Cerebellar Disease Mimicking Cerebrotendinous Xanthomatosis: Langerhans Cell Histiocytosis

Bianca M.L. Stelten, Marjo S. van der Knaap, Ron A. Wevers, Aad Verrips

Research output: Contribution to journalArticleAcademicpeer-review

Abstract

Background This report highlights the differential diagnosis of predominant cerebellar white matter abnormalities with dentate nuclei involvement. Patient description We describe two individuals with Langerhans cell histiocytosis in whom the diagnosis of cerebrotendinous xanthomatosis was initially considered. The clinical picture consisted of a progressive cerebellar syndrome with typical magnetic resonance imaging abnormalities. In both individuals, the cerebellar syndrome preceded the diagnosis of Langerhans cell histiocytosis. Conclusions The magnetic resonance imaging abnormalities and neurological features in patients with Langerhans cell histiocytosis can be strikingly similar to those with cerebrotendinous xanthomatosis. In cerebrotendinous xanthomatosis, the cerebellar symptoms and cerebellar white matter abnormalities are usually seen in adult patients. In a pediatric patient with a cerebellar syndrome, showing these cerebellar white matter abnormalities a diagnosis of Langerhans cell histiocytosis is more likely.

Original languageEnglish
Pages (from-to)98-100
Number of pages3
JournalPediatric Neurology
Volume73
DOIs
Publication statusPublished - 1 Aug 2017

Cite this

Stelten, Bianca M.L. ; van der Knaap, Marjo S. ; Wevers, Ron A. ; Verrips, Aad. / Cerebellar Disease Mimicking Cerebrotendinous Xanthomatosis : Langerhans Cell Histiocytosis. In: Pediatric Neurology. 2017 ; Vol. 73. pp. 98-100.
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title = "Cerebellar Disease Mimicking Cerebrotendinous Xanthomatosis: Langerhans Cell Histiocytosis",
abstract = "Background This report highlights the differential diagnosis of predominant cerebellar white matter abnormalities with dentate nuclei involvement. Patient description We describe two individuals with Langerhans cell histiocytosis in whom the diagnosis of cerebrotendinous xanthomatosis was initially considered. The clinical picture consisted of a progressive cerebellar syndrome with typical magnetic resonance imaging abnormalities. In both individuals, the cerebellar syndrome preceded the diagnosis of Langerhans cell histiocytosis. Conclusions The magnetic resonance imaging abnormalities and neurological features in patients with Langerhans cell histiocytosis can be strikingly similar to those with cerebrotendinous xanthomatosis. In cerebrotendinous xanthomatosis, the cerebellar symptoms and cerebellar white matter abnormalities are usually seen in adult patients. In a pediatric patient with a cerebellar syndrome, showing these cerebellar white matter abnormalities a diagnosis of Langerhans cell histiocytosis is more likely.",
keywords = "cerebellar white matter abnormalities, cerebrotendinous xanthomatosis (CTX), Langerhans cell histiocytosis (LCH), nucleus dentatus",
author = "Stelten, {Bianca M.L.} and {van der Knaap}, {Marjo S.} and Wevers, {Ron A.} and Aad Verrips",
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Cerebellar Disease Mimicking Cerebrotendinous Xanthomatosis : Langerhans Cell Histiocytosis. / Stelten, Bianca M.L.; van der Knaap, Marjo S.; Wevers, Ron A.; Verrips, Aad.

In: Pediatric Neurology, Vol. 73, 01.08.2017, p. 98-100.

Research output: Contribution to journalArticleAcademicpeer-review

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T1 - Cerebellar Disease Mimicking Cerebrotendinous Xanthomatosis

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AU - Stelten, Bianca M.L.

AU - van der Knaap, Marjo S.

AU - Wevers, Ron A.

AU - Verrips, Aad

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AB - Background This report highlights the differential diagnosis of predominant cerebellar white matter abnormalities with dentate nuclei involvement. Patient description We describe two individuals with Langerhans cell histiocytosis in whom the diagnosis of cerebrotendinous xanthomatosis was initially considered. The clinical picture consisted of a progressive cerebellar syndrome with typical magnetic resonance imaging abnormalities. In both individuals, the cerebellar syndrome preceded the diagnosis of Langerhans cell histiocytosis. Conclusions The magnetic resonance imaging abnormalities and neurological features in patients with Langerhans cell histiocytosis can be strikingly similar to those with cerebrotendinous xanthomatosis. In cerebrotendinous xanthomatosis, the cerebellar symptoms and cerebellar white matter abnormalities are usually seen in adult patients. In a pediatric patient with a cerebellar syndrome, showing these cerebellar white matter abnormalities a diagnosis of Langerhans cell histiocytosis is more likely.

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