LHERMITTE-DUCLOS DISEASE: A RARE CEREBELLAR HAMARTOMA PRESENTING FOLLOWING TRAUMATIC BRAIN INJURY AND A REVIEW OF THE LITERATURE

Authors

  • Mohammad Ashraf Department of Neurosurgery, Allama Iqbal Medical College, Jinnah Hospital Lahore, Pakistan
  • Usman Ahmad Kamboh Department of Neurosurgery, Allama Iqbal Medical College, Jinnah Hospital Lahore, Pakistan
  • Muhammad Asif Raza Department of Neurosurgery, Allama Iqbal Medical College, Jinnah Hospital Lahore, Pakistan
  • Nabeel Choudhary Department of Neurosurgery, Allama Iqbal Medical College, Jinnah Hospital Lahore, Pakistan
  • Mehreen Mehboob Department of Neurosurgery, Allama Iqbal Medical College, Jinnah Hospital Lahore, Pakistan
  • Syed Shahzad Hussain Department of Neurosurgery, Allama Iqbal Medical College, Jinnah Hospital Lahore, Pakistan
  • Naveed Ashraf Department of Neurosurgery, Allama Iqbal Medical College, Jinnah Hospital Lahore, Pakistan

DOI:

https://doi.org/10.55519/JAMC-03-S1-9900

Keywords:

Lhermitte-Duclos Disease, Dysplastic Gangliocytoma, Cowden's Disease

Abstract

Lhermitte-Duclos Disease (LDD) is an extremely rare hamartoma of the cerebellum and is associated with the cancer syndrome Cowden’s disease. We report such a patient whose disease was diagnosed incidental to traumatic brain injury. A 40-year-old male presented after fall from stairs. CT scan revealed a large lesion in the right cerebellar hemisphere. Clinical history recounted multiple short episodes of vomiting (>10 a week) for the past 30 years and development of posterior fossa symptoms over the recent months. Neither of these had him referred due to lack of access to primary healthcare. T1 MRI with contrast showed an isointense focal mass, enhancement along the folia, and distortion of the 4th ventricle. On T2 MRI, tiger striped appearance was noted. Endoscopic third ventriculostomy was performed followed by gross total resection of the hamartoma. Histology confirmed LDD. All reported symptoms resolved following surgery. Due to lack of access to the expensive genetic testing for Cowden’s he is in regular biannual follow up to be evaluated clinically for associated malignancies. We present this case to highlight the clinical-pathological characteristics of LDD, its treatment, and discuss management in the absence of genetic testing in our socio-economic demographic.

Author Biographies

Mohammad Ashraf, Department of Neurosurgery, Allama Iqbal Medical College, Jinnah Hospital Lahore, Pakistan

Neurosurgery Clinical Research Fellow 

Usman Ahmad Kamboh, Department of Neurosurgery, Allama Iqbal Medical College, Jinnah Hospital Lahore, Pakistan

Assistant Professor of Neurosurgery

Muhammad Asif Raza, Department of Neurosurgery, Allama Iqbal Medical College, Jinnah Hospital Lahore, Pakistan

Assistant Professor of Neurosurgery

Nabeel Choudhary, Department of Neurosurgery, Allama Iqbal Medical College, Jinnah Hospital Lahore, Pakistan

Assitant Professor of Neurosurgery

Mehreen Mehboob, Department of Neurosurgery, Allama Iqbal Medical College, Jinnah Hospital Lahore, Pakistan

Senior Resident Neurosurgeon

Syed Shahzad Hussain, Department of Neurosurgery, Allama Iqbal Medical College, Jinnah Hospital Lahore, Pakistan

Associate Professor of Neurosurgery (at the time of writing)

Naveed Ashraf, Department of Neurosurgery, Allama Iqbal Medical College, Jinnah Hospital Lahore, Pakistan

Professor and Head of Department

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Published

2022-06-24

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