Search Results for: rare dementia

The Rare Dementias: Fatal Familial Insomnia

Fatal familial insomnia (FFI) is one of the rarest – the dominant gene that causes this type of dementia has been identified in only 28 families worldwide since its discovery in 1990 – dementias that have been identified so far.

Its first identifiable symptom occurs typically between the ages of 40 and 60 with a sudden increase in trouble falling asleep that quickly progresses to a greater inability to sleep and then just as quickly degenerates into total – and fatal – insomnia. The lifespan of a person once symptoms emerge is very short (around one year).

FFI is in a class of neurological diseases caused by prions ((Creutzfeldt-Jakob disease and its bovine equivalent, Mad Cow disease are other examples). Prions are abnormal pathogens that can be transmitted (in both humans and animals). They cause abnormal folding of prion proteins (normal cellular proteins that are found most abundantly in the brain). Prions are caused by a mutation of the PRNP gene on chromosome 20.

Prion diseases fall into three categories: sporadic, acquired, and genetic.

Sporadic prion diseases are random occurrences of these neurological diseases in people without any known risks or genetic mutations that could cause them. To date, the only prion disease that has sporadic incidences is Creutzfeldt-Jakob disease.

Acquired prion diseases occur because of exposure to the abnormal prion protein. These account for less than 1% of all prion diseases.

Genetic prion diseases are the most common form of prion diseases. FFI, along with Creutzfeldt-Jakob disease and Gerstmann-Sträussler-Scheinker Syndrome (GSS), fall into this type of prion disease.

Since the mutated gene that causes FFI is a dominant gene, if only one parent has the mutated gene, the likelihood of his or her children having the mutated gene is 50%. If both parents have the mutated gene, then all their children will have it as well.

The areas of the brain most severely affected by FFI include the thalamus, the frontal cortex, and the central nervous system.

Because the first symptoms of FFI usually occur at or near the end of childbearing years, parents aren’t even aware that they are passing the defective gene on to their children. However, a diagnostic test has been developed to test for the mutated gene.

FFI has four discrete and degenerative stages.

In the first stage of FFI, the onset of sudden and unexplained persistent insomnia leads to panic attacks and irrational phobias. This stage lasts approximately four months.

During the second stage of FFI, continued sleep deprivation increases the number and severity of panic attacks and hallucinations become pervasive. This stage lasts approximately five months.

In the third stage of FFI, total insomnia occurs, resulting in rapid weight loss and very limited mental functioning. This stage can last for up to three months.

During the fourth and final stage of FFI, dementia and unresponsiveness occur. This stage can last up to six months.

There is no treatment or cure for FFI, and the mortality rate is 100%.

The Atlantic has an very interesting article on FFI that involves a 30-year-old woman whose mother died of FFI at age 52. The daughter is now working and hoping to find a way to intercept and neutralize the genetic mutation before she reaches the age where her symptoms begin. It’s a intriguing story, which I highly recommend.

For an in-depth look at the biology of prions, an excellent reference book is The Family That Couldn’t Sleep.

The Rare Dementias: Corticobasal Degeneration (CBD)

Apr23

Corticobasal degeneration (CBD) – also known as corticobasal ganglionic degeneration (CBGD) – is a rare (occurs in less than 1% of the population) and progressive form of dementia.

The onset of symptoms typically occurs after the age of 60 and the average duration of the disease from onset of symptoms to death is six years.

Although the underlying cause of CBD is unknown, what is known is that CBD is the result of extensive and severe damage in multiple areas of the brain.

Research into this form of dementia is relatively new (it was discovered in 1968), but the most current research has found that there are similar, but not identical, changes in the brain protein tau to the changes observed in progressive supranuclear palsy and Pick’s Disease.

These areas of the brain where damage is extensive include the cortex (especially in the frontal lobe and parietal lobes) and the deep-brain basal ganglia region of the brain, with the hallmark feature in that area being significant neuron degeneration and the loss of pigment in dopaminergic neurons (signifying a decrease in dopamine production) in the substantia nigra, which controls movement.

Dopamine is a chemical produced by the brain (a neurotransmitter) that plays a leading role in movement, memory, pleasure, cognition, behavior, attention, sleep, and mood.

When dopamine production decreases in the substantia nigra, movement is severely affected.

