Diffuse reduction of cerebral grey matter volumes in Erdheim-Chester disease

Table 1 Patient characteristics

Patient	Organ systems affected by ECD	Co-morbid illnesses	Cognitive complaints	Concurrent Medications	Inflammatory Markers^a	CSF Examination^b
66 Female	Myocardium, bones	None	No	None	ESR >100	1 WBC; Protein 28; Glucose 57;
66 Female	Myocardium, bones	None	No	None	CRP 4.39	1 WBC; Protein 28; Glucose 57;
75 Male	Mesentery, orbit, bones, skin	Chronic myelomonocytic leukemia	Yes	Prednisone	CRP 0.98	-
54 Male	Brainstem, retroperitoneum, bones	Diabetes mellitus	No	Prednisolone	ESR 31	2 WBC; Protein 42; Glucose 78
54 Male	Brainstem, retroperitoneum, bones	Diabetes mellitus	No	Prednisolone	CRP 1.8	2 WBC; Protein 42; Glucose 78
51 Male	Retroperitoneum, aorta, pericardium, pleura, bones	Essential thrombocytosis	Yes	Interferon-alpha, anakinra	-	1 WBC; Protein 21; Glucose 54
51 Male	Orbit, retroperitoneum, epidural soft tissues, bones	Ulcerative colitis	Yes	None	CRP 1.63	-
60 Male	Retroperitoneum, lung, skin, bones	MALT lymphoma	Yes	Prednisone	ESR 92	2 WBC; Protein 33; Glucose 58
60 Male	Retroperitoneum, lung, skin, bones	MALT lymphoma	Yes	Prednisone	CRP 1.49	2 WBC; Protein 33; Glucose 58
48 Female	Orbit, retroperitoneum, bones	None	Yes	Prednisone	CRP 3.81	-
66 Female	Brainstem, retroperitoneum, bones	None	Yes	Prednisone	-	2 WBC; Protein 71; Glucose 57
68 Male	Retroperitoneum, bones, subcutaneous soft tissues	None	No	None	ESR 61	-
68 Male	Retroperitoneum, bones, subcutaneous soft tissues	None	No	None	CRP 6.29	-
58 Male	Orbit, retroperitoneum, bones	None	No	Prednisone	CRP 3.42	-
52 Female	Subcutaneous soft tissues, maxillary sinus, bones, skin	None	Yes	None	CRP 2.56	-

^aNormal range for ESR 0-15 mm/h; Normal range for C-Reactive Protein <0.80 mg/dl
^bNormal WBC count in CSF <4 cells per μL; Normal CSF protein 21–38 mg/dL; Normal CSF glucose 38–82 mg/dL

ISSN: 1750-1172