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Case Reports
. 2019 Jun 14:33:118.
doi: 10.11604/pamj.2019.33.118.16809. eCollection 2019.

The Lhermitte-Duclos disease: a rare bilateral cerebellar location of a rare pathology

Mehdi Borni  1 , Brahim Kammoun  1 , Fatma Kolsi  1 , Souhir Abdelmouleh  1 , Mohamed Zaher Boudawara  1
Affiliations
Case Reports

The Lhermitte-Duclos disease: a rare bilateral cerebellar location of a rare pathology

Mehdi Borni et al. Pan Afr Med J. .

Abstract

Dysplastic gangliocytoma or Lhermitte-Duclos disease is a rare disorder characterized by a slowly progressive unilateral tumour mass of the cerebellar cortex. It is probably hamartomatous, although the exact pathogenesis remains unknown. Lhermitte-Duclos disease was recently encountered to be part of a multiple hamartoma-neoplasia complex (Cowden's syndrome). It typically presents in young adults, although it has been encountered at all ages. We present the case of bilateral cerebellar location of this pathology in a 50-year-old man presented with a progressive onset and worsening of headaches accompanied by nuchal rigidity, photophobia and nausea awakening each morning. Upon physical examination, the patient was awake with a discrete right vestibular syndrome made of positive Romberg without nystagmus. Magnetic Resonance Imaging (MRI) was performed and revealed salient "tiger stripe" appearance of the bilateral cerebellar cortex relevant to a Lhermitte-Duclos disease.

Keywords: Dysplastic gangliocytoma; cowden's syndrome; gangliocytoma.

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Conflict of interest statement

The authors declare no competing interests.

Figures

Figure 1
Figure 1
Cerebral MRI: two cerebellar lesions: iso signal weighted T1, hyper signal weighted T2, seat of small area of necrosis, heterogeneously enhanced, with hyper signal diffusion without decrease of the ADC and surrounded by oedema
See this image and copyright information in PMC

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