Functional symptoms in neurology

Transcription

1 Neurology in Pratie THE BARE ESSENTIALS Funtional symptoms in neurology Jon Stone Correspondene to: Dr J Stone, Consultant Neurologist and Honorary Senior Leturer in Neurology, Department of Clinial Neurosienes, Western General Hospital, Edinburgh EH4 2XU, UK; Jon.Stone@ed.a.uk IS IT YOUR JOB AS A NEUROLOGIST TO DEAL WITH THIS PROBLEM? If you find people with neurologial symptoms but no disease tiresome and not really what you ame in to the speialty for, then you are going to find large parts of your job tiresome and worse your attitude will filter through in a negative way to the patients regardless of the form of words you use to talk to them. On the other hand, if you allow yourself to be interested by the omplexity of the problem and an see the potential for benefit that you, as a neurologist, an make to some patients then you may disover that this is a worthwhile area in whih to improve your knowledge and skills. TERMINOLOGY None of the urrent terms is perfet. It is best to hoose words based on (a) how you see the ause or mehanism of the symptoms and (b) how this affets your ability to ommuniate the diagnosis helpfully to the patients (preferably also inluding opying your lini letter to them). Ultimately the label is not as important as the neurologist s attitude to the patient. Psyhiatri terminology Dissoiative seizure/motor disorder (onversion disorder) (ICD-10) suggests dissoiation as an important mehanism in symptom prodution, whih is true for many patients but not all (see below). Dissoiation has many meanings but in this ontext often refers to two partiular experienes: depersonalisation, a feeling of disonnetion from one s own body, and derealisation, a feeling of disonnetion from one s environment (see Clinial approah, below). Conversion disorder (DSM-IV) is a reli of Freudian psyhoanalyti theory in the Amerian bible of psyhiatry (DSM) based on the idea of onversion of mental distress to physial symptoms. It is defined as: a motor or sensory symptom or blakouts not ompatible with disease whih is not thought to be onsiously manufatured whih auses distress and is related to psyhologial fators. Criteria 2 and 4 are unworkable in linial pratie and the definition is in need of hange. Somatisation disorder (DSM-IV) is applied to a patient with a longstanding tendeny to have symptoms unexplained by disease, usually starting before the age of 30. It is somewhat arbitrarily defined as someone who has arued at least one onversion symptom, four pain symptoms, two gastrointestinal symptoms (usually irritable bowel syndrome) and 1 sexual symptom (dyspareunia, dysmenorrhoea or hyperemesis gravidarum indiating that women an be labelled with this more easily). Hypohondriasis means exessive and intrusive health anxiety about the possibility of serious disease whih the patient has trouble ontrolling. Typially the patient seeks repeated medial reassurane but this only has a short-lived effet; in this situation it is a form of addition whih an only be overome by a better explanation for symptoms, and ultimately disussion of health anxiety itself. Fatitious disorder means that symptoms are onsiously fabriated for the purpose of medial are (not money). These patients often have a personality disorder. Munhausen syndrome desribes someone with fatitious disorder who wanders between hospitals, typially hanging their name and story. Malingering is not a psyhiatri diagnosis but means making up symptoms for material gain (eg, benefit fraud). Other terminology Funtional implies in the broadest possible sense a problem due to a hange in funtion (of the nervous system) rather than struture. Non-organi, non-epilepti et all have the problem of desribing what the problem is not, rather than what it is. Psyhosomati is supposed to mean an interation between mind and body but in pratie is interpreted in the same way as somatisation, the psyhologial influene on the body. Psyhogeni suggests an entirely psyhologial explanation for symptoms. Medially unexplained is a neutral term but one that patients may easily interpret as the dotor not knowing what the diagnosis is (rather than not knowing why they have the problem). Like many neurologial diseases (eg, 2009;9: doi: /jnnp

2 Neurology in Pratie multiple slerosis, motor neuron disease, migraine) we an diagnose funtional symptoms without knowing why the patient has them. Abnormal illness behaviour is a term suggesting behaviour out of keeping with the severity of the illness, rather than the normal sort of illness behaviour that we all have when we have, for example, flu. As it is not lear what a normal response to funtional problems should be, I personally find this an ambiguous and unhelpful term. Hysteria is an anient term originating from the idea of the wandering womb. As Aubrey Lewis pointed out, it has frequently outlived its obituarists. The terms that I personally prefer for motor, sensory symptoms and blakouts unexplained by disease are funtional and dissoiative beause they: desribe a mehanism and not an aetiology sidestep an illogial debate about whether symptoms are in the mind or the brain map onto newer findings from funtional imaging studies allow for the possibility of improvement an be used easily with patients. For simpliity I will use the term funtional symptoms in this artile. FUNCTIONAL SYMPTOMS IN MEDICINE These aount for around one third of all patients seen in hospital medial linis (box 1). Many of these problems overlap substantially with eah other in terms of symptoms, aetiologial fators and response to treatment. Lumping these problems together is helpful from