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 ↓↓↓

Swap!

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

Thank you!

(and so

me Path…)