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A. Introduction to the concept of voluntary and involuntary movements and the notion of free will
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We experience ourselves as having free will. This experience leads us to classify behaviors intuitively as voluntary and involuntary. But then we must puzzle over behaviors such as breathing, bladder and bowel emptying, and postural reflexes that straddle the voluntary-involuntary dichotomy. For example, you can freely will yourself to hold your breath for a period of time, but ultimately you simply have to breathe. You have no choice. The physiologic imperative to act (i.e., to emit a certain behavior) overpowers the will. Mentally ill Pts often experience their behaviors and even their very thoughts as involuntary or directed by external forces. Thus, Pts come to the physician because they experience behaviors and thoughts that they cannot willfully control.
By virtue of operational definitions, we can identify certain movements and behaviors that we can agree to class as voluntary or involuntary.
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B. Working definition of voluntary and involuntary movements (behaviors)
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A voluntary movement is one that the standard normal person can start or stop at the person's own command or an observer's command.
An involuntary movement is one that the standard normal person does not start and cannot stop at the person's own or an observer's command.
As strictly construed by neurologists, involuntary movements mean those patterns of muscle contractions (tremors and other movement sequences) caused by identifiable structural or biochemical lesions in the circuitry of the basal motor nuclei, reticular formation, and cerebellum.
Broadly construed, the concept of involuntary movements can also include the gamut of muscle fiber contractions of peripheral and central origins, extending from fibrillations to epileptic seizures.
Write out the definition of behavior given in Chapter 1, page 1:
__________________________________________________________________
_________________________________.
Reread the statements in B1 to 2 a and 2b above and substitute the word behavior for movement. It will give you a different feeling for the definition of behavior.
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C. Clinical operations for identifying voluntary and involuntary movements
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Neurologic lesions result in patterns of involuntary movements, recognizable from the history, inspection, and sometimes requiring laboratory tests, as for fasciculations. The operations for clinical analysis of involuntary movements follow.
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Find out when the movements started, what conditions trigger or alleviate the movements, their relation to sleep and emotion, and their evolution over time. In other words, what is the history?
Describe the pattern of the movements, their distribution, rate, amplitude, and force. In other words, what are the physical findings?
Inspection is pivotal, allowing recognition without recourse to the Pt's testimony, in most cases. To see is to diagnose, because most involuntary movements fall into stereotyped, identifiable patterns (Fig. 15-4).
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D. Some normal involuntary movements
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Physiologic synkinesia (syn = with; kinesis motion): A synkinesis is an involuntary or automatic movement that accompanies a voluntary movement. If you voluntarily close your eyes, your eyeballs automatically roll up (Bell's phenomenon). Walk and your arms swing. Lean forward and your leg muscles automatically brace. Use these examples to identify other synkinesias (Hint: What about convergence of the eyes?). The degree of volition in the synkinesias varies. You can't stop Bell's phenomenon, but you can stop your arms from swinging when you walk.
Myoclonic jerks
Myoclonic jerks are sudden, brief, shock-like, involuntary twitches of individual muscles or sets of muscles. A myoclonic twitch may appear in a single muscle, such as the biceps, perhaps after unaccustomed work. Or a more widespread startle response may cause an upright jerk of the head as when the person is falling asleep. A sudden discharge in the reticular activating system of the brainstem causes the lightening, myoclonic jerk of the head and the sudden restoration of consciousness. Myoclonus may occur at rest or provoked by voluntary action (action myoclonus), or triggered by diverse stimuli (stimulus-sensitive or reflex myoclonus).
Epileptic myoclonic jerks, often refractory to treatment, occur in many CNS diseases. What is physiologic under one circumstance is pathologic under another.
Myoclonic jerks are separate from fasciculations. Define a fasciculation.
_______________________________________________________________
_______________________________________________________________
A fasciculation is a muscular twitch caused by random discharge of an LMN and its fascicle of muscle fibers (a motor unit discharge).
A twitch of the entire muscle or groups of muscles is called a _____________________ jerk, whereas a twitch of a single fascicle of muscle is called a _____________________.
myoclonus; fasciculation
Benign fasciculations
Fasciculations appear in some normal persons, particularly after exercise. If the person has no weakness or other signs of LMN disease, the diagnosis is benign fasciculations. Twitching of an eyelid is an example.
What clinical and EMG findings would differentiate pathologic from benign fasciculations?
_______________________________________________________________
_______________________________________________________________
If LMN disease causes the fasciculations, the Ex should find the full syndrome of LMN disease: weakness, hypoor areflexia, hypotonia, and denervation atrophy, as supported by fibrillations and giant polyphasic motor units in the EMG.
Physiologic tremor: See next section.
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Definition: Tremors are rhythmic, involuntary, oscillations of one or more regions of the body (Fig. 7-38).
Clinical characteristics of tremors: Types of tremors differ in distribution, rate, and amplitude and whether they occur at rest or during voluntary muscular contractions. They also differ in response to drugs, in response to sleep and emotion, and in pathogenesis.
Distribution: Tremors most commonly affect the head, jaw, tongue, palate, and hands but may affect the trunk or legs.
Rate: Tremors vary in rate between 3 and 12 cps.
Amplitude: Tremors vary from barely perceptible to gross.
Relation to rest and volitional muscular contractions: Tremors dichotomize into two main groups: rest tremor and action tremors. Action tremors appear during voluntary muscular contractions, either to move a part or to maintain a voluntary posture. Action tremors include postural, kinetic, isometric, and task-specific types (Fig. 7-38).
