Neurodevelopmental Medicine

Question 1

What are conditions that can mimic cerebral palsy?

Answer 1

Muscular dystrophies

• Becker’s
  
  • Proximal myo[pathy
  • Gowers sign
  • Calf hypertrophy

Mitochondrial disease

• Leigh syndrome
  
  • Regression

Metabolic conditions

• Glutaric acidaemia type 1
  
  • Tx: low protein, L carnitine + riboflavin
• Lesh Nyhan
  
  • Self mutilate
• Sjogren Larsson
  
  • Ichthyosis
• Glucose transporter 1 deficiency (dyskinesia CP)
  
  • Dx: LP CSF BSL < 2.2
  • Tx: ketogenic diet

Malformations syndrome

• Miller Dieker lissencephaly
• Rett Syndrome
  
  • Microcephaly
  • hand wringing
  • Hyperventilation

Question 2

Surgical procedures for spastic hip displacement

Answer 2

Preventative (soft tissue surgery)

• Adductor longus release
• Gracilis release
• Adductor brevis release
• Iliopsoas lengthening
• Obturator neurectomy

Reconstructive (redirectional osteotomy)

• Femoral varus derotation osteotomy
• Pelvic osteotomy
• Combined procedure

Salvage

• Excision of proximal femur
• Valgus osteotomy
• Arthroplasty
• Arthrodesis
• Replacement

Question 3

Management of spasticity in CP

Answer 3

Localised / Segmental Spasticity

• Botulinum toxin A is effective + generally safe un upper + lower extremities
• Conflicting evidence RE functional improvement as severe generalised weakness can occur

Generalised Spasticity

• Diazepam short term (SE= sedation, drowsiness, hypersalivation, weakness)

Question 4

Poor Salivary Control

• Consequences
• Contributors
• Treatment

Answer 4

Consequences

• URTI
• Aspiration
• Sleep disturbance
• Skin excoriation
• Social

Contributors

• Poor dental health
• Hypotonia
• Upper airway issues
• Pain

Treatment

• Referrals / reviews
  
  • Dental + speech
• Non-pharm
  
  • Positioning, dental, hygiene, bibs
• Pharmacological
  
  • Intranasal steroids
  • Anticholinergics
  • Antimuscarinics
  • Tricyclic antidepressants (amitriptyline)
  • Alpha adrenergic agonists
• Surgical
  
  • Botox, excision/ ligation fo salivary glands, relocation of ducts

Question 5

Risk of T21 for future pregnancies if mother or father parent has robertsonian translocation

Answer 5

Mother = 10-15%

Father = 2%