one perspetive, but it is also important to appreiate that there is a lot of heterogeneity within them and that patients with the same symptoms may have entirely different reasons for developing them. Box 1 Funtional symptoms in medial speialities Gastroenterology: irritable bowel syndrome (IBS) Endorinology: borderline thyroid funtion Respiratory: hroni ough, brittle asthma Rheumatology: fibromyalgia, hroni bak pain (some) Gynaeology: hroni pelvi pain, dysmenorrhoea (some) Cardiology: atypial/non-ardia hest pain, palpitations (some) Infetious diseases: (post-viral) hroni fatigue syndrome (CFS/ME) Ear, nose and throat: globus, funtional dysphonia Neurology: dissoiative seizures, funtional weakness and sensory symptoms FREQUENCY OF FUNCTIONAL SYMPTOMS IN NEUROLOGY Neurology outpatients About 50% have a funtional symptom/somatoform problem of some kind, even if it is not their main problem. About 30% of new neurology outpatients have main presenting symptoms that are only somewhat or not at all explained by disease. This inludes patients with neurologial disease and funtional overlay. About 15% have a primary funtional/psyhologial diagnosis (inluding pain and fatigue unexplained by disease). About 5% have seizures, weakness, sensory symptoms or movement disorder whih is thought by the neurologist to be funtional/ non-organi (sometimes alled onversion symptoms ). On average new neurology patients with funtional symptoms are just as disabled as and even more distressed than those with a neurologial disease. Neurology inpatients 1 10% of neurology inpatients have a primary funtional diagnosis. Conversion symptoms Dissoiative/non-epilepti seizures aount for about 20% of referrals to first fit linis and to speialist epilepsy linis. Up to 50% of admissions to hospital with status epileptius are in fat dissoiative seizures/ pseudostatus. Funtional weakness has an annual inidene of at least 5/ , similar to multiple slerosis. Funtional movement disorders aount for 5 10% of patients seen in a movement disorders lini. CLINICAL APPROACH TO THE PATIENT WITH FUNCTIONAL SYMPTOMS In a 30 minute appointment, the prospet of a talkative patient with 20 symptoms and three volumes of notes an be daunting. The following are some tips to help you ope. Things to do List all the urrent symptoms at the beginning. Say that you will ome bak to eah one later. Ask everyone about fatigue, pain, sleep and onentration. Avoid desriptions of past events at this stage get to the end of the list first. The more symptoms the patient has the more likely it is that the primary symptom will not be due to a reognised disease ;9: doi: /jnnp

3 Neurology in Pratie Find out what the patient an do. The patient may be keener to tell you what they an no longer do but it may be more revealing to ask how he or she does spend their time. Are there good days and bad days? Ask about onset and time ourse. If the onset was sudden, look arefully for symptoms of dissoiation or pani. Triggers may be physial (injury, infetion, disease), psyhologial (pani attak, speifi thoughts, depressive illness), soial (life stress) or not present at all (a random event). Look for dissoiation Derealisation is a feeling that the world around is unreal, disonneted, far away. The patient may feel alone or in a plae of their own. Depersonalisation is a feeling of detahment or disonnetion from ones own body. Patients may say that their body doesn t feel part of them or feels distorted. Dissoiative symptoms are ommon in pani attaks and persistent fatigue but an be experiened in isolation. They may our briefly as part of epilepsy and migraine. They are espeially ommon in patients with dissoiative seizures (non-epilepti attaks) and in patients with sudden onset funtional motor symptoms. Patients are rarely able/willing to desribe these symptoms spontaneously, partly beause they don t know what words to use but also beause they fear that they will be thought to be losing their mind. So you will often have to ask diret questions to reveal these symptoms. If someone desribes dizziness or spinning they may atually be desribing dissoiative symptoms. Ask about previous funtional symptoms/ syndromes. As desribed above, the more of these there are, the more likely it is that the presenting symptom is also funtional. Ask what the patient thinks is wrong. Does he or she have any thoughts about why they have these symptoms? If not, what do family or friends think? If they (or their family) are onvined they have multiple slerosis or Lyme disease or rumbling bones in the spine you need to know this, so you an tailor your later explanation. If they think it s all psyhologial this is a little unusual but does sometimes happen (espeially in primary are). Find out why the patient is in your lini. Try to deide to what extent the patients are anxious about their symptoms (health anxiety; see above) and to what extent they just have the symptoms and want to tell you about them. Do they atually want you to try to get them better or are they just there beause their general pratitioner sent them? What do they think will help? If a patient says nothing then they are usually diffiult to help. Ask what happened with other dotors. What did the last dotor say? If they tell you angrily that Dr X said it was all in their mind, then this tells you that your patient will be sensitive to disussions about psyhologial fators. Show early familiarity with the problem. If possible, make the patient aware early on that their 27 symptoms are all familiar and that you have not automatially assumed they are mad. Use humour where possible and appropriate. Things to wath out for Don