Health anxiety, malingering, or factitious illness, different from a psychological point of view.
Bleed the symptoms dry! [John Stone, Glasgow].
In a 1965 paper by Eliot Slater, more than half of patients diagnosed as having “hysteria” later turned out to have “organic” disease – but John Stone’s study of adult neurology referrals found very few who turned out to have an occult disease.
Louise Stone in Australia has done a lot of work in primary care. She identifies negative feelings and a lack of diagnostic language and frameworks as barriers to managing these patients effectively. The negative feelings (such as frustration, shame and helplessness), are shared between doctors and patients…
Managing your own feelings and frustrations, and finding ways of understanding and managing the therapeutic relationship important.
Let family feel validated for all concerns – at least in the first instance. Helps develop a therapeutic alliance.
Commit to the patient, which includes advocacy and support.
Family response to symptoms?
Explore beliefs, specific worries (eg cancer). May then allow broadening out to more general worries.
Manage uncertainty – including managing the need for a disease name! Not having a predictable outcome is hard.
Harm minimisation. Shift from diagnosis to coping with ongoing symptoms.
Good to offer a tentative preamble to difficult conversations! “This is something we as doctors have to deal with every day – signs and symptoms that are very real, with a real impact on a child/family, but where physical examination and investigations do not offer any clues to what the underlying problem might be…”
Paed psychology if issues mostly seem related to child and this is a new problem; CAMHS if new problem adding to existing child/parent issues.
Can be rewarding in the long term!
[Louise Stone, Aust Fam Physician 2013 Jul;42(7):501-2]
Involvement of family members and other carers can be really useful, if the young person agrees. Should be collaborative, of course, giving them opportunities to contribute to planning. But not just about minimising self harm behaviour – empowering and supporting (during acute distress and also in recovery) vital too.
Assessment
Discuss removing the method of self harm – with therapeutic collaboration or negotiation, to keep the person safe
Discuss current support network, any safety plan (see below) or coping strategies
Refer
Refer to mental health professionals urgently where:
the person’s levels of concern or distress are rising, high or sustained
the frequency or degree of self-harm or suicidal intent is increasing
the person asks for further support from mental health services
levels of distress in family members or carers of children, young people and adults are rising, high or sustained, despite attempts to help.
Treatment
Work collaboratively with the person, using a strengths-based approach to identify solutions to reduce their distress that leads to self-harm.
Consider developing a safety plan:
recognise the triggers and warning signs
individualised coping strategies, including problem solving any barriers to those strategies
social contacts and settings that can distract from suicidal thoughts or escalating crisis
family members or friends to provide support and/or help resolve the crisis
contact details for the mental health service, including out-of-hours services and emergency contact details
keep the environment safe by working collaboratively to remove or restrict lethal means of suicide.
Underlying depression, anxiety, learning disabilities, autism, eating disorders should all be addressed.
For children and young people with significant emotional dysregulation difficulties who have frequent episodes of self-harm, consider dialectical behaviour therapy adapted for adolescents (DBT-A).
Early adolescence appears to be a critical time for determining the long term direction of biopsychosocial development. US studies found that those who reported their first alcoholic drink before age 14 (or drug use before age 15) were 3x more likely to develop dependence.
Regular use of cannabis before age 15 seems linked to subsequent psychosis, and risk appears to be 5x higher in daily users of high strength cannabis. But longitudinal study did not find a link after controlling for confounders. Risk probably highest where genetic or other vulnerability.
Large studies in Australia and New Zealand found frequent cannabis use predicts poor educational outcomes – some evidence for poorer memory performance (for days or even weeks after use), which might explain it (controlled for socioeconomic factors).
Inconsistent moderate associations with suicide and attempted suicide.
Have you ever ridden in a Car driven by yourself or someone else who was high or who had been using alcohol/drugs?
Do you ever use alcohol/drugs to Relax, feel better about yourself or fit in?
Do you ever use alcohol/drugs when you are Alone?
Do you ever Forget things you did while using alcohol/drugs?
Do your family/friends ever tell you that you should cut down?
Have you been in Trouble while using alcohol/drugs?
2 or more yes answers “suggests an important problem”.
