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.
Highlights
Dysplastic gangliocytoma or Lhermitte-Duclos disease is a rare disorder characterized by a slowly progressive unilateral tumour mass of the cerebellar cortex
The Lhermitte-Duclos disease, or dysplastic cerebellar gangliocytoma, is an unusual tumour arising from the cerebellar cortex
Regarding the nature of the disease, the original opinion expressed by Lhermitte and Duclos was that the tumour was a combination of a congenital malformation and a neoplasm arising from ganglion cells [5]
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, the exact pathogenesis remains unknown. Cerebral MRI (Figure 1) was performed showing a first right cerebellar convexity lesion which was hyper intense on T2 weighted images containing small zones of necrosis in hyper intense T2 weighted signal and discrete hyper intense T1. This lesion is enhanced heterogeneously and moderately and surrounded by an edematous reaction. The spectroscopy was performed in both of the 2 lesions revealing a choline peak and N-Acety Aspartate (NAA) drop
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