Who was the first person diagnosed with Alzheimer’s disease
Who was the first person ever diagnosed with Alzheimer’s disease? What do we know about this person? And is that person still relevant today?
Let’s find out.
Most people interested in the history of dementia will know about the doctor who first described Alzheimer’s disease for the first time – Dr Alois Alzheimer. (If you do not know about Alois Alzheimer, I recommend my article on him here.) But fewer people will actually know about the first person ever diagnosed with Alzheimer’s disease, despite this person’s role is as important as Alzheimer’s in the ‘discovery’ of the disease. For many diseases, we did not know much about the first person diagnosed with Alzheimer’s disease. The main reason was that the medical file of that person was thought to have been lost. The original hospital where Alzheimer saw this person was demolished in the 1920s and it was unclear whether all the medical files were also destroyed at the time. It took until the 1990s when the former director of the psychiatric hospital in Frankfurt (Prof Konrad Maurer) made it his mission to find this file and indeed they found it in their archives. The files not only confirmed the person’s name but also provided a lot of information on the person’s background as well as the transcribed conversations Alzheimer had with this person when conducting his clinical examinations.
So, who is this person, you might wonder?
The first person ever diagnosed with Alzheimer’s disease was a 51 year old woman names Mrs Auguste Deter.
What do we know about Mrs Deter?
Mrs Auguste Deter
Mrs Deter was born on the 16th May 1850 in Kassel, a city in Germany, where she also grew up. She married her husband August Wilhelm Karl Deter in 1873 and moved with him at some stage to Frankfurt, ~200km/120miles south of Kassel. Mrs Deter was a housewife and took care of her daughter Thekla, until the daughter married and moved to Berlin. Her husband August Wilhelm Carl Deter (called Carl by the family to avoid confusion between Auguste and August) was a low-ranking administrator in the fledgling railway system in Germany. We know this from the information provided by her husband to the doctors when Mrs Deter was admitted to the psychiatric hospital on the Affensteiner Huegel, just outside Frankfurt, on the 25th November 1901.
Mr Deter reported that they had been happily married since 1873 and that his wife was a diligent and hard-working woman who always took care of their daughter. However, he started to notice some changes in her from March 1901 onwards. First of all, Mrs Deter was claiming to have seen him with a (female) neighbour walking in the park, which he strenuously denied. He noticed that she became increasingly suspicious of people, and also accused a delivery man that he was ‘after her’. Mr Deter also noticed that his wife’s memory started to become worse and she misplaced items in the household, which led to great confusion. He further noticed that she started to struggle to perform her household chores, in particular cooking, where she became confused as to what to do at which stage. Recently, she had become increasingly agitated and even angry, which was completely out of character and they, therefore, went to see their family doctor Dr Leopold L (Unfortunately, we do not have the surname of the family doctor from the files). Dr L decided that she might be best examined at the psychiatric hospital for further investigations.
In his referral letter, Dr Lmentioned that Auguste Deter had been “suffering for some time from memory problems, delusions, sleep problems, restlessness” based on the information given by her husband. Dr L diagnosed her with ‘chronic brain paralysis’ (German: “chronische Hirnparalyse”), a medical term no longer in use. Literally translated, it means that her brain seemed to have stopped working normally for a prolonged period of time. Dr L suggested that she should be treated at the psychiatric hospital. The admitting doctor at the psychiatric hospital, Dr Nitsche, also noted that according to her husband Mrs Deter had been in general good health and that there was no history of alcohol abuse or venereal diseases.
This requires a bit of explanation, as it might seem strange to our modern ears for a psychiatrist to ask for a history of sexually transmitted diseases. However, asking for this kind of information was very much routine at that time. It was known by then that many sexually transmitted diseases, such as syphilis, can cause severe changes to a person’s mental health in their late stages. We should not forget that syphilis was a common ailment until the invention of penicillin in 1910, with estimates showing that at least 10% of the population had sexually transmitted diseases at any given time. Therefore, it is likely that a significant proportion of psychiatric patients were carrying the syphilis bacterium which caused their symptoms and eventual death. Not unsurprisingly, then, research into neurosyphilis was a considerable part of the fledgling field of psychiatry and so it made sense for Nitsche to enquire about this, as it could potentially explain the changes in Mrs Deter. However, there was no indication that she had syphilis or any other sexually transmitted disease.
Finally, Dr Nitsche asked Mrs Deter direclty, what she thought of her symptoms. She simply answered: “I have lost myself.”
