JMD 4 1 21 4
JMD 4 1 21 4
JMD 4 1 21 4
Psychogenic Movement Disorders (PMD) are one of the most challenging neuropsychiat-
ric disorders to both the Neurologist and the Psychiatrist which have baffled the specialists
of both these disciplines, time and again, over more than a century. These disorders cannot
be fully accounted for by any known organic syndrome and which appear, as based on avail-
able clinical evidence, to have significant psychological and or psychiatric contributions.
Elegant descriptions are available in writings of Charcot,1 Gowers,2 and Head.3
The psychogenic neurological disorders include psychogenic hemiplegia, paraplegia, blind-
ness, seizures, pain syndromes, and a variety of movement disorders PMD. The latter include
Received December 15, 2010 hyperkinetic syndromes of tremor, jerks, and spasms (dystonias), gait disorders, and less com-
Accepted December 22, 2010 monly a hypokinetic extrapyramidal syndrome mimicking parkinsonism. PMD has also been
Corresponding author sub classified into dystonic and non-dystonic.4 While clinical acumen by trained neurologist
Pramod Kumar Pal, MD, DM is often sufficient to diagnose some PMDs, there are clinical situations when clinical charac-
Additional Professor of Neurology, teristics may be inconclusive to make a firm diagnosis of PMD, which is required for man-
National Institute of Mental agement of the patient. There are no specific laboratory investigations to diagnose PMD and
Health & Neurosciences (NIMHANS),
Hosur Road, Bangalore-560011, appropriate investigations need to be carried out to rule out an organic basis of these neuro-
Karnataka, India logical disorders. However, over the past two decades, several investigators have reported
E-mail [email protected] usefulness of electrophysiological tests to differentiate between true and psychogenic move-
he author has no financial conflicts of
T
•- ment disorders. This article will review the role of electrophysiology in diagnosis of PMD.
interest. The exact prevalence or incidence of PMD is unknown. Small cohorts of patients and in-
adequate follow up preclude adequate information on the epi- affected part, 2) decrease or resolve when not the clear focus
demiology of PMD. The prevalence of psychogenic neurologi- of attention or enquiry and during tests requiring concentration
cal disorders have been reported to be 1-9% among patients or other tasks, 3) triggered or relieved with unusual nonphysi-
attending Neurology clinics.5-7 Among the patients attending ological interventions (such as trigger points on the body, tun-
specialty clinics of movement disorders, the prevalence has ing fork), 4) deliberate slowness of movements, 5) rhythmical
been reported to vary from 2.1% (4) to 3.3% (8). The preva- and often violent shaking, 6) changing characteristics of move-
lence of PMD is higher in women compared to that in men ments -severity, frequency, type, and distribution, 7) entrainment
(-3 : 2).4,8 Age of the patients vary from 17-83 years with a (see below), 8) selective disability, 9) demonstration of fatigue,
mean age of 50.8 The age of onset of PMD is quite variable, 10) presence of multiple movement disorders, 11) presence of
and has been reported to be 8-58 years.4 bizarre movements which are difficult to classify, 12) excessive
Tremor is the most commonly observed PMD8,9 though in a startle response, 13) paroxysmal movement disorders, and 14) bi-
large series10 dystonia was found to be commonest. On the con- zarre gait
trary, Monday and Jankovic11 reported psychogenic myoclo-
nus to be the commonest PMD (20.2%) of the 89 PMD pa- Psychogenic tremor
tients seen in the movement disorder clinic at Baylor College Psychogenic tremor most often involves the right hand
of Medicine, Houston, Texas, USA, from 1981 to 1991. The oth- (84%), followed by legs (28%), generalized (20%), left arm and
er PMDs are by gait disturbances, parkinsonism, stiff person head (8% each).15 Tremor of voice, face, tongue, and fingers is
syndrome, tics, belpharospasm, hemifacial spasm and other fa- distinctly uncommon. Patient with psychogenic tremor may
cial movements, paroxysmal dyskinesias or shaking, tics, cho- have a selective disability, e.g., normal writing but tremulous-
rea and undifferentiated movements. In addition to a PMD, a ness on drawing a spiral. Suggestion and placebo administra-
patient may have another PMD (e.g., tremor and dystonia), an tion can be used diagnostically to exacerbate or relieve tremor.
organic movement disorder, or a psychogenic neurological dis- A patient with psychogenic tremor of one limb, when asked
order other than movement disorder (e.g. hysterical paralysis, to perform rapid or slow movements with his “unaffected”
blindness, or pain). A combination of PMD and organic move- limb, may fail to continue with the original frequency of the
ment disorder has been reported in 10-25% of cases8,12 and “abnormal” movement of the “affected” limb. The frequency
usually the organic disorder precedes the PMD by years. of tremor of the affected limb may be entrained by the new fre-
Coexistent psychiatric diagnosis in the form of anxiety quency of the unaffected limb (entrainability of tremor). This
(38.%) and major depression are common (19.1%) in patients clinical observation can be confirmed by coherence analysis,
with PMD, and they also have a higher chance of other life-time described below.
