Lhermitte-Duclos disease

Uygur, Saygı; Andrade, Marina Coelho de; Brum, Carolina de Almeida Ito; Monerat, Andrea Lima Cruz; Landeiro, José Alberto; Acioly, Marcus André

doi:10.1590/0004-282x20140026

Cited by 3 publications

(4 citation statements)

References 3 publications

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“…Histological hallmarks of the disease are thickening and hypermyelination of the outer molecular layer, loss of Purkinje cells and white matter, dysplastic ganglion cells with rounded nuclei, and abundant mitochondria invading the inner granular layer. 2 5…”

Section: Discussionmentioning

confidence: 99%

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A Radiological Curiosity of a Rare Diagnosis: Lhermitte-Duclos Disease

Wilson

Koudouhonon

Igombe

et al. 2022

ASJO

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Lhermitte–Duclos disease (LDD) is a rare cerebellar lesion, described in 1920 by two French physicians: Lhermitte and Duclos. The clinical presentation is usually made of neurological symptoms. This lesion is characterized by a hamartomatous lesion in the posterior fossa. Mainly diagnosed by MRI, when it comes to preoperative, the T2-weightened MRI demonstrates the classical “tiger-striped” pattern. The definitive diagnosis, nonetheless, is histopathological. The treatment for LDD consists of surgical decompression or excision. We present here a rare case of a woman who developed neurological symptoms that led to LDD diagnosis to describe protocol MRI imaging, the main findings and their pathophysiological meanings.

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Section: Discussionmentioning

confidence: 99%

“…1 It is an extremely rare benign tumor of specific cerebellar localization, with poorly understood etiopathogeny. 2 It was first described in 1920 by two French people, Lhermitte and Duclos. 3 There is a recognition of a higher female preponderance.…”

Section: Introductionmentioning

confidence: 99%

A Radiological Curiosity of a Rare Diagnosis: Lhermitte-Duclos Disease

Wilson

Koudouhonon

Igombe

et al. 2022

ASJO

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“…While some patients are asymptomatic, LDD most commonly presents with chronic and progressive symptoms, with symptoms typically developing after a few months to more than 10 years prior to diagnosis [ 1 , 5 ]. LDD most commonly presents with cerebellar ataxia, dysarthria, cranial nerve dysfunction, and psychiatric symptoms [ 6 ]. In more severe cases, obstructive hydrocephalus and intracranial hypertension may develop, leading to macrocephaly and bilateral papilledema [ 2 , 3 , 7 ].…”

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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“…Of these, 5 reports are Brazilian. [9][10][11][12][13][14] Therefore, this article is the 6th report of LDD and the first to report the association with CS in Brazil.…”

Section: Discussionmentioning

confidence: 99%

Lhermitte-Duclos Disease and Cowden Syndrome: A Case Report and Literature Review

et al. 2019

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Lhermitte-Duclos disease (LDD), also known as dysplastic gangliocytoma of the cerebellum, is a rare, usually benign, slow-growing tumor, that commonly affects patients aged 30 to 50 years-old. The manifestations of dysplastic cerebellar gangliocytoma are nonspecific and are related both to the mass effect produced by its growth and to the location of the lesion. Cerebellar symptoms such as ataxia are often present. In 40% of cases, the tumor is associated with Cowden syndrome, which is part of a group of genetic disorders called polypoid hamartoma complex. In this case report, the patient presented expansive lesion in the posterior fossa, compatible with LDD, associated with macrocephaly. These findings are considered major criteria for Cowden syndrome. When together, they confirm the diagnoses. To our knowledge, this is the first report of the association of LDD and Cowden syndrome in Brazil.

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Lhermitte-Duclos disease

Cited by 3 publications

References 3 publications

A Radiological Curiosity of a Rare Diagnosis: Lhermitte-Duclos Disease

A Radiological Curiosity of a Rare Diagnosis: Lhermitte-Duclos Disease

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

Lhermitte-Duclos Disease and Cowden Syndrome: A Case Report and Literature Review

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