Functional neurology

Functional neurology : 

Functional neurology Dr Jeff Kimber Paget 1873 "The patient says he cannot, it looks like he will not, but the truth is that the patient cannot will".

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TERMINOLOGY None of the current terms is perfect. It is best to choose words based on (a) how you see the cause or mechanism of the symptoms (b) how this affects your ability to communicate the diagnosis helpfully to the patients (preferably also including copying your clinic letter to them). Ultimately the label is not as important as the neurologist’s attitude to the patient.

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Psychiatric terminology Dissociative seizure/motor disorder (conversion disorder) (ICD-10) suggests dissociation as an important mechanism in symptom production, which is true for many patients but not all. Dissociation has many meanings but in this context often refers to two particular experiences: depersonalisation, a feeling of disconnection from one’s own body, and derealisation, a feeling of disconnection from one’s environment

Conversion disorder (DSM-IV) is a relic of Freudian psychoanalytic theory in the American bible of psychiatry (DSM) . It is defined as: –a motor or sensory symptom or blackouts not compatible with disease –which is not thought to be consciously manufactured–which causes distress and –is related to psychological factors. A traumatic experience often sexual is rendered more innoccuous by transformation into a somatic symptom. The resolution of the conflict is the primary gain.The advantages of the sick role are the secondary gain : 

Conversion disorder (DSM-IV) is a relic of Freudian psychoanalytic theory in the American bible of psychiatry (DSM) . It is defined as: –a motor or sensory symptom or blackouts not compatible with disease –which is not thought to be consciously manufactured–which causes distress and –is related to psychological factors. A traumatic experience often sexual is rendered more innoccuous by transformation into a somatic symptom. The resolution of the conflict is the primary gain.The advantages of the sick role are the secondary gain

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Somatisation disorder (DSM-IV) is applied to a patient with a longstanding tendency to have symptoms unexplained by disease, usually starting before the age of 30. It is somewhat arbitrarily defined as someone who has accrued at least one "conversion" symptom, four pain symptoms, two gastrointestinal symptoms (usually irritable bowel syndrome) and 1 sexual symptom (dyspareunia, dysmenorrhoea or hyperemesis gravidarum—indicating that women can be labelled with this more easily). Hypochondriasis means excessive and intrusive health anxiety about the possibility of serious disease which the patient has trouble controlling. Typically the patient seeks repeated medical reassurance but this only has a short-lived effect; in this situation it is a form of addiction which can only be overcome by a better explanation for symptoms, and ultimately discussion of health anxiety itself.

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Factitious disorder means that symptoms are consciously fabricated for the purpose of medical care (not money). These patients often have a personality disorder. Munchausen syndrome describes someone with factitious disorder who wanders between hospitals, typically changing their name and story. Malingering is not a psychiatric diagnosis but means making up symptoms for material gain (eg, benefit fraud).

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Other terminology Functional implies in the broadest possible sense a problem due to a change in function (of the nervous system) rather than structure. Non-organic, non-epileptic etc all have the problem of describing what the problem is not, rather than what it is. Psychosomatic is supposed to mean an interaction between mind and body but in practice is interpreted in the same way as "somatisation", the psychological influence on the body. Psychogenic suggests an entirely psychological explanation for symptoms. Medically unexplained is a neutral term but one that patients’ may easily interpret as the doctor not knowing what the diagnosis is (rather than not knowing why they have the problem). Like many neurological diseases (eg, multiple sclerosis, motor neuron disease, migraine) we can diagnose functional 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 clear what a normal response to functional problems should be, Hysteria is an ancient term originating from the idea of the "wandering womb". As Aubrey Lewis pointed out, it has "frequently outlived its obituarists".

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Functional describes a mechanism and not an aetiology sidesteps an illogical debate about whether symptoms are in the mind or the brain maps onto newer findings from functional imaging studies allows for the possibility of improvement can be used easily with patients.

