Dentate nucleus lesions

Written and reviewed by Dr Ibrahim Imam, Consultant Neurologist. Last reviewed .

Dentate nucleus lesions. Fahr disease, Leigh syndrome, Glutaric aciduria type 1, L2 hydroxyglutaric aciduria. Fully referenced neurology checklist.

Metabolic causes

  • Fahr disease
  • Leigh syndrome
  • Glutaric aciduria type 1
  • L2 hydroxyglutaric aciduria
  • Maple syrup urine disease
  • Succinic semialdehyde dehydrogenase deficiency (SSADH)
  • Zellweger syndrome
  • Neuronal ceroid lipofuscinosis (NCL)
  • Krabbe disease
  • Alexander disease
  • Wilson’s disease
  • Gaucher disease

Neurodegenerative causes

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Other neurological causes

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Toxic causes

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Systemic causes

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Acronyms

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References

  1. Bond KM, Brinjikji W, Eckel LJ, Kallmes DF, McDonald RJ, Carr CM. Dentate update: imaging features of entities that affect the dentate nucleus. Am J Neuroradiol 2017; 38:1467-1474. 
  2. Bhattacharya K, Pendharkar H, Gupta AK. Imaging of dentate nucleus pathologies; a pictorial essay. Indian J Radiol Imaging 2018; 28:152-160. 
  3. Usha Chowdary MK, UC, Naveen Prasad SV, Gudla S, Bhuma V. Imaging of dentate nucleus pathologies: a case series. J Neurosci Rural Pract 2022; 13:146-150.
  4. Ziyeh S, Berlis A, Korinthenberg R, Spreer J, Schumacher M. Selective involvement of the globus pallidus and dentate nucleus in succinic semialdehyde dehydrogenase deficiency. Pediatr Radiol 2002; 32:598-600. 
  5. van der Knaap MS, Wassmer E, Wolf NI, et al. MRI as diagnostic tool in early-onset peroxisomal disorders. Neurology 2012; 78:1304-1308.
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