t believe all the physial diagnoses in the medial notes. They may not be orret. Asthma may be pani disorder, the appendix or uterus may have been normal even though surgially removed. Don t wade in early with blunt questions about depression or anxiety. You will probably just annoy the patient without finding out anything useful (see below). Don t make a diagnosis of funtional symptoms beause someone has an obvious psyhiatri problem/personality disorder. Patients with psyhosis are not espeially liable to funtional symptoms, and patients with any psyhiatri disorder may be harbouring a neurologial disease. Don t avoid a diagnosis of a funtional problem beause someone seems too normal. Normal people, inluding those with no depression/anxiety or previous history an get funtional symptoms too (even neurologists!). Don t misinterpret exaggeration to onvine as exaggeration to deeive. The patient who groans and sighs in an exessive way is more likely to be doing so to show you how bad their symptoms are (when they really do have them) rather than making up their symptoms from srath in an attempt to deeive you Looking for depression, pani and anxiety Leave this until the end of the history unless the patient volunteers it. Patients with funtional symptoms may not have depression, pani or anxiety. Look at the patient, are they agitated or miserable? Do they have poor eye ontat that might suggest depression? Depression (major depression DSM-IV) is defined as five of the following nine persisting and marked symptoms for two weeks (inluding one of the first two): low mood most of the time, loss of interest/ pleasure in most things (anhedonia), hange in weight/appetite, agitation/slowing down, fatigue, sleep disturbane, guilt/worthlessness, redued onentration, suiidal ideation. Ask Is there anything you an still enjoy despite having all these symptoms? (anhedonia). Loss of libido is a useful question, espeially for men. 2009;9: doi: /jnnp

4 Neurology in Pratie For low mood instead of asking Are you depressed? try Do your symptoms get you down? with neurologial disease are sometimes surprisingly heerful too. Pani attak symptoms are defined in DSM-IV as four of the following: palpitations, sweating, trembling/shaking, shortness of breath, hoking sensation, hest pain/pressure, nausea/feeling of imminent diarrhoea, dizziness, derealisation/depersonalisation, afraid of going razy/losing ontrol, afraid of dying, tingling, flushes/hills. Generalised anxiety (DSM-IV) is defined as persistent worry that the person finds hard to ontrol for over six months in ombination with three of the following six symptoms: restlessness/ on edge, insomnia, fatigue, irritability, poor onentration, tense musles. If you suspet pani/anxiety attaks, ask Do you ever get symptoms all at one? What happens? Is it frightening? Does it make you feel as if you re going to die/lose ontrol? Patients with severe pani attaks often don t know or aept that s what they are having that s why the attaks are so frightening. Asking about other life events/hildhood adversity The frequeny of preeding hildhood and adult sexual and physial trauma may be higher (eg, dissoiative seizures 20 30%, weakness 10 20%) than in the general population (5 10%) but still only applies to a minority of patients. The ombination of previous selfharm and multiple somati symptoms make a history of sexual abuse muh more likely. Childhood emotional neglet may sometimes be important in the absene of physial/sexual abuse. Getting patients to dislose trauma in the first or early onsultations has not been shown to be therapeuti and neither is it neessary for the important early steps of treatment, even if it is relevant. Therefore it is usually not neessary to explore this early on with the patient. Do not be put off by a lak of reent life events. There may not be any. Examination Make a diagnosis based on the presene of positive physial signs or a familiar pattern of symptoms, not beause tests are normal, the problem looks bizarre or the patient has a psyhiatri history. Positive physial evidene of a funtional problem only tells you that there is a funtional problem. There may also be a neurologial disease. La belle indifferene, smiling indifferene to disability, is of no diagnosti value. It may mean that the patient is fearful and upset but trying hard to put on a brave fae (usually), or has a fatitious disorder (rarely). Patients ASSESSMENT OF SPECIFIC SYMPTOMS Dissoiative seizures In younger patients females predominate 3:1. In middle aged and older patients the male:female ratio is 1:1 and there is often a history of health anxiety, espeially worry about ardia problems. Semiology,70% thrashing,,25% fall down lie still,,5% other (table 1). Movements during a thrashing dissoiative seizure are a form of severe tremor rather than loni movements, typially there is no isolated toni phase. Very few onditions (other than death) lead to a state of suddenly falling down and lying still for over five minutes. Subjetive symptoms Patients with dissoiative seizures typially keep their desriptions minimal, desribing where they were and what happened afterwards but with no desription of prodromal symptoms, and no language to desribe the seizures themselves. They may turn to ask their relative what happened at an early stage. Patients with epilepsy make muh more effort to desribe the nature of the warning and tend to desribe seizures as an external fore. One reason for the relutane of patients with dissoiative seizures to talk about their attaks is that they have had a prodrome, typially of rising anxiety, with dissoiative and autonomi arousal symptoms but