Parkinson’s Definition:
Parkinson's
disease (also known as Parkinson disease or PD) is a degenerative
disorder of the central nervous system that often impairs the
sufferer's motor skills and speech.
Parkinson's disease belongs to a
group of conditions called movement disorders. It is characterized by
muscle rigidity, tremor, a slowing of physical movement (bradykinesia)
and, in extreme cases, a loss of physical movement (akinesia). The
primary symptoms are the results of decreased stimulation of the motor
cortex by the basal ganglia, normally caused by the insufficient
formation and action of dopamine, which is produced in the dopaminergic
neurons of the brain. Secondary symptoms may include high level
cognitive dysfunction and subtle language problems. PD is both chronic
and progressive.
PD is the most common cause of Parkinsonism, a
group of similar symptoms. PD is also called "primary parkinsonism" or
"idiopathic PD" (having no known cause). While most forms of
parkinsonism are idiopathic, there are some cases where the symptoms
may result from toxicity, drugs, genetic mutation, head trauma, or
other medical disorders.
HISTORY:
Symptoms of Parkinson's disease
have been known and treated since ancient times. However, it was not
formally recognized and its symptoms were not documented until 1817 in
An Essay on the Shaking Palsy the British physician James Parkinson.
Parkinson's disease was then known as paralysis agitans, the term
"Parkinson's disease" being coined later by Jean-Martin Charcot. The
underlying biochemical changes in the brain were identified in the
1950s due largely to the work of Swedish scientist
Arvid Carlsson,
who later went on to win a Nobel Prize,. L-dopa entered clinical
practice in 1967, and the first study reporting improvements in
patients with Parkinson's disease resulting from treatment with L-dopa
was published in 1968.
Symptoms
Parkinson disease affects
movement (motor symptoms). Typical other symptoms include disorders of
mood, behavior, thinking, and sensation (non-motor symptoms).
Individual patients' symptoms may be quite dissimilar and progression
of the disease is also distinctly individual.
Motor symptoms
The cardinal symptoms are:
•
tremor: normally 4-7 Hz tremor, maximal when the limb is at rest, and
decreased with voluntary movement. It is typically unilateral at onset.
This is the most apparent and well-known symptom, though an estimated
30% of patients have little perceptible tremor; these are classified as
akinetic-rigid.
• Rigidity: stiffness; increased muscle tone. In
combination with a resting tremor, this produces a ratchety, "cogwheel"
rigidity when the limb is passively moved.
•
bradykinesia/akinesia: respectively, slowness or absence of movement.
Rapid, repetitive movements produce a dysrhythmic and decremental loss
of amplitude. Also "dysdiadokinesia", which is the loss of ability to
perform rapid alternating movements
• postural instability: failure of postural reflexes, which leads to impaired balance and falls.
Other motor symptoms include:
• Gait and posture disturbances:
o
Shuffling: gait is characterized by short steps, with feet barely
leaving the ground, producing an audible shuffling noise. Small
obstacles tend to trip the patient
o Decreased arm swing: a form of bradykinesia
o
Turning "en bloc": rather than the usual twisting of the neck and trunk
and pivoting on the toes, PD patients keep their neck and trunk rigid,
requiring multiple small steps to accomplish a turn.
o Stooped,
forward-flexed posture. In severe forms, the head and upper shoulders
may be bent at a right angle relative to the trunk.
o Festination:
a combination of stooped posture, imbalance, and short steps. It leads
to a gait that gets progressively faster and faster, often ending in a
fall.
o Gait freezing: "freezing" is another word for akinesia,
the inability to move. Gait freezing is characterized by inability to
move the feet, especially in tight, cluttered spaces or when initiating
gait.
o Dystonia (in about 20% of cases): abnormal, sustained,
painful twisting muscle contractions, usually affecting the foot and
ankle, characterized by toe flexion and foot inversion, interfering
with gait. However, dystonia can be quite generalized, involving a
majority of skeletal muscles; such episodes are acutely painful and
completely disabling.