Intervention
Brief motivational intervention almost halved frequency of alcohol bingeing among 13-17yr olds presenting to an Emergency department. But rate also reduced in control groups.
A “confiding” parent-child relationship is linked to lower substance abuse rates. Parental knowledge of the child’s whereabouts is protective, although also likely to be proxy for confiding relationship.
Sympathetic, informed, supportive counselling as good as CBT for adolescent depression and adult alcoholism. Flexibility of services is important.
FRAMES –
Feedback – personalised, about risk/harm
Responsibility – emphasis personal responsibility for change
Advice – give clear advice to change habits
Menu – offer menu of strategies
Empathic – and non judgmental
Self efficacy – aim to increase patient’s confidence to change behaviour
Doesn’t seem to be associated with severity of initial illness.
Long COVID is less common if you are vaccinated already. In a community based study of adults aged 18 to 69 years infected with SARS-CoV-2 before vaccination against covid-19, the odds of experiencing long covid symptoms decreased by an average of 13% after a first covid-19 vaccine dose, with a further 9% decrease in the odds of long covid after a second dose. [https://doi.org/10.1136/bmj-2021-069676]
If you already have long COVID, further COVID immunisations seem to be beneficial, rather than harmful:
In a non-controlled study of 900 social media users with long COVID, more than half experienced an improvement in symptoms after vaccination compared with 7% who reported a deterioration.19
A study of 44 vaccinated patients and 22 unvaccinated controls previously admitted to hospital with covid-19 in the UK, which inevitably had limited power to detect clinically relevant effects, found no evidence for vaccination being associated with worsening of long covid symptoms or quality of life.20
A French study of 455 self-selected participants found reduced long COVID symptom burden and double the rate of remission at 120 days in vaccinated participants compared with unvaccinated controls.
All about symptoms, excluding treatable conditions, then self management. For adults:
Sedentary time spent in front of screens programs metabolism and brain neurochem. Similar to addiction.
But it’s complicated! Partly because there are so many different kinds of activity that can now be done with mobile phones and tablets, and because it’s difficult to control or randomize.
How people interact with screens is changing too. In the mid-2000s in the US, children over 8 spent an average of 6.43 hours a day on electronic media, but this was mainly watching TV. Evidence of stress response (reduced cortisol increase) on waking after using screens for average 3 hours a day.
But now mobile devices are the centre piece of young people’s social lives. Boys tend to spend more time gaming, girls more time on social networking.
Effect of high levels of screen time does not seem to be attenuated by equivalent exercise.
High levels of screen time (over 4 hours daily) is associated with poorer school performance. Social skills are poorer. These children tend to form cliques with shared interest, that create further social isolation.
Violent games and media are associated with aggression in children as young as pre-school. Aggression in children can be manifest physically, or verbally, or relationally (ignoring, excluding, spreading rumours). There is also significantly more hostile attribution bias, where you interpret behaviour (such as not being invited to a party) as hostile, even when it is not, or at least ambiguous.
Parental involvement matters – how frequently parents watch TV with their children, discuss content with them, and set limits on time spent playing video games.
Exposure to media violence must also be seen within a risks/resilience approach. [Gentile and Coyne, Aggressive Behaviour 2010] ]
Some evidence that some “social” games themed around cooperation and construction eg Animal crossinghave benefits. Similarly, links between media and relational violence pretty weak, but that could be because relational violence content isn’t really examined! Actual content probably more important than time spent playing. Note that in that study of Animal Crossing, background mental health problems seemed to reduce benefit.
Longitudinal research shows association between progressively higher glycaemic index diet and incidence of depressive symptoms. Experimental exposure to diets with high glycaemic load increases depressive symptoms in healthy volunteers, with moderately large effect.
Mechanism could be repeated and rapid changes in blood glucose, triggering counter regulatory hormones such as cortisol, adrenaline, growth hormone, glucagon.
Appears to be an inflammatory response to high glycaemic index foods too. Adherence to Mediterranean diet reduces markers. Mood disorders have been linked to heightened inflammation, although only in a minority. Observational studies show people with depression score higher for “dietary inflammation” viz trans fats, refined carbohydrates, lower intake of omega 3 fats. Mediated through polyphenols, polyunsaturated fatty acids?