they either an t or don t want to remember it or disuss it. They may need enouragement to desribe these symptoms whih may only emerge at later visits. Patients with dissoiative seizures may (when they trust you) also admit that they welome the blakout as it provides a means of esape from these very distressing warning symptoms. Finding a prodrome is of ruial importane in treatment (and is relevant for other aute funtional symptoms in neurology too) but is not always possible. Investigations Video EEG is the gold standard 50% will have an attak during a short video EEG with suggestion, espeially those who have attaks in medial situations. Chek you have reorded their usual seizure. A normal surfae ital EEG does not exlude epilepsy. As well as a normal EEG have you seen a typial dissoiative attak on the video, one that onvines you that it is not epilepsy? ;9: doi: /jnnp

5 Neurology in Pratie Table 1 Dissoiative versus epilepti seizures, helpful and less helpful distinguishing features Distinguishing feature Dissoiative seizures Epilepti seizures Helpful Duration over 5 min Common Rare Gradual onset Common Rare Flutuating ourse Common Rare Eyes and mouth losed Common Rare Resisting eye opening Common Very rare Thrashing, violent movements Common Rare Side-to-side head movement Common Rare Opisthotonus, ar de erle Oasional Very rare Visible large bite mark on side of tongue Rare Oasional Disloated shoulder Rare Oasional Respiration Often fast Ceases Grunting sounds Oasional Common Reall for period of unresponsiveness Common Very rare Weeping/upset after a seizure Oasional Rare{ Not so helpful Stereotyped attaks Common Common Attak arising from sleep Oasional Common Aura Common Common Inontinene of urine or faees Oasional Common Injury* Common* Common Report of tongue biting Oasional Common Pelvi thrusting Oasional Oasional{ *Espeially arpet burns and bruising; {frontal lobe epilepsy; {normally sleepy. Ask a relative, friend or arer to video an attak, perhaps using a mobile phone. Serum prolatin measurement has many pitfalls. Prolatin has been found to be elevated rarely after dissoiative seizures (and synope). Its use is therefore delining. Rarer auses eg, insulinoma, paroxysmal dyskinesia. Weakness Half start suddenly, half more gradually often with pain or fatigue. If sudden onset, may arise after a physial injury, dissoiative seizure, from sleep paralysis or general anaestheti. Look for evidene of inonsisteny a limb that appears weak but then moves normally in another irumstane, eg: Diagnosti pitfalls Coexistent epilepsy is present in 5 20% of those with dissoiative seizures. Frontal lobe epilepsy may present as weird attaks but usually their brevity should alert one to epilepsy. Pani/fear as part of temporal lobe seizures. Sleep disorders suh as REM sleep behaviour disorder. Figure 1 Hoover s sign. weakness on the bed (for example of plantar flexion) that is inonsistent with abilities when walking (for example walking on tiptoes). observe the patient outside the formal examination oming in and out of the onsulting room, getting dressed/undressed, reahing for mediation lists in bags, et. Hoover s sign (weakness of hip extension whih returns to normal with ontralateral hip flexion against resistane, often easier to demonstrate in the seated position rather than lying down (fig 1)); onsider showing Hoover s sign to the patient and their family (eg, This test shows that when you are trying to make the movement, your brain is not sending the message properly to your leg, but when I test your other leg you an see that it omes bak to normal so the nervous system an t be damaged ). May be false positive in patients with neglet and in patients with a lot of limb pain. 2009;9: doi: /jnnp

6 Neurology in Pratie A similar finding of hip abdution weakness whih returns to normal with ontralateral hip abdution against resistane may also be helpful. most patients with funtional sensory symptoms tend to have a mild degree of funtional weakness whih makes it possible to base the diagnosis on positive signs (of weakness). A gait in whih the leg is dragged with the hip internally or externally rotated, with the foot dragging along the ground. A give-way quality to the weakness whih an be enouraged briefly to normal (eg, at the ount of three, push push ). An inverted pyramidal pattern of weakness in the legs (ie, extensors weaker than flexors). A limb that when left suspended in the air may hover for a fration of a seond before ollapsing. Faial weakness: there may be an appearane of ptosis when the problem is atually one of overontration of orbiularis oulis (usually in assoiation with photophobia). In the lower fae the mouth is sometimes pulled down by overontration of platysma giving an appearane of lower faial weakness. Sensory symptoms Patterns of sensory disturbane inlude: Feeling split in half down one side of the body, with altered temperature, vibration sense and light touh down the affeted side (be areful, this also ours in thalami lesions). There is invariably mild funtional weakness in assoiation with this, and sometimes ipsilateral diminished hearing and vision too. Limb sensory symptoms that stop at the groin or the shoulder. The patient may be less tiklish on the affeted foot (and the plantar response may be orrespondingly rather mute). Tests of funtional sensory symptoms are desribed inluding: Movement disorders These often start rather suddenly, espeially after physial injury. This is an area where getting the funtional diagnosis wrong is more likely Tremor Disappears with distration eg, ounting bakwards in sevens. Tremor of Parkinson s disease may be more notieable