Response of tremors to drugs: Drugs may increase or decrease tremor. Tremors commonly accompany lithium treatment. Anticholinergic drugs or dopamine decrease the tremor of parkinsonism. Alcohol and propranolol decrease physiologic or essential tremor, whereas adrenalin increases them. Tremors, "the shakes," commonly follow withdrawal from alcohol and other drugs.
Response of tremors to sleep and emotion: Like virtually all involuntary movements, tremors generally increase during emotional stress but dampen or disappear during tranquility and cease during sleep (Chokroverty et al., 2002).
Lesion site for tremors: No single "tremorogenic" center exists. Tremors generally arise from disruption of the feedback circuits of the basal motor nuclei (Fig. 2-31), inferior olivary nucleus, and the cerebellum. Occasionally tremors occur with peripheral neuropathies.
Clinical characteristics of tremor types: Inspect the Pt for tremors under three conditions: rest, maintaining a posture, and during movement (Bain, 2002).
Rest (or resting) tremor is a tremor when the body parts are at complete rest, supported against gravity. Have the Pt sit with the arms relaxed and the forearms supported by the thighs or recline. Look for a tremor of the fingers and hands.
Rest tremor generally disappears during voluntary movement but increases during mental stress such as counting backward or when walking or moving another body part.
The Ex can bring out a minimal or inapparent tremor or increase the amplitude of a rest tremor of the hands by having the Pt move the head from side to side (Froment's maneuver).
Rest tremor is highly characteristic of parkinsonism.
Postural or position maintenance tremor occurs during the maintenance of any intentional posture, such as holding the head up, the trunk erect, or the arms out stretched. Postural tremor qualifies as an action tremor (Fig. 7-38) because the "action" refers to the sustained volitional contraction to hold the part in position. The hands, when extended in front, show a regular, rhythmic tremor of several cycles per second. If the Pt brings the finger in to touch his nose, intention tremor may appear. As the finger approaches the nose and the Pt attempts to stop the finger to maintain a position, the tremor reappears or heightens as an end point or terminal tremor. When the finger actually touches the nose, the tremor may or may not dampen. When the Pt returns his hands to his lap, at rest, any such action tremor disappears.
Kinetic tremor or intention tremor (also called ataxic tremor) may appear during any voluntary movement. At rest, the hand remains still, but upon movement, as when the Pt does the finger-to-nose test, mild to moderate deviations detour the part from a straight line path. Intention and end-point tremor appear in various degrees and combinations. They implicate a lesion of the cerebellum or its efferent pathways.
Task-specific tremors appear during defined tasks, such as writing. An orthostatic tremor, a very fine, rapid, 16-cps tremor of the legs appears mainly when the Pt stands, usually combined with a general feeling of unsteadiness. Some patients experience sudden falls. Listening to the muscles with the bell of the stethoscope may demonstrate the tremor (Gershlager et al., 2004). Isometric tremor appears during a sustained muscular contraction against a stationary object.
Mixed tremors: A particular type of mixed tremor that appears at rest, while maintaining a posture, and that becomes severe during voluntary movement often follows trauma to the midbrain or other midbrain lesions (Samie et al., 1990). Because the lesion affects the region of the red nucleus, this mixed type of tremor is called rubral or Holmes' tremor, implying a lesion of the dentatorubral or dentatothalamic tract, in the superior cerebellar peduncle pathway (Samie et al., 1990). Holmes' tremor mainly involves the hand and proximal arm, and is mostly unilateral. Dystonic tremor is a postural and kinetic tremor, usually not present at rest, and typically involving the body part affected by dystonia (Deuschl, 2003).
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F. Kymographic records of tremors
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From these kymographic recordings, identify the type of tremor and the pathophysiologic basis:
The Pt is sitting quietly in a chair. An accelerometer attached to a hand records this tremor (Fig. 7-39).
The rate of the tremor is about ____ cps.
5 or 6
Because the tremor occurs at rest but disappears during intentional movement, it qualifies as a □ rest/□ intention/□ postural tremor.
☑ rest
This type of tremor signifies a lesion of the extrapyramidal pathways and is characteristic of the disorder called _________________ (eponym).
parkinsonism
The next Pt was sitting quietly, holding her arms extended in front, at shoulder level. Only a faint instability appeared. Figure 7-40 shows the tremor when the Pt attempted to touch her index finger to her nose. After she reached her nose, the tremor increased somewhat.
The tremor illustrated in Fig. 7-40 is called __________________ tremor.
kinetic, intention, or ataxic
It signifies a lesion of the □ pyramidal/□ basal ganglia/□ cerebellar pathways.
☑ cerebellar
The next Pt had no tremor when sitting still, but when she held her arms straight out, a tremor appeared (Fig. 7-41).
After she reached her nose, the tremor was accentuated. The tremor shown in the initial phase (Fig. 7-41), when the Pt was holding her arms extended and still, is called □ resting/□ physiological/□ postural tremor.
☑ postural
It signifies a lesion of the ___________________ or its efferent pathways.
cerebellum
Because it may dampen during movement, postural tremor is like □ parkinsonian/□ intention/□ essential tremor.
☑ parkinsonian
Give the full clinical characteristics of parkinsonian tremor.
_______________________________________________________________
_______________________________________________________________
Parkinsonian tremor appears at rest, has a low amplitude and a regular frequency of around 5 cps, disappears during intentional movement and sleep, and increases during emotional stress.