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Neurology outpatients About 50% have a functional 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 includes patients with neurological disease and "functional overlay". 25% of chronic epilepsy patients have NEA About 15% have a primary functional/psychological diagnosis (including pain and fatigue unexplained by disease). About 5% have seizures, weakness, sensory symptoms or movement disorder which is thought by the neurologist to be functional/non-organic (sometimes called "conversion symptoms"). On average new neurology patients with functional symptoms are just as disabled as and even more distressed than those with a neurological disease. 1–10% of neurology inpatients have a primary "functional" diagnosis. J Stone Practical Neurology 2009;9:179-189

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An average British Neurologist will fail to find an adequate physiological explanation for 1 in 5 out- patients. (~36, 000 patients per annum in UK). Mace CJ & Trimble M JRSoc Med (1991) 84:471-5 In a study of 100 consecutive Neurological admissions in Denmark an organic explanation for all symptoms was made in only 40 Ewald H et al Acta Psych Scand (1994) 89:174-9

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Conversion symptoms Dissociative/non-epileptic seizures account for about 20% of referrals to "first fit" clinics and to specialist epilepsy clinics. Up to 50% of admissions to hospital with "status epilepticus" are in fact dissociative seizures/"pseudostatus". Functional weakness has an annual incidence of at least 5/100 000, similar to multiple sclerosis. Functional movement disorders account for 5–10% of patients seen in a movement disorders clinic.

What should we call them? Number needed to offend : 

What should we call them? Number needed to offend Stone J et al. BMJ (2002) 325 1449-59

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There is no common aetiology for all patients. Severity also varies enormously Functional imaging and cognitive neuropsychological studies of patients with functional paralysis do not yet provide a convergent model but suggest an altered brain state in which there may be a combination of altered and overactive premotor areas and hypoactive thalamic areas

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Dissociative 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, especially worry about cardiac problems. Semiology 70% thrashing, 25% fall down lie still, 5% other . Movements during a "thrashing" dissociative seizure are a form of severe tremor rather than clonic movements, typically there is no isolated "tonic" phase. Very few conditions (other than death) lead to a state of suddenly falling down and lying still for over five minutes. Diagnostic pitfalls Coexistent epilepsy is present in 5–20% of those with dissociative seizures. Frontal lobe epilepsy may present as "weird" attacks but usually their brevity should alert one to epilepsy. Panic/fear as part of temporal lobe seizures. Sleep disorders such as REM sleep behaviour disorder. Rarer causes—eg, insulinoma, paroxysmal dyskinesia.

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Weakness Half start suddenly, half more gradually often with pain or fatigue. If sudden onset, may arise after a physical injury, dissociative seizure, from sleep paralysis or general anaesthetic. Look for evidence of inconsistency—a limb that appears weak but then moves normally in another circumstance, eg: –weakness on the bed (for example of plantar flexion) that is inconsistent with abilities when walking (for example walking on tiptoes). –observe the patient outside the formal examination—coming in and out of the consulting room, getting dressed/undressed, reaching for medication lists in bags, etc. –Hoover’s sign . May be false positive in patients with neglect and in patients with a lot of limb pain. –A similar finding of hip abduction weakness which returns to normal with contralateral hip abduction against resistance may also be helpful. A gait in which 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 which can be encouraged briefly to normal (eg, "at the count of three, push...1...2...3...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 fraction of a second before collapsing.

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Copyright ©2002 BMJ Publishing Group Ltd. Stone, J et al. J Neurol Neurosurg Psychiatry 2002;73:241-245 Hoover's sign. (A) Hip extension is weak when tested directly. (B) Hip extension is normal when the patient is asked to flex the opposite hip.

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Vision Complete blindness—look for normal pupillary reaction or optokinetic nystagmus. Be careful of cortical blindness. Reduced vision—often ipsilateral to any hemisensory disturbance. Look for a tubular visual field by examining fields to confrontation at the bedside close (eg, 30 cm) and far away (eg, 150 cm). There may be spiral or pinpoint fields on Goldmann perimetry. Diplopia may be due to convergent spasm of one or both eyes which sometimes looks like a sixth nerve palsy. Beware midbrain lesions Monocular diplopia can be caused by ocular disease

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Facial weakness: there may be an appearance of ptosis when the problem is actually one of overcontraction of orbicularis oculis (usually in association with photophobia). In the lower face the mouth is sometimes pulled down by overcontraction of platysma giving an appearance of lower facial weakness