during distration. Variable frequeny is more helpful than variable amplitude. Entrainment ask the patient to make a rhythmial movement with their good side. They will either not be able to do it (and be unable to explain why) or the rhythm will entrain to the same rhythm as the affeted limb. Alteration with weight or attempted immobilisation funtional tremor typially worsens when an arm is weighted or if an examiner attempts to make it still by holding on to it. Dystonia Typially fixed with a lenhed fist or an inverted and plantarflexed foot (fig 2). Usually assoiated with pain, and often a omplex regional pain type 1 piture, whih usually arises after a minor injury. There is debate about the extent to whih fixed dystonia is a funtional disorder. Patients who have been ured with hypnosis or sedation indiate that this debate needs to ontinue. Figure 2 dystonia. Fixed Altered vibration sense aross either side of the forehead Say yes when you feel it and no when you don t or lose your eyes and touh your nose when I touh your hand However, studies have found that these are also ommon in patients with neurologial disease and so none an be onsidered reliable. Fortunately Gait disorders Astasia-abasia inability to stand and walk despite normal power on the bed. Dragging leg gait (as above). Crouhing gait with uneonomial movement (often a patient who is frightened of falling and wants to be loser to the ground). A tightrope-walkers gait with arms outstrethed typified by the patient falling in to the relative s or examiner s arms. Be areful like movement disorders, gait disorders may be mistaken as funtional when in fat they are organi. Pain Symptoms and signs of bak pain behaviour whih an be useful markers of a pain syndrome where funtional symptoms predominate inlude: Bak pain on simulated testing ;9: doi: /jnnp

7 Neurology in Pratie Rotation. Ask the patient to stand with their feet planted on the ground and swing their whole body. Pain in the bak is at odds with the manoeuvre whih does not really mobilise the bak. Axial loading. Low bak pain on pressing on the head. Dysphonia typially a whispering speeh pattern. Drop attaks in young people an be a form of dissoiative attak without loss of onsiousness, typially with overwhelming fear of falling. Superfiial tenderness extreme pain from light pressure over a wide area of the bak Inonsisteny in pain response eg, a patient with very painful straight leg raising lying flat who is able to sit up omfortably at 90u on a ouh with legs outstrethed. Fatigue Fatigue is the ommonest symptom in assoiation with other funtional neurologial symptoms. Chroni fatigue syndrome (also referred to in UK government douments as ME or myalgi enephalomyelitis)) an be diagnosed in a patient with disabling fatigue lasting longer than six months in the absene of another ause. Cognitive symptoms Some absent-mindedness is entirely normal eg, putting keys in the fridge, going upstairs and forgetting why but may be interpreted as early dementia by people with health anxiety, hene their appearane in your lini. As a symptom of anxiety or depression poor onentration seen in depression or anxiety may be lathed on to by a patient as the primary symptom. A psyhiatrist used to deteting anxiety or depression in these situations may be required. Pure retrograde amnesia the patient reports prolonged retrograde memory loss with normal anterograde memory. This overlaps with fugue states and may be assoiated with a desire to return to a previous time in life. INVESTIGATIONS Investigations like an MR brain san in patients with funtional symptoms are often unavoidable beause: you may not be sure yourself that there is no additional disease proess you know that the general pratitioner or patient will find the diagnosis hard to believe without negative investigations. Performing any investigation an promote an unhelpful feeling of diagnosti unertainty in the patient regardless of what you say to him or her; minimise this by doing all the tests as soon as possible, prediting that the tests will be normal and being very expliit about why you are doing them. Although tests may be neessary, the neurologist must, wherever possible, eventually have the ourage to make a lear diagnosis and draw a firm line under investigations. Patients need to be warned about the hane (10 15%) of inidental findings on MR brain sanning (inreasing with age), and that after age 30 most people have age related hange in their spine (just as a lot of people start getting grey hair). Remember that normal MR brain imaging does not exlude neurologial disease and that a few white dots annot explain a positive Hoover s sign. For dissoiative seizures a negative video EEG an be helpful in persuading the patient that you have the right diagnosis. Visual symptoms Complete blindness look for normal pupillary reation or optokineti nystagmus. Be areful of ortial blindness. Redued vision often ipsilateral to any hemisensory disturbane. Look for a tubular visual field by examining fields to onfrontation at the bedside lose (eg, 30 m) and far away (eg, 150 m). There may be spiral or pinpoint fields on Goldmann perimetry. Diplopia may be due to onvergent spasm of one or both eyes whih sometimes looks like a sixth nerve palsy. Beware midbrain lesions. Other funtional symptoms seen in neurology Globus a sensation of something being stuk in the throat, typially not when swallowing. ARE THE PATIENTS MAKING IT UP? While neurologists may be onerned that they are being hoodwinked by some patients, patients are frequently onerned that no-one believes them (and sometimes are seretly worried themselves that they are doing it on purpose, even when they are not). Distinguishing symptoms under voluntary ontrol from symptoms whih are not is linially diffiult beause: inonsisteny on examination will be found in both it is not a blak and white question, there is probably a spetrum between the two where someone is on that spetrum an hange over time some patients may be in a state of selfdeeption dotors are not trained to detet deeption. 2009;9: doi: /jnnp

