Creutzfeldt-Jakob disease with florid plaques after cadaveric dural graft in a Japanese woman

Abstract

The Japanese National Committee for Creutzfeldt-Jakob disease (CJD) reported there are 43 patients with CJD in Japan after implantation of cadaveric dura mater, and that no case of new-variant CJD (nvCJD) has been detected. We report a case with similar features to nvCJD. At the age of 38 years, a Japanese woman received a cadaveric dural graft (Lyodura, B Braun Melsungen AG, Germany) after a clipping procedure of the right middle cerebral artery aneurysm in September, 1985. In November, 1994, 110 months after the procedure, she became unsteady and had blurred vision. She was admitted to hospital because of progressing ataxia. Examination disclosed dementia, visual disturbance, hyperreflexia, and cerebellar ataxia. Blood chemistry and cerebrospinal fluid were normal. Brain computed tomography (CT) showed no abnormal lesions. There was slow activities without periodic discharge on her electroencephalogram (EEG). gradually decreased and could not be detected after 40 months. In the second patient, the IgA antibody value remained low. None of the remaining eight patients developed detectable IgA antibodies. Thus far, a total of 1707 infusions have been given and only six (0·4%) mild systemic adverse reactions (headache, nausea, and/or light dizziness) have been noted. Our preliminary experience indicates that prophylactic IgG replacement therapy can safely be given to patients with selective IgA deficiency including those with IgA antibodies by subcutaneous administration. The treatment seems to be effective as the frequency of bacterial respiratory tract infections was reduced. One possible explanation why administration of IgG is effective in IgA deficiency could be that the patients are given additional specific antibodies that they themselves are unable to produce in sufficient amounts. According to the estimated prevalence, the number of IgA deficient individuals is 20 000 in Sweden, 120 000 in UK, and at least 250 000 in the USA. Given that 30% of these have an increased susceptibility to infections, further studies are needed to decide whether to broaden the indications for administration of subcutaneous IgG.