Abstract
Lhermitte-Duclos disease (LDD) (dysplastic cerebellar gangliocytoma) is a rare disorder of unknown pathogenesis, presenting with signs and symptoms resulting from obstruction of cerebrospinal fluid flow and mass effect in the posterior fossa. Magnetic resonance imaging is the diagnostic modality of choice allowing preoperative diagnosis with characteristic findings. Surgery is the choice of treatment. The typical histopathological findings of LDD are characterized by widening of the molecular layer, absence of the Purkinje cell layer and hypertrophy in the granule cell layer. Herein we report an adolescent girl with LDD diagnosed preoperatively by the conventional and advanced MR techniques.
MeSH terms
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Adolescent
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Cerebellar Neoplasms / diagnostic imaging
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Cerebellar Neoplasms / pathology*
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Cerebellar Neoplasms / physiopathology
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Cerebellum / diagnostic imaging
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Cerebellum / pathology*
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Cerebellum / physiopathology
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Cranial Fossa, Posterior / pathology*
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Cranial Fossa, Posterior / physiopathology
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Female
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Fourth Ventricle / diagnostic imaging
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Fourth Ventricle / pathology
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Fourth Ventricle / physiopathology
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Hamartoma Syndrome, Multiple / diagnostic imaging
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Hamartoma Syndrome, Multiple / pathology*
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Hamartoma Syndrome, Multiple / physiopathology
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Headache / etiology
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Humans
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Hydrocephalus / diagnostic imaging
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Hydrocephalus / pathology
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Hydrocephalus / physiopathology
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Intracranial Hypertension / etiology
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Intracranial Hypertension / pathology
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Intracranial Hypertension / physiopathology
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Magnetic Resonance Imaging
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Magnetic Resonance Spectroscopy
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Neurosurgical Procedures
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Tomography, X-Ray Computed
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Treatment Outcome