8 Neurology in Pratie Patients simulating symptoms solely in order to obtain medial are (fatitious disorder) are akin to someone with severe self-harming behaviour. They will be found by definition in hospital linis but are generally thought to represent no more than 5% of patients with funtional symptoms. Patients with malingering are more likely to be seen in mediolegal senarios. The main lues to malingering or fatitious disorder are: inonsisteny in the history on different oasions (between dotors or between relative and patient) an admission from the patient that they have lied about other things in the past avoidane of tests a diret onfession evidene of gross inonsisteny from overt surveillane (for example a wheelhair patient who is seen playing tennis) simulation of symptoms that mimis disease very losely (for example a patient who has toni then loni movements in their seizure rather than just shaking). In favour of the idea that most patients with funtional symptoms are not malingering/fatitious are: follow-up studies finding that most patients remain symptomati and disabled in the long term the similarities in the way patients desribe their symptoms and their onfusion/frustration with their symptoms the keenness of most patients to pursue investigations (eg, have seizures during telemetry) ompliane with mediation by patients with dissoiative seizures, evidene of shoe wear marks in patients with gait problems. Paget s observation of 1873 is still relevant: The patient says he annot, it looks like he will not, but the truth is that the patient annot will. MISDIAGNOSIS Misdiagnosis for onversion symptoms in studies sine 1970 has on average been around only 4% at 5 years. This is the same as for other neurologial and psyhiatri onditions suh as multiple slerosis and shizophrenia. Gait disorders, movement disorders, frontal lobe epilepsy, psyhiatri presentations of multiple slerosis, oexisting disease and funtional symptoms, and patients with obvious psyhiatri problems/reent life events are over-represented in ases when neurologists do get it wrong. PROGNOSIS Patients with dissoiative seizures, funtional weakness and outpatients with milder symptoms as few as one third beome seizure or symptom-free after several years follow-up. It may be that work status or a measure of disability is a better measure of outome. Poor prognosti fators strong beliefs in lak of reversibility of symptoms/damage, anger at the diagnosis of a non-organi disorder, delayed diagnosis, multiple other physial symptoms/somatisation disorder, onurrent organi disease, personality disorder, older age, sexual abuse, reeipt of finanial benefits, litigation. Good prognosti fators willingness to aept reversibility/self-help, young, reent diagnosis, lak of other physial symptoms, hange in marital status (divore/marriage) after diagnosis, onurrent anxiety/depression. In pratie it an be surprising to find that patients with a host of poor prognosti fators an do well and vie versa. AETIOLOGY There is no ommon aetiology for all patients. Severity also varies enormously Formulate an individual patient s problems using table 2 but be willing to aept there are unknowables there too. Funtional imaging and ognitive neuropsyhologial studies of patients with funtional paralysis do not yet provide a onvergent model but suggest an altered brain state in whih there may be a ombination of altered and overative premotor areas and hypoative thalami areas MANAGEMENT Explanation This an go a long way. Sometimes a single good explanation is suffiient for reovery. However, the way you say things is probably more important than the terminology you use. There is no one size fits all and no right way to do it. Nonetheless, there do seem to be some important ingredients towards a suessful explanation (table 3). Like ooking or skiing though, you need to pratise and to want to do it better next time. Follow-up neurology visit What is the patient s understanding of the problem? What did they think of the lini letter/leaflet? If they flatly disbelieve your diagnosis or have no memory of the letter or leaflet then further treatment is unlikely to be worthwhile. If they are making some effort to understand it but need more help, then onsider how you are going to supply this. If the patient now feels believed, they may be notieably less defensive about emotional symptoms and important life events, often mentioning these things spontaneously. Disuss how they are going to tell friends/ employers about their illness. Close friends/ ;9: doi: /jnnp

