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Handbook of Clinical Neurology, Vol.

139 (3rd series)


Functional Neurologic Disorders
M. Hallett, J. Stone, and A. Carson, Editors
http://dx.doi.org/10.1016/B978-0-12-801772-2.00017-5
2016 Elsevier B.V. All rights reserved

Chapter 17

Neurologic diagnostic criteria for functional neurologic disorders


C. GASCA-SALAS1, 2 AND A.E. LANG1*
1
Morton and Gloria Shulman Movement Disorders Clinic and the Edmond J. Safra Program in Parkinsons Disease,
University Health Network, Toronto Western Hospital, Toronto, Ontario, Canada
2
HM CINAC-Hospital Universitario HM Puerta del Sur, Mstoles, Universidad CEU San Pablo, Madrid, Spain

Abstract
The diagnosis of functional neurologic disorders can be challenging. In this chapter we review the diagnostic
criteria and rating scales reported for functional/psychogenic sensorimotor disturbances, psychogenic nonepi-
leptic seizures (PNES) and functional movement disorders (FMD). A recently published scale for sensorimotor
signs has some limitations, but may help in the diagnosis, and four motor and two sensory signs have been
reported as highly reliable. There is good evidence using eight specific signs for the differentiation of PNES
from seizures. Recently, diagnostic criteria were developed for PNES; their sensitivity and specificity need to be
evaluated. The definitive diagnosis of PNES can be made by recording typical positive features during the
spells, and in a low proportion of cases, where the distinction with an organic etiology cannot easily be done,
a normal electroencephalogram suggests the diagnosis. FMD diagnosis relies on diagnostic criteria, which have
been refined over time and may be supplemented by laboratory tests in some phenotypes. Rating scales for
PNES and FMD could be useful for severity measures, but several limitations remain to be addressed.

INTRODUCTION functional/psychogenic neurologic disorders, emphasiz-


ing sensory/motor disturbances, movement disorders,
Functional disorders are common in neurology; their
and nonepileptic events. Other symptoms, such as distur-
diagnosis is not always easy and is commonly based
bances in level of consciousness, cognitive dysfunction,
on the neurologists experience. Diagnosis should rely
visual loss, and speech, eye movement, and auditory
on the presence of positive signs and there must not be
abnormalities, are dealt with in other chapters.
a better explanation for the symptoms, as emphasized
in the recent published fifth edition of the Diagnostic
and Statistical Manual of Mental Disorders (DSM-5: FUNCTIONAL MOTOR, SENSORY,
Stone et al., 2011; American Psychiatric Association, AND GAIT DISORDERS
2013; Daum et al., 2015). For that purpose, we need signs
that are both sufficiently reliable in the detection of func- Reliable clinical signs and diagnostic criteria
tional disorders and reproducible by different examiners. Diagnostic criteria for functional disorders in neurology
Moreover, DSM-5 does not require the presence of psy- have been proposed in the fields of movement disorders
chologic stressors as criteria for a diagnosis. and epilepsy. However, no criteria exist for other neuro-
It is also important to take into account that patients logic presentations, namely weakness, sensory or gait
may have a combination of an organic disease with a disorders (Daum et al., 2014). Therefore, we need valid
functional overlay (Stone et al., 2012, 2013). positive signs suggestive of functional disorders to sup-
In this chapter we will describe the validity and port the diagnosis, as negative signs of neurologic dis-
reliability of clinical signs as well as the diagnostic ease do not exclude an organic etiology. More than
criteria and rating scales reported for different 50 positive signs have been identified for weakness,

*Correspondence to: Anthony E. Lang, MD, Movement Disorders Clinic, Toronto Western Hospital, 399 Bathurst St, 7 McL,
Toronto, Ontario M5T 2S8, Canada. Tel: +1-416-603-5112, Fax: +1-416-603-5004, E-mail: lang@uhnres.utoronto.ca
194 C. GASCA-SALAS AND A.E. LANG
sensory or gait disorders; however, not all have been studies with a minimum class III evidence level, and a
properly assessed and there is no gold standard against controlled design.
which to compare these tests. Because of this, many stud- Subsequently, the authors further attempted to validate
ies have used the sign of interest in the diagnostic pro- 10 signs previously suggested as valid and 28 previously
cess, leading to an overestimated reported specificity unvalidated positive signs (13 motor/sensory, 14 gait, and
(e.g., Tinazzi et al., 2008). This diagnostic suspicion one general sign) in a pilot study. The pilot study included
bias and the lack of blinding in most of these studies 20 functional patients and 20 patients with an organic neu-
reduce their validity generally, along with a number of rologic disorder as controls. For the first time, they calcu-
generic limitations in studies of physical signs in this area lated the inter-rater agreement of the positive signs in a
(Table 17.1). Keeping these issues in mind, we will controlled blind design (Daum et al., 2015). Twenty-three
describe the most common and assessed signs, since out of 38 signs had an acceptable inter-rater agreement.
some of them can still be regarded as helpful despite The sternocleidomastoid test (see Table 17.2 for expla-
the absence of good validation. nation) and the presence of falls that are always to-
In this regard, Daum et al. (2014) have recently wards support showed the highest inter-rater agreement
reported 14 clinically validated positive signs in a sys- (k Cohens 0.83). Six bedside positive signs that
tematic review; yet, none of these signs has been subject showed high specificity and good or excellent inter-rater
to really rigorous blind testing. Overall, these signs had agreement (k Cohens > 0.6) in their study were pro-
good specificity but low sensitivity. The authors included posed as highly reliable, whereas 13 signs with high
specificity and moderate to excellent inter-rater agreement
(k Cohens > 0.4) were considered as reliable signs
Table 17.1 (Tables 17.217.4). However, even though this study
Limitations of the methodology of studies assessing the was blinded, it is a single study with a low sample size.
validity of clinical signs for the diagnosis of functional Furthermore, the raters used videos instead of different
motor, sensory and gait disorders* clinicians examining the same patients, limiting the valid-
ity of inter-rater agreement assessment. Therefore, conclu-
1. No gold standard against which to compare these tests sions from this study should be interpreted with caution.
2. Diagnostic suspicion bias: many studies have used the
studied sign in the diagnostic process, leading to an
overestimated reported specificity
Positive signs for functional weakness,
3. Most studies have based their validation on a single sensory and gait disorders
evaluation SUGGESTED SIGNS AND THEIR RELIABILITY
4. Very few studies have been blinded (e.g., only Daum et al.,
2015) Weakness (Table 17.2)
5. Possibility for some signs to be found in organic patients
Fifteen motor signs have been reported as valid; however,
(e.g., give-way weakness: Gould et al., 1986, midline
splitting and splitting of vibration: Rolak, 1988; Stone et al., for many of them the data have been reported from only
2010) one study. Hoovers test has been considered the most use-
6. Potential for false positives: due the inability to understand ful test for nonorganic weakness (Stone et al., 2002). In
the instructions, the presence of pain compromising patient addition, in certain case-control studies describing this test
compliance or even patients eagerness to convince the the main aim was not to evaluate Hoovers sign (Sonoo,
doctor of their limitations. In all of these circumstances, 2004; Tinazzi et al., 2008; McWhirter et al., 2011). Other
patients with organic symptoms can demonstrate false- signs, such as the abductor sign, the abductor finger test,
positive functional features (e.g., Hoovers sign, yes/no test) drift without pronation, and the spinal injury test, have
7. Some studies did not specifically look at the reported deficit. shown high sensitivity and specificity, but the evidence
For instance, in midline splitting sign for sensory disorders,
comes from only one study each. Given their high speci-
only Rolak (1988) looked specifically at sensory deficit,
ficity and good or excellent inter-rater agreement, Daum
whereas in Stone et al. (2010) and Chabrol et al. (1995)
sensory deficits were not the main focus and coworkers (2015) proposed that give-way weakness,
8. Precise description of the clinical signs and interpretation of drift without pronation, and co-contraction are highly
the findings is not always provided (e.g., BowlusCurrier reliable signs. Hoovers sign was also highly reliable;
test, nonanatomic sensory loss) no inter-rater agreement was assessed, but it has a strong
9. Rating by videos instead of clinicians examining the same validation in several studies.
patients: limits the validity of inter-rater reliability
assessment Sensory disorders (Table 17.3)
*All these limitations could have introduced errors in the estimated Despite many claims and attempts to demonstrate
sensitivity and specificity. validity, sensory signs generally have poor specificity.
Table 17.2
Suggested positive signs for functional motor disorders

Inter-rater Comments (see


agreement Table 17.1 for
(k Cohens)* generic
(Daum et al., Reliability (Daum methodologic
Sign Description/assumption Sensitivity Specificity 2015) et al., 2015) issues) References

