Академический Документы
Профессиональный Документы
Культура Документы
Neurological Symptoms
Jon Stone, Alan Carson
1992
Downloaded for Departemen THT (thtrscm@indo.net.id) at Universitas Indonesia from ClinicalKey.com by Elsevier on October 20, 2017.
For personal use only. No other uses without permission. Copyright 2017. Elsevier Inc. All rights reserved.
Functional and Dissociative (Psychogenic) Neurological Symptoms 1993
BOX 113.1 DSM-5 Diagnostic Criteria: Conversion TABLE 113.1 Functional Symptoms and Syndromes According to
Medical Specialty 113
Disorder (Functional Neurological
Symptom Disroder) Specialty Symptoms
Gastroenterology Irritable bowel syndrome
A. One or more symptoms of altered voluntary motor or sensory Respiratory Chronic cough, brittle asthma (some)
function.
B. Clinical findings provide evidence of incompatibility between Rheumatology Fibromyalgia, chronic back pain (some)
the symptom and recognized neurological or medical Gynecology Chronic pelvic pain, dysmenorrhea (some)
conditions. Allergy Multiple chemical sensitivity syndrome
C. The symptom or deficit is not better explained by another
medical or mental disorder. Cardiology Atypical/noncardiac chest pain, palpitations
D. The symptom or deficit causes clinically significant distress or (some)
impairment in social, occupational, or other important areas of Infectious diseases (Postviral) chronic fatigue syndrome,
functioning or warrants medical evaluation chronic Lyme disease (where physician
disagrees that there is ongoing infection)
Coding note: The ICD-9-CM code for conversion disorder is
300.11, which is assigned regardless of the symptom type. The Ear, nose, and throat Globus sensation, functional dysphonia
ICD-10-CM code depends on the symptom type (see below). Neurology Dissociative (nonepileptic) attacks,
Specify symptom type: functional weakness and sensory
(F44.4) With weakness or paralysis symptoms
(F44.4) With abnormal movement (e.g., tremor, dystonic Psychiatry Depression, anxiety
movement, myoclonus, gait disorder)
(F44.4) With swallowing symptoms
(F44.4) With speech symptom (e.g., dysphonia, slurred speech)
(F44.5) With attacks or seizures simplicity, the term functional is used in this chapter, although
(F44.6) With anesthesia or sensory loss psychogenic remains a popular term, especially among US neu-
(F44.6) With special sensory symptom (e.g., visual, olfactory, or rologists (Espay etal., 2009; Fahn and Olanow, 2014).
hearing disturbance)
Psychogenic, psychosomatic, and somatization all describe an
(F44.7) With mixed symptoms
exclusively psychological etiology.
Specify if: Functional describes in the broadest possible sense a problem
Acute episode: Symptoms present for less than 6 months. due to a change in function (of the nervous system)
Persistent: Symptoms occurring for 6 months or more. rather than structure. It can be criticized for being too
broad a term.
Specify if:
Nonorganic, nonepileptic describes what the problem is not,
With psychological stressor (specify stressor) rather than what it is.
Without psychological stressor No diagnosis refers to the fact that many neurologists, even
when faced with clear evidence of a functional/psychogenic
Reprinted with permission from the Diagnostic and Statistical Manual of
Mental Disorders, Fifth Edition, (Copyright 2013). American Psychiatric
neurological problem, are in the habit of making no diag-
Association. nosis at all and simply conclude that there is no evidence
of neurological disease (Friedman and LaFrance, 2010).
Medically unexplained superficially appears to be a neutral
term but is often interpreted by patients and doctors as not
Illness Anxiety Disorder describes excessive and intrusive knowing what the diagnosis is, rather than not knowing
health anxiety about the possibility of serious disease, why they have the problem. Furthermore, many neurologi-
which the patient has trouble controlling. Typically the cal diseases have uncertain etiology.
patient seeks repeated medical reassurance, which only has Hysteria, an ancient term originating from the idea of the
a short-lived effect. Health anxiety (previously called hypo- wandering womb causing physical symptoms, is generally
chondriasis) is often present to varying degrees in patients viewed as pejorative.
with psychogenic/functional symptoms but may be com-
pletely absent.
Factitious disorder (DSM-5) describes symptoms that are con-
sciously fabricated for the purpose of medical care or other EPIDEMIOLOGY IN NEUROLOGY AND OTHER
nonfinancial gain. MEDICAL SPECIALTIES
Munchausen syndrome describes someone with factitious dis- A number of studies of neurological practice have found that
order who wanders between hospitals, typically changing around one-third of neurological outpatients present with
their name and story. There is a strong association with symptoms the neurologist does not think relate to neurological
severe personality disorder. disease. In half of these (around one-sixth) the neurologist
Malingering is not a psychiatric diagnosis but describes the makes a primary functional or psychogenic diagnosis. The
deliberate fabrication of symptoms for material gain. rest have some neurological disease but symptoms out of pro-
portion to that disease (Stone etal., 2010a). These figures mirror
those in other medical specialties where functional symptoms
Other Terminology comprise around a third to half of patients seeing a cardiologist,
Our preferred terms for motor/sensory symptoms and black- gastroenterologist, rheumatologist, and other specialty prac-
outs unexplained by disease are functional and dissociative tices. Table 113.1 lists functional symptoms and syndromes
because (a) they describe a mechanism and not an etiology according to specialty. Patients with functional neurological
and (b) they sidestep an illogical debate about whether symp- symptoms have much higher rates of these other non-
toms are in the mind or the brain (Edwards etal., 2014). For neurological functional symptoms (Crimlisk etal., 1998).
Downloaded for Departemen THT (thtrscm@indo.net.id) at Universitas Indonesia from ClinicalKey.com by Elsevier on October 20, 2017.
For personal use only. No other uses without permission. Copyright 2017. Elsevier Inc. All rights reserved.
1994 PART III Neurological Diseases and Their Treatment
Studies of patients with functional neurological symptoms 7. Avoid blunt questions about depression and anxiety. It is not
have shown that they report just as much physical disability necessary for the purposes of neurological diagnosis to
and have higher rates of anxiety and depression than patients make an accurate assessment of a patients psychological
with neurological disease. Patients with these symptoms are state on the first visit since the diagnosis of a functional
more likely than the general population to be out of work disorder should be made on the basis of the physical exam-
because of ill health (Carson etal., 2011). Findings are similar ination, not the presence or absence of psychological
in other specialties. comorbidity. It often may be wise to leave questions about
emotions for later; only a minority of patients with func-
CLINICAL ASSESSMENT OF FUNCTIONAL AND tional symptoms believe that stress or psychological factors
have anything to do with their symptoms, in contrast to
DISSOCIATIVE (PSYCHOGENIC) SYMPTOMS patients with disease who commonly attribute their symp-
General Advice in History Taking toms to stress (Stone etal., 2010b). Patients with functional
disorders do have high rates of depression and anxiety but
Clinical assessment of the patient with functional symptoms
are often wary of questions about their emotions. They
requires a somewhat different approach to the standard neu-
often feel that the doctor is angling to blame their physical
rological assessment, especially when there are time con-
symptoms on them personally. Blunt questions like Are
straints. We suggest the following to improve the efficiency of
you depressed or anxious? may not therefore yield accu-
assessment:
rate answers. Instead try the following:
1. Start by making a list of all physical symptoms. Patients with For depression, ask about activities they can do and
functional symptoms typically have multiple physical whether they get enjoyment from them; if not, they may
symptoms. Making a list at the beginning avoids symptoms have anhedonia. If hospitalized, do they look forward to
cropping up later, helps build rapport, and allows an early visits from friends and family? Look at the patientare
appreciation of the main difficulties. Always ask about they miserable or avoiding eye contact? Or try framing
fatigue, pain, sleep disturbance, memory and concentration questions around the physical symptoms: Does your
symptoms, and dizziness. It may seem counterintuitive to weakness get you down? Depression is likely when there
be seeking more symptoms in someone who is already is persistent anhedonia or low mood most of the
polysymptomatic, but sometimes these symptoms, espe- time, with four or more of the following: fatigue, sleep
cially fatigue, are reluctantly volunteered even though they disturbance, suicidal ideation, poor memory or concen-
often cause the most limitation. tration, psychomotor retardation/agitation, or feelings of
2. Dissociative symptoms. Dizziness, if present, may turn out worthlessness/guilt and/or suicidal ideation.
to be dissociative in nature (e.g., feeling spaced out, For anxiety, look for three out of the following six symp-
there but not there, or unreal). Patients have trouble toms: restlessness/on edge, insomnia, fatigue, irritability,
describing dissociation, partly because it is hard to describe poor concentration, and/or tense muscles combined
but also because they fear the symptoms indicate crazi- with a history of worry that is persistent and hard to
ness. Depersonalization describes feeling disconnected from control. Worry will often be primarily focused on health.
