Professional Documents
Culture Documents
Functional Neurological Disorder
Functional Neurological Disorder
often considered elusive, since they have traditionally been tion techniques and signs that aim to differentiate functional
seen as diagnoses of exclusion, characterized by psychiatric from pathophysiological weakness and numbness have been
described since early work on "hysteria." Those signs are
comorbidities such as anxiety, neuroticism, or traumatization.
passed on through clinical mentoring and are dutifully collated
However, the diagnostic criteria for functional neurological
in literature reviews, but only a few of them are sufficiently
disorder have fundamentally changed with the last revision
specific and reliable to contribute to the differential diagnosis
of the Diagnostic and Statistical Manual of Mental Disorders,
(Table 1). Many signs are not proven to be helpful (or are
replacing what was a principle of exclusion with a phenotype-
proven to be unhelpful) and should thus be explicitly discour-
based diagnosis supported by specific clinical signs that dem-
aged for emergency decision-making, as they lead to misdiag-
onstrate inconsistency, reversibility, and top-down modulation
nosis (Table 2).
of symptoms.8 As the utility of examination techniques and
In functional limb weakness, the patient’s motor deficit is
clinical signs to identify functional weakness, numbness, and chiefly determined by an abnormal (computational) prediction
speech disorders are increasingly established, it is now timely of weakness that can be encoded at various levels of the motor
to provide a state-of-the-art review, tailored to the needs of the control pathway.19 The presentation is thus determined by
clinician working in acute stroke care. attention and expectation, with voluntary movements affected
more so than automatic ones. Most bedside tests aim to tease
Functional Limb Weakness out this difference and demonstrate the inconsistency between
and Sensory Deficits voluntary and involuntary movements. However, in stroke-
Presentations with limb weakness and sensory disturbances related paresis, movement can also be modulated by attention
comprise about 70% of all functional stroke mimics.1 Like or emotion, harboring the potential for false positives. It is
Received January 14, 2020; final revision received March 4, 2020; accepted March 13, 2020.
From the Department of Neurology, University Hospital Knappschaftskrankenhaus Bochum, Ruhr University Bochum, Germany (S.P.);
Wissenschaftskolleg zu Berlin–Institute for Advanced Study, Germany (S.P., A.M.B.); Centre for Clinical Brain Sciences, University of Edinburgh,
Royal Infirmary of Edinburgh, United Kingdom (J.S.); and Acute Stroke Programme, Radcliffe Department of Medicine, University of Oxford, United
Kingdom (A.M.B.).
Correspondence to Stoyan Popkirov, MD, Universitätsklinikum Knappschaftskrankenhaus Bochum, In der Schornau 23-25, 44892 Bochum, Germany.
Email popkirov@gmail.com
(Stroke. 2020;51:1629-1635. DOI: 10.1161/STROKEAHA.120.029076.)
© 2020 American Heart Association, Inc.
Stroke is available at https://www.ahajournals.org/journal/str DOI: 10.1161/STROKEAHA.120.029076
1629
1630 Stroke May 2020
Table 1. Examination Techniques for the Diagnosis of Functional Stroke Mimics With Motor Symptoms
thus advisable to seek confirmation from several clearly posi- A pattern of global or inverse pyramidal weakness is com-
tive signs before reaching a final diagnosis. mon in functional limb weakness instead of the usual pattern
Give-way weakness describes the sudden loss of tone or of stronger flexors in the arms and extensors in the legs. In
strength during isometric muscle strength testing. It has good studies of patients with a variety of upper and generalized
interrater reliability, as well as excellent specificity and pos- lower motor neuron weakness (eg, Guillain-Barré syndrome),
itive predictive value for functional limb weakness.16,18 In this pattern is consistently found. Achieving global or inverse
patients with leg weakness, it can manifest during gait assess- pyramidal weakness requires a process that preferentially
Downloaded from http://ahajournals.org by on January 9, 2024
ment as knee buckling. An important caveat is that it can occur affects the strongest voluntary muscles, in keeping with a dis-
due to joint or limb pain, especially in patients with preexisting order of willed movement.