Diet also affects microbiome, which interacts with the brain in bidirectional ways using neural, inflammatory and hormonal signalling pathways. High fibre, polyphenol, unsaturated fats promotes microbial taxa that generate anti-inflammatory metabolites such as short chain fatty acids.
Study of probiotics in healthy volunteers found altered response to a task that requires emotional attention, and may even reduce symptoms of depression.
But no benefit in large trial of Medierranean diet with subclinical depressive symptoms, only small trials of current depression showed benefit. Note context of people’s expectations regarding food/diet, which will likely have a marked effect on wellbeing.
Danger too of stigmatisation if trying to change an individual’s dietary choices.
One of the major criteria for Rheumatic fever but can sometimes be seen in isolation. An acute neuropsychiatric condition, that can cause severe functional impairment, but that mostly resolves spontaneously. See Jelly Jumps page for videos and family support.
Classically, involuntary, non-rhythmic movements, associated with emotional lability. Often misdiagnosed initially eg psychogenic [Mary King, ADC 2015]. Adults can get it rarely – tends to be relapse of childhood disease, female hormones seem to a trigger (eg pregnancy, oral or other contraceptives).
Chorea is a particular kind of movement – varies from smooth writhing (athetosis) to rapid, high amplitude jerks (ballism). Typical signs are repeated pouting of lips, milk maid sign (ask to squeeze fingers in hand), hyperextension of wrists, piano playing movements. Fine motor control usually lost, due to these extra movements. Gait disturbance common, can look like hip hop dancing! Ask to stick tongue out (unable to maintain – “motor impersistence”). Movements disappear in sleep. Can be hard to differentiate sometimes from stereotypies and tics, and of course these things are not uncommon so might co-exist.
Can be one side of the body predominantly in 20-30% of cases (hemichorea). Underlying the involuntary movements is often a loss of tone, which may not become obvious until treatment started to suppress the chorea.
In severe cases, the loss of tone and weakness predominate (chorea paralyticum).
Variable severity. May just be some instability on walking, some difficulty with hand writing. Or unable to walk, talk, feed yourself.
The “psychiatric” part of the neuropsychiatric condition is a mixture of different issues. Emotional lability common, mild anxiety and poor attention less so – although developing a new disability without any cognitive impairment may explain some of it. Tics (new) often seen.
Family history often seen, at least in historical reports, where it was part of the diagnosis! But perhaps cross infection rather than genetic predisposition.
Risk of cardiac involvement, as related to rheumatic fever – 20% of cases in BPSU study, but 71% of cases in Turkish study. Half if not more are subclinical (no findings on clinical examination). Significant risk of long term morbidity, probably more important than chorea itself, so always echo. Penicillin prophylaxis important for carditis (see below).
A new case every 2.5 weeks in the UK, according to BPSU study.
History
Previously called St Vitus’ dance by Thomas Sydenham, but confusing, because there were epidemics of uncontrollable dancing in the middle ages which probably weren’t all related to rheumatic fever.
In the late 1800s, Sydenham’s chorea was the fourth most common reason for children to be admitted to the Great Ormond Street hospital, London. Often there would have been a family history, probably due to cross infection.
Diagnosis
Essentially clinical, with supportive evidence of recent streptococcal infection (history, ASO titre, throat swab). But recognised that infection can be up to 6 months before, or too mild to really notice, and ASO hardly reliable.
Other tests are directed at differential diagnosis – lumbar puncture, MRI brain (putaminal enlargement described in SC but not diagnostic) etc.
Although there is evidence of anti-neuronal antibodies directed against the basal ganglia (eg anti D2R, see Church 2003), these are not specific or sensitive (see Sugar 2003, same time as Church) so not used in clinical practice. Swedo and Cunningham (also 2003) found cross reactive antibodies that recognised N-acetyl Beta D glucosamine, the major strep surface epitope, and also lysoganglioside, activating CAMK II which may regulate neurotransmitters. “Cunningham panel” is private test, see PANDAS.
