Examination of the Patient With a Movement Disorder
Authors: Lewis,
Steven L.
Steven L.
Title: Field
Guide to the Neurologic Examination, 1st Edition
Guide to the Neurologic Examination, 1st Edition
Copyright ©2004 Lippincott Williams &
Wilkins
Wilkins
> Table of Contents > Section 3 –
Neurologic Examination in Common Clinical Scenarios >Chapter 46 –
Examination of the Patient With a Movement Disorder
Neurologic Examination in Common Clinical Scenarios >Chapter 46 –
Examination of the Patient With a Movement Disorder
Chapter 46
Examination of the Patient With a Movement Disorder
GOAL
The main goal of the examination of the patient with a movement
disorder is to characterize the disorder of increased or decreased movements to
better understand the diagnosis and management of the patient’s
symptoms.
disorder is to characterize the disorder of increased or decreased movements to
better understand the diagnosis and management of the patient’s
symptoms.
PATHOPHYSIOLOGY OF MOVEMENT DISORDERS
Disorders of movement can be divided into those that cause
diminished (hypokinetic) movement and those that cause abnormal (hyperkinetic)
involuntary movements.
diminished (hypokinetic) movement and those that cause abnormal (hyperkinetic)
involuntary movements.
The hypokinetic movement disorders comprise mainly Parkinson’s
disease and other parkinsonian syndromes. Parkinson’s disease occurs due to
degeneration of substantia nigra neurons in the midbrain that project to the
basal ganglia. Parkinsonism, on the other hand, is
the generic name for any clinical syndrome that includes Parkinson’s
disease-like symptoms.
The hyperkinetic movement disorders include tremors, dystonia,
chorea, myoclonus, and tic disorders. Some of these symptoms occur due to
disorders of basal ganglia (extrapyramidal) function (e.g., chorea,
parkinsonian tremors, and, probably, dystonia), and some occur due to
disorders of cortical or spinal cord function (e.g., myoclonus), but the
pathophysiology of some of the hyperkinetic movement disorders (e.g.,
essential tremor and tic disorders) remains unclear.
TAKING THE HISTORY OF A PATIENT WITH A MOVEMENT DISORDER
More than any other neurologic disorder, the diagnosis of movement
disorders depends primarily on observation of the findings on examination.
Nonetheless, like any neurologic disorder, the history is integral to the
diagnostic process.
disorders depends primarily on observation of the findings on examination.
Nonetheless, like any neurologic disorder, the history is integral to the
diagnostic process.
In any patient with a movement disorder, take a good medication
history because many medications can cause extrapyramidal symptoms of
parkinsonism, dystonia, and chorea (e.g., neuroleptics and antiemetics), and
many can cause or worsen postural tremor (e.g., valproic acid, amiodarone, and
lithium). Make sure you ask about past medications and not just medicines the
patient is currently taking.
The family history can give important clues for some of the
movement disorders, such as Huntington’s disease and familial essential
tremor.
Have the patient describe the abnormal movements, the age of
onset (e.g., tic disorders usually begin in childhood), and the course of
symptoms (e.g., worsening over time suggests a degenerative
disorder).
Ask about any exacerbating or relieving factors (e.g., essential
tremor may be worse with stress and improved with alcohol; patients with
cervical dystonia may have found maneuvers to temporarily relieve the
symptoms; patients with focal dystonias may only develop their symptoms during
certain activities). Ask if there is any control over the movements (e.g., tic
disorders).
Inquire about other associated symptoms that may provide
diagnostic clues in the appropriate clinical situations, such as cognitive
symptoms (e.g., Huntington’s disease and Wilson’s disease), autonomic symptoms
(e.g., Shy-Drager syndrome), and systemic problems (e.g., liver dysfunction in
Wilson’s disease or a recent streptococcal infection in Sydenham’s
chorea).