conditions. Moreover, patients might want to help out during For functional leg weakness, Hoover's sign has the best
assessment or demonstrate their symptom to the examiner. clinical utility.16 It can be conducted when the patient is
seated or lying down (Figure) and reveals a normalization of
Table 2. Examination Techniques Not Suited for Acute Stroke Workup hip extension on the weak side during hip flexion of the non-
Due to Insufficient Specificity, Reliability, Evidence or Practicability (See paretic lower limb. In a prospective study of 124 patients
References16–18 for Descriptions) with suspected stroke and leg weakness, Hoover's sign was
Abduction finger sign positive in 5 of 8 functional stroke mimics and in none of
the definite stroke cases, yielding a sensitivity of 63% (CI,
Arm drop test
24%–91%) and specificity of 100% (CI, 97%–100%).20 The
Atypical distribution of weakness positive predictive value was 100% (CI, 46%–100%); the
Thigh trunk test negative predictive value was 99% (CI, 96%–100%). This
prospective study found no false positives, but those defi-
Bowlus-Currier test
nitely occur in acute stroke, particularly in supplementary
Co-contraction sign motor area lesions,21,22 or in cases of so-called pseudopare-
Forced choice/systematic failure sis due to parietal lobe lesions.23
La belle indifférence The hip abductor sign is similar to Hoover's sign and
describes weakness of voluntary hip abduction, which returns,
Midline splitting of sensory loss
through automatic movements, to normal during contralateral
Monrad-Krohn test hip abduction against resistance24 (Figure). In a study of 33
Non digiti quinti sign patients with lateralized leg weakness (16 functional and 17
organic, of whom 8 with stroke), this sign achieved 100% sen-
Nonanatomic distribution of sensory symptoms
sitivity and specificity.24
Pseudo-waxy flexibility Downward drift with pronation indicates cortical weak-
Sensitivity to suggestion ness. Drift without pronation is a typical finding in patients
Splitting of vibration sense with functional arm weakness (Figure). In a study of 26
patients with functional arm weakness and 28 controls with
Sternocleidomastoid test
organic paresis (23 due to stroke), drift without pronation
Popkirov et al Functional Neurological Disorder Mimicking Stroke 1631
Downloaded from http://ahajournals.org by on January 9, 2024
Figure. Positive clinical signs of functional neurological disorder. A, Hoover's sign: right hip extension is weak but normalizes during contralateral flexion
(gray and white arrows indicate examiner’s and patient’s active movements, respectively; striped arrows indicate patient’s automatically/involuntarily gener-
ated movement in the affected limb). B, Hip abductor sign: right hip abduction is weak but normalizes during contralateral abduction (arrows same as above).
C, Drift without pronation in functional arm weakness, with pronation in stroke. D, Unilateral lip pulling with ipsilateral platysma contraction in functional facial
dystonia. With permission from the person pictured.