An echo can confirm presence of carditis (typically mitral/aortic valvulitis) if actually rheumatic fever, not just Sydenham’s. Jones criteria suggest repeat echo in 2-4 weeks if initially normal.
Management
There is a UFMG rating scale for SC, from Brazilian Universidade Federal de Minas Gerais (UFMG), only looks at motor function, 27 items, so for research purposes only. Walker-Wilmshurst-Wendy scale just 16 yes/no, with 1 point for emotional lability, 1 for OCD and 1 for other behavioural disturbance.
Occupational and physiotherapy useful for maintaining function and muscle tone, especially for getting back to school.
Treatment with valproate is effective for controlling symptoms but doesn’t speed up recovery. May reveal hypotonia. Haloperidol used previously but prob more side effects. Case reports to support carbamazepine and levetiracetam.
One RCT supporting steroids from Paz, Brazil 2006, 22 cases of SC, remission reduced to 54 days from 119 days. Various other reports of use of oral or IV steroids from Israel, Italy [Fusco 2012, 2017], Brazil [Cardoso 2005], immunoglobulin [Holland, 2016, South Africa 2016]. Some of these studies report response with days, and remission within 7 to 54 days, even where cases are severe and have already been treated with anticonvulsants. South African group found less neuropsychiatric complications at 6 months with IVIG treatment (IVIG preferred due to fear of TB reactivation). [Review by Deans and Singer, 2017]
Prophylaxis
A course of penicillin is usually given at diagnosis, to definitively clear any remaining strep but no evidence this really achieves anything and active infection probably long gone. Penicillin prophylaxis, on the other hand, essential if you have other features of rheumatic fever – regimens vary globally.
If Sydenham’s chorea is not part of broader rheumatic fever diagnosis, then practice varies regarding offering prophylaxis. Evidence is that recurrence is less where penicillin prophylaxis is used, and used reliably, but that it doesn’t always prevent it. Given the high rate of recurrence, the level of disability and potential for long term complications, the benefits seem to outweigh the costs (review in 2017 favours it but does not seem to strictly distinguish non-RF Sydenham’s) and American Heart Association 2009 guidelines recommend it wholeheartedly, but not straightforward.
Patients find injections of benzathine penicillin painful. Downside of oral twice daily penicillin is the restrictions around meal times (absorption affected by food, so advised best given at least 1 hour before or 2 hours after), which can be challenging. But remembering to take it probably more important!
Recurrence
Recurrence seen in 16-40%. More likely if poor compliance with penicillin prophylaxis, of course. Sometimes associated with rise in ASO or other evidence of new streptococcal infection but certainly not always the case. No obvious clinical parameter that might predict those at risk of recurrence. More likely if failure to remit in initial 6 months. Can recur with pregnancy and possibly with other female hormone treatments eg oral contraceptives or HRT.
Higher recurrence rates seen in longest follow up – can recur up to 10 years after the initial episode, so might be underestimated by series with shorter follow up.
Usually recurrence is just chorea, even if you had other features of rheumatic fever to begin with. Just two reports of heart disease worsening after recurrence of chorea [Israel and Thailand]. The Thailand study also had 2 cases where carditis, which had improved after initial diagnosis, came back again. Some suggest that perhaps recurrent chorea is a different disease altogether. [Israel, Arch Neurol. 2004; Turkey, PMID 27209549]
Prognosis
Most resolve within 2-4 months. Improvement tends to be rapid once it begins.
10% reported long term tremor in one study (10 years follow up). Long term neuropsychiatric difficulties increasingly recognised (49 studies so far, {Michael Morton and Nadine Mushet 2016 PMID 25926089] esp Obsessive-compulsive disorder but also Attention-deficit-hyperactivity disorder, affective disorders, tic disorders, executive function disturbances, psychotic features, language impairment.
Heart involvement improves in about a third of cases (whether silent or not).[PMID 22734303]
Benign hereditary chorea (BHC) – rare. In infants low muscle tone, chorea, lung infections, and respiratory distress. In older children, delayed motor and walking milestones, myoclonus, dystonia (esp upper limb), motor tics, and vocal tics. The chorea often improves with time, in some cases myoclonus persists or worsens. Some have learning and behaviour problems, thyroid problems and recurring chest infections. Caused by mutations in the NKX2-1 gene (autosomal dominant)
Bilateral striatal necrosis is a rare condition where similar symptoms but chronic and permanent. Various causes, has been seen in association with streptococcus. Has been described in a case of Sydenham’s where symptoms recurred and then persisted, so not clear whether coincidence or it wasn’t really Sydenham’s in the first place.