Often this is the first visible symptom of CBD. It presents as stiff movement, shaky movement, jerky movement, slow movement, and increased lack of balance, increased lack of coordination, and clumsiness. Generally, movement problems affect one side of the body almost exclusively, but as CBD progresses, both sides of the body are affected.

Since these movement disorders can mimic both Parkinson’s Disease and the effects of a deep-brain stroke – one of the classic movement disorders associated with these is ideomotor apraxia (a common example is the inability to initiate walking where the foot seems to be stuck to the floor and can’t be lifted spontaneously to take a step forward) – those must be ruled out as the causes of the movement disorders.

Other early symptoms of CBD can include difficulty controlling the mouth muscles, cognition problems, and behavioral problems. Language and speech difficulties – dysphasia (an impaired ability to understand or use the spoken word) and dysarithia (an impaired ability to clearly articulate the spoken word) – are also early CBD symptoms.

(In my latest book, You Oughta Know: Recognizing, Acknowledging, and Responding to the Steps in the Journey Through Dementias and Alzheimer’s Disease, I devote a whole chapter to a comprehensive and in-depth discussion of the communication problems, including the different types of dysphasia, that occur with dementias and Alzheimer’s Disease, and ways to work with our loved ones to keep the lines of communication open for as long as possible.)

It is not unusual for CBD to be initially diagnosed, if the first symptoms are cognitive impairment and/or behavioral issues, as Alzheimer’s Disease or frontotemporal dementia. Similarly, if movement disorders are the first symptoms, CBD is often initially diagnosed as Parkinson’s Disease.

However, a clear diagnosis of CBD is usually made when both movement disorders and cognitive impairment and/or behavior problems appear simultaneously.

There is no known treatment for CBD. Unlike Parkinson’s Disease where dopamine-enhancing or dopamine-mimicking medications prove to be effective for some of the duration of the disease, these drugs have proven to be ineffective for treating CBD (this is likely because of the very different pathologies in the development and progression of the two diseases).

In the early stages of CBD, speech therapy and physical therapy may help with communication and stiffness and movement. However, as the disease progresses, these will become less effective and, in the end stage, they will be completely ineffective.

As CBD progresses, other symptoms appear and worsen, including:

Rigidity
Tremors
Involuntary muscle contractions
Involuntary eyelid spasms
Loss of sensory functions
“Alien hand/limb” syndrome (hand or limb movement that the person isn’t aware of nor has control over)

Because of the increased rigidity and lack of muscle coordination and use as CBD progresses, usually within five years of onset, sufferers will be unable to swallow and will be completely immobile. Even before this, though, one of the potentially-fatal risks associated with CDB is aspiration of food into the lungs because of impaired swallowing and the high likelihood of pneumonia as a result.

While a feeding tube may be considered as an alternative when CBD has progressed to the point where swallowing is significantly affected, it is, in my opinion, inhumane because it only prolongs the suffering from a disease that is ultimately fatal.

This is a quality-of-life choice. I can’t imagine for myself a life prolonged where I am completely immobile and completely dependent on everyone else for everything and I can do nothing for myself.

A feeding tube would be my worst nightmare. And for me, it would be the most cruel thing those in charge of making medical decisions for me could do to me.

Fortunately, I already have all my documents in place to make sure this can’t and won’t happen to me when and if the time comes that the choice needs to be made, because I’ve already made the choices.

So, as an aside, I would strongly urge everyone who reads this to get your wishes formalized and signed and communicated so that you have control over the end game of your life in this area.

Not only is the wise and prudent thing to do, but it eliminates the agony of wondering what to do so often seen in families where the person affected never talked about what he or she wanted and never took the time to answer these questions when he or she could.

The Rare Dementias: Progressive Supranuclear Palsy

Apr3

This post will begin a continuing series of posts that looks at rare types of dementia. Today’s post will look at the dementia of Progressive Supranuclear Palsy.

Since this blog is primarily original and unique content that provides real education and real resources and understandable information that you can actually use (you’ve no doubt noticed that, unlike many of the blogs that purport to “talk” about Alzheimer’s Disease and dementias, Going Gentle Into That Good Night does not flood your email each day with many, many blog posts that are either old news, political advocacy, or simply forwarded items from some other source, but instead does the medical and scientific research and translates that into usable and practical information for you), I will provide a clarification that I often do here.

