Auguste Deter
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Auguste Deter (; 16 May 1850 – 8 April 1906) was a
German German(s) may refer to: * Germany (of or related to) **Germania (historical use) * Germans, citizens of Germany, people of German ancestry, or native speakers of the German language ** For citizens of Germany, see also German nationality law **Ge ...
woman notable for being the first person to be diagnosed with
Alzheimer's disease Alzheimer's disease (AD) is a neurodegenerative disease that usually starts slowly and progressively worsens. It is the cause of 60–70% of cases of dementia. The most common early symptom is difficulty in remembering recent events. As ...
.


Life

Auguste was born in
Kassel Kassel (; in Germany, spelled Cassel until 1926) is a city on the Fulda River in northern Hesse, Germany. It is the administrative seat of the Regierungsbezirk Kassel and the district of the same name and had 201,048 inhabitants in December 2020 ...
,
Hesse-Kassel The Landgraviate of Hesse-Kassel (german: Landgrafschaft Hessen-Kassel), spelled Hesse-Cassel during its entire existence, was a state in the Holy Roman Empire that was directly subject to the Emperor. The state was created in 1567 when the L ...
on May 16, 1850. Born into a working-class family, Auguste had three siblings and was a daughter to Johannes Hohmann. Auguste's father died when she was young. Even though Auguste's family was impoverished, she was well-educated. She attended school in
Cassell Cassell may refer to: Companies * ''Cassell Military Paperbacks'', an imprint of Orion Publishing Group * ''Cassell's National Library'' * Cassell (publisher) (Cassell Illustrated or Cassell & Co.), a British book publisher now owned by the Orion ...
, and it is speculated that she may have been a student of Dr. Alois Alzheimer's grandfather, Johann. He was a schoolmaster in Cassell during the time Auguste attended school. Further education was not possible for Auguste due to social norms and her family's financial situation. Auguste started work as a full-time
seamstress A dressmaker, also known as a seamstress, is a person who makes custom clothing for women, such as dresses, blouses, and evening gowns. Dressmakers were historically known as mantua-makers, and are also known as a modiste or fabrician. Not ...
assistant at the age of 14. She continued this career until she married Carl August Wilhelm Deter on 1 May 1873, at the age of 23. In 1888, Carl began work as a railway clerk. After marrying Carl, Auguste moved to
Frankfurt Frankfurt, officially Frankfurt am Main (; Hessian: , " Frank ford on the Main"), is the most populous city in the German state of Hesse. Its 791,000 inhabitants as of 2022 make it the fifth-most populous city in Germany. Located on it ...
, Germany, where she was a full-time
housewife A housewife (also known as a homemaker or a stay-at-home mother/mom/mum) is a woman whose role is running or managing her family's home—housekeeping, which includes caring for her children; cleaning and maintaining the home; making, buying an ...
. Carl described their marriage as "happy and harmonious". The couple had one daughter named Thekla. Auguste became ill in the spring of 1901 at the age of 50. She was admitted to a
psychiatric hospital Psychiatric hospitals, also known as mental health hospitals, behavioral health hospitals, are hospitals or wards specializing in the treatment of severe mental disorders, such as schizophrenia, bipolar disorder, eating disorders, dissociat ...
later that year in November, where she lived for the rest of her life. Auguste and Carl were married for 33 years until her death on 8 April 1906 at the age of 55, just 5 weeks shy of her 56th birthday.


Onset of disease

During the late 1890s, Auguste exhibited a rapid escalation in memory loss and started showing symptoms of
dementia Dementia is a disorder which manifests as a set of related symptoms, which usually surfaces when the brain is damaged by injury or disease. The symptoms involve progressive impairments in memory, thinking, and behavior, which negatively affe ...
, such as loss of memory,
delusion A delusion is a false fixed belief that is not amenable to change in light of conflicting evidence. As a pathology, it is distinct from a belief based on false or incomplete information, confabulation, dogma, illusion, hallucination, or som ...
s, and even temporary
vegetative state A persistent vegetative state (PVS) or post-coma unresponsiveness (PCU) is a disorder of consciousness in which patients with severe brain damage are in a state of partial arousal rather than true awareness. After four weeks in a vegetative stat ...
s. In March 1901, Auguste's behavior started to become out of control. She began to accuse Carl of being adulterous and soon became jealous. Auguste started to become inattentive with housework, purposely hid objects and lost her capacity to cook. She also developed
insomnia Insomnia, also known as sleeplessness, is a sleep disorder in which people have trouble sleeping. They may have difficulty falling asleep, or staying asleep as long as desired. Insomnia is typically followed by daytime sleepiness, low energy, ...
, which caused her to drag sheets outside the house and scream for hours in the middle of the night. She became paranoid over neighbors and strangers as she believed someone was out to kill her. As a railway worker, Carl was unable to provide adequate care for his wife and was given recommendations by a local doctor to admit her into a mental hospital. She later was admitted to a mental institution, the (''Irrenschloss'') in Frankfurt on 25 November 1901. There, she was examined by Dr.
Alois Alzheimer Alois Alzheimer ( , , ; 14 June 1864 – 19 December 1915) was a German psychiatrist and neuropathologist and a colleague of Emil Kraepelin. Alzheimer is credited with identifying the first published case of "presenile dementia", which Kraep ...
. Carl visited Auguste whenever possible, though he struggled to make payments for her care and stay. It would have been more financially efficient to spend the time at work. Having difficulty keeping up with the payments, Carl continued insisting on getting her into a more affordable facility. Such a transfer would remove Auguste from Alzheimer's care, but Carl continued to persist in transferring Auguste. When asking Alzheimer for an arrangement of hospital transfers, Alzheimer discouraged him from such a decision; instead, he offered him an agreement for her to continue to receive care without cost in exchange for her medical records and brain after death, to which Carl gave a signed consent.