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Sensory symptoms Patterns of sensory disturbance include: Feeling "split in half" down one side of the body, with altered temperature, vibration sense and light touch down the affected side (be careful, this also occurs in thalamic lesions). There is invariably mild functional weakness in association 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 ticklish on the affected foot (and the plantar response may be correspondingly rather mute). Tests of functional sensory symptoms are described including: Altered vibration sense across either side of the forehead "Say yes when you feel it and no when you don’t" or "close your eyes and touch your nose when I touch your hand" However, studies have found that these are also common in patients with neurological disease and so none can be considered reliable.

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Tremor Disappears with distraction—eg, counting backwards in sevens. Tremor of Parkinson’s disease may be more noticeable during distraction. Variable frequency is more helpful than variable amplitude. Entrainment—ask the patient to make a rhythmical 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 affected limb. Alteration with weight or attempted immobilisation—functional tremor typically worsens when an arm is weighted or if an examiner attempts to make it still by holding on to it.

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Copyright ©2002 BMJ Publishing Group Ltd. Stone, J et al. J Neurol Neurosurg Psychiatry 2002;73:241-245 Functional gait. The leg is dragged at the hip. External or internal rotation of the hip or ankle inversion/eversion is common. Uneconomic posture. Pseudoataxia. "Walking on ice" gait

Functional Symptoms in NeurologyGait disorders : 

Functional Symptoms in NeurologyGait disorders Monoplegic "dragging" gait Fluctuation of impairment Excessive slowness of movements or hesitation "Psychogenic Romberg" test "Walking on ice" pattern Uneconomic postures with waste of muscle energy Sudden knee buckling Nb.chorea and cataplexy

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Dystonia Typically "fixed" with a clenched fist or an inverted and plantarflexed foot Usually associated with pain, and often a complex regional pain type 1 picture, which usually arises after a minor injury. There is debate about the extent to which "fixed dystonia" is a functional disorder. Patients who have been cured with hypnosis or sedation indicate that this debate needs to continue.

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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, hence their appearance in your clinic. As a symptom of anxiety or depression—"poor concentration" seen in depression or anxiety may be latched on to by a patient as the primary symptom. A psychiatrist used to detecting 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 associated with a desire to return to a previous time in life.

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MISDIAGNOSIS In 1965 Slater found 21/85 patients at NHNN diagnosed with hysteria had this confirmed over time Misdiagnosis for "conversion symptoms" in studies since 1970 has on average been around only 4% at 5 years. Better investigations Some hysterical diseases (CFS, torticolis) now recognised This is the same as for other neurological and psychiatric conditions such as multiple sclerosis and schizophrenia. Gait disorders, movement disorders, frontal lobe epilepsy, psychiatric presentations of multiple sclerosis, coexisting disease and functional symptoms, and patients with obvious psychiatric problems/recent life events are over-represented in cases when neurologists do get it wrong

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The main clues to malingering or factitious disorder are: inconsistency in the history on different occasions (between doctors or between relative and patient) an admission from the patient that they have lied about other things in the past avoidance of tests a direct confession evidence of gross inconsistency from covert surveillance (for example a wheelchair patient who is seen playing tennis) simulation of symptoms that mimics disease very closely (for example a patient who has tonic then clonic movements in their seizure rather than just shaking).

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Treatment Silas Weir Mitchell (1829-1914) “yes she will run out of the door in two minutes I will set her sheets on fire” “I urged and scolded and teased and bribed and decoyed along the road to health: but this is what it means to treat hysteria”

Functional symptoms ‘Management in neurological practice’ : 

Functional symptoms ‘Management in neurological practice’ Thomas W. Salmon (1876-1927), The Care and Treatment of Mental Diseases and War Neuroses ("Shell Shock") in the British Army, War Work Committee of the National Committee for Mental Hygiene, New York, 1917.

