or Opsoclonus Myoclonus Syndrome, which is a much less suggestive name, and not only a partially correct description!
Features:
But also
Typically in young children, under 3. About half will have neuroblastoma. The rest assumed to be autoimmune.
=enterobius. Common in young children, probably due to hygiene issues. Also called pinworms. Small (1-2cm), like white threads. Can be intensely itchy, usually in night when worms emerge to lay eggs.
Mebendazole effective at killing live worms in gut, not effective if eggs in vagina. Licensed from age 2 but can be used off license below that. Does not kill eggs however, and main issue is reinfection so consider treating whole family, with repeat doses after 2 weeks when any eggs will have hatched. NHS Inform advice on hygiene:
Eggs probably become unviable after a few days if not in warm and moist environment… Children may of course pick up another threadworm infection from school/nursery…
Sometimes you just have to keep repeating treatment over months/years! Mebendazole is harmless (v little systemic absorption), although symptoms may be caused by clearance of large worm burdens.
Typically P. aeruginosa. A biofilm producing gram negative bacterium important in multiresistant infections, particularly in the immunocompromised and in cystic fibrosis.
Pretty ubiquitous in the environment, especially in lakes and rivers. Often found in vases of cut flowers, and in spa/whirlpool baths, where it is associated with folliculitis.
The ability to make biofilms makes infection difficult to treat, as the biofilm prevents penetration by antibiotics. The biofilm allows the bugs to survive in low nutrient environments.
Antibiotic resistance is a major issue, with specific anti-pseudomonal antibiotics often required viz tobramycin, ceftazidine.
Ingesting pseudomonas doesn’t pose much of a hazard, unless you are on antibiotics already and have a disturbed intestinal flora. Aerosolizing pseudomonas on the other hand can be lethal to mice.
[DOI: 10.1007/978-1-4419-0032-6_3]Common in children esp teenagers, often at rest, sharp but brief. Extremely rare to find a cause…
Differential:
So red flags would be syncope, colour change, sudden dizziness/confusion, sweating/clamminess suggesting cardiovascular compromise.
Assuming normal physical examination, and no family history of inherited cardiac problems (or sudden death), if not exertional then can be reassured. Pain killers not usually helpful as pain settles so quickly.
If exertional then needs ECG. Unlikely to be significant cardiac problem if normal.
[Archives 2014]Exclusively females (lethal in males? Or rate of germ cell mutations higher in male germ cells?). Virtually always sporadic – so not exactly X linked dominant.
MECP2 gene on X chromosome.
Developmental arrest at 6-18 months, then regression, loss of speech, stereotypies esp hands.
Often epilepsy, then complications of severe neurodisability eg chronic lung issues.
Not degenerative however – can live into middle life.
Gate theory useful – it’s not just about the stimulus coming to your nerve endings. That signal has to pass through a gate, to register in your brain, and different things affect whether the gate is more or less open or closed.
A feature of many chronic health conditions, eg juvenile idiopathic arthritis, migraine. Often a feature of chronic fatigue syndrome. Can be part of a functional disorder such as functional abdominal pain. In a limb, can lead to reflex sympathetic dystrophy. But often unexplained.
Think about PTSD – it’s all “in your head” but that doesn’t mean it’s nothing. Or phantom limb pain – there isn’t even a true signal coming from the limb, but you still feel pain.
Pain is performative – no one gets credit for trying to cope and hide it. But you can be accuse of “laying it on thick”.
Having a diagnosis helps socially.
Pain can make you a different person from who you were. Impatience and irritability can be understood, besides an inability to do some activities that might be important to identity (and to relieving stress). Social isolation is common.
Chronic pain also steals any sense of your future because it is too intolerable to imagine more pain.
Fear needs to be detached from pain.
Acceptance therapy – not to accept eternity of pain but to focus on progress and function.
[Haider Warraich podcast with Kate Bowler]
Children growing up on farms are less likely to develop allergies and asthma. Farming has been part of human culture for probably 7000 years.
It is widely accepted now that a symbiotic relationship with a diverse population of microbes in the environment, on the skin, in the gut and in the lung is necessary for a healthy immune system (“microbiome“). These microbes influence the balance between inflammation and immune tolerance. That relationship needs to be developed in early life, and nutrition is a major part.
Big European cross sectional studies – PARSIFAL and GABRIEL. Amish and Hutterites in US are genetically similar but Hutterites use industrial rather than traditional farming techniques (and have 4-6x the rate of hay fever and atopic sensitization).
