CHARACTERISTIC BRAIN MRI APPEARANCE OF ERDHEIM-CHESTER DISEASE

Neurology ◽  
2009 ◽  
Vol 73 (24) ◽  
pp. 2120-2122 ◽  
Author(s):  
F. Bianco ◽  
E. Iacovelli ◽  
E. Tinelli ◽  
N. Locuratolo ◽  
F. Pauri ◽  
...  
Keyword(s):  
Brain Mri ◽  
Erdheim Chester Disease ◽  
Erdheim Chester ◽  
Chester Disease

scholarly journals Neuroradiologic manifestations of Erdheim-Chester disease

2018 ◽  
Vol 8 (1) ◽  
pp. 15-20 ◽  
Author(s):  
Natalie E. Parks ◽  
Gaurav Goyal ◽  
Ronald S. Go ◽  
Jay Mandrekar ◽  
W. Oliver Tobin
Keyword(s):  
Spinal Cord ◽  
Diabetes Insipidus ◽  
Brain Mri ◽  
Urine Osmolality ◽  
Erdheim Chester Disease ◽  
Hypothalamic Pituitary Axis ◽  
Spine Mri ◽  
Erdheim Chester ◽  
Chester Disease

BackgroundWe describe the neuroradiologic features of a cohort of patients with Erdheim-Chester disease.MethodsWe assessed patients at Mayo Clinic Rochester between January 1, 1990, and July 31, 2016, with pathologically confirmed Erdheim-Chester disease (n = 53).ResultsNeuroimaging, including head CT (n = 17), brain MRI (n = 39), orbital MRI (n = 15), and spine MRI (n = 16), was available for 42 participants. Median age at diagnosis was 55 years (interquartile range 46–66) with higher male prevalence (33:20). Neurologic symptoms were identified in 47% (25/53); BRAFV600E mutation in 58% (15/26). Median follow-up was 2 years (range 0–20) with 18 patients deceased. Radiologic disease evidence was seen in dura (6/41), brainstem (9/39), cerebellum (8/39), spinal cord (2/16), spinal epidura (2/16), hypothalamic-pituitary axis (17/39), and orbits (13/42). T2 white matter abnormalities (Fazekas score ≥1) were present in 21/34 patients. Diabetes insipidus was present in 30% (16/53); 8 had abnormal hypothalamic–pituitary axis imaging. Radiographic evidence of CNS involvement (i.e., dural, brain, including Fazekas score >1, or spinal cord) occurred in 55% (22/40) and was unassociated with significantly increased mortality.ConclusionsErdheim-Chester disease commonly and variably involves the neuraxis. Patients with suspected Erdheim-Chester disease should undergo MRI brain and spine and screening investigations (serum sodium, serum and urine osmolality) for diabetes insipidus to clarify extent of neurologic disease.

scholarly journals Misdiagnosis: CNS Erdheim–Chester disease mimicking CLIPPERS

2017 ◽  
Vol 31 (4) ◽  
pp. 399-402 ◽  
Author(s):  
Jillian Berkman ◽  
Caleb Ford ◽  
Emily Johnson ◽  
Beth A Malow ◽  
Joseph M Aulino
Keyword(s):  
Case Report ◽  
Clinical Symptoms ◽  
Brain Mri ◽  
Subcortical White Matter ◽  
Mri Findings ◽  
Erdheim Chester Disease ◽  
Benign Process ◽  
Magnetic Resonance Imaging Mri ◽  
Erdheim Chester ◽  
Chester Disease

Introduction Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) is an inflammatory central nervous system (CNS) disorder with particular involvement of the pons. Diagnostic criteria include a range of clinical symptoms related to the underlying brainstem pathology, visible with magnetic resonance imaging (MRI). MRI findings include the appearance of punctuate and curvilinear gadolinium enhancement ‘peppering’ the pons. We discuss a patient presenting with clinical and radiographic characteristics of CLIPPERS who was diagnosed with Erdheim–Chester disease (ECD). Case report A 52-year-old male presented with 2 years of progressive spasticity, dysarthria, and gait instability. Initially, he was diagnosed with Parkinson’s disease at an outside hospital, based on tremor, rigidity, and gait instability; however, he failed to improve with a trial of levodopa. Brain MRI showed small enhancing parenchymal nodules coalescing in the central pons, but also affecting the cerebellum and cerebellar peduncles, with more punctate enhancing lesions in the cerebral lobar subcortical white matter. When the patient’s response to steroids was inadequate, further imaging was done, revealing perinephric processes. Subsequent biopsy revealed ECD. Conclusions A review of the literature for cases of CLIPPERS demonstrated a subset of patients later found to have various malignancies involving the CNS. This case report uses the patient’s unique radiographic and clinical presentation to demonstrate the importance of the exclusion criteria within the CLIPPERS diagnostic requirements and stresses red flags suggestive of alternative diagnoses. This distinction is of high importance when differentiating a relatively benign process such as CLIPPERS from more malignant diseases.

