In 1901, Auguste Deter, a 51-year-old woman, was admitted to the Asylum for the Insane and Epileptic in Frankfurt am Main. She had been brought to the doctor by her husband for evaluation of pathological jealousy that had progressed to rapid memory loss. In addition, she had become disoriented in her home and had a fixed delusion that someone was trying to kill her.
In the asylum, she was examined by Alzheimer and found to be confused and disoriented to time and place, with reduplicative paramnesia (acting as if the hospital was her home). She also evidenced intermittent auditory hallucinations and lengthy bouts of screaming, that worsened when anyone approached. She alternated between believing that her attending physician was trying to harm her and then being overly familiar with him. Her short-term memory deficits were profound, forgetting objects shown to her almost immediately. Her language functioning was characterized by fluent, paraphasic, somewhat empty speech, with poor comprehension, impaired reading, and dysgraphia containing repeated or omitted syllables. She also was agnosic and apraxic.
Four and a half years after admission to the asylum, Auguste Deter died at age 56, following a steady, downhill course.
A psychiatrist and neuropathologist named Alois Alzheimer received Deter’s brain for analysis from the asylum’s director. He found “an evenly atrophic brain without macroscopic focal degeneration.” After preparing over 250 histological slides and utilizing silver staining methods, he identified extracellular plaques and, for the first time, intracellular neurofibrillary tangles.
On November 3, 1906, at a meeting of Southwest German psychiatrists in Tübingen, Alzheimer presented the case of Auguste Deter. Alzheimer’s paper, entitled “On an Unusual Malady of the Cerebral Cortex,” described the clinical manifestations of this new syndrome and also “anatomical characteristics [found in the brain on autopsy] which set it apart from all recognized cases.” Eighty-eight individuals attended the talk; no questions were asked at its conclusion. Alzheimer’s 1907 publication of the Auguste Deter case also received little attention.
For decades, the histological slides of Deter’s brain were lost. Then, in 1997, a year after psychiatrists at the University of Frankfurt discovered her original hospital records, a team of researchers located a trove of slides, each labeled “Deter,” in the basement of the Institute of Neuropathology of the University of Munich. Microscopic review revealed that Auguste Deter indeed had the classical plaques and tangles that we now associate with Alzheimer’s disease.
Importance of this case:
Franz Nissl wrote of Alzheimer that he “was first and foremost a psychiatrist who strove to advance psychiatry by using a microscope.” At the time of Alzheimer’s case report, Alzheimer’s disease had not been differentiated from other forms of mental illness. As recently as 1975, Medline indexed only 40 papers in which “Alzheimer’s disease” was a keyword.
Auguste Deter was the first of several cases Alzheimer would investigate using the clinico-anatomic method, establishing the core elements of the clinical description and pathology of Alzheimer’s disease that remain valid today. Auguste Deter’s disease was almost certainly an aggressive, familial, early-onset variety.
In 1910, Emil Kraepelin introduced the term “Alzheimer’s presenile dementia” in his 8th edition of the Handbook of Psychiatry. Kraepelin, who headed the Munich Royal Psychiatric Clinic in which Alzheimer was chief of neuropathology, may have named the disease for Alzheimer to differentiate it from the more common senile-onset dementias and to promulgate the idea of biological causes of mental illness. It would take decades for Alzheimer’s “unusual malady” to become recognized as a common dementia.
While Deter’s brain exhibited the widespread plaques and tangles now known to be characteristic of Alzheimer disease, Deter’s clinical presentation was atypical for Alzheimer disease; agitation and psychotic symptomatology overshadowed her memory loss. Alzheimer’s work demonstrated the value of investigating microscopic cellular pathology in association with behavioral alterations, and this has led to the elucidation of large classes of neurodegenerative diseases..
Excerpted from: “Six Landmark Case Reports Essential for Neuropsychiatric Literacy”
Authors: Sheldon Benjamin, M.D., Lindsey MacGillivray, M.D., Ph.D., Barbara Schildkrout, M.D., Alexis Cohen-Oram, M.D., Margo D. Lauterbach, M.D., Leonard L. Levin, M.S. L.I.S., M.A.