CNS complications in a girl with angioimmunoblastic lymphadenopathy with dysproteinemia (AILD)

A 13-year-old girl presented with doughy swelling of both hands, a pruritic maculopapular rash, fever, malaise, lymphadenopathy and splenomegaly. Examination of an enlarged cervical lymph node revealed typical histopathological features of AILD. Partial remission was achieved by treatment 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 manifestation, the other two with generalized lymphadenopathy. At the age of 17 years, an acute reduction of visual acuity occurred in both eyes in the absence of lymphadenopathy or cutaneous manifestations. Plasma viscosity was elevated in connection with high levels of IgM and IgG. Fundoscopy revealed papilledema compatible with hyperviscosity syndrome. Plasmapheresis resulted in a slow recovery of vision when suddenly a bilateral loss of vision occurred. MRI at this time revealed a lesion within the optic chiasm and additional high-signal lesions in parietal white matter of brain. All of these regions showed enhancement after i.v. application of Gd-DTPA. Again, high-dose corticosteroids were introduced and a partial restoration of vision could be achieved. This was paralleled by an improvement of the changes on followup MRI examinations. The last examination ten months after onset of CNS complications revealed a single small hyperintense residual area positioned in left parietal white matter. Enhancement of contrast medium was absent. These cerebral and retinal complications are so far undescribed complications of AILD which occurred in a childhood case.