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.
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.
Emotional lability well described, mild anxiety and poor attention less common – although developing a new disability without any cognitive impairment may explain some of it.
Pregnancy is associated with first onset but also relapse!
High risk of cardiac involvement, as type of rheumatic fever – 71% of cases in Turkish study, of which nearly half silent (no findings on clinical examination). Significant risk of long term morbidity, even if chorea never comes back, so always echo and give penicillin prophylaxis.
Diagnosis
Essentially clinical, with supportive evidence of recent streptococcal infection (ASO titre, throat swab). But recognised that infection can be up to 6 months before, or too mild to really notice.
Other tests are directed at differential diagnosis – lumbar puncture, MRI brain etc. Putaminal enlargement described.
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.
Management
There is a UFMG rating scale for SC, from Brazilian Universidade Federal de Minas Gerais (UFMG), for research purposes but only looks at motor function.
Occupational and physiotherapy useful for maintaining function and muscle tone.
Treatment with valproate is effective for controlling symptoms but doesn’t speed up recovery. 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 [2012, 2017], Brazil [Cardoso 2005], immunoglobulin [Holland, 2016, South Africa 2016]. Some improvement can be seen within a few days of IV steroids. In Italy, prednisolone reduced average duration of symptoms from 9 weeks to 4 weeks, and these were severe cases [Fusco 2012]. 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]
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. If isolated chorea, arguable whether cardiac risk justifies or not.
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.
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 being 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
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.
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
US IOM expert panel have rejected this name, 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 doi: http://dx.doi.org/10.1136/bmj.h775]
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.
Recent 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.
Relaxation techniques recommended by NICE. CBT also effective. Analysis of both CBT and graded exercise suggests that benefit comes from reducing inactivity.
Sleep hygiene important. 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.
But are these cures? PACE trial aimed for less than full restoration of health as “recovery”, future trials should use clinically relevant improvement and patient self-perception.
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.
Binge Eating Disorder (without the compensatory behaviours of bulimia eg vomiting, exercise)
Selective/Restrictive Eating
DSM-IV Criteria for Anorexia Nervosa
Body weight at or below 85% of that expected
Fear of gaining weight or becoming fat, even though underweight
Disturbed perception of body weight or shape
Amenorrhea, at least 3 consecutive cycles
Disturbance in way one’s body shape is experienced (?)
ICD-10 adds a couple of things:
BMI < 15
Weight loss caused by food avoidance, self induced vomiting, purging, excessive exercise (not in DSM)
loss of libido in men
Pubertal delay if early onset
For children, these strict criteria can overlook significant disordered eating – wide variation in weight and height gain through puberty; menses not present else irregular. Levels of cognitive development obviously vary. Abnormal food behaviours eg slow eating, hiding may have been present from a young age. One type of eating disorder can change into another.
Interest in healthy eating and exercise may initially give impression that child is well. Dieting is miserable for most people, but for some it appears to relieve anxiety, which can lead to a vicious cycle. Sometimes it is specific foods that are feared.
BMI <2nd centile prob more useful for kids. GOSH Criteria (Nicoles, Chater & Lask 2000) talks about determined wt loss, abnormal cognitions about weight/shape but also morbid preoccupation. Similarly, Bulimia Nervosa defined as Recurrent binges and purges, Sense of lack of control, Morbid preoccupation with weight or shape. Purging is any behaviour to prevent weight gain including self induced vomiting, fasting, dieting, excessive exercise, misuse of medicines such as diuretics or laxatives.
Questions:
What did you eat yesterday? Are there foods you don’t eat any more?
Are you more interested in food and cooking?
Are you trying to cut back?
Does it ever feel like your eating gets out of control?
What happens if you can’t exercise?
Have you been making yourself sick? Do you drink water to prevent hunger?
What do you see when you look in the mirror? (Body dysmorphia = perception of shape, size that is unrelated to reality). Is there a weight you would like to be? What sort of things do you look at online?
Explore mood and risk of self harm. Anhedonia (inability to enjoy anything)common. Ask direct questions about abuse or neglect (explain this is routine).
Early intervention associated with better outcomes. Poor outcome in anorexia if patient does not receive effective treatment in first 3 years.
Principles –
Give diagnosis – may not be appreciated. Anosognosia = inability to see weight loss or failing health (and therefore others with that viewpoint are irrational or unkind)
Strengthening family relationships away from food (in many families tends to be around meals, snacks, eating out, and many conversations around favourite foods etc)
Conceptualising the eating problem as being separate from the young person. Eating problem as “bullying voice”. Avoid discussions of weight or body image (“you look healthier” can be misinterpreted as “you look fat” by eating problem).
Not a choice, not rational. Alexithymia (inability to express feelings) common. Genetic heritability accounts for approximately 50–80% of the risk of developing an eating disorder, often pre-existing tendencies towards anxiety, inflexibility, difficulties with emotional regulation, enhanced sensitivity towards punishment. [Proposed mechanism here]
Families are not to blame!
Food is medicine. Enforcing regular, balanced meals and snacks (3 of each daily) as a family improves mood, behaviour as well as physical symptoms. Terrifying at first, of course. Metabolism often ramps up once refeeding begins, so a huge increase in intake is often required to achieve restoration of healthy weight (and catch up growth) in anorexia. For bulimia, regular pattern of eating more important.
Don’t allow meal choices or negotiation, discourage involvement in or observation of food preparation, which reinforces disordered thinking. Reduces anxiety when not required to make “difficult” choices about problem foods.