your own body; derealization is a feeling of being discon- For panic attacks, look for four of the following: palpita-
nected from your surroundings (Video 113.1). tions, sweating, trembling/shaking, shortness of breath,
3. Onset. The onset in patients with weakness and movement choking sensation, chest pain/pressure, nausea/feeling of
disorders is sudden in around half of patients. Physical imminent diarrhea, dizziness, derealization/depersonali-
injury, pain, or acute symptoms of dissociation or panic are zation, afraid of going crazy/losing control, afraid of dying,
common in this situation. More gradual-onset symptoms tingling, flushes/chills. Panic is a very common problem
are often associated with fatigue. in patients with functional symptoms, especially nonepi-
4. What can the patient do? Patients with functional symptoms leptic attacks. Typically they are not reported as panic
have a tendency to report what they can no longer do rather attacks at all, but rather attacks where the patient unexpect-
than what they can do. While it is helpful to hear about edly had multiple symptoms all at once. The emotional
previous function, ask what they are able to dodo they component of the panic attack is experienced but errone-
enjoy it? ously attributed by the patient as being an understandable
5. Look for other functional symptoms and syndromes (see Table fear about the physical attack that is occurring.
113.1). The more they have, the more likely it is that the 8. Do not always expect psychological comorbidity or life events.
presenting neurological complaint is functional. Some Depression and anxiety are common, but around one-third
patients rotate between different specialists, with none of patients will have neither. Likewise, although some
appreciating their vulnerability to functional symptoms in patients have a history of a recent life event or stress, this
general. is not always present. Sometimes the panic attack or physi-
6. Ask the patient what they think is wrong and what should be cal injury that triggered the symptom is the most stressful
done. If they or their family have been concerned or life event, and the presence of the symptom then serves to
wondering about a specific neurological disease such as perpetuate the anxiety. Avoiding a diagnosis of functional
multiple sclerosis, Lyme disease, or trapped nerves, this symptoms in someone because they are psychologically
information is important to tailoring an explanation for normal is as great an error as making the diagnosis
the diagnosis later on. Do they have health anxiety? Do simply because the patient has a lot of obvious psychologi-
they think they are irreversibly damaged? Efforts at reha- cal comorbidity.
bilitation may be futile unless beliefs about damage can be
altered. In one prospective study of outpatients, beliefs
about irreversibility predicted outcome more than age, Advice in Specific Physical Diagnosis
physical disability, and distress (Sharpe etal., 2009). What
The diagnosis of functional symptoms should always be made
happened with previous doctors and why has the patient
on the basis of either:
come to see you? Some patients seek diagnosis and treat-
ment; others are simply looking for a label for a problem Clinical features typical of a functional/dissociative diagno-
they do not expect to resolve. sis (e.g., a typical thrashing dissociative (nonepileptic)
Downloaded for Departemen THT (thtrscm@indo.net.id) at Universitas Indonesia from ClinicalKey.com by Elsevier on October 20, 2017.
For personal use only. No other uses without permission. Copyright 2017. Elsevier Inc. All rights reserved.
Functional and Dissociative (Psychogenic) Neurological Symptoms 1995
attack with side-to-side head movements and eyes closed TABLE 113.2 Differentiating Dissociative (Nonepileptic) Attacks from
for 5 minutes); or Generalized Tonic-Clonic Epileptic Seizures 113
Physical signs demonstrating internal inconsistency (e.g.,
Dissociative Epileptic
Hoover sign for functional weakness, entrainment in func- attacks seizures
tional tremorsee later discussion).
HELPFUL
Mistakes are more likely when (1) too much weight is
placed on the presence of a psychiatric history; (2) the diag- Duration over 2 minutes* Common Rare
nosis is made just because the problem is bizarre or unfamil- Fluctuating course* Common Rare
iar; (3) there is failure to consider the possibility of a comorbid Eyes and mouth closed* Common Rare
neurological disease (e.g., nonepileptic attacks and epilepsy in
the same patient); or (4) when the assessing clinician is unfa- Resisting eye opening Common Very rare
miliar with a wide range of unusual neurological disorders Side-to-side head or body Common Rare
(Stone etal., 2013). movement*
La belle indifference (smiling indifference to disability) has Opisthotonus, arc de cercle Occasional Very rare
no diagnostic value, since it is just as commonly present in
neurological disease (Stone etal., 2006). When it is present, Visible large bite mark on side of Very rare Occasional
it often reflects a conscious desire on the patients behalf to tongue/cheek/lip
appear happy in a situation where they are concerned that Dislocated shoulder Very rare Occasional
someone will make a psychiatric diagnosis, or alternatively Fast respiration during attack Common Ceases
may indicate factitious disorder.
Grunting/guttural ictal cry sound Rare Common
Weeping/upset after a seizure* Occasional Very rare
Blackouts/Dissociative (Nonepileptic) Attacks Recall for period of Common Very rare
Dissociative (nonepileptic) attacks, also commonly called psy- unresponsiveness*
chogenic nonepileptic seizures, are the most common type of Thrashing, violent movements Common Rare
symptom unexplained by disease seen in neurological practice
Postictal stertorous breathing* Rare Common
(Schacter and LaFrance, 2010). Studies have estimated that up
to 1 in 7 patients in a first fit clinic, 50% of patients brought Pelvic thrusting*,
Occasional Rare
in by ambulance in apparent status epilepticus, and around Asynchronous movements* ,
Common Rare
20% to 50% of patients admitted for videotelemetry have this
diagnosis. Peak age of onset is in the mid-20s; females Attacks in medical situations Common Rare
predominate 3:1. Later-onset patients in their 40s and 50s NOT SO HELPFUL
have a 1:1 gender ratio and typically have health anxiety Stereotyped attacks Common Common
and a history of recent organic health problems (Duncan
etal., 2006). Attack arising from sleep Occasional Common
Dissociative attacks most frequently involve shaking move- Aura Common Common
ments of the limbs with impaired awareness for the attack.
Incontinence of urine or feces* Occasional Common
The movement seen is usually a tremor rather than a jerk.
Around 20% of attacks resemble syncope more than epilepsy Injury* Common Common
and consist of the patient falling down and lying still Report of tongue biting* Common Common
with their eyes shut for a prolonged period (Benbadis and
Chichkova, 2006); very few other conditions lead to this clini- *Endorsed by a recent systematic review (Avbersek, A., Sisodiya, S.,
2010. Does the primary literature provide support for clinical signs
cal scenario. Occasionally, attacks similar to complex partial
used to distinguish psychogenic nonepileptic seizures from
seizures may be seen. Drop attack semiology without loss of epileptic seizures? J Neurol Neurosurg Psychiatry 81, 719725).
awareness can also be seen in patients who are recovering from
These signs unhelpful in distinguishing nonepileptic attacks from
or subsequently develop dissociative attacks, suggesting a con- frontal lobe seizures.
tinuity of these phenotypes in some patients.
Normally sleepy.
The diagnosis is usually made on the basis of the observ-
Frontal lobe epilepsy. Nonepileptic attacks do appear to arise from
able features of an attack, preferably recorded using video sleep, but video electroencephalogram (EEG) usually shows this
electroencephalography (EEG) (Table 113.2) (Lafrance etal., not to be true sleep. Attacks arising from EEG-documented sleep
2013). No one feature should be used on its own to make a are suggestive of epilepsy.
Especially carpet burns and bruising.
diagnosis, but some are more reliable than others (Avbersek
and Sisodiya, 2010). Data on the reliability of these signs have
largely been taken from studies of videotelemetry; these signs
are less reliable when based on witness descriptions. this is useful information that gives the clinician windows into
Attention has shifted in recent years to diagnosis using understanding both the nature of the attacks (a mechanism
subjective experience of the attack. Patients with dissociative related to panic attacks in which the patient dissociates) and
attacks typically do not volunteer a prodrome. Indeed, studies possible treatment (teaching the patient distraction tech-
analyzing dialogue between neurologists and patients have niques to use during this warning phase to avert the attack and
shown that the lack of any attempt to describe a prodrome following treatment principles for panic disorder). As some
may be of diagnostic value in itself, since patients with epi- patients recover, they may experience awareness during the
lepsy usually do attempt to describe their prodrome when attack itself.
present, compared to patients with dissociative attacks who Video EEG may be supplemented by an open suggestion
describe the disability associated with the attack (Reuber etal., protocol to help record an attack (McGonigal etal., 2002).
