Pick’s Disease

Dementia is a group of medical conditions indicative by impairment of at least two brain function such as loss of judgment and shot term memory losses; a group of thinking and social symptoms that interferes with daily routines.

Pick’s disease is a very uncommon medical condition that causes progressive and irreversible dementia. This complex neurodegenerative disease is due to damages and shrinkage of the frontal and temporal lobes of the brain.

The frontal and temporal lobes of the brain, whose damage or shrinkage is the primary etiology for the pick’s disease, the lobes are responsible for controlling speech and emotion, and as a consequence, people affected with pick’s disease always have difficulty speaking and understanding speech, controlling of emotions especially in gatherings and social settings.

History of Pick’s Disease: Arnold Pick

The characterization of persons with various types of frontal and temporal lobe dementia-associated symptoms were first described in late 19th and early 20th century in Prague by a professor of psychiatry, by name Arnold Pick.

Arnold Pick was known by colleagues, an intelligent, modest and very principled individual, who made valued contributions to medicine especially in field of neurology and psychiatry. His contributions were numerously publicized in both German and English languages.

Arnold Pick had a patient in his early days, August H., who he studied and reported some of the observed clinical symptoms. August H. suffered from severe aphasia (loss of language skills) due to degenerations in his temporal lobes and was later thought to be the first published case of aphasia, and later reported, was Anna H., whose symptoms included behavioral abnormalities and loss of cognitive function that were consistent with frontal dementia.

A third case was Anna J, a 75-year-old woman with temporal deterioration who exhibited language impairment and loss of conceptual knowledge such as appropriate use of daily household items.

All these cases together, formed the basis for Arnold Pick’s hypothesis that deterioration of specific brain regions causes dementia and not due to generalized loss of brain function as medically suspected.

Classification of Pick Disease

Although Arnold studies often included autopsy of the brain to study size and shrinkage of specific regions, his reports however did not entail the histo-pathological data at cellular level.

Later studies by Alois Alzheimer in 1911, identified protein accumulations and swollen neurons, which was later termed Pick bodies and Pick cells that characterized the Pick’s disease.

In the 1920s, other medical researchers publicly classified Pick’s disease based on the clinical and pathologic findings of Arnold Pick and Alois Alzheimer.

Decades later, the medical term was instituted internationally to describe both the clinical manifestation of dementia associated with frontal or temporal lobe degeneration as well as pathological presence of Pick bodies.

Today, clinical personnel use the general term frontotemporal dementia (FTD) is mostly employed.

Causes of Pick’s Disease

Pick’s disease is a subdivision of a larger category of neurodegenerative condition known as Frontotemporal dementia (FTD).

This category of dementia grouping is based on their associated shrinkage of the temporal lobe and the frontal lobe region, the category consists of Primary Progressive Aphasia (PPA), Semantic dementia, Corticobasal degeneration (CBD), Argyrophillic grain disease (AGD), Globular glial tauopathy (GGT), Primary age-related tauopathy (PART) and Pick’s disease.

Pick’s disease in particular is distinctive by the presence of Pick bodies, which are abnormal aggregations of the neuronal protein tau, and swollen neuronal cells (pick cells). Pick cells and these pick bodies result in the loss of neuronal connections and cell death within the two lobes of the brain, leading to visible shrinkage of these brain regions.

Pick’s disease is rare. It can occur in people as young as 20 years old, but it usually begins between ages 40 and 65, the average age at which it begins is 54. The exact cause of the abnormal proteins to form is remains unknown till date.

Many different abnormal genes that cause frontotemporal dementia, which leads to Picks disease, have been discovered. Some of this genes and cases of Pick disease are passed down through families.

Symptoms of Pick’s Disease

Symptoms of Pick’s disease are progressive and worsen over time. Most of these symptoms include behavioral and personalities changes that make social interaction very difficult.

Persons with Pick’s disease tend to behave wrongly in social settings, these changes in behavior continues to worsen and are often one of the most disturbing symptoms of the disease.

Early personality changes, however, can assist clinicians tell Pick’s disease (FTD) from Alzheimer disease; as one of them been memory loss been the main and earliest symptom of Alzheimer disease.