psychiatric diagnosis, including adjustment disorder, schizoaf- Another feature which is often characteristic of psychogen-
fective disorder, bipolar disorder, and alcohol or sedative ab- ic tremor is the inability of the patient to maintain the same fre-
use.13 In general, patients with PMD generally fall into one of quency or pattern of abnormal movements when observed over
the two broad categories of psychiatric dysfunctions:14 1) symp- prolonged periods. A flexion-extension movement of the fingers
tom production not under conscious voluntary control which may change to a pronation-supination movement of the fore-
include conversion disorder and somatization disorder (Hyste- arm. While a patient of organic tremor can maintain the same
ria or Briquet’s syndrome); and 2) symptom production under frequency of tremor even on performing mental tasks such as
voluntary control which include patients either suffering from serial-7 subtraction or when engaged in conversation, a patient
factitious disorder or malingering. will psychogenic tremor usually fails to maintain the same fre-
quency. This phenomenon, called “distractibility” was present
Clinical characteristics of PMD in 86-100% of patients with psychogenic tremor15,16 and may
A detailed clinical history with separate interviews of the pa- sometimes need electrophysiological evaluation for documen-
tient and the caregiver, meticulous neurological examination, tation.
and prolonged observation of the patient over several sessions “Coactivation sign” described to be a characteristic feature
usually provide sufficient clues to make a diagnosis of PMD. of psychogenic tremor, consists of presence of voluntary coact-
Patients with PMD usually have acute or subacute onset of ivation of agonist and antagonist muscles of the respective joint
symptoms with maximum severity at the onset and thereafter a with overlying rhythmic trembling.15 This sign can be elicited
static course. In a large majority of patients a clear precipitating by palpating the muscles when testing for rigidity. In the trem-
factor, often in the form of psychosocial stress, is present and ulous hand, there is active resistance against passive, arrhyth-
there may be associated past or present psychiatric illness. The mic movements performed by the examiner about the involved
PMD may be paroxysmal, mimicking organic paroxysmal joint in opposite directions (e.g., flexion and extension of wrist).
movement disorders. Clinical features of PMD include: 1) There is fluctuation of the tone with reduction or increase in the
increase or become elaborate when examination is focused on tremor. Finally there may be a temporary but complete normal-
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Psychogenic Movement Disorders ▐ Pal PK
ization of the tone with disappearance of tremor. This co-acti- fered from idiopathic Parkinson’s disease. Apart from the char-
vation sign can be electrophysiologically documented (see acteristics of psychogenic tremor described above, the “rest”
below). tremor may fail to change with posture or action. Early maxi-
mum disability with a static course, voluntary resistance (often
Psychogenic dystonia reducing when distracted) rather than a true rigidity, absence of
Among the patients diagnosed as dystonia, the prevalence of micrographia, normal facial expression and voice, arms held
psychogenic dystonia may range from 2.2% to 4.6% depending stiffly at the sides while walking, and extreme or bizarre re-
on the criteria used (documented, clinically definite, probable sponse to minimal displacement on “pull test” are other charac-
or possible psychogenic dystonia).10,17 It is important to note that teristic features of psychogenic parkinsonism.24
in 25-52% of cases organic dystonia may be misdiagnosed as
psychogenic dystonia.16,18-21 The reasons for this misdiagnosis Red flags in diagnosis of psychogenic tremor
are several common characteristics between organic and psy- Appropriate caution should be exerted in labeling an organ-
chogenic dystonias, such as features of 1) varied nature of ab- ic tremor syndrome as psychogenic tremor. Psychogenic trem-
normal movements, alone or in combination, or co-occurrence or may coexist with organic movement disorders including
of organic and psychogenic movements in the same patient 2) tremor. Tremor following vascular strokes and trauma may
spontaneous remissions (in organic dystonia up to 20% cases, have abrupt onset, and that secondary to exposure to toxins or
especially cervical dystonia), 3) task specificity, 4) paroxysmal drugs may have fluctuation or spontaneous remissions, simu-
course, 5) absence of other neurological deficits and normal in- lating psychogenic tremor. “Rubral” or midbrain tremor fol-
vestigations (e.g., in idiopathic torsion dystonia), 6) temporary lowing demyelination, trauma, or that in Wilson’s disease can
relief by “sensory tricks” (geste antagoniste) (though character- have a combination of coarse rest and action (postural or kinet-
istic of organic dystonia, has been also reported in psychogen- ic) tremor can be sometimes mistaken for psychogenic tremor.
ic dystonia,22 7) relief or reduction by relaxation and hypnosis, Finally a feeling of tremulousness in legs when standing, inabil-
8) even minor trauma (head/neck or peripheral) may precede ity to stand, but ability to walk normally after few steps can be
onset of true dystonia. In addition, patients with psychogenic a manifestation of orthostatic tremor, which may be overlooked.