Too many times, I hear the term “Alzheimer’s Disease” used to characterize any and all neurological damage, decline, and death.

That characterization is wrong.

Dementias refer to the full category of unique terminal neurological diseases that result in irreparable damage and destruction in the brain that lead to cognitive impairment, cognitive decline, and are always ultimately fatal.

Alzheimer’s Disease is one type of dementia. However, not everyone who suffers from dementia has Alzheimer’s Disease. And sometimes people who suffer with dementia have more than one type of dementia happening concurrently (these are called mixed dementia diagnoses).

I’ve had people who’ve never dealt directly with dementias kind of wave me off with a “whatever!” reaction when I correct them on this. And I understand that if someone hasn’t had to deal with these dementias day in and day out, they really don’t know and it doesn’t seem like a big deal.

However, for those of us who are dealing with it day and day out – or who may be in the future dealing with it day in and day out – it’s a really big deal.

Why? Precision in defining and understanding the types of dementia is absolutely critical both in caring for our loved ones, but also in effectively addressing the symptoms and behaviors associated with each dementia.

I’ll explain this in an analogy.

Let’s say you or I got diagnosed today with hypertension (high blood pressure). The normal pharmacological treatment is a medication to lower the blood pressure and possibly statins or beta channel blockers to supplement the efficacy of the main medication.

Your physician gives you the diagnosis of hypertension followed by the statement “it’s about the same thing as cancer,” and then sends you to the local hospital for radiation treatment, to be followed by chemotherapy.

Absurd, right? And yet while this may be obviously absurd to nearly everybody, a lot of those people don’t make the connection to the same absurdity of classifying all dementias as a single type of dementia.

But this underscores why it’s just as important to know about the different types of dementias and how to address and respond to the unique areas of the brain they affect as it is to know that you don’t treat hypertension with radiation and chemotherapy.

I have full-information posts on this blog about some of the more common types of dementias and symptoms/behavioral treatments:

And that’s why I want to cover some of the rarer types of dementia now. There’s no substitute for education.

We’re all, if we haven’t already been, going to be personally effected by these diseases at some point in our lives.

We can either be knowledgeable or we can spend time that we should be focusing on our loved ones playing catch-up because we didn’t take advantage of this opportunity to learn about these neurological diseases now.

Progressive supranuclear palsy (also known as Steele-Richardson-Olszewsky syndrome) is a rare neurological disease that, over time, irreversibly damages neurological cells, primarily in the brain stem, causing a severe degradation of gait, balance, complex eye movements, and thinking.

Because gait and balance are primary symptoms of progressive supranuclear palsy, this neurological disease is often misdiagnosed as Parkinson’s Disease. However, progressive supranuclear palsy has unique characteristics that differentiate it from Parkinson’s Disease.

The brain stem handles many of the critical functions of the body, including eye and mouth movement, balance, breathing, consciousness, swallowing, sensitivity to external conditions (heat, cold, pain, pleasure, etc.), and hunger.

The earliest symptoms to emerge with progressive supranuclear palsy are with gait and balance, leading to unexplained falls. Frequently the falls are described by those experiencing them as sudden attacks of dizziness.

Other early symptoms include marked personality changes, including apathy, increased irritability, increased aggravation, inexplicable angry outbursts, and increased forgetfulness.

As progressive supranuclear palsy advances, the characteristics that distinguish it from Parkinson’s Disease begin to appear in vision problems.

The first two vision problems to materialize are blurry vision and difficulty controlling eye movement. It is usually when these symptoms appear that progressive supranuclear palsy can be accurately diagnosed.

People suffering with this neurological disease will, by this point in the disease process, experience severe difficulty voluntarily shifting their eyes to a downward gaze and in controlling their eyelid movements.

This lack of control over eye movement can result in exaggerated or infrequent blinking (infrequent blinking can cause serious damage to the eyes including severe dryness and ulcers on the surface of the eye) and not being able to close the eyelids or to open the eyelids.

As these symptoms appear, other symptoms also emerge. These include increasingly slurred speech and frequent difficulty in swallowing solid food and liquids (dysphagia).

There are currently no definitive treatments for progressive supranuclear palsy.

There is some research that has shown botulinum injections (Botox) can be an effective treatment for the involuntary eye movements associated with this neurological disease. However, Botox carries its own risks and the benefits of using this as a potential therapy must be weighed against those.