First meetings with Alois Alzheimer
The day after the admission by Dr Nitsche, Alois Alzheimer decided to examine Mrs Deter in more detail. Alzheimer was at that time one of the consultant ‘nerve doctors’ at the hospital and saw most people after admission. After introducing himself, he asked her a variety of questions, some of which are still used in the examination of patients these days, such as where she thought she was, what her name was and where she lived. Due to Alzheimer’s diligent note-keeping, we can actually follow their verbatim conversation (The square brackets are my additions):
Alzheimer: “What is your name?”
A: “Family name?”
A: “What is the name of your husband?”
D: “I think Auguste.” [It is unclear whether she was really referring to her husband who, confusingly, was called August Wilhelm Carl]
A: “Your husband?”
D: “I see, my husband…”
A: “Are you married?”
D: “To Auguste.”
A: “To Mrs Deter?”
D: “Yes, to Mrs Deter.”
A: “How long have been here?”
D: “Three weeks.”
A: “What do I have in my hand?”
D: “A cigar.”
A: “Correct. …”
Aside, from the bizarre image that Alzheimer is examining a patient while smoking a cigar – common at that time but definitely unheard of in our times – we can see that Alzheimer is probing Mrs Deter’s memory. In particular, he probes for her personal events memory (so-called episodic memory) by asking her name, her marital status, duration of hospital stay, but he also asks her more everyday questions (concerning so-called semantic memory) by asking for the names of objects, such as the cigar. From these first few exchanges, it is clear that Mrs Deter is quite disorientated, as she had only been admitted the day before, but believes she has been in the hospital for three weeks. Contrast this with her knowledge of the whom she is married – although the name overlap with her husband clearly does not help – and her naming of the cigar in Alzheimer’s hand.
To conclude his first examination, Alzheimer’s asks her to write several things, including her name and address. One symptom he instantly notices is that in the middle of writing her name, she has forgotten what she was meant to write. This is a memory symptom, which piques Alzheimer’s interest. He notes it down as ‘amnestic writing disturbance’ (original German: “amnestische Schriftstörung”) in his report, while also noting that she still understood the meaning of the words and could name everyday objects without any problems.
Another common symptom in Alzheimer’s disease is how disorientated people often are regarding time and place. As we can see in the previous exchange, Mrs Deter can’t remember the year she was born and also gets her birthday confused with the current and previous year.
A: “What did I ask you?”
D: “Well, Deter Auguste…”
A: “Do you have children?”
D: “Yes, one daughter.”
A: “What is her name?”
A: “How old is she?”
D: “She is married in Berlin, Mrs Wilke.”
A: “Where does she live?”
D: “We live in Kassel.”
A: “Where does your daughter live?”
D: “Waldemarstreet – no, different…”
A: “What is the name of your husband?”
D: “I don’t know…”
A: “What name does your husband have?”
D: “My husband isn’t here at the moment.”
A: “What is your husband called?”
D: [suddenly quickly]: “August Wilhelm Karl – I do not know if I can tell you like that.”
A: “What is your husband[’s profession]?”
D: “Administrator [original German: “Kanzlist”] – I am so confused, so confused – I cannot.”
In a prior part of their conversation, Alzheimer had probed for information about the people close to Mrs Deter, asking for her husband’s and daughter’s names and details. From the conversation, it becomes clear that she can remember her daughter and her name. She also knows that the daughter is now married under a different surname and lives in Berlin. However, she gets this information confused when asked for her daughter’s address, in answer to which she actually names the street where she grew up in Kassel. She has difficulty remembering her husband’s name, but once the memory is triggered, she quickly remembers his full name and profession. This suggests that her memories are intact but she cannot access them anymore, which is a very common symptom in early Alzheimer’s disease. Therefore, people are often unable to remember information but once the memory is triggered, they can recall the required information.
Let’s rejoin them:
A: “How long have you been here?”
D: “About two days…”
A: “Where are you?”
D: “This must be Wilhelmshöhe…”
A: “Where is your flat?”
D: “Well, Frankfurt am Main…”
A: “In which street?”