Hoovers sign Weakness of hip extension that resolves 63100% 86100% Highly reliable Ziv et al., 1998; Sonoo,
during contralateral hip flexion against 2004; Tinazzi et al., 2008;
resistance Stone et al., 2010;
McWhirter et al., 2011;
Daum et al., 2015
Abductor sign Weakness of hip abduction that resolves with 100% 100% No inter-rater Sonoo, 2004
contralateral hip abduction against agreement
resistance provided
information
Spinal injury center In patients unable to lift up their knees, legs 100% 1398% Moderate Reliable Test limitations: Yugue et al., 2004; Daum
test are passively lifted up in a flexed posture. (0.52) only suitable for et al., 2015
The paretic leg will not fall in nonorganic patients with
weakness bilateral leg
paralysis
Abduction finger test In healthy subjects, abduction finger 100% 100% Test limitations: it Tinazzi et al., 2008
movements of one hand against resistance can only be
for 2 minutes will display a synkinetic applied to
abduction of the fifth finger of the patients with
contralateral (nonorganic paretic) hand severe hand
paresis
Drift without Arms stretched out and palms in supinated 61100% 9395% Good (0.78) Highly reliable Only examined in Babinski, 1907; Daum and
pronation position. Downward drift without one study of Aybek, 2013; Daum
pronation was described as a sign of 26 patients et al., 2015
functional paresis
Collapsing weakness In nonorganic weakness a limb collapses 4470% 98100% Moderate Reliable Stone et al., 2010; Daum
from a normal position with a light touch (0.45) et al., 2015
Give-way weakness In functional paresis strength initially is 2085% 95100% Good (0.61) Highly reliable Gould et al., 1986; Rolak,
normal during testing and then suddenly (Daum et al., 1988; Daum et al., 2015
collapses 2015); highly
unreliable
(Gould et al.,
1986)

Continued
Table 17.2
Continued

Inter-rater Comments (see


agreement Table 17.1 for
(k Cohens)* generic
(Daum et al., Reliability (Daum methodologic
Sign Description/assumption Sensitivity Specificity 2015) et al., 2015) issues) References

Co-contraction sign Simultaneous contraction of the antagonist 1730% 100% Good (0.77) Highly reliable False positive in Knutsson and Martensson,
muscle (i.e., triceps) when the agonist spastic patients 1985; Baker and Silver,
muscle is being tested (biceps) (excessive 1987; Daum et al., 2015
antagonist
activation)
Motor inconsistency The impossibility to do a movement while 13% 98% Chabrol et al., 1995
another movement using the same muscle
is possible
Sternocleidomastoid Weakness of head turning to the affected side 3180% 89100% Excellent Reliable Diukova, 2001; Daum et al.,
test in a patient with functional hemiparesis. (0.83) 2015
(Sternomastoid has bilateral innervation)
Irregular drift Same maneuver as drift without pronation. 11% 95% Good (0.72) Suggestive Daum et al., 2015
In a nonorganic paresis the arm drifts
irregularly
Nonconcavity of the Same maneuver as drift without pronation. 89% 65% Good (0.65) Reliable Daum et al., 2015
palm of hand No concave/flexed position is observed in
functional paresis
Inconsistence of Same maneuver as drift without pronation. 39% 95% Moderate Reliable Daum et al., 2015
direction An oscillation of the arm is seen in (0.42)
functional paresis (downward, upward,
downward)
Non digiti quinti sign Same maneuver as drift without pronation. 0% 95% Moderate Suggestive Daum et al., 2015
In nonorganic paresis no abduction of the (0.48)
fifth finger is seen
Mingazzini: irregular Mingazzini maneuver: patient in supine 47% 95% Moderate Reliable Daum et al., 2015
drift position, legs are bent 90 in the knees, and (0.60)
hips and eyes are closed for 5 seconds. In
an organic paresis the leg drifts regularly
whereas it drifts irregularly in a functional
paresis

Data from Daum et al. (2014, 2015).


*Inter-rater agreement: poor (<0.20), fair (0.210.40), moderate (0.410.60), good (0.610.80), excellent (0.811).
Table 17.3
Suggested positive signs for functional sensory disorders

Inter-rater
agreement
(k Cohens)* Reliability in Daum Comments (see Table 17.1 for
Sign Description/assumption Sensitivity Specificity (Daum et al., 2015) et al. (2015) study generic methodologic issues) References

Midline Exact splitting of sensory loss 1842% 85100% Good (0.63) Highly reliable This sign can exist with Rolak, 1988; Chabrol et al.,
splitting of half of the body cannot thalamic lesions 1995; Stone et al., 2010;
occur in organic disease, Daum et al., 2015
except thalamic lesions
Splitting of There should not be differences 5095% 1488% Good (0.66) Highly reliable Gould et al., 1986; Rolak,
vibration in the sensation of a turning 1988; Stone et al., 2010;
sense fork placed over the left or Daum et al., 2015
right side of the sternum or
frontal bone as the same bone
is involved
Nonanatomic Sensory deficits with 7480% 90100% Fair (0.23) Reliable Gould et al., 1986; Baker
sensory nonanatomical distribution and Silver, 1987
loss
Inconsistency Findings are not consistent and 70% 100% Sensory inconsistency not Baker and Silver, 1987
not reproducible on repeated well defined in this small
sensory testing study. Parietal lesions can
produce inconsistences in
sensory testing (Magee,
1962; Critchley 1964)
Changing Changing boundaries of 46% 2052.5% Gould et al., 1986; Chabrol
pattern of sensory loss et al., 1995
sensory
loss
Systematic The test is considered positive 8.715% 100% Poor (0.16) Reliable Reported as general sign Baker and Silver, 1987;
failure when the subject fails 100% Daum et al., 2015
of the time on a
discrimination task (i.e.,
upgoing or downgoing joint)

*Inter-rater agreement: poor (<0.20), fair (0.210.40), moderate (0.410.60), good (0.610.80), excellent (0.811).
Table 17.4
Suggested positive signs for functional gait disorders and general sign

Inter-rater
agreement Comments (see
(k Cohens)* Reliability (Daum Table 17.1 for generic
Sign Description/assumption Sensitivity Specificity (Daum et al., 2015) et al., 2015) methodologic issues) References

Dragging In functional gait the leg is dragged 811% 100% Moderate (0.44) Reliable Ehrbar and Waespe, 1992;
monoplegic after the patient as it was Stone et al., 2010; Daum
leg inanimate matter without et al., 2015
circumduction seen in
pyramidal weakness and
typically with the forefoot in
contact with the ground at all
times (Todd, 1856)
Chair test Based on Blocqs (Blocq, 1888) 89% 100% No inter-rater reliability Okun et al., 2007
description. Patients with study
nonorganic astasia-abasia
despite apparently normal
power can propel a swivel chair
while sitting
Falls always The patient falls in the direction of 19% 93% Excellent (0.83) Suggestive Daum et al., 2015
towards the examiner or another support
support
Psychogenic Constant falls towards or away 39% 100% Moderate (0.54) Reliable In the study of Lempert Lempert et al., 1991; Daum
Romberg from the observer, large- et al. (1991), this sign et al., 2015
amplitude body sway after a was present in 12 out of
latency of a few seconds and 25 patients and none of
improvement with distraction 13 healthy drama
students simulators
Noneconomic A walking pattern that requires 21% 100% Moderate (0.53) Suggestive Lempert et al., 1991; Okun
posture waste of muscle energy in order et al., 2007; Daum et al.,
to maintain balance (for 2015
instance, standing and walking
with flexion of hips and knees)
Sudden knee Sudden knee buckling, usually 21% 95% Moderate (0.52) Suggestive Infrequent sign. It can be Keane, 1989; Lempert
buckling with no falls found in chorea (Daum et al., 1991; Baik and
et al., 2015) Lang, 2007; Okun et al.,
2007; Daum et al., 2015
Hesitation The beginning of the movement is 37% 100% Good (0.66) Reliable Organic freezing of gait or Lempert et al., 1991; Daum
delayed or not possible start hesitation could be et al., 2015
confused with this
Tremulousness Body tremor with up-and-down 16% 100% Good (0.64) Suggestive Lempert et al., 1991; Daum
shaking of the body (flexion/ et al., 2015
extension of the knees), not
compatible with an orthostatic
tremor
Bizarre Bizarre excursions of the trunk, 21% 100% Moderate (0.48) Reliable Lempert et al., 1991; Daum
excursion of often building up over a few et al., 2015
the trunk seconds; legs often unaffected
Excessive The slow motion is not consistent 94% 32% Moderate (0.47) Common in functional Lempert et al., 1991; Baik
slowness with an organic neurologic gait disorders. and Lang, 2007; Okun
disorder moderate inter-rater et al., 2007; Daum et al.,
agreement and high 2015
specificity (Daum et al.,
2015)
Expressive Suffering or strained facial 55% 95% Good (0.62) Reliable Lempert et al., 1991; Daum
behavior expression, moaning, mannered et al., 2015
(general posture of hands,
sign) hyperventilation or grasping of
the leg