2009). However, if questioned, many patients with nonepilep- Deceptive placebo induction with saline or a tuning fork is
tic attacks will admit to a brief prodrome with features of more controversial. Postictal prolactin measurement (to detect
panic (Goldstein and Mellers, 2006) (Fig. 113.1). If obtained, high prolactin after a generalized seizure) has fallen out of
Downloaded for Departemen THT (thtrscm@indo.net.id) at Universitas Indonesia from ClinicalKey.com by Elsevier on October 20, 2017.
For personal use only. No other uses without permission. Copyright 2017. Elsevier Inc. All rights reserved.
1996 PART III Neurological Diseases and Their Treatment
Feeling that the person pyramidal weakness, with the flexors weaker in the arms
was going crazy and the extensors weaker in the legs.
Numbness or tingling Inconsistency during examination. This may be obviousfor
in arms, legs, or face example, a patient who can walk to the examination table
but cannot raise the leg against gravity on examination.
Sweating More commonly there is weakness of ankle movements, but
the patient can stand on tiptoes or on their heels. Arm weak-
Shortness of breath or ness may be incompatible with performance, such as remov-
smothering sensation ing shoes or carrying a bag.
Racing or pounding Hoover sign. Hip extension must be weak for this test to
heart work. The presence of hip extension weakness itself in an
ambulant patient is a positive sign of functional weakness.
Chest pain
If hip extension returns to normal during contralateral hip
flexion against resistance, this demonstrates structural integ-
0 10 20 30 40 50 60 70 80 rity of the motor pathways (Fig. 113.2) (Video 113.3). The
% with symptom test is easiest to do with the patient in the sitting position.
We find it useful to demonstrate this sign to the patient and
Epileptic seizures (focal) (n = 19) relatives to indicate that the diagnosis is being made on the
Dissociative attacks (n = 25)
basis of positive criteria. This test may be false positive when
Fig. 113.1 Prodromal symptoms of panic are much more common in there is cortical neglect.
dissociative (nonepileptic) attacks than epilepsy. Although they may Hip abductor sign. A similar test involves demonstrating
not initially be disclosed, they provide an opportunity for treatment. weakness of hip abduction which returns to normal with
(Redrawn from Goldstein, L.H., Mellers, J.D., 2006. Ictal symptoms of contralateral hip abduction against resistance.
anxiety, avoidance behaviour, and dissociation in patients with dis- Dragging gait. If there is moderate or severe unilateral leg
sociative seizures. J Neurol Neurosurg Psychiatry 77, 616621, by weakness, the patient may walk with a dragging gait in
permission of BMJ publications.) which the foot does not leave the ground. Often the hip is
externally or internally rotated (Fig. 113.3).
Give-way weakness. This is a pattern of weakness in which
the patient transiently has normal power but then the limb
favor owing to problems with the reliability and timing of the
gives way, sometimes just before it is touched. If the arm is
test. Common diagnostic pitfalls include coexistent epilepsy
very weak it may hover for a second before collapsing.
(present in 5%20% of patients), frontal lobe seizures,
Normal power can be produced by saying to the patient, At
sleep-related movement disorders, and paroxysmal movement
the count of 3, push1 2 3 push. This is a less
disorders.
reliable sign and occurs more commonly in painful limbs
or occasionally in myasthenia gravis.
Drift without pronator sign. Sometimes patients with func-
Weakness/Paralysis tional arm weakness will demonstrate a downward drift
Weakness as a functional symptom is more common in of the arm without the pronation seen in conditions such
females and typically presents in the mid-thirties but like all as stroke.
functional symptoms can occur in children and the elderly. Facial spasm (looking like weakness) (Video 113.4). It is not
Estimates of incidence are around 5/100,000, comparable to uncommon to see patients who apparently have weakness
multiple sclerosis. Comorbidity with other functional symp- of their face, usually ipsilateral to a functional hemiparesis
toms, especially fatigue and pain, is almost invariable. The (Fasano etal., 2012). In fact, the appearance is nearly
most common presentation is unilateral weakness, followed always due to unilateral overactivity of the platysma
by monoparesis and paraparesis. There is no good evidence muscle which pulls the side of the lip downwards. There
for left-sided or nondominant preponderance. Complete may be jaw deviation and sometimes an upwards devia-
paralysis is less common clinically than weakness (Stone tion of the mouth instead. Contraction of the orbicularis
etal., 2010b). oculi muscle can lead to an appearance with a depressed
The onset is sudden in around 50% of patients. In the acute eyebrow (Fig. 113.4), which may be interpreted incorrectly
presentation, there are often symptoms of a panic attack, dis- as ptosis, although true functional ptosis does occur more
sociative seizure, or an immediate trigger such as a physical rarely. These features can sometimes be enhanced on
injury, acute pain, migraine, a general anesthetic, or an episode examination by sustained voluntary contraction of facial
of sleep paralysis (Stone etal., 2012). When the onset is more or periocular muscles.
gradual, there is typically a history of fatigue, pain, or immo- Altered reflexes. Occasionally, patients with functional
bility on which the weakness becomes superimposed gradu- weakness may have what appears to be ankle clonus, which
ally over time. The weakness seen in complex regional pain on closer inspection has features of functional tremor. There
syndrome type 1 (CRPS1) (Birklein etal., 2000) has the same may also appear to be reflex asymmetry if the patient is
clinical features as functional weakness. co-contracting agonist and antagonist muscles on one side
Subjectively, patients with functional weakness report that of their body. Finally, in our experience it is not that unusual
the affected limb doesnt feel as if it belongs to them or in for the plantar response to be relatively mute on the affected
extreme situations as if it is not there or is someone elses side if there is marked sensory disturbance.
limb (Video 113.2). They commonly report that the leg gives
away or that they keep dropping things unexpectedly. The
diagnosis depends on demonstrating internal inconsistency
Movement Disorders
and incongruence with disease: Functional movement disorders have been increasingly recog-
nized by movement disorder specialists, especially over the
Pattern of weakness. In functional weakness, the limb is past decade (Thenganatt and Jankovic, 2015). In specialist
usually globally weak or often demonstrates the inverse of clinics, these symptoms account for up to 10% of new referrals
Downloaded for Departemen THT (thtrscm@indo.net.id) at Universitas Indonesia from ClinicalKey.com by Elsevier on October 20, 2017.
For personal use only. No other uses without permission. Copyright 2017. Elsevier Inc. All rights reserved.
Functional and Dissociative (Psychogenic) Neurological Symptoms 1997
113
Downloaded for Departemen THT (thtrscm@indo.net.id) at Universitas Indonesia from ClinicalKey.com by Elsevier on October 20, 2017.
For personal use only. No other uses without permission. Copyright 2017. Elsevier Inc. All rights reserved.
1998 PART III Neurological Diseases and Their Treatment
B
Fig. 113.4 Functional facial spasm leading to (A) deviation of the
chin and downward movement of the corner of the mouth from
platysma contraction and (B) upward contraction of the mouth and
orbicularis.
Downloaded for Departemen THT (thtrscm@indo.net.id) at Universitas Indonesia from ClinicalKey.com by Elsevier on October 20, 2017.
For personal use only. No other uses without permission. Copyright 2017. Elsevier Inc. All rights reserved.
Functional and Dissociative (Psychogenic) Neurological Symptoms 1999
Myoclonus
Brief jerky movements may appear to be myoclonus. More
commonly, patients have more complex hyperkinetic move-
ments that are hard to accurately classify. Functional myo-
clonus may be stimulus sensitive, especially during deep
tendon reflex testing, where myoclonus may occur even A
before the reflex hammer has made contact (Hallett, 2010).
Functional/psychogenic myoclonus is often associated with
a bereitschaft potential (BP) prior to the movement. This
requires recording multiple events using EEG and back-
averaging according to an electromyogram (EMG) (van der
Salm etal., 2012). The presence of a BP does not provide
evidence of conscious intention to move but does indicate that
the voluntary motor system is being utilized for the move-
ment. In recent years it has become recognized that a large
proportion of patients with axial myoclonic syndromes, com-
monly described as propriospinal myoclonus, have a BP prior
to their jerks and other features in keeping with a functional
movement disorder (Erro etal., 2014; van der Salm etal.,
2010). In addition, tics as a functional/psychogenic movement
disorder also rarely occur. Clinical features include a late age B
of onset, lack of premonitory symptoms, inability to suppress
tics, and comorbidity with other functional movement disor- Fig. 113.5 Functional/psychogenic dystonia typically presents with
ders (Baizabal-Carvallo and Jankovic, 2014). (A) a clenched fist or (B) an inverted plantar-flexed ankle. (From Stone,
J., 2009. The bare essentials: functional symptoms in neurology. Pract
Dystonia Neurol 9, 179189, by permission of BMJ publications.)
