Contents
Bbilirubin-induced brain dysfunction.
Aetiology
Risk factors
- Premature birth
- Rh incompatibility
- Polycythemia – often present in neonates
- Sulfonamides (e.g. co-trimoxazole) – displaces bilirubin from serum albumin
- Crigler-Najjar syndrome type I
- Gilbert’s syndrome
- G6PD deficiency
- Bruising
Clinical features
I. Acute bilirubin encephalopathy (ABE)
Acute state of elevated bilirubin in the CNS.
- Clinical features:
- Lethargy, decreased feeding, hypotonia or hypertonia, a high-pitched cry, spasmodic torticollis, opisthotonus, setting-sun sign, fever, seizures, and even death
- If the bilirubin is not rapidly reduced, ABE quickly progresses to chronic bilirubin encepalopathy.
II. Chronic bilirubin encephalopathy (CBE)
Chronic state of severe bilirubin-induced neurological lesions.
- Reduction of bilirubin in this state will not reverse the sequelae.
- Clinical features:
- Movement disorders
- Athetoid cerebral palsy and or dystonia, 60% have severe motor disability (unable to walk).
- Auditory dysfunction
- Auditory neuropathy (ANSD)
- Oculomotor impairments
- Nystagmus, strabismus, Impaired upward or downward gaze, and/or cortical visual impairment
- Dental enamel hypoplasia/dysplasia of the deciduous teeth
- Gastroesophageal reflux
- Impaired digestive function
- Movement disorders
III. Subtle bilirubin encephalopathy (SBE)
SBE is a chronic state of mild bilirubin-induced neurological dysfunction.
- Clinical features:
- Neurological, learning and movement disorders
- Isolated hearing loss and auditory dysfunction
Diagnosis
Imaging
MRI