*Inter-rater agreement: poor (<0.20), fair (0.210.40), moderate (0.410.60), good (0.610.80), excellent (0.811).
200 C. GASCA-SALAS AND A.E. LANG
Although six sensory signs have been reported as valid, common feature, and a second group with pure psycho-
other studies have found high rates in controls with genic gait, where buckling of the knee was the most
organic disease. For example, midline splitting and split- common pattern, followed by astasia-abasia.
ting of vibration showed a high inter-rater reliability in Eleven gait signs have been more carefully evaluated;
one study (Daum et al., 2015). However, this was based most have low sensitivity and high specificity. The
on a small sample size and other studies demonstrated chair test presented the highest sensitivity and specifi-
these features in fairly high numbers of controls with city (89% and 100% respectively), but was only evalu-
neurologic disease (Rolak, 1988; Stone et al., 2010). ated in a single study (Okun et al., 2007). Daum and
Only two of the signs, the nonanatomic pattern sign colleagues (2015) recently validated eight of these
and inconsistency, have demonstrated high sensitivity signs; however, none was considered as highly reliable
and specificity (Daum et al., 2014). and the chair test was not assessed. Lempert et al.
(1991) identified six positive signs related to functional
Gait disorders (Table 17.4) gait disorders (most were studied by Daum and col-
leagues but only in small numbers: momentary fluc-
In 1989, Keane assessed 60 patients with hysterical gait tuations of stance and gait, excessive slowness or
disorders and found that patterns rarely duplicated those hesitation, psychogenic Romberg test, uneconomic pos-
of neurologic disability and were promptly suspected as tures, walking on ice, and sudden buckling of the
functional by an experienced physician. Moreover, a dra- knees), that were present alone or in combination in
matic cure was the best diagnostic evidence. Jordbru and 97% of functional patients.
colleagues (2012) provided a valuable categorization of
psychogenic gait into three patterns limping of one
LESS INVESTIGATED SIGNS (TABLE 17.5)
leg, limping of two legs, and truncal imbalance and
the reliability between independent raters was high. Ten motor, two sensory, and ten gait signs have been
Baik and Lang (2007) found that 118 out of 279 reported but not yet been evaluated in detail. Daum
(42.3%) patients with functional movement disorder and coworkers (2015) aimed to validate some of
(FMD) had an abnormal gait. Here they differentiated these but, unfortunately, it was determined that further
two groups of patients: those with psychogenic move- validation was required due to the poor to fair inter-rater
ment symptoms unrelated to walking but combined with agreement or because they are uncommon signs in func-
an abnormal gait, where slowness of gait was the most tional patients.

Table 17.5
Less investigated positive signs for functional weakness, sensory and gait disorders

Description/assumption Comments References

Weakness
Nonpyramidal In organic lesions the weakness is Not helpful to differentiate a Freud, 1895; Koehler, 2003;
distribution greater distal > proximal and in peripheral lesion. Attempted Daum et al., 2015
of paresis flexor > extensor muscles. In validation by Daum et al. (2015).
functional paresis weakness is Fair inter-rater agreement (0.21)
approximately equally distributed in
all muscle groups
Arm drop Patient lying supine, the limb is held Attempted validation by Daum et al. Reeves and Bullen, 1994;
test/hand over the patients face and dropped by (2015); further validation required Greer et al., 2005; Marcus
strike the examiner. In an organic paresis, because it is a rare sign. It can only et al., 2010; Stone et al.,
the arm hits the patients face. In be applied in cases of complete 2010; Daum et al., 2015
functional paresis the arm regularly upper-limb paralysis
falls to the side, avoiding the face
Barre test Patient in prone position and legs bent at Daum et al. (2015). Poor inter-rater Barre, 1919; Daum et al., 2015
90 in the knees. In pyramidal agreement (0.03)
weakness, leg falls accompanied by
contraction of hamstring muscle. In
functional paresis, leg falls without
contraction of the hamstrings or is
maintained in the flexed position
NEUROLOGIC DIAGNOSTIC CRITERIA FOR FUNCTIONAL NEUROLOGIC DISORDERS 201
Table 17.5
Continued

Description/assumption Comments References

Wrong-way In organic hemiparesis a slight tongue Keane, 1986


tongue deviation towards the paresis can be
deviation seen. A strong deviation away from
the hemiparesis supports a functional
paresis
Platysma sign In organic paresis there is an asymmetry Expert opinion Babinski, 1900
of platysma contraction when opening
the mouth wide or when flexing the
chin towards the chest against the
examiners pressure. This asymmetry
is absent in functional cases
Babinski The patient in supine position and arms Expert opinion. Daum et al. (2015) Babinski, 1900; Daum et al.
trunk-thigh across chest, is asked to sit up. In attempted to validate; a poor inter- (2015)
test organic paresis, the weak limb raises rater agreement (0.18)
above the bed and the contralateral
shoulder makes a forward movement.
In functional cases, the patient cannot
sit or will sit but no asymmetry is seen
Supine catch Patient with a wrist drop is asked to put Reported in a case report of functional Sethi et al., 2010
sign the hand in supination. In organic wrist drop
cases, the hand is maintained in
neutral position with fingers flexed. In
functional paresis, the wrist
hyperextends with fingers extended
Mingazzini: Mingazzini maneuver (see Table 17.1). Attempted validation by Daum et al. Daum et al., 2015
drift without In organic paresis there is a hip and (2015). Fair inter-rater agreement
extension knee extension with the downward (0.27)
drift. In functional paresis only the
knee bends and drifts without an
extension movement of the hip
Drift against Patient in supine position, arm held at 45 Attempted validation by Daum et al. Daum et al. (2015)
gravity from the horizontal. In organic paresis (2015). Fair inter-rater agreement
arm drifts with the gravity. In functional (0.36)
cases arm raises against gravity
Elbow flex- Patient with unilateral arm weakness Lombardi et al., 2014
ex keeps elbows flexed at 30 and the
examiner holds both forearms near
the wrists. First part: Patient flexes or
extends the normal arm at the elbow;
in patients with nonorganic paresis
the examiner simultaneously feels
flexion or extension of the
contralateral (paretic) arm. In organic
paresis this contralateral movement is
not significantly detectable. Second
part: Patient flexes or extends the
paretic arm at the elbow. In
nonorganic weakness there is a
simultaneous poor strength of
extension of the normal limb. In
organic weakness patient displays
normal effort of the contralateral arm

Continued
202 C. GASCA-SALAS AND A.E. LANG
Table 17.5
Continued

Description/assumption Comments References

Sensory
Bowlus Palms together, wrist crossed with Single study of 36 patients that did not Bowlus and Currier, 1963
Currier test thumbs down, interlock fingers provide a precise interpretation of
(thumbs uncrossed), rotate hands, and the findings and whether it was
keep them in front of the chest. The independently tested
examiner starts touching on the fifth
finger up to the thumb (which is
uncrossed). In functional numbness,
the patient will report anesthesia to all
the fingers on that line, including the
thumb, even though it belongs to the
nonaffected side
Yes/no test Patient, with eyes closed, is asked to Implies trickery on the part of the Magee, 1962; Stone et al.,
state yes when appreciating a touch physician. The patient may think 2010; Daum et al., 2015
stimulus and no when the stimulus no means no, I dont feel it as
is not appreciated. A no response in much, so it can be confusing use
an anesthetic limb strongly suggests a with caution, is recommended.
functional deficit, because some Attempted validation by Daum
degree of touch perception is et al. (2015); further validation
preserved required because it is a rare sign
Gait
Fluctuation Gait disturbance with periods of normal May occur in neurologic disease, for Lempert et al., 1991; Okun
gait. Often in response to suggestion instance, myasthenia gravis et al., 2007
Walking Patient walking pattern mimics ice Attempted validation by Daum et al. Lempert et al., 1991; Daum
on ice skating or as if on slippery grounds (2015); further validation required et al., 2015
because it is a rare sign
Staggering Patient appears very unstable but doesnt Attempted validation by Daum et al. Keane, 1989; Daum et al.,
long fall, and will eventually find support, (2015); further validation required 2015
distance even if far out of reach because it is a rare sign
Exaggerated Similar to the psychogenic Romberg Keane, 1989
swaying sign (Table 17.3)
without
falling
Astasia-abasia Inability to stand and walk despite In thalamic astasia the patient is also Knapp, 1891; Blocq, 1888;
normal leg function in bed unable to stand Lempert et al., 1991; Baik
and Lang, 2007
Opposite of Inability to move legs in bed despite Ehrbar and Waespe, 1992
astasia- preserved capacity of stance and gait
abasia
Sudden side Patient with functional gait will display Attempted validation by Daum et al. Diukova and Stoliarova, 2001;
steps a big displacement in his trajectory (2015). Fair inter-rater agreement Daum et al., 2015
with sudden side steps, without (0.37)
falling
Cross legs Functional patient will walk with Keane, 1989; Diukova and
crossed legs or scissoring pattern Stoliarova, 2001
Flailing arms Exaggerated large-amplitude Attempted validation by Daum et al. Lempert et al., 1991; Daum
movements of the arms during (2015). Poor inter-rater agreement et al., 2015
walking apparently to maintain (0.03)
stability
Robot walk Robotic, stiff-legged, and square-cut Southard, 1919; Keane, 1989;
walk Daum et al., 2014

