PT - JOURNAL ARTICLE AU - H G Lidov AU - L W Duchen AU - P K Thomas AU - D C Thrush TI - Progressive medullary failure associated with neurofibrillary degeneration. AID - 10.1136/jnnp.52.5.643 DP - 1989 May 01 TA - Journal of Neurology, Neurosurgery & Psychiatry PG - 643--647 VI - 52 IP - 5 4099 - http://jnnp.bmj.com/content/52/5/643.short 4100 - http://jnnp.bmj.com/content/52/5/643.full SO - J Neurol Neurosurg Psychiatry1989 May 01; 52 AB - A clinicopathological report is presented of a British male, aged 59 years, who died after an illness of 10 years, manifested by progressive respiratory failure, ptosis, and dysphagia. At no time was there evidence of ophthalmoplegia, Parkinsonism or dementia. At necropsy the main finding was of neurofibrillary tangles in the neurons of the pontine and medullary reticular formation, with particularly severe involvement of the nucleus ambiguus, dorsal motor nucleus of the vagus and nucleus tractus solitarius. Morphologically, by light and electron microscopy and immunostaining, the tangles were similar to those of other neurofibrillary degenerative diseases. Although similar in some respects to progressive supranuclear palsy and amyotrophic lateral sclerosis of the Guam type, the combination of clinical and neuropathological features suggest that this is a distinct disease entity.