Curriculum standards

Knowledge guides

LG25: Neonatal neurology

Key presentations and conditions

Advanced Trainees will have a comprehensive depth of knowledge of these presentations and conditions.

View

Epidemiology, pathophysiology, and clinical sciences

Advanced Trainees will have a comprehensive depth of knowledge of the principles of the foundational sciences.

View

Investigations, procedures, and clinical assessment tools

Advanced Trainees will know the scientific foundation of each investigation and procedure, including relevant anatomy and physiology. They will be able to interpret the reported results of each investigation or procedure.

Advanced Trainees will know how to explain the investigation or procedure to patients, families, and carers, and be able to explain procedural risk and obtain informed consent where applicable.

View

Important specific issues

Advanced Trainees will identify important specialty-specific issues and the impact of these on diagnosis and management and integrate these into care.

View

Presentations

Abnormal movements
Acidosis
Bowel changes, such as:
- diarrhoea
- reduced bowel motions
- stained meconium
Breathing difficulties, such as:
- apnoea
- respiratory distress
Cyanosis / Pallor
Developmental and/or growth delays
Fever or low body temperature
Hyperflexibility / Hypotonia
Intrauterine growth restriction (IUGR)
Lethargy
Low heart rate
Microcephaly
Muscle weakness
Neonatal seizures
Poor reflexes
Spasms
Tonic motor activity and posturing
Vomiting and/or poor feeding

Conditions

Congenital and neonatal infections
Disorders of prematurity
Hypotonia:
- disorders of the anterior horn cell, such as:
  - spinal muscular atrophy
- disorders of the peripheral nerve
- genetic and central nervous system disorders, including:
  - Prader–Willi syndrome
- myasthenic syndromes
- myopathy
- myotonic dystrophy
- systemic disease / illness
Hypoxic ischaemic encephalopathy, including:
- periventricular leukoencephalopathy
Inborn errors of metabolism
Neonatal seizures
Postnatal complications of maternal antenatal disease
Stroke:
- haemorrhagic
- ischaemic

For each presentation and condition, Advanced Trainees will know how to:

Synthesise

recognise the clinical presentation
identify relevant epidemiology, prevalence, pathophysiology, and clinical science
take a comprehensive clinical history
conduct an appropriate examination
establish a differential diagnosis
plan and arrange appropriate investigations
consider the impact of illness and disease on patients and their quality of life when developing a management plan

Manage

provide evidence-based management
prescribe therapies tailored to patients’ needs and conditions
recognise potential complications of disease and its management, and initiate preventative strategies
involve multidisciplinary teams

Consider other factors

identify individual and social factors and the impact of these on diagnosis and management

Clinical correlates, pathophysiology, and prognosis of neonatal encephalopathy, including:
- hypoxic ischaemic encephalopathy
- periventricular leukomalacia
Differential diagnosis, investigation, and management plans for neonatal seizures
Disorders of metabolism in the newborn period
Evaluation and management of perinatal stroke
Impact of maternal disorders and prenatal neurologic diagnoses on the fetus
Neurodevelopmental features and examination findings of normal preterm and term infants

Cerebrospinal fluid (CSF) procedures and investigations

Lumbar puncture (LP) and interpretation of investigation results, such as:
- basic CSF analysis:
  - cell count
  - cytology
  - immunological tests
  - microbiological tests
  - paired serum and:
    - CSF lactate
    - CSF glucose level
  - protein level
  - xanthochromia
- Special tests:
  - CSF amino acids
  - neurotransmitters, including:
    - neopterin
  - paired serum and CSF oligoclonal bands

Clinical neurophysiology investigations

EEG:
- amplitude integrated EEG (aEEG)
- standard EEG
- video EEG
Electromyography (EMG):
- motor and sensory studies
- needle EMG
- nerve conduction studies (NCS)
- repetitive nerve stimulation
- single-fibre EMG

Neurogenetic investigations

Genetic testing, including, but not limited to:
- chromosomal testing, such as:
  - karyotype
  - microarray
- genomic testing, including whole exome or genome sequencing
- mitochondrial genome sequencing
- targeted panel testing
Referral to a neurogeneticist

Neuroimaging investigations

CT, including:
- CT angiography
Magnetic resonance:
- angiography (MRA)
- spectroscopy (MRS)
- venogram (MRV)
MRI

Other investigations

Cardiac investigations:
- 24-hour electrocardiogram monitoring
- ECG
- echocardiography
Other laboratory tests:
- ammonia
- blood gas
- creatine kinase
- liver function tests
- thrombophilia screen
- thyroid function tests
- transferrin isoforms
- white cell enzymes
Polysomnography

Approach to breaking bad news and future counselling of a child with neurological diagnosis at birth
Approach to emergency management and escalation of therapy for neonatal seizures
Approach to introduction of the palliative care team
Approach to neurological assessment of a newborn, including:
- antenatal
- birth
- comprehensive examination
- genetic history
- maternal
Approach to the hypotonic newborn
Discussion of life-limiting diagnosis and end-of-life considerations