Auguste Deter: Difference between revisions

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→‎Treatment: I thought it would be good to add information about her daughter's chance of developing Alzheimer's given that it is talking about DNA.
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Dr. Alzheimer asked her many questions, and later asked again to see if she remembered. He told 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."<ref>{{cite journal |doi=10.1016/S0140-6736(96)10203-8 |title=Auguste D and Alzheimer's disease |journal=The Lancet |volume=349 |issue=9064 |pages=1546–9 |year=1997 |last1=Maurer |first1=Konrad |last2=Volk |first2=Stephan |last3=Gerbaldo |first3=Hector }}</ref>
Dr. Alzheimer asked her many questions, and later asked again to see if she remembered. He told 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."<ref>{{cite journal |doi=10.1016/S0140-6736(96)10203-8 |title=Auguste D and Alzheimer's disease |journal=The Lancet |volume=349 |issue=9064 |pages=1546–9 |year=1997 |last1=Maurer |first1=Konrad |last2=Volk |first2=Stephan |last3=Gerbaldo |first3=Hector }}</ref>


After many years, she became completely demented, 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 and Sydney. The results were published in the journal ''[[The Lancet Neurology]]''. According to this paper, a mutation in the [[PSEN1]] gene was found, which alters the gamma-secretase function and is a known cause of early-onset Alzheimer's disease.<ref>{{cite journal |doi=10.1016/S1474-4422(12)70307-1 |pmid=23246540 |title=A presenilin 1 mutation in the first case of Alzheimer's disease |journal=The Lancet Neurology |volume=12 |issue=2 |pages=129–30 |year=2013 |last1=Müller |first1=Ulrich |last2=Winter |first2=Pia |last3=Graeber |first3=Manuel B }}</ref> 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 mutation in the exons of [[Amyloid precursor protein|APP]], [[PSEN1]], and [[PSEN2]] genes comprising the already known familial [[Alzheimer%27s_disease#Genetic|AD mutations]]."<ref>{{cite journal |doi=10.1016/j.jalz.2014.06.005 |pmid=25130656 |title=A presenilin 1 mutation in the first case of Alzheimer's disease: Revisited |journal=Alzheimer's & Dementia |volume=10 |issue=6 |pages=869–72 |year=2014 |last1=Rupp |first1=Carsten |last2=Beyreuther |first2=Konrad |last3=Maurer |first3=Konrad |last4=Kins |first4=Stefan }}</ref> 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. No doubt that any of her decedents would develop Alzheimer’s
After many years, she became completely demented, 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 and Sydney. The results were published in the journal ''[[The Lancet Neurology]]''. According to this paper, a mutation in the [[PSEN1]] gene was found, which alters the gamma-secretase function and is a known cause of early-onset Alzheimer's disease.<ref>{{cite journal |doi=10.1016/S1474-4422(12)70307-1 |pmid=23246540 |title=A presenilin 1 mutation in the first case of Alzheimer's disease |journal=The Lancet Neurology |volume=12 |issue=2 |pages=129–30 |year=2013 |last1=Müller |first1=Ulrich |last2=Winter |first2=Pia |last3=Graeber |first3=Manuel B }}</ref> 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 mutation in the exons of [[Amyloid precursor protein|APP]], [[PSEN1]], and [[PSEN2]] genes comprising the already known familial [[Alzheimer%27s_disease#Genetic|AD mutations]]."<ref>{{cite journal |doi=10.1016/j.jalz.2014.06.005 |pmid=25130656 |title=A presenilin 1 mutation in the first case of Alzheimer's disease: Revisited |journal=Alzheimer's & Dementia |volume=10 |issue=6 |pages=869–72 |year=2014 |last1=Rupp |first1=Carsten |last2=Beyreuther |first2=Konrad |last3=Maurer |first3=Konrad |last4=Kins |first4=Stefan }}</ref> 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. No doubt that any of her decedents would develop Alzheimer’s <ref>{{cite journal |last1=Keohane & Grace |first1=Kieran & Victoria |title=What is 'Alzheimer's Disease'? The 'Auguste D' Case Re-opened |journal=Cult Med Psychiatry |date=Feb 2019 |volume=43 |page=359 |pages=336 |doi=https://doi.org/10.1007/s11013-019-09622-z |url=https://doi.org/10.1007/s11013-019-09622-z}}</ref>


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, 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."
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, 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."

Revision as of 20:06, 17 March 2021

Auguste Deter
Born
Johanna Auguste Caroline Hochmann

(1850-05-16)16 May 1850
Kassel, Germany
Died8 April 1906(1906-04-08) (aged 55)
Frankfurt, Germany
NationalityGerman
Known forFirst diagnosis of Alzheimer's
SpouseCarl August Wilhelm Deter

Auguste Deter (German pronunciation: [aʊ̯ˈɡʊstə ˈdeːtɐ]; 16 May 1850 – 8 April 1906) was a German woman notable for being the first person to be diagnosed with Alzheimer's disease.