=inflammation of the meninges. Clinically neck stiffness/pain, headache, photophobia. Almost always vomiting. Usually infective, so usually fever too. Can be viral, bacterial or tuberculosis.
This clinical picture gets confused with the diagnosis of meningococcal disease. Meningococcus (gram negative diplococcus, very distinctive under the microscope) commonly causes meningitis but tends to cause a relatively mild disease with good outcome. It can also cause sepsis that is rapid onset and often fatal, meningitis is rarely a feature of this disease (indeed, having meningitis at the same time is a good prognostic feature).
Diagnosis is by lumbar puncture. Bugs often seen under microscope, which will usually give organism too, else rapid antigen tests available. White cells will be high (often in thousands if bacterial), protein high (can be over 2 if bacterial). Neutrophil predominance suggests bacterial but this is not v reliable esp in babies. Low glucose v suggestive of bacterial.
Can be complicated by raised intracranial pressure and seizures.
Organisms
In neonates, mostly Group B streptococcus, else gram negative bacilli. Listeria can present with sepsis or meningitis in young infants (90% under 30 days).
In older infants and children, mostly meningococcal disease, else pneumococcal or haemophilus. All declining rapidly as a result of immunisation, currently conjugate Hib, PCV-13 and MenACWY plus 4CMenB.
Treatment
Antibiotics to kill bugs. Steroids to reduce damage.
Ceftriaxone is ideal, broad spectrum, good CSF penetration, once daily. But listeria resistant, and gets chelated by calcium so contraindicated if likely HDU/ICU care where calcium infusions often necessary. Also contraindicated in preterm infants under 41/40 corrected, and in neonates esp jaundice, acidosis, hypoalbuminaemia.
For listeria, amoxicillin or ampicillin for 21 days in total, plus gentamicin for at least the first 7 days.
Dexamethasone has been shown to reduce complications eg deafness. Regimen is 0.15 mg/kg (max 10 mg) qds x 4 days. Only given to children ≥ 3 months old. Ideally given before or with first dose antibiotics – NICE says within 12 hours, assuming positive LP viz frankly purulent CSF, or CSF WCC > 1000/μl, or raised CSF WCC and protein > 1 g/L, or bacteria on Gram stain. Steroids should not be used in developing countries.
For unconfirmed bacterial, NICE says minimum 10/7 for over 3/12, 14/7 (with amoxicillin) for under 3/12 – but depending on signs, symptoms and clinical course. Discuss with expert if complicated clinical course.
TB meningitis is a whole different ball game. See NICE NG33 before administering steroids.
Complications
Hydrocephalus, epilepsy, deafness. Particularly seen with Pneumococcal disease.
Recent evidence highlights that meningitis in early childhood is associated with higher depressive and anxiety symptoms, psychological and behavioural problems, and increased risk of psychotic experiences. Not just that, higher risk of ADHD, and lower IQ on average. Follow up therefore very important for young babies, and probably appropriate to warn families.
persistent difficulties with social communication and social interaction, and
restricted and repetitive patterns of behaviours, activities or interests (this includes sensory behaviour),
present since early childhood,
to the extent that these limit and impair everyday functioning
Sensory behaviour may be meltdown or withdrawal or other challenging behaviour when too much information or sensation is experienced. There can be hyper (or hypo) sensitivity to lighting, problems with depth perception, noises or crowds, smells (or licking), pain, taste/textures.
