Neurodegenerative langerhans cell histiocytosis (ND-LCH)

Zoran Rumboldt, Andrea Rossi

Research output: Chapter in Book/Report/Conference proceedingChapter

Abstract

Specific Imaging Findings. Neurodegenerative Langerhans cell histiocytosis (ND-LCH) develops years after the initial disease presentation and presents on CT as symmetric nonenhancing hypodensities in the dentate nuclei region of the cerebellum, sometimes with calcifications. On MRI, the symmetric cerebellar lesions are T2 hyperintense with hypointense and/or hyperintense signals on T1-weighted images. The abnormalities may be limited to the deep cerebellar gray matter or extend to the surrounding white matter, and can eventually result in CSF-like “holes”. T2 hyperintensities may also be found in the pontine tegmentum and/or pontine pyramidal tracts. T1 hyperintensity of the basal ganglia is another characteristic finding, which may be limited to globus pallidus. All of these lesions do not show mass effect or contrast enhancement, and are the second most frequent presentation of CNS LCH, after the extra-axial involvement of the pituitary gland and hypothalamus. Slight hyperintensity of the dentate nucleus on T1WI may be the initial finding, followed by T2 hyperintensity and subsequent slow extension of T2 hyperintensity to the cerebellar white matter. In the cerebral hemispheres, dilated perivascular spaces and white matter T2 hyperintensities may be encountered. The entire spectrum of intracranial LCH findings can be classified into four groups: (1) osseous lesions in the craniofacial bones and/or skull base with or without soft-tissue extension; (2) intracranial extra-axial disease in the hypothalamic–pituitary region, pineal gland, meninges, choroid plexus, and ependyma; (3) intra-axial degenerative disease in the gray and/or white matter with a striking symmetry of the lesions and a clear predominance in the cerebellum and basal ganglia; (4) localized or diffuse atrophy.

Original languageEnglish
Title of host publicationBrain Imaging with MRI and CT: An Image Pattern Approach
PublisherCambridge University Press
Pages71-72
Number of pages2
ISBN (Print)9781139030854, 9780521119443
DOIs
Publication statusPublished - Jan 1 2010

Fingerprint

Langerhans Cell Histiocytosis
Cerebellar Nuclei
Basal Ganglia
Cerebellum
Ependyma
Meninges
Pyramidal Tracts
Choroid Plexus
Globus Pallidus
Pineal Gland
Skull Base
Cerebrum
Pituitary Gland
Hypothalamus
Atrophy
Bone and Bones
White Matter
Gray Matter

ASJC Scopus subject areas

  • Medicine(all)

Cite this

Rumboldt, Z., & Rossi, A. (2010). Neurodegenerative langerhans cell histiocytosis (ND-LCH). In Brain Imaging with MRI and CT: An Image Pattern Approach (pp. 71-72). Cambridge University Press. https://doi.org/10.1017/CBO9781139030854.036

Neurodegenerative langerhans cell histiocytosis (ND-LCH). / Rumboldt, Zoran; Rossi, Andrea.

Brain Imaging with MRI and CT: An Image Pattern Approach. Cambridge University Press, 2010. p. 71-72.

Research output: Chapter in Book/Report/Conference proceedingChapter

Rumboldt, Z & Rossi, A 2010, Neurodegenerative langerhans cell histiocytosis (ND-LCH). in Brain Imaging with MRI and CT: An Image Pattern Approach. Cambridge University Press, pp. 71-72. https://doi.org/10.1017/CBO9781139030854.036
Rumboldt Z, Rossi A. Neurodegenerative langerhans cell histiocytosis (ND-LCH). In Brain Imaging with MRI and CT: An Image Pattern Approach. Cambridge University Press. 2010. p. 71-72 https://doi.org/10.1017/CBO9781139030854.036
Rumboldt, Zoran ; Rossi, Andrea. / Neurodegenerative langerhans cell histiocytosis (ND-LCH). Brain Imaging with MRI and CT: An Image Pattern Approach. Cambridge University Press, 2010. pp. 71-72
@inbook{0e8ea8ee506146838b89b3e48761858e,
title = "Neurodegenerative langerhans cell histiocytosis (ND-LCH)",
abstract = "Specific Imaging Findings. Neurodegenerative Langerhans cell histiocytosis (ND-LCH) develops years after the initial disease presentation and presents on CT as symmetric nonenhancing hypodensities in the dentate nuclei region of the cerebellum, sometimes with calcifications. On MRI, the symmetric cerebellar lesions are T2 hyperintense with hypointense and/or hyperintense signals on T1-weighted images. The abnormalities may be limited to the deep cerebellar gray matter or extend to the surrounding white matter, and can eventually result in CSF-like “holes”. T2 hyperintensities may also be found in the pontine tegmentum and/or pontine pyramidal tracts. T1 hyperintensity of the basal ganglia is another characteristic finding, which may be limited to globus pallidus. All of these lesions do not show mass effect or contrast enhancement, and are the second most frequent presentation of CNS LCH, after the extra-axial involvement of the pituitary gland and hypothalamus. Slight hyperintensity of the dentate nucleus on T1WI may be the initial finding, followed by T2 hyperintensity and subsequent slow extension of T2 hyperintensity to the cerebellar white matter. In the cerebral hemispheres, dilated perivascular spaces and white matter T2 hyperintensities may be encountered. The entire spectrum of intracranial LCH findings can be classified into four groups: (1) osseous lesions in the craniofacial bones and/or skull base with or without soft-tissue extension; (2) intracranial extra-axial disease in the hypothalamic–pituitary region, pineal gland, meninges, choroid plexus, and ependyma; (3) intra-axial degenerative disease in the gray and/or white matter with a striking symmetry of the lesions and a clear predominance in the cerebellum and basal ganglia; (4) localized or diffuse atrophy.",
author = "Zoran Rumboldt and Andrea Rossi",
year = "2010",
month = "1",
day = "1",
doi = "10.1017/CBO9781139030854.036",
language = "English",
isbn = "9781139030854",
pages = "71--72",
booktitle = "Brain Imaging with MRI and CT: An Image Pattern Approach",
publisher = "Cambridge University Press",

