergammaglonerve. Compound muscle action potentials were slightly reduced in bulinemia due to myeloma or Waldenström's macroglobulinemia amplitude. Sensory nerve action potentials were diminished or missing.are unlikely causes, because organ complications remitted with Needle electromyography showed no denervation signs.antibiotic treatment. After 3 days, the patient developed respiratory impairment due toIn conclusion, in our immunocompetent patient with L. monocymuscle weakness (vital capacity, 1.4 L) and bilateral pleural effusions.togenes infection, the unusual manifestations of polyradiculitis, Blood tests revealed hypoalbuminemia (albumin level, 23 g/L) and nephrotic syndrome, and perimyocarditis took a benign course and nonselective proteinuria (protein excretion rate, 10 g/day), which sugshould be considered as possible complications in the management gested a nephrotic syndrome. The serum urea level was slightly eleof patients with L. monocytogenes infection. vated, whereas the creatinine level and creatinine clearance remained normal. Kidney biopsy showed mild focal and segmental glomerulo- 5. Bolton CF. Sepsis and the systemic inflammatory response syndrome: neu-
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