identified functional arm weakness with a sensitivity of 100% low interrater reliability,34,35 and most diagnostic signs that
(CI, 84%–100%) and a specificity of 93% (CI, 76%–98%).25 have been suggested for identifying functional sensory distur-
Functional motor disorders of the face are generally easy bance do not have the required specificity. For example, non-
to distinguish from facial symptoms of stroke. The unilat- anatomic distributions of acute sensory symptoms, such as a
eral lip-pulling sign (Figure) reveals a characteristic func- glove-like distribution, are found in patients with distal arm
tional dystonic movement disorder of the face that may give paresis due to cortical stroke.36 Another proposed feature of
a superficial appearance of weakness but is actually caused functional hemisensory disturbance is so-called midline split-
by overactivity, typically of platysma or the muscles of jaw ting. Since cutaneous nerves of the trunk typically overlap
deviation.26 Ipsilateral orbicularis oculis contraction may be a couple of centimeters at the midline, an exact splitting of
an accompanying feature. Other facial or axial signs include deficits is often attributed to a functional disorder. However,
the trunk-thigh test,27 which has a low interrater reliability,18 midline splitting can be found in patients with clear organic
and the wrong-way tongue deviation away from paretic side28 causes of sensory loss,37–39 particularly in pure sensory stroke,
(toward hemiparesis in most strokes29), which remains to be which can remain magnetic resonance imaging (MRI) neg-
evaluated systematically and can be false positive in medullary ative.40 Other proposed signs for functional sensory symp-
infarctions.30 The proposed sternocleidomastoid muscle test, in toms, such as splitting of vibration sense (vibration is felt less
which head rotation is more likely to be weak toward the side on the numb side of the forehead or sternum despite intact
of hemiparesis in patients with functional paresis,31 is prob- bone conduction), similarly lack specificity for use in stroke
lematic as it is documented as a common finding in stroke.32,33 workup.38,39 It should be noted that these clinical signs may
In most cases, functional limb weakness is accompanied show better specificity when combined with the above men-
by sensory deficits. However, sensory testing in general has tioned motor signs.16
1632 Stroke May 2020
distinguishing features of functional stuttering is its quick and were retrospectively determined to have had functional symp-
complete response to therapy, though that will not be available toms.57 True DWI-negative stroke cases all corresponded to la-
in the emergency setting. cunar or posterior circulation stroke syndromes such as ataxic
Dysarthria (difficulty with articulation) is a common hemiparesis or isolated internuclear ophthalmoplegia.57 Thus,
symptom of stroke but occurs in isolation in only 1.3% to a DWI-negative stroke that does not correspond to a known
2.8% of cases.46,47 In cases of moderate-to-severe isolated stroke syndrome58 should prompt specific reexamination re-
dysarthria, the patient will always report some degree of dys- garding functional neurological deficits.
phagia as well, since the motor components of speaking and In summary, neuroimaging can only support but not prove
swallowing overlap. In functional dysarthria, patients might the diagnosis of functional neurological disorder, and it can
complain of a globus sensation but will rarely have any other detect an acute infarction but cannot exclude it with absolute
swallowing difficulties. Functional dysarthria is only rarely an certainty. Although it cannot thus establish the diagnosis of a
isolated presenting symptom, so diagnosis can usually be de- functional stroke mimic on its own, it can tip the scales suffi-
termined by its variability and by close examination of accom- ciently to guide treatment.
panying symptoms.
An inability to produce language, aphasia, is another com- Deciding on a Diagnosis
mon presenting sign of stroke, usually in the context of clearly The diagnostic decision of acute-onset functional neurolog-
identifiable anterior circulation stroke syndromes. When ical disorder versus imaging-negative/atypical stroke has to be
aphasia presents without limb motor deficits, additional signs reached as quickly as possible. While the above mentioned ex-
such as facial weakness, hemianopia, or sensory disturbance amination techniques can be incorporated into the neurolog-
are usually found.48 Truly isolated aphasia accounts for only ical examination without significant delay, further information
3% of acute stroke presentations and has an 86% likelihood from collateral history or short-term observation might not be
of being a stroke mimic.49 Functional aphasia is also rare and available acutely, leading to variable levels of diagnostic con-
usually presents as nonfluent aphasia with preserved compre- fidence at the end of the emergency workup.11 Considering
hension and naming.50 Its agrammatism can sound like broken the proven safety of thrombolysis for stroke mimics, only a
English or baby talk ("me sleepy"), compared with Broca tele- high level of certainty regarding the phenotype-based diag-
graphic speech ("I sleepy"), and speech patterns are generally nosis of a functional stroke mimic, supported by positive clin-
