Year : 1999 | Volume
: 9 | Issue : 1 | Page : 25--27
K Surekha, AK Gupta, Santhosh Joseph, C Kesavadas, NKK Prabhu
Department of Radiology, Sree Chitra Tirunal Institute for Medical Sciences & Technology, Thiruvananthapuram-695001, India
A K Gupta
Department of Radiology, Sree Chitra Tirunal Institute for Medical Sciences & Technology, Thiruvananthapuram-695001
|How to cite this article:|
Surekha K, Gupta A K, Joseph S, Kesavadas C, Prabhu N. Radiological quiz-neuroradiology.Indian J Radiol Imaging 1999;9:25-27
|How to cite this URL:|
Surekha K, Gupta A K, Joseph S, Kesavadas C, Prabhu N. Radiological quiz-neuroradiology. Indian J Radiol Imaging [serial online] 1999 [cited 2020 Jan 19 ];9:25-27
Available from: http://www.ijri.org/text.asp?1999/9/1/25/28368
A fifty-seven-years old man was well until one year prior to admission to the hospital. He developed recurrent attacks of headache and vomiting with increased frequency of symptoms about one month prior to admission. He also complained of swaying to either side. Examination revealed bilateral papilledema. In addition he also had multiple subcutaneous lipomas and papules over the body.
CT and MR were performed [Figure 1],[Figure 2].
Lhermitte-Duclos Disease (LDD)
CT shows an ill-defined hypodense lesion of the right cerebellum with significant mass effect [Figure 1]. MR reveals a hypointense mass on the T1W images [Figure 2]a and a laminated, striated or folial pattern of increased signal on the T2W images [Figure 2]b.
The patient underwent decompression and partial resection of the lesion. On surgery, the cerebellum showed thickened folia. Pathological features demonstrated were suggestive of Lhermitte-Duclos disease (LDD). Dermatological consultation with biopsy of skin lesions was consistent with Cowden's disease.
In 1920, Lhermitte and Duclos  described a cerebellar abnormality consisting of focally enlarged hemispheric folia containing abnormal ganglion cells in the granular layer, thickening and hypermyelination of the molecular layer and atrophy of cerebellar white matter with loss of Purkinje cells. Microcalcification within the folia and microvascular proliferation in the leptomeninges and outer cortex were also described.
It is generally considered to be a developmental disorder resulting from abnormal neuronal growth , rather than a true neoplasm. As the pathogenesis is not clear, a number of names have been used to describe this lesion. These include dysplastic gangliocytoma of cerebellum, purkinjeoma, ganglioneuroma, hamartoma of the cerebellum, etc. Associated abnormalities have also been described, including megalencephaly, heterotopia, microgyria, hydromyelia, polydactyly, perithelioma, partial gigantism, macroglossia and leontiasis ossea. Co-existing conditions in LDD include Cowden's disease, which is also called multiple hamartoma syndrome. It is a rare hereditary condition characterized by multiple hamartomas and neoplasms of ectodermal, mesodermal and endodermal origin. Other reported associations include gastro-intestinal polyps, breast disease, thyroid abnormalities, ovarian cysts, hepatic lesions, neuromas and neurofibromas.
Typically, LDD presents in young adults with features of raised intracranial pressure. Cerebellar signs are much less prominent. Neuroimaging with CT and MRI demonstrates abnormal laminated patterns of cortical architecture. Although alternating isodense and hypodense layers are discernible by CT, the architecture of the lesion is more clearly evident on MR .
The presence of parallel linear striations on the surface of the lesion presumably representing thickened dysplastic cerebellar folia provides a diagnostic image on MR ,,, which is unlikely to be confused with any other pathological process in the cerebellum. This capability allows for management options independent of a biopsy. Accurate MR diagnosis of incidental LDD, in patients, evaluated for unrelated reasons obviates the need for craniotomy for tissue diagnosis. In all patients with LDD, long-term follow up is advisable because of occasional symptomatic recurrence .
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