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G. Clinical features of several common tremor syndromes
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Physiologic tremor
Self-demonstration of physiologic tremor: Insert a large sheet of paper between your index finger and the adjacent finger and hold your arm straight out in front of you. The rustling of the paper demonstrates physiologic tremor. The tremor has a frequency of about 10 cps.
Physiologic tremor is generally low in amplitude, relatively rapid (6 to 13 cps), and is most evident during movement or when a part sustains a posture. It varies from 6 cps in childhood, to 8 to 13 cps in adulthood, and back to around 6 cps in senility.
Physiologic tremor arises from a combination of neurally mediated oscillations and the ballistic effects of respiratory and cardiac actions (ballistocardiogram). Thus, it has neurologic and mechanical origins.
Emotional tremor, a normal phenomenon, to be distinguished from psychogenic tremor, is an enhanced physiologic tremor. It occurs at rest, but it worsens during volitional movement: Witness the quivering knees and quivering voice of the novice orator. From personal experience you know that emotional tremor is □ rapid/ □ very slow and of □ very great/□ relatively low amplitude.
☑ rapid; ☑ relatively low
Essential (familial) tremor
This autosomal dominant or sporadic disorder resembles physiologic tremor in frequency, with a range of 4 to 12 cps, but has a greater amplitude.
It affects the hands predominantly but may affect the head, bulbar muscles, and voice (Lou and Jankovic, 1989; Louis, 2001). It typically appears during a sustained posture but may appear during voluntary movements. It generally dampens or disappears with the Pt at rest. Tremors involving the tongue, trunk and lower limbs are rarely encountered.
The lesion site is unknown (Rajput et al., 1991).
Essential palatal tremor versus symptomatic palatal tremor (palatal myoclonus)
Palatal tremor has the characteristics of essential tremor. The Pt may experience an audible click. The tremor disappears during sleep.
A disorder formerly called palatal myoclonus has a frequency of 110 to 160 beats/min. It may affect other bulbar muscles derived from the branchial arches. It violates the law of disappearance of involuntary movements during sleep because it persists. It follows, with variable delays, lesions in the triangle between the cerebellum red nucleus, central tegmental tract, and inferior olivary nucleus (Mollaret's triangle).
Senile tremor has a frequency of about 6 to 10 cps. Senile and familial tremors merge in their clinical manifestations, but senile tremor, although frequently familial, may result from nonfamilial lesions of aging.
Parkinsonian tremor (Jankovic and Tolosa, 1998)
Parkinsonian tremor has a frequency of 3 to 6 cps and low to moderate amplitude. Drum your fingertips on the table, timing 25 beats per 5 seconds, to observe two features of this tremor: moderate frequency and relatively low amplitude. It appears when the part is at rest, increases during mental and emotional tension, but disappears or dampens during intentional movement, and is absent during sleep. Often it appears asymmetrically. Typically affecting the hands and digits, the rustling of the thumb against the pads of the fingers resembles pill-rolling; hence its name, pill-rolling tremor. Voluntary head movements enhance the tremor (Froment's maneuver).
A 4- to 8-cps postural tremor occurs also about as often as the classic rest tremor (Brooks, 2002).
Degeneration of the dopaminergic pathway that runs from the substantia nigra of the midbrain to the striatum causes parkinsonism, but other neurons also degenerate. The parkinsonian triad of rest tremor, lead-pipe rigidity, and hypokinesia may appear as an entity or with associated widespread neuronal degeneration, known as the Parkinson plus syndromes (Victor and Ropper, 2001).
Parkinsonian tremor differs from most tremors by □ increasing/□ decreasing during volitional movement, and resembles other involuntary movements by □ increasing/□ decreasing during emotional stress and □ increasing/□ disappearing during sleep.
☑ decreasing; ☑ increasing; ☑ disappearing
The rest tremor of parkinsonism, a hyperkinesia, contrasts with a reduction in the overall mobility of the Pt, called bradykinesia or hypokinesia. Paradoxically, an irresistible need to move, a physiologic imperative called akathisia (see Section XII P) also plagues the Pt, requiring abrupt, restless shifts of position against the background of bradykinesis and muscular rigidity. In a given Pt, one or more of the signs may predominate. Thus, one Pt displays mainly tremor and the next mainly rigidity. The basic motor signs of parkinsonism include a quatrain of:
Rest tremor
Lead-pipe rigidity, often with a "cogwheeling," ratchet-like yielding due to the superimposition of the beats of the tremor
Overall bradykinesia
Postural instability
Derivative signs of the rigidity of the laryngeal muscles are loss of inflections of the voice, resulting in a characteristic monotonous tone, i.e., plateau speech, and words running together (Caekebeke et al., 1991). Rigidity of the facial muscles results in an absence of emotional expression, i.e., a masked face.
Oculogyric crises are spasms of upward deviation of the eyes or of the eyes and head. Common in post-encephalitic parkinsonism, oculogyric crises may occur in other disorders of the basal motor nuclei. Summarize for yourself the motor manifestations of parkinsonism.
Neurologic evaluation scales for following the course of parkinsonism document changes in their status (Gollomp, 2002; www.wemove.org).
Neuropathic tremor: This tremor, usually an action type, occurs with a variety of acquired or hereditary peripheral neuropathies, more commonly with demyelinating than with axonal types (Bain, 2002). The tremors are mainly postural and action tremors.
Drug-induced and toxic tremors: A number of tremors, as with hyperthyroidism, lithium treatment, delirium, and drug over dosage or withdrawal (including alcohol), share features of one or more of the foregoing types of tremors or come from an enhancement of physiologic tremor.
