Princess Sissi’s abnormal mental manifestations of depression, anorexia, and ADHD and her family history
Empress Elisabeth of Austria suffered from atypical depression, which was not the only illness she suffered from. She was also believed to have anorexia. This eating disorder has a psychological basis and is characterized by conscious fasting. The effects of the disease are obvious, as the patient’s weight is far below the optimal level. The sharp weight loss leads to problems with daily body functions.
Sissi was 172 cm tall and weighed only 50 kg. Her obsession with slimness was reflected in her daily standing on the scale, her great attention to the quality of the food she prepared, and her slim figure was also the result of her love of sports. She loved walking, horseback riding and gymnastics, activities that made her feel happy at least for a little while.
Princess Sissi’s abnormal mental performance can be diagnosed as anorexia nervosa, depression with attention deficit hyperactivity disorder (ADHD) in modern medicine. All these abnormal mental performances made her seem out of place in the strict court etiquette after marriage. She yearned for her free life in the countryside before marriage, riding freely in the fields, which made her frequently leave her husband and court life. These mental performances of Princess Sissi are very similar to her father’s “weird” pedigree performance. At the same time, her son also suffers from depression. This series of abnormal mental performances reflects the sensitivity of her family’s inheritance. Sissi was born in the ducal royal branch of the Bavarian Telsbach family. This family is famous for its members’ inbreeding and bad behavior. In addition, Princess Sissi also intermarried with her cousin, so the abnormal mental performance of Sissi, Sissi’s father, and Sissi’s son is likely to be caused by some kind of inherited metabolic disorder (IEM).
一、Family History of Empress Elisabeth of Austria — Princess Sissi
Empress Elisabeth of Austria (Princess Sissi) was probably first cousins (due to inbreeding, they also had an aunt/uncle relationship), and she later married her aunt’s son, Franz Joseph of the Habsburg dynasty.
Born into the ducal branch of the House of Telsbach in Bavaria, a family known for inbreeding and bad behavior among its members, Elisabeth enjoyed an informal upbringing before marrying her cousin Emperor Franz Joseph I at the age of 16. The marriage thrust her into the more formal Habsburg court life, for which she was unprepared and found suffocating. Soon after the marriage, she had a dispute with her mother-in-law, Archduchess Sophie, who was also her aunt, and Sophie took over the raising of Elisabeth’s daughters, one of whom died in infancy. The birth of her son, Crown Prince Rudolf, raised Elisabeth’s status at court, but her health suffered as a result. As a result, she frequently visited Hungary because the environment there was more relaxed. She developed a deep affinity with Hungary and helped establish the dual monarchy of Austria-Hungary in 1867.
In 1889, the suicide of Sisi’s only son and mistress, Marie Vitsela, in a hunting lodge in Mayerling was a huge blow to Sisi, and she never recovered. She withdrew from court affairs and traveled extensively without her family. In 1890, she built the Achilles Palace on the Greek island of Corfu. The palace features elaborate mythological motifs and was a frequent refuge for Elizabeth. She was extremely concerned about maintaining her youthful figure and beauty, and instituted a strict diet plan and wore very tight corsets to make her waist look slender.
In 1898, Elizabeth was stabbed through the heart by Italian anarchist Luigi Lucheni while traveling in Geneva, causing her death. She reigned for 44 years, the longest of any Austrian empress.
1. “Strange” family history
Elizabeth, nicknamed Sissi, is the third child of Bavarian Duke Maximilian Joseph and Bavarian Princess Ludovica.
Her father was a bit of an eccentric and had a history of alcoholism, and he was fascinated by the circus and Bavarian folk music. At the same time, he did not much enjoy his ducal duties and made it his personal mission to escape them whenever possible.
Her mother was the half-sister of King Ludwig I of Bavaria, who was known for his unconventional passions, his penchant for building grand buildings in the style of ancient Greece and Renaissance Italy, and his terrible poetry. She was also the cousin of King Ludwig II of Bavaria, who was sometimes called the “Swan King” and was considered a lunatic by his contemporaries.