Downloaded for Departemen THT (thtrscm@indo.net.id) at Universitas Indonesia from ClinicalKey.com by Elsevier on October 20, 2017.
For personal use only. No other uses without permission. Copyright 2017. Elsevier Inc. All rights reserved.
2000 PART III Neurological Diseases and Their Treatment
Downloaded for Departemen THT (thtrscm@indo.net.id) at Universitas Indonesia from ClinicalKey.com by Elsevier on October 20, 2017.
For personal use only. No other uses without permission. Copyright 2017. Elsevier Inc. All rights reserved.
Functional and Dissociative (Psychogenic) Neurological Symptoms 2001
113
Tubular field
Examination at Examination at
A Normal field 50 cm 150 cm
120 105 90 75 60
70
135 45
60
50
150 30
40
30
165 15
20
10
90 80 70 60 50 40 30 20 10 10 20 30 40 50 60 70 80 90 0
180
10
20 345
195
30
40
210 330
B
Fig. 113.7 Functional visual symptoms can be associated with (A) tubular visual fields at the bedside or (B) spiral fields on Goldmann perimetry.
(From Stone, J., 2009. The bare essentials: functional symptoms in neurology. Pract Neurol 9, 179189, by permission of BMJ publications.)
seen a man who used a computer to speak for 4 years before this symptom can be explained by gastroesophageal reflux
making a good recovery. In organic foreign accent syn- disease and investigation by gastroenterology or otolaryn-
drome, for example after stroke, there is usually a breakdown gology is usually appropriate (Selleslagh etal., 2014).
of prosody, the melody of speech, which as a byproduct Attributed by the Egyptians to a wandering womb (i.e.,
leads to a new accent. In functional foreign accent syndrome, globus hystericus), globus sensation is one of the oldest
the accent is more of a parody or imitation of foreign speech, recorded medical problems.
although it is experienced as involuntary by the patient.
Dysphonia. Functional dysphonia is a common presenting
symptom to otolaryngologists but may be seen by neurolo- Memory and Cognitive Symptoms
gists in combination with other functional symptoms.
Cognitive symptoms are common among patients with func-
Speech is usually whispering in nature and may follow a
tional neurological symptoms. They may be attributable (by
genuine or perceived episode of laryngitis. At least six ran-
both doctor and patient) to associated fatigue, anxiety, or low
domized controlled trials in this area have suggested benefit
mood or become a presenting symptom in their own right.
of voice therapy (Ruotsalainen etal., 2008).
Presentations include the following:
Globus pharynges. This describes the symptom of something
sticking in the throat, even when the patient is not swal- Normal absentmindedness and functional memory symptoms.
lowing anything. There is controversy regarding how often In someone who is usually not absentminded, forgetting
Downloaded for Departemen THT (thtrscm@indo.net.id) at Universitas Indonesia from ClinicalKey.com by Elsevier on October 20, 2017.
For personal use only. No other uses without permission. Copyright 2017. Elsevier Inc. All rights reserved.
2002 PART III Neurological Diseases and Their Treatment
why they went upstairs, losing their keys, or losing track without limb pain. The neurologist may also have to assess
of conversation may be interpreted as abnormal. Anxiety back pain in someone with functional symptoms. Certain
about the cause and attention paid to the symptom can signs, including some described by Gordon Waddell, do
amplify the problem and lead to neurological referral. A provide useful information (Waddell, 2004):
subgroup of patients in any memory clinic presents without
Straight leg raising while lying and sitting. If a patient can sit
obvious anxiety, depression, or stress apart from anxiety
comfortably on an examination table with their legs
about their memory symptoms (Schmidtke etal., 2008). In
stretched out at 90 degrees to their body, any pain induced
addition to escalating absentmindedness as described, the
by straight leg raising in the supine position cannot be due
patient with functional memory symptoms usually reports
to true sciatic nerve pain.
variability in their memory problems and episodes when
Simulated rotation. Ask the patient to stand with their
they forgot familiar information such as their own address
feet flat on the floor and rotate the trunk with the
and then remembered it again.
arms stabilized at the sides. This movement occurs at the
Word-finding difficulty is a common symptom among
knees and not the back and should not cause significant
patients with other functional neurological symptoms. They
back pain.
may also report mixing words up with spoonerisms or neol-
Localized tenderness. Some patients with back pain are exqui-
ogisms, but true dysphasia is rare.
sitely tender to superficial palpation.
Poor concentration as part of a psychological disorder. Attention
Axial loading. Pressure on the head should not cause signifi-
and concentration may be noticeably impaired in anxiety
cant back pain.
and depression. In severe depression, the presentation may
be that of a pseudodementia. Routine neuropsychological These signs are not, as is commonly assumed, proof of
tests may give spuriously low values. Attempting to control malingering or even exaggeration, but they do suggest that
for this with the use of self-reported anxiety and depression reports of back pain are heightened when the patient and
scales is unreliable in the authors experience. doctor are paying attention to it.
Pure retrograde functional/psychogenic amnesia. This memory
syndrome, common to fiction, also happens occasionally in Fatigue
real life. It presents with normal anterograde memory but
with a large chunk of absent memory prior to a certain point Fatigue may turn out, for all of the patients more obvious
(pure retrograde amnesia) (Staniloiu etal., 2010). When not symptoms, to be their most limiting symptom. Chronic per-
associated with obvious gain (e.g., a criminal who cannot sistent fatigue in the absence of a disease cause has been
remember the crime), it can occur in response to stress, labeled, defined, and conceived of in many waysfor example,
perhaps as a self-deceptive phenomenon. It may be a feature chronic fatigue syndrome (CFS), neurasthenia, and myalgic
in patients who have apparent catastrophic cognitive impair- encephalomyelitis (ME), some of which (ME) are based on
ment secondary to minor head injury. The authors have also the belief that the symptoms are due to an underlying neuro-
seen several patients with this syndrome who wished to be logical disease process yet to be fully elucidated. The fact that
at a previous time in their life, and their amnesia was best ME is listed as a neurological disorder and neurasthenia as a
seen as compatible with that wish. Neurologists may also psychiatric disorder in the current ICD-10 classification indi-
be asked to see patients in fugue states who characteristically cates the nature of the controversy. One of the simpler defini-
have moved from where they normally live and then cannot tions of CFS is persistent fatigue lasting longer than 6 months
remember who they are or where they live. which is not due to another cause. The relevance of this discus-
sion is that there have been large positive randomized trials
In the assessment of patients with functional cognitive of cognitive behavioral therapy and graded exercise for CFS
symptoms, approximate answers to questions (e.g., How (White etal., 2011) which have potentially helped to inform
many legs has a horse got? Answer: 3) are classically treatment for patients with functional neurological symptoms
described. This has been called Ganser syndrome after the (where the evidence is more limited).
nineteenth-century German psychiatrist who first reported it.
In our experience, this is rare and is a marker of factitious
symptoms. More common is the early loss of relatively pro- FEIGNING AND MALINGERING
tected knowledge such as the names of spouses or children,
The issue of feigning remains topical in this area, firstly
discrepancies between real-life function and test results, or
because these are symptoms without verifiable disease, and
discrepancies between results on cognitive tests that localize
secondlyunlike, for example, irritable bowel syndrome
to the same anatomical region (e.g., memory). The individual
they are symptoms that relate to the voluntary nervous system
may do better in more complex tasks in the same domain than
(Kanaan etal., 2009). Distinguishing symptoms that are
simple ones. In such situations, cognitive effort tests may be
under conscious intentional voluntary control from those that
useful. These are very simple tests that even patients with
are not is difficult because: (1) the positive signs used to make
severe dementia or head injury should be able to perform well.
a diagnosis of functional symptoms would be the same if
For example, the coin-in-the-hand test involves 10 trials of
someone was feigning, (2) doctors are not trained to detect
showing a patient which hand a coin is held in, asking them
deception, and (3) some patients may be in a state of
to close their eyes for 10 seconds and then choose the hand
self-deception.
with the coin (Kapur, 1994). A score at chance indicates poor
Clues to feigning include a documented history of lying in
effort. A score below chance is sometimes used as evidence of
the past or use of multiple different names, major inconsisten-
factitious disorder/malingering, although in reality it cannot
cies in the history given to different clinicians, and avoidance
distinguish between conscious or unconscious deception.
of investigations. The only definitive ways to be confident that
feigning is an explanation for functional neurological symp-
Overlap with Pain and Fatigue toms are if the patient is covertly observed doing something
highly discrepant with what they have claimed to be able to
Pain do (e.g., playing tennis when they claim to be in a wheelchair)
We have discussed the similarity of functional motor and or if they confess to feigning. Feigning of other symptoms such
sensory symptoms seen in CRPS1 to those seen in patients as post-traumatic stress disorder, pain states, or depression
Downloaded for Departemen THT (thtrscm@indo.net.id) at Universitas Indonesia from ClinicalKey.com by Elsevier on October 20, 2017.