Inter-rater agreement: poor (< 0.20), fair (0.210.40), moderate (0.410.60), good (0.610.80), excellent (0.811).
NEUROLOGIC DIAGNOSTIC CRITERIA FOR FUNCTIONAL NEUROLOGIC DISORDERS 203
Rating scales and their reliability are signs with good evidence from literature that differen-
tiate PNES from seizures. An occurrence from EEG-
A sensorimotor scale for functional disorders was sug-
confirmed sleep and postictal confusion support an
gested by Daum and colleagues (2015) on the basis of
epileptic seizure. There is also good evidence from
their study. This scale was developed to help with the
primary studies that postictal stertorous breathing sup-
diagnosis and not as a severity scale. It combines
ports epileptic seizure, but only in the case of convulsive
six motor and four sensory validated positive signs.
events. Around 20% of nonepileptic events actually
This scale has a maximum score of 14 points. Two
resemble syncope more than epilepsy. Here, a sudden
points are attributed to signs with a robust validation
collapse to the ground with eyes closed for more than
(Hoovers sign, the give-way weakness sign, the drift
2 minutes is highly characteristic of a psychogenic
without pronation sign, and splitting the midline sign)
nonepileptic event.
and the remaining signs are given a score of 1 each (col-
In addition, there are other potentially useful clinical
lapsing weakness, co-contraction, spinal injury test,
signs that have been described. For example, ictal stutter-
splitting of vibration sense, nonanatomic sensory loss,
ing (Vossler et al., 2004) and the bringing of an age-
and systematic failure always choosing a wrong
inappropriate toy animal to the video-EEG monitoring
answer during sensory testing). A score of  4 showed
(teddy bear sign) (Burneo et al., 2003) were found in
100% specificity and 95% sensitivity, suggesting that
single studies to have a specificity of 100%, but they
this scale can help in differentiating between organic
are not frequent, leading to a low sensitivity (9% and
and functional sensorimotor disorders. However, these
5.2%, respectively).
results need to be interpreted with caution given the small
The presence of somatic symptoms of anxiety during
study sample size and the use of a video for rater evalu-
attacks seems to be more frequent in PNES compared to
ation, which limits the assessment of inter-rater agree-
epileptic seizures, and could help in the diagnosis
ment (Daum et al., 2015).
(Goldstein and Mellers, 2006). Hendrickson et al.
(2014) recently reported that the presence, whether
PSYCHOGENIC NONEPILEPTIC before, during, or after an attack, of at least four of the
SEIZURES 13 panic attack symptoms of anxiety of the Diagnostic
and Statistical Manual IV Text Revision (DSM-IV-
The most reliable diagnosis of psychogenic nonepi- TR: American Psychiatric Association, 2000) has a sen-
leptic seizures (PNES) is provided by the clinical pic- sitivity of 83% and specificity of 65%. By increasing the
ture with the presence of clinical features consistent number of these symptoms to five and six, the sensitiv-
with a psychogenic/dissociative nonepileptic event ity reduces, but the specificity improves. These symp-
(described below). In some cases where this distinction toms include: (1) palpitations, pounding heart, or
cannot be made easily (e.g., because the attack has not accelerated heart rate; (2) sweating; (3) trembling or
been witnessed), a video-electroencephalogram (EEG) shaking; (4) sensations of shortness of breath; (5) feeling
recording of typical events will confirm the lack of of choking; (6) chest pain or discomfort; (7) nausea
electrographic changes and allow clinical features to or abdominal distress; (8) feeling dizzy, unsteady,
be recorded (Syed et al., 2011). When trying to reach lightheaded, or faint; (9) derealization or depersonaliza-
the diagnosis of PNES, it is important to consider tion; (10) fear of losing control or going crazy; (11) fear
that 10% of patients with PNES also have seizures of dying; (12) paresthesias; and (13) chills or hot
(Benbadis et al., 2001) and, in these cases, clinical flushes.
and laboratory criteria should be applied (Drazkowski Finally, there are six relatively common signs
and Chung, 2010). with insufficient evidence to support their useful-
ness: gradual onset, nonstereotyped movements, flail-
ing or thrashing movements, opisthotonus, tongue
Reliable clinical signs
biting, and urinary incontinence (Avbersek and Sisodiya,
Clinical signs that reliably distinguish between PNES 2010).
and seizures have been extensively evaluated The final diagnosis of PNES will require all available
(Avbersek and Sisodiya, 2010) (Table 17.6). Selected data and should not be led by only one clinical sign
motor signs have been reported in at least two controlled (Avbersek and Sisodiya, 2010). As mentioned above,
studies where a video-EEG was used to diagnose the all these signs are useful in the diagnosis of PNES, but
events. Long duration, fluctuating course, asynchronous the gold standard for the diagnosis is video-EEG moni-
movements, pelvic thrusting, side-to-side head or body toring and recording of typical positive features during
movement, closed eyes, ictal crying, and memory recall the episodes.
204 C. GASCA-SALAS AND A.E. LANG
Table 17.6
Clinical signs differentiating between epileptic seizure (ES) and nonepileptic events (NEE)/psychogenic nonepileptic
seizures (PNES)

Sensitivity Sensitivity Specificity Specificity References from controlled


per event per patient per event per patient Comments studies

Signs supporting NEE/PNES


Fluctuating 69% 4788% 96% 96100% Vinton et al., 2004; Chen
course et al., 2008
Asynchronous 4496% 956% 9396% 93100% Limitation: frontal-lobe Gates et al., 1985; Azar et al.,
movements partial seizures are 2008; Chen et al., 2008
excluded
Pelvic trusting 131% 7.444% 96100% 92100% Limitation: frontal-lobe Gates et al., 1985; Saygi et al.,
partial seizures are 1992; Devinsky et al.,
excluded 1996; Geyer et al., 2000;
Azar et al., 2008; Chen
et al., 2008
Side-to-side 2563% 1536% 96100% 92100% Only applied for generalized Gates et al., 1985; Saygi et al.,
head or body tonic clonic seizures 1992; Pierelli et al., 1989;
movement Azar et al., 2008; Chen
et al., 2008
Closed eyes 3488% 5296% 74100% 97% DeToledo and Ramsay, 1996;
Chung et al., 2006; Azar
et al., 2008; Chen et al.,
2008; Syed et al., 2008
Ictal crying 1314% 3.737% 100% 37% Slater et al., 1995; Devinsky
et al., 1996; Walczak and
Bogolioubov, 1996; Chen
et al., 2008
Memory recall 63% 7788% 96% 90% Bell et al., 1998; Devinsky
et al., 1996
Long duration Events longer than 2 minutes Gates et al., 1985; Pierelli
are highly suggestive of et al., 1989; Brown et al.,
NEE/PNES. However, 1991; Saygi et al., 1992;
partial ES may also last Henry and Drury, 1998;
more than 2 minutes Jedrzejczak et al., 1999;
Azar et al., 2008
Signs supporting ES
Occurrence 3159% 100% Electroencephalogram is Pierelli et al., 1989; Saygi
from sleep required to verify et al., 1992; Azar et al.,
wakefulness 2008
Postictal 61100% 67% 88% 84% Azar et al., 2008; Slater et al.,
confusion 1995
Postictal 6191% 100% Partial seizures are excluded Sen et al., 2007; Azar et al.,
stertorous 2008; Chen et al., 2008
breathing

Modified from Avbersek and Sisodiya (2010), with permission from BMJ Publishing Group Ltd.

Diagnostic criteria
et al., 1999). Specifically, a prospective study showed
For the diagnosis of PNES, video-EEG monitoring has that epileptic seizures were misdiagnosed as PNES more
demonstrated a moderate inter-rater agreement frequently than the reverse (57% vs. 12%) (Parra et al.,
(k 0.57) (Benbadis et al., 2009). In the absence of 1999). Moreover, epileptic seizures of frontomesial origin
video-EEG recording, the diagnosis of PNES can be unre- are often misdiagnosed as PNES, since they can semiolog-
liable, with resulting false-positive diagnoses (Ramani ically mimic them and often fail to display an identifiable
et al., 1980; King et al., 1982; Gates et al., 1985; Parra electrographic ictal pattern (Saygi et al., 1992).
NEUROLOGIC DIAGNOSTIC CRITERIA FOR FUNCTIONAL NEUROLOGIC DISORDERS 205
Table 17.7
Proposed diagnostic levels of certainty for non-epileptic events (NEE)/ psychogenic nonepileptic seizures (PNES)

Diagnostic level Witnessed event EEG findings Comments

Possible NEE/PNES Self-reported and/or witness No epileptiform activity (routine EEG or


description of the events sleep-deprived interictal EEG)
Probable NEE/PNES Physician witnessed the event or No epileptiform activity (routine EEG or Home video recording
reviewed a video showing sleep-deprived interictal EEG) does not usually include
semiologic findings of PNES the beginning of the
event
Clinically established Clinician experienced in No epileptiform activity (routine EEG or
NEE/PNES epilepsy reviewed the video ambulatory ictal EEG) during a typical
or witnessed the event, event in which the semiology would
typical of PNES expect epileptiform EEG activity during
equivalent epileptic seizures
Documented NEE/ Clinician experienced in No epileptiform activity immediately The recorded event on the
PNES epilepsy reviewed the video before, during, and after the event EEG has to be typical of
or witnessed the event, captured on ictal video EEG the patients habitual
typical of PNES ones

Reproduced from LaFrance et al. (2013), with permission from John Wiley.
EEG, electroencephalogram.