Avoid regular weighing and other forms of body checking
Full recovery is possible, especially when detected early eg months rather than years.
Can be helpful to offer option not to be told weight. Beware concealing weights on body, water loading before weighing day. Praise honesty, highlight confidentiality, agree sharing of information with parents.
Parents can become used to “new normal” of disordered eating, and might not appreciate risks. Alternatively, young person might feel threatened by alliance between doctor and parents.
Target weight is tricky – what is required for normal bodily function? What was growth/puberty trajectory before eating restricted? So healthy thoughts about food, normal periods (often 9 months or more), return of premorbid personality etc. Fluctuating weight gain may be due to metabolism, fluid shifts, concealed weights or water loading, concealed purging (silent exercise eg crunches in bed).
Where food refusal is an issue, energy dense food is required – increase fat content, avoid water or diet drinks, leave fruit/veg till after other foods eaten. Smoothies or milkshakes often better tolerated. Bloating and nausea with refeeding common initially but should improve.
Family based treatment is recommended by NICE as first line. Emphasises that parents initially take back responsibility for feeding, then gradually handing it back to the young person. Minimisation of blame. If ineffective then CBT.
Psychotropic medication not recommended – metanalysis found no benefit from antidepressants in anorexia.
No evidence based guidelines for re-introduction of nutrition/energy in adolescents!
For the majority of patients, 40kcal/kg/day (1200kcal/day) appears to be safe – don’t start a meal plan with less calorific content than they were receiving prior to admission, although difficult when history of the amount taken is unclear.
The meal plan should comply with normal macronutrient guidelines (10-15% protein, 30-35% fat, 50-60% carbohydrates).
Increase the meal plan by 200kcal/day until 2000kcal/day is achieved.
Fluid 50ml/kg/d for 15yr+, 55ml/kg/d 11-14yr (Shaw et al). GOSH use standard paediatric fluid requirement calculations.
Promote weight gain 0.5kg/week (NICE 2004, Junior Marsipan 2012). Ignores malnutrition, of course. Percentage weight for height used, but easiest to divide BMI by median BMI for age/sex – 85% is underweight, 90% is satisfactory.
Check electrolytes, calcium, phosphate, magnesium, liver function, vit D on admission. QTc must be calculated MANUALLY (to find end of T, draw tangent through steepest part of curve).
All patients should be prescribed prophylactic dose of Vitamin D at a dose of 800IU/day whilst waiting for Vitamin D levels to be reported.
Prophylactic phosphate should not be routinely prescribed, however it should be considered where:
There has been a previous history of re-feeding syndrome.
Multiple risk factors
Consider Thiamine where starvation has been very prolonged (e.g. greater than one year at very low weight and poor intake) or there is a concern about vitamin deficiency.
A low phosphate (<1.1 mmol/L) before initiating feeds is unusual (see below) and should be corrected as soon as is possible on the day of admission:
Low phosphates should be discussed with the responsible consultant.
Give two sandoz-phosphate tablets and commence TDS regular phosphate regime.
Recheck U&E in 12 hours and monitor clinically (see below).
Do not make any increases on the feeding regime until phosphate has been corrected.
If phosphate is still low at 12 hours then consider repeated double dose, or IV correction. This is unusual.
Other causes of low phosphate should be excluded – in particular Vitamin D deficiency and hypoparathyroidism: check PTH and Vitamin D with next set of bloods (if hasn’t already been checked). These bloods should not hold up commencing of feeding once phosphate is normalised.
If phosphate is significantly low (<0.5) consider IV replacement – this will generally mean transfer to a medical ward.
Phosphates that are potentially dangerously low (<0.3) should be managed on a medical ward/PICU and discussions should occur with the consultant and CSPs about transfer before commencing feeding.
Refeeding syndrome
Biochemical abnormalities AND cardiovascular and neurological findings. For most children and young people, the most significant early finding is a fall in phosphate. Increased requirement for phosphate as the body switches back to carbohydrate metabolism, plus chronic phosphate depletion due to starvation. Phosphate levels in the blood begin to fall, and sequelae may follow. Potassium, Mg also fall. Onset is in first 48 hours, up to first five days of initiating feeding. Cardiac arrest has occurred.
Very low weight (Junior MARSIPAN defines as <70% median BMI (red) or between 70 and 80% median BMI (Amber)) or faster rates of weight loss (recent loss of weight of 1kg or more/week for two consecutive weeks (red) or loss of weight of 500g-999g/week for two consecutive weeks (amber))
Minimal or no feeding in 3-4 days prior to admission, or before commencing re-feeding, defined as acute food refusal or estimated calorie intake 400-600kcal per day (red) or severe restriction (less than 50% of required intake: amber)). If either is in combination with vomiting or laxative misuse this will increase the risk.
Previous history of re-feeding syndrome.
Neutropenia (low white cell count) on full blood count (FBC)
All patients considered at risk of re-feeding syndrome should be monitored for clinical signs of the re-feeding syndrome:
Resting tachycardia (differential for this includes anxiety).
Oedema or swelling, especially in the legs.
Confusion or altered conscious state (always check glucose in this case).
Patients should have:
Daily inspection for any signs of oedema (in particular peripheral oedema) for first five days.
Three times/day resting pulse and lying and standing blood pressure for first five days.
Monitor for biochemical/blood parameters of the re-feeding syndrome:
Daily urea, creatinine, sodium, potassium, phosphate, magnesium daily for five days. The drop in phosphate seen when re-feeding will normally occur within 48-72 hours.
