Understanding psychological Disorders -II

PARKINSON’S DISEASE
Parkinson’s disease (PD), originally described by Parkinson in 1817 and previously known as
“paralysis agitans,” is a degenerative disorder of the subcortical areas in the basal ganglia
that serve nonvoluntary (extrapyramidal) movement. Parkinson's disease is named after a
British doctor, James Parkinson. In 1817, he published a detailed description of the
condition in a work titled "An Essay on the Shaking Palsy". The term "Parkinson's
disease" itself wasn't coined until 1865 by William Sanders, and later popularized by a
French neurologist.
Parkinson’s disease is characterized by motor symptoms such as resting tremors or rigid
movements. Parkinson disease (PD) is a brain condition that causes problems with
movement, mental health, sleep, pain and other health issues.PD gets worse over time.
There is no cure, but therapies and medicines can reduce symptoms. Common symptoms
include tremors, painful muscle contractions and difficulty speaking.
PREVALENCE
Its prevalence is also around 1 per 1,000 of the population, making it nearly as common as
MS. Parkinson’s disease is a movement disorder of middle and old age that affects 1 percent
of the population over the age of 55.
Only about 12% of patients with PD who are in their 50s have dementia, compared with
about 70% of those older than 80 (Mayeux et al.,1992). Younger patients with PD are likely
to function well, but dementia is more likely with increased age and disease severity.
Risk factors for PD include:
 Age:The average age of onset is about 70 years, and the incidence rises significantly
with advancing age. However, a small percent of people with PD have “early-onset”
disease that begins before the age of 50.
 Sex:PD affects more men than women.
 Heredity:People with one or more close relatives who have PD have an increased risk
of developing the disease themselves. An estimated 15 to 25 percent of people with
PD have a known relative with the disease. Some cases of the disease can be traced to
specific genetic mutations.
 Exposure to pesticides:Studies show an increased risk of PD in people who live in
rural areas with increased pesticide use.
CAUSES
The cause of PD is unknown, and the disease is therefore called idiopathic, it is known to
selectively affect the substantia nigra and the dopaminergic systems of the brain.
Like epilepsy, Parkinson’s disease seems to have no single cause; faulty DNA, brain
infections, strokes, tumors, traumatic brain injury, and neurotoxins have all been implicated
in specific cases.
  Biological
The most prominent signs and symptoms of Parkinson’s disease occur when nerve cells in the
basal ganglia, an area of the brain that controls movement, become impaired and/or die.
Parkinson’s disease is associated with widespread degeneration, but it is particularly severe in
the substantia nigra—the midbrain nucleus whose neurons project via the nigrostriatal
pathway to the striatum of the basal ganglia.

Image 1: Representing substantia nigra and neural transmission of dopamine

Dopamine is the chemical messenger responsible for transmitting signals between the
substantia nigra and the next “relay station” of the brain, the corpus striatum, to produce
smooth, purposeful movement. Loss of dopamine results in abnormal nerve firing patterns
within the brain that cause impaired movement. Studies have shown that most people with
Parkinson’s have lost 60 to 80 percent or more of the dopamine-producing cells in the
substantia nigra by the time symptoms appear.
Dopaminergic degeneration in PD is several times that of normal aging. Perhaps the reason
that noticeable parkinsonian symptoms do not appear in older adults is because there is a
“dopamine threshold,” estimated to be breached at between 50% and 80% cell loss
(Bradshaw & Mattingly, 1995), before symptoms appear.
Autopsy often reveals clumps of proteins in the surviving dopaminergic neurons of the
substantia nigra—the clumps are called lewy bodies, after the German pathologist who first
reported them in 1912. Although the pattern of concentration of Lewy bodies in the substantia
nigra indicates PD, the presence of Lewy bodies in the brain is not specific to PD. They may
also appear in normally aging people, individuals with AD, and those with other progressive
neurodegenerative conditions. This leads to speculation that Lewy bodies are either (1)
indicators of a general disease process or (2) markers of cell death.
People with PD also lose the nerve endings that produce the neurotransmitter
norepinephrine— the main chemical messenger to the part of the nervous system that
controls many automatic functions of the body, such as pulse and blood pressure. The loss of
norepinephrine might explain several of the non-motor features seen in PD.
  Genetic
Several genetic mutations are associated with PD, including the alpha-synuclein gene, and
many more genes have been tentatively linked to the disorder. The same genes and proteins
that are altered in inherited cases may also be altered in sporadic cases by environmental
toxins or other factors.Some genes associated with PD are SCNA,LRRK2,DJ-1,PINK-1 etc.
 Environmental
Exposure to certain toxins has caused parkinsonian symptoms in rare circumstances (such as
exposure to MPTP, an illicit drug, or in miners exposed to the metal manganese). Other still-
unidentified environmental factors may also cause PD in genetically susceptible individuals.
DIAGNOSIS
Initial symptoms
PD is insidious, slowly sneaking up on its victim. The patient may first sense vague aches and
pains and wonder whether arthritis is developing. A general feeling of tiredness or malaise
may come first, which could easily be attributed to overwork or “burnout.”
Other patients with PD may first report feeling irritable or depressed. As the disease
continues to progress, subtle motor symptoms begin to appear. Perhaps the person notices
weakness in an arm or leg, including problems in writing, holding a pen, or typing.
There is no X-ray or blood test that can confirm the disease. However, non-invasive
diagnostic imaging, such as positron emission tomography (PET) can support a doctor's
diagnosis.

