Appendix C
First Assessment Checklist

Written and medically reviewed by
Prof. Dr. Burak TatlıSpecialist in Pediatric Neurology & Developmental Pediatrics
İstanbul University-Cerrahpaşa Faculty of Medicine · Nörogender Association
Last reviewed:
Intended for healthcare professionals
History
- Prenatal: reduced fetal movement, polyhydramnios, congenital contractures.
- Perinatal: hypotonia, weak cry, feeding difficulty, respiratory support.
- Motor milestones with dates; delay, plateau or loss.
- Fatigability and diurnal variation.
- Family history, consanguinity, signs of myotonia in the mother, early cardiac death in the family.
Examination
- Observation: gait, rising from the floor (Gowers) — video where possible.
- Strength (MRC), distribution, facial involvement; deep tendon reflexes.
- Muscle bulk: atrophy or pseudohypertrophy; tongue fasciculations.
- Sensory examination.
- Spine, hips, contractures, foot deformity.
- Breathing pattern, chest wall shape, cough strength; growth measurements.
First-line investigations
- Creatine kinase (at rest); AST, ALT, GGT.
- Full blood count, electrolytes, calcium-phosphate-ALP.
- Thyroid function, 25-OH vitamin D.
- In selected cases, lactate and dried blood spot acid alpha-glucosidase.
Plan
- Has genetic testing been directed by localization?
- Has the baseline functional measure been recorded?
- Have respiratory and cardiac assessments been arranged?
- Has referral for genetic counselling been made?
- Has the anaesthetic risk card been issued and a written summary given to the family?
- Has the date of the next appointment been set?
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