T. Cymes & W. Cymes Stage 3 student doctor University of Cambridge.
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Transcript of T. Cymes & W. Cymes Stage 3 student doctor University of Cambridge.
PaediatricsNeurological and musculoskeletal systems
T. Cymes & W. CymesStage 3 student doctorUniversity of Cambridge
Plan
Examination
Tumours Cerebral palsy Epilepsy Neurocutaneous
syndromes Meningitis
Common problems at birth Developmental dysplasia
of hip Pain Limp Muscular dystrophies Juvenile idiopathic arthritis
Examination
Unlikely to get a child to examine
Videos of conditions Pictures of conditions Case based discussion including a neurological
disorder History pointing towards neurological disorder
Remember the viva!
Brain tumours
Pathology Most primary and infratentrorial Astrocytomas (40%) and
glioblastomas (20%) most common
Symtpoms Headaches Morning vomiting Increased circumference in infants Behaviour change Clumsiness / change in gait Slowed learning
Signs Can be none Papilloedema Focal neurology
Investigations MRI
Management Need surgery ± chemotherapy
Palliation might be the only option
Cerebral palsy
Non-progressive movement and posture abnormality due to insult in utero / by 2yo
2 per 1000 live births Causes
80% antenatal▪ Vascular occlusion▪ Brain maldevelopment
10% hypoxic-ischaemic injury 10% postnatal
Spastic CP “clasp knife” hyperreflexia Hemi/di/quadriplegic Stiff crossing legs
Dyskinetic CP Chorea, dysthonia, athetosis etc
Ataxic (hypotonic) CP ± cereberral ataxia later in life
Intelect can be intact!
Cerebral palsy
Management MDT approach▪ Doctor▪ Nurses▪ Specialist nurses▪ Physiotherapist▪ Psychologists▪ Teachers▪ And more!
MRI might identify cause
Be honest with parents Treat the cause if possible▪ Hydrocephalus▪ Kernicterus
Physiotherapy to avoid contracures, maximise mobility
Psychological help
NB medicolegal issues
Epilepsy
Epidemiology 0.05% incidence, 0.5% prevalence
Types Generalised vs focal Simple vs complex ? Secondary generalisation
Generalised Absence Myotonic Tonic-clonic
Focal seizures Frontal – Jacksonian march Temporal – aura, automatism,
deja/jamai vu
Causes Idiopathic Febrile Secondary to space occupying lesion
Assessment Before – during - after Great to get video! Look out for underlying disease
Investigations EEG MRI / fMRI
Epilepsy
Syndromes West syndrome (4-6mo) Lennox-Gastaut syndrome
(1-3yo) Childhood absence epilepsy
(4-12yo) Juvenile myoclonic epilepsy
(adolescent – young adults)
Management Anti-epileptics▪ Different drugs work on different
types▪ Can exacerbate some types of
seizures▪ Often can stop when 2y seizure-free
Surgery if tumours School needs to know▪ And how to deal with seizure!
Some have educational problems Driving issues around adulthood
Neurocutaneous syndromes
Neurofibromatosis type 1 1:3000 live births Cutaneous features after
puberty Optic glioma Lisch nodule (iris
hamartoma) Eye protrusion (sphenoid
dysplasia)
NF2 associated with bilateral vestibular schwannomas
Tuberous sclerosis 1:9000 live births (70% new
mutation) Neurological features:▪ Infantile spasm▪ Developmental delay▪ Focal epilepsy▪ Retinal phakomata
Other features:▪ PKD▪ Heart rhabdomyomata
CT / MRI for further lesions
Source: webmd.com
Source: actasdermo.org
Source: meded.ucsd.edu
Source: mynotes4usmle.tumblr.com
Source: e-ijd.org
Source: consultantlive.com
Meningitis
5-10% mortality 10% survivors have impairment
Clinical features Fever Non-specific in infants / young
child▪ Bulging fontanelle
Irritable, drowsy etc Meningism if old enough ± septicaemia▪ Purpura in menigococcal
Need LP unless contraindicated
Management Empirically – benzilpenicillin IM
before transfer to hospital IV antibiotic therapy if bacterial Aciclovir IV if herpes Dexamethasone to reduce
complications Complications
Hearing loss Local cerebral infarcion Hydrocephalus Subdural abscess
Source: meningitisnow.org
Age Pathogens
<3 months GBSE. Coli
1month – 6yo MeningococciPneumococciH. Influenzae
>6yo MeningococciPneumococci
Viral (67%) EnterovirusesEBVMumpsadenoviruses
Bacterial Viral TB
Appearance
turbid clear turbid/clear/viscous
WBC neutrophils lymphocytes
lymphocytes
Protein ↑↑ ↑/normal ↑↑↑
Glucose ↓↓ ↓/normal ↓↓↓
Common problems at birth
Positional talipes Normal, can correct with passive manipulation
Talipes equinovarus Fixed foot position, not correctible with manipulation▪ Adducted foot with inwardly rotated heel
1 in 1000, 2 male : 1 female Ponsetti method (plaster casting and bracing) Corrective surgery CHECK: neuromuscular disorder? DDH?
©http://www.kkh.com.sg
Developmental Dysplasia of the Hip Variable disorder of the hip: dysplasia ---- subluxation ---- dislocation
1.3 per 1ooo live births (6-10 on screening) EARLY DETECTION KEY
Baby check▪ Barlow manoeuvre - hip dislocates posteriorly?▪ Ortholani manoeuvre – hip relocates into acetabulum on abduction?▪ F/U – hip USS
Risk factors: FH Breech presentation
Treatment Splint in flexed and abducted position Surgery if above fails
©http://en.wikipedia.org
©www.orthopediatrics.com
Pain?
Chronic Growing pains
3-12 years old Symmetrical throughout LL (bones + muscles) Normal examination; no limit to daily activities NOT present at the start of the day
Hypermobility ‘Complex regional pain syndromes’
Acute Osteomyelitis
Fever, painful immobile limb, swelling Staph. aureus, Strep, H. Influenzae▪ In SCD: Salmonella
Blood cultures and IV Abx!!!
Malignant disease ALL (often at night) Bone tumours (rare!)
Pain?
Source: http://jrheum.org/
Limp?
MSK: Transient synovitis Perthes disease Slipped Upper Femoral Epiphysis Missed DDH
Neurological?? Remember ↑ICP▪ Space-occupying lesion▪ Hydrocephalus▪ Brain tumour
Source: .wheelessonline.comSource: radiopaedia.org
Muscular dystrophies
Duchenne muscular dystrophy X-linked: dystrophin deletion muscle necrosis ↑CPK in serum 1 in 4,000 male infants Gowers sign, calves pseudohypertrophy Management: MDT!!!▪ Symptomatic▪ Corticosteroids in ambulant!
Becker muscular dystrophy Some functional dystrophin present SLOWER progression Life expectancy – even normal!
©www.alison-burke.com
©www.prsharma.com.np
Juvenile Idiopathic Arthritis
Persistent joint swelling (>6/52) presenting before 16 years of age in the absence of infection or any other cause
1 in 1000
Morning joint pain and stiffness Long term: genu valgus, different length digits, swelling of the
joints
Treatment: analgesia + immunosuppression + MDT
Complications Chronic anterior uveitis
©bestpractice.bmj.com
©www.nras.org.uk