Diagnostic Criteria

1. Diagnosis of parkinsonism
a. Bradykinesia
Plus one of
b. Tremor
c. Rigidity
2. Exclusion criteria
a. Cerebellar abnormalities
b. Supranuclear gaze palsy
c. Diagnosis of behavioral variant of frontotemporal dementia or primary
progressive aphasia within 5 years of disease onset
d. Parkinsonian features restricted to the lower limbs for more than 3 years
e. Treatment with a dopamine receptor blocker or dopamine depleting agent
consistent with drug-induced parkinsonism
f. Absence of a response to high-dose levodopa despite at least moderate disease
severity
g. Cortical sensory loss, clear limb ideomotor apraxia, or progressive aphasia
h. Normal functional imaging of the dopaminergic system
i. Diagnosis of alternative condition causing parkinsonism which could be
causing the symptoms
 3. Supportive criteria
a. Clear beneficial response to dopaminergic therapy
b. Presence of levodopa-induced dyskinesia
c. Rest tremor of a limb
d. The presence of either olfactory loss or cardiac sympathetic denervation on
MIBG scintigraphy (although the latter is rarely done in current practice)

Conventional methods for diagnosis include:

 The presence of two of the three primary symptoms

 The absence of other neurological signs upon examination
 No history of other possible causes of parkinsonism, such as the use of tranquilizer
medications, head trauma or stroke
 Responsiveness to Parkinson's medications, such as levodopa

SYMPTOMS
Motor symptoms
There are five primary motor symptoms of PD: tremor, rigidity, bradykinesia (slow
movement), postural instability (balance problems), and walking/gait problems. Observing
one or more of these symptoms is the main way that physicians diagnose PD.
 Tremor
The characteristic tremor in PD is a slow, rhythmic tremor that typically starts in one hand,
foot, or leg and can eventually affect both sides of the body. Tremor can also occur in the jaw,
chin, mouth, or tongue. The classic tremor of PD is a resting tremor, which is strongest
when the affected limb is at rest, and may become less apparent or even disappear during a
purposeful movement.
An action tremor (a tremor that occurs with intentional movement) may also be a feature of
PD. In addition, some people with PD can experience a feeling of internal tremor, which is
not necessarily noticeable to others.
 Rigidity
Rigidity refers to a tightness or stiffness of the limbs or torso. Rigidity, especially in the early
stages of PD, may be wrongly attributed to arthritis or orthopedic problems, such as a rotator
cuff injury.
 Bradykinesia
Greek for “slow movement,” bradykinesia is a frequent symptom of PD and related
movement disorders. In addition to a general slowness of movement, the bradykinesia of PD
is typically demonstrated by a reduced or mask-like expression of the face (hypomimia), a
decreased blink rate of the eyes, and problems with fine motor coordination (for example,
difficulties buttoning a shirt). Having trouble turning over in bed and slow, small handwriting
(micrographia) are other signs of bradykinesia.
 Postural Instability
More pronounced in the later stages of PD, postural instability includes the inability to
maintain a steady, upright posture or to prevent a fall. Such balance problems in PD are
associated with a tendency to list or fall backward (retropulsion). In fact, a light push can
cause the individual with PD to continue stepping backward or to even fall down. Prominent
postural instability early in the disease may indicate that the correct diagnosis is one of the
other parkinsonian syndromes rather than PD.
 Walking or Gait Difficulties
Bradykinesia and postural instability both contribute to walking, or gait, difficulties in PD,
particularly as the disease progresses. A common, early symptom of PD is a decrease in the
natural swing of one or both arms when walking. Later, steps may become slow and small,
and a shuffling gait may appear. Gait problems in PD can also include a tendency to propel
forward with rapid, short steps (festination).
Patients with PD also describe difficulty in initiating movement, or hypokinesia, and may
have to consciously think to begin walking, to turn around, or to lift a fork.
Non motor symptoms
Autonomic dysfunction:
 Urinary problems or constipation:Bladder and bowel problems can occur due to the
improper functioning of the autonomic nervous system, which is responsible for
regulating smooth muscle activity.
 Sexual dysfunction: Because of its effects on nerve signals from the brain, PD may
cause sexual dysfunction. PD-related depression or use of certain medications may
also cause decreased sex drive and other problems.
 Excessive sweating:Sweating is a relatively common sign of PD, particularly if the
disease is untreated. It happens most often in the upper body.
Neuropsychiatric symptoms:
 Depression and Anxiety: It seems generally agreed that about 20% of PD patients
suffer from a major depression with about a further 20% experiencing a more minor
depression. Anxiety occurs in PD as well and, like depression, can be mild or severe.
In some cases, anxiety may require medication. As with depression, psychotherapy
such as CBT can help to address anxiety.
 Impulse-control disorders (e.g., preoccupations, hypersexuality, compulsive
shopping, binge eating)
 Panic disorder
 Psychosis (e.g., hallucinations, delusions)
 Emotional changes:Some people with PD become fearful and insecure, while others
may become irritable or uncharacteristically pessimistic.
 Cognitive dysfunction: Cognitive problems become more severe in late stages of PD,
and some people are diagnosed with Parkinson’s disease dementia (PDD). Memory,
social judgment, language, reasoning, or other mental skills may be affected.

