Advanced MR imaging in Lhermitte-Duclos disease: moving closer to pathology and pathophysiology

Thomas, Bejoy; Thamburaj, Krishnamoorthy; Radhakrishnan, V. V.; Kesavadas, Chandrasekharan

doi:10.1007/s00234-007-0241-1

Cited by 55 publications

(49 citation statements)

References 16 publications

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“…In one of our patients, the lesion exhibited diffuse hyperintensity on the ADC maps, indicative of a benign lesion. Proton MR spectroscopy demonstrated reduced NA̸Cho and NA̸Cr ratios compared to the controls, with peaks attributable to lactate (13,17), which was consistent with one of our cases. The low NA̸Cho and NA̸Cr ratios may be attributed to the apparent lack of neuronal architecture (a hallmark of hamartoma) and the .…”

Section: Discussionsupporting

confidence: 88%

“…All the cases in our study shared similar MRI characteristics, whether aLDD was sporadic or associated with CD. The appearance of LDD on MRI is pathognomonic and enables a definitive preoperative diagnosis (11)(12)(13)(14). The hyperintense signal observed on T2-weighted images corresponds to the inner molecular and granular cell layers and the loss of central white matter within the folia (12).…”

Section: Discussionmentioning

confidence: 99%

“…Advanced MRI with novel imaging capabilities may provide insight into the underlying pathophysiology and the nature of LDD (13). Variable diffusion properties of LDD have been reported.…”

Section: Discussionmentioning

confidence: 99%

“…Magnetic resonance imaging (MRI) is considered to be the imaging modality of choice for the diagnosis of LDD and an Aunt Minnie diagnosis of LDD may be confirmed due to the characteristic 'tiger-striped' appearance of LDD on MRI (11)(12)(13)(14). An improved understanding of the underlying molecular pathogenesis and affected signaling pathways of aLDD and its association with CD may have direct implications for optimizing current treatments and developing novel therapeutic approaches.…”

Section: Introductionmentioning

confidence: 99%

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Magnetic resonance characteristics of adult-onset Lhermitte-Duclos disease: An indicator for active cancer surveillance?

Wei

Zhang

et al. 2014

Molecular and Clinical Oncology

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Lhermitte-Duclos disease (LDD) is a rare, non-cancerous entity characterized by enlarged, abnormally developed cerebellar folia containing dysplastic cells. Symptomatic LDD is commonly observed in adults (adult-onset LDD, aLDD) as an isolated condition or associated with Cowden's disease (CD). The present study aimed to investigate the magnetic resonance imaging (MRI) characteristics and the underlying pathological findings in 7 cases of aLDD, with emphasis on the association with CD and the need for active cancer surveillance once the diagnosis of LDD is confirmed. The MRI findings along with the clinical and histopathological data collected from 7 patients with aLDD were retrospectively reviewed. The diagnosis of CD was based on a range of clinical characteristics, according to the International Cowden Consortium Criteria. A thorough review of the published data was conducted and our results indicated that all 7 cases shared similar MRI characteristics, whether the aLDD was sporadic (2 cases) or associated with CD (5 cases), including a highly typical non-enhancing striated MRI appearance of thickened folia, consisting of alternating bands on T1- and T2-weighted images. On gross examination, the involved cerebellar folia were distorted and enlarged, whereas the histopathological examination revealed that the molecular layer was widened and occupied by abnormal ganglion cells. Moreover, a reduction in the number or absence of the Purkinje cells and hypertrophy of the granular cell layer were observed. Our findings were consistent with the diagnosis of LDD. Variable levels of vacuolization of the white matter and the molecular layer were observed in all the cases. Notably, CD34 immunohistochemical analysis revealed the presence of angiogenesis within the lesions. aLDD associated with CD exhibited no pathological or immunohistochemical characteristics that were distinct from those of isolated aLDD. Of the 7 cases of aLDD, 5 presented with symptoms suggestive of CD, which is a syndrome associated with a high risk of multiple benign and malignant neoplasms. In conclusion, aLDD exhibits characteristic MRI and histopathological findings and displays a strong association with CD. Therefore, we recommend that the MRI diagnosis of aLDD triggers active cancer surveillance and preventive care.

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Section: Discussionsupporting

confidence: 88%

Section: Discussionmentioning

confidence: 99%

“…Advanced MRI with novel imaging capabilities may provide insight into the underlying pathophysiology and the nature of LDD (13). Variable diffusion properties of LDD have been reported.…”

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

See 2 more Smart Citations

Magnetic resonance characteristics of adult-onset Lhermitte-Duclos disease: An indicator for active cancer surveillance?

Wei

Zhang

et al. 2014

Molecular and Clinical Oncology

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“…[15,16] On diffusion-weighted imaging, Lhermitte-Duclos disease shows increased signal intensity due to T2 shine-through effect rather than restricted diffusion as reß ected by lack of hypointensity on ADC mapping. [13,17,18] In our case, the findings closely resembled that of Lhermitte-Duclos disease with thickened folia. Mild contrast enhancement has also been reported in Lhermitte-Duclos disease.…”

Section: Discussionsupporting

confidence: 77%

Adult medulloblastoma mimicking Lhermitte-Duclos disease: Can diffusion weighted imaging help?

et al. 2009

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Lhermitte-Duclos disease, also known as dysplastic cerebellar gangliocytoma, is a rare cerebellar benign tumor with characteristic appearance of thickened cerebellar folia giving a laminated or striated appearance, quite diagnostic of the condition. We had seen a patient with medulloblastoma with imaging findings suspicious for thickened cerebellar folia reminiscent of Lhermitte-Duclos disease. However, it showed patchy contrast enhancement and restricted diffusion on diffusion-weighted imaging, which are not typically associated with Lhermitte-Duclos disease. Presence of restricted diffusion proved to be a useful diagnostic feature for differentiation between the two lesions. This patient highlights the importance of obtaining all the MR imaging sequences in suspected Lhermitte-Duclos disease and histological diagnosis if there is doubt.

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Novel Approaches to Imaging Brain Tumors

Mittal

Haacke

2011

Susceptibility Weighted Imaging in MRI

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Advanced MR imaging in Lhermitte-Duclos disease: moving closer to pathology and pathophysiology

Abstract: MRI with the newer imaging capabilities can demonstrate the pathology and pathophysiology in Lhermitte-Duclos disease better. SWI helps in detecting the veins around the thickened folia.

Cited by 55 publications

References 16 publications

Magnetic resonance characteristics of adult-onset Lhermitte-Duclos disease: An indicator for active cancer surveillance?

Magnetic resonance characteristics of adult-onset Lhermitte-Duclos disease: An indicator for active cancer surveillance?

Adult medulloblastoma mimicking Lhermitte-Duclos disease: Can diffusion weighted imaging help?

Novel Approaches to Imaging Brain Tumors

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