For personal use only. No other uses without permission. Copyright 2017. Elsevier Inc. All rights reserved.
Functional and Dissociative (Psychogenic) Neurological Symptoms 2003
% misdiagnosis
often exaggeration to convince the doctor there is a problem
rather than exaggeration to deceive. This exaggeration may 30
paradoxically get worse when they feel disbelieved by the
doctor. Also, some discrepancies between reported and 20
observed function occur because of a mismatch between the
patients genuine perception of how they are (terrible) com- 10
pared to the reality of how they actually are (not quite so bad).
For example in one study, ten patients with functional tremor
who explicitly knew that a tremor watch would record how 0
much of the time they were shaking grossly overestimated the
9
result (84% diary estimate vs 4% recorded tremor). A bit like
50
55
60
65
70
75
80
85
90
95
19
19
19
19
19
19
19
19
19
19
looking inside the fridge to see if the light is on, some patients
with functional disorders may feel as if their symptoms are Year of diagnosis
always there because they are whenever they think about them Fig. 113.8 Frequency of misdiagnosis of conversion symptoms
(Parees etal., 2012). and hysteria (mean %, 95% confidence intervals) in 27 studies
Although clinicians estimate feigning to account for around (n = 1466), mean follow-up 5 years, plotted at midpoint of 5-year
5% of patients with functional symptoms, it is impossible for intervals according to when patients were diagnosed. (From
anyone to truly know. Most neurologists will come across Stone, J., 2009. The bare essentials: functional symptoms in neurol-
patients in their career who have hoodwinked them or they ogy. Pract Neurol 9, 179189, by permission of BMJ publications.)
may boast about those they caught out. It may be tempting to
start believing that most patients are feigning. Several argu-
ments stand in the way of this hypothesis: (1) the homogene-
ity of patient experiences as described in clinic, both of their Dissociative (nonepileptic) seizures probably have a better
symptoms and of their general bewilderment; (2) follow-up overall outcome than motor disorders (Durrant etal., 2011).
studies showing symptom persistence over decades; (3) the Good prognostic factors for functional neurological symp-
high frequency of nonepileptic attacks during video EEG even toms are a willingness to accept the potential reversibility of
when patients have been told this is the suspected diagnosis; the symptoms, a good interaction with the doctor, short dura-
(4) the persistence of positive signs such as the Hoover tion of symptoms, and a lack of other physical symptoms. The
sign even when the patient has been shown how it operates; presence of anxiety and depression and change in marital
(5) evidence of shoe wear in patients with functional gait status have been found to predict positive outcome in some
disorders and contractures in patients with fixed dystonia; studies but not others.
(6) similar prevalence figures between industrialized nations Poor prognostic factors include strong beliefs in lack of
with welfare benefit systems and nonindustrialized countries reversibility of symptoms/damage, anger at the diagnosis of
without (Simon etal., 1996); and (7) historical consistency a nonorganic disorder, delayed diagnosis, multiple other
in clinical presentation over centuries (Stone etal., 2008). physical symptoms/somatization disorder, concurrent organic
disease, personality disorder, older age, sexual abuse, receipt
of financial benefits, and litigation. However, in most studies
MISDIAGNOSIS these prognostic factors only explained a limited amount of
While neurologists tend to worry about feigning, doctors other the variance. In practice, some patients with many poor prog-
than neurologists, especially psychiatrists, tend to be preoc- nostic factors respond well to treatment, and some with many
cupied by the opposite concern of misdiagnosis. Studies in the good prognostic features do badly.
1950s and 1960s suggested high rates of the misdiagnosis of
hysteria of up to 60%. A systematic review of 27 studies
included 1466 patients with a mean follow-up of 5 years and
ETIOLOGY AND MECHANISM
found a frequency of misdiagnosis of around 4% since 1970, Patients often ask, Why has this happened? It is useful to
before the advent of CT scans and videotelemetry (Stone etal., rephrase this question into two separate questions as we do
2005) (Fig 113.8). This is a frequency of misdiagnosis compa- for other neurological disorders such as multiple sclerosis:
rable to other neurological and psychiatric disorders. A study Why has it happened? (etiology) and How has it hap-
of 1144 patients in Scotland found an even lower misdiagnosis pened? (mechanism).
rate at 18 months of only 4 patients (Stone etal., 2009). This The etiology of functional symptoms is multifactorial and
is not a reason for complacency, however, and we would varies hugely between patients. Although one can individually
recommend that neurologists continue to be responsible formulate an etiology for patients based on the factors shown
for these diagnoses. For neurologists, relying on obvious in Table 113.3, this model is likely to be incomplete. If there
psychiatric comorbidity, forgetting about neurological disease is one rule here, it is avoid generalizing. The notion that all
comorbidity or making a diagnosis using gait disorder are patients with functional symptoms have been abused or suf-
common pitfalls (Stone etal., 2013). fered some sort of trauma is not supported by the evidence.
Likewise, although many patients with functional symptoms
believe that stress is not relevant to their symptoms, between
PROGNOSIS 25% and 50% do think it is relevant (Stone etal., 2010b).
Long-term follow-up studies have suggested that functional More common and relevant to understanding mechanism
motor symptoms persist in the majority and improve in a than stress is the presence of a physiological trigger such as
third (Gelauff etal., 2014). As expected, sensory symptoms minor injury, infection, or drug side effect, as discussed earlier
have a better prognosis than weakness, which in turn has a (Pares etal., 2014; Stone etal., 2012). Having a neurological
better outcome than fixed dystonia (Ibrahim etal., 2009). disease is an important and powerful risk factor for functional
Downloaded for Departemen THT (thtrscm@indo.net.id) at Universitas Indonesia from ClinicalKey.com by Elsevier on October 20, 2017.
For personal use only. No other uses without permission. Copyright 2017. Elsevier Inc. All rights reserved.
2004 PART III Neurological Diseases and Their Treatment
symptoms. Understanding prior vulnerabilities may help fill incidental or age-related findings (Petrie etal., 2007). It is
in general understanding about the problem, but it is really especially worth anticipating the 15% risk of nondiagnostic
the perpetuating factors in Table 113.3 that are the most high-signal lesions on magnetic resonance imaging (MRI) and
important targets for treatment. other incidentalomas (Morris etal., 2009) and the presence
The neural mechanisms of functional neurological symp- of age-related degenerative change on spinal MRI. If this must
toms are not yet well understood, but functional imaging be done, an analogy with gray hair may be usefuleveryone
studies of functional motor symptoms combined with other gets it but it doesnt necessarily mean anything. Try to do all
neurophysiological techniques are adding to our understand- the necessary tests at the same time and not sequentially,
ing. A functional imaging study of unilateral weakness and which tends to prolong the agony of diagnostic limbo. Even
sensory disturbance in four patients whose symptoms subse- if there are abnormalities on a scan, they cannot explain most
quently improved showed dose-responsive hypoactivation of the positive signs described in this chapter.
in contralateral thalamic and basal ganglia areas (Vuilleumier
etal., 2001) (see Fig. 113.7). Other studies are beginning to
converge on a hypothesis that preemptor areas are overactive
TREATMENT
and not properly integrating with feed-forward areas in the Figure 113.9 shows a treatment algorithm for functional neu-
brain, such as the parietal lobe, that may be responsible for rological symptoms described in more detail below.
the sense of agency of movement. This might help explain the
apparent voluntariness of functional motor symptoms in
the absence of a sense of intention on the part of the patient
Explanation
(Voon etal., 2010). Another study found neural differences in The diagnosis of a functional disorder is made on the basis of
patients feigning weakness compared to patients with actual positive neurological features on assessment combined with a
functional weakness (Cojan etal., 2009). Somatosensory knowledge of the range of presentations of neurological
evoked potentials may be altered in functional disorders com- disease, and not on the basis of psychiatric symptomatology
pared to feigning (Blakemore etal., 2013) although changes even though the latter may be relevant to etiology and
in transcranial magnetic stimulation parameters thought to treatment. Neurologists are therefore in a good position to
relate to functional limb weakness (Liepert etal., 2011) have explain the diagnosis of functional disorders. A really success-
also been reproduced in subjects consciously feigning weak- ful explanation can alter outcome dramatically, even with
ness (Liepert etal., 2014). In patients with dissociative (non- long-standing symptoms. Most authors agree that a good
epileptic) seizures some intriguing preliminary findings in explanation is a prerequisite to successful treatment with phys-
interictal EEG are also emerging (Knyazeva etal., 2011). Neu- iotherapy or psychological therapy, and there is some evidence
roimaging and advances in neuroscience do hold out a that it does affect outcome (Carton etal., 2003; Jankovic etal.,
promise of understanding symptoms in parallel neurological 2006). How the diagnosis is explained to the patient will
and psychiatric ways, with the hope of potentially being able depend on the clinicians own views of why and how the
to abandon the artificial distinction between the two. symptoms are present; no method is suitable for all patients.
