The heart sink patient – keeping the doctor and patient in a reasonable frame of mind

Some of our most time consuming patients present with symptoms and/or signs which do not fulfill conventional ‘organic’ nor psychiatric diagnostic schema. This paper summarises the neurological aspects of ‘medically unexplained’ or ‘functional’ disorders, which are common in both general and specialist acute and office based services. Such patients are often erroneously labelled with disease states, particularly epilepsy, stroke or multiple sclerosis – with serious implications and may follow a path of peripatetic, resource consuming and unhelpful medical interventions. Managing these protean disorders is challenging.

I am much less likely to reach a specific, ‘organic’ diagnosis when the consultation includes some of the following attributes: a third or subsequent opinion; the patient has an extensive prior medical career and multiple volumes of records, without clear pathology; chronic pain as the basis for referral; several surgical procedures have been performed for the same complaint (eg thoracic outlet release, ulnar transposition and carpal tunnel release); “my health is getting worse and worse doctor” – yet the patient is strong on symptoms, short on signs; prior psychiatric illness, especially depression and anxiety; an histrionic or dependent personality; multiple psychosocial stressors, often in a solo parent; sheets of foolscap notes; prior complaints about doctors failing to diagnose, especially alleged poisonings; conspicuous appliances such as dark glasses, collars and splints; no dental amalgam; poor tolerance of any drug treatment; much grunting and groaning during the examination.’Unexplained’ neurological symptoms cover the gamut of neurological function especially: pain – usually in the limbs, where a ‘trapped nerve’ is the often a concern; ‘memory’ loss, in young adults; hemiparesis, or one sided heavy limbs, often with migraine; paroxysmal turns; abnormal movements; sensory symptoms and gait disturbance – so called ‘astasia basia’. Sometimes an incorrect diagnosis of multiple sclerosis, as an explanation for fatigue and sensory symptoms, or epilepsy for unusual turns, has been suggested. Myasthenia gravis may be entrenched into thinking and behaviour, as an explanation for fatigue.In my area of practice, clinical neurology, functional problems of this kind (there are many more examples) account for as much as 30% of referrals to neurology outpatient clinics [1] – depending on how you define this group. Until recently, only limited data have been available to guide us in the diagnosis and management of ‘functional’ disorders, however a recent resurgence of interest in this difficult area of medicine is apparent [2].Part of the problem for research is defining this diverse patient group, for example should primary headache, which does not fit specific syndromes, be considered ‘medically unexplained’? Another problem is what terms to use. The literature is awash with labels to describe symptoms unexplained by disease, including pure symptomatic labels, such as chronic fatigue; descriptions of what the diagnosis is not, rather than what it is, for example ‘non epileptic attacks’; quasi diagnoses without a pathological basis, such as ‘fibromyalgia’ and ‘neurosensitisation’; diagnoses that imply a psychological cause for example ‘psychosomatic’, ‘hypervigilance’; traditional terms that do not fit any of these categories especially ‘hysteria’ and finally, official DSM based psychiatric diagnoses including conversion, somatisation, dissociative disorder, hypochondriasis. The term, ‘functional’ as a non-judgemental concept has been advocated and popularised recently. Patients may well appreciate that disordered function can occur without other objective evidence of disease, for example on tests. This is also the term patients find least offensive [3].Functional problems are part of the human predicament, one of the core elements of of dis-ease. Charcot demonstrated hysterical collapse at the Saltpetriere in the 1860s and George Beard’s described of neuroaesthenia in 1869. Biological, psychodynamic social and behavioural schemas have all been suggested to explain illness behaviour but none of these is entirely satisfactory. The situation is further complicated by evidence that functional disorders of the nervous system may involve active processes in the brain, blurring the line between psychodynamic/psychological theories and ‘organic’ neurological disease [4]. Concerns that the label ‘hysteria’ often leads to an eventual organic explanation for the presenting symptoms are unfounded. In fact, there is a high degree of reliability between neurologists when the diagnosis is of a functional illness (which may, of course, reflect a mind set). We probably make the same proportion of errors with this diagnosis as with any other neurological disorder. Functional illness is not just a diagnosis of exclusion, some useful signs can be sought to positively identify functional aspects. Space does not permit detailed description of these and there are excellent review articles in the literature [3]. Neurologists are accustomed to identifying the quality of weakness (eg collapsing) as well as and its pattern or distribution, looking for inconsistencies in the presentation and importantly getting the best from the patient’s performance during the examination, especially with respect to strength and gait (I admit at times the need to bully along when co-operation seems poor). For many but not all, a clear diagnosis of a functional disorder of the nervous system can be made after the first visit or two and an appropriate investigative