dystonia may have relief of dystonia by trigger point pressure
or injection (e.g., of saline). Diagnostic Criteria of PMD
Psychogenic myoclonus There are no diagnostic laboratory criteria for PMD. A val-
A diagnosis of psychogenic myoclonus is made when patients id positive criteria of PMD based on clinical examination is
have jerks inconsistent or incongruous with typical myoclonus rare, and currently the diagnosis is based on negative criteria,
and have clinical characteristics of PMD described previously, viz. absence of symptoms indicative of an underlying organic
such as diminished movement with distraction, periods of spon- disease.
taneous remission or remissions following suggestion and pla- Fahn and Williams10 and Fahn4 categorized patients into four
cebo, presence of other psychogenic symptomatology, and ev- levels of certainty as to the likelihood of their having a PMD.
idence of psychopathology by testing or by past psychiatric hi- The degrees of certainty are documented PMD, clinically es-
story.11 The distribution of psychogenic myoclonus has been tablished PMD, probable PMD, and possible PMD. With
reported to be predominantly segmental or generalized and availability of advanced electrophysiological techniques to
less often focal.11 As with other PMDs, women are more often evaluate certain PMDs, Brown and Thompson25 proposed a
affected and patients may have additional PMDs such as gait, combined clinical and electrophysiological classification of
tremor and dystonia. Patients with psychogenic myoclonus in- PMD. The classification included definite, probable and possi-
volving the whole body and precipitated by stimulus, habituate ble PMD, with supporting electrophysiological evidence of
with repeated stimuli, like normal startle response.23 However, PMD in the first two categories.
psychogenic myoclonus may mimic closely propriospinal my-
oclonus and in such situation electrophysiological evaluation Electrophysiological Evaluation
may be helpful (see below). of Movement Disorders
Psychogenic parkinsonism Electrophysiological evaluation of movement disorders help
Parkinsonism as a primary psychogenic manifestation is to identify and characterize a variety of abnormal movements
rare. In the series by Lang et al.24 the mean age of presentation which include tremor, myoclonus, dystonia, tics, asterexis,
was 47 years and 71% noted symptoms after work-related in- ataxias, etc. It also helps to characterize mixed movement dis-
jury or motor vehicle accident. Majority (57%) had bilateral orders or clinically bizarre movements. In certain cases, such
symptoms and the nature of tremor, rigidity, bradykinesia dif- as myoclonus or tremor, advanced techniques of electrophysi-
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Journal of Movement Disorders ▐ 2011;4:21-32
ology may also help to precisely locate the origin of these mo- The patient is comfortably seated in a chair and explained
vements in the central nervous system. about the test and the different maneuvers which need to be
With advances in electrophysiologic techniques there has performed while tremor recording. It is recommended to ac-
been greater understanding of the pathophysiology of move- quire EMG and accelerometry data for sufficient lengths of
ment disorders, identification of characteristic physiological time (100 seconds to even few minutes) for each condition (de-
changes of many of these disorders, and recognizing patterns scribed below).
of movement that could be under voluntary control.25 On the affected limb, surface electrodes are placed in a ten-
Electrophysiological evaluation of different types of PMD don-belly arrangement to record EMG activities from the an-
is well established. Currently available electrophysiological tagonistic muscles. The actual movement of the body part can
tests are most useful for tremor, and to some extent for myoc- be recorded by a lightweight piezoresistive accelerometer
lonus, and least for dystonia. The commonly used tests are mul- strapped over body parts involved in tremor. Single axis accel-
tichannel surface electromyographic (EMG) recording, accel- erometers are commonly used, but triaxial ones are available
erometry, electroencephalography (EEG) time locked with to record the movements in three axes (vertical, horizontal, and
EMG, premovement potential (Bereitschaftspotential), and so- antero-posterior). On the unaffected side, two-channel sur-
matosensory evoked potentials. Other tests which may be of val- face EMG, examining the flexors and extensors of the wrist or
ue include long-loop responses, H-reflex recovery curve, and fingers may be sufficient along with one single-axis accelerom-
transcranial magnetic stimulation (TMS). eter placed over the moving part. This will help to capture the
voluntary movement which the patient will be required to do
Evaluation of psychogenic tremor to study the entrainability and, distractibility of the tremor of
The basic electrophysiological evaluation of tremor usually the affected side and coherence.