Life

Auguste was born and raised in Cassell (Kassell), Germany, into a working-class family on May 16th, 1850. She had three siblings and was a daughter to Johannes Hohmann. He died when Auguste was at a young age. Even though Auguste’s family was impoverished, she was well educated. She attended school in Cassell, and it is speculated that she may have been a student of Alzheimer’s grandfather, Johann. He was a schoolmaster in Cassell during the time Auguste attended school. Further education would have never been considered for Auguste due to the social climate. Education is based upon social class & gender rather than skill. Auguste started work as a full-time seamstress assistant when she was at the age of 14. She continued this career until She married Carl (Karl) August Wilhelm Deter on 1st, 1873, at the age of 23.

Carl worked as a railway clerk since its opening in 1888. After marrying Carl, she moved to Frankfurt, Germany, to start her duty as a full-time housewife. Carl described their marriage as “Happy and harmonious.” Together they had one daughter named Thekla. Auguste regularly cooked, cleaned and raised her daughter Thekla until she became ill in the spring of 1901 at the age of 50. She was later that year in November admitted to a mental hospital where she lived the rest of her life. Auguste and Carl have been married for 33 years until her death on April 8th, 1906 is the age of 55, being 5 weeks shy of her 56th birthday.[1]

Onset of disease

During the late 1890s, Auguste exhibited a rapid escalation in memory loss & started showing symptoms of dementia, such as loss of memory, delusions, and even temporary vegetative states. [2] 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 & loss her capacity to cook. She developed insomnia 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 Institution for the Mentally Ill and for Epileptics (Irrenschloss) in Frankfurt, Germany on 25 November 1901. There, she was examined by Dr. Alois Alzheimer.

Carl visited Auguste whenever possible, though he struggled to make payments for her care & 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. 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 & brain after death, to which Carl gave a signed consent. [3]

Treatment

Dr. Alzheimer asked her many questions, and later asked again to see if she remembered. He told 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."[4]

After many years, she became completely demented, 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 and Sydney. The results were published in the journal The Lancet Neurology. According to this paper, a mutation in the PSEN1 gene was found, which alters the gamma-secretase function and is a known cause of early-onset Alzheimer's disease.[5] 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 mutation in the exons of APP, PSEN1, and PSEN2 genes comprising the already known familial AD mutations."[6] 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. No doubt that any of her decedents would develop Alzheimer’s [7]

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, 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, but made frequent calls to Frankfurt inquiring about Deter's condition. On 9 April 1906, Alzheimer received a call from Frankfurt 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 caused by an infected bedsore. With the aid of Italian physicians, Gaetano Perusini & Francesco Bonfiglio they carefully examined her brain to discover senile plaques and neurofibrillary tangles. 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.[8]

Rediscovery of medical record

In 1996, Dr. Konrad Maurer and his colleagues, Drs. Volk and Gerbaldo, rediscovered the medical records of Auguste Deter.[9] 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'." [German: 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

  1. ^ Page & Fletcher, Sean & Tracey (November 1, 2006). "Auguste D: One hundred years on: 'The person' not 'the case'". Sage journals. 5 (4): 571-583. doi:https://doi.org/10.1177/1471301206069939. {{cite journal}}: Check |doi= value (help); External link in |doi= (help)
  2. ^ Budelier & Bateman, Melissa & Randall (2021 Jan 1). "Biomarkers of Alzheimer Disease". J Appl Lab Med. 5 (1): 208. doi:10.1373/jalm.2019.030080. {{cite journal}}: Check date values in: |date= (help); More than one of |pages= and |page= specified (help)
  3. ^ Yang, Hyun Duk; et al. (2016 Dec). "History of Alzheimer's Disease". Dementia and Neurocognitive Disorders. 15 (4): 121. doi:https://doi.org/10.12779/dnd.2016.15.4.115. {{cite journal}}: Check |doi= value (help); Check date values in: |date= (help); Explicit use of et al. in: |last1= (help); External link in |doi= (help); More than one of |pages= and |page= specified (help)
  4. ^ Maurer, Konrad; Volk, Stephan; Gerbaldo, Hector (1997). "Auguste D and Alzheimer's disease". The Lancet. 349 (9064): 1546–9. doi:10.1016/S0140-6736(96)10203-8.
  5. ^ Müller, Ulrich; Winter, Pia; Graeber, Manuel B (2013). "A presenilin 1 mutation in the first case of Alzheimer's disease". The Lancet Neurology. 12 (2): 129–30. doi:10.1016/S1474-4422(12)70307-1. PMID 23246540.
  6. ^ Rupp, Carsten; Beyreuther, Konrad; Maurer, Konrad; Kins, Stefan (2014). "A presenilin 1 mutation in the first case of Alzheimer's disease: Revisited". Alzheimer's & Dementia. 10 (6): 869–72. doi:10.1016/j.jalz.2014.06.005. PMID 25130656.
  7. ^ Keohane & Grace, Kieran & Victoria (Feb 2019). "What is 'Alzheimer's Disease'? The 'Auguste D' Case Re-opened". Cult Med Psychiatry. 43: 359. doi:https://doi.org/10.1007/s11013-019-09622-z. {{cite journal}}: Check |doi= value (help); External link in |doi= (help); More than one of |pages= and |page= specified (help)
  8. ^ Dahm, Ralf (November 7, 2006). "Alzheimer's discovery". Current Biology. 16 (21). doi:10.1016/j.cub.2006.09.056.
  9. ^ Deutsche Alzheimer Gesellschaft Archived 2011-07-18 at the Wayback Machine

External links