Lymphomatoid granulomatosis (LYG)/angiocentric immunoproliferative lesions (AIL) consist of angiocentric and angiodestructive lymphoreticular proliferation predominantly involving the lungs and other extranodal sites, such as the central nervous system (CNS). This clinical entity is considered as a B cell process related to Epstein-Barr virus (
EBV) infection and EBV positive
diffuse large B-cell lymphoma. The CNS is involved in 20% of cases of LYG, but initial involvement is rare. In cases without pulmonary symptoms, diagnosis may be difficult. We report a rare case involving initial progression of CNS symptoms followed by a pulmonary abnormality.A 14-year-old girl suffered from high
fever, ataxic gait and
paraparesis. MRI revealed diffuse T2 high signals with multiple
gadolinium enhancements in the cerebellum, brain stem and cerebral white matter. Her symptoms briefly improved after
steroid therapy, but
ataxia gradually progressed.
Dyspnea due to pulmonary interstitial involvement appeared when she was 18 years old.
Steroid therapy proved effective for respiratory symptoms. At 20 years old she suffered from disseminated intravascular coagulopathy (
DIC) and
hemophagocytic syndrome (HPS) with respiratory symptoms and repeated
seizures. Her symptoms improved after the administration of
cyclophosphamide. Mild
hemiparesis and gait disturbance appeared when she was 22 years old. MRI revealed new lesions at the basal ganglia and subcortical white matter, brain
atrophy and diffuse T2 high intensity of cerebral white matter.
Cyclophosphamide was effective and there has been no recurrence of symptoms in the last 5 years. We reviewed the non-tumorous LYG/AIL involving the CNS, and discussed the clinical features, MRI imaging and diagnosis of the LYG/AIL.