Rosai–Dorfman–Destombes disease of the nervous