Case report of Erdheim-Chester disease in a female cardiac surgery patient

2011 ◽  
Vol 59 (S 01) ◽  
Author(s):  
HR Mahoozi ◽  
A Zittermann ◽  
K Hakim-Meibodi ◽  
J Gummert ◽  
N Mirow
Keyword(s):  
Cardiac Surgery ◽  
Case Report ◽  
Cardiac Surgery Patient ◽  
Erdheim Chester Disease ◽  
Surgery Patient ◽  
Erdheim Chester ◽  
Chester Disease

Erdheim-Chester Disease Presenting with Histiocytic Colitis and Cytokine Storm

2017 ◽  
Vol 26 (2) ◽  
pp. 183-187
Author(s):  
George P. Christophi ◽  
Yeshika Sharma ◽  
Quader Farhan ◽  
Umang Jain ◽  
Ted Walker ◽  
...  
Keyword(s):  
Periodic Acid ◽  
Poor Response ◽  
Rare Type ◽  
Periodic Acid Schiff ◽  
Hematopoietic Stem ◽  
Hemodynamic Compromise ◽  
Erdheim Chester Disease ◽  
Erdheim Chester ◽  
Chester Disease ◽  
Segmental Colitis

Background: Non-Langerhans histiocytosis is a group of inflammatory lymphoproliferative disorders originating from non-clonal expansion of hematopoietic stem cells into cytokine-secreting dendritic cells or macrophages. Erdheim-Chester Disease (ECD) is a rare type of non-Langerhans cell histiocytosis characterized by tissue inflammation and injury caused by macrophage infiltration and histologic findings of foamy histiocytes. Often ECD involves the skeleton, retroperitoneum and the orbits. This is the first report documenting ECD manifesting as segmental colitis and causing cytokine-release syndrome.Case presentation: A 68-year old woman presented with persistent fever without infectious etiology and hematochezia. Endoscopy showed segmental colitis and pathology revealed infiltration of large foamy histiocytes CD3-/CD20-/CD68+/CD163+/S100- consistent with ECD. The patient was empirically treated with steroids but continued to have fever and developed progressive distributive shock.Conclusion: This case report describes the differential diagnosis of infectious and immune-mediated inflammatory and rheumatologic segmental colitis. Non-Langerhans histiocytosis and ECD are rare causes of gastrointestinal inflammation. Prompt diagnosis is imperative for the appropriate treatment to prevent hemodynamic compromise due to distributive shock or gastrointestinal bleeding. Importantly, gastrointestinal ECD might exhibit poor response to steroid treatment and other potential treatments including chemotherapy, and biologic treatments targeting IL-1 and TNF-alpha signaling should be considered.Abbreviations: AFB: acid-fast bacilli; ECD: Erdheim-Chester Disease; IBD: inflammatory bowel disease; PASD: periodic acid-Schiff with diastase; TB: tuberculosis

Erdheim-Chester Disease Involving Bilateral Lower Extremities

2000 ◽  
Vol 174 (3) ◽  
pp. 875-876 ◽  
Author(s):  
Tamio Kushihashi ◽  
Hirotsugu Munechika ◽  
Masayuki Sekimizu ◽  
Etsuo Fujimaki
Keyword(s):  
Lower Extremities ◽  
Erdheim Chester Disease ◽  
Erdheim Chester ◽  
Chester Disease

scholarly journals Isolated thoracic intramedullary Erdheim-Chester disease presenting with paraplegia: a case report and literature review

2021 ◽  
Vol 22 (1) ◽  
Author(s):  
Ikchan Jeon ◽  
Joon Hyuk Choi
Keyword(s):  
Surgical Resection ◽  
Fdg Pet ◽  
Rare Case ◽  
Systemic Disease ◽  
Mass Lesion ◽  
Erdheim Chester Disease ◽  
Pet Ct ◽  
Fdg Pet Ct ◽  
Erdheim Chester ◽  
Chester Disease

Abstract Background Erdheim-Chester disease (ECD) is a rare, idiopathic, systemic non-Langerhans cell histiocytosis involving long bone and visceral organs. Central nervous system (CNS) involvement is uncommon and most cases develop as a part of systemic disease. We present a rare case of variant ECD as an isolated intramedullary tumor. Case presentation A 75-year-old female patient with a medical history of diabetes and hypertension presented with sudden-onset flaccid paraparesis for 1 day. Neurological examination revealed grade 2–3 weakness in both legs, decreased deep tendon reflex, loss of anal tone, and numbness below T4. Leg weakness deteriorated to G1 before surgery. Preoperative magnetic resonance imaging (MRI) and 18F-fluorodeoxyglucose positron emission tomography/computed tomography (FDG-PET/CT) showed an intramedullary mass lesion at T2-T4 with no systemic lesion, which was heterogeneous enhancement pattern with cord swelling and edema from C7 to T6. Gross total removal was achieved for the white-gray-colored and soft-natured intramedullary mass lesion with an ill-defined boundary. Histological finding revealed benign histiocytic proliferation with foamy histiocytes and uniform nuclei. We concluded it as an isolated intramedullary ECD. The patient showed self-standing and walkable at 18-month with no evidence of recurrence and new lesion on spine MRI and whole-body FDG-PET/CT until sudden occurrence of unknown originated thoracic cord infarction. Conclusions We experienced an extremely rare case of isolated intramedullary ECD, which was controlled by surgical resection with no adjuvant therapy. Histological examination is the most important for final diagnosis, and careful serial follow-up after surgical resection is required to identify the recurrence and progression to systemic disease.

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