Daiki Uchida1, Hirokazu Nakatogawa2, Chikanori Inenaga2, Tokutaro Tanaka2. 1. Department of Neurosurgery, Seirei Hamamatsu General Hospital, Hamamatsu, Japan. Electronic address: d-uchida@sis.seirei.or.jp. 2. Department of Neurosurgery, Seirei Hamamatsu General Hospital, Hamamatsu, Japan.
Abstract
BACKGROUND: Lhermitte-Duclos disease (LDD) is a rare neurologic disease that causes a hamartomatous lesion in the cerebellum. LDD is usually associated with mass lesion effects, but with appropriate surgical treatment, the clinical course is usually benign. We report a rare case of a patient with LDD with contrast enhancement on magnetic resonance imaging (MRI) who died as a result of intratumoral hemorrhage. CASE DESCRIPTION: LDD was diagnosed in a 59-year-old woman after a routine MRI. She did not present with any symptoms initially or after follow-up MRI suggested minor hemorrhage in the tumor. Eleven months after her first visit to our department, she suddenly lost consciousness, and computed tomography revealed massive intratumoral hemorrhage. Surgical decompression was done, but she died 17 days after surgery. Histopathologic findings were consistent with LDD. CONCLUSIONS: Hemorrhagic events can occur with LDD, even though the growth of the lesion is slow. Long-term close follow-up of patients with LDD is needed, especially patients in whom MRI shows atypical enhancement. To avoid possible hemorrhagic events, surgical treatment should be considered even if only subtle changes are seen on neuroimaging.
BACKGROUND:Lhermitte-Duclos disease (LDD) is a rare neurologic disease that causes a hamartomatous lesion in the cerebellum. LDD is usually associated with mass lesion effects, but with appropriate surgical treatment, the clinical course is usually benign. We report a rare case of a patient with LDD with contrast enhancement on magnetic resonance imaging (MRI) who died as a result of intratumoral hemorrhage. CASE DESCRIPTION: LDD was diagnosed in a 59-year-old woman after a routine MRI. She did not present with any symptoms initially or after follow-up MRI suggested minor hemorrhage in the tumor. Eleven months after her first visit to our department, she suddenly lost consciousness, and computed tomography revealed massive intratumoral hemorrhage. Surgical decompression was done, but she died 17 days after surgery. Histopathologic findings were consistent with LDD. CONCLUSIONS: Hemorrhagic events can occur with LDD, even though the growth of the lesion is slow. Long-term close follow-up of patients with LDD is needed, especially patients in whom MRI shows atypical enhancement. To avoid possible hemorrhagic events, surgical treatment should be considered even if only subtle changes are seen on neuroimaging.