Characteristics of patients with variably protease-sensitive
phonopathy whose brain samples were selected for transmission studies * Patient, Age at Disease Clinical signs sex death, duration, and symptoms y mo NL-VV, 57 20 Progressive dementia, M([dagger]) spastic paraplegia, sensorimotor polyneuropathy UK-VV, 59 42 Progressive dementia, F([double emotional and obsessive dagger]) behavior (early), very occasional myoclonus (late) UK-MV, 76 12 Forgetfulness, visuospatial M([section]) perceptual problems, difficulties walking, action tremor, akinetic mutism Patient, Neuropathologic sex features NL-VV, Mild to moderate spongiform change M([dagger]) in basal ganglia, and cerebral and cerebellar cortices.