The normal treatment for gait and balance problems – medication and physical therapy – have, to-date, proven ineffective. Therefore, sufferers of progressive supranuclear palsy must use weighted walkers and must have human assistance when walking. Eventually, the problems become severe enough and safety becomes a high enough concern that people suffering from progressive supranuclear palsy are confined to wheelchairs.

The symptom of dysphagia is the most dangerous and the most likely to have a fatal outcome. This is because of choking and the high likelihood of aspirating food back into the lungs. Aspiration often leads to pneumonia, and in this class of neurological diseases, pneumonia is very often fatal.

The only way to address this aspect of progressive supranuclear palsy is to change to the texture of the food. The National Dysphagia Diet Levels is the standard progressive list used to determine food consistency levels as dysphagia worsens.

However, at some point, all ability to swallow anything will be lost, so even if pneumonia is avoided or successfully treated, the time will come when people suffering from progressive supranuclear palsy will not be able to take any kind of nourishment or hydration. This, then, would be the other eventual cause of death from this neurological disease.

Profiles in Dementia: Charmian Carr (1942 – 2016)

Sep25

Charmian Carr was born in 1942 to parents who were performing artists (her mother was a vaudeville actress and her father was an orchestra leader) and she followed in their footsteps in her early life, landing the role of Liesl Von Trapp at 21 in Sound of Music in 1965.

Although Carr had a lauded role in the classic film and the promise of a good career as an actress – her only other film was with Anthony Perkins in 1966’s musical Evening Primrose – Carr made the choice to pursue a more private life.

She married, raised two daughters, and ran her own design business.

However, Carr happily embraced her role in the Sound of Music throughout her life, writing two books – Being Liesl and Letters to Liesl – about the lasting effect of her seminal character on her own life and participating in singalong performances of the Sound of Music soundtrack at the Hollywood Bowl.

Before a 2005 performance at the Hollywood Bowl, Carr
commented: “I tell people that they should consider singalong Sound of Music like going to a therapist. It’s just a kind of therapy. They can move around. They can dance and talk back to the screen. They can skip their appointment with the shrink that week.”

Carr died on September 19, 2016 at the age of 73 from an undisclosed rare form of dementia.

Profiles in Dementia: Gene Wilder (1933 -2016)

Sep4

Gene Wilder was a comedic actor best known for his performances in movies such Willy Wonka and the Chocolate Factory, Blazing Saddles, Stir Crazy, Young Frankenstein, and The Producers.

Wilder got his start in acting in 1961 in off-Broadway productions, but his acting career began to take off in 1967 when he landed a role in Bonnie and Clyde, which starred Warren Beatty and Faye Dunaway.

Wilder’s career peaked in the 1970’s, as he became a household name after masterfully capturing the essence of the title character in 1971’s Willy Wonka and the Chocolate Factory.

Wilder’s performance in Willy Wonka and the Chocolate Factory caught the attention of Mel Brooks, a filmmaker well-known for making movies that are farces or comedic parodies (perhaps because I’m too much on the serious side of things, I have never really gotten Brooks’ movies and don’t find them appealing, but they are very popular), and Wilder was featured in starring roles in the string of hit movies that Brooks made in the late 1960’s and throughout the 1970’s.

Wilder’s last movie role was in 1999, after which the actor began a more private life, with rare cameos in television shows and interviews (the last of which was in 2008 with Alec Baldwin in a Turner Classic Movie biography entitled Role Model).

According to statement from Wilder’s nephew, Wilder had been officially diagnosed with Alzheimer’s Disease in 2013, but had been showing increasingly more obvious behaviors and symptoms of the neurological disease for several years before that.

There have been many critics of Wilder’s desire to keep his diagnosis private because the critics believe that celebrities with a dementia diagnosis will bring more awareness about dementia and will spur more public and political action.

I strongly disagree with the critics and I support Wilder’s desire to keep his diagnosis private.

There is plenty of information – this blog and the books I’ve written with in-depth and practical information about dementia (what it looks like, how to travel through the journey day-to-day with our loved ones, and invaluable information on resources to best facilitate that journey), in addition to other blogs and books that focus on certain aspects of dementia or are devoted to a certain angle of dementia awareness – available to raise awareness.

I also believe that each person, and their families, should have the discretion to choose whether to make a dementia diagnosis and journey public or private.