It is no exaggeration to say, therefore, that both sides of Sisi’s family had a “genetic history” of eccentric personalities. As a result, she and her siblings enjoyed great freedom during their childhood, away from the restraints of the court. Sisi often skipped classes to go for walks in the countryside or horseback riding. It was this freedom, lack of restraint, and a lot of outdoor activities that left a deep impression on her character.
Photo: Engagement portrait of Duke Maximilian Joseph and Princess Ludovica/Wikimedia Commons
2. Descendants of Princess Sissi
Sissi, 16, married the 23-year-old Austrian Emperor Franz Joseph. Sissi’s husband was the son of her aunt, which was considered a close relative marriage. They had four children after marriage.
2.1 Grand Duchess Sophie:
Their eldest daughter, Grand Duchess Sophie, died of fever and illness at the age of two.
2.2 Archduchess Gisela:
The second daughter, Archduchess Gisela, married Prince Leopold of Bavaria. This was a profound return of Princess Sissi to her Bavarian ancestry and a highly respected marriage among the royal family and aristocracy at the time.
The couple had four children:
The eldest daughter, Princess Elizabeth Marie (1874–1957), married Count Otto of Seefried and Buttenheim. They had 5 children, all of whom were honored as counts and countesses, and adopted the title of von Seefried used by many nobles after the abolition of the German monarchy and the establishment of the republic.
The second daughter, Princess Augusta Maria (1875–1964), married Archduke Joseph August of Austria in 1893. They had six children, but two died in infancy.
Prince Georg of Bavaria (1880–1943), married Archduchess Isabella of Austria
Prince Conrad of Bavaria (1883–1969), married Princess Bona Margaret of Savoy-Genoa
2.3 Crown Prince Rudolf:
Crown Prince Rudolf suffered from depression (probably due to family hereditary reasons), and the Crown Prince committed suicide at the age of 30. However, the family lineage was passed on through the Crown Prince’s only daughter, Elizabeth Marie (also known as Elche), and the many descendants of the Crown Prince’s sisters, Archduchesses Gisela and Valerie.
2.4 Archduchess Valerie, the fourth daughter:
The youngest child of Princess Sissi was Archduchess Valerie (April 22, 1868 — September 6, 1924), who is widely regarded as the Queen’s favorite child, probably because the other older children were raised in the formal Habsburg way by Archduchess Sophie, Franz Joseph’s mother. Young Valerie had a closer relationship with her mother and accompanied her on her travels around Europe.
On July 31, 1890, Valerie married her third cousin Archduke Franz Salvator in Bad Ischl.
This youngest child was perhaps also the one who made the greatest contribution to consolidating her royal parents and the Habsburg bloodline, and she later gave birth to ten more children.
Grand Duchess Valeria’s descendants, and grandchildren of Sissi, include Grand Duchess Elisabeth Francesca, Grand Duchess Franz Karl, Grand Duchess Hubert Salvatore, Grand Duchess Hedwig, Grand Duchess Theodore Salvatore, Grand Duchess Gertrude, Grand Duchess Maria Elisabeth, Grand Duchess Clemens Salvatore, Grand Duchess Mathilde, and Grand Duchess Agnes.
These children grew up to form their own extended families, forming a large number of noble and royal descendants who are still alive today.
Although the legend of Princess Sissi is a bittersweet fairytale marriage with tragedy, her undoubted legend lives on today, both in history books and among the many descendants of this great and beautiful queen, who have carried her bloodline into the modern world.
二、Princess Sissi’s abnormal mental performance
Princess Sissi controlled her diet to a great extent, exercised a lot and behaved abnormally. These abnormal mental performances can be diagnosed as anorexia nervosa in modern medicine. Princess Sissi also suffered from depression and ADHD. All these abnormal mental performances made her seem out of tune with the strict court etiquette after marriage. She yearned for her free life in the countryside before marriage, riding freely in the fields, which made her frequently leave her husband and court life. These mental performances of Princess Sissi were very similar to her father’s “weird” pedigree performance. At the same time, her son also suffered from depression. This series of abnormal mental performances reflected her family’s inherited sensitivity.