Psychogenic tremors have inconsistent, complicated patterns that change with circumstances (Koller et al., 1989). Most of these tremors are action tremors. They often develop suddenly and sometimes have spontaneous remissions. The tremor decreases during distraction or volitional movement of the contralateral hand. When the Ex tests for muscle tone, the Pt shows resistance to passive movement of the joint, indicating increased tone of the part, and the tremor stops briefly, which is known as the coactivation sign (Deuschl et al., 1998). See Chapter 14 for other clinical features of psychogenic disorders.
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H. Disorders to differentiate from tremors
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Clonus is a repetitive MSR.
Asterixis consists of sudden lapses of a sustained posture, which may have a periodic or pseudo-rhythmic frequency. To elicit asterixis, ask the Pt to extend the arms straight out, with the wrists dorsiflexed. The wrist will then periodically drop and immediately re-extend. First described in hepatic encephalopathy, it also appears in other metabolic-toxic encephalopathies and after structural lesions of the cerebello-thalamo-cortical circuits (Victor and Ropper, 2001; Kim, 2002).
Myoclonus
Myoclonus means shock-like, lightning fast contractions of parts of muscles or groups of muscles. Individual movements are very brief but may be repetitive. They are irregular in rate and amplitude and symmetric or asymmetric (Victor and Ropper, 2001).
When restricted to one group of muscles, it is called segmental myoclonus clonus or myoclonus simplex.
When widespread, the movements are myoclonus multiplex or polymyoclonus.
Myoclonus appears in a large number of metabolic, toxic, and degenerative diseases and types of epilepsy and may arise from lesions at the spinal level. Many different disorders that do not belong together are semantically linked by the term myoclonus.
The distinction between polymyoclonus, chorea, severe ataxia, hyperexplexia, and multiple tics is sometimes difficult.
A distinctive syndrome of opsoclonus and polymyoclonus affects children as an autoimmune disorder and as a distant effect of neuroblastoma. Personality changes are usual, particularly irritability.
Rhythmic myoclonus versus so-called cortical tremor: The Pt shows intermittent brief jerks, irregular or rhythmic, of slow frequency and often limited to segmental levels (Deuschl et al., 1998). Cortical tremor consists of high-frequency jerks (7 to 18 cps) similar to high-frequency postural tremor. Epileptiform discharges appear in the electroencephalogram (EEG).
Partial continual epilepsy (of Kozhevnikoff/Kojewnikow): The Pt shows continuous low-frequency jerks of one muscle group days and nights for weeks, months to years. The EEG shows focal epileptiform discharges.
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Before proceeding with the text, review the tremor hyperkinesias by actually acting them out (Figs. 7-38 to 7-41). If you feel the need to, organize your own table or make a personal differential diagnostic dendrogram.
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BIBLIOGRAPHY · Tremor and Parkinsonism
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Bain
PG. The management of tremor.
J Neurol Neurosurg Psychiatry 2002;72 (suppl 1): i3–i9.
[PubMed: 11870197]
+
Brooks
DJ. Diagnosis and management of atypical parkinsonism syndromes.
J Neurol Neurosurg Psychiatry 2002;72 (suppl 1):i10–i16.
[PubMed: 11870198]
+
Caekebeke
JFV,, Jennekens-Schinkel
A,, van
der, Linden
ME, et
al. The interpretation of dysprosody in patients with Parkinson's disease.
J Neurol Neurosurg Psychiatry 1991;54:145–148.
CrossRef
[PubMed: 2019840]
+
Chokroverty
S,, Hening
WA,, Walters
AS, et al, eds. Sleep and Movement Disorders. Philadelphia, Butterworth-Heinemann, 2002.
+
Deuschl
G,, Bain
P,, Brin
M,, Ad
Hoc Scientific Committee. Consensus statement of the Movement Disorder Society.
Mov Disord 1998;13(suppl 3):2–23.
[PubMed: 9827589]
+
Deuschl
G,, Dystonic
tremor. Revue Neurologique 2003;159:900–905.
+
Gershlager
W,, Munchau
A,, Katzenschlager
R, et al. Natural history and syndromic association of orthostatic tremor: a review of 41 patients. Movement Disorders 2004;19(7):788–795.
CrossRef
[PubMed: 15254936]
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Jankovic
J,, Tolosa
E. Parkinson's Disease and Movement Disorders, 3rd ed. Hagerstown, Lippincott Williams & Wilkins, 1998.
+
Koller
W,, Lang
A,, Vetere-Overfield
B, et al. Psychogenic tremors. Neurology 1989;41:234–238.
+
Lou
JS,, Jankovic
J. Essential tremor: clinical correlates in 350 patients.
Neurology 1989;39:1094–1099.
CrossRef
[PubMed: 2761704]
+
Rajput
AH,, Rozdilsky
B,, Ang
L, et al. Clinicopathologic observations in essential tremor: report of six cases.
Neurology 1991;51:1422–1424.
CrossRef +
Victor
M,, Ropper
AH. Adams and Victor's Principles of Neurology. 7th ed. New York, McGraw-Hill, 2001.
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J. Non-tremor types of hyperkinesias
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Several hyperkinesia display characteristic clinical features that predict the lesion site (Table 7-8). (Chokroverty, 1990; Klawans et al., 1988; Lang and Weiner, 1992).
