Tag Archive | alcohol-related dementia

We Had Seasons in the Sun (Debra “Deb” Lynn Ross – February 29, 2020)

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“This is the way the world ends
Not with a bang but a whimper.”
“The Hollow Men” – T. S. Eliot

My fraternal twin sister, Deb, died of complications from liver failure at 7:49 a.m. EST on February 29, 2020. I am heartbroken writing this.

T.S. Eliot is one of my favorite poets, and although I love the depth of “The Wasteland” and the profundity of “The Love Song of J. Alfred Prufrock,” “The Hollow Men” has always been my favorite. The last two lines always run through my mind when someone I know dies, as does Ecclesiastes 9:5-6 – “For the living know that they will die; but the dead know nothing, and they have no more reward, for the memory of them is forgotten. Also their love, their hatred, and their envy have now perished; nevermore will they have a share In anything done under the sun.” Continue reading

The Layperson’s Guide to Hepatic Encephalopathy

One of the conditions that occurs with advanced liver disease caused by chronic alcohol abuse is hepatic encephalopathy. Hepatic encephalopathy is different from Wernicke-Korsakoff syndrome, another condition associated with long-term alcohol abuse that is caused by a severe thiamine (B1) deficiency. However, the two conditions may coexist and lead to alcohol-related dementia. Continue reading

Profiles in Dementia: David Cassidy (1950 – 2017)

David CassidyDavid Cassidy was a singer, songwriter, and guitarist, who catapulted to fame as Keith Partridge in the 1970’s television show The Partridge Family.

Although Cassidy aspired to have the musical chops and freedom of Mick Jagger (The Rolling Stones) and John Lennon and Paul McCartney (The Beatles), his long-running stint on The Partridge Family relegated him to performing “bubblegum” music. It was something he hated, but depended on after the TV series ended to continue to make a living. Continue reading

Profiles in Dementia: Malcolm Young (1953 – 2017)

Malcolm Young suffered from alcohol-related dementiaMalcolm Young, along with his younger brother Angus, founded AC/DC, one of the first metal rock groups, in 1973.

AC/DC quickly gained traction with its driving rhythm and shouted vocals and moved into the spotlight of the music scene after just a couple of years in the recording studio.
Continue reading

Confabulation, Alcohol Abuse, and Alcohol-Related Dementia

Confabulation breaks trustOne of the lifelong struggles we, as human beings, do – or should be doing – battle with is being consistently honest both with ourselves (I submit this may be the hardest part of this battle because our capacity for self-deception seems to have no limits) and with others.

I have written here before about the interconnected relationship between honesty and trust. When we are dishonest with someone, we break their trust. Continue reading

Profiles in Dementia: Rita Hayworth (1918 – 1987)

Rita Hayworth Alzheimer's Disease Alcohol-Related DementiaRita Hayworth was an American actress whose career peaked in the 1940’s as Columbia Pictures’ most lucrative female lead, with a career that included 61 movies over a 37-year span.

One of Hayworth’s most acclaimed performances was in 1946’s Gilda, starring opposite leading man Glenn Ford. Hayworth also starred in two movies with Fred Astaire, who said she was his favorite dancing partner on screen.

However, fame and fortune could not stave off Hayworth’s personal demons, one of which was alcoholism.

By the late 1950’s, Hayworth’s chronic abuse of alcohol had ravaged the beauty of her younger years and had aged her considerably, making her no longer as attractive to Columbia as a leading lady even though she wasn’t 40 years old yet.

Alcoholism also created havoc in Hayworth’s personal life – she married and divorced five times (to men who in their own rights were not good choices).

By the 1970’s, when Hayworth was in her mid-50’s, the ravages of years of alcohol abuse began to also affect her brain. From 1972 until her death 1987, Hayworth’s cognitive impairment, memory loss, and repetitive outrageous behavior were what kept her name in the news headlines.

Rita Hayworth as an older womanHayworth died in February 1987 at the age of 68. Although she was the first public face of Alzheimer’s Disease (then a relatively-unheard-of form of dementia), there is absolutely no doubt that Hayworth also had alcohol-related dementia (also in 1987, mixed dementias and the many types of dementia were relatively unheard of as well), which probably hastened both her neurological decline and her death.

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

Normal brain cellMost 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.  

Structure of cell membraneThese 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).

Anatomy of a sphingolipidSpingolipids 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.

Chemistry of sphingolipidIn 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.

A cherry red spot on the macula is a common denominator in Tay Sachs Disease and Niemann Pick disease Type 1Along 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.