Due to her nervous attacks, fasting therapy, strict exercise regime and frequent coughing, her health became so worrying that in October 1860, it was reported that she not only suffered from “green disease” (anemia), severe swelling of the feet, but also physical fatigue and migraines. Pulmonologist Dr. Skoda was worried that she had severe tuberculosis and suggested staying in Madeira.
1. The troubles of anorexia
She can be said to be the ancestor of the anorexic girl on the Internet. She once collected her own anorexic photo collection. She always carried a scale with her, and like many anorexics, she carefully and compulsively recorded her weight and measurements every day. A man named Count Wilczek once recalled that when he walked into the room of Sissi and French Queen Eugenie, “they stood behind the door with their backs to the door… and were measuring the most beautiful calves in Europe at that time with two measuring tapes.”
The details of her diet and exercise plan were recorded by those who knew her, and later her biographers also recorded them meticulously because these details circulated on the anorexic Internet. She often refused to have dinner together, and even if she did, she ate “astonishingly little,” said Count von Rechberg, one of her entourage. He complained: “We also suffer from this because the entire dinner, including four courses, four desserts and coffee, lasts no more than twenty-five minutes.” Her diet sometimes included milk, orange juice, beef broth or a mixture of egg white and salt, and sometimes she would eat violet-flavored ice. For a while, she alternated between “milk days” and “orange days”, during which she ate nothing.
In the last stage of Sissi’s life, a doctor performed an autopsy on her and found that she suffered from edema caused by hunger, a disease more common among soldiers in wartime, and it can be seen that she had been hungry for a long time.
2. Obsession with beauty
Elizabeth was obsessed with beauty and she was very careful about her love of beauty. She was famous for her waist (19 inches) and hair (almost floor-length), and her weight fluctuated between under 100 pounds and over 110 pounds before her death, which was always too light for her 172 cm tall woman. It took an hour to tighten the corset, and it took three hours to wash her hair with raw eggs and brandy once a month. During this time she also had a language teacher with her, and every time she washed her hair she would count the hair that fell off her head.
Image: Portrait of Sisi by Franz Xaver Winterhalter/Wikimedia Commons
Her niece, Marie Larisch-Wallasey, wrote in her memoirs that her aunt’s “life mission was to preserve her youth, and she was always thinking about the best way to preserve her beauty.” She would sometimes sleep with a mask lined with raw beef and wrapped herself in wet towels to keep her waist slim. She would smear strawberries on her face when they were ripe. “She would take warm baths with olive oil, which she thought helped to keep her figure supple, but on one occasion the olive oil nearly boiled and she, like many Christian martyrs, nearly died,” Marie Wallasey wrote. She starved herself to maintain her famous waist. Her mother worried that starvation “had become an obsession,” and her husband often wrote to her expressing his concerns about “this terrible diet” and that she was becoming “too thin.”
She forbad herself to have her photograph or portrait taken after the age of 32. She wanted the public to remember her at her best
3. Fanaticism for various sports
Another consequence of Sissi’s obsession with appearance was her fanatical participation in various sports activities.
In addition to her intense exercise in her custom-built gym, she was also an equestrian enthusiast. In fact, she was a superb rider and was considered the best female equestrian of her time.
When she had to give up riding due to health problems, she began walking, and not just any walking. She insisted on walking briskly for several hours every day. Her walks were more like forced marches, as she never let bad weather stop her from going out. She chose her companions based on one key criterion: whether they could keep up with her pace or whether they would collapse from exhaustion after a few miles.
In her fifties, she began to train in fencing with the same passion and discipline she showed in all other sports, and became quite good at it. She would train for hours on end because she needed to release her abundant energy (hyperactivity), a trait she had throughout her life.
Image: Picture of Queen Elizabeth riding a horse/Wikimedia
4. Sissi’s life and personality
Princess Sissi’s personality was shy and introverted. She grew up in the countryside and loved playing outdoors, even playing in the mud with the farm children. She was described as restless and unable to sit still in her married life. She had little interest in learning, except for a few things she liked, such as writing poetry or painting. When she arrived at the palace in Vienna after her marriage, she was very unhappy. She hated all the strict royal etiquette and hated sitting for hours at banquets. She also wrote — “I woke up in prison with my hands chained”. Sissi took every opportunity to leave the palace and go hiking or horseback riding until she was exhausted. She began to smoke and drink. She also developed an obsession with her physique, not starving herself, exercising constantly, and keeping knee-length hair. Later, she even let her husband have a mistress so that she could travel freely and refused to participate in royal activities.