9 Neurology in Pratie Table 2 A range of potential aetiologial fators in patients with funtional symptoms Fators Biologial Psyhologial Soial Ating at all stages Organi disease Emotional disorder Soioeonomi/deprivation History of previous funtional symptoms Personality disorder Life events and diffiulties Predisposing Geneti fators affeting personality Pereption of hildhood experiene as adverse Childhood neglet/abuse Biologial vulnerabilities in the nervous system? Personality traits Poor family funtioning Poor attahment/oping style Symptom modelling (via media or personal ontat) Preipitating Abnormal physiologial event or state (eg, Pereption of life event as negative, unexpeted hyperventilation, sleep deprivation, sleep paralysis) Physial injury/pain Aute dissoiative episode/pani attak Perpetuating Plastiity in CNS motor and sensory (inluding pain) pathways Deonditioning Neuroendorine and immunologial abnormalities similar to those seen in depression and anxiety Illness beliefs (patient and family) Pereption of symptoms as being due to disease/ damage/outwith the sope of self-help Not feeling believed Avoidane of symptom provoation (eg, by learned onditioning) The presene of a welfare system Soial benefits of being ill Availability of legal ompensation Stigma of mental illness in soiety and from medial profession Ongoing medial investigations and unertainty family may need to see some written information, espeially if it stops them paniking about dissoiative seizures. Nosey olleagues at work may be told that they are seeing speialists at the hospital for neurologial symptoms whih they hope will slowly improve. Insurane ompanies may need to have the diagnosis spelled out aording to reognised ICD-10 or DSM-IV lassifiations Consider the use of further self-help material eg, the website written by the author of this hapter. Referral to psyhiatry/psyhology Any patient with disabling symptoms who is not showing early signs of improvement may benefit from further psyhiatri/psyhologial assessment. You should preferably refer to a psyhiatrist/ psyhologist with experiene in this area (eg, liaison psyhiatrist if available). Referral to a general psyhiatrist with little experiene may be ounterprodutive. Cognitive behavioural therapy (CBT) is the most popular psyhologial approah although there is room for other forms of therapy, espeially in younger patients, if these resoures are available. What is ognitive behavioural therapy? The basi priniple is not ompliated it just means helping people hange the way they think and behave. Table 4 shows examples of hanges in thought and behaviour that neurologists themselves an help with during the onsultation. Table 3 Ingredient Ingredients of a suessful explanation for patients with funtional symptoms Example Explain what they do have Emphasise the mehanism of the symptoms rather than the ause Explain how you made the diagnosis Explain what they don t have Indiate that you believe them Emphasise that it is ommon Emphasise reversibility Emphasise that self-help is a key part of getting better Metaphors may be useful Introduing the role of depression/anxiety Use written information Stop the antiepilepti drug in dissoiative seizures Suggesting antidepressants Making the psyhiatri referral Involve the family/friends You have funtional weakness You have dissoiative seizures Weakness: Your nervous system is not damaged but it is not funtioning properly Seizures: You are going into a trane-like state a bit like someone being hypnotised Show the patient their Hoover s sign or dissoiative seizure video. You do not have MS, epilepsy et I do not think you are imagining/making up your symptoms/mad I see lots of patients with similar symptoms Beause there is no damage you have the potential to get better This is not your fault but there are things you an do to help it get better The hardware is alright but there s a software problem ; It s like a ar/piano that s out of tune ; It s like a short iruit of the nervous system (dissoiative seizures) If you have been feeling stressed/low/worried that will tend to make the symptoms even worse (often easier to ahieve on a seond visit) Send the patient their lini letter. Give them some written information If you have diagnosed dissoiative seizures and not epilepsy, stop the antiepilepti drug. Leaving the patient on the drug is illogial, makes no sense to the patient and will hamper reovery So-alled antidepressants often help these symptoms even in patients who are not feeling depressed. They are not additive. I don t think you re mad but Dr X has a lot of experiene and interest in helping people like you to manage and overome their symptoms. Are you willing to overome any misgivings about their speialty to try to get better? Explain it all to them as well 2009;9: doi: /jnnp

10 Neurology in Pratie Table 4 Examples of hanges in thoughts and behaviour that an help in patients with funtional symptoms Dissoiative seizures Funtional weakness Chroni bak pain Old thought New thought Old behaviour New behaviour Oh no what s happening to me. Am I going to die during one of these attaks? I ve got MS; I m going to end up in a wheelhair. No one believes me My spine is damaged, I must avoid moving too muh in ase it makes it worse I m having something a bit like a pani attak Mmm this is odd but it looks as if I an get better. My bones are fine, its my musles that are stiff and out of ondition Avoid going out, tendeny to Seeing lots of speialists, not doing Avoiding exerise/bak movement suumb to blakout as a way of very muh in ase it makes it getting rid of horrible warning worse symptoms Try out distration tehniques during Gradually exerise, learn to expet Gradually exerise, hange sleep warning symptoms relapses patterns Self-help soures for patients What else an a psyhiatrist/psyhologist do? Spend longer on speifi tehniques to deal with anxiety and pani symptoms. Reinfore explanations you have given about how the neurologial symptoms fit with their other symptoms. Disuss how previous life events/personality traits may help explain their vulnerability to symptoms. Monitor antidepressant treatment. Detet and treat other omorbid psyhiatri disorder bipolar disorder, obsessive ompulsive disorder, post-traumati stress disorder, eating disorder. Involve other relevant