Chorea refers to incessant, random, moderately quick movements—a grimace, elevation of a finger or arm, a misstep when walking, an interruption when speaking. Overall, chorea resembles the "fidgets." One part or another of the body is flickering into motion all of the time. The movements resemble a choreographer working out the movements for a dance or, perhaps better described, they simulate fragments of normal movements. For instance, at one time or another, after you start to make an inappropriate movement (perhaps reaching up to pick your nose), you may suddenly decide to arrest it midway. Or after starting such a movement, you may have diverted it to brushing back your hair. Patients with chorea may employ this ruse. Nevertheless, an observer can perceive the stoppage or the diversion in the continuity of the initial movement.
Athetosis refers to slow, writhing movements of the fingers and extremities. If severe, athetosis affects speech and some proximal movements. These movements wax and wane and generally do not hold the part in a fixed posture. Athetosis often accompanies or follows partial interruption of the pyramidal tracts, particularly in Pts with cerebral palsy and spastic quadriplegia or diplegia. The quick random fidgety movements, called ________________, contrast with the slow writhing distal movements, called __________________.
chorea; athetosis
Dystonia refers to prolonged, slow, alternating contraction and relaxation of agonists and antagonists. The prolonged muscular contractions hold the part in one position for periods and may lead to pretzel-like body positions with fixed scoliosis and fixed contractures of joints.
Focal dystonia, such as spasmodic torticollis and writer's cramp, may affect more or less restricted groups of muscles.
The sustained postural deviations of dystonia differ from the quick movements of chorea and the slower writhing, mainly distal movements called ____________________.
athetosis
Although dystonia is traditionally classed as an extrapyramidal movement disorder, the lesion site and pathophysiology of the hereditary form are unknown (Zeman and Dyken, 1968), but dystonia can result from known, acquired lesions of the basal motor nuclei (Zeman and Whitlock, 1968).
Hemiballismus refers to violent flinging movements of one-half of the body. Ballista means to throw, as in ballistics. The Pt's arm thrashes about as if it were trying to fling away a handful of snakes. Hemiballismus usually appears abruptly in elderly hypertensive Pts. The lesion is predictable. A peculiar, sharply delimited hemorrhage destroys the contralateral subthalamic nucleus of Luys or its immediate surrounding pathways. This almond-sized and almond-shaped diencephalic nucleus belongs to the basal motor nuclei. Hemiballismus may also result from lesions in the caudate, putamen, globus pallidus, precentral gyrus, or thalamic nuclei.
Tics are quick, lightning fast, stereotyped, involuntary movements of face, tongue, upper extremities, or phonations. (Jankovic and Tolosa, 1998). In contrast to the preceding hyperkinesias, the sequence of movements is identical each time if the Pt has one type of tic. However, the Pt may have multiple kinds of tics. All of us display minor tics: wrinkling of the forehead, followed by blinking the eyes, or hitching up the trousers, or a shrug of the shoulder. Athletes display a number of tic-like maneuvers, as when a basketball player prepares to shoot a free throw, or a tennis player prepares to serve. Tics increase during emotional stress, are less prominent during periods of concentration, and often abate during sleep. Although mostly of low amplitude, tics, when violent, may throw the Pt to the floor, thus resembling an exaggerated startle response called hyperexplexia. Of the movement disorders discussed, tics, being quick and usually of low to moderate amplitude, most closely resemble □ chorea/□ athetosis/□ hemiballismus.
☑ chorea
Multiple tic syndrome of Gilles de la Tourette: Some tics are regarded as psychogenic, but they figure prominently in an organic disorder called Tourette's syndrome, which has three major features:
Multiple tics that change from time to time.
Involuntary respiratory actions and vocalizations with squawks, barks, howls or sniffs, humming, and sometimes the involuntary utterance of expletives.
Personality traits: sometimes rigid, obsessive-compulsive, or abrasive (Kurlan, et al., 2002; Leckman and Cohen, 1999) and attention deficit hyperactivity disorder (Tourette's Syndrome Study Group, 2002).
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K. Continuum between the named types of hyperkinesias
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Dystonia, athetosis, chorea, and tics represent way stations along a continuum of involuntary movements, not necessarily discrete entities. They differ perhaps more in their speed than in any other way. Tics and chorea are the fastest, with each movement measured in a second or even less. Next comes athetosis, lasting just a little longer, in seconds. Then comes dystonia, which lasts many seconds to minutes or even longer, as in spasmodic torticollis, a form of dystonia in which the head remains deviated for long periods.
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As an oversimplified, 1, 2, 3 mnemonic, think of each tic as shorter than 1 second (but the same tic may be repetitive), individual choreiform movements as lasting 1 second, athetosis as lasting 1 to 3 seconds (but the movements may flow into one another), and dystonia as lasting from 3 seconds to minutes or longer.
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Hemiballismus differs from chorea in its more violent amplitude and unilaterality. Hemichorea may occur, but it is most frequently bilateral, as in Huntington's or Sydenham's chorea.
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L. Drug-induced extrapyramidal movement syndromes and the tardive dyskinesias
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Various tranquilizers, antipsychotics, and antidepressant drugs alter the balance of neurotransmitters in the basal motor circuits (Yassa et al., 1990). These chemical lesions mimic the effect of anatomic lesions in producing hypo- and hyperkinesias, ranging from parkinsonism to dystonia. The involuntary movements often predominately affect facial, oral, buccal, and pharyngeal movements, with dysphonia and dysphagia (Meige's syndrome; Lang and Weiner, 1992). Unfortunately, tardive dyskinesia may be permanent and very resistant to therapy. Levodopa, used to treat parkinsonism, also produces dyskinesias (Olanow, 2000).