5. Sissi Syndrome
Sissi syndrome is an unusual form of depression that combines irritability, nervousness, high energy, physical hyperactivity, mood swings, fasting, sleep disorders, exaggerated body worship, lack of achievement and satisfaction, self-esteem problems, and countless attempts at self-treatment in the form of a depressive syndrome.
Researchers are divided on whether Sissi syndrome should be classified as a separate mental illness: In 1998, a pharmaceutical company allegedly launched a new depressive syndrome called “Sissi Syndrome” after the former Empress of Austria. The company’s advertising campaign provided information about the classification of the disease, symptoms, and recommended treatments. When medical researchers reviewed the relevant literature on this syndrome, they could not confirm the statements about it. Therefore, there is a lack of scientific evidence that it is an independent depressive syndrome. It is said that about one-third of female depression patients have Sissi syndrome, which makes it difficult to diagnose depression correctly. In Germany, about 3 million people are said to suffer from Sissi syndrome.
The depression that Princess Sissi suffered from did not lead to the typical symptoms of mental disorders. She lived a wealthy and seemingly happy life, and although she was dissatisfied, she did not show it. Sissi syndrome is a special type of depression that does not manifest itself as inactivity, the need for isolation or even ending one’s life, but quite the opposite. People with this disease are energetic and willing to act. The problem is that doing all kinds of activities does not bring satisfaction to yourself and your work.
6. The association between these abnormal mental manifestations of Princess Sissi and anorexia nervosa:
For the Austrian Queen Sissi, a luxurious and affluent life with servants and spacious rooms for her use, but neither material wealth nor the love of her beloved made her feel fully happy. The strict rules and etiquette that prevailed in the court often made Sissi nervous. She did not feel at home there, and almost no one understood her, so she felt lonely. She sought comfort through various entertainments. The death of her eldest daughter in childhood, the mother-in-law’s snatching of her custody and the suicide of her only son aggravated her depression symptoms.
Psychological symptoms: mainly manifested in fear and anxiety about weight gain, happiness about weight loss, obviously already very thin, but still thinks she is fat, and has no satisfaction with her life.
Physical symptoms: manifested as extreme weight loss, malnutrition and insomnia, depression with irritability, impulsivity and hyperactivity, and Sissi has bipolar-like emotional manifestations with “outbursts” of anger, extreme excitement or extreme sadness.
Abnormal behavior: restricting or refusing to eat, vomiting, catharsis, etc., and reduced interpersonal communication is often a secondary behavioral feature.
Gastrointestinal symptoms of inherited metabolic disorders IEM: the appearance of gastrointestinal symptoms such as diarrhea, vomiting, abdominal pain or exercise intolerance. Gastrointestinal symptoms such as abdominal pain, nausea and vomiting may occur at the same time as psychiatric symptoms. This is especially true for porphyria, which has been widely described in the literature. In addition, our results show that vomiting has also been described in patients with late-onset OTCD. This is consistent with previous studies that have shown that nausea and vomiting are often observed in patients with urea cycle disorders. Therefore, the combination of atypical psychiatric symptoms and gastrointestinal symptoms may be the key to identifying and distinguishing psychiatric disorders caused by potential IEM from primary psychiatric disorders. In patients presenting with gastrointestinal and abdominal symptoms, failure to establish a gastrointestinal diagnosis after ultrasound, CT, endoscopy, and blood analysis may help to raise suspicion of IEM. Frequently, these patients are incorrectly diagnosed with functional disorder, personality disorder, bipolar disorder, or Munchausen syndrome.
