CNS COMPLICATIONS IN A GIRL WITH ANGIOIMMUNOBLASTIC LYMPHADENOPATHY WITH DYSPROTEINEMIA (AILD)

A 13-year-old girl presented with doughy swelling of both hands, a pru ritic maculopapular rash, fever, malaise, lymphadenopathy and splenome galy Examination of an enlarged cervical lymph node revealed typical h istopathological features of AILD. Partial remission was achieved by t reatment with prednisolone. During the next 4 years 3 flare-ups of the disease could be controlled by corticosteroids, one going along with an enlargement of the right tonsil, histologically proven as an AILD m anifestation, the other two with generalized lymphadenopathy. At the a ge of 17 years, an acute reduction of visual acuity occurred in both e yes in the absence of lymphadenopathy or cutaneous manifestations. Pla sma viscosity was elevated in connection with high levels of IgM and I gG. Fundoscopy revealed papilledema compatible with hyperviscosity syn drome. Plasmapheresis resulted in a slow recovery of vision when sudde nly a bilateral loss of vision occurred. MRI at this time revealed a l esion within the optic chiasm and additional high-signal lesions in pa rietal white matter of brain. All of these regions showed enhancement after i.v. application of Gd-DTPA. Again, high-dose corticosteroids we re introduced and a partial restoration of vision could be achieved. T his was paralleled by an improvement of the changes on follow-up MRI e xaminations. The last examination ten months after onset of CNS compli cations revealed a single small hyperintense residual area positioned in left parietal white matter. Enhancement of contrast medium was abse nt. These cerebral and retinal complications are so far undescribed co mplications of AILD which occurred in a childhood case.