D: “Waldemarstrasse… Not, it has to be another, just wait a moment – I am just so, so…”
Again, she seems disoriented as to time and place but more importantly, we can see here the typical symptoms of a person with Alzheimer’s disease ‘living in the past’ (see also my article on this symptom here). Mrs Deter seems to think that she is back in Kassel, where she was born and grew up, although she actually lived most of her life in Frankfurt. Instead of realising that she is in Frankfurt, she thinks that she is on the Wilhelmshöhe, which is a hill close to Kassel, similar to the psychiatric hospital in Frankfurt which was located on a hill (Affensteiner Hügel) overlooking the city. She must have deduced from the view she had from the institution that she was on top of a hill and because she believes that she lives in Kassel, “This must be Wilhelmshöhe…”
One curious thing worth noticing is that Alzheimer painstakingly makes notes of all the interviews with Auguste Deter and also asks a photographer to take some photos of her at the beginning of 1902 – the most famous one showing her sitting on the bed in her white gown, which was the prescribed clothing for all patients on the wards (the pictures is shown at the top of the article). Alzheimer clearly paid close attention to Mrs Deter, but it is not clear whether Alzheimer instantly knew whether Mrs Deter was a ‘special’ patient. On the one hand, it is unlikely, as Alzheimer and other clinicians were seeing dozens of patients every week, each with their own symptoms and no diagnostic criteria existed at that time for most psychiatric diseases. It must have been very difficult to identify whether one particular case was a variation of the other cases or a completely different disease.
Still, Alzheimer saw something in Mrs Deter, as he paid close attention to her, as proven by his extensive note-taking and the photographs. He was particularly taken by her ‘writing amnesia’, that she forgot mid-sentence what she was writing, while at the same time being very lucid in her knowledge of objects and facts. In most other psychiatric cases, it’s more likely that they would be confused on a general level and would have had problems answering any of Alzheimer’s questions. Nonetheless, Mrs Deter, despite being quite confused, showed this dissociation of episodic and semantic memory, which piqued his interest. Another reason for his particular attention to her case might be that he had seen a similar case three years prior, in 1898. He even published details of the case, along with other cases of psychiatric diseases, but we have very little information on the actual person and the detailed symptoms in this original case. Still, this previous encounter might have piqued Alzheimer’s interest when he met Mrs Deter. He likely made the connections between the patient he had seen in 1898 and Mrs Deter and thought that these were not isolated cases but potentially a different disease was underlying the same symptoms. There seemed to be a pattern to the specific episodic memory symptoms in both of them, which was worth investigating further. This could explain Alzheimer’s painstaking note-taking in the case of Mrs Deter. Instead of seeing Mrs Deter, therefore, as the first person with Alzheimer’s disease, we should regard Mrs Deter as rather the first well-documented case of Alzheimer’s disease. Alzheimer and other clinicians had likely seen and even reported similar cases before this case.
Alzheimer only ‘knew’ Mrs Deter for ~6 months, since he left in mid-1902 Frankfurt and moved first to Heidelberg and eventually Munich. Before he left, he went to see Mrs Deter to say a final goodbye. We can follow their, rather brief, conversation again, based on Alzheimer’s notes:
Alzheimer: “Good day, Mrs Deter.”
Deter: “Ah well, just get away. I – cannot – speak.”
It is not perhaps the fondest farewell but we should realise that Mrs Deter’s condition had by that point deteriorated significantly and she could recognise only very few people. It is, therefore, likely that she did not recognise Alzheimer and simply wanted to be left in peace. Still, it’s further evidence of Alzheimer’s empathy towards his patients that he went around the wards for a final farewell before he left. Importantly, he also asked the director of the hospital Prof Emil Sioli to keep him updated on Mrs Deter’s symptoms and the progression of the disease.
Mrs Deter’s legacy
Sioli indeed kept Alzheimer informed on Mrs Deters disease progression over the next years. On the 9th of April 1906, Alzheimer received a phone call from Sioli. This was in itself a highly unusual event since the national telephone network in Germany was still in development at that time and calling ‘long-distance’ from Frankfurt to Munich was not only difficult to arrange, but also exceedingly expensive. But Sioli had good reason to make this call urgent as he told Alzheimer that Mrs Deter had died the day before.
Alzheimer asked Sioli to conduct a post-mortem on Mrs Deter and to send him her brain to examine it further. Sioli obliged and Alzheimer received her brain shortly thereafter. Now, for our modern ears, this clearly is highly unethical behaviour, since – to our knowledge – neither Mrs Deter nor her family was asked for this to happen. But we should see it in the light of the time, when this was common practice and only after the Second World War, ethical procedures for consent became stricter in medical research.
Once, Alzheimer received everything, he straightaway starting analysing Mrs Deter’s brain with two colleagues. It took them a good 6 months to conclude their analyses and Alzheimer presented his findings of Mrs Deter’s symptoms and the changes in her brain at a scientific meeting at the end of 1906. A summary of his talk was published in 1907 and this slim, 2-page summary is still considered the foundation stone for Alzheimer’s disease. This means that Mrs Deter was indeed the first diagnosed person with Alzheimer’s disease, although many people before her had likely the same condition.