Treatment

Dr. Alzheimer asked her many questions, then later asked again to see if she would remember. He asked her to write her name. She tried to, but would forget the rest and repeat: "I have lost myself." (German: "Ich habe mich verloren.") He later put her in an isolation room for a while. When he released her, she would run out screaming, "I will not be cut. I do not cut myself." After many years, she became completely addled with dementia, muttering to herself. She died on 8 April 1906. More than a century later, her case was re-examined with modern medical technologies, where a genetic cause was found for her disease by scientists from
Gießen Giessen, spelled Gießen in German (), is a town in the German state (''Bundesland'') of Hesse, capital of both the district of Giessen and the administrative region of Giessen. The population is approximately 90,000, with roughly 37,000 univers ...
and
Sydney Sydney ( ) is the capital city of the state of New South Wales, and the most populous city in both Australia and Oceania. Located on Australia's east coast, the metropolis surrounds Sydney Harbour and extends about towards the Blue Mounta ...
. The results were published in the journal of ''
The Lancet Neurology ''The Lancet'' is a weekly peer-reviewed general medical journal and one of the oldest of its kind. It is also the world's highest-impact academic journal. It was founded in England in 1823. The journal publishes original research articles ...
''. According to this paper, a
mutation In biology, a mutation is an alteration in the nucleic acid sequence of the genome of an organism, virus, or extrachromosomal DNA. Viral genomes contain either DNA or RNA. Mutations result from errors during DNA or viral replication, m ...
in the
PSEN1 Presenilin-1 (PS-1) is a presenilin protein that in humans is encoded by the ''PSEN1'' gene. Presenilin-1 is one of the four core proteins in the gamma secretase complex, which is considered to play an important role in generation of amyloid beta ...
gene In biology, the word gene (from , ; "...Wilhelm Johannsen coined the word gene to describe the Mendelian units of heredity..." meaning ''generation'' or ''birth'' or ''gender'') can have several different meanings. The Mendelian gene is a b ...
was found, which alters the gamma-secretase function and is a known cause of early-onset
Alzheimer's disease Alzheimer's disease (AD) is a neurodegenerative disease that usually starts slowly and progressively worsens. It is the cause of 60–70% of cases of dementia. The most common early symptom is difficulty in remembering recent events. As ...
. However, the results could not be replicated in a more recent paper published in 2014 where "Auguste D's DNA revealed no indication of a nonsynonymous hetero- or
homozygous Zygosity (the noun, zygote, is from the Greek "yoked," from "yoke") () is the degree to which both copies of a chromosome or gene have the same genetic sequence. In other words, it is the degree of similarity of the alleles in an organism. Mo ...
mutation In biology, a mutation is an alteration in the nucleic acid sequence of the genome of an organism, virus, or extrachromosomal DNA. Viral genomes contain either DNA or RNA. Mutations result from errors during DNA or viral replication, m ...
in the
exons An exon is any part of a gene that will form a part of the final mature RNA produced by that gene after introns have been removed by RNA splicing. The term ''exon'' refers to both the DNA sequence within a gene and to the corresponding seque ...
of APP, PSEN1, and
PSEN2 Presenilin-2 is a protein that (in humans) is encoded by the ''PSEN2'' gene. Function Alzheimer's disease (AD) patients with an inherited form of the disease carry mutations in the presenilin proteins (PSEN1; PSEN2) or the amyloid precursor p ...
genes comprising the already known familial AD mutations." It is suggested that Auguste's daughter Thekla would have had a 50% chance of inheriting the PSEN1 gene and developing Alzheimer's, although there is no recorded information of her developing such an illness. Alzheimer concluded that she had no sense of time or place. She could barely remember details of her life and frequently gave answers that had nothing to do with the question and were incoherent. Her moods changed rapidly between
anxiety Anxiety is an emotion which is characterized by an unpleasant state of inner turmoil and includes feelings of dread over anticipated events. Anxiety is different than fear in that the former is defined as the anticipation of a future threat wh ...
, mistrust, withdrawal and 'whininess.' They could not let her wander around the wards because she would accost other patients who would then assault her. It was not the first time that Dr. Alzheimer had seen a complete degeneration of the psyche in patients, but previously the patients had been in their seventies. Ms. Deter piqued his curiosity because she was much younger. In the weeks following, he continued to question her and record her responses. She frequently responded, "Oh, God!" and, "I have lost myself, so to say." She seemed to be consciously aware of her helplessness. Alzheimer called it the "Disease of Forgetfulness".