In my mom’s case, while she was alive, I kept her diagnosis confined to a small group of people whom she and I were close to and who would want to know. I don’t really know what Mama would have chosen (it’s not a question you can really ask), but I knew I wanted to protect her and to maintain her dignity, so I made that choice with no regrets.

It was only after Mama’s death that I chose to make her dementia public, but only because I knew that our journey could help other people and that Mama would have supported that. I haven’t ever given all the details of our journey and I never will (some things just need to stay private).

Gene Wilder died at the age of 83 from complications of Alzheimer’s Disease on August 29, 2016.

2015 Research Offers New Insights Into Lewy Body Dementia – Part 1

Jan10

In 2015, a significant amount of research was dedicated to better understanding what I believe is the least understood of the major types of dementia. That type of dementia is Lewy Body dementia.

This post will will begin to summarize the findings of this latest research in way that is both accessible and will hopefully better educate us as caregivers of loved ones with Lewy Body dementia. Continue reading →

Profiles in Dementia: Norman Rockwell (1894 – 1978)

Nov20

Normal Rockwell’s artistic creations are an entrenched part of Americana. He captured everyday life with a unique emotional component that seemed and seems to touch the American soul deeply.

Rockwell is best known for the 47 years that his iconic cover illustrations graced The Saturday Evening Post, but his body of work also included other magazines, several books, and prints for both the common man and collectors.

Growing up, I remember frequently picking up the oversized, hardcover edition of Norman Rockwell: Artist and Illustrator that my parents had purchased and immersing myself in a thoughtful journey through the large body of his work. There were some that stand out in my mind still. A sampling of those include:

But the four-part Freedoms From… illustrations that Rockwell did in 1943 (during World War II to shore up and promote American involvement in the war) are probably the ones that tug, even today, at American heartstrings.

In part, I believe, the reason is they represent an ideal that we all wish existed, but which, in fact, never did and never will in our lifetimes.

They invoke a nostalgia for our imagined past as a nation, as families, as people. And I suppose that we want to be those people is a good thing, but the reality that we’re not is, for me, quite disheartening.

But I still love the pictures even in my discouragement at how far we all fall short of the ideals they portray. My hope lies in a better fulfillment in another life, while my despair comes from knowing it won’t be this one.

Rockwell struggled with severe depressive episodes, as it seems most creative people do, throughout his life, but it rarely, if at all, is seen in his art, which is, frankly, amazing and admirable.

In the last decade of his life, Rockwell developed dementia and for the last six years of his life lost the ability completely to do any artwork or illustrations at all.

Rockwell died in 1978 at the age of 84 from emphysema.

The Layperson’s Guide to Neural Disorders That Often Lead to Neurodegeneration and Dementia

Oct15

Most dementias – Lewy Body dementia, vascular dementia, early-onset dementias, alcohol-related dementia, and Alzheimer’s Disease among them – appear seemingly suddenly as primary and distinct neurodegenerative processes without definitive causes (except in the case of genetic inheritance, which primarily occurs in rare dementias like Corticobasal Degeneration, Progressive Supranuclear Palsy, and Fatal Familial Insomnia and some of the early-onset dementias).

However, there are a group of neural disorders, which are caused by the same genetic mutation that affects lipid storage in the body, that often have dementia as a secondary symptom as the diseases progress.

These neural disorders (all these have sphingolipid metabolism dysfunction in common) – which include Niemann-Pick disease, Tay Sachs disease, and Gaucher disease – are characterized by by increased levels of a particular type of sphingolipid.

There is no cure for these neural disorders and they are all fatal (in many cases, during childhood).

Spingolipids are the biological product of a chemical process that creates a protective layer on nerve cell membranes and ensures proper – and protective – cell signaling and are critical to optimal brain function.

The genesis of sphingolipids are long-chain – also known as sphingoid – bases that normally have a length of 18 carbons, although they can also have lengths of 16 or 20 carbons. The length of long-chain bases is determined by serine palmitoyltransferase (STP), a multiprotein enzyme.

In neural disorders like Niemann-Pick disease, Tay Sachs disease, and Gaucher disease, a mutation (known as Stellar) in one of the proteins that makes up STP creates an abnormally high number of 20 carbon long-chain bases, which dramatically interferes with sphingolipid metabolism.

This causes neurodegeneration to occur. In all these neural disorders, much of the neurodegeneration begins soon after birth.