SIGN guidance is that (145):
children under 3 with regression in language or social skills should be referred
not to screen population
that screening instruments are not 100% reliable but have their uses
that ASD should be considered in any child with developmental, emotional, psychiatric or behaviour issues, or a genetic syndrome
in preschool children typical features may be absent
gender differences are important in terms of symptoms and level of impairment
Assessment
Lack of shared attention (or late development) eg pointing
repetitive behaviour/play
resistance to change
violent or self injurious behaviour, pica
Types
Aspergers – social difficulties in absence of learning or communication problems
Pathological demand avoidance – where underlying problem is high level of anxiety about conforming to social demands or not being in control
Therapy based on physical exercise should NOT be offered “as a cure”, nor should graded exercise programmes (which by definition use fixed increments in exercise) be used!
Instead, self management, flexible and tailored
CBT should only be offered to manage symptoms, improve functioning and reduce distress.
Talks about “energy management” – includes emotional, social, cognitive.
“Care and support plan” – physical activity including mobility but also activities of daily living. Plan periods of rest and activity, and incorporate the need for pre-emptive rest. Management of relapses and flares.
Main thrust of update is that CFS/ME is a complex, chronic medical condition affecting multiple body systems and its pathophysiology is still being investigated. It affects everyone differently and its impact varies widely – for some people symptoms still allow them to carry out some activities, whereas for others they cause substantial incapacity. It is a fluctuating condition in which a person’s symptoms can change unpredictably in nature and severity over a day, week or longer.
Often it profoundly affects different aspects of the lives of both people with ME/CFS and their families/carers including social life, emotional wellbeing and education.
Another big theme is prejudice, disbelief and stigma experienced by patients.
US IOM expert panel have rejected the name “chronic fatigue syndrome”, as patients hate it! Myalgic encephalitis (ME) also rejected on basis of insufficient evidence that this is the pathological process. They suggest “Systemic exertion intolerance”, which is probably even more rubbish, in my opinion.
Diagnostic criteria: all of the following 3 [BMJ2015; 350]
Substantial reduction/impairment in pre-illness levels of activity, that persists for more than six months [NICE 2007 says 3/12 for children], and accompanied by fatigue (often profound, new or definite onset, not the result of ongoing excessive exertion and not substantially alleviated by rest)
Worsening of symptoms after any type of exertion (including cognitive and emotional stress) – “post-exertional malaise“
Unrefreshing sleep, and/or sleep disturbance.
In addition, should have at least one of:
Cognitive impairment
Orthostatic intolerance.
Doesn’t mention chronic pain?! NICE says reconsider diagnosis in absence of cognitive difficulties or chronic pain.
Causes
Evidence (reproducible) implicating certain infections as a trigger. Co-existing mood disorder in substantial proportion of patients, sometimes sleep-wake disorder – likely to perpetuate/exacerbate.
Brain imaging has identified alternations suggesting that it is a brain problem.
Cochrane review of graded exercise therapy – may benefit sleep, physical function, self-perceived general health, and no evidence that it worsens outcomes. Curiously, no evidence for loss of aerobic fitness! Perhaps graded exercise tackles a hyper-reactive CNS response to exercise-related physiological signals. Note that fear of physical activity becomes conditioned when it commonly exacerbates symptoms.
Warn that exercise programmes can make things worse rather than better. Exercise should only be done as part of supervised programme, with physiotherapist – don’t just tell them to go the gym more! Start below baseline activity level.
Other
Relaxation techniques recommended by NICE.
CBT – should only be offered to manage symptoms, improve functioning and reduce distress. Again, not a “cure”. Analysis of both CBT and graded exercise suggests that benefit comes from reducing inactivity.
Sleep hygiene important. Include rest periods in plan but avoid day time naps, especially since sleep doesn’t usually help anyway!
Many people find exclusion diets useful, esp bowel symptoms, not recommended but involve dietician if attempted anyway.
Equipment to maintain independence can improve quality of life and should be part of overall management.
Beware boom-bust! Many patients over do it when they have a period of relative wellness. Flares and relapses are to be expected. Trigger? New medical problem? Adjust plan as necessary.
Severe CFS can increase risk of pressure ulcers, DVT, vitamin D deficiency and contractures.
Prognosis
Important to be honest at time of diagnosis. More optimistic in young people. Most adults improve, some are able to return to usual activities but others experience long term symptoms or relapse.
PACE trial aimed for less than full restoration of health as “recovery”, future trials should use clinically relevant improvement and patient self-perception.