more inconsistent than in stroke aphasia. There is some evi- ical signs and normal neuroimaging, can justify withholding
dence that a proportion of patients with foreign accent syn- acute stroke treatment, that is, thrombolysis. In all other cases,
drome have a primary functional neurological disorder.51 the possibility of a functional disorder can be communicated
Popkirov et al Functional Neurological Disorder Mimicking Stroke 1633
early on, but a competing suspicion of acute ischemia should well as the fact that the diagnosis is based not just on normal
prompt stroke treatment algorithms in consideration of the imaging but primarily on specific clinical signs. There may be
short therapeutic windows. Follow-up examinations will usu- particular value in showing patients the positive clinical signs
ally settle the final diagnosis. Studies of functional motor dis- that establish the diagnosis: it emphasizes that the diagnosis is
order including stroke-like presentations suggest a low rate of not reached by exclusion and reveals the nature of the problem
misdiagnosis (<1%) at follow-up,59–61 although more studies (a deficit of voluntary but not automatic movement), as well as
from a hyperacute setting are required. the potential for improvement.64 Provision of information, for
example, from websites such as www.neurosymptoms.org or
Management patient organizations such as www.fndhope.org may be helpful
Treatment begins with an open and clear communication of but should not be regarded as a substitute for treatment.
the problem (Table 3).62 A simple explanation of the disorder As the patient achieves a degree of confidence in the di-
can be offered, making clear that the loss of motor control or agnosis, treatment is more likely to be helpful. Patients with
sensation results from abnormal brain functioning rather than functional limb weakness have been shown to benefit from
structural damage (software, not hardware); as such, it is po- specialized physiotherapy that relies on retraining movement
tentially reversible through appropriate therapy. It is important emphasizing the promotion of automatic movements with
to keep in mind that a functional neurological disorder is just as diverted attention.65 A feasibility trial of this approach com-
real, worrisome, and disabling as stroke can be. Terms such as pared to a standard neurophysiotherapy showed high rates of
hysteria, psychogenic, and conversion imply an unproven path- improvement,66 and a multicenter randomized controlled trial
ogenesis and are likely to offend.63 Psychological risk factors, of specialized physiotherapy for functional motor disorder is
when present, should not be ignored but are best addressed later currently underway.67 Importantly, physiotherapy is different
in treatment once patients have a secure understanding of their than that for stroke. Techniques that focus attention on the im-
condition. This is similar to addressing smoking or lifestyle pairment, such as strengthening or balancing exercises, are un-
issues in relation to ischemic stroke later as part of rehabilita- likely to help, and walking aids should be avoided. Instead,
tion. If patients have been managed under the working diag-
normalization of movement is aimed for through distraction
nosis of stroke to date, the distinction should be made clear, as
techniques and task-oriented exercises.65 Functional dysarthria
or aphasia responds well to specialized speech therapy.42 Stroke
Table 3. Principles of Treatment for Functional Neurological Disorder clinicians are well suited to manage acute multidisciplinary
Presenting to the Stroke Service
treatments and initiate appropriate long-term rehabilitation.
Downloaded from http://ahajournals.org by on January 9, 2024
Positive clinical diagnosis and communication If acute psychiatric symptoms such as severe anxiety or
Make a diagnosis based on positive diagnostic features, not on normal suicidality are present, (neuro)psychiatric assessment is nec-
investigations essary. Some patients with functional disorders will benefit
Explain diagnosis clearly, preferably demonstrating positive features
from psychotherapy, exploring how risk factors may have
contributed to vulnerability and perpetuation of symptoms,
Provide accessible material to allow the patient to understand the diagnosis
but clinicians should avoid the idea that all patients need or
Physiotherapy benefit from such treatment, and it is typically best delivered
Link principles of physiotherapy to ideas of brain retraining: promotion after some improvement in motor function has occurred. As
of normal automatic movements and suppression of abnormal voluntary with stroke, successful treatment of functional neurological
movement disorder is a multidisciplinary endeavor, which may include
Emphasis on goal-directed task-specific movement, eg, kick a football, occupational68 and speech therapy42 in addition to modali-
rather than specific limb movements ties already mentioned. It should be stressed that functional
Use of full-length mirror or video for visual feedback motor and sensory symptoms have a prognosis comparable to
that of neurological control cases, with about half of patients
Psychological therapy
showing no improvement in the long term.59 The associated
Assessment/formulation of potential predisposing, precipitating, and disability should not be underestimated, and the rate of spon-
perpetuating factors
taneous remissions should not be overestimated.
