TY - JOUR T1 - Progressive medullary failure associated with neurofibrillary degeneration. JF - Journal of Neurology, Neurosurgery & Psychiatry JO - J Neurol Neurosurg Psychiatry SP - 643 LP - 647 DO - 10.1136/jnnp.52.5.643 VL - 52 IS - 5 AU - H G Lidov AU - L W Duchen AU - P K Thomas AU - D C Thrush Y1 - 1989/05/01 UR - http://jnnp.bmj.com/content/52/5/643.abstract N2 - 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. ER -