Table 113.4 lists a series of components of explanation that
we believe provide a constructive basis for further treatment.
INVESTIGATIONS A successful explanation leaves a patient feeling they have
Investigations will usually be necessary, partly because the finally got to the bottom of what the problem is, with con-
presence of a functional symptom does not exclude a comor- fidence that they do have something genuine, but potentially
bid underlying neurological disease, but it is worth consider- reversible with rehabilitation and determination. Even in
ing how to perform them in the patients best interest if results those who do not improve there is value in the peace of mind
are likely to be normal. If there is any delay in tests, patients brought by a clear diagnosis. In our own practice, showing
can benefit from being told the likely diagnosis and that clini- patients their positive signs, such as the Hoover sign or the
cal investigations will probably be normal or show only tremor entrainment test, is the most transparent and effective
Downloaded for Departemen THT (thtrscm@indo.net.id) at Universitas Indonesia from ClinicalKey.com by Elsevier on October 20, 2017.
For personal use only. No other uses without permission. Copyright 2017. Elsevier Inc. All rights reserved.
Functional and Dissociative (Psychogenic) Neurological Symptoms 2005
113
Neurology/Rehabilitation Medicine
Step 3 Psychiatry/Psychology
Complex Care Physio/occupational/speech therapy
Chronic pain services
Hypnosis/Sedation/? TMS
Fig. 113.9 A treatment algorithm for functional neurological symptoms. Note that the neurologist has a role not just in diagnosis but in initial
explanation and triaging further treatment. Patients who cannot engage with the diagnosis often do not benefit from attempts at further treatment.
(Adapted with permission from Health Improvement Scotland (2012).)
way of explaining that this is not a diagnosis of exclusion, but and is not as important as the totality of the explanation. A
one in which the physical signs indicate the potentially revers- psychological explanation has the advantage of being clear-
ible nature of the problem (Stone and Edwards, 2012). cut, compatible with psychiatric referral, and consistent with
The issue of whether one tells the patient they have psy- psychiatric terminology. Unfortunately, words such as psycho-
chogenic symptoms, conversion disorder, functional symp- genic are commonly interpreted by a patient as meaning
toms, or dissociative symptomsterminology discussed at the crazy or making symptoms up, so even if psychogenic is a
beginning of this chapteris only one of these components given neurologists preferred term for theoretical reasons, it is
Downloaded for Departemen THT (thtrscm@indo.net.id) at Universitas Indonesia from ClinicalKey.com by Elsevier on October 20, 2017.
For personal use only. No other uses without permission. Copyright 2017. Elsevier Inc. All rights reserved.
2006 PART III Neurological Diseases and Their Treatment
important to consider whether the patient is translating the may be helpful to use distraction techniques during move-
words into meanings never intended. A further disadvantage ment to allow better movement. In someone with a func-
is that such terms are based on only one aspect of evidence tional tremor, asking the patient to make a voluntary large
concerning etiology and not really compatible with a biopsy- amplitude movement on top of their tremor and then to
chosocial model. Finally, studies in primary care attempting reduce the amplitude may allow them to gain control of an
to help patients reattribute their functional symptoms psycho- involuntary tremor. Principles of graded exercise as used in
logically have not been successful (Gask etal., 2011). Func- chronic fatigue syndrome are also likely to be helpful. As with
tional is a more acceptable term (Edwards etal., 2014), which back pain, the patient should be told to expect relapses as
along with dissociative describes a mechanism and leaves the they increase activity. Mental imagery techniques and mirror
etiology more open. These terms have the advantage of allow- therapy as used in CRPS may also be useful (Nielsen etal.,
ing a more integrated description involving biological, psy- 2013). Consensus open access recommendations for physio-
chological, and social factors as stressors on neural function therapy for functional motor disorders are now available
and allow treatment aimed at restoring nervous system func- (Nielsen et al., 2014).
tion. The common criticism is that they are too broad and
open to confusion (Fahn and Olanow, 2014). One option is
to use a functional explanation by default and introduce dis- Psychological Treatment
cussion of psychological factors later if relevant or necessary.
Since up to one-third of all neurology outpatients have func-
Even an unspoken suspicion that most patients with func-
tional symptoms to some degree, it is unlikely they could all
tional symptoms are feigning will likely be picked up on,
have specialist psychological treatment, nor do they all need
regardless of what is said out loud.
it. Patients with mild symptoms may just need to be steered
There are many barriers to a successful explanation other
in the right direction, given sensible information, and they
than the words used. Patients with functional disorder have
will do the rest themselves. Patients who are struggling with
often gone through a phase themselves of wondering, Is it
disabling symptoms are likely to benefit from further treat-
me? because of the variability of the symptoms but, in con-
ment. Randomized controlled trials have shown benefit of a
trast, also experience feelings of being out of control. They can
cognitive behavioral treatment (CBT) over a 6-month period
therefore be particularly sensitive to a diagnosis that suggests
in nonepileptic attacks (Goldstein etal., 2010), a wider range
that they are doing it on purpose or in control. Even when
of functional (psychogenic) symptoms (Sharpe etal., 2011),
they are comfortable with the diagnosis of functional symp-
and in somatization disorder (Allen and Woolfolk, 2010).
toms, this is a hard thing to explain to friends, family, and
Other uncontrolled studies have shown similar treatment
employers. Neurologists for their part are often unsure what
effects for more broad-based psychotherapy in a range of func-
to think about this area of their practice and may prefer to
tional neurological symptoms (Reuber etal., 2007). But what
dodge the whole issue by only explaining that there is no
does (and can) a psychiatrist/psychologist actually do with
neurological disease (Friedman and LaFrance, 2010). Patient
patients with functional symptoms?
frustration is the inevitable upshot; they want to know what
is wrong with them, and not just what isnt. Further explanation. A psychiatrist or psychologist must be
familiar with the area and able to give the same kind of
explanation the patient has received from the neurologist.
Further Neurological Treatment This in itself may take some time.
Traditionally many neurologists have ceased their involve- Detection and treatment of comorbid psychiatric problems
ment after the diagnosis, handing care back to the family such as anxiety, depression, post-traumatic stress disorder,
doctor or referring on to a psychiatrist. However, neurologists or obsessive compulsive disorder.
can play an important role, with a second or further visit to Cognitive behavioral treatment. This involves developing the
reinforce the explanation and rationale for the diagnosis, stop patients diagnosis to change how they think about their
anticonvulsants in patients with dissociative attacks, and con- symptoms and behave as a consequence of them (Table
sider onward referral. If a thorough explanation has been 113.5). It is an approach based in learning theory and aims
offered, and the patient has been given some sources of self- to provide a detailed examination of the interactions
help yet returns with no idea what their diagnosis is, then it between physical symptoms, thoughts, behavior, and mood.
is likely they will be difficult to help. Conversely, the patient It applies a model that patients will gain immediate reward
who trusts his or her clinicians expertise will start to under- for their actions that influence future behavior; a patient
stand how physiotherapy or psychological therapy may be with back pain may rest at the first signs of exacerbation,
helpful in breaking established symptom patterns. removing the pain in the short term but leading to long-
term poorer function. Illness and other beliefs will also
feature; the patient may believe that acute exacerbation of
Physiotherapy their back pain is a sign of new damage and thus strive to
For a patient with a symptom like weakness, gait, or move- avoid this and become fearful of it. This can in turn result
ment disorder, there is increasing evidence that an approach in increased muscle tension and poor posture, making the
which focuses on changing physical function and which actual occurrence more likely. Such vicious circles are pos-
doesnt necessarily have to involve a talking therapy can be tulated as contributing to the genesis of functional symp-
effective. It is now our practice to refer eligible patients with toms, and the therapy aims to unpick them.
