CASE 11760 Published on 21.04.2014

Lhermitte-Duclos disease

Section

Neuroradiology

Case Type

Clinical Cases

Authors

DR. CHIRAG KANJIBHAI GHODASARA, DR. NISHA SATISHKUMAR DOSHI, DR. PINKAL PATEL, DR. JAY THAKKAR, DR. MANISH YADAV.

SANYA IMAGING CENTRE ,RAJKOT CIVIL HOSPITAL ,RAJKOT ,GUJARAT,INDIA 360001
Email:drchiragghodasara@gmail.com
Patient

20 years, female

Categories
Area of Interest Neuroradiology brain ; Imaging Technique MR, MR-Diffusion/Perfusion, MR-Spectroscopy
Clinical History
Patient has a headache since 20 days, then suddenly the patient developed double vision. There is no history of vomiting or any focal neurological deficit.
Imaging Findings
Altered signal intensity is noted involving left-sided superior cerebellar hemisphere, cerebellar vermis and posterior part of cerebellum in mid line.

Lesion appears hyperintense on T2W images and partially suppressed on FLAIR images and hypointense on T1W images. Lesion shows striated appearance with hypo and isointense band on T1W images and hyper and isointense band on T2W images. The lesion shows internal dysplastic arrangement of cerebellar folia. The lesion does not show any post contrast (gadolinium) enhancement.

The lesion causes significant compression over the 4th ventricle and brain stem, predominantly in the mid-brain on the left side. There is mild dilatation of bilateral and 3rd ventricles. It suggests obstructive hydrocephalus. The lesion causes mass effect in posterior fossa. There is cerebellar tonsillar herniation through posterior part of foramen magnum. It causes mild compression over the medulla.

On 144TE MR spectroscopy, there is reduction of Cho/Cr ratio and MI/Cr ratio. NAA is not significantly reduced. There is elevation of lactate and MI.
Discussion
Lhermitte-Duclos disease is rare tumour of the cerebellum. It is also called as dysplastic cerebellar gangliocytoma. It is hamartomatous lesion on histology. It is considered as a WHO grade I tumour [1].
Symptoms of the tumour is mostly due to compression over the 4th ventricle or brain stem. The tumour is mostly presented in a younger age group [2].
Tumour is associated with Cowden’s syndrome, disorder of cortical malformation, polydactyly, macroglossia, hydromyelia, localised gigantism.[2]

On MR imaging, tumour appears hyperintense on T2 and hypointense on T1 with preservation of normal cortical striation. Tumours shows a striated pattern. Tumours do not show any post contrast (gadolinium) enhancement. Other cerebellar tumours show post contrast (gadolinium) enhancement. In contrast to cerebellitis, tumours do not show restricted diffusion. On MR spectroscopy, the tumour usually shows reduction of NAA and reduction of Cho/Cr & MI/Cr ratio. The tumour shows increased lactate on spectroscopy. On FDG-PET, tumour shows hyper metabolism and increased FDG uptake [1].

Initially, shunting is recommended to relive obstructive hydrocephalus. Surgical resection is often curative and there is less chances of recurrence [2].
Differential Diagnosis List
probable Lhermitte-Duclos disease
cerebellitis
cerebellar glioma
Final Diagnosis
probable Lhermitte-Duclos disease
Case information
URL: https://www.eurorad.org/case/11760
DOI: 10.1594/EURORAD/CASE.11760
ISSN: 1563-4086