work up. The notable exception is suspected epilepsy – a longer period of observation and ictal EEG recordings to differentiate partial seizures from panic attacks and other non-epileptic minor turns, may be needed. Many but not all patients with functional symptoms have primary or secondary anxiety, or are depressed but it is just not practicable to send all patients to the psychiatrist – in any case many decline the suggestion. Furthermore, some psychiatrists limit involvement to confirming there is no mental illness, or simply an absence of psychological problems, another non-illness – which is hardly helpful. One reason for neurologists to play a pivotal role in the approach to functional disorders is the necessity to recognise rare disorders and unusual presentations of common disorders and to be prepared to review the diagnosis, especially in the early stages of evaluation. The neurologist or relevant specialist and general practitioner remain in the best position to reassure, guide and manage most patients with functional illness.After a ‘therapeutic’ scan or other test, the temptation may be to dismiss and discharge as someone else’s responsibility. However, explanation to the patient, in a clear, logical, transparent and non offensive way is the key to a therapeutic relationship and thence recovery, at least for some. Indicate you believe the patient – most are immediately aware of any hint that you think this is ‘all in the mind’; explain what they don’t have; show the patient their positive signs which cannot be explained by neurological disease; emphasise that the problem is common, potentially reversible and that self help is the key to recovery. Metaphors and comparisons may be useful (for example ‘the hardware is fine but there is a software problem’); introduce the potential role of depression and anxiety late in the consultation – the patient may well do this; talk to family and carers and consider a psychiatric referral [5]. If it works well, this approach can come as an enormous relief to the sufferer – one of my recent patients said that she had only used multiple sclerosis as a convenient label, as no other schema existed with which she could identify. Cognitive behavioural therapy is a term of which we have all heard but few understand. The principals of CBT can be incorporated into standard medical care and need not necessitate hours of intensive therapy with a psychologist. Evidence exists that CBT is effective for a wide range of functional somatic symptoms and emphasises the interaction of cognitive, behavioural, emotional and physiological factors in perpetuating symptoms [5]. CBT principals which might encourage recovery, for example from functional hemiparesis could include: accepting all the symptoms at face value (avoiding a mind body dichotomy); persuading the patient they are not damaged and therefore have the potential to recover; providing a rationale for treatment eg exercise to recondition muscles and tune up the nervous system; promoting a positive, encouraging dialogue – bad days don’t mean that all is lost and that failure to improve is an event not a person; establishing a good sleep regime; encouraging the patient to reconsider unhelpful and negative thoughts, for example modest pain during exercise is not likely to be harmful; recognising any warning symptoms of decompensation and learning to control these; identifying obstacles to recovery – if you ‘always do what you have always done, you always get what you’ve always had’. Sometimes self help material is useful. Peripatetic consultations should be limited as much as possible. Antidepressants can help many patients with functional symptoms, even those who are not depressed, however their use may be limited by side effects. The mode of introduction of medications is just as important as writing the prescription, counselling the patients as to the reasons for choices of any medications (usually antidepressants) and delaying fears of “addiction”. Co-existing personality disorders and entrenched abnormal behaviour make recovery much less likely [6]. Some will follow a life long course of symptoms and disability, in which case ‘damage control’ (especially to the health care budget!) is the paradigm for management. For those with repeated presentations, regular review by a general practitioner regardless of whether the symptoms are new or not may help to contain behaviour, recognise and treat depression early. Steering a sensible path between over medicalising and appropriate review is very difficult. Dr Andrew Chancellor MD, FRACPTauranga HospitalVirtual Neuro Centre Editorial Advisory Board MemberReferences:1. Carson A, Ringbauer B Stone J et al. Do medically unexplained symptoms matter? A prospective cohort study of 300 new referrals to neurology outpatient clinics. J Neurol Neurosurg Psychiatry 2000; 68: 207-10. 2. Reuber M, Mitchell AJ, Howlett SJ, Crimlisk HL, Grünewald RA. Functional symptoms in neurology: questions and answers. J Neurol Neurosurg Psychiatry 2005; 76: 307-314.3. Stone J, Carson A, Sharpe M. Functional symptoms and signs in neurology: Assessment and diagnosis. J Neurol Neurosurg Psychiatry 2005; 76 (Suppl 1):i2-i12.4. Vuilleumier P, Chicherio, Assal F et al Functional neuroanatomical correlates of hysterical sensorimotor loss. Brain 2001; 124: 1077-90.5. Stone J, Carson A, Sharpe M. Functional symptoms and signs in neurology: Management. J Neurol Neurosurg Psychiatry 2005; 76 (Suppl 1):i13-i21.6. J Allanson, C Bass and D T Wade. Characteristics of patients with persistent severe disability and medically unexplained neurological symptoms: a pilot study. Journal of Neurol Neurosurg Psychiatry 2002;73:307-309.