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EXAMINING THE PATIENT WITH A MOVEMENT DISORDER
Parkinsonism
Parkinson’s disease usually presents as asymmetric slowness
(bradykinesia), rigidity, and tremors. Other parkinsonian syndromes are more
likely to present symmetrically.
Observe for diminished facial expression consistent with masked
facies and listen to the speech, which may be soft and even stuttering in some
patients.
Observe the patient for a resting tremor in the hands, although
not all patients have a tremor. The typical parkinsonian tremor is described
as pill-rolling because of the characteristic
oscillating movements between the thumb and the other fingers. It is often
asymmetric, especially early in the course of Parkinson’s disease, and it
tends to occur at rest and diminishes when the arm is performing a task. The
tremor may be particularly prominent when the patient is preoccupied with
another task, such as walking or manipulating the opposite hand. In some
patients, a similar resting tremor may be seen in a foot as it dangles off the
examining table.
Test for cogwheel rigidity as described in Chapter
27, Examination of Tone. The ability to perform fine movements and
rapid alternating movements (see Chapter 34, Testing of
Upper Extremity Cerebellar Function) may be impaired in parkinsonism due to
slowness from basal ganglia dysfunction. Strength should be normal.
Ask the patient to write a sentence (not their signature);
patients with Parkinson’s disease usually have small (micrographic), shaky
handwriting. Also ask the patient to draw a spiral from the inside out (draw a
sample of this for the patient, by drawing a loose spiral a few inches in
diameter); patients with Parkinson’s disease usually draw a small, tightly
packed spiral.
Examine the gait (see Chapter 39,
Examination of Gait) for typical signs of parkinsonism, including diminished
arm swing; multiple small, narrowbased, shuffling steps; and a stooped
posture. The patient may have difficulty getting up out of a chair to initiate
gait. Festination of gait refers to the tendency of
parkinsonian patients to keep speeding up when walking, with difficulty
stopping their forward progress. Make sure you observe the patient turn and
walk back to you, because patients with parkinsonism may show typical en bloc turning, taking multiple extra steps as they
attempt to change direction, rather than a normal pivot. There may also be a
tendency to freeze at doorways and with turns.
In any patient with suspected parkinsonism, assess postural
reflexes:
Ask the patient to stand still. Inform the patient that you
will be testing balance by pulling him or her backward and that the patient
should maintain balance as best as he or she can and may take steps backward
if necessary.
Stand directly behind the patient, so if the patient were to
fall backward, you would be able catch him or her.
Reach around and pull back quickly on the patient’s shoulders
and observe the patient’s ability to retain his or her balance. Obviously,
catch the patient if he or she begins to fall into you.
Impairment of postural reflexes would manifest as a series of
backward steps or as the patient falling backward.
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Patients with the parkinsonian syndrome of progressive
supranuclear palsy may have difficulty with voluntary conjugate eye movements
(see Chapter 14, Examination of Eye Movements) and
especially may have difficulty looking down as the earliest eye sign of the
disease. In these patients, the supranuclear (i.e.,
above the cranial nerve nuclei in the brainstem) cause of the dysfunction can
usually be detected by finding that the downward eye movements are intact when
the patient’s head is passively tilted upward while the patient is asked to
fixate his or her eyes forward on your face.
When the Shy-Drager syndrome (a form of multiple-system atrophy)
is suspected, be certain to look for evidence of orthostatic hypotension (see
Chapter 44, Examination of the Dizzy
Patient).
Postural (Essential) Tremor
Unlike parkinsonian tremors, patients with essential tremor or
other postural tremors have a tremor that is worse with activities that
involve maintaining their arm in a specific posture or with action, rather
than at rest. Holding a cup of coffee or a soup spoon may be difficult and
embarrassing to the patient.
To assess for a postural tremor, have the patient hold his or her
hands directly in front with the palms down and fingers spread (abducted), and
observe the hands. Depending on the severity, there may be a subtle fine
tremor of the fingers or more prominent, higher amplitude shakiness. Giving
the patient a cup of water to hold can also be a good way for you to see the
real-world severity of the tremor.
