Sustained response to subcutaneous immunoglobulins in chronic ataxic neuropathy with anti-disialosyl IgM antibodies (CANDA): report of two cases and review of the literature.

INTRODUCTION: Chronic ataxic neuropathy with anti-disialosyl IgM antibodies (CANDA) is a rare disorder for which the pathological, neurophysiological, and therapeutic evidence remains anecdotal and controversial. METHODS: This report on CANDA focuses on the neurophysiological patterns and treatment responses shared by two cases. One patient underwent nerve ultrasound follow-up. A comprehensive review of the literature highlighted the diverse experiences with different treatment options. RESULTS: Response to different therapies was similar in both patients: intravenous immunoglobulins achieved a favorable response albeit with significant wearing-off fluctuations; treatment with subcutaneous immunoglobulins (SCIg) was an effective alternative leading to a clinical response for at least 2 years. Rituximab, which was trialed in both patients, was not continued long enough to determine its efficacy in modifying the disease course and/or modulating responsiveness to immunoglobulins. Steroids caused clinical worsening in both patients. CONCLUSIONS: Immunoglobulin therapy appeared as the most effective in the treatment of these two patients. SCIg provided an effective treatment option for the long-term management of CANDA.

[Lyophilized bone homografts in reconstructive surgery of the middle ear]. / Omotrapianti ossei liofilizzati nella chirurgia ricostruttiva dell'orecchio medio.

The authors present their experience with stored lyophilized bone allografts utilized in middle ear reconstructive surgery. The implants were cut from the cortical substance of the long bone in subjects deceased. A demineralization in 0.6 N HCl solution was performed in order to induce osteoinductive capacity to the bone matrix. From November 1986 to December 1988 this material was utilized in 45 operations: 31 canal up techniques; 7 canal down techniques; 4 anatomical and functional rehabilitation of old radical cavities. Good anatomical results without perforation of the tympanic membrane and without signs of inflammation or retraction were obtained in 33 cases (79%). Furthermore, no extrusion was observed in cases with retraction of the graft and protrusion of the prosthesis. In cases in which the homografts were utilized for ossiculoplasty, the post-operative air-bone gap was 21 dB and the mean hearing gain was 15 dB. The histological examination of two homografts, removed 13-18 months after implantation, appeared to be coated with normal mucosa and to contain areas of active bone formation.