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

Overview of attention for article published in The Cerebellum, June 2012
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Title
Acute-Onset Cerebellar Symptoms in Lhermitte–Duclos Disease: Case Report
Published in
The Cerebellum, June 2012
DOI 10.1007/s12311-012-0394-2
Pubmed ID
Authors

Omid R. Hariri, Arsineh Khachekian, Dan Muilli, Jenny Amin, Tanya Minassian, Blake Berman, Yoav Ritter, Javed Siddiqi

Abstract

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.

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Geographical breakdown

Country Count As %
Korea, Republic of 2 10%
Unknown 18 90%

Demographic breakdown

Readers by professional status Count As %
Researcher 2 10%
Librarian 1 5%
Student > Doctoral Student 1 5%
Lecturer > Senior Lecturer 1 5%
Professor 1 5%
Other 3 15%
Unknown 11 55%
Readers by discipline Count As %
Medicine and Dentistry 6 30%
Neuroscience 2 10%
Agricultural and Biological Sciences 1 5%
Social Sciences 1 5%
Biochemistry, Genetics and Molecular Biology 1 5%
Other 0 0%
Unknown 9 45%
Attention Score in Context

Attention Score in Context

This research output has an Altmetric Attention Score of 1. This is our high-level measure of the quality and quantity of online attention that it has received. This Attention Score, as well as the ranking and number of research outputs shown below, was calculated when the research output was last mentioned on 15 June 2012.
All research outputs
#19,495,804
of 23,975,976 outputs
Outputs from The Cerebellum
#659
of 957 outputs
Outputs of similar age
#131,830
of 169,758 outputs
Outputs of similar age from The Cerebellum
#9
of 15 outputs
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