professionals ommunity psyhiatri nurse, psyhotherapist et. Referral to pain management One way in whih patients with funtional symptoms may be able to aess a CBT-based approah to their symptoms is via a Pain Management Programme (if you have one). Pain is often a big part of their symptoms and the priniples of rehabilitation for pain/fatigue/weakness are similar. Drug treatment Disuss whether to try antidepressants regardless of mood/anxiety; they an help some patients with a new (non-profit-making!) website by the author of this artile, designed as a soure of information for patients with funtional and dissoiative neurologial symptoms. Sharpe M, Campling F. Chroni Fatigue Syndrome (CFS/ME): The Fats. Oxford: Oxford University Press, The nek book Waddell et al and The bak book RCGP et al, both published by The Stationery Offie. Non-epilepti attaks. Self-help material is improving on the web (eg, www. epilepsyonnetions.org.uk and a good leaflet by Markus Reuber at www. shef.a.uk/ontent/1/6/08/82/45/nest%20patient%20booklet.pdf). Good self-help literature for pani, anxiety, depression and espeially health anxiety from Newastle/Northumberland trust ( leaflet.php?s = selfhelp). General literature on anxiety and depression ( Fibromyalgia, nek and bak pain leaflets at funtional symptoms. A triyli is a good hoie for someone with pain and insomnia. A seletive serotonin reuptake inhibitor (SSRI) may be better for someone with hypersomnia. Typially there will be adverse effets for several weeks after starting or inreasing the dose along with a delayed treatment effet. Explaining this arefully to the patient is worthwhile. If treatment fails then onsider a liaison psyhiatry referral. Consider beta blokers as a treatment for somati symptoms of anxiety. Physiotherapy A patient with mobility problems who is deonditioned needs physial as well as psyhologial treatments. Physiotherapists are often well plaed to advise on graded exerise but they must give an explanation that is onsistent with your own. Patients with funtional weakness may be best doing exerises that enourage bilateral leg movement rather than fousing on the affeted limb (whih will make it worse). Hypnosis or light sedation These an transiently, and sometimes permanently, improve the posture of a dystoni limb or improve a ompletely paralysed limb. Video this and show it to the patient afterwards to help them believe that it an be reversed. Physial aids/wheelhairs These an be an obstale to reovery but also improve independene and morale. Explain that the same arguments apply in multiple slerosis too and disuss openly with the patient. Disability benefits As with physial aids they an be an obstale to reovery but disability should be the riterion not the diagnosis. THE PATIENT WHO DOES NOT GET BETTER Do not expet to help all patients; just beause they have no disease does not mean they should get better. Perhaps only 1 in 4 severely affeted patients will do well. As in progressive multiple slerosis, patients with longstanding symptoms still benefit from assessment, treatment of interurrent depression and symptom management ;9: doi: /jnnp

11 Neurology in Pratie Further reading redue the number of new symptoms and unneessary referrals to hospital. Hallett M, Cloninger CR, Fahn S, et al. Psyhogeni movement disorders. Lippinott Williams & Wilkins and the Amerian Aademy of Neurology, Stone J, Carson A, Sharpe M. Funtional symptoms and signs in neurology: assessment and diagnosis/management. J Neurol Neurosurg Psyhiatry 2005;76(Suppl 1):i2 i21. Stone J. Dissoiation: what is it and why is it important? Prat Neurol 2006;6: Reuber M. Psyhogeni nonepilepti seizures: answers and questions. Epilepsy Behav 2008;12: Sharpe M, Campling F. Chroni fatigue syndrome (CFS/ME): The fats. Oxford, UK: Oxford University Press, Baker R, Shaw EJ. Diagnosis and management of hroni fatigue syndrome or myalgi enephalomyelitis (or enephalopathy): summary of NICE guidane. BMJ 2007;335: Mayou R, Sharpe M, Carson A. ABC of psyhologial mediine. London, UK: BMJ Publishing Group, Halligan PW, Bass C, Oakley DA. Malingering and Illness Deeption. Oxford, UK: Oxford University Press, Halligan P, Bass C, Marshall JC. Contemporary approahes to the siene of hysteria: linial and theoretial perspetives. Oxford, UK: Oxford University Press, Do not let your most severely affeted patients make you hopeless/negative about all patients with funtional symptoms. If their general pratitioner an agree a plan to see the patient on a regular basis, this may CONCLUSIONS Patients with funtional symptoms make up a large proportion of an average neurologist s workload. They are, on the riteria of distress, disability and persistene of symptoms, as deserving as patients with pathologially defined disease. Although the history an point in the right diretion, the diagnosis should be made on the basis of typial findings on examination or during an attak. Look for dissoiative symptoms they will help manage the patient. Investigations should be done quikly with a lear message that they are likely to be normal. A lear explanation of what is wrong (and not just what is not wrong) supplemented by a lini letter opied to the patient, and written information an be remarkably therapeuti. Neurologists need aess to speialist psyhiatry/psyhology support for many but not all these patients, but in many parts of the world this is not available Aknowledgements: I have learnt from many, espeially Mihael Sharpe, Alan Carson, Rod Dunan, Markus Reuber, Charles Warlow and John Mellers. This artile was reviewed by Mark Hallett, NIH, Washington DC, USA. Competing interests: None. 2009;9: doi: /jnnp