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M. The concept of deficit and release phenomena after lesions of the basal motor connections
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For general discussion, see Section XI of this chapter.
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Deficit phenomena after lesions of the basal motor circuitry include overall bradykinesia, gait impairment, masked facies, and loss of voice inflection.
Release phenomena include the hypertonia called lead-pipe rigidity, tremor (generally tremor of the part at rest), akathisia, and the patterned hyperkinesias such as tics, chorea, athetosis, dystonia, and hemiballismus. Whether the concept of release phenomena applies to these phenomena requires some stretch of the imagination. Even less does the concept apply to the other syndromes of excessive behavior, such as hyperactivity.
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N. Rating scales for involuntary movement disorders
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Various rating scales such as the Abnormal Involuntary Movement Scale enable the Ex to quantify various involuntary movements to determine the degree of disability, to follow the course of the disease, and to document the effects of treatment (Gollomp, 2002; Herndon, 1997; Marsden and Fahn, 1984, 1987, 1994).
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O. Some simple, general tests for motor dysfunction: writing, finger-tapping speed, the Archimedes spiral, and activities of daily living
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These tests are useful in analyzing most motor disorders, especially in Pts with hemiparesis, tremor, rigidity, or ataxia, not only for differential diagnosis but also to appreciate functional disability.
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Writing: Watch the Pt write spontaneously and, if a motor disorder or cerebral disorder is suspected, write a sentence to dictation. Like the gait, most motor disorders affect writing. The ataxic dysgraphia of cerebellar disease, the micrographia of rigidity, and the tremulous dysgraphia of essential tremor stand out vividly from each other.
Finger-tapping speed
Technique: For a convenient bedside test in lieu of an actual counter, place an ordinary audiocassette on the table and have the Pt grasp it between the thumb and third digit, leaving the index finger free to tap (Fig. 7-42). Ask the Pt to tap as rapidly as possible for several seconds. The Ex should demonstrate the test first. Normal subjects tap at rate of around 50 taps per 10 seconds. Children and the aged tap at slower rates.
The cassette serves three purposes:
The Ex will quickly learn about the Pt's fine motor skills.
The dyspraxic Pt has difficulty assuming the position or even in picking up and arranging the box.
Most important, the cassette amplifies the sound so that the Ex can estimate the speed and the rhythm. The ear can detect subtle differences between the right and left hands far better than the eye.
Because the test engages the entire central and peripheral motor systems, spasticity, rigidity, ataxia, and neuromuscular disorders will slow finger-tapping speed discernibly, and the Ex will easily hear the disturbance in rhythm of the cerebellar Pt.
Archimedes spiral
Technique: The Pt places a pen in the middle of a sheet of paper and makes a spiral line encircling the center point, making several winds of the pen, out to the periphery.
The difference in rigidity that narrows the spiral but leaves its lines fairly regular, ataxia in which the spiral's lines irregularly weave in and out, and the continuous wavering of lines of essential tremor are striking.
Tasks of daily living: Frequently the Ex should elect to watch the Pt button, use scissors, tie shoelaces, drink a glass of water, and arrange clothes as if to put them on.
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BIBLIOGRAPHY · Finger Tapping
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Blessing
W. Alternating two finger tapping as part of the neurological motor examination.
Aust N Z J Med 2000;30:506–507.
CrossRef
[PubMed: 10985519]
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Cousins
MS,, Corrow
C,, Finn
M, et al. Temporal measures of human finger tapping: effects of age.
Pharmacol Biochem Behav 1998;59:445–449.
CrossRef
[PubMed: 9476994]
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Moritz
CH,, Haughton
VM,, Cordes
D, et al. Whole-brain functional MR imaging activation from a finger-tapping task examined with independent component analysis.
Am J Neuroradiol 2000;21:1629–1635.
[PubMed: 11039341]
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P. Akathisia, restless legs, and hyperactivity: the urge to move
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Akathisia refers to motor unrest manifested by continual shifting of positions and sometimes by restlessly moving about. When questioned, Pts often report an actual feeling in their muscles of an urge to move (Sachdev, 1995). Akathisia appears typically in Parkinson's disease, in other diseases of the basal motor nuclei, and with psychotropic medications.
Restless-legs syndrome (Ekbom's syndrome; Chokroverty and Jankovic, 1999; Ekbom, 1960): When attempting to rest or sleep, these Pts feel an irresistible urge, a necessity, to move their legs around. No force of will can hold the legs still against the pathophysiologic imperative, and the aimless, incessant wandering of the legs prevents the onset of sleep. Symptoms are partially or totally relieved by movements such as walking or stretching (Allen et al., 2003). Exhaustive exercise or some drugs, such as antihistamines, may induce restless legs.
The hyperactive child: Hyperactive children display incessant, rapidly changing motor activity, often starting in infancy. Their driven, inappropriate, and usually annoying activity consists of fidgeting, pushing, pulling, banging, rummaging, clamoring, whining, and running. The hyperactivity continues with little regard for other people, danger, and reward and punishment. Rather than a standard, stereotyped pattern like athetosis or dystonia, the disorder consists of the sheer quantity of activity. Usually these children are so clamorous that my secretary can already diagnose them by the noise they make as they clatter down the long hall to my office. By stretching the imagination, we might think of restless legs as a localized type of akathisia and of childhood hyperactivity as its generalized childhood counterpart. No specific known brain lesion underlies hyperactivity, but many hyperkinetic children have other subtle or overt evidence of brain damage or frank mental retardation. The foregoing syndromes of driven behavior border on a variety of cursive states such as compulsive walking or running and cursive epilepsy. All have in common an irresistible drive, a pathophysiologic imperative, to move.