III. Inherited metabolic disorder IEMS
1. Inherited metabolic disorders IEMS
IEM diseases can occur at any age, from the fetal period to the elderly, and are often divided into early-onset and late-onset diseases at the age of 1. Late-onset patients generally have higher enzyme activity than early-onset patients, so the degree of late-onset disease will be milder than that of early-onset patients. However, at present, the prognosis of some late-onset patients is not as good as that of early-onset patients, because some early-onset patients can be screened out at birth heel blood, or early acute attacks can be diagnosed under the diagnosis of professional pediatric metabolic doctors in first-tier cities. Early diagnosis and disease management or surgical intervention (liver transplantation) can mostly lead to normal development. However, late-onset patients have good metabolic capacity at birth, and many heel blood screenings cannot detect abnormal metabolites. They often have acute attacks in late childhood or adulthood. There are many twists and turns in clinical diagnosis, which makes it impossible to diagnose and cannot be diagnosed in the first time. As a result, some late-onset patients may be disabled or even die in the acute phase.
The clinical manifestations of the nervous system in late-onset IEM disease are highly heterogeneous, mainly manifested as epilepsy, cognitive impairment, mental and behavioral abnormalities, acute/chronic myelopathy, movement disorders, peripheral neuropathy and myopathy. Moreover, most of these patients have normal growth and development before the first onset of the disease, so they are easily misdiagnosed as acquired diseases, such as encephalitis, myelitis or other degenerative diseases.
If some IEM diseases are not diagnosed in time and are treated incorrectly, the patient’s disease process will be aggravated, leading to disability and death. For example, patients with urea cycle disorders (UCD) are treated as encephalitis with symptoms such as coma, delirium, epilepsy, and vomiting in the acute stage, and corticosteroids and large-dose intravenous immunoglobulin are used as incorrect treatment methods, resulting in brain edema and brain damage in patients.
For more information about IEMs, a genetic metabolic disorder, please refer to previous articles: Diagnostic methods for IEMs, a genetic metabolic disorder: genetic metabolomics testing
2. Benefits of IEMS diagnosis
Many family members of patients said, “These genetic metabolic disorders are rare diseases. Even if they are diagnosed, there is no medicine to treat them. What’s the point of spending so much money to diagnose them?” We believe that diagnosis has the following benefits
It can evaluate the disease status of family patients: for example, if the elder brother is diagnosed with a certain disease, then according to the genetic pattern, it can be analyzed whether the younger brother and grandparents are patients (mild symptoms) or carriers. Then, according to the diagnosis, disease management and fertility risk assessment of family members can be carried out.
For example, if the eldest daughter of this family in the previous article can be diagnosed earlier and receive standardized treatment for the disease, she may not die. At the same time, if the younger brother Jiajia is diagnosed earlier, it is very likely that the consequences of this acute crisis can be avoided. At the same time, the disease status of the second daughter and the father can be diagnosed, and the risk of the second daughter’s marriage and childbirth can be avoided in the future.
Previous article: A family of three with mental disorders as the first symptom, one of them died and the other was disabled.
Help other patients get diagnosed: Sometimes in the clinic, patients are diagnosed with some new mutations or new disease genes. These points will be added to the gene bank under the evaluation of doctors and researchers, so that more other patients can be diagnosed. Once diagnosed, there will be a circle of patients, so patients will not feel so lonely in the journey of the disease.
Get treatment: With the development of medical care, some genetic metabolic disorders have some standardized treatment methods, such as supplementing coenzyme factors, optimizing metabolism through circulating substrates, and reducing non-metabolizable substances through dietary management. Early diagnosis of such patients and timely standardized treatment can prevent brain nerve damage, and the patient’s intelligence and physical development can develop normally. After diagnosis, good management of the current disease can sometimes prevent the disease from worsening, giving patients a time difference and waiting for gene therapy to cure them in the future.
In the family introduced in the previous article, the eldest sister, the fourth brother and the father had mental disorders. They had been receiving palliative treatment for mental illness (treatment for mental disorders). Therefore, this type of mental disorders will recur. Because there is no diagnosis, there is no correct treatment method to manage the disease. During the repeated acute period, the grassroots hospital treated them as mental illness, so that the siblings later had an acute metabolic period. Some patients with this type of IEM disease who were not diagnosed in time in the clinic died in the acute period without a confirmed cause, such as Jia Jia’s eldest sister in the article.