Treatment of comorbid anxiety, depression, and other psychiatric
disorders
Acknowledgments
Retraining of aberrant cognitions and behaviors in relation to movement We thank Dr Natalie Zadrozny for assistance with the Figure.
Occupational therapy
Careful use of aids and appliances, only as short-term solutions; Disclosures
requires planning to stop using Dr Popkirov received a speaker’s honorarium (Novartis). Dr Stone is
supported by an National Health Service (NHS) National Research
Focus on goal-directed activities, which may often involve overcoming
Scotland Career Fellowship and reports independent expert testi-
fear avoidance
mony work for personal injury and medical negligence claims, royal-
Speech and language therapy ties from UpToDate for articles on functional neurological disorder,
and runs a free nonprofit self-help website, www.neurosymptoms.
Identification and promotion of normal automatic sounds and
org. A.M. Buchan is a cofounder of Brainomix, which is developing
identification of aberrant involuntary speech sounds
eASPECTS.
1634 Stroke May 2020
diffusion-weighted imaging study. Cerebrovasc Dis. 2007;23:331–338. 59. Gelauff JM, Carson A, Ludwig L, Tijssen MAJ, Stone J. The prog-
doi: 10.1159/000099131 nosis of functional limb weakness: a 14-year case-control study. Brain.
47. Beliavsky A, Perry JJ, Dowlatshahi D, Wasserman J, Sivilotti ML, 2019;142:2137–2148. doi: 10.1093/brain/awz138
Sutherland J, et al. Acute isolated dysarthria is associated with a 60. Simhan S, Thijs V, Mancuso S, Tsivgoulis G, Katsanos A, Alexandrov
high risk of stroke. Cerebrovasc Dis Extra. 2014;4:182–185. doi: AV, et al. The outcome of acute functional neurological disorder: a meta-
10.1159/000365169 analysis of stroke-mimic presentations [published online January 22,
48. Denier C, Chassin O, Vandendries C, Bayon de la Tour L, Cauquil C, 2020]. J Neurol. 2020. https://link.springer.com/article/10.1007/s00415-
Sarov M, et al. Thrombolysis in stroke patients with isolated aphasia. 020-09709-3. Accessed March 4, 2020.
Cerebrovasc Dis. 2016;41:163–169. doi: 10.1159/000442303 61. Binzer M, Andersen PM, Kullgren G. Clinical characteristics of patients
49. Casella G, Llinas RH, Marsh EB. Isolated aphasia in the emergency
with motor disability due to conversion disorder: a prospective con-
department: The likelihood of ischemia is low. Clin Neurol Neurosurg.
trol group study. J Neurol Neurosurg Psychiatry. 1997;63:83–88. doi:
2017;163:24–26. doi: 10.1016/j.clineuro.2017.10.013
10.1136/jnnp.63.1.83
50. Mendez MF. Non-neurogenic language disorders: a preliminary
62. Carson A, Lehn A, Ludwig L, Stone J. Explaining functional disorders
classification. Psychosomatics. 2018;59:28–35. doi: 10.1016/j.
