An unusual cause of posterior fossa mass: Lhermitte-Duclos disease

Yağcı-Küpeli, Begül; Oğuz, Kader K.; Bilen, M; Yalçın, Bilgehan; Akalan, Nejat; Büyükpamukçu, Münevver

doi:10.1016/j.jns.2009.12.010

Cited by 9 publications

(9 citation statements)

References 18 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…[ 2 3 10 12 ] Acute-onset of cerebellar symptoms, characterized by a rapid neurological deficit is rarely reported. [ 4 5 ] Our patient showed this acute-onset symptom.…”

Section: Discussionmentioning

confidence: 57%

“…[ 6 7 15 ] Establishing preoperative diagnosis of LDD with MRI obviates the need for biopsy and this allows neurosurgeons to plan for appropriate treatment. [ 4 5 7 ] The CT scan appearance of LDD, consists of hypo-dense and calcified areas is nonspecific for this disease. [ 2 3 9 ] In our case, preoperative diagnosis was uncertain because MRI was not available yet at our hospital.…”

Section: Discussionmentioning

confidence: 99%

“…Our literature review suggests that surgical resection is the treatment of choice for this particular disease. [ 1 2 3 5 6 7 ] Many reports have confirmed that decompressive craniotomy for LDD has been successful not only in relieving symptoms[ 9 ] but also improving long-term survival. [ 3 10 ]…”

Section: Discussionmentioning

confidence: 99%

“…The unique anatomical features of LDD, which include preservation and widening of the gyral pattern can be diagnosed preoperatively with MRI. [ 1 2 3 4 5 6 7 8 ] Other imaging studies such as CT scan are not considered sufficiently sensitive to make the diagnosis. [ 2 3 9 ] However, there are reports of successful surgical resection of this particular disease even the preoperative diagnosis is uncertain.…”

Section: Introductionmentioning

confidence: 99%

See 3 more Smart Citations

Management of unusual dysplastic gangliocytoma of the cerebellum (Lhermitte-Duclos disease) in a developing country: Case report and review of the literature

et al. 2016

View full text Add to dashboard Cite

Dysplastic cerebellar gangliocytoma or Lhermitte-Duclos disease (LDD) especially in children are extremely rare. In this report we add one further case to this rare entity. A three year old boy with a history of cerebellar and brain stem compression signs was presented in unconscious condition. Computerized tomography (CT) scan revealed a well defined lesion mixed with area of calcification in the right cerebellum and severe obstructive hydrocephalus. Ventricular shunting was performed followed by gross total tumor removal three days after shunting. In the follow up period, the patient showed almost total resolution of all neurological deficits. MRI has been an imaging tool to preoperatively diagnose this disease; otherwise it would be uncertain preoperative diagnosis. However, in this report we would like to emphasize that not all uncertain diagnosis of LDD leads to palliative treatment. Well demarcated lesion may account for the safe surgical resection of this disease.

show abstract

“…[ 2 3 10 12 ] Acute-onset of cerebellar symptoms, characterized by a rapid neurological deficit is rarely reported. [ 4 5 ] Our patient showed this acute-onset symptom.…”

Section: Discussionmentioning

confidence: 57%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

See 2 more Smart Citations

Management of unusual dysplastic gangliocytoma of the cerebellum (Lhermitte-Duclos disease) in a developing country: Case report and review of the literature

et al. 2016

View full text Add to dashboard Cite

show abstract

“…Subsequently, Lhermitte-Duclos disease has become a surgically treatable and even curable condition. One of the major challenges for the surgeon during surgical exploration of Lhermitte-Duclos cerebellar tumors is the lack of clear margins with the normal cerebellar tissue which are often not well defined [3][4][5].…”

Section: Introductionmentioning

confidence: 99%

Acute Hydrocephalus Revealing Unusual Cerebellar Mass: Dysplastic Cerebellar Gangliocytoma or Lhermitte-Duclos Disease (LDD)

Karekezi¹,

Boutarbouch²,

Coulibaly³

et al. 2013

View full text Add to dashboard Cite

Lhermitte-Duclos disease (LDD) or dysplastic gangliocytoma of the cerebellum is a rare benign lesion of uncertain pathogenesis characterized by overgrowth of cerebellar ganglion cells which replace granular cells and Purkinje cells; this results in gross thickening of the cerebellar folia. It is revealed by symptoms of raised intracranial pressure, cerebellar impairment and obstructive hydrocephalus. We reported the case of a 41-year-old male who presented with symptoms of acute raised intracranial pressure. Brain computed tomography (CT) scan revealed hydrocephalus due to compression of the 4th ventricle by a large non-enhancing left cerebellar mass. Magnetic resonance imaging (MRI) showed a space-occupying lesion within the left cerebellar hemisphere with unusual striation. Radical surgery was retained though the margins with normal cerebellar tissue were not distinct. Clinical complications after gross total or partial removal of Lhermitte-Duclos lesions have been rarely reported in the literature; herein we stress the importance of extreme caution in removing these lesions in cerebellar areas that have no distinct margins between the lesion and normal tissue.

show abstract

Acute-Onset Cerebellar Symptoms in Lhermitte–Duclos Disease: Case Report

et al. 2012

View full text Add to dashboard Cite

Adult-onset Lhermitte-Duclos disease (LD), or dysplastic cerebellar gangliocytoma, is a hamartoma considered pathognomonic for Cowden disease. Classically, LD has a progressive and insidious onset of symptoms. In this case report, we present a patient having rapid neurological deterioration from acute-onset LD. There are only three reported cases of acute LD presentation. A 22-year-old female presented to the emergency department with diplopia, dysarthria, dysphagia, and gait instability which developed within 6 h. A non-contrast CT scan revealed diffuse attenuation in the left cerebellum and mild ventricular dilatation. LP revealed no organisms. Magnetic resonance imaging revealed salient "tiger stripe" appearance of the left cerebellar cortex and effacement of the fourth ventricle. The patient subsequently underwent suboccipital craniotomy and gross total resection of the lesion. The tumor histology showed distortion of normal cerebellar architecture with dysplastic ganglion cells, loss of Purkinje cells, atrophy of the white matter, and expansion of cerebellar folia. Findings were consistent with adult-onset Lhermitte-Duclos disease.

show abstract

An unusual cause of posterior fossa mass: Lhermitte-Duclos disease

Cited by 9 publications

References 18 publications

Management of unusual dysplastic gangliocytoma of the cerebellum (Lhermitte-Duclos disease) in a developing country: Case report and review of the literature

Management of unusual dysplastic gangliocytoma of the cerebellum (Lhermitte-Duclos disease) in a developing country: Case report and review of the literature

Acute Hydrocephalus Revealing Unusual Cerebellar Mass: Dysplastic Cerebellar Gangliocytoma or Lhermitte-Duclos Disease (LDD)

Acute-Onset Cerebellar Symptoms in Lhermitte–Duclos Disease: Case Report

Contact Info

Product

Resources

About