involves recording the actual movement of body parts using
an accelerometer and the EMG activity from the involved Protocol and analysis
muscles using surface electrodes. Multi-channel surface elec- The protocol for evaluation of psychogenic tremor is given
tromyography and at least two accelerometers are required for in Table 1. Tremor is recorded in different conditions to docu-
evaluation of psychogenic tremor. Clinical examination and ment changes of the characteristics of tremor with posture,
prolonged observation usually help the examiner to identify voluntary movements of unaffected body part, distraction,
the muscles which are “tremulous” and require to be studied. weight-loading, peripheral and central stimulation, etc. After
24
Psychogenic Movement Disorders ▐ Pal PK
acquisition of EMG and accelerometric data, further analysis tremor at the onset of tremor. Electrophysiologically, there is a
is done to characterize the tremor (Table 2): a) determine the tonic coactivation phase of the wrist flexor and extensor mus-
frequency and power spectrum of the tremor, b) pattern of EMG cles -300 ms before the reciprocal alternating tremor bursts
bursts, c) modulation of frequency and amplitude of tremor by develop.15
mental tasks, and movement of other parts of body, and d) de-
termine whether tremor with different frequencies coexist. Tremor amplitude
The amplitude of tremor is variable and is not of much diag-
Pattern of EMG bursts nostic significance to differentiate between organic and psycho-
The majority of patients with ET (type A), cerebellar, and en- genic tremor. In psychogenic tremor apart from a change of fre-
hanced physiological tremor have a synchronous pattern of quency with distraction or suggestion, there may be a change is
muscle activation while an alternating activation pattern is seen amplitude also. However, amplitude of tremor is very variable
in patients with PD rest tremor, rubral tremor, some patients in organic tremors. In contrast to organic tremors, in psychogenic
with ET (type B). In psychogenic tremor usually an alternating tremor there is an increase in tremor amplitude with peripher-
pattern is seen. It should be remembered that in 10-15% of PD al loading.
patients, a synchronous pattern can be found during action but
a reciprocal alternating pattern during rest and this feature Tremor frequency
should not point towards a non-organic basis. In addition, it has Analysis of the frequency of tremor is probably the most im-
also been observed in the same patient both patterns of muscle portant step to differentiate between organic and functional
activity can occur. tremors. Based on the frequency, tremor is classified as low,
medium and high frequency. Though the frequency of tremor
Duration of EMG bursts can be determined by counting the number of EMG bursts over
The duration of EMG bursts are often helpful to differentiate a given length of time, power spectrum analysis is preferable.
between organic and psychogenic tremors. The EMG bursts in The accelerometer recording and the rectified and digitally fil-
psychogenic tremors is usually > 70-80 ms. However, it should tered EMG (below 50-100 Hz to exclude movements artifacts)
be noted that in organic tremors with EMG recorded from larg- of each muscle is subjected to Fast Fourier Transformation to
er muscles as well as dystonic tremor can have longer duration extract the peak frequency and the total power in the frequen-
of EMG bursts. In psychogenic tremor, duration of EMG bursts cy range between 2 and 30 Hz.26 Tremors > 11 Hz are rare and
are often varying (Figure 1). However, this feature can also be are likely to be pathologic (e.g., in orthostatic tremor), tremor < 6
present in dystonic tremor. Hz are almost always pathologic.26
The characteristics of psychogenic tremor are given in Table
Coactivation sign 3. These include 1) usually a low frequency tremor which is < 11
This sign is often observed in patients with a psychogenic Hz, 2) varying frequency of tremor (Figure 2), 3) change of fre-
quency with distraction (usually on a mental-arithmetic task),
Table 2. Electrophysiologic analysis of psychogenic tremor 4) a dissipated frequency spectrum of tremor rather than a peak
A) Pattern of EMG bursts frequency, 5) positive entrainment of tremor (when asked to
a) Temporal relationships of the EMG bursts from the
antagonistic muscles (alternate or synchronous)
b) Duration of EMG bursts and their variability Rt. FCR
tasks, voluntary movements of other limb Figure 1. Tremorogram from the right hand of a patient with psy-
D) Amplitude
chogenic tremor of the right hand. Surface electromyographic re-
cording from the right flexor carpi radialis (Rt. FCR) and right exten-
a) Variable and of less diagnostic significance sor carpi radialis (Rt. ECR). The accelerometric recording from the
b) Change with peripheral loading wrist is shown in lowest trace. Note the marked variation in the du-
ration of EMG bursts recorded from Rt. ECR, which varied from 176.8
E) Coherence analysis ms (A) to 79.2 ms (B).
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Journal of Movement Disorders ▐ 2011;4:21-32
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Psychogenic Movement Disorders ▐ Pal PK
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Journal of Movement Disorders ▐ 2011;4:21-32
4.3 Hz 0.75
4.3 Hz
0.50
0.25
0.00
1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16
Frequency Hz Frequency Hz Frequency Hz
A B C
3.8 Hz 2.3 to 2.6 Hz 2.3 Hz
1 2 3 4 5 6 7 8 9 10 11 12 1 2 3 4 5 6 7 8 9 10 11 12 1 2 3 4 5 6 7 8 9 10 11 12
Frequency Hz Frequency Hz Frequency Hz
D E F
Figure 5. The figure demonstrates the phenomenon of coherence in a patient with psychogenic tremor of right hand. Simultaneous EMG
and accelerometry were performed on both hands. The right hand was at rest (A and D) and the patient did fast tapping (B) and later slow tap-
ping (E) on the table with the index finger of left hand. During fast tapping with left index finger, the tapping frequency as well as the right hand
tremor frequency had identical peaks at 4.3 Hz with harmonics at 8.6 to 8.7 Hz. Coherence was seen at 4.5 Hz to 5.2 Hz. During slow tapping
with left index finger (at around 2.3 to 2.6 Hz) the right hand tremor at rest was at 3.8 Hz with a harmonic at 7.8 Hz. The coherence between
the movements of the two sides was at 2.3 Hz. This coherence test proves the entrainment of the tremor of right hand with the tapping frequen-
cy of left hand, thereby suggesting a psychogenic tremor.
