SPINAL CORD - Neurofibromatosis

This consists of the lower part of the spinal cord and the cauda equina. There are numerous nodular expansions of the nerve fibres, ranging in size from a few mm. to just over 1 cm
The patient suffered from congenital neurofibromatosis. His mother had died of this disease, some seven years before he died at the age of 22, following removal of a second acoustic neuroma. He had also had bilateral acoustic neuromas, together with peripheral lesions. He developed a complete block at the level of 8th thoracic vertebra due to a neurofibroma, and he became paraplegic. Following exploration of a left-sided cerebello-pontine tumour, his cerebellum became infarcted, and death occurred. At autopsy, a number of subcutaneous nodules were seen, but no other abnormality than that in the central nervous system was noted.