In Tay Sachs disease, neurodegeneration of the brain and spinal cord begins at about six months of age. The average lifespan is four years.

Gaucher disease has three subtypes.

In Type 1 Gaucher disease, symptoms, which include anemia, bone deterioration, and liver and spleen impairment, are non-neurological and do not materialize until middle age. The average life expectancy for Type 1 is 68 years.

Type 2 and Type 3 Gaucher disease are both neuropathic forms of the disease. Neurodegenerative symptoms include abnormal eye movements, seizures, and systemic brain damage.

In Type 2 Gaucher disease, the onset of symptoms is within three to six months of age. Deterioration is rapid; the average life expectancy is about two years of age.

Type 3 Gaucher disease is a slower onset and involving version of Type 2. The average onset of neurological involvement is late childhood into adolescence. Life expectancy ranges from the mid-twenties to, in extremely rare cases, the early forties.

Niemann-Pick disease has four types: Type A, Type B, Type C1 and Type C2.

Niemann-Pick disease Type A occurs in infants. Symptoms include enlargement of the liver and spleen (around three months of age) and a failure to thrive during the first year of life. At one year, widespread damage to the lungs occurs, and there is a progressive loss of neurological and motor function.

Along with Tay Sachs disease, Niemann-Pick disease Type A also has a common eye deformity consistent with neurometabolic disease, known as a cherry spot, that occurs within the macula and is often what initially identifies the two neural disorders.

While most children born with Niemann-Pick disease Type A die in infancy, a few may live as long as four years.

Niemann-Pick disease Type B includes most of the same symptoms as Type A (motor skills are not usually affected), but the onset of symptoms is during adolescence. Most people with Niemann-Pick disease Type B survive into adulthood, but mortality rates climb dramatically between twenty and thirty years of age.

Niemann-Pick disease Type C (C1 and C2 are caused by different gene mutations, but the symptoms are the same) is characterized by severe liver disease, severe pulmonary infections, progressive neurodegeneration, and increasing difficulty with speech and swallowing that deteriorates completely over time.

The onset of Niemann-Pick disease Type C can be at any age, but it is most commonly seen by the age of five. The life expectancy with this type is under twenty years of age when symptoms appear in childhood. When symptoms appear later, the life expectancy is ten to twenty years after symptoms begin.

Profiles in Dementia: Miss Daisy of “Driving Miss Daisy”

Sep15

Driving Miss Daisy, the 1990 Academy Award-winning movie, is one of those rare movies that is both expansive and intimate as we take a journey through 25 years of the elderly life of Miss Daisy.

Miss Daisy, portrayed by one of the finest actresses I’ve ever seen, Jessica Tandy, is both a student and an educator (she’s a former schoolteacher) in this multilayered drama that takes her and us through the sweeping political and social changes in the three decades following the end of World War II.

The unlikely, and initially unwanted, relationship and friendship that develops between the 72-year-old lively and independent Miss Daisy and the calm and steady Hoke Colburn, portrayed by Morgan Freeman, who is hired by Miss Daisy’s son, Boolie, portrayed by Dan Akroyd, as Miss Daisy’s driver after she wrecks her car for a second time in a short time span is both poignant and endearing.

Although they come from completely different worlds in every sense of the word, they learn a lot from each other and they grow because of each other.

When Hoke comes in one morning after 23 years with Miss Daisy and finds her frantically running around the house because she believes she’s late for school to teach her students, he calls Boolie, who immediately puts Miss Daisy in a nursing home.

Hoke, who now depends on others to drive him around, isn’t able to visit Miss Daisy often, but he goes to see her in the nursing home when he gets the chance.

Boolie has a mostly hands-off relationship with his mother: he loves her and takes care of the practical things she needs, but she’s mainly a nuisance who often gets in the way of the rest of his life. Watching their interaction – and the lack of time Boolie has for Miss Daisy – is reminiscent of what many of us see in our own family dynamics with caregiving in general.

Hoke, on the other hand, is nurturing and compassionate and provides the time and the companionship that is missing from Boolie’s relationship with his mother.

We see the difference in these relationships with Miss Daisy, as well as the rapid decline of dementia, near the end of the movie when Boolie and Hoke go to see her in the nursing home on Thanksgiving Day.

