Auguste Ds dementia

In addition to Alzheimer disease, other hypothetical diagnoses of Auguste D.'s disease have been put forward, especially arteriosclerosis and, astonishingly, metachromatic leukodystrophy. Many postmortem diagnoses listed arteriosclerosis at this time. In Auguste D.'s file, Alzheimer himself noted "Arteriosklerotische Gehirnatrophie." The question mark is interesting and also appeared in the autopsy report: "Arteriosklerose der kleinen Hirngefäße." However, the histopathologic details in the 1907 and 1909 publications always pointed to vessels without arteriosclerosis: Perusini found "that the large vessels, the arterial circle of Willis, and the Sylvian arteries showed no significant sign of arteriosclerosis"; only "some regressive alterations of the arterial walls" were described. In both papers the presence of the neuritic plaques and neurofibrillary tangles was confirmed.

There are a number of convincing arguments against the assumption of a metachromatic leukodystrophy in Auguste D.'s case, as suggested by Amaducci (Amaducci et al. 1991). The clinical picture of Auguste D. bears only a limited resemblance to the symptoms of metachromatic leukodystrophy. In particular, key symptoms caused by involvement of the peripheral nervous system are lacking. Alzheimer was an experienced neuropsychiatrist, and it is unlikely that he would have missed clear symptoms concerning the disease.

All discussions about Auguste D.'s illness should end now that the tissue sections from Auguste D., discovered by Graeber and co-workers in 1998, have been examined (Fig. 13) "There were numerous neurofibrillary tangles and many amyloid plaques, especially in the upper cortical layer of this patient. Yet, there was no microscopic evidence for vascular—i.e., arteriosclerotic lesions (Graeber et al., 1998). Thus, considering the publications and the file of Auguste D., it becomes more and more evident that she is the initial case of Alzheimer's disease."

RalfDahm

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