• Speech and swallowing disturbances
o
Hypophonia: soft speech. Speech quality tends to be soft, hoarse, and
monotonous. Some people with Parkinson's disease claim that their
tongue is "heavy".
o Festinating speech: excessively rapid, soft, poorly-intelligible speech.
o Drooling: most likely caused by a weak, infrequent swallow and stooped posture.
o
Non-motor causes of speech/language disturbance in both expressive and
receptive language: these include decreased verbal fluency and
cognitive disturbance especially related to comprehension of emotional
content of speech and of facial expression
o Dysphagia: impaired ability to swallow. Can lead to aspiration, pneumonia.
• Other motor symptoms:
o fatigue (up to 50% of cases);
o masked faces (a mask-like face also known as hypomimia), with infrequent blinking;
o difficulty rolling in bed or rising from a seated position;
o micrographia (small, cramped handwriting);
o impaired fine motor dexterity and motor coordination;
o impaired gross motor coordination;
o
Poverty of movement: overall loss of accessory movements, such as
decreased arm swing when walking, as well as spontaneous movement.
Non-motor symptoms
Mood disturbances
•
Estimated prevalence rates of depression vary widely according to the
population sampled and methodology used. Reviews of depression estimate
its occurrence in anywhere from 20-80% of cases. Estimates from
community samples tend to find lower rates than from specialist
centres. Most studies use self-report questionnaires such as the Beck
Depression Inventory, which may overinflate scores due to physical
symptoms. Studies using diagnostic interviews by trained psychiatrists
also report lower rates of depression.
• More generally, there is
an increased risk for any individual with depression to go on to
develop Parkinson's disease at a later date.[
• 70% of individuals
with Parkinson's disease diagnosed with pre-existing depression go on
to develop anxiety. 90% of Parkinson's disease patients with
pre-existing anxiety subsequently develop depression; apathy or abulia.
Cognitive disturbances
• slowed reaction time; both voluntary and involuntary motor responses are significantly slowed.
•
Executive dysfunction, characterized by difficulties in: differential
allocation of attention, impulse control, set shifting, prioritizing,
evaluating the salience of ambient data, interpreting social cues, and
subjective time awareness. This complex is present to some degree in
most Parkinson's patients; it may progress to:
• dementia: a later
development in approximately 20-40% of all patients, typically starting
with slowing of thought and progressing to difficulties with abstract
thought, memory, and behavioral regulation. Hallucinations, delusions
and paranoia may develop.
• short term memory loss; procedural memory is more impaired than declarative memory. Prompting elicits improved recall.
•
medication effects: some of the above cognitive disturbances are
improved by dopaminergic medications, while others are actually
worsened.
Sleep disturbances
• Excessive daytime somnolence
• Initial, intermediate, and terminal insomnia
•
Disturbances in REM sleep: disturbingly vivid dreams, and REM Sleep
Disorder, characterized by acting out of dream content - can occur
years prior to diagnosis
Sensation disturbances
• impaired
visual contrast sensitivity, spatial reasoning, colour discrimination,
convergence insufficiency (characterized by double vision) and
oculomotor control
• dizziness and fainting; usually attributable
orthostatic hypotension, a failure of the autonomous nervous system to
adjust blood pressure in response to changes in body position
• impaired proprioception (the awareness of bodily position in three-dimensional space)
• reduction or loss of sense of smell (microsmia or anosmia) - can occur years prior to diagnosis,
•
pain: neuropathic, muscle, joints, and tendons, attributable to
tension, dystonia, rigidity, joint stiffness, and injuries associated
with attempts at accommodation
Autonomic disturbances
• oily skin and seborrheic dermatitis
• urinary incontinence, typically in later disease progression
• nocturia (getting up in the night to pass urine) - up to 60% of cases
• constipation and gastric dysmotility that is severe enough to endanger comfort and even health
•
altered sexual function: characterized by profound impairment of sexual
arousal, behavior, orgasm, and drive is found in mid and late Parkinson
disease. Current data addresses male sexual function almost exclusively
• weight loss, which is significant over a period of ten years - 8% of body weight lost compared with 1% in a control group
Prevalence:
INDIA
A
NIMHANS study concentrated on the ethnic roots of Parkinson's disease.
In India, Anglo-Indians are found to be less prone to the disease.
While 19.5 per cent of Indians at large have Parkinson's disease, only
4 per cent of Anglo-Indians are affected.
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