tools used to differentiate psychogenic from organic jerks. BP ous EMG is also recorded from the proximal muscles of the
likely reflects the cortical activity prior to voluntary movement same limb and also from muscles of the non-stimulated hand.
and thus represents movement preparation. It is a slow positive In addition, simultaneous median SSEP is recorded from the
potential beginning around one second prior to movement and scalp. In certain types of myoclonus, especially cortical reflex
maximum over the vertex31 and is measured using back-aver- myoclonus, a markedly enhanced long-latency reflex is usual-
aging epochs of EEG preceding the EMG accompanying spon- ly recorded from the thenar muscle at a latency of around 45 ms
taneous jerks. This can be compared to BP recorded from vol- after stimulation of the median nerve at the wrist,32 which cor-
untary, self-paced movements. For detecting such a potential responds to the C reflex named by Sutton and Mayer.33 In some
several trials need to be averaged (at least 40 trials) to improve patients, the enhanced C reflexes can also be recorded from
the signal-to-noise ratio, and it is unreliable if the movements more proximal muscles of the stimulated upper extremity with
occur more than every 2 seconds25 A jerk preceded by a BP is shorter latency and even from the opposite (nonstimulated)
likely to be of voluntary origin. hand muscle.
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Psychogenic Movement Disorders ▐ Pal PK
a cortical activity with the EMG burst. Three patterns of tem- an organic jerk, particularly when there is co-contraction of ag-
poral relationship can be observed: a spike or sharp wave 1) onist and antagonist muscle pairs.
precedes a jerk in cortical myoclonus by 20-40 ms depending A well organized triphasic pattern activation of agonist and
on whether the muscle under investigation is in the upper or antagonist muscle pairs, and a prolonged duration of EMG
lower limb,34 2) follows a jerk in brainstem or reticular myoc- burst favours psychogenic jerk. Spontaneous jerks preceded
lonus, or is 3) absent that may suggest spinal myoclonus. Most by a BP (premovement potential) is likely to be psychogenic,
organic jerks including majority of patients with tics do not but BP may be recorded in chorea of choreocacanthocytosis,38
have a BP prior to the movements.35 However a BP preceding and an abbreviated BP can be seen in tics39 and in patients with
the choreic movements may be observed in patients of choreo- myoclonus accompanying dystonia.40
acanthocytosis and some patients with tics and those with my- Stimulus sensitive psychogenic jerks, unlike organic jerks,
oclonus accompanying dystonia may have an abbreviated BP have varied latency of onset of jerks (muscle activity) from the
preceding the abnormal movements.25 causative stimulus (latency) from trial to trial. The latencies are
Stimulus-evoked jerks, such as that seen in stimulus-sensi- greater than seen in reflex myoclonus of cortical or brainstem
tive myoclonus of cortical origin have giant cortical SSEP, short origin or longer than the fastest voluntary reaction times of nor-
latency (usually < 100 ms, depending on the muscle analyzed), mal subjects.23 The mean latency of > 100 ms suggests volun-
short duration EMG bursts, and a characteristic descending tary or psychogenic jerks.25 Other features may include vari-
(craniocaudal) pattern of muscle recruitment.23 In pathological able patterns of muscle recruitment within each jerk and,
enhancement of startle response or hyperekplexia there is ste- significant habituation with repeated stimulation.23
reotyped non-habituating early response in sternocleidomastoid In psychogenic jerks mimicking generalized myoclonus, the
to sound or tap to the mantle region.23 response may reduce or stop after repeated stimuli, mimicking
In cortical reflex myoclonus, LLR is enhanced, with a C re- normal (physiological) startle response. The presence of giant
flex recorded from the thenar muscle at a latency of around 45 cortical somatosensory-evoked potentials, short EMG bursts,
ms after stimulation of the median nerve at the wrist32 (Figure and characteristic descending pattern of muscle recruitment
5B). In some patients, the enhanced C reflexes can be recorded point towards cortical myoclonus.23
also from more proximal muscles of the stimulated upper ex-
tremity with shorter latency and even from the opposite (non- Evaluation of psychogenic dystonia
stimulated) hand muscle.30 Compared to tremor and jerks, electrophysiological charac-
In reticular reflex myoclonus, the LLR is enhanced without terization of dystonia is difficult. The protocol of evaluation is
enhancement of cortical SSEP. The reflex myoclonus first in- similar, consisting of multichannel surface EMG from the sym-
volves bulbar muscles such as the sternocleidomastoid and tra- ptomatic muscles for prolonged periods during abnormal move-
pezius muscles, and subsequently the more rostral cranial mus- ments (spasms), rest, and with voluntary contraction. Effort
cles (such as the facial muscles) and caudal muscles (such as should be made to include the agonists and antagonists to de-
limb muscles) are involved.36 tect the presence of co-contraction, and the unaffected muscles
of the same or opposite limbs to document the presence of over-
Psychogenic jerks flow dystonia. Finally, the patients should be asked to perform
Electrophysiological evaluation is helpful to differentiate various tasks which can induce or modify the ongoing spasms,
psychogenic from organic jerks25 In psychogenic jerks a well such as mental arithmetic, finger tapping and opening and clos-
organized triphasic pattern of activation of agonist and antag- ing fist of the unaffected hand, writing (in patients with writer’s
onist muscles are common and the EMG bursts are of long du- cramp), etc.
