MR imaging features of Lhermitte–Duclos disease

Zhang, Hanwen; Zhang, Yuanqing; Mo, Yong-Qian; Yi, Lei; Lin, Fan; Yang, Feng

doi:10.1097/md.0000000000028667

Cited by 12 publications

(22 citation statements)

References 29 publications

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“…e appearance of the Lac peak, low Cho/NAA ratio, and comprehensive presence of the conventional MRI tiger striped sign can improve the diagnostic accuracy of LDD. [11] e MRI features often correlate with the histopathological features of LDD. e central core of T1 hypointensity and T2 hyperintensity corresponds to the thinned white matter, widened granular cell layer, and the inner portions of the dysplastic molecular layer.…”

Section: Discussionmentioning

confidence: 99%

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Lhermitte-Duclos disease: A series of six cases

Kolhe

Shenoy

Tayal

et al. 2023

JNRP

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The Lhermitte-Duclos disease (LDD), also known as dysplastic cerebellar gangliocytoma, is a rare lesion characterized by variable enlargement of cerebellar folia. The pathological basis of LDD has long been debated, as it has overlapping features of both, a neoplasm and hamartoma. Association between LDD and Cowden syndrome (CS) has been established based on the presence of phosphatase and tensin homologue germline mutation in both. We present a series of six cases of LDD: Four females and two males, aged between 16 and 38 years, presenting with headache and imbalance on walking of 1–7 months duration. Histomorphology showed thickening and vacuolation of the molecular layer, loss of Purkinje cells, and replacement of granular cell layer by large dysplastic ganglion cells. Awareness of histological features of this rare entity and a higher level of suspicion is required for the correct diagnosis, which, in turn, should prompt thorough investigations to exclude features of associated CS. LDD is a rare entity, awareness of its histological features and correlating them with radiology is essential, especially in tiny biopsies; to render the correct diagnosis. Diagnosis of LDD warrants further clinical workup and close follow-up for the associated features of CS.

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Section: Discussionmentioning

confidence: 99%

“…The appearance of the Lac peak, low Cho/NAA ratio, and comprehensive presence of the conventional MRI tiger striped sign can improve the diagnostic accuracy of LDD. [ 11 ]…”

Section: Discussionmentioning

confidence: 99%

Lhermitte-Duclos disease: A series of six cases

Kolhe

Shenoy

Tayal

et al. 2023

JNRP

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“…Imaging is commonly a diagnostic tool when assessing the disease onset of LDD, as MRI is primarily used to detect the dysplastic gangliocytoma [ 8 ]. Cerebral MRIs have been used to capture images of the cerebellar lesions, which can rarely enhance heterogeneously and are surrounded by a noticeable edematous reaction [ 5 ].…”

Section: Discussionmentioning

confidence: 99%

Subacute Posterior Inferior Cerebellar Artery Stroke Radiographically Mimicking Lhermitte-Duclos Disease

Mehkri¹,

Poe²,

Nasrullah³

et al. 2022

Cureus

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Lhermitte-Duclos disease (LDD) is a rare cerebellar lesion characterized by a hamartomatous lesion of the cerebellum. Mainly diagnosed by MRI, the clinical presentation is usually made of neurological symptoms. Modern neuroimaging techniques such as MRI have led to accurate diagnosis of this disease in both its preand post-operative periods. We present the case of a 68-year-old male with a past medical history of cardiac stenting and coronary artery disease who originally presented to the emergency department as a transfer for evaluation of possible obstructing hydrocephalus and left posterior inferior cerebellar artery (PICA) infarct. Based on the clinical presentation and imaging, the favored diagnosis of his left cerebellar abnormality was LDD rather than an unusual acute/subacute infarct or a metastatic lesion. The rapid progression of symptoms with rapidly progressive cytotoxic edema on serial CTs helped exclude LDD, which is nearly always more of a chronic process. The classic neuroimaging findings and clinical presentation of LDD are also discussed.

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“…However, all currently available evidence shows that MRI, including magnetic resonance spectroscopy, could safely set the diagnosis. 12 Unfortunately, there is no diagnostic accuracy data on the performance of MRI in LDD, since the available evidence originate from a limited number of case reports, due to the rarity of the clinical entity under study. On top of that, an expectant approach with a regular patient follow-up seems to be a more legally defensible strategy than to proceed with an aggressive posterior fossa surgery, considering the potential perioperative risk the patient's preference.…”

Section: Discussionmentioning

confidence: 99%

Posterior Fossa Intra-Axial Lesion: There Are Pathologies Beyond Metastases

et al. 2022

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Cerebellar metastases represent the most common lesion requiring surgical treatment in the daily neurosurgical praxis. We report a rare case with Lhermitte–Duclos disease presenting to the neurosurgical outpatient department and describe our diagnostic and treatment challenges. A 57-year-old man presented with transient cerebellar manifestations, including walking difficulties, dizziness, and a persistent occipital headache. The magnetic resonance imaging demonstrated a high-intensity lesion in the right cerebellar hemisphere on T2-weighted images. We initially considered surgery to establish a histopathological diagnosis. However, the magnetic resonance spectroscopy was compatible with a dysplastic gangliocytoma of the cerebellum. Therefore, we preferred a more conservative approach. Posterior fossa contrast-enhancing lesions are much more than metastases. The role of advanced magnetic resonance in avoiding unnecessary surgical interventions is priceless.

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MR imaging features of Lhermitte–Duclos disease

Cited by 12 publications

References 29 publications

Lhermitte-Duclos disease: A series of six cases

Lhermitte-Duclos disease: A series of six cases

Subacute Posterior Inferior Cerebellar Artery Stroke Radiographically Mimicking Lhermitte-Duclos Disease

Posterior Fossa Intra-Axial Lesion: There Are Pathologies Beyond Metastases

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