Über eine eigenartige herdförmige Erkrankung des Zentralnervensystems.

Berlin: Springer, 1920.

First edition, in the original printed wrappers, of the first description of what later became known as Creutzfeldt-Jakob disease, or CJD, following its independent identification by Alfons Jakob one year later. “Hans Gerhard Creutzfeldt and Alfons Jakob independently authored clinical and pathologic descriptions of a new syndrome in the 1920s. This syndrome, which subsequently came to be named after them, was characterized by dementia, motor and coordination abnormalities, a fatal course, and pathologic findings of diffuse spongiform neuronal degeneration. Although it appeared for many years to be little more than a medical curiosity, Creutzfeldt-Jakob disease attained widespread attention by its pathologic similarity to kuru and bovine spongiform encephalopathy, ‘mad cow disease.’ Because there are sporadic, familial, and iatrogenic forms of Creutzfeldt-Jakob disease, it is considered to have both genetic and infectious aspects. Although its causation has for some time been ascribed to ‘slow viruses,’ the etiology of Creutzfeldt-Jakob disease is currently thought to be due to prions, small proteinaceous infectious particles that have genetic encoding. The debate regarding whether the appearance of atypical Creutzfeldt-Jakob disease can be linked to the epidemic of ‘mad cow disease’ is currently unresolved” (Sternbach et al, p. 701).

“While working at Alzheimer’s neuropsychiatric clinic, ‘Creutzfeldt studied the case of a 22-year old woman suffering from tremors, spasticity, and pyramidal signs. She became ataxic and demented. Her later symptoms included nystagmus, rigidity, myoclonus, and mutism; she ultimately died in status epilepticus 12 months after the onset. At Autopsy, Creutzfeldt found moderate cerebral atrophy with a patchy diffuse neuronal loss, pronounced astroglial hypertrophy, and bilateral degeneration of the corticospinal tracts. Creutzfeldt did not mention either neuronal vacuolation or a spongy state. Because of the war, publication was delayed until 1920. In 1921, Alfons Jakob (1884-1931) described three cases of ‘spastic pseudosclerosis, disseminated encephalomyelopathy’, and subsequently reported two other cases [‘Über eigenartige Erkrankungen des Zentralnervensystems mit bemerkenswerten anatomischen Befunden,’ Zeitschrift für die gesamte Neurologie und Psychiatrie 64, 147–228]. Jakob had examined Creutzfeldt’s slides, and noting similarities among some of their cases, grouped them together as examples of ‘spastic pseudosclerosis’, a term Creutzfeldt disagreed with, believing their cases constituted a new clinical entity” (Encyclopedia of the Neurological Sciences, Vol. 1, p. 898). Creutzfeldt’s material was lost during World War II.

“Hans-Gerhard Creutzfeldt (1885-1964) was born in Harburg, Germany, and studied medicine in Jena, Rostock, and Kiel, where he received his MD in 1908. He wrote his thesis on tumors of the pituitary gland. From 1910 to 1912, he worked as a ship’s doctor and then studied neuropathology with Ludwig Edinger in 1912. He spent 2 years with Alois Alzheimer in Breslau (present Wroclaw, Poland). After World War I, he worked with Walther Spielmeyer in Munich and with Ernst Siemerling in Kiel. For the next 14 years, he worked in the Department of Psychiatry of the Charité Hospital in Berlin with Karl Bonhoeffer. He returned to Kiel in 1938, was appointed director of Neuropsychiatry at the Christian-Albrechts University, and served there until 1953. He was one of the few academics who refused to join the Nazi party. He became the first postwar Rector of the University (1945-46). He ended his academic career at the Psychiatric Institute of Munich in 1955. He died after a long illness in 1964” (ibid.).

Garrison-Morton 4719.1. Sternbach, Dibble & Varon, ‘From Creutzfeldt-Jakob disease to the mad cow epidemic,’ Journal of Emergency Medicine 15 (1997), 701-5.

Large 8vo (244 x 163 mm), pp. 1-18 in Zeitschrift für die gesamte Neurologie und Psychiatrie, 57 Band, entire issue offered here; pp. [vi], 358, [iv]. Original printed wrappers, unopened. Some small dents to margins from having been tied with string.

Item #3853

Price: $1,500.00

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