Recently, using different combinations of patient scale may be a valid assessment of PNES, it has several
history, witness description, clinician observation, limitations, such as how the severity or the time of the
and EEG findings, diagnostic criteria have been devel- episode should be judged or the evaluation of only one
oped for PNES. These four categories of certainty event per patient that prevents the assessment of the con-
have been reported: possible, probable, clinically sistency or stereotyped nature of the events. In addition,
established, and documented PNES (LaFrance some of the associated features support a diagnosis
et al., 2013). All levels need history characteristics con- (but are nonspecific for PNES, i.e., crying), while others
sistent with PNES (Table 17.7). However, these are such as sphincteric incontinence are not specific for epi-
proposed criteria and their sensitivity and specificity lepsy. Finally, this test evaluated responsiveness during
have not yet been evaluated. atonic/akinetic fits only, and not when other motor phe-
nomena (i.e., tremor/oscillation, hypermotor/agitation,
Rating scales and their reliability automatisms) were present, and so consciousness was
not properly assessed.
Inspired by the Psychogenic Movement Disorders Scale
(Hinson et al., 2005), Cianci et al. (2011) aimed to
develop a rating scale for PNES (Table 17.8). For this FUNCTIONAL MOVEMENT DISORDERS
purpose, 60 PNES patients were included; they had no
Diagnostic criteria and their degree
epileptiform activity (ictal or interictal) and no postictal
of certainty
slowing. The diagnosis of PNES was confirmed by sug-
gestion, which has shown a high sensitivity and specific- When dealing with FMDs or psychogenic movement
ity (Popkirov et al., 2015). This scale showed a good disorders, the clinical picture should guide the diagnosis.
inter-rater reliability (measured by AC1 statistic, this ran- Although no absolutely pathognomonic findings exist,
ged from 0.69 to 1 for the presence or absence of the there are important unequivocal clinical features, dis-
motor phenomena and associated features). There was cussed in the following chapters (e.g., inconsistency
a moderate inter-rater agreement for the three scores of and/or incongruity in the exam), that serve as critical cues
this scale (Kendalls concordance coefficients (KCC) to the diagnosis and are applicable to all types of move-
0.530.71 and intraclass correlation coefficient (ICC) ment disorders. (Table 17.9 provides a list of the historic
0.510.56). The results from this scale were compared and general examination clues to the diagnosis.) In addi-
to the Clinical Global Impression (CGI) scale, a nonspe- tion to these features, there is a new sign that could be
cific scale, to test the validity. There was a strong corre- helpful in the diagnosis of any kind of hyperkinetic
lation (Spearman correlation score 0.69) between the FMD, the whack-a-mole sign. This sign is character-
mean CGI and the mean total PNES score. Although this ized by the development of an abnormal movement in
Table 17.8
Rating scale for psychogenic nonepileptic seizures

Motor phenomena Scale factors

Tremor/oscillation Presence of motor phenomena Severity Duration

Tonic 0 absent 0 none 0 none


Clonic/jerking 1 present 1 minimal 1 < 25% of the time
Hypermotor/agitation 2 mild 2 2550% of the time
Atonic/akinetic 3 moderate 3 5075% of the time
Automatisms 4 severe 4 > 75% of the time

Body parts considered: upper face, lips/perioral, jaw, neck, head, left shoulder, right shoulder, left upper extremity, right upper extremity, left lower
extremity, right lower extremity, pelvis, trunk.

Associated features Presence of associated features

Incontinence 0 absent
Tongue biting 1 present
Drooling
Eye closure
Hyperventilation
Lament/crying

Modified from Cianci et al. (2011).


There are three scores for this scale: (1) the total scores for phenomena, calculated as the sum of the severity and duration rating of all phenomena
considering all body regions affected; (2) the total scores for associated phenomena, calculated as the sum of the scores for the presence of the
different signs; and (3) the total psychogenic nonepileptic seizure score, calculated as the addition of the total phenomenology score and the total
associated phenomena score.

Table 17.9
Clues suggesting a functional/psychogenic cause of a movement disorder

Historic General examination

Abrupt onset (symptoms often maximal at that time) Movement inconsistent


Static course Variability over time (frequency, amplitude, direction/distribution of
Spontaneous remissions/cures movement)
Paroxysmal symptoms (generally nonkinesigenic)* Distractibility reduces or resolves, attention increases movement
Psychiatric comorbidities Selective disability
Secondary gain (often not apparent) Entrainment (especially with tremor)
Risk factors for conversion disorder (sexual and Movement incongruous with organic movement disorders
physical abuse, trauma) Mixed (often bizarre) movement disorders
Psychological stressors Paroxysmal attacks (including pseudoseizures)
Multiple somatizations/undiagnosed conditions Precipitated paroxysms (often suggestible/startle)
Employed in allied health professions (infrequent) Suggestibility
Effortful production or deliberate slowness (without fatiguing)
of movement
Self-inflicted injury (caution: tic disorders)
Delayed and excessive startle response to a stimulus
Burst of verbal gibberish or stuttering speech
False (give-away) weakness
Nonanatomic sensory loss or spread of movement
Certain types of abnormal movements common in individuals with
functional movement disorders{
Functional disability out of proportion to examination findings

Data from Gupta and Lang (2009).


*Separation from organic paroxysmal dyskinesias can be challenging, particularly if they occur infrequently with prolonged symptom-free periods.

Psychiatric diseases can also coincide with organic illness or present as part of the organic movement disorder.
{
Such movements include dystonia that begins as a fixed posture (particularly if abrupt onset, painful, and early contractures are seen); bizarre gait;
twisting facial movements that move mouth to one side or the other (organic dystonia of the facial muscles usually does not pull the mouth sidewise).
NEUROLOGIC DIAGNOSTIC CRITERIA FOR FUNCTIONAL NEUROLOGIC DISORDERS 207
another limb when the affected limbs movement is Secondary criteria included multiple somatizations and
restricted by the examiner holding it (Park et al., 2015). obvious psychiatric disturbance. These criteria had a
Unlike the field of epilepsy, where a normal EEG can high sensitivity (83%) and specificity (100%) for the iden-
be useful in the diagnosis of PNES in specific cases, in tification of probable FMD. Sensitivity of this scale was
FMD the laboratory is only of help in providing positive higher (97%) when considering possible, with a specific-
diagnostic information in cases of tremor and myoclonus. ity of 96% (Shill and Gerber, 2006).
The diagnosis of FMD should not be considered a However, this study had several limitations. One
diagnosis of exclusion. Instead, it should rely on positive was the emphasis placed on the presence of psycho-
signs and other features for which laboratory findings logic factors which are not part of the FMD diagnosis
may help (Espay et al., 2009; Gupta and Lang, 2009). and that others have argued should not influence the
In 1988, Fahn and Williams first proposed criteria for diagnosis (Espay et al., 2009). In this study these features
the diagnosis of psychogenic dystonia, that can be applied enhanced the sensitivity and specificity of the criteria.
to all movement disorders. They categorized patients into There were also important methodologic concerns, such
four levels of certainty: documented, clinically as the retrospective design, the lack of provided evidence
established, probable, and possible (Table 17.10). that all FMD patients and controls were evaluated for all
Later, Williams et al. (1994) proposed the combination the features that were eventually used as diagnostic cri-
of documented and clinically established degrees as teria (creating diagnostic suspicion bias), and finally,
clinically definite, since both may imply a definite the fact that Shill and Gerber suggested that the diagnosis
diagnosis. of FMD can be made without consideration of the neu-
Shill and Gerber proposed criteria in 2006. They rologic symptoms, given the emphasis placed on the
defined the following levels of certainty: clinically proven presence of psychologic factors. These concerns make
FMD when it remits with psychotherapy, while unob- the application of their criteria less useful (Voon
served, or if there is premovement Bereitschaftspotential et al., 2007).
on EEG (myoclonus only). According to the presence of A subsequent study attempted to assess diagnostic
primary and secondary criteria, they defined diagnoses agreement in 14 clinicians who provided a dichotomous
as clinically definite, clinically probable, and clinically judgment (psychogenic or organic) following review of a
possible. Primary criteria included factors suggesting a video and standardized clinical information (Morgante
movement disorder inconsistent with organic disease et al., 2012). Both sets of clinical criteria (Fahn and
(i.e., distractibility), excessive pain or fatigue, and previous Williams, 1988; Shill and Gerber, 2006) showed poor
exposure to neurologic disease (usually family history). inter-rater agreement when considering the possible

Table 17.10
Levels of certainty for functional movement disorders

Fahn and Williams (1988) criteria Proposed revision (Gupta and Lang, 2009)

1. Documented 1. Documented (as in original)


Remittance with suggestion, physiotherapy, psychotherapy,
placebos, while unobserved
2. Clinically established 2a. Clinically established plus other features (as in original)
Inconsistent over time/incongruent with clinical condition 2b. Clinically established minus other features: unequivocal
+ other manifestations: other false signs, multiple features incompatible with organic disease with no features
somatizations, obvious psychiatric disturbance suggesting another underlying neurologic or psychiatric
problem
3. Probable 1 + 2a + 2b Clinically definite
a. Inconsistent/incongruent with no other features
b. Consistent/congruent + false neurological signs 3. Laboratory-supported definite
c. Consistent/congruent + multiple somatizations Electrophysiologic evidence proving a psychogenic movement
disorder (primarily in cases of tremor and myoclonus)
4. Possible
Consistent/congruent + obvious emotional disturbance

Data from Gupta and Lang (2009).