- Visuospatial dysfunction
 Although it is not clear whether all patients with PD experience visuospatial
dysfunction, a sizable proportion does. Certain subgroups of patients, or those in
more advanced stages of the disease, for example, may show the most difficulty.

- Language/Speech dysfunction
About 70% of patients with PD have difficulties with articulation and the neuro
mechanical aspects of speech production .In PD, the voice may become softer, or
it may start off strong and then fade away.Patients with PD lose voice amplitude
and vocal emotional expression (dysphonia), which results in monotonous voice.
Other speech irregularities may include segmented accelerated bursts of speech
(tachyphemia) and compulsive word or phrase repetition (palilalia).

- Memory functioning
Memory functioning is relatively spared in PD, even in patients with PD with
dementia (Sagar et al., 1988). Patients with PD do show a recall deficit but
demonstrate encoding and registration of declarative material through recognition
tasks.Nondeclarative learning, which relies on intact motor or executive
functioning, is often deficient. The ability to learn new motor skills declines as the
disease progresses.
Skin problems:The skin on the face may become oily, particularly on the forehead and at the
sides of the nose. The scalp may become oily too, resulting in dandruff. In other cases, the
skin can become very dry.
Sleep problems:Common sleep problems in PD include difficulty staying asleep at night,
restless sleep, nightmares and emotional dreams, and drowsiness or sudden sleep onset during
the day.
Sensory Symptoms:
- Olfactory dysfunction:A reduced sensitivity to odors (hyposmia) or a loss of
smell (anosmia) is often an early symptom of PD.
- Pain:Muscles and joints may ache because of the rigidity and abnormal postures
often associated with the disease.
TREATMENT
There is currently no drug that will permanently block the progressive development of
Parkinson’s disease or permanently reduce the severity of its symptoms.Indeed, current
evidence suggests that by the time the motor symptoms of Parkinson’s disease become
apparent, and a diagnosis is made, irreversible damage has already occurred.
Drug therapy
Medications for PD fall into three categories:
 Drugs that increase the level of dopamine in the brain. The most common drugs for
PD are dopamine precursors.Other drugs mimic dopamine or prevent or slow its
breakdown.
  Drugs that affect other neurotransmitters in the body in order to ease some of the
symptoms of the disease. For example, anticholinergic drugs interfere with production
or uptake of the neurotransmitter acetylcholine. These can be effective in reducing
tremors.
 Medications that help control the non-motor symptoms of the disease, that is, the
symptoms that don’t affect movement. For example, people with PD-related
depression may be prescribed antidepressants.
Medications for Parkinson’s include:
 Levodopa/Carbidopa:The cornerstone of therapy for PD is the drug levodopa (also
called L-dopa). Nerve cells can use levodopa to make dopamine and replenish the
brain’s reduced supply.
People cannot simply take dopamine pills because dopamine does not easily pass through the
bloodbrain barrier.Usually, people are given levodopa combined with another substance
called carbidopa. When added to levodopa, carbidopa prevents the conversion of levodopa
into dopamine except for in the brain; this stops or diminishes the side effects due to
dopamine in the bloodstream.
Levodopa/carbidopa is often very successful at reducing or eliminating the tremors and
other motor symptoms of PD during the early stages of the disease. Levodopa usually helps
most with the slowing of movement and rigidity.
 Dopamine agonists:These mimic the role of dopamine in the brain and can be given
alone or with levodopa. They are somewhat less effective than levodopa in treating
PD symptoms but work for longer periods of time. In rare cases, they can cause an
uncontrollable desire to gamble, hypersexuality, or compulsive shopping.
 MAO-B inhibitors: These drugs block or reduce the activity of the enzyme
monoamine oxidase B, or MAO-B, which breaks down dopamine in the brain. MAO-
B inhibitors cause dopamine to accumulate in surviving nerve cells and reduce the