Functional symptoms ‘Management in neurological practice’ : 

Functional symptoms ‘Management in neurological practice’ Positive diagnosis Exclusion of organic disease Close collaboration between Neurologists and Psychiatrists Assessment and treatment of associated psychiatric illness (avoiding chicken egg arguments) Cognitive behaviour programmes Avoid unnecessary investigations and iatrogenic damage

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Referral to pain management CBT-based approach to their symptoms via a Pain Management Programme. Pain is often a big part of their symptoms and the principles of rehabilitation for pain/fatigue/weakness are similar. Drug treatment Discuss whether to try antidepressants regardless of mood/anxiety; A tricyclic is a good choice for someone with pain and insomnia. A selective serotonin reuptake inhibitor (SSRI) may be better for someone with hypersomnia. Typically there will be adverse effects for several weeks after starting or increasing the dose along with a delayed treatment effect. Explaining this carefully to the patient is worthwhile. If treatment fails then consider a liaison psychiatry referral. Consider beta blockers as a treatment for somatic symptoms of anxiety. Physiotherapy A patient with mobility problems who is deconditioned needs physical as well as psychological treatments. Physiotherapists are often well placed to advise on graded exercise but they must give an explanation that is consistent with your own. Patients with functional weakness may be best doing exercises that encourage bilateral leg movement rather than focusing on the affected limb (which will make it worse). Hypnosis or light sedation These can transiently, and sometimes permanently, improve the posture of a dystonic limb or improve a completely paralysed limb. Video this and show it to the patient afterwards to help them believe that it can be reversed. Physical aids/wheelchairs These can be an obstacle to recovery but also improve independence and morale. Explain t hat the same arguments apply in multiple sclerosis too and discuss openly with the patient. Disability benefits As with physical aids they can be an obstacle to recovery but disability should be the criterion

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What else can a psychiatrist/psychologist do? Spend longer on specific techniques to deal with anxiety and panic symptoms. Reinforce explanations you have given about how the neurological symptoms fit with their other symptoms. Discuss how previous life events/personality traits may help explain their vulnerability to symptoms. Monitor antidepressant treatment. Detect and treat other comorbid psychiatric disorder—bipolar disorder, obsessive compulsive disorder, post-traumatic stress disorder, eating disorder. Involve other relevant professionals—community psychiatric nurse, psychotherapist etc.

Paradoxes in illness belief and disability in patients with functional and neurological paresis : 

Paradoxes in illness belief and disability in patients with functional and neurological paresis Self rated distress and disability are similar Patients with functional paresis had significantly more psychiatric illness Despite this they were less likely to agree that stress was a cause of their illness (24% vs 57%) And are twice as likely to have given up work (59% vs 29%) Stone J et al.(2004) JNNP 75:519

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PROGNOSIS Patients with dissociative seizures, functional weakness and outpatients with milder symptoms—as few as one third become 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 outcome. Poor prognostic factors—strong beliefs in lack of reversibility of symptoms/damage, anger at the diagnosis of a "non-organic" disorder, delayed diagnosis, multiple other physical symptoms/somatisation disorder, concurrent organic disease, personality disorder, older age, sexual abuse, receipt of financial benefits, litigation. Good prognostic factors—willingness to accept reversibility/self-help, young, recent diagnosis, lack of other physical symptoms, change in marital status (divorce/marriage) after diagnosis, concurrent anxiety/depression.

Photographic reproduction of schematic representation of specificity in the etiology of the peptic ulcerFranz Alexander (1891-1964), Psychosomatic Medicine, New York, 1950. : 

Photographic reproduction of schematic representation of specificity in the etiology of the peptic ulcerFranz Alexander (1891-1964), Psychosomatic Medicine, New York, 1950.

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CONCLUSIONS Patients with functional symptoms make up a large proportion of an average neurologist’s workload. They are, on the criteria of distress, disability and persistence of symptoms, as deserving as patients with pathologically defined disease. Although the history can point in the right direction, the diagnosis should be made on the basis of typical findings on examination or during an attack. Look for dissociative symptoms—they will help manage the patient. Investigations should be done quickly with a clear message that they are likely to be normal. A clear explanation of what is wrong (and not just what is not wrong) supplemented by a clinic letter copied to the patient, and written information can be remarkably therapeutic. Neurologists need access to specialist psychiatry/psychology support for many but not all these patients, but in many parts of the world this is not available