208 C. GASCA-SALAS AND A.E. LANG
and probable judgment, but they yielded a substantial specificity (both 73%), followed by distraction with
agreement when considering the clinically definite serial 7 s, with a sensitivity of 58% and specificity of
level, suggesting that only this level is useful for the diag- 84% (Kenney et al., 2007). Suggestibility with a tuning
nosis of FMD (Morgante et al., 2012). fork also seemed to be a good predictor for functional
In contrast, another recent study evaluating 29 videos tremor; it showed a high specificity (88%) but a relatively
of movement disorders on YouTube showed a high low sensitivity (42%). On the other hand, entrainment
inter-rater agreement (inter-rater reliability coefficient seemed to be less predictive for the diagnosis of func-
0.89) as well as a high level of certainty (4.33 0.60 tional tremor (Kenney et al., 2007).
86.6%) on the part of seven experts who independently With respect to laboratory tests, one study found
reviewed them. However, there was no independent con- that the diagnosis of functional tremor required a combi-
firmation of the diagnosis in these cases (Stamelou nation of several tests to reach adequate sensitivity and
et al., 2011). specificity, since no single measure was sufficiently reli-
The utility of laboratory testing in support of the clin- able to differentiate it from an organic tremor
ical diagnosis (Morgante et al., 2012) has led to subse- (Schwingenschuh et al., 2011). The following tests were
quent modifications adding the idea that the diagnosis included in the study: (1) incorrect tapping performance
of FMD can be laboratory-supported (adding a at 1 Hz, 3 Hz, and 5 Hz (1 point each); (2) entrainment,
laboratory-supported definite category to the diagnos- suppression, or pathologic frequency shift at 1 Hz,
tic criteria in a fashion similar to the field of multiple 3 Hz, and 5 Hz (1 point each); (3) pause or 50% reduction
sclerosis) (Gupta and Lang, 2009). In particular, this in amplitude of tremor with contralateral ballistic move-
category applies to electrophysiologic tests capable of ments (1 point); (4) tonic coactivation before tremor onset
supporting the diagnosis of functional/psychogenic (1 point); (5) coherence of bilateral tremors (1 point); and
tremor and functional/psychogenic myoclonus. (6) increase of total power (as surrogate of tremor ampli-
Gupta and Lang proposed a further revision of the tude) with 500-g weight loading (1 point). Attributing a
Fahn and Williams criteria. They noted the possibility score to every laboratory measure, the authors devised a
of establishing a diagnosis of FMD with only the clinical cut-off of 3 out of 10 points for a diagnosis of
findings (clinically established minus other features; laboratory-supported functional tremor which had sensi-
i.e., other false signs, multiple somatic symptoms, tivity and specificity of 100% in their sample of 13 patients
other psychiatric disturbances, required by Fahn and with functional tremor and 25 patients with organic
Williams criteria, are not present) and proposed elimi- tremor. This tool was recently validated by the same group
nating the possible category because it may represent in a larger sample (38 patients with functional tremor and
an organic movement disorder with superimposed psy- 73 with organic tremor), showing good inter-rater reliabil-
chiatric symptoms (Table 17.10) (Schrag and Lang, 2005). ity, testretest reliability, and very high sensitivity (89.5%)
Some studies have focused on the diagnosis of specific and specificity (95.9%) (Schwingenschuh et al., 2016).
FMDs. Van der Salm and colleagues (2013) designed a sur- More recently, van der Salm and colleagues (2014)
vey to assess selected functional hyperkinetic movement found that 104 out of the 179 cases (58%) of propriosp-
disorders (psychogenic jerks, myoclonus, or tics). When inal myoclonus in the literature were actually functional,
the diagnostic steps applied by experienced movement dis- concluding that an FMD is far more frequent than previ-
orders physicians included a short video, medical history, ously assumed. Based on their clinical experience and
neurologic exam, and neurophysiologic information, the review of literature, they proposed specific diagnostic
agreement was moderate (k value 5.6  0.1) and the criteria for propriospinal myoclonus and suggested three
diagnostic certainty was relatively high (3.5  1.2) (where categories: idiopathic, secondary, and functional. The
1 very uncertain and 5 absolutely certain). Impor- proposed criteria for functional propriospinal were: (1)
tantly, when psychiatric evaluation was added, it did not clinical clues (for instance, previous somatizations);
increase either the agreement or the certainty. (2) coexistence of facial movements or vocalizations
(they dont concur with a spinal origin); (3) normal imag-
ing of the spinal axis with no evidence of myelopathy;
Diagnostic criteria focused on the different
and (4) presence of Bereitschaftspotential or inconsistent
types of movement disorders
electromyogram pattern. However, the latter can be
Functional tremor is the most frequent presentation of a absent or not recordable and caution is recommended
FMD (in at least 50% of cases) (Factor et al., 1995; Cubo (van der Salm et al., 2014).
et al., 2005). The reliability of some measures for the Two recent studies proposed hints for the diagnosis of
diagnosis of functional tremor was investigated in order functional tics, a rarely reported phenotype (i.e., adult
to distinguish it from essential tremor. The tapping task onset, inability to suppress the movements, lack of pre-
for distraction reached the highest sensitivity and monitory sensations). However, given the small sample
NEUROLOGIC DIAGNOSTIC CRITERIA FOR FUNCTIONAL NEUROLOGIC DISORDERS 209
size reported in both studies (9 patients in the first and Table 17.11
11 in the second), retrospective analysis, and no Rating scale for psychogenic movement disorders*
Bereitschaftspotential assessment to support a functional
origin, these clues should be interpreted with caution and Part 1:
confirmed in larger studies (Baizabal-Carvallo and Phenomena Part 2: Functions Part 3: Total scores
Jankovic, 2014; Demartini et al., 2015).
Likewise, a retrospective study explored the typical Rest tremor Gait Total phenomenology
Action Speech score
clinical characteristics of functional (psychogenic) parox-
tremor Total function score
ysmal movement disorders based on 26 cases. The authors
Dystonia Total psychogenic
found that, even though the phenotypic presentation can Chorea movement disorder
be highly diverse, 11 characteristics help in distinguishing Bradykinesia score (1 + 2)
this condition from the three classic forms of primary par- Myoclonus
oxysmal dyskinesias (paroxysmal kinesigenic dyskinesia, Cerebellar
paroxysmal nonkinesigenic dyskinesia, and paroxysmal Ballism
exercise-induced dyskinesia): (1) an adult age of onset; Athetosis
Tics
(2) the presence of paroxysmal tremor; (3) high within-
Severity Duration Incapacitation
subject phenomenologic variability; (4) marked increases 0 none 0 none 0 none
in attack frequency and severity during examination; 1 minimal 1 < 25% of the time 1 minimal
(5) highly variable attack duration; (6) numerous and 2 mild 2 2550% of the time 2 mild
unusual triggers; (7) alteration of responsiveness during 3 moderate 3 5075% of the time 3 moderate
attacks; (8) odd precipitating factors; (9) odd relieving 4 severe 4 > 75% of the time 4 severe
maneuvers; (10) medically unexplained somatic symp-
Modified from Hinson et al. (2005), with permission from John
toms; and (11) atypical response to medication. However,
Wiley.
the reliability of these features has not been evaluated, *Retaining the terminology of the original report.
since no control group with organic paroxysmal dyskine-
Body parts considered: upper face, lips/perioral, jaw, tongue,
sias was included (Ganos et al., 2014). neck, head, left shoulder, right shoulder, left upper extremity, right
Regarding functional chorea, there are not specific upper extremity, left lower extremity, right lower extremity, trunk,
criteria, probably because of the very low frequency of other region
this phenotype (Thomas and Jankovic, 2004).

