Biological Characteristics of a Cerebellar Mass Regrowing after Removal in a Patient with Lhermitte-Duclos Disease: Emission Tomography Studies

Lhermitte-Duclos disease is a rare disorder characterized by a slowly enlarging mass in the cerebellum. The exact nature of this lesion remains unclear, but it has been variously characterized as hypertrophy, hamartoma or benign neoplasm. Single-photon emission computed tomography (SPECT) with 123I-...

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Bibliographic Details
Main Authors: Hideo Saito, Kuniaki Ogasawara, Takaaki Beppu, Hideki Matsuura, Kazunori Terasaki
Format: Article
Language:English
Published: Karger Publishers 2014-03-01
Series:Case Reports in Neurology
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Online Access:http://www.karger.com/Article/FullText/362118
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Summary:Lhermitte-Duclos disease is a rare disorder characterized by a slowly enlarging mass in the cerebellum. The exact nature of this lesion remains unclear, but it has been variously characterized as hypertrophy, hamartoma or benign neoplasm. Single-photon emission computed tomography (SPECT) with 123I-iomazenil and 99mTc-ethyl cysteinate dimer (ECD) display central benzodiazepine receptors that are widely distributed in the normal brain cortex, a combination of brain perfusion, enzymatic activity and blood-brain barrier function, respectively. This report describes the case of a 30-year-old man who developed Lhermitte-Duclos disease. The mass regrew after a subtotal removal 13 years earlier. The cerebellar lesion exhibited hyperperfusion and hypermetabolism on blood flow and a metabolic rate of oxygen imaging on 15O-gas positron emission tomography (PET), as well as a hyperactivity and a defect in the uptake on 99mTc-ECD and 123I-iomazenil SPECT imaging, respectively. The present Lhermitte-Duclos lesion might have the biological characteristics of both hypertrophy and neoplasm, from the perspective of results from 15O-gas PET, 99mTc-ECD and 123I-iomazenil SPECT.
ISSN:1662-680X