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

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 o...

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Bibliographic Details
Published in:The Pan African medical journal 2019, Vol.33 (118), p.118
Main Authors: Borni, Mehdi, Kammoun, Brahim, Kolsi, Fatma, Abdelmouleh, Souhir, Boudawara, Mohamed Zaher
Format: Article
Language:English
Subjects:
Case Report
cowden´s syndrome
dysplastic gangliocytoma
gangliocytoma
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Summary: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.
ISSN:1937-8688
1937-8688
DOI:10.11604/pamj.2019.33.118.16809