functional weakness or movement disorder for physiotherapy Psychodynamic psychotherapies have been historically
as a first-line treatment. A randomized controlled trial of popular in treatment of functional neurological symptoms
physiotherapy and other physically oriented programs have in neurology, but empirical support is lacking. There is,
shown sustained benefit even for patients with chronic symp- however, evidence of benefit of short psychodynamic psy-
toms (Czarnecki etal., 2012; Jordbru etal., 2014). Physiother- chotherapy in other somatic symptoms such as irritable
apy for patients with functional disorders does have specific bowel syndrome and chronic pain (Abbass etal., 2009).
features compared to those used in other neurological disor- Such therapy is based on the premise that symptom devel-
ders. For example, if someone has limb weakness, rather than opment occurs as a means of escape from an interpersonal
paying a lot of attention to the weak limb, as after a stroke, it conflict. These conflicts are seen in the context of
Downloaded for Departemen THT (thtrscm@indo.net.id) at Universitas Indonesia from ClinicalKey.com by Elsevier on October 20, 2017.
For personal use only. No other uses without permission. Copyright 2017. Elsevier Inc. All rights reserved.
Functional and Dissociative (Psychogenic) Neurological Symptoms 2007
TABLE 113.5 Examples of Changes in Thoughts and Behavior That Can Help Patients with Functional Symptoms
113
Dissociative attacks Functional weakness Chronic back pain
Old thought Oh no, whats happening to me? Am I Ive got MS, Im going to end up in a My spine is damaged. I must
going to die during one of these wheelchair. No one believes me. avoid moving too much in case
attacks? it makes it worse.
New thought Im having something a bit like a panic Hmm this is odd, but it looks as if I My bones are fine, its my
attack where Im losing control. can get better. That doctor is right. muscles that are stiff and out of
When Im not thinking about the leg, it condition.
does seem to move better.
Old behavior Avoid going out. Worry constantly about Seeing lots of specialists. Not doing very Avoiding exercise/back
attacks. much in case it makes it worse. movement.
New behavior Try out distraction techniques during Gradually exercise, trying not to focus on Gradually exercise, expecting
warning symptoms. limb weakness. Learn to expect exacerbations of pain.
relapses.
abnormally learned patterns of interpersonal relationships should have a wheelchair or go on to health-related finan-
during childhood, which then go on to distort social inter- cial benefits. The advice here is not really different from any
actions in adulthood. For example, a patient who has suf- other patient with a disability that may improve. Wheel-
fered an abusive upbringing may be prone to emotional chairs and sickness benefits definitely improve independ-
instability, anger, or passivity which determine their inter- ence and morale in some patients but can also create a
pretation and response to physical sensations as well as further obstacle to rehabilitation by discouraging day-to-day
their interactions with health professionals when seeking movement and an early return to work.
help for those symptoms. Therapy based on these principles Hypnosis. This treatment has a long association with func-
includes learning to recognize these subconscious maladap- tional neurological symptoms especially suited to func-
tive patterns, allowing for a more socially skilled approach tional motor symptoms. Two randomized controlled trials
to interpersonal conflict resolution, and discarding a pre- found it to be of benefit in patients with motor symptoms
sumed psychic need to develop physical symptoms. (Moene etal., 2003). Patients may be able to learn self-
hypnosis and other relaxation techniques.
Sedation. Patients with prolonged paralysis or fixed dysto-
Specific Advice for Dissociative nia may benefit from examination under sedation. Rather
(Nonepileptic) Attacks than quiz the patient (abreaction), we suggest that the
patient is held just at the point of anesthesia, to demon-
In addition to the approaches described, some additional strate (with video) better function under sedation than
helpful measures include: during wakefulness and to kick start some improvement
Stop anticonvulsant drugs. Studies have shown this is safe to (Video 113.6) (Stone etal., 2014). A secondary function of
do (Oto etal., 2005). Not stopping them sends a very mixed the procedure is to look for evidence of contractures in
message to the patient about how confident the physician patients with fixed dystonia. We find that it is important
is about the diagnosis. for the patient to be given physiotherapy immediately
Look carefully for warning symptoms and teach distraction afterwards if they have made some improvement during
techniques. As described earlier, symptoms of panic are the procedure.
common, although often not initially reported, in patients Transcranial magnetic stimulation (TMS). Some centers have
with dissociative (nonepileptic) attacks. Patients need help reported remarkable results using TMS not only in acute
recognizing and sometimes remembering these warning functional motor disorders (where improvement may have
symptoms and with distraction techniques to avert an occurred anyway) (Chastan and Parain, 2010) but also
attack. When they have averted just one attack or developed chronic patients (Garcin etal., 2013). Most authors agree
more awareness of shaking, this will reinforce their confi- that the effect is unlikely to relate to direct alteration of
dence in the diagnosis. neural pathways. Instead TMS provides a way for a patient
Recognize triggers for attacks. Patients commonly report rec- to see the potential reversibility of symptoms as well as the
ognizing no pattern at all to their attacks. However, they desire of the physician to help the patient. Peripheral nerve
may be helped to see that attacks are more likely in situa- stimulation is also associated with good anecdotal out-
tions where the consequences are especially embarrassing comes. Electrical stimulation for patients with these disor-
or inconvenient, like in shops or on stairs (because they ders is nothing new and has been a feature of medicine
have been worrying that the attack would happen), and since the early 1800s (Adrian and Yealland, 1917).
conversely when they are sitting quietly undistracted (when
trance-like symptoms are more likely to take hold). When Drug Treatment
they are highly distracted or focused on a task, they may be
less likely to occur. There is no good evidence to guide the use of antidepressant
drugs for patients with dissociative (nonepileptic) attacks or
motor and sensory symptoms. There is, however, good evi-
Specific Advice for Functional Motor Symptoms dence supporting the use of antidepressant drugs across a
range of other functional symptoms, with the balance of evi-
In addition to the approaches described, some additional
dence favoring the use of tricyclic drugs (OMalley etal.,
issues include:
1999). Outcomes do not appear to be affected by the presence
Physical aids and appliances/sickness benefits. Patients with dis- or absence of depressed mood. Where comorbid anxiety,
ability from functional symptoms may ask whether they depression, or panic is present, drug treatment can be
Downloaded for Departemen THT (thtrscm@indo.net.id) at Universitas Indonesia from ClinicalKey.com by Elsevier on October 20, 2017.
For personal use only. No other uses without permission. Copyright 2017. Elsevier Inc. All rights reserved.
2008 PART III Neurological Diseases and Their Treatment
discussed on its own merits. Similarly, there is a good evidence possible. The patients primary physician has an important
base for the use of tricyclic antidepressants in pain or insom- role in recognizing vulnerability to symptoms, treating inter-
nia in the absence of mood disorder. Patients with functional current mood and anxiety problems, and protecting them
symptoms do seem to be unusually sensitive to drug side from unnecessary investigations and treatments in secondary
effects, possibly via nocebo mechanisms. We advise any drugs care where possible.
be started at a low dose and increased slowly. It can be helpful
to caution the patient that they may experience side effects
over the first few weeks, but if they stick with it, these will
SUMMARY
often settle down. Functional and dissociative disorders in neurology are
common, disabling, and distressing. The diagnosis should be
made on the basis of positive physical signs or observation of
When Nothing Helps attacks, combined with a sound knowledge of neurological
diseasenot on psychological grounds. Neurologists are in a
Just because a patient has no disease does not mean they
good position to alter the illness trajectories of many patients
should automatically get better (see the earlier section Prog-
with a careful and rational explanation of the diagnosis and
nosis). Neurologists should bear in mind that only one in
onward appropriate referral.
three patientsor much less than that for symptoms like fixed
Sources of free self-help for patients, created by researchers,
dystoniawill improve spontaneously. It is important to
can be found online at:
maintain reasonable expectations of ones therapeutic abilities
in a situation where multiple powerful perpetuating factors http://www.neurosymptoms.org. Self-help material for all
may exist. If despite all best efforts, a patient does not really of the symptoms described in this chapter.
believe or is unable to understand the diagnosis, the neurolo- http://www.nonepilepticattacks.info. Free self-help material
gist has perhaps done everything possible in the circumstances. specifically about nonepileptic attacks.