Watch the patient write a sentence of his or her choice. Patients
with essential tremor usually have tremulous handwriting that, unlike
parkinsonian writing, is not micrographic. Ask the patient to draw a spiral
(draw a sample for the patient), which will be shaky but not micrographic as
would occur in Parkinson’s disease.
Look for accentuation of essential tremor during the
finger-to-nose maneuver (see Chapter 34, Testing of
Upper Extremity Cerebellar Function) as an endpoint tremor. This usually
manifests as a fine shakiness when the patient’s finger gets close to your
finger or his or her nose but without the clumsiness (dysmetria) throughout
the maneuver seen in cerebellar disorders.
Dystonia
Dystonic movements are abnormal, sustained muscle contractions
that result in abnormal postures. Depending on the syndrome, these dystonic
postures may be focal or generalized, and they may involve the limbs, neck,
body, or face.
Patients with dystonia have abnormal postures of the involved
body part evident simply by observation during the examination.
Patients with focal cervical dystonia (torticollis) show abnormal
posturing of the neck, resulting in a combination of head turning, tilting,
flexion, or extension. The patient may have the ability to prevent the posture
by performing a self-learned simple maneuver such as touching the chin (called
a geste antagoniste).
Patients with symptoms suggestive of writer’s cramp or other
focal or occupational dystonias should be observed in the act of performing
the inciting activity (such as writing), so that you can observe the dystonic
movements.
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Chorea
Chorea manifests on examination as
rapid, fidgety-appearing movements, primarily of the extremities, but it may
also involve the face. These movements, which are often described as
dance-like, are not stereotyped and move unpredictably from one area of the
body to another (unlike myoclonus or tics).
Patients with generalized chorea (such as from Huntington’s
disease) look fidgety and may be misdiagnosed as simply “nervous.” Patients
with choreiform movements often, probably inadvertently, try to hide their
choreiform movements by incorporating them into maneuvers such as brushing
their hair with their hand.
Hemichorea is chorea involving only one side of the body.
Hemichorea and hemiballismus (defined as flinging
movements of the extremities on one side) are essentially clinically
indistinguishable from each other.
Tic Disorders
Tics are rapid, stereotyped movements or vocalizations. Motor
tics may involve the face (e.g., eye blinking), head (e.g., shaking), or
extremities. Unlike chorea, tics are generally stereotyped for each patient
(i.e., the patient usually manifests the same types of behavior
intermittently). Vocal tics may be simple sounds (e.g., throat clearing) or
more complex vocalizations, including obscenities (coprolalia).
Patients may be observed to be able to voluntarily suppress the
tics at least to some extent, but they usually describe a build-up of tension
that is released as they allow the tics to express themselves
again.
Myoclonus
The movements of myoclonus are rapid muscle jerks. Myoclonus may be
focal, involving a single extremity or part of an extremity, or generalized.
Generalized myoclonus is a common clinical finding, especially in patients with
metabolic encephalopathies.
focal, involving a single extremity or part of an extremity, or generalized.
Generalized myoclonus is a common clinical finding, especially in patients with
metabolic encephalopathies.
ADDITIONAL COMMENTS
The term athetosis is used to describe
slow, writhing movements of the extremities. These movements can occur within
the context of chorea (hence the common term choreoathetosis), but athetosis can also be seen in the
setting of dystonia or even peripheral nerve disorders affecting position
sense (pseudoathetosis), as described in Chapter 30,
Examination of Vibration and Position Sensation.
Wilson’s disease is an important, treatable (but otherwise
progressive) cause of a (hypokinetic or hyperkinetic) movement disorder. When
this disorder is a consideration, the finding of copper deposition around the
edge of the cornea (Kayser-Fleischer rings) needs to be sought on examination,
but it requires slit-lamp examination by an ophthalmologist to be
observed.