This scale showed an excellent inter-rater reliability for


Rating scales for severity of functional
the presence or absence of each phenomenon (k range
movement disorders
0.630.86) and also showed a high rate of agreement
To date, only one scale has been developed with the spe- when measuring total phenomenology (ICC 0.87,
cific intention of rating the severity of FMD (Hinson KCC 0.91), function (ICC 0.89, KCC 0.92) and
et al., 2005). It includes 10 phenomena (rest tremor, total score (ICC 0.88, KCC 0.93). It also demonstrated
action tremor, dystonia, chorea, bradykinesia, myoclo- the ability to capture changes due to a therapeutic interven-
nus, cerebellar incoordination, ballism, athetosis, and tion. Moreover, the mean total score showed a high corre-
tics), anatomic distribution, severity, and duration, along lation with the mean CGI (Pearson correlation 0.79),
with two functions (gait and speech), incapacitation due supporting its validity as a measure of severity.
to the abnormal movement/function, and total severity On the other hand, this scale is problematic as it
score (Table 17.11). combines all movement disorders rather than assessing
Each phenomenon is scored as absent or present. isolated movement disorders. This results in long evalu-
When it is present, the severity of each phenomenon is ation times and also the potential for overrating and
scored from 0 (none) to 4 (severe), the duration factor underrating the severity of the disorder (e.g., a patient
is rated from 0 (none) to 4 (>75% of the time), and who has only tremors of moderate severity, present
the incapacitation ranges from 0 (none) to 4 (severe). 75% of the time, with moderate incapacitation would
This scale includes three scores: (1) the total phenom- score less than a patient with more than three different
enology score: the sum of the severity, duration, and types of movement disorders but each with mild severity
incapacitation rating of all phenomena in affected and a duration of less than 25% of the time and minimal
regions; (2) the total function score: the sum of the scores incapacitation). Besides, there is no other gold standard
of duration and incapacitation rating for gait and/or to compare the scale to. Thus, simpler scales are war-
speech; and (3) the total Psychogenic Movement Disor- ranted, permitting the evaluation of single movement
der score is obtained from the addition of 1 and 2. disorder phenomena.
210 C. GASCA-SALAS AND A.E. LANG
CONCLUDING REMARKS Babinski J (1907). De la pronation de la main dans
lhemiplegie organique. Rev Neurol (Paris) 15: 755.
In summary, functional neurologic disorders are common Baik JS, Lang AE (2007). Gait abnormalities in psychogenic
in clinical practice, and diagnosis should rely on the pres- movement disorders. Mov Disord 22 (3): 395399.
ence of positive signs. Clinical criteria have been proposed Baizabal-Carvallo JF, Jankovic J (2014). The clinical features
for PNES and FMD, whereas to date, no criteria exist for of psychogenic movement disorders resembling tics.
other neurologic presentations, namely weakness, sensory J Neurol Neurosurg Psychiatry 85 (5): 573575.
or gait disorders. On the other hand, there are a significant Baker JH, Silver JR (1987). Hysterical paraplegia. J Neurol
number of clinical signs that can be helpful in the diagno- Neurosurg Psychiatry 50 (4): 375382.
sis of these clinical presentations, but with some limita- Barre J (1919). La manoeuvre de la jambe; Nouveau signe
objectif des paralysies ou paresis dues aux perturbations
tions that reduce their general validity.
du faisceau pyramidal. Presse Med 79: 793795.
In addition, although numerous signs have been val- Bell WL, Park YD, Thompson EA et al. (1998). Ictal cognitive
idated for the diagnosis of PNES, the final diagnosis in assessment of partial seizures and pseudoseizures. Arch
generalized shaking events is provided by the gold- Neurol 55 (11): 14561459.
standard video-EEG monitoring during the episodes Benbadis SR, Agrawal V, Tatum 4th WO (2001). How many
where the diagnosis relies on assessing positive signs patients with psychogenic nonepileptic seizures also have
captured during video as much as the negative EEG. In epilepsy? Neurology 57 (5): 915917.
FMD, no gold standard exists; therefore, diagnosis relies Benbadis SR, LaFrance Jr WC, Papandonatos GD et al. (2009).
on diagnostic criteria, which have been refined over time Interrater reliability of EEG-video monitoring. Neurology
and which may be supplemented by laboratory findings 73 (11): 843846.
Blocq P (1888). Sur une affection caracterisee par de lastasie
in selected phenotypes. The phenomenology of FMD is
et dabasie, Vol. 43. Progrs Medical, Paris.
broad; however, surprisingly, when each movement is
Bowlus WE, Currier RD (1963). A test for hysterical hemia-
studied in isolation (e.g., tremor, dystonia), clinical cri- nalgesia. N Engl J Med 269: 12531254.
teria have only been proposed for functional tremor, pro- Brown MCL, Ramsay BE, Katz E et al. (1991). Characteristics
priospinal myoclonus, tics, and paroxysmal dyskinesias. of patients with nonepileptic seizures. J Epilepsy 4:
The Fahn and Williams criteria were initially proposed 225229.
for the diagnosis of functional/psychogenic dystonia but Burneo JG, Martin R, Powell T et al. (2003). Teddy bears: an
were subsequently applied to all FMDs. observational finding in patients with non-epileptic events.
The scales for PNES and FMD may be useful as sever- Neurology 61 (5): 714715.
ity measures, but still have some limitations. The recently Chabrol H, Peresson G, Clanet M (1995). Lack of specificity of
proposed sensorimotor scale showed a high specificity the traditional criteria for conversion disorders. Eur
Psychiatry 10 (6): 317319.
for a score of only 4 out of 14 points, but was only tested
Chen DK, Graber KD, Anderson CT et al. (2008). Sensitivity
in one study. Future studies designed to validate these
and specificity of video alone versus electroencephalogra-
scales in other cohorts with larger samples are needed. phy alone for the diagnosis of partial seizures. Epilepsy
Furthermore, future studies should also assess which Behav 13 (1): 115118.
signs should be routinely evaluated as part of shorter, Chung SS, Gerber P, Kirlin KA (2006). Ictal eye closure is a
more concise, and easily applicable scales. reliable indicator for psychogenic nonepileptic seizures.
Neurology 66 (11): 17301731.
Cianci V, Ferlazzo E, Condino F et al. (2011). Rating scale for
REFERENCES psychogenic nonepileptic seizures: scale development and
clinimetric testing. Epilepsy Behav 21 (2): 128131.
American Psychiatric Association (2000). Diagnostic and sta- Critchley M (1964). Psychiatric symptoms and parietal dis-
tistical manual of mental disorders (4th edn., text revision). ease: differential diagnosis. Proc R Soc Med 57: 422428.
American Psychiatric Association, Washington, DC. Cubo E, Hinson VK, Goetz CG et al. (2005). Transcultural
American Psychiatric Association (2013). Diagnostic and sta- comparison of psychogenic movement disorders. Mov
tistical manual of mental disorders. 5th edn. American Disord 20 (10): 13431345.
Psychiatric Association, Washington, DC. Daum C, Aybek S (2013). Validity of the Drift without
Avbersek A, Sisodiya S (2010). Does the primary literature pronation sign in conversion disorder. BMC Neurol 13: 31.
provide support for clinical signs used to distinguish psy- Daum C, Hubschmid M, Aybek S (2014). The value of posi-
chogenic nonepileptic seizures from epileptic seizures? tive clinical signs for weakness, sensory and gait disorders
J Neurol Neurosurg Psychiatry 81 (7): 719725. in conversion disorder: a systematic and narrative review.
Azar NJ, Tayah TF, Wang L et al. (2008). Postictal breathing J Neurol Neurosurg Psychiatry 85 (2): 180190.
pattern distinguishes epileptic from nonepileptic convul- Daum C, Gheorghita F, Spatola M et al. (2015). Interobserver
sive seizures. Epilepsia 49 (1): 132137. agreement and validity of bedside positive signs for func-
Babinski J (1900). Diagnostic differentiel de lhemiplegie tional weakness, sensory and gait disorders in conversion
organique et de lhemiplegie hysterique. Gazette des disorder: a pilot study. J Neurol Neurosurg Psychiatry 86:
H^opitaux de Paris 73: 533537. 425430.
NEUROLOGIC DIAGNOSTIC CRITERIA FOR FUNCTIONAL NEUROLOGIC DISORDERS 211
Demartini B, Ricciardi L, Parees I et al. (2015). A positive epileptiform EEG abnormalities. Epilepsia 39 (2):
diagnosis of functional (psychogenic) tics. Eur J Neurol 175182.
22527e36. Hinson VK, Cubo E, Comella CL et al. (2005). Rating scale
DeToledo JC, Ramsay RE (1996). Patterns of involvement of for psychogenic movement disorders: scale development and
facial muscles during epileptic and nonepileptic events: clinimetric testing. Mov Disord 20 (12): 15921597.
review of 654 events. Neurology 47 (3): 621625. Jedrzejczak J, Owczarek K, Majkowski J (1999). Psychogenic
Devinsky O, Sanchez-Villasenor F, Vazquez B et al. (1996). pseudoepileptic seizures: clinical and electroencephalo-
Clinical profile of patients with epileptic and nonepileptic gram (EEG) video-tape recordings. Eur J Neurol 6 (4):
seizures. Neurology 46 (6): 15301533. 473479.
Diukova G (2001). Sternocleidomastoid (SCM) muscle test in Jordbru AA, Smedstad LM, Moen VP et al. (2012). Identifying
patients with hysterical and organic paresis. J Neurol Sci patterns of psychogenic gait by video-recording. J Rehabil
187 (Suppl 1): S109. (PO312). Med 44 (1): 3135.
Diukova GM, Stoliarova AV (2001). Psychogenic disorders of Keane JR (1986). Wrong-way deviation of the tongue with
stance and gait as seen in videotaping. Zh Nevrol Psikhiatr hysterical hemiparesis. Neurology 36 (10): 14061407.
