We Shall Not Be Forgotten: Alzheimer's Disease - Rita and Auguste
Lovers of old Hollywood and vintage fashion would easily recognise the images of Rita Hayworth. One of the most popular actresses in the 1940s, Rita was known for her beauty, her fashionable looks, and her elegant dancing. She was a starlet who graced the silver screens with big names such as Fred Astaire and Gene Kelly. Fans couldn’t get enough of her and she was prolific, appearing in 61 films over 37 years. In her 50s, however, a personal tragedy struck - she lost two brothers within a week of each other and started drinking heavily. This new habit was thought to be the source of her decline - she struggled to memorise her lines and had emotional outbursts. It is now believed that alcohol sped the progression of the real underlying disease. Hayworth was diagnosed with Alzheimer’s Disease after collapsing and was taken to a doctor by her adult daughter Princess Yasmin Aga Khan. The diagnosis was confirmed through a brain scan and memory testing by a neurologist – the scan showed shrinkage to her brain and she failed her short-memory tests.
When she came out publicly with her diagnosis in 1981 she was the first public figure to do so, gaining attention to this disease that was forgotten for decades since its discovery in 1906. In the 1980s, there was no treatment available so Rita soothed herself through painting and listening to music. A confirmation of the disease was done after her death at age 67 in 1987, where they found the pathological signatures of the disease in her brain. Upon hearing about her death, the US President at the time, Ronald Reagan, issued a statement acknowledging her legacy on stage and on screen as well as praising her and her family for raising awareness of Alzheimer’s Disease. Ironically, Reagan himself was diagnosed with the same disease in 1994.
Every year, the Alzheimer’s Association in the US hosts the Rita Hayworth Gala to raise funding towards the research effort investigating this disease. This gala was organised by Rita’s daughter Princess Khan, who became a caregiver to her mother towards the end of her life. She personally witnessed Rita’s decline and wanted to raise awareness and funds towards Alzheimer’s Disease research. While Alzheimer’s Disease has been known for more than a century, we have yet to find a cure for this devastating disease. The incidence of Alzheimer's Disease and other diseases causing dementia are reported to have increased worldwide, as people are living longer and diagnoses are continuously improving.
Alzheimer’s Disease is a physical disease affecting the human brain, causing the brain to develop dementia. Dementia refers to a set of symptoms that include memory loss and difficulties with thinking or problem-solving. Alzheimer’s Disease is responsible for 60-70% of cases of dementia, and symptoms worsen as the disease develops. Alzheimer’s Disease commonly affects individuals over 65, although 4-5% of cases are early-onset. The cause is poorly understood as this disease is complicated and currently there is no treatment to stop or reverse its progression. Dementia is often mistaken as a normal part of ageing or as symptoms of stress, delaying diagnoses and care.
The main risk factors for Alzheimer’s are age and gender, with more women developing this disease than men. One theory suggests that the mitochondria (the powerhouses of the cells inside the human body) in young females are protected against the disease by oestrogen, which is the main female sex hormone. As women age and reach menopause, oestrogen level decreases and this protection disappears, rendering the brain cells vulnerable to Alzheimer’s Disease.
In fact, the diagnosis of this disease was first described in a female patient named Auguste Deter. Auguste developed dementia (memory loss, delusions, and trouble sleeping) in the late 1890s, and her rail worker husband was unable to provide care for her. Auguste was admitted to a mental institution in Frankfurt Germany in November 1901 and was examined by Dr Alois Alzheimer. She was repeatedly asked questions by the doctor to test her memory, and as the disease worsen, she failed to recall basic questions such as her family name, her husband’s name, as well as where she lived. Auguste had no sense of time or place, but was aware of her helplessness and developed anxiety. She died in 1906 at the age of 55 and her brain was sent to Dr Alzheimer to examine. Upon this examination, he found senile plaques and neurofibrillary tangles, the pathological features that were also found in Hayworth more than 80 years later. It is now commonly believed that Auguste suffered from early-onset Alzheimer’s Disease.
These pathological features are what the doctors and scientists look for to confirm and study Alzheimer’s Disease. The first features, the senile plaques, are caused by an abnormal build-up of amyloid beta peptides which is a type of short proteins present in the brain. The second features, the neurofibrillary tangles, are formed when another type of proteins called tau is overly-modified, causing it to clump. This clumping disrupts the tau proteins’ function to stabilise the microtubules, the structural part of the cells. This destabilisation causes various diseases called tauopathies, one of them being Alzheimer’s Disease. These features still baffle scientists as it is not clear whether they are caused by the disease or produced by the brain to somehow fight the disease.
There are other risk factors associated with Alzheimer’s Disease – these include cholesterol, lack of exercise, alcohol and smoking. Genetics also plays a big role, family history (especially grandparents or parents) increases the risk of developing Alzheimer’s. The diagnosis is a multi-step process – it is complicated but in the past few years has become more accurate. This has also allowed improvement in providing care and support. Diagnoses can be devastating but are often necessary for the patient and caregivers – they prepare both parties better to manage the condition. Dementia research aims to find treatments to delay the onset of diseases, slow progression, and ultimately cure the diseases.
While Alzheimer's Disease is often described as the disease of forgetfulness, we should always remember Auguste Deter and Rita Hayworth - without them, we would not have the same understanding of this disease as we do today.
References and further reading:
Alzheimer's Association - Rita Hayworth Gala
Daniel P. Perl, MD. Neuropathology of Alzheimer's Disease. Mt Sinai J Med. 2010 Jan-Feb; 77(1): 32–42.
Deutsche Alzheimer Gesellschaft
FoxNews - Yasmin Khan interview
The Globe and Mail - Yasmin Khan interview
Müller U, Winter P, Graeber MB. A presenilin 1 mutation in the first case of Alzheimer's disease. Lancet Neurol. 2013 Feb;12(2):129-30. doi: 10.1016/S1474-4422(12)70307-1. Epub 2012 Dec 14.
Pathology of Alzheimer's Disease. Science Directs