Role of autoimmunity in retinitis pigmentosa.

Twenty-five cases of retinitis pigmentosa were investigated to assess the cell-mediated immunity and for evidence of autoimmunity by measuring peripheral blood T-cell rosette count, cutaneous DTH response to recall antigens (Candida and purified protein derivative [PPD]), and 2:4 dinitrochlorobenzene (DNCB) and antiretinal antibody by tanned red cell hemagglutination technique. It was observed that cell-mediated immunity was significantly depressed, and antiretinal antibody was found in 60% of the cases, which correlated with the duration and severity of the disease. We conclude that although retinitis pigmentosa is genetically determined, patients develop autoimmunity against retinal tissue due to suppression of cell-mediated immunity. Association of rheumatoid factor in 8% of the cases further confirms the loss of homeostatic control owing to suppressed cell-mediated immunity.

The radiological features of craniofacial neurofibromatosis.

Three cases of neurofibromatosis are reported showing a wide spectrum of roentgen abnormalities affecting the skull and facial bones. One case had macrocrania and multiple calvarial defects; enlargement of the sella turcica and middle cranial fossa was also present. All the three cases had orbital changes either in the form of enlargement, reduced anteroposterior width or loss of bony outlines with an appearance of emptiness. The other two cases showed predominantly facial involvement with hypoplasia of the mandibular body and ramus, abnormal coronoid process, zygomatic arch, hypoplastic maxilla, cystic mandible with abnormal dentition and absence of the osseous external auditory meatus. Intra-orbital calcification was present in one case. The radiological projections required to evaluate such cases are discussed. The 20 degrees occipito-mental view has been found extremely useful and is recommended.