symptoms of PD.
 COMT inhibitors: COMT stands for catechol-methyltransferase, another enzyme
that breaks down dopamine. The drugs entacapone and tolcapone prolong the effects
of levodopa by preventing the breakdown of dopamine. COMT inhibitors can
decrease the duration of “off periods” of one’s dose of levodopa.
 Anticholinergics: These drugs, which include trihexyphenidyl, benztropine, and
ethopropazine, decrease the activity of the neurotransmitter acetylcholine and can be
particularly effective for tremor associated with PD.
Surgery: Studies in the past few decades have led to great improvements in surgical
techniques, and surgery is considered for people with PD for whom drug therapy is no longer
sufficient.
The earliest types of surgery for PD involved selectively destroying specific parts of the brain
that contribute to PD symptoms. Surgical techniques have been refined and can be very
effective for the motor symptoms of PD. The most common lesion surgery is called
pallidotomy. In this procedure, a surgeon selectively destroys a portion of the brain called
the globus pallidus. Pallidotomy can improve symptoms of tremor, rigidity, and
bradykinesia.
Deep brain stimulation: A treatment in which low-intensity electrical stimulation is
continually applied to an area of the brain through an implanted electrode . DBS is primarily
used to stimulate one of three brain regions: the subthalamic nucleus, the globus pallidus
interna, or the thalamus. Stimulation of either the globus pallidus or the subthalamic nucleus
can reduce tremor, bradykinesia, and rigidity.
However, if the stimulation is turned off, the therapeutic improvements dissipate very
quickly. Unfortunately, deep brain stimulation can cause side effects such as cognitive,
speech, and gait problems.
CASE STUDY
Patient: Gail Brown, a 68-year-old female.
She is a retired farmer who lives at home alone with her dog. Mrs. Brown's husband
passed away 5 years ago. She experienced a minor fall (3 months ago) after tripping over
her dog and landed on an outstretched right hand, leading to wrist pain.
She saw her family doctor regarding her wrist, but also complained of some recent
trouble with balance and a small hand tremor. She was referred to a neurologist and
diagnosed with early-stage idiopathic Parkinson’s Disease.
History of present illness: Diagnosis of idiopathic Parkinson’s Disease 1 month ago,
left-hand tremor (5 months), right hand dominant and decreased handwriting size (5
months), decreased balance (1 year). She also has a history of depression.

Health Habits: Non-smoker, no longer drinks alcohol (3 years)

Previous functional status:

- Prior to the onset of PD symptoms (decreased balance and tremor): Able to

walk about 200m to her friend's house, gardening, performed activities of
daily living independently, driving often (grocery store, recreation center).
- Prior to husband passing (5 years ago): attended dance classes, was very
active with farm work.

Current functioning status since the onset of PD symptoms: Drives when necessary
but less confident with reaction time, less confident walking outside, no issues with
dressing/bathing, no problems with stairs, no problems with bed mobility.

Symptoms

 Slight masked face, slight muscular deconditioning, mild dysarthria, mild left
resting hand tremor which increased while discussing history of diagnosis
 Moderate kyphotic forward head posture
 Mild bradykinesia

Treatment plan
She received a referral for physiotherapy to perform a falls risk assessment, maintain her
functional status, and address her concerns regarding the condition.After the initial
assessment, it was decided that Mrs. Brown would receive 12 weeks of physiotherapy to
address her impairments and implement a home exercise program.
Based on Mrs. Brown’s current status after 12 weeks of treatment, it was decided that the
frequency of her appointments would be reduced. A 1 month follow up appointment was
scheduled for continued evaluation and treatment progression.
Although she is in an early stage of PD, research supports both early intervention and the
involvement of a multidisciplinary team will facilitate communication between
healthcare professionals and the patient .