Alternatively, a patient may be fully accepting of the diagnosis,
but it may be too difficult for anyone to help. Patients can be
told that they have done their best for the time being and that REFERENCES
their symptoms may improve in the future, but for now the The complete reference list is available online at https://expertconsult
management is to learn to manage the symptoms as much as .inkling.com/.
Downloaded for Departemen THT (thtrscm@indo.net.id) at Universitas Indonesia from ClinicalKey.com by Elsevier on October 20, 2017.
For personal use only. No other uses without permission. Copyright 2017. Elsevier Inc. All rights reserved.
Functional and Dissociative (Psychogenic) Neurological Symptoms 2008.e1
Downloaded for Departemen THT (thtrscm@indo.net.id) at Universitas Indonesia from ClinicalKey.com by Elsevier on October 20, 2017.
For personal use only. No other uses without permission. Copyright 2017. Elsevier Inc. All rights reserved.
2008.e2 PART III Neurological Diseases and Their Treatment
Nielsen, G., Stone, J., Edwards, M.J., 2013. Physiotherapy for func- Staniloiu, A., Markowitsch, H.J., Brand, M., 2010. Psychogenic
tional (psychogenic) motor symptoms: A systematic review. J. Psy- amnesiaa malady of the constricted self. Conscious. Cogn. 19,
chosom. Res. 75, 93102. 778801.
Nielsen, G., Stone, J., Matthews, A., etal., 2014. Physiotherapy for Stone, J., Carson, A., Duncan, R., etal., 2009. Symptoms unexplained
functional motor disorders: a consensus recommendation. J. by organic disease in 1144 new neurology out-patients: how often
Neurol. Neurosurg. Psychiatry 10.1136/jnnp-2014-309255. does the diagnosis change at follow-up? Brain 132, 28782888.
OMalley, P.G., Jackson, J.L., Santoro, J., etal., 1999. Antidepressant Stone, J., Carson, A., Duncan, R., etal., 2010a. Who is referred to
therapy for unexplained symptoms and symptom syndromes. neurology clinics?the diagnoses made in 3781 new patients. Clin.
J. Fam. Pract. 48, 980990. Neurol. Neurosurg. 112, 747751.
Oto, M., Espie, C., Pelosi, A., etal., 2005. The safety of antiepileptic Stone, J., Edwards, M., 2012. Trick or treat? Showing patients with
drug withdrawal in patients with non-epileptic seizures. J. Neurol. functional (psychogenic) motor symptoms their physical signs.
Neurosurg. Psychiatry 76, 16821685. Neurology 282284.
Pares, I., Kojovic, M., Pires, C., etal., 2014. Physical precipitating Stone, J., Hewett, R., Carson, A., etal., 2008. The disappearance of
factors in functional movement disorders. J. Neurol. Sci. 338, hysteria: historical mystery or illusion? J. R. Soc. Med. 101, 1218.
174177. Stone, J., Hoeritzauer, I., Brown, K., Carson, A., 2014. Therapeutic
Parees, I., Saifee, T.A., Kassavetis, P., etal., 2012. Believing is perceiv- sedation for functional (psychogenic) neurological symptoms.
ing: mismatch between self-report and actigraphy in psychogenic J. Psychosom. Res. 76, 165168.
tremor. Brain 135, 117123. Stone, J., LaFrance, W.C., Brown, R., etal., 2011. Conversion disorder:
Petrie, K.J., Muller, J.T., Schirmbeck, F., etal., 2007. Effect of providing current problems and potential solutions for DSM-5. J. Psychosom.
information about normal test results on patients reassurance: ran- Res. 71, 369376.
domised controlled trial. BMJ 334, 352. Stone, J., Reuber, M., Carson, A., 2013. Functional symptoms in neu-
Reuber, M., Burness, C., Howlett, S., etal., 2007. Tailored psycho- rology: mimics and chameleons. Pract. Neurol. 13, 104113.
therapy for patients with functional neurological symptoms: a pilot Stone, J., Smyth, R., Carson, A., etal., 2005. Systematic review of
study. J. Psychosom. Res. 63, 625632. misdiagnosis of conversion symptoms and hysteria. BMJ 331, 989.
Reuber, M., Monzoni, C., Sharrack, B., Plug, L., 2009. Using interac- Stone, J., Smyth, R., Carson, A., etal., 2006. La belle indiffrence in
tional and linguistic analysis to distinguish between epileptic and conversion symptoms and hysteria: systematic review. Br. J. Psychia-
psychogenic nonepileptic seizures: a prospective, blinded multirater try 188, 204209.
study. Epilepsy Behav. 16, 139144. Stone, J., Warlow, C., Sharpe, M., 2010b. The symptom of functional
Rommel, O., Gehling, M., Dertwinkel, R., etal., 1999. Hemisensory weakness: a controlled study of 107 patients. Brain 133,
impairment in patients with complex regional pain syndrome. Pain 15371551.
80, 95101. Stone, J., Warlow, C., Sharpe, M., 2012. Functional weakness: clues to
Ruotsalainen, J., Sellman, J., Lehto, L., Verbeek, J., 2008. Systematic mechanism from the nature of onset. J. Neurol. Neurosurg. Psychia-
review of the treatment of functional dysphonia and prevention of try 83, 6769.
voice disorders. Otolaryngol. Head Neck Surg. 138, 557565. Thenganatt, M.A., Jankovic, J., 2014. Psychogenic tremor: A video
Rika, E., Zrubov, K., Nutt, J.G., Bloem, B.R., 2011. Silly walks guide to its distinguishing features. Tremor. Other Hyperkinet. Mov.
in Parkinsons disease: unusual presentation of dopaminergic- (N Y) 4, 253.
induced dyskinesias. Mov. Disord. 26, 17821784. Thenganatt, M.A., Jankovic, J., 2015. Psychogenic movement disor-
Schacter, S., LaFrance, W.C. Jr., 2010. Gates and Rowans Nonepileptic ders. Neurol. Clin. 33, 205224.
Seizures, third ed. Cambridge University Press, Cambridge. Van der Salm, S.M., Koelman, J.H., Henneke, S., etal., 2010. Axial
Schmidtke, K., Pohlmann, S., Metternich, B., 2008. The syndrome jerks: a clinical spectrum ranging from propriospinal to psychogenic
of functional memory disorder: definition, etiology, and natural myoclonus. J. Neurol. 257, 13491355.
course. Am. J. Geriatr. Psychiatry 16, 981988. Van der Salm, S.M., Tijssen, M.A., Koelman, J.H., van Rootselaar, A.-F.,
Schrag, A., Trimble, M., Quinn, N., Bhatia, K., 2004. The syndrome 2012. The bereitschaftspotential in jerky movement disorders. J.
of fixed dystonia: an evaluation of 103 patients. Brain 127, Neurol. Neurosurg. Psychiatry 83, 11621167.
23602372. Voon, V., Gallea, C., Hattori, N., etal., 2010. The involuntary nature
Selleslagh, M., van Oudenhove, L., Pauwels, A., etal., 2014. The com- of conversion disorder. Neurology 74, 223228.
plexity of globus: a multidisciplinary perspective. Nat. Rev. Gastro- Vuilleumier, P., Chicherio, C., Assal, F., etal., 2001. Functional neuro-
enterol. Hepatol. 11, 220233. anatomical correlates of hysterical sensorimotor loss. Brain 124,
Sharpe, M., Stone, J., Hibberd, C., etal., 2010. Neurology out-patients 10771090.
with symptoms unexplained by disease: illness beliefs and financial Waddell, G., 2004. The Back Pain Revolution. Churchill Livingstone,
benefits predict 1-year outcome. Psychol. Med. 40, 689698. Edinburgh.
Sharpe, M., Walker, J., Williams, C., etal., 2011. Guided self-help for White, P.D., Goldsmith, K.A., Johnson, A.L., etal., 2011. Comparison
functional (psychogenic) symptoms: a randomized controlled effi- of adaptive pacing therapy, cognitive behaviour therapy, graded
cacy trial. Neurology 77, 564572. exercise therapy, and specialist medical care for chronic fatigue
Simon, G., Gater, R., Kisely, S., Piccinelli, M., 1996. Somatic symptoms syndrome (PACE): a randomised trial. Lancet 377, 823836.
of distress: an international primary care study. Psychosom. Med.
58, 481488.
Downloaded for Departemen THT (thtrscm@indo.net.id) at Universitas Indonesia from ClinicalKey.com by Elsevier on October 20, 2017.
For personal use only. No other uses without permission. Copyright 2017. Elsevier Inc. All rights reserved.