Death and legacy

In 1902, Alzheimer left the "Irrenschloss" (Castle of the Insane), as the Institution was known colloquially, to take up a position in
Munich Munich ( ; german: München ; bar, Minga ) is the capital and most populous city of the German state of Bavaria. With a population of 1,558,395 inhabitants as of 31 July 2020, it is the third-largest city in Germany, after Berlin and ...
, but made frequent calls to
Frankfurt Frankfurt, officially Frankfurt am Main (; Hessian: , " Frank ford on the Main"), is the most populous city in the German state of Hesse. Its 791,000 inhabitants as of 2022 make it the fifth-most populous city in Germany. Located on it ...
inquiring about Deter's condition. On 9 April 1906, Alzheimer received a call that Auguste Deter had died. He requested that her medical records and brain be sent to him. Her chart recorded that in the last years of her life, her condition had deteriorated considerably. Her death was the result of
sepsis Sepsis, formerly known as septicemia (septicaemia in British English) or blood poisoning, is a life-threatening condition that arises when the body's response to infection causes injury to its own tissues and organs. This initial stage is follo ...
caused by an infected
bedsore Pressure ulcers, also known as pressure sores, bed sores or pressure injuries, are localised damage to the skin and/or underlying tissue that usually occur over a bony prominence as a result of usually long-term pressure, or pressure in combi ...
. With the aid of Italian physicians, Gaetano Perusini and Francesco Bonfiglio they carefully examined her brain to discover
senile plaques Amyloid plaques (also known as neuritic plaques, amyloid beta plaques or senile plaques) are extracellular deposits of the amyloid beta (Aβ) protein mainly in the grey matter of the brain. Degenerative neuronal elements and an abundance of micr ...
and
neurofibrillary tangles Neurofibrillary tangles (NFTs) are aggregates of hyperphosphorylated tau protein that are most commonly known as a primary biomarker of Alzheimer's disease. Their presence is also found in numerous other diseases known as tauopathies. Little is ...
. These would be the hallmark of Alzheimer's Disease as scientists know it today. Auguste would have been diagnosed with early-onset Alzheimer's disease if seen by a current-day doctor.


Rediscovery of medical record

In 1996, Dr. Konrad Maurer and his colleagues, Drs. Volk and Gerbaldo, rediscovered the medical records of Auguste Deter. In these documents, Dr. Alzheimer had recorded his examination of his patient, including her answers to his questions: :''"What is your name?"'' :''"Auguste."'' :''"Family name?"'' :''"Auguste."'' :''"What is your husband's name?"'' - she hesitates, finally answers: :''"I believe ... Auguste."'' :''"Your husband?"'' :''"Oh, my husband."'' :''"How old are you?"'' :''"Fifty-one."'' :''"Where do you live?"'' :''"Oh, you have been to our place."'' :''"Are you married?"'' :''"Oh, I am so confused."'' :''"Where are you right now?"'' :''"Here and everywhere, here and now, you must not think badly of me."'' :''"Where are you at the moment?"'' :''"We will live there."'' :''"Where is your bed?"'' :''"Where should it be?"'' Around midday, Frau Auguste D. ate pork and cauliflower. :''"What are you eating?"'' :''"Spinach."'' (She was chewing meat.) :''"What are you eating now?"'' :''"First I eat potatoes and then horseradish."'' :''"Write a '5'."'' erman: ''fünf'':''She writes: "A woman"'' 'Frau'':''"Write an '8'."'' 'acht'':''She writes: "Auguse"'' ('' sic'', while she is writing she repeatedly says, "I have lost myself, so to say.")


References


External links

*
Alois Alzheimer
Who Named It?
Alzheimer's: 100 years on

Alois Alzheimer's Biography
International Brain Research Organization
Ludwig-Maximilians-University Munich, Laboratory for Neurodegenerative Disease Research
– Prof. Dr. Christian Haass
Bibliography of secondary sources
on Alois Alzheimer and Alzheimer's disease, selected from peer-reviewed journals. * Graeber Manuel B
"Alois Alzheimer (1864–1915)"
International Brain Research Organization
"The Story of Auguste Deter"
ref> {{DEFAULTSORT:Deter, Auguste 1850 births 1906 deaths People from Kassel People from the Electorate of Hesse Alzheimer's disease 1901 in Germany Deaths from dementia in Germany Deaths from Alzheimer's disease