40 y/o, History of Seizures

Case History: A 40-year-old patient presented with history of seizures.

Figure 1A. FLAIR images show striated hyperintensity left cerebellar hemisphere.

Figure 1B. FLAIR images show striated hyperintensity left cerebellar hemisphere.

Figure 1C. FLAIR images show striated hyperintensity left cerebellar hemisphere.

Figure 2. T2W image shows hyperintensity in left cerebellar hemisphere.

Figure 3. T1WI shows low signal in left cerebellar hemisphere.

Figure 4. DWI shows no diffusion restriction.

Figure 5. T1 contrast shows no enhancement.

Diagnosis: Lhermitte-Duclos disease

CT may show a nonspecific hypo attenuating cerebellar mass; calcification is sometimes seen.

FDG-PET shows increased uptake.

Lhermitte-Duclos disease (LDD), also known as dysplastic cerebellar gangliocytoma, is a rare tumor of the cerebellum.

Lhermitte-Duclos disease typically presents in young adults.

Symptoms are typically related to raised intracranial pressure, obstructive hydrocephalus and, to a lesser degree, cerebellar dysfunction.

The dysplastic mass grows very slowly, and initial treatment revolves around treating hydrocephalus.