The disease can be paid attention to by more medical research and development groups: If the probability of diagnosis of patients is small, then the number of people diagnosed with this disease will not be large, and many opportunities will be lost, such as: pharmaceutical companies develop drug treatments, medical teams pay attention to the disease and develop nursing opportunities for the disease.
3. IEMS disease and future radical treatment
The treatment goal of IEMs is to correct metabolic defects. The main treatment principles include dietary therapy to limit the intake of defective enzyme substrates, coenzyme factor therapy, nitrogen excretion agents to promote the excretion of toxic metabolites, cocktail therapy and liver transplantation therapy. A large part of IEMs are preventable and controllable. If detected early and properly intervened, patients can even live healthily without the disease.
Current treatments for inherited metabolic disorders (IEM):
Nutritional management (diet therapy) for inherited metabolic disorders
Coenzymes, nitrogen-removing agents, and mitochondrial cocktail therapy for inherited metabolic disorders
Liver diseases caused by inherited metabolic disorders: the evolution of liver transplantation and the future prospects of hepatocyte transplantation
Future treatments for inherited metabolic disorders (IEM):
Except for some types of IEM diseases that can be cured by liver transplantation, the current treatments for IEM are not therapies that can cure such diseases, and some patients suffer from sequelae of brain damage due to failure to receive timely treatment. Because brain damage is irreversible, current treatments cannot cure brain damage. Future treatments will mainly explore curative therapies: gene therapy, enzyme replacement therapy, stem cell therapy, etc. Among them, gene therapy and enzyme replacement therapy can cure such metabolic disorders in patients, and stem cell therapy and brain-machine interface stimulation can repair human nerves (brain nerves and muscle nerves, etc.). The combined treatment of these future therapies can change the current medical predicament of patients. Most of these therapies are currently in the research and development stage. I believe that they will be clinically applied to patient treatment in the near future. At this stage, patients need to manage their diseases and nutrition and wait for the day of medical progress.
Future curative treatment for inherited metabolic disorders
References for this article:
Burgmer M, Driesch G, Heuft G. Das “Sisi-Syndrom” — eine neue Depression? [The “Sisi syndrome”: a new form of depression?]. Nervenarzt. 2003 May;74(5):440–4. German. doi: 10.1007/s00115–003–1489–2. PMID: 12966819.
Vandereycken W, Abatzi T. Das anorektische Leben der Kaiserin Elisabeth von Osterreich (1837–1898). Schlankheitskult im Hause Habsburg [The anorectic life of Empress Elisabeth of Austria (1837–1898). Slenderness cult of the Habsburg family]. Nervenarzt. 1996 Jul;67(7):608–13. German. PMID: 8927201.
家谱：https://gw.geneanet.org/castel974?n=de+wittelsbach&oc=0&p=elisabeth+sissi&type=tree
https://en.geneastar.org/genealogy/vonwittelsbache/elisabeth-von-wittelsbach
https://www.royaltitles.net/blog/who-are-empress-sissis-descendants-today/
厌食症：https://lareviewofbooks.org/article/empress-elisabeth-and-the-archives-of-anorexia/
https://medium.com/new-writers-welcome/empress-sisi-of-austria-the-eccentric-royal-with-a-tragic-fate-b11363193011
https://www.reddit.com/r/adhdwomen/comments/11m1z4t/the_sisi_syndrome_women_in_history_with_adhd/
茜茜综合征：https://flexikon.doccheck.com/de/Sissi-Syndrom
https://www.medonet.pl/psyche/zaburzenia-psychiczne,syndrom-sisi---szczegolna-odmiana-depresji--historia-cesarzowej-austrii,artykul,67542971.html
https://www.geni.com/people/Empress-Elisabeth-of-Austria-Elisabeth-von-%C3%96sterreich-Ungarn/6000000002459854330
Chinese version of this article:茜茜公主的抑郁症、厌食症、多动症的异常精神表现与家族遗传史