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Diffusion Tensor Magnetic Resonance Imaging in the Analysis of Neurodegenerative Diseases
Published on: July 28, 2013
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Neuroimaging Spectrum of Erdheim-Chester Disease: An Image-Based Review.
Pranjal Rai1, Hayden J Swartz2, Neetu Soni3
1From the Department of Radiology (P.R.), Tata Memorial Hospital, Maharashtra, India.
AJNR. American Journal of Neuroradiology
|November 22, 2024
Summary
Erdheim-Chester disease (ECD) can affect the central nervous system (CNS), leading to diverse neurological symptoms and imaging findings. Recognizing CNS-ECD manifestations is crucial due to its association with poorer prognosis and higher mortality.
Area of Science:
- Neurology
- Radiology
- Histiocytic Disorders
Background:
- Erdheim-Chester disease (ECD) is a rare multisystemic histiocytic disorder.
- Central nervous system (CNS) involvement occurs in about half of ECD patients, often worsening prognosis.
Purpose of the Study:
- To review the diverse clinical and imaging manifestations of CNS involvement in ECD.
- To highlight the importance of recognizing CNS-ECD due to its prognostic implications.
Main Methods:
- Literature review of clinical presentations and neuroimaging findings in CNS-ECD.
- Discussion of differential diagnoses for histiocytic and nonhistiocytic processes.
Main Results:
- CNS-ECD presents with variable neurological symptoms (cognitive decline, motor deficits, neuropathies).
- Neuroimaging reveals diverse findings: volume loss, lesions, meningeal infiltration, hypothalamo-pituitary axis involvement, and perivascular changes.
- Craniofacial, orbital, and spinal involvement are also documented.
Conclusions:
- CNS involvement in ECD is common and associated with increased mortality.
- Awareness of CNS-ECD's varied manifestations and imaging features is vital for timely diagnosis and management.