General symptoms of Pick’s disease (FTD) include:

Behavioral changes

  • Compulsive behaviors
  • Impulsive or inappropriate behavior
  • Inability to interact properly in social or personal situations.
  • Repetitive behavior
  • Lack of personal hygiene
  • Social interaction withdrawal (Social Anxiety Disorder)
  • Agoraphobia

Language changes

  • Mutism (inability to make speech when needed)
  • Difficulty finding words.
  • Decreased ability to read and write.
  • Shrinking vocabulary
  • Echolalia (repetition of sound/words spoken to them)
  • Weak, uncoordinated speech sounds
  • Aphasia (difficulty speaking or understanding speech)

Neurological changes

  • Increased muscle tone (rigidity)
  • Weakness
  • Memory loss that worsens with time
  • Movement /coordination difficulties (apraxia)
  • Urinary incontinence

Emotional Changes

  • Inappropriate mood
  • Decreased interest in daily living routine.
  • Abrupt mood changes
  • Failure to recognize changes in behavior.
  • Failure to exhibit emotional warmth, empathy, concern or sympathy.
  • Decreased interest about events and environment.

Prognosis of Pick’s Disease

Pick’s disease Frontotemporal Dementia causes death within 8 to 10 years, usually from infection or sometimes due to body system failure. The disorder quickly and progressively becomes worse as the patient becomes totally disabled early in the course of the disease.

Diagnosis of Pick’s Disease

Health care providers usually ask for medical history both individually and family history. Laboratory tests may be conducted to help rule out other causes of dementia, including dementia due to metabolic causes like vitamin B-12 deficiency, syphilis and hypothyroidism.

Though there is not a particular test for Pick’s disease, FTD is diagnosed based on exhibited symptoms and result of tests, including:

  • Physical examinations and detailed neurological examination
  • Neuropsychological assessment (assessment of the mind and behavior)
  • Brain MRI (Magnetic Resistance Imaging)
  •  Head CT scan
  • Examination of the fluid around the central nervous system (cerebrospinal fluid) after a lumbar puncture
  • Tests for cognitive functions (sensation, thinking and reasoning) and motor function.
  • Position emission tomography (PET) scan of brain
  • Newer methods that test brain metabolism or protein deposits may better allow for more accurate diagnosis in the future.

Note that pick’s disease condition can only be confirmed by brain biopsy.

Treatment of Pick’s Disease

No treatment has effectively stopped or slows the progression of Pick’s disease. Doctors may prescribe treatments to help ease some of the symptoms; use of antidepressant and antipsychotic medications to help treat emotional and behavioral changes can be advised.

Test may reveal other problems that could worsen the situation and if treatable, these situations can also be attended to, for instance:

  • Anemia; which can cause fatigue, headaches and mood swings.
  • Depression
  • Nutritional disorders
  • Thyroid disorders
  • Decreased oxygen levels
  • kidney or liver failure
  • Heart conditions
  • Infections

Medications may be required to control aggressive, dangerous or agitated behaviors. In some situations, stopping or changing medicines that worsen confusion or that are not required can improve thinking and general mental functions.

Medications including;

  • Analgesics
  • Anticholinergics
  • Cimetidine
  • Lidocaine
  • CNS depressants (Central Nervous System)

Management of Pick’s Disease

Depending on the severity of the symptoms of Pick disease, monitoring and assistance with personal hygiene and self care may be needed.

Eventually, there may be a needed “all-round-the-clock” care and monitoring at home or in a special facility dedicated to this disease and other related conditions. Family counseling can help individuals cope with the changes needed for home care.

Care may include:

  • Adult protective services
  • Homemakers
  • Community resources
  • Visiting nurses or aides
  • Volunteer services
  • Legal advice early in the course of the disorder may be needed for people with Pick’s disease and their families. These legal actions can make it easier to make decisions regarding the cre of the person with the disease.

Prevention’s of Pick Disease

There is no known prevention of Pick’s Disease as most of the cases, about 90% of known cases are due to inherited gene factor.

Summary: Morality and Morbidity

Pick’s disease runs a shorter course than Alzheimer disease, on average, about 6years in some cases whose main symptoms are disturbance of speech and language (PPA).

The clinical course can be gradual. In a small series, these patients survived an average of 5 years longer than patients with behavioral symptoms variant.

A patient with PPA (Primary Progressive Aphasia) may preserve the ability to function at home for 10 or more years after onset.