Lhermitte-Duclos disease (Dysplastic gangliocytoma of the cerebellum)

Nowak, Dennis A.; Trost, H. A.; Porr, Astrid; Stölzle, A.; Lumenta, Christianto B.

doi:10.1016/s0303-8467(01)00124-x

Cited by 51 publications

(49 citation statements)

References 29 publications

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“…The pathologic changes in gangliocytoma of the cerebellum that cause LDD are the loss of the middle Purkinje cell layer and infiltration of the internal granular cell layer with large neurons with vesicular nuclei and marked nucleoli, which enlarge cerebellar folias (1,5). Findings of light and electron microscopes show that these cells are hypertrophic granular cells (2).…”

Section: Discussionmentioning

confidence: 99%

“…Papilledema, cranial nerve palsies, ataxia, and confusion can be found in the neurologic examination (1,3). Neuroimaging is enough to make the diagnosis (7).…”

Section: Discussionmentioning

confidence: 99%

“…Dysplastic gangliocytoma of the cerebellum, also known as Lhermitte-Duclos disease (LDD), is a rare hamartomatous disorder of the cerebellar cortex (1,2). It typically presents in young adults but can also appear at all ages (3).…”

Section: Introductionmentioning

confidence: 99%

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Lhermitte-Duclos Disease with Orthostatic Hypotension

Yılmaz¹,

Şeker²,

Omaygenç³

et al. 2016

tnd

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130Lhermitte-Duclos disease is a rare cerebellar dysplastic gangliocytoma. The most common symptoms include headache, nausea, vomiting, blurred vision, and imbalance. The typical appearance on cranial magnetic resonance imaging is hyper-intensity on T2-weighted images and hypo-intensity on T1-weighted images. The disease generally presents with benign progress. Development of obstructive hydrocephalus is an indication for urgent surgical intervention and surgery outcomes are satisfactory. Orthostatic hypotension is a very rare clinical presentation of this syndrome and ours is the second case of orthostatic hypotension to be reported in the literature.

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Section: Discussionmentioning

confidence: 99%

“…Papilledema, cranial nerve palsies, ataxia, and confusion can be found in the neurologic examination (1,3). Neuroimaging is enough to make the diagnosis (7).…”

Section: Discussionmentioning

confidence: 99%

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Lhermitte-Duclos Disease with Orthostatic Hypotension

Yılmaz¹,

Şeker²,

Omaygenç³

et al. 2016

tnd

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“…Despite the controversy regarding its pathogenesis, imaging and histopathological findings are rather typical 1,2,3,4 . A 17-year-old female presented with a 2-year history of progressive headaches and gait imbalance.…”

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confidence: 99%

Lhermitte-Duclos disease

Uygur

Andrade

Brum

et al. 2014

Arq. Neuro-Psiquiatr.

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Lhermitte-Duclos disease (dysplastic gangliocytoma of the cerebellum) is a rare benign cerebellar mass of unknown etiology which is characterized by enlargement of the cerebellar folia 1,2 . Despite the controversy regarding its pathogenesis, imaging and histopathological findings are rather typical 1,2,3,4 . A 17-year-old female presented with a 2-year history of progressive headaches and gait imbalance. Cranial CT revealed a cerebellar expanding mass and hydrocephalus. The patient underwent shunt insertion and was referred to our department for treatment. MRI demonstrated a non-enhancing left hemisphere cerebellar tumor (Figure 1) which was subtotally resected during surgical procedure. Figure 2 shows the histological findings. Postoperative course was uneventful.

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“…LDD represents a rare harmatomatous disorder linked to germline loss of one allele of the PTEN gene with subsequent loss of the remaining allele 3,4 . Cranial nerve palsies, gait ataxia and obstructive hydrocephalus secondary to a slowly progressive unilateral cortical cerebellar tumor represents the most common clinical findings 5 . …”

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confidence: 99%

Brain MRI features in Lhermitte-Duclos disease

Pinto

Souza

2014

Arq. Neuro-Psiquiatr.

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A 24-year-old woman presented with long-standing headache, blurred vision, and a 2-week-history of progressive ataxia and vomiting with papilledema and Parinaud syndrome, suggestive of raised intracranial pressure. Neuroimaging features were highly suggestive of dysplastic gangliocytoma of the cerebellum or Lhermitte-Duclos disease (LDD) 1,2 ( Figure), which was confirmed in postsurgical histopathological evaluation. LDD represents a rare harmatomatous disorder linked to germline loss of one allele of the PTEN gene with subsequent loss of the remaining allele 3,4 .

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Lhermitte-Duclos disease (Dysplastic gangliocytoma of the cerebellum)

Cited by 51 publications

References 29 publications

Lhermitte-Duclos Disease with Orthostatic Hypotension

Lhermitte-Duclos Disease with Orthostatic Hypotension

Lhermitte-Duclos disease

Brain MRI features in Lhermitte-Duclos disease

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