Prenatal maternal exposure to farm animals is protective against eczema in the first 2 years of life, and against asthma symptoms pre-school.
Farm milk consumption in the first year of life is protective against respiratory allergies. Not clear what it is about it – more whey? Higher levels of cytokines or polyunsaturated fatty acids?
In children, exposure to cows and hay was protective against asthma. Some evidence for pigs, but risk seems to go up for sheep.
Mediators thought to potentially be N-gylcolylneuraminic acid (animals/pets) and arabinogalactan (plants).
Lipopolysaccharide (endotoxin) is widespread in the farm environment. Levels in mattresses inversely associated with hay fever, atopic sensitisation and asthma.
Lack of gut microbial diversity in first month of life predicts school age asthma.
Dietary diversity in first 2 years of life protects against asthma and allergic rhinitis. The link between gut microbes and lung health is thought to be short chain fatty acids, such as acetate and butyrate.
[Ped Allergy and Imm 2022]In a study of 589 children, 1-year microbiota maturation (based on metagenomics – genetic material of a community of micro-organisms – and metabolomics – metabolites in environment) closely related to eczema, asthma, food allergy and allergic rhinitis at age 5 years. Found a core set of “functional and metabolic imbalances” characterized by compromised mucous integrity, elevated oxidative activity, decreased secondary fermentation, and elevated trace amines. [Hoskinson, BC, Canada – Nature communications . 14(1):4785, 2023 08 29.]
Not great, particularly in young infants, but probably the best method of testing for type 1 allergy other than direct challenge.
You do need a patch of healthy skin however, so if bad eczema all over then not an option. Easiest is the medial forearm, but failing that, the back.
Some medicines interfere with skin prick testing (suppress it) – most commonly antihistamines. Non sedating should ideally be stopped 7 days before testing, sedating 48 hours. Other problem medicines are:
BSACI have SOP.
Anergy is the failure for testing to confirm allergy after a recent severe reaction – this is well recognised with venom allergy but in theory could affect both skin prick and IgE testing within a few weeks/months of a reaction.
Upper, lower, atypical, recurrent – all have specific definitions;
Diagnosis is ideally by culture – minimum of 1.25 ml, pref 2.5ml of urine in a 7ml red top boric acid container. White top only acceptable if received at lab on same day or day before, within hospital. Collection method important, ideally clean catch.
But given delay in getting culture result, urinalysis more practical, if less sensitive/specific. Nitrites have high positive predictive value so treatment usually started pending culture. Microscopy (for white cells and bacteria) is routinely done in infants under 3 months but needs to be requested between 3 months and 3 years. Greater than 100 wcc or 1000 organisms considered “numerous”, above 40 wcc or 500 organisms “moderate”.
All methods with exception of condoms more than 99% effective – if you use it as directed, of course! Combined – Rigevidon has v safe progesterone. Evra is a patch (replace each week for 3 weeks then week free). Nuvaring is monthly ring, less effected by GI problems but more expensive.
Contraindications for any combined product – migraine with aura, first 6 weeks breast feeding. DVT risk related to which progesterone is in combination – risk triples with levonorgestrel (Rigevidon), norethisterone, norgestimate (Cilest) but quadruples for others. But cf risk in pregnancy, more than 10x higher. UKMEC has risk table for family history etc.
Move towards only 4 pill free days – to avoid risk of ovulation if you miss day 1. Ultimately going to 63-84 days continuously (3-4 packs) but potentially confusing as need to stop and start on different days of the week.
Progesterone only pills were just barrier methods, due to effect on mucus. Cerazette (desogestrel) different, inhibits ovulation without other oestrogen effects. Bleeding is quite common in early days. Good for controlling cycle related problems eg menorrhagia, catamenial migraine. Good for young people because continuous. Depot good as lead in for implant (else weight gain as side effect).
Nexplanon is implant, under local, lasts 3 years. But side effects include irregular bleeding.
Enzyme inducers – cbz, phenytoin, topimarate! And st john’s wort! Rifampicin. Lamotrigine is not an inducer, but interacts with COCP/POP so avoid unless no other option, in which case needs dose adjustment and must be continuous method.
Consent to sexual activity often confused! Under 13 cannot consent (so different from medical treatment consent). Consent must be “free agreement” so sleep, coercion, under influence not allowed. Disclosure of child protection concerns is tricky due to fear of disengagement with service.