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.