}

TY - CHAP

T1 - Neurodegenerative langerhans cell histiocytosis (ND-LCH)

AU - Rumboldt, Zoran

AU - Rossi, Andrea

PY - 2010/1/1

Y1 - 2010/1/1

N2 - Specific Imaging Findings. Neurodegenerative Langerhans cell histiocytosis (ND-LCH) develops years after the initial disease presentation and presents on CT as symmetric nonenhancing hypodensities in the dentate nuclei region of the cerebellum, sometimes with calcifications. On MRI, the symmetric cerebellar lesions are T2 hyperintense with hypointense and/or hyperintense signals on T1-weighted images. The abnormalities may be limited to the deep cerebellar gray matter or extend to the surrounding white matter, and can eventually result in CSF-like “holes”. T2 hyperintensities may also be found in the pontine tegmentum and/or pontine pyramidal tracts. T1 hyperintensity of the basal ganglia is another characteristic finding, which may be limited to globus pallidus. All of these lesions do not show mass effect or contrast enhancement, and are the second most frequent presentation of CNS LCH, after the extra-axial involvement of the pituitary gland and hypothalamus. Slight hyperintensity of the dentate nucleus on T1WI may be the initial finding, followed by T2 hyperintensity and subsequent slow extension of T2 hyperintensity to the cerebellar white matter. In the cerebral hemispheres, dilated perivascular spaces and white matter T2 hyperintensities may be encountered. The entire spectrum of intracranial LCH findings can be classified into four groups: (1) osseous lesions in the craniofacial bones and/or skull base with or without soft-tissue extension; (2) intracranial extra-axial disease in the hypothalamic–pituitary region, pineal gland, meninges, choroid plexus, and ependyma; (3) intra-axial degenerative disease in the gray and/or white matter with a striking symmetry of the lesions and a clear predominance in the cerebellum and basal ganglia; (4) localized or diffuse atrophy.

AB - Specific Imaging Findings. Neurodegenerative Langerhans cell histiocytosis (ND-LCH) develops years after the initial disease presentation and presents on CT as symmetric nonenhancing hypodensities in the dentate nuclei region of the cerebellum, sometimes with calcifications. On MRI, the symmetric cerebellar lesions are T2 hyperintense with hypointense and/or hyperintense signals on T1-weighted images. The abnormalities may be limited to the deep cerebellar gray matter or extend to the surrounding white matter, and can eventually result in CSF-like “holes”. T2 hyperintensities may also be found in the pontine tegmentum and/or pontine pyramidal tracts. T1 hyperintensity of the basal ganglia is another characteristic finding, which may be limited to globus pallidus. All of these lesions do not show mass effect or contrast enhancement, and are the second most frequent presentation of CNS LCH, after the extra-axial involvement of the pituitary gland and hypothalamus. Slight hyperintensity of the dentate nucleus on T1WI may be the initial finding, followed by T2 hyperintensity and subsequent slow extension of T2 hyperintensity to the cerebellar white matter. In the cerebral hemispheres, dilated perivascular spaces and white matter T2 hyperintensities may be encountered. The entire spectrum of intracranial LCH findings can be classified into four groups: (1) osseous lesions in the craniofacial bones and/or skull base with or without soft-tissue extension; (2) intracranial extra-axial disease in the hypothalamic–pituitary region, pineal gland, meninges, choroid plexus, and ependyma; (3) intra-axial degenerative disease in the gray and/or white matter with a striking symmetry of the lesions and a clear predominance in the cerebellum and basal ganglia; (4) localized or diffuse atrophy.

UR - http://www.scopus.com/inward/record.url?scp=84924678312&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=84924678312&partnerID=8YFLogxK

U2 - 10.1017/CBO9781139030854.036

DO - 10.1017/CBO9781139030854.036

M3 - Chapter

AN - SCOPUS:84924678312

SN - 9781139030854

SN - 9780521119443

SP - 71

EP - 72

BT - Brain Imaging with MRI and CT: An Image Pattern Approach

PB - Cambridge University Press

ER -