Self-mutilation: The Pt compulsively inflicts self-injury by biting, scratching, or pounding despite punishments or rewards. The head-banging infant or child particularly distresses parents. Although generally seen in mentally retarded Pts, some individuals with normal intelligence scratch, bite fingernails, pick their nose or lips, or otherwise injure themselves compulsively (neurodermatitis) in response to some pathophysiologic imperative that overcomes willpower. Smoking with its intermittent irritation of the lungs may fall into the same category. The Pt scratches, as it were, the respiratory mucosa, but of course chemical addiction belongs to the scenario.
Stereotyped behavioral mannerisms: Many retarded, autistic, and some otherwise normal children and psychotic adults display repetitive behaviors, including spinning, rocking, patting, touching, grimacing, licking, and mouthing. Repetitive back arching and thigh squeezing may act as masturbatory equivalents. Children with Rett's syndrome display a characteristic hand wringing. Where these behaviors, in addition to so many of the behaviors discussed above, fall in the scale between voluntary and involuntary further stretches the notion of free will.
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Definition: Epilepsy is any change in the mental, motor, or sensory state of the Pt caused by an abnormal hypersynchronous discharge of neurons.
Involuntary epileptic motor activity may consist of tonic or clonic spasms and myoclonic jerks that affect all or part of the body or of complex automatisms with laughter and cursive states during complex partial (psychomotor) seizures.
If epilepsy causes the abnormal motor activity, the Pt usually will lose consciousness and have amnesia for the episode, and EEG monitoring usually will record epileptiform activity. However, during some epileptiform movements, the Pt retains consciousness (see Chapter 13).
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R. Avoiding pitfalls (pratfalls) in distinguishing psychogenic from organic motility disturbances
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Hysterical movement disorders or malingering may imitate organic movement disorders. See Chapter 14.
Anxiety or emotional tension makes virtually all hyperkinesias worse, but most dampen or disappear when the Pt is relaxed or asleep. Only a few abnormal involuntary movements occur during sleep: sleep myoclonus, palatal myoclonus (actually palatal tremor), somnambulism, and some epileptic seizures.
During the evolution of one illness, the form of the hyperkinesias or the degree of hypo- or hypertonia may change. Hence, what starts as a flaccid hemiparesis may end as spastic hemiparesis. Simple athetosis may end as dystonia or so-called tension athetosis, in which the Pt's efforts to make a voluntary movement is paralleled by the degree of an athetoid or dystonic spasm. Torsion dystonia and tension athetosis are classic examples of pathophysiologic imperatives that determine the contraction of the muscles, rather than the Pt's "will."
Psychotropic medications, alcohol, and street drugs frequently cause hypokinetic or hyperkinetic movement disorders. Consider drugs in the differential diagnosis of tremors or any other movement disorder of new onset.
Mothers of hyperactive children invariably appear distraught, depressed, and defeated by their inability to cope with their children. Usually the child's hyperactivity discloses itself by the slapdash, frenetic way the child executes the Ex's requests. If, however, the child appears calm during the period of examination, as a few will, the Ex may mistakenly conclude that the mother is "overanxious." The usual problem is an underanxious physician, not an overanxious mother. Thus the immediate inspection of the Pt, which serves so well to recognize the standard hyperkinesias of extrapyramidal origin, may, on some occasions, fail in recognizing the hyperactive child if the observation period is too brief. On the next visit, detain the child in the waiting room for a considerable period before the appointment. Then, after the child has defaced your office décor, scattered the toys all over the reception room (without actually playing with any), exasperated your other Pts, and driven your receptionist mad, you will understand the mother's plight.
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The Pt is a mentally normal 47-year-old woman who was born with mild spastic cerebral palsy. Her motor disorder has not changed in the decades since childhood. For the serial photographs, she was requested to extend her arms out in front of her and to hold them as still as possible. Similar movements appear when she is at rest or walking. The individual movements last 2 to 3 seconds and have low to moderate force and amplitude, but may merge with each other in a continuous pattern. The movements shown in Fig. 7-43 would best be classified as __________________.
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T. A summary of involuntary movements (hyperkinesias) by operational definition
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For clinical diagnosis, I have defined hyperkinesias broadly to include any extra muscular activity of peripheral or central origin caused by a lesion of the nervous system.
For the clinical characteristics in frames a to v, write down the proper descriptive diagnosis in the blank. Where possible, act out the motility disorder described.
