Monday 29 June 2015

LHERMIETTE DUCLOS DISEASE

T2-AXIAL IMAGE

T1-AXIAL IMAGE

FLAIR-AXIAL IMAGE

CONTRAST ENHANCED T1 AXIAL IMAGE
A 7 year old child presented with ataxia and dizziness. MRI of brain shows increased volume and abnormal signal in right cerebellar hemisphere with a characteristic "striated" appearance on T2. There is mass effect on the 4th ventricle. It shows mild patchy enhancement.

SALIENT RADIOLOGICAL FEATURES:
  • It is also called dysplastic cerebellar gangliocytoma
  • Well defined cerebellar lesion involving usually unilateral hemisphere. 
  • It may be large and involve the entire hemisphere as in this patient.
  • Widened cerebellar folia with a characeteristic "layered" or "striated"appearance on T2
  • It is hypointense on T1 and may show striated appearance with alternating layers of isointense and hypointense signal
  • Calcification may be seen rarely
  • These may show bright signal on DWI due to T2" shine through"
  • There is no diffusion disturbance on ADC map.
  • Rarely, some lesions may enhance
  • MRS may show elevated lactate. There is diminished NAA, choline and myoinositol
REFERENCES:


Jones BV: Dysplastic Cerebellar Gangliocytoma. Diagnostic Imaging Brain: First Edition. Amirsys: I-6-70, 2005.  

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