SIGNS AND SYMPTOMS IN PARKINSON'S DISEASE

At the end of this tutorial you should be able to:

1. Define Parkinsons disease
2. List the classical triad iof Parkinsons disease
3. Take a history from a patient with suspected Parkinsons disease or
Parkinsonism, addressing the points which will help to confirm the
diagnosis
4. Perform physical examination focussing on the features which may occur
in Parkinsons disease or Parkinsonsim.
5. Choosing and justifying appropriate investigations of the patient.

DEFINITION: Parkinsons disease (PD) is a progressive neurodegenerative

disorder associated with a loss of dopaminergic nigrostriatal neurons. It is
named after James Parkinson, the English physician who described the shaking
palsy in 1817.

PATHOPHYSIOLOGY:
The major neuropathologic findings in Parkinson disease are a loss of
pigmented dopaminergic neurons in the substantia nigra and the presence of
Lewy bodies. The loss of dopaminergic neurons occurs most prominently in the
ventral lateral substantia nigra. Approximately 60-80% of dopaminergic neurons
are lost before the motor signs of Parkinson disease emerge.

DIFFERENTIAL DIAGNOSIS
1. Parkinsonism can be caused by a variety of degenerative disorders, as
well as toxins, infections, and vascular or structural lesions.
2. Parkinsonism also can be induced by medications that
a. block dopamine receptors (e.g., neuroleptics, anti-emetics)
b. deplete dopamine stores (e.g., reserpine, tetrabenazine)

3. Differentials to consider include:

a. Essential tremor
b. Cardioembolic stroke
c. Alzheimers Dementia
d. Multisystem Atrophy
 e. Normal Pressure Hydrocephalus
f. Parkinsons Plus syndromes
g. Wilsons Disease

HISTORY:
Parkinson disease may have a long premotor phase. Mid-life risk factors for the
later development of Parkinson disease include developing symptoms of
constipation and daytime sleepiness. These may well be the first clinical
manifestations of the disease but are non-specific. Additional features that
commonly precede onset of motor signs include decreased sense of smell.
The initial symptoms of Parkinson disease may be non-specific and
include fatigue, depression, constipation, and sleep problems.
Some patients experience a subtle decrease in dexterity and may notice a
lack of coordination with activities such as playing golf or dressing
Onset of motor signs in Parkinson disease is typically asymmetric, with
the most common initial finding being an asymmetric resting tremor in an
upper extremity. About 20% of patients first experience clumsiness in one
hand. Over time, patients notice symptoms related to progressive
bradykinesia, rigidity, and gait difficulty.
Tremor usually begins in one upper extremity and initially may be
intermittent. As with most tremors, the amplitude increases with stress
and resolves during sleep. After several months or years, the tremor may
affect the extremities on the other side, but asymmetry is usually
maintained. Parkinson disease tremor may also involve the lower
extremities, tongue, lips, or chin.
Some patients complain of aching or tightness in the calf or shoulder
region.
The first affected arm may not swing fully when walking, and the foot on
the same side may scrape the floor.
Over time, axial posture becomes progressively flexed and strides become
shorter.
Decreased swallowing may lead to excess saliva in the mouth and
ultimately drooling.
Symptoms of autonomic dysfunction are common and include
constipation, sweating abnormalities, sexual dysfunction, and seborrheic
dermatitis.
Sleep disturbances are common.
The best clinical predictors of a pathology diagnosis of Parkinson disease
are the following:

1. Asymmetry
2. Presence of resting tremor
3. Good response to dopamine replacement therapy
EXAMINATION

Parkinson's disease diagnosis is based on signs and symptoms and ruling out
other disorders that produce similar symptoms. The 3 cardinal signs of
Parkinson disease are resting tremor, rigidity, and bradykinesia. Of these
cardinal features, 2 of 3 are required to make the clinical diagnosis. Postural
instability (balance dysfunction) is the fourth cardinal sign, but it emerges late
in the disease, usually after 8 years or more.

Primary Symptoms
 1. Bradykinesia is slowness in voluntary movement. It produces difficulty
initiating movement, as well as difficulty completing movement once it is
in progress. The delayed transmission of signals from the brain to the
skeletal muscles, due to diminished dopamine, produces bradykinesia.
Bradykinesia and rigidity that affects the facial muscles can result in an
expressionless, "mask-like" appearance.
2. Tremors in the hands, fingers, forearm, or foot tend to occur when the
limb is at rest, but not when the patient is performing tasks. Tremor may
occur in the mouth and chin as well.
3. Rigidity, or stiff muscles, may produce muscle pain and facial masking.
Rigidity tends to increase during movement.
4. Poor balance is due to the impairment or loss of the reflexes that adjust
posture in order to maintain balance. Falls are common in people with
Parkinson's disease.
5. Parkinsonian gait is the distinctive unsteady walk associated with
Parkinson's disease. There is a tendency to lean unnaturally backward or
forward, and to develop a stooped, head-down, shoulders-drooped stance.
Arm swing is diminished or absent and people with Parkinson's tend to
take small shuffling steps (called festination). Patient's with Parkinson's
may have trouble starting to walk, may appear to be falling forward as
they walk, may freeze in mid-stride, and may have difficulty making a
turn.

Secondary Symptoms

The progressive loss of voluntary and involuntary muscle control produces a

number of secondary symptoms associated with Parkinson's. Most patients do
not experience all of them, and symptoms vary in intensity from person to
person.

Some secondary symptoms of Parkinson's disease include the following:

1. Constipation
2. Depression
3. Difficulty swallowing (dysphagia)saliva and food may collect in the
mouth or back of the throat may cause choking, coughing, or drooling
4. Excessive salivation (hypersalivation)
5. Excessive sweating (hyperhidrosis)
6. Loss of intellectual capacity (dementia)late in the disease
7. Psychosocial: anxiety, depression, isolation
8. Scaling, dry skin on the face and scalp (seborrhoea)
9. Slow response to questions (bradyphrenia)
10. Small, cramped handwriting (micrographia)
11. Soft, whispery voice (hypophonia)

INVESTIGATIONS:

Laboratory investigations
No laboratory biomarkers exist for Parkinson disease.
Serum caeruloplasmin concentration is obtained as a screening test for
Wilson disease. It should be obtained in patients who present with
 Parkinsonian symptoms when younger than 40 years. In cases in which
Wilson disease is suspected, 24-hour urinary copper and slit lamp
examination of the eyes also should be obtained.

Imaging Studies
Magnetic resonance imaging (MRI) and computed tomography (CT) scan
are unremarkable in Parkinson disease.
o No imaging study is required in patients with a typical
presentation. Such patients are aged 55 years or older; have a
slowly progressive, asymmetric parkinsonism with resting tremor
and bradykinesia or rigidity; and demonstrate a good response to
dopamine replacement therapy.
o MRI is useful to exclude multi-infarct state, hydrocephalus, and the
lesions of Wilson disease.