Spontaneous random contractions of denervated muscle fibers detected by EMG: ________________________.
fibrillations
Spontaneous random twitches of small parts of muscles detected by clinical inspection and EMG: ________________________.
fasciculations
Sudden spontaneous contraction of a muscle or group of muscles, which may simulate a startle reaction: ________________________.
myoclonic jerks
Spontaneous stereotyped sequence of muscular contractions, most prominent in facial muscles, in patients with obsessive-compulsive personality traits: ________________________.
tics
Spontaneous tonic or clonic jerking of the body, often accompanied by loss of consciousness: ________________________.
epilepsy
Spontaneous, quick movements simulating fragments of normal movements, usually most prominent in extremities: ________________________.
chorea
Spontaneous, writhing movements of fingers and extremities that may affect facial and axial muscles: ________________________.
athetosis
Spontaneous, long-sustained deviations of appendicular and axial parts, with alternating agonist-antagonist contractions, that may ultimately lead to fixed deformities: ________________________.
dystonia
More or less incessant (during waking hours), wild, flinging movements of one-half of the body, seen usually in elderly hypertensive patients: ________________________.
hemiballismus
Spontaneous upward deviation of the eyes and head, seen usually with rest tremor and lead-pipe rigidity: ________________________.
oculogyric crisis
Irresistible wandering of the legs, especially when the patient tries to rest or to sleep: ________________________.
restless-legs syndrome of Ekbom
Incessant, driven, usually annoying or aggressive behavior in a child: ________________________.
hyperkinesia
A tremor at rest of 6 cps, which dampens or disappears on intentional movement: ________________________.
parkinsonian (rest) tremor
Irregular tremor of a movement in progress, but no tremor at rest: ________________________.
intention (ataxic) tremor
Rapid tremor of an outstretched hand that dampens when a movement is in progress and reappears when a new posture is held: ________________________.
postural tremor
A 10-cps tremor of the hands that often has an autosomal dominant hereditary pattern: ________________________.
familial (essential) tremor
An 8-cps tremor of the head or of the head and hands occurring in an elderly patient: ________________________.
senile tremor
A therapy-resistant hyperkinesia usually with predominant face, lip, and tongue movements that appear after prolonged ingestion of psychotropic medications: ________________________.
tardive dyskinesia
A state of motor unrest in a parkinsonian Pt characterized by irresistible restless shifting of postures: ________________________.
akathisia
Sudden yielding of a sustained posture, as of the dorsiflexed wrist with the arms extended: ________________________.
asterixis
Prolonged rippling or undulating, worm-like contractions of muscles, distributed focally or in widespread groups, associated with repetitive discharges of groups of motor units: ________________________.
myokymia
Irregular, quick, low amplitude, widespread movements of trunk and extremities, often associated with opsoclonus: ________________________.
polymyoclonus
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BIBLIOGRAPHY · Involuntary Movements
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Allen
RP,, Picchietti
D,, Hening
WA, et al. Restless legs syndrome: diagnostic criteria, special considerations, and epidemiology. A report from the restless leg syndrome diagnosis and epidemiology workshop and the National Institutes of Health. Sleep Medicine 2003; 4:101–119.
+
Chokroverty
S. Movement Disorders. Great Neck, PMA Publishing Corp., 1990.
+
Chokroverty
S,, Jankovic
J. Restless legs syndrome. A disease in search of identity.
Neurology 1999;52:907–910.
CrossRef
[PubMed: 10102404]
+
DeMyer
W. Spasmodic torticollis, status marmoratus and status dysmyelinatus. Consensus statement of the Movement Disorder Society. Mov Disord 1998;13(suppl 3):2–23.
+
Gollomp
S. Track Parkinson's progression with this handy tool. Practical Neurol 2002;1:42–51.
+
Herndon
RM, ed. Handbook of Neurologic Rating Scales. New York, Demos Vermande, 1997.
+
Klawans
HL,, Goetz
CG,, Tanner
CM. Common Movement Disorders. New York, Raven, 1988.
+
Kurlan
R,, Como
PG,, Miller
B, et al. The behavioral spectrum of tic disorders. A community-based study.
Neurology 2002;59:414–420.
CrossRef
[PubMed: 12177376]
+
Lang
AE,, Weiner
W. Drug-induced Movement Disorders. Mount Kisco, Futura Publishing, 1992.
+
Leckman
JF,, Cohen
DJ, eds. Tourette's Syndrome—Tics, Obsessions, Compulsions: Developmental Psychopathology and Clinical Care. New York, John Wiley & Sons, 1999.
+
Marsden
CD,, Fahn
S, eds. Movement Disorders. 3 vols. Oxford, Butterworth-Heinemann, 1984, 1987, 1994.
+
Meyers
R. Ballismus. In: Vinken PJ, Bruyn GW, eds. Handbook of Clinical Neurology. Vol 6. Amsterdam: North-Holland, 1968; 476–490.
+
Olanow
CW, ed. Levodopa-induced dyskinesias. Ann Neurol 2000;47(suppl 1):S1–S203.
+
Sachdev
P. Akathisia and Restless Legs. New York, Cambridge University Press, 1995.
+
Sawle
G, ed. Movement Disorders in Clinical Practice [with CD-ROM]. Oxford, Isis Medical Media Ltd, 1999.
+
Vitek
JL,, Giroux
M. Physiology of hypokinetic and hyperkinetic movement disorders: model for dyskinesia. Ann Neurol 2000;47(suppl 1):S131–S140. Also see: http://www.wemove.org.
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Yassa
R,, Nair
NPV,, Iskandar
H,
et al. Factors in the development of severe forms of tardive dyskinesia.
Am J Psychiatry 1990;147:1156–1163.
[PubMed: 1974745]
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Zeman
W,, Dyken
P. Dystonia musculorum deformans. In Vinken PJ, Bruyn GW, eds. Diseases of the Basal Ganglia. Handbook of Clinical Neurology, vol. 6. Amsterdam, Elsevier, 1968.
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Zeman
W,, Whitlock
C. Symptomatic dystonias. In Vinken PJ, Bruyn GW, eds. Diseases of the Basal Ganglia. Handbook of Clinical Neurology, vol 6. Amsterdam, Elsevier, 1968.