Miss Daisy looks and acts like a shell of her former self. She is unable to communicate and doesn’t seem to recognize either Boolie or Hoke.

Boolie makes quick contact and disappears, while Hoke goes in to the dining room to sit with Miss Daisy while she mostly doesn’t eat Thanksgiving dinner.

Hoke talks with her, both about the past and about his present life. He notices that a piece of pumpkin pie sits in front of Miss Daisy uneaten.

Sensing that Miss Daisy may not remember how to eat the pie, Hoke cuts it into pieces and feeds it to her. We see just the briefest of sparkles return to Miss Daisy’s eyes as she savors each bite and the brief glimpses of recognition Miss Daisy has for her old friend Hoke even through the mostly impenetrable walls of dementia that have enveloped her mind.

I highly recommend Driving Miss Daisy. It is a tear-jerker at unexpected places throughout the movie, so grab the tissues because you’re going to need them, but it’s well worth it.

Profiles in Dementia: Zelda Fitzgerald (1900-1948)

Jun15

I recently finished reading Sometimes Madness is Wisdom: Zelda and Scott Fitzgerald – A Marriage. I highly recommend it, although it’s a harrowing book in so many ways.

Two of those harrowing aspects are Scott’s alcoholism and how he deliberately and consciously broke Zelda, who had her own demons and a genetic predisposition toward mental illness, for good.

A third harrowing aspect of this book is the barbaric state of psychiatry – diagnosis and treatment – in the 1930’s and 1940’s.

Zelda, who was diagnosed in 1930 with schizophrenia (most likely an inaccurate diagnosis, since it’s highly unusual for schizophrenic’s first symptoms to not appear during adolescence), was repeatedly subjected to three types of treatment routinely used for schizophrenia.

Not only did they, in many ways, exacerbate Zelda’s mental illness as well as qualify for legalized torture, but two of them eventually caused irreversible neurological damage, including cognitive impairment and memory loss consistent with all forms of dementia.

The first of these that Zelda endured over the course of 18 years was insulin coma therapy. Begun accidentally by Viennese physician Manfred Sakel, it involved giving large doses of insulin hourly daily for several weeks to keep the patients in a comatose state. It was believed that this gave the brain a chance to rest and heal itself.

Of course, other than the obvious risks of not being able to bring patients out of the comas (happened regularly) or death (also happened regularly), the high doses of insulin produced a prolonged state of hypoglycemia, resulting in permanent neurological damage.

The second treatment that Zelda was routinely given was chemically induced seizures. This treatment, based on very iffy logic at best, was pioneered by Hungarian pathologist Ladislas Joseph von Meduna.

von Meduna made the unscientific leap with his observation that because people diagnosed with epilepsy rarely were diagnosed with schizophrenia. Therefore, he concluded that the epileptic seizures – and their aftereffects, which to von Meduna’s mind seemed to indicate blissful happiness – must be the reason for rare schizophrenic diagnoses. von Meduna then concluded that the seizures could cure schizophrenia.

By the time Zelda was subjected to chemically induced seizures, the original substances used to induce seizures – strychnine, absinthe, caffeine, and camphor – had been abandoned in favor of the drug Metrazol.

Given to schizophrenics in a regimen of 30-40 injections, with the injection rate of two to three times a week, the first injection produced such a powerful seizure within a minute of injection that torn muscles and fractured bones were not uncommon.

Beyond the high physical risks of violent injury, Metrazol also caused permanent memory loss.

Zelda died in a tragic fire at Highland Hospital, a psychiatric hospital, in Asheville, North Carolina in March of 1948, during the latest of on-and-off commitments to the facility she had from 1936 onward.

However, in many ways, Zelda was gone years before the fire took her physical life. Zelda spent the last several years of her life devoid of memory, devoid of personality, and devoid of any sort of intellectual spark.

Zelda’s physical appearance was so drastically changed that friends and acquaintances had a hard time recognizing her.

Even in her hometown of Montgomery, Alabama, where she lived with her mother when she wasn’t at Highland Hospital, Zelda was unrecognizable to people who’d know her and her family all their lives.

Zelda spent the majority of the daylight hours in Montgomery restlessly and aimlessly wandering around town in old, ragged, dirty clothes with no evidence of any care for personal grooming until her mother would find her and get her home before darkness fell.

It was a sad end of days for Zelda Fitzgerald.

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