psym.2017.08.006 in the neurology clinic: a photo story. Pract Neurol. 2016;16:56–61. doi:
51. McWhirter L, Miller N, Campbell C, Hoeritzauer I, Lawton A, Carson A, 10.1136/practneurol-2015-001242
et al. Understanding foreign accent syndrome. J Neurol Neurosurg 63. Stone J, Wojcik W, Durrance D, Carson A, Lewis S, MacKenzie L, et
Psychiatry. 2019;90:1265–1269. doi: 10.1136/jnnp-2018-319842 al. What should we say to patients with symptoms unexplained by di-
52. Bègue I, Adams C, Stone J, Perez DL. Structural alterations in func- sease? The “number needed to offend”. BMJ. 2002;325:1449–1450. doi:
tional neurological disorder and related conditions: a software and 10.1136/bmj.325.7378.1449
hardware problem? Neuroimage Clin. 2019;22:101798. doi: 10.1016/j. 64. Stone J, Edwards M. Trick or treat? Showing patients with func-
nicl.2019.101798 tional (psychogenic) motor symptoms their physical signs. Neurology.
53. Barber PA, Hill MD, Eliasziw M, Demchuk AM, Pexman JH, 2012;79:282–284. doi: 10.1212/WNL.0b013e31825fdf63
Hudon ME, et al; ASPECTS Study Group. Imaging of the brain in acute 65. Nielsen G, Stone J, Matthews A, Brown M, Sparkes C, Farmer R, et
ischaemic stroke: comparison of computed tomography and magnetic al. Physiotherapy for functional motor disorders: a consensus recom-
resonance diffusion-weighted imaging. J Neurol Neurosurg Psychiatry. mendation. J Neurol Neurosurg Psychiatry. 2015;86:1113–1119. doi:
2005;76:1528–1533. doi: 10.1136/jnnp.2004.059261 10.1136/jnnp-2014-309255
54. Simon JE, Morgan SC, Pexman JH, Hill MD, Buchan AM. CT 66. Nielsen G, Buszewicz M, Stevenson F, Hunter R, Holt K, Dudziec M, et
assessment of conjugate eye deviation in acute stroke. Neurology. al. Randomised feasibility study of physiotherapy for patients with func-
2003;60:135–137. doi: 10.1212/01.wnl.0000042086.98735.75 tional motor symptoms. J Neurol Neurosurg Psychiatry. 2017;88:484–
55. Popkirov S, Grönheit W. Hyperperfusion can identify epileptic stroke 490. doi: 10.1136/jnnp-2016-314408
mimics. Pract Neurol. 2019;19:352–353. doi: 10.1136/practneurol-
67. Nielsen G, Stone J, Buszewicz M, Carson A, Goldstein LH, Holt K,
2019-002255
et al; Physio4FMD Collaborative Group. Physio4FMD: protocol for a multi-
56. Edlow BL, Hurwitz S, Edlow JA. Diagnosis of DWI-negative acute
centre randomised controlled trial of specialist physiotherapy for functional
ischemic stroke: a meta-analysis. Neurology. 2017;89:256–262. doi:
10.1212/WNL.0000000000004120 motor disorder. BMC Neurol. 2019;19:242. doi: 10.1186/s12883-019-1461-9
57. Watts J, Wood B, Kelly A, Alvaro A. Stroke syndromes associated 68. Gardiner P, MacGregor L, Carson A, Stone J. Occupational therapy for
Downloaded from http://ahajournals.org by on January 9, 2024
with DWI-negative MRI include ataxic hemiparesis and isolated in- functional neurological disorders: a scoping review and agenda for re-
ternuclear ophthalmoplegia. Neurol Clin Pract. 2013;3:186–191. doi: search. CNS Spectr. 2018;23:205–212. doi: 10.1017/S1092852917000797
10.1212/CPJ.0b013e318296f288
58. Balami JS, Chen RL, Buchan AM. Stroke syndromes and clinical man- Key Words: conversion disorder ◼ diagnosis ◼ functional neurological
agement. QJM. 2013;106:607–615. doi: 10.1093/qjmed/hct057 disorder ◼ imaging ◼ psychogenic ◼ stroke mimic