NR AKNX
AU Schoene,W.C.; Masters,C.L.; Gibbs,C.J.Jr.; Gajdusek,D.C.; Tyler,H.R.; Moore,F.D.; Dammin,G.J.
TI Transmissible spongiform encephalopathy (Creutzfeldt-Jakob disease). Atypical clinical and pathological findings.
QU Archives of Neurology 1981 Aug; 38(8): 473-7
PT journal article
AB A middle-aged neurosurgeon had an 18-month illness characterized by abnormal sleep patterns, paresthesias, and necrotizing cutaneous lesions with vasculitis and signs of cerebral, brainstem, vestibulocerebellar, and progressive spinal cord involvement. Biopsy specimens of nerve and skin showed an acute vasculitis with endovascular cellular proliferation in the pattern of a Kohlmeier-Degos lesion and focal epidermal necrosis. Mental changes and cranial-nerve signs developed. Myoclonus occurred occasionally during sleep. Akinetic mutism ensued. At autopsy, major abnormalities were limited to the nervous system and skin. Spongiform encephalopathy typical of Creutzfeldt-Jakob disease was found with amyloid kuru plaques. A cribriform change distinct from the spongiform change was seen focally in the white matter. Scarred skin lesions and a healed, partially obliterative arteritis were noted. Inoculation of brain and lung into nonhuman primates resulted in a spongiform encephalopathy.
IN Ein Neurochirurg mittleren Alters starb an der Creutzfeldt-Jakob-Krankheit nach 18-monatiger Krankheit mit gestörtem Schlafmuster, Erschlaffungslähmungen, mentalen Veränderungen, Myoklonien im Schlaf, akinetischem Mutismus und nekrotischen Hautarealen mit Vasculitis und Anzeichen für zentralnervöse Beteiligung. Im Gehirn wurden die typische schwammförmige Degeneration sowie amyloide Kuru-Plaques gefunden. Mit Hirn und Lunge dieses Patienten ließ sich die Creutzfeldt-Jakob-Krankheit auf Primaten übertragen.
MH Brain Stem/pathology; Case Report; Creutzfeldt-Jakob Syndrome/*pathology; Ethmoid Bone/pathology; Human; Male; Middle Age; Paresthesia/etiology; Skin/pathology; Sleep Disorders/etiology; Spinal Cord/pathology; Sural Nerve/pathology
SP englisch
PO USA