ration (usually > 70 ms). Stimulus-evoked jerks or jumps with EMG is analyzed to determine: 1) the pattern of EMG activ-
a mean latency in excess of 100 ms suggest voluntary or psy- ity in agonists and antagonists during spasm 2) duration of each
chogenic jerks.25 A BP may precede psychogenic jerks.37 EMG burst 3) the regularity of occurrence of EMG bursts, 4)
The following findings point against a psychogenic jerk: presence of overflow activity in remote muscles while per-
1) giant cortical somatosensory evoked potentials, 2) short forming discrete voluntary acts, and, 5) the difference in the de-
duration EMG bursts, 3) characteristic descending patterns gree of EMG activity (area of rectified EMG) between epochs
of muscle recruitment (suggesting a cortical myoclonus), and with and without muscle spasms.
4) presence of LLR/C reflex. Other investigations which are often useful to characterize
Clinically it may be difficult to separate psychogenic jerks an organic dystonia or a dystonic syndrome include: 1) H-reflex
from tics and myoclonus. Differentiation of psychogenic jerks recovery curve, 2) mechanically and electrically induced long-
from organic jerks has been often based on duration of EMG latency muscle stretch reflex, 3) reciprocal inhibition between
activity of a burst, and the pattern of EMG burst (Table 3). antagonistic muscles, 4) brainstem reflexes such as blink reflex,
Jerks associated with EMG duration of < 70 ms is likely to be 5) cortical SEP 6) BP and contingent negative variation (CNV),
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Journal of Movement Disorders ▐ 2011;4:21-32
and 7) transcranial magnetic stimulation. er excitability of motor cortex in patients with primary dysto-
The following electrophysiological findings are often pres- nia, reorganization of cortical excitability in patients with writ-
ent, in varying combinations, in organic dystonia. Therefore, in er’s cramp, reduced short interval intracortical inhibition in
a given patient suspected of psychogenic dystonia, presence of patients with focal, task-specific primary dystonia when tested
any of the following should warrant a revision in diagnosis and at rest, and reduced EMG silent period and changes in long in-
search for underlying cause of dystonia. terval intracortical inhibition in patients with dystonia.58-60
Several electrophysiological abnormalities have been report-
Abnormalities of EMG and kinematic studies ed in dystonia, but the specificity and sensitivity of these find-
The abnormalities include 1) Co-contraction (often up to sev- ings are not enough to differentiate between organic and psy-
eral seconds) of agonist and antagonist muscle pairs during chogenic dystonia.51 In cervical dystonia EMG recording show-
dystonia. However, this feature is true for voluntary (psycho- ed a pathological common drive manifest as significant cohe-
genic) spasm and may not be of value, 2) repeated short bursts rence at 4-7 Hz band between sternocleidomastoid and splenius
of EMG activity superimposed on the prolonged spasms, which capitis.61
may result in superimposed action and postural tremors,41,42 Abnormalities in dystonic torticollis include abnormal low-
slow myorhythmia43 or myoclonic jerks,44 depending on the du- frequency common drive. A similar abnormality has been re-
ration and regularity of these jerks, 3) replacement of the nor- ported in the lower limbs of 10 out of 12 symptomatic pa-
mal di-or tri-phasic pattern of activation of agonists and antag- tients with DYT1 dystonia, but not psychogenic dystonia or
onists muscles during voluntary movements by prolonged bur- normal controls.62 Other abnormality in organic dystonia in-
sts with a resultant overlap of agonist and antagonist activities, clude impaired reciprocal inhibition of H-reflexes.63
4) the voluntary movements of the distal part of the limbs may
be accompanied by inappropriate activity of remote proximal Psychogenic parkinsonism
muscles,45 and 5) an abnormal synchronizing drive at certain Electrophysiological evaluation of psychogenic parkinson-
frequencies in dystonic muscles. ism is difficult and there is paucity of reports in the literature.