There is an irony that Mrs Deter lived her life without realising how much of a legacy she would leave, even after her death. As so often, we do not know what we will be remembered for. We should therefore celebrate the woman who ‘allowed’ Alzheimer to make his discovery and like other people, such as Henrietta Lacks (if you do not know how this is, I urge you to find out more here), there is still an under-recognition for people who made such a significant contribution (unknowingly) to medical research. That clearly needs to change. Why are we not celebrating Mrs Deter birthday (16th May) or death (8th April) to celebrate her legacy and critical contribution to Alzheimer’s disease?
Is that then the legacy of Mrs Deter?
The final twist
The eagle-eyed of my readers will have spotted that there are a few ‘odd’ things about Mrs Deter’s case. For example, her age when she presented at the psychiatric hospital – 51. Clearly, it is very rare for someone to have Alzheimer’s disease at this age and it might have been her young age which is also piqued Alzheimer’s interest in her case. The other aspect is that her progression was very fast with a clear clinical progression, even during the 6 months before she was admitted to the psychiatric hospital and Alois Alzheimer saw her. Both these factors, her young age when the disease started and the fast disease progression, raised eyebrows in the scientific community. Not during Alzheimer’s time but certainly over the recent two decades. Scientists started asking how ‘typical’ Mrs Deter’s case was really for Alzheimer’s disease or even worse, did Alois Alzheimer ‘misdiagnosed’ her case?
As with her clinical files, it was assumed that the brain samples of Mrs Deter which Alois Alzheimer analysed were lost or destroyed. However, in 2013 scientists tracked down samples of Mrs Deter’s brain tissue, which incredibly was still stored in the medical school in Munich where Alzheimer analysed her brain 100 years earlier. After extensive analysis, the scientists confirmed Alzheimer’s findings in Mrs Deter’s brain, confirming that she had indeed Alzheimer’s disease.
How can we then explain her young age at the disease onset?
For that, we have to understand that a younger age onset for Alzheimer’s disease, can be an indicator of a genetic mutation having caused Alzheimer’s disease. Many people are worried that they have inherited genes from their family to develop Alzheimer’s disease but such so-called familial Alzheimer’s disease (because it is inherited within families), is exceedingly rare. Current estimates show that only about 1% of all Alzheimer cases are familial Alzheimer’s disease. Rare indeed!
This rarity makes it even more astonishing to find out that Mrs Deter had familial Alzheimer’s disease. This was not known to Alzheimer and his contemporaries, since genetic analysis did not exist at the time. However, the scientists who investigated Mrs Deter’s brain in 2013 also ran a genetic analysis on her brain tissue and found that Mrs Deter had a PSEN 1 mutation affecting one letter of her genetic code on chromosome 14, indicating that there she had a familial Alzheimer’s disease.
It is astonishing that Alzheimer literally found the ‘needle in the haystack’ when identifying Mrs Deter since the prevalence of familial Alzheimer’s disease is so low. But retrospectively it makes sense, as Alzheimer noticed Mrs Deter particularly because she was so young (51 years of age) when she presented at the hospital in Frankfurt. At the time, Alzheimer and his contemporaries saw many people who showed sign of dementia at an older age, which they called ‘senile dementia’. However, Mrs Deter struck Alzheimer as being too young for senile dementia. He therefore often wrote about pre-senile dementia in his notes with reference to Mrs Deter; or as we would say today younger onset dementia (referring to people developing dementia before the age of 65). It is therefore not only the presentation of Mrs Deter but also her age – 51 when he first encountered her – which caught Alzheimer’s eye and made him examine and follow her more closely. Still, there is some irony in the fact that the ‘discovery’ of the disease was actually based on its rarest form and not the more common form.
Mrs Auguste Deter was a 51-year-old woman when she developed symptoms, which we now know are Alzheimer’s disease. She was referred to the psychiatric hospital in Frankfurt/Germany where she was seen by Dr Alois Alzheimer. Alzheimer followed the case closely, even when he moved away and after Mrs Deter’s death, he analysed her brain and published his findings, which have become the foundation stone for Alzheimer’s disease diagnosis and research. More than 100 years after her death, it was discovered that Mrs Deter had one of the rarest forms of Alzheimer’s disease, familial Alzheimer’s disease, caused by a mutation in one of her genes on chromosome 14. This means that ironically, that the first person diagnosed with the disease had one of its rarest forms and not the more typical Alzheimer’s disease which the vast majority of people have. There is still a significant under-recognition of Mrs Deter’s contribution to the ‘discovery’ of Alzheimer’s disease, which affects so many people’s life. We should rectify that and celebrate this remarkable woman.
- A brilliant video and interview with Prof Konrad Maurer, who ‘re-discovered’ the clinical file of Mrs Deter: https://www.bbc.co.uk/news/av/magazine-35279750