Im S S Korsakova 101 (12): 1318. Keane JR (1989). Hysterical gait disorders: 60 cases.
Drazkowski JF, Chung SS (2010). Differential diagnosis of Neurology 39 (4): 586589.
epilepsy. Continuum (Minneap Minn) 16 (3 Epilepsy): Kenney C, Diamond A, Mejia N et al. (2007). Distinguishing
3656. psychogenic and essential tremor. J Neurol Sci 263 (1-2):
Ehrbar R, Waespe W (1992). Functional gait disorders. 9499.
Schweiz Med Wochenschr 122 (22): 833841. King DW, Gallagher BB, Murvin AJ et al. (1982).
Espay AJ, Goldenhar LM, Voon V et al. (2009). Opinions and Pseudoseizures: diagnostic evaluation. Neurology 32 (1):
clinical practices related to diagnosing and managing 1823.
patients with psychogenic movement disorders: an interna- Knapp P (1891). Astasia-abasia. J Nerv and Ment Dis XVII
tional survey of movement disorder society members. Mov 673701.
Disord 24 (9): 13661374. Knutsson E, Martensson A (1985). Isokinetic measurements of
Factor SA, Podskalny GD, Molho ES (1995). Psychogenic muscle strength in hysterical paresis. Electroencephalogr
movement disorders: frequency, clinical profile, and Clin Neurophysiol 61 (5): 370374.
characteristics. J Neurol Neurosurg Psychiatry 59 (4): Koehler PJ (2003). Freuds comparative study of hysterical
406412. and organic paralyses: how Charcots assignment turned
Fahn S, Williams DT (1988). Psychogenic dystonia. Adv out. Arch Neurol 60 (11): 16461650.
Neurol 50: 431455. LaFrance Jr WC, Baker GA, Duncan R et al. (2013). Minimum
Freud S (1895). Studies in hysteria. Hogarth Press, London. requirements for the diagnosis of psychogenic nonepileptic
Ganos C, Aguirregomozcorta M, Batla A et al. (2014). seizures: a staged approach: a report from the International
Psychogenic paroxysmal movement disorders clinical League Against Epilepsy Nonepileptic Seizures Task
features and diagnostic clues. Parkinsonism Relat Disord Force. Epilepsia 54 (11): 20052018.
20 (1): 4146. Lempert T, Brandt T, Dieterich M et al. (1991). How to iden-
Gates JR, Ramani V, Whalen S et al. (1985). Ictal characteris- tify psychogenic disorders of stance and gait. A video study
tics of pseudoseizures. Arch Neurol 42 (12): 11831187. in 37 patients. J Neurol 238 (3): 140146.
Geyer JD, Payne TA, Drury I (2000). The value of pelvic Lombardi TL, Barton E, Wang J et al. (2014). The elbow flex-
thrusting in the diagnosis of seizures and pseudoseizures. ex: a new sign to detect unilateral upper extremity non-
Neurology 54 (1): 227229. organic paresis. J Neurol Neurosurg Psychiatry 85
Goldstein LH, Mellers JD (2006). Ictal symptoms of anxiety, (2): 165167.
avoidance behaviour, and dissociation in patients with dis- Magee KR (1962). Hysterical hemiplegia and hemianesthesia.
sociative seizures. J Neurol Neurosurg Psychiatry 77 (5): Postgrad Med 31: 339345.
616621. Marcus H, Aldam P, Lennox G et al. (2010). Medically
Gould R, Miller BL, Goldberg MA et al. (1986). The validity of unexplained neurological symptoms. JRSM Short Rep 1
hysterical signs and symptoms. J Nerv Ment Dis 174 (10): (3): 25.
593597. McWhirter L, Stone J, Sandercock P et al. (2011). Hoovers
Greer S, Chambliss L, Mackler L et al. (2005). Clinical inqui- sign for the diagnosis of functional weakness: a prospective
ries. What physical exam techniques are useful to detect unblinded cohort study in patients with suspected stroke.
malingering? J Fam Pract 54 (8): 719722. J Psychosom Res 71 (6): 384386.
Gupta A, Lang AE (2009). Psychogenic movement disorders. Morgante F, Edwards MJ, Espay AJ et al. (2012). Diagnostic
Curr Opin Neurol 22 (4): 430436. agreement in patients with psychogenic movement disor-
Hendrickson R, Popescu A, Dixit R et al. (2014). Panic attack ders. Mov Disord 27 (4): 548552.
symptoms differentiate patients with epilepsy from those Okun MS, Rodriguez RL, Foote KD et al. (2007). The chair
with psychogenic nonepileptic spells (PNES). Epilepsy test to aid in the diagnosis of psychogenic gait disorders.
Behav 37: 210214. Neurologist 13 (2): 8791.
Henry TR, Drury I (1998). Ictal behaviors during nonepileptic Park JE, Maurer CW, Hallett M (2015). The Whack-a-mole
seizures differ in patients with temporal lobe interictal sign in functional movement disorders. Mov Disord Clin
epileptiform EEG activity and patients without interictal Pract 2: 286288.
212 C. GASCA-SALAS AND A.E. LANG
Parra J, Iriarte J, Kanner AM (1999). Are we overusing the Stone J, LaFrance Jr WC, Brown R et al. (2011). Conversion
diagnosis of psychogenic non-epileptic events? Seizure disorder: current problems and potential solutions for
8 (4): 223227. DSM-5. J Psychosom Res 71 (6): 369376.
Pierelli F, Chatrian GE, Erdly WW et al. (1989). Long-term Stone J, Carson A, Duncan R et al. (2012). Which neurolo-
EEG-video-audio monitoring: detection of partial epileptic gical diseases are most likely to be associated with symp-
seizures and psychogenic episodes by 24-hour EEG record toms unexplained by organic disease. J Neurol 259 (1):
review. Epilepsia 30 (5): 513523. 3338.
Popkirov S, Gronheit W, Wellmer J (2015). A systematic Stone J, Reuber M, Carson A (2013). Functional symptoms in
review of suggestive seizure induction for the diagnosis neurology: mimics and chameleons. Pract Neurol 13 (2):
of psychogenic nonepileptic seizures. Seizure 31: 124132. 104113.
Ramani SV, Quesney LF, Olson D et al. (1980). Diagnosis of Syed TU, Arozullah AM, Suciu GP et al. (2008). Do observer
hysterical seizures in epileptic patients. Am J Psychiatry and self-reports of ictal eye closure predict psychogenic
137 (6): 705709. nonepileptic seizures? Epilepsia 49 (5): 898904.
Reeves RR, Bullen JA (1994). Misuse of the face-hand test Syed TU, LaFrance Jr WC, Kahriman ES et al. (2011). Can
for psychogenic neurologic deficits. J Clin Psychiatry 55 semiology predict psychogenic nonepileptic seizures?
(8): 363. A prospective study. Ann Neurol 69 (6): 9971004.
Rolak LA (1988). Psychogenic sensory loss. J Nerv Ment Dis Thomas M, Jankovic J (2004). Psychogenic movement
176 (11): 686687. disorders: diagnosis and management. CNS Drugs 18 (7):
Saygi S, Katz A, Marks DA et al. (1992). Frontal lobe 437452.
partial seizures and psychogenic seizures: comparison of clin- Tinazzi M, Simonetto S, Franco L et al. (2008). Abduction
ical and ictal characteristics. Neurology 42 (7): 12741277. finger sign: a new sign to detect unilateral functional
Schrag A, Lang AE (2005). Psychogenic movement disorders. paralysis of the upper limb. Mov Disord 23 (16):
Curr Opin Neurol 18 (4): 399404. 24152419.
Schwingenschuh P, Katschnig P, Seiler S et al. (2011). Moving Todd RB (1856). Clinical lectures on paralysis, certain
toward laboratory-supported criteria for psychogenic diseases of the brain, and other affections of the nervous
tremor. Mov Disord 26 (14): 25092515. system. 2nd edn. Lecture 1, London. 20.
Schwingenschuh P, Saifee TA, Katschnig-Winter P et al. van der Salm SM, de Haan RJ, Cath DC et al. (2013). The eye
(2016). Validation of laboratory-supported criteria for of the beholder: inter-rater agreement among experts on
functional (psychogenic tremor). Mov Disord 31 (4): psychogenic jerky movement disorders. J Neurol
555562. Neurosurg Psychiatry 84 (7): 742747.
Sen A, Scott C, Sisodiya SM (2007). Stertorous breathing is a van der Salm SM, Erro R, Cordivan C et al. (2014).
reliably identified sign that helps in the differentiation of Propriospinal myoclonus: Clinical reappraisal and review
epileptic from psychogenic non-epileptic convulsions: an of literature. Neurology 83 (20): 18621870.
audit. Epilepsy Res 77 (1): 6264. Vinton A, Carino J, Vogrin S et al. (2004). Convulsive
Sethi NK, Sethi PK, Torgovnick J (2010). Supine catch sign a nonepileptic seizures have a characteristic pattern of
simple clinical test to differentiate between true and rhythmic artifact distinguishing them from convulsive
false (pseudo) radial nerve palsy. Clin Neurol Neurosurg epileptic seizures. Epilepsia 45 (11): 13441350.
112 (5): 441442. Voon V, Lang AE, Hallett M (2007). Diagnosing
Shill H, Gerber P (2006). Evaluation of clinical diagnostic psychogenic movement disorders which criteria should
criteria for psychogenic movement disorders. Mov be used in clinical practice? Nat Clin Pract Neurol 3 (3):
Disord 21 (8): 11631168. 134135.
Slater JD, Brown MC, Jacobs W et al. (1995). Induction of Vossler DG, Haltiner AM, Schepp SK et al. (2004). Ictal
pseudoseizures with intravenous saline placebo. Epilepsia stuttering: a sign suggestive of psychogenic nonepileptic
36 (6): 580585. seizures. Neurology 63 (3): 516519.
Sonoo M (2004). Abductor sign: a reliable new sign to detect Walczak TS, Bogolioubov A (1996). Weeping during psycho-
unilateral non-organic paresis of the lower limb. J Neurol genic nonepileptic seizures. Epilepsia 37 (2): 208210.
Neurosurg Psychiatry 75 (1): 121125. Williams DT, Ford B, Fahn S (1994). Phenomenology
Southard E (1919). Shell-shock and other neuropsychiatric and psychopathology related to psychogenic movement
problems. WM Leonard, Boston. disorders. In: WJ Weiner, AE Lang (Eds.), Behavioural
Stamelou M, Edwards MJ, Espay AJ et al. (2011). Movement neurology in movement disorders. Raven Press, New
disorders on YouTube caveat spectator. N Engl J Med York, pp. 231257.
365 (12): 11601161. Yugue I, Shiba K, Ueta T et al. (2004). A new clinical evalu-
Stone J, Zeman A, Sharpe M (2002). Functional weakness and ation for hysterical paralysis. (Phila Pa 1976) 29 (17):
sensory disturbance. J Neurol Neurosurg Psychiatry 73 (3): 19101913. discussion 1913.
241245. Ziv I, Djaldetti R, Zoldan Y et al. (1998). Diagnosis of non-
Stone J, Warlow C, Sharpe M (2010). The symptom of func- organic limb paresis by a novel objective motor assess-
tional weakness: a controlled study of 107 patients. Brain ment: the quantitative Hoovers test. J Neurol 245 (12):
133 (Pt 5): 15371551. 797802.