Electrophysiology can help in differentiating a true rest trem-
Abnormalities of spinal cord reflexes or from a psychogenic tremor by methods described previous-
These include abnormalities of H-reflex recovery curve,46 ly. Parkinson’s disease is characterized by altered cortical ex-
LLR,47 and breakdown of normal pattern of reciprocal inhibi- citability by TMS studies, which if present may support a
tion between opposing muscles.48 diagnosis of organic parkinsonism.
Zeuner et al.64 measured postural wrist tremor with accel-
Abnormalities of brainstem reflexes erometry in 6 patients with psychogenic tremor, 11 with es-
These include abnormalities of blink reflex and its recovery sential tremor and 12 with parkinsonian tremor. Tremor was
cycle in cranial dystonia49,50 as well as in cervical and general- measured in one hand, while the other hand either rested or
ized dystonia even without blepharospasm.51 tapped to an auditory stimulus at 3 and 4 or 5 Hz. The patients
with psychogenic tremors showed larger tremor frequency
Abnormalities of SSEPs changes and higher intraindividual variability while tapping.
The findings are controversial. Late components of SSEP to The authors concluded that accelerometry could be an useful
median nerve stimulation (N30) has been reported to be enlarged tool to differentiate psychogenic from essential and parkinso-
in writer’s cramp,52 but normal53 or reduced in size in spasmod- nian tremor.
ic torticollis without hand dystonia.54 Benaderette et al.65 evaluated the concordance between inde-
pendent clinical, electrophysiological, and [123I]-FP-CIT SPECT
Abnormalities of premovement potentials scan explorations as a staged procedure for an accurate diagno-
In primary as well as secondary dystonias abnormalities sis in 9 patients referred with a diagnosis of suspected psycho-
(mainly reduced amplitudes) of the initial slow and/or the later genic parkinsonism. Three patients were reclassified as pure
steep components of BP.55,56 The amplitude of the late compo- psychogenic parkinsonism, 6 with a form of combined psycho-
nent of CNV is reduced in torticollis when patients are asked to genic parkinsonism and Parkinson’s disease, and none with
rotate their head to either side depending on a signal, and in pure Parkinson’s disease (PD). Electrophysiological record-
patients with writer’s cramp while performing hand move- ings showed the characteristics of psychogenic tremor in 5 of 7
ments.57 patients with tremor. In two of these 5, PD tremor was also
recorded. SPECT scan results were abnormal in five of 9 pa-
Abnormalities of transcranial magnetic stimulation tients. The authors concluded that electrophysiology contrib-
A number of abnormalities have been described in dystonia, utes to the clinical diagnosis of psychogenic tremor and may
which include abnormal recruitment pattern, suggesting great- help confirm associated organic PD tremor.
30
Psychogenic Movement Disorders ▐ Pal PK
www.e-jmd.org 31
Journal of Movement Disorders ▐ 2011;4:21-32
Presence of Bereitschaftspotential preceding psychogenic myoclo- 54. Mazzini L, Zaccala M, Balzarini C. Abnormalities of somatosensory
nus: clinical application of jerk-locked back averaging. J Neurol evoked potentials in spasmodic torticollis. Mov Disord 1994;9:426-430.
Neurosurg Psychiatry 1995;58:745-747. 55. Fève A, Bathien N, Rondot P. Abnormal movement related potentials
38. Shibasaki H, Sakai T, Nishimura H, Sato Y, Goto I, Kuroiwa Y. Invol- in patients with lesions of basal ganglia and anterior thalamus. J Neurol
untary movements in chorea-acanthocytosis: a comparison with Hun- Neurosurg Psychiatry 1994;57:100-104.
tington’s chorea. Ann Neurol 1982;12:311-314. 56. Van der Kamp W, Rothwell JC, Thompson PD, Day BL, Marsden CD.
39. Karp BI, Porter S, Toro C, Hallett M. Simple motor tics may be preced- The movement-related cortical potential is abnormal in patients with
ed by a premotor potential. J Neurol Neurosurg Psychiatry 1996;61: idiopathic torsion dystonia. Mov Disord 1995;10:630-633.
103-106. 57. Kaji R, Ikeda A, Ikeda T, Kubori T, Mezaki T, Kohara N, et al. Physio-
40. Quinn NP, Rothwell JC, Thompson PD, Marsden CD. Hereditary myo- logical study of cervical dystonia. Task-specific abnormality in contin-
clonic dystonia, hereditary torsion dystonia and hereditary essential my- gent negative variation. Brain 1995;118:511-522.
oclonus: an area of confusion. Adv Neurol 1988;50:391-401. 58. Thompson ML, Thickbroom GW, Sacco P, Wilson SA, Stell R, Mast-
41. Yanagisawa N, Goto A. Dystonia musculorum deformans. Analysis aglia FL. Changes in the organisation of the corticomotor projection to
with electromyography. J Neurol Sci 1971;13:39-65. the hand in writer’s cramp [abstract]. Mov Disord 1996;11Suppl 1:219.
42. Jedynack CP, Bonnet AM, Agid Y. Tremor and idiopathic dystonia. 59. Ridding MC, Sheean G, Rothwell JC, Inzelberg R, Kujirai T. Changes
Mov Disord 1991;6:230-236. in the balance between motor cortical excitation and inhibition in focal,
43. Herz E. Dystonia. 1. Historical review: analysis of dystonic symptoms task specific dystonia. J Neurol Neurosurg Psychiatry 1995;59:493-498.
and physiologic mechanisms involved. Arch Neurol Psychiatry 1944; 60. Chen R, Wassermann EM, Caños M, Hallett M. Impaired inhibition in
51:305-318. writer’s cramp during voluntary muscle activation. Neurology 1997;49:
44. Obeso JA, Rothwell JC, Lang AE, Marsden CD. Myoclonic dystonia. 1054-1059.
Neurology 1983;33:825-830. 61. Tijssen MA, Marsden JF, Brown P. Frequency analysis of EMG activ-
45. van der Kamp W, Berardelli A, Rothwell JC, Thompson PD, Day BL, ity in patients with idiopathic torticollis. Brain 2000;123:677-686.
Marsden CD. Rapid elbow movements in patients with torsion dysto- 62. Grosse P, Edwards M, Tijssen MA, Schrag A, Lees AJ, Bhatia KP, et
nia. J Neurol Neurosurg Psychiatry 1989;52:1043-1049. al. Patterns of EMG-EMG coherence in limb dystonia. Mov Disord
46. Panizza M, Lelli S, Nilsson J, Hallett M. H-reflex recovery curve and 2004;19:758-769.
reciprocal inhibition of H-reflex in different kinds of dystonia. Neurol- 63. van de Beek WJ, Vein A, Hilgevoord AA, van Dijk JG, van Hilten BJ.
ogy 1990;40:824-828. Neurophysiologic aspects of patients with generalized or multifocal
47. Naumann M, Reiners K. Long-latency reflexes of hand muscles in id- tonic dystonia of reflex sympathetic dystrophy. J Clin Neurophysiol 2002;
iopathic focal dystonia and their modification by botulinum toxin. Brain 19:77-83.
1997;120:409-416. 64. Zeuner KE, Shoge RO, Goldstein SR, Dambrosia JM, Hallett M. Accel-
48. Chen RS, Tsai CH, Lu CS. Reciprocal inhibition in writer’s cramp. erometry to distinguish psychogenic from essential or parkinsonian
Mov Disord 1995;10:556-561. tremor. Neurology. 2003;61:548-550.
49. Berardelli A, Rothwell JC, Day BL, Marsden CD. Pathophysiology of 65. Benaderette S, Zanotti Fregonara P, Apartis E, Nguyen C, Trocello JM,
blepharospasm and oromandibular dystonia. Brain 1985;108:593-608. Remy P, et al. Psychogenic parkinsonism: a combination of clinical,
50. Tolosa E, Montserrat L, Bayes A. Blink reflex studies in focal dystoni- electrophysiological, and (123I)-FP-CIT SPECT scan explorations im-
as: enhanced excitability of brainstem interneurones in cranial dystonia proves diagnostic accuracy. Mov Disord 2006;21:310-317.
and spasmodic torticollis. Mov Disord 1988;3:61-69. 66. Raethjen J, Kopper F, Govindan RB, Volkmann J, Deuschl G. Two dif-
51. Berardelli A, Rothwell JC, Hallett M, Thompson PD, Manfredi M, ferent pathogenic mechanisms in psychogenic tremor. Neurology 2004;
Marsden CD. The pathophysiology of primary dystonia. Brain 1998;121: 63:812-815.
1195-1212. 67. Williams DR, Cowey M, Tuck K, Day B. Psychogenic propriospinal
52. Reilly JA, Hallett M, Cohen LG, Tarkka IM, Dang N. The N30 compo- myoclonus. Mov Disord 2008;23:1312-1313.
nent of somatosensory evoked potentials in patients with dystonia. Elec- 68. Kang SY, Sohn YH. Electromyography patterns of propriospinal my-
troencephalogr Clin Neurophysiol 1992;84:243-247. oclonus can be mimicked voluntarily. Mov Disord 2006;21:1241-1244.
53. Nardone A, Mazzini L, Zaccala M. Changes in EMG response to per- 69. van der Salm SM, Koelman JH, Henneke S, van Rootselaar AF, Tijssen
turbations and SEPs in a group of patients with idiopathic spasmodic MA. Axial jerks: a clinical spectrum ranging from propriospinal to psy-
torticollis [abstract]. Mov Disord 1992;7Suppl 1:25. chogenic myoclonus. J Neurol 2010;257:1349-1355.
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