Bilateral basal ganglia lesions

Evidence-based neurology checklist on bilateral basal ganglia lesions: Metabolic causes: hereditary Wilson’s disease Glutaric aciduria type I Methylmalonic acidemia Propionic aciduria Cockayne disease Hypomyelination with atrophy of the basal ganglia and cerebellum GM2 gangliosidosis Tay Sachs…

Metabolic causes: hereditary

  • Wilson’s disease
  • Glutaric aciduria type I
  • Methylmalonic acidemia
  • Propionic aciduria
  • Cockayne disease
  • Hypomyelination with atrophy of the basal ganglia and cerebellum
  • GM2 gangliosidosis
  • Tay Sachs disease
  • Infantile bilateral striatal necrosis (IBSN)
  • Canavan disease
  • Krabbe disease
  • Kearns Sayre syndrome (KSS)
  • Leigh disease
  • Citrullinaemia
  • Biotin responsive basal ganglia disease
  • Glucose 6 phosphate dehydrogenase (G6PD) deficiency
  • Fahr disease: causing calcification

Metabolic causes: acquired

Neurodegenerative causes

Infective causes

Toxic causes

Autoimmune causes

Other causes

Acronym

References

  1. Bekiesinska-Figatowska M, Mierzewska H, Jurkiewicz E. Basal ganglia lesions in children and adults. Eur J Radiol 2013; 82:837-849.
  2. Ho VB, Fitz CR, Chuang SH, Geyer CA. Bilateral basal ganglia lesions: pediatric differential considerations. Radiographics 1993; 13:269-292.
  3. Lim CC. Magnetic resonance imaging findings in bilateral basal ganglia lesions. Ann Acad Med Singap 2009; 38:795-798.
  4. Van Cauter S, Severino M, Ammendola R, et al. Bilateral lesions of the basal ganglia and thalami (central grey matter)-pictorial review. Neuroradiology 2020; 62:1565-1605. 
  5. Değerliyurt A, Gündüz M, Ceylaner S, Ünal Ö, Ünal S. Neonatal form of biotin-thiamine-responsive basal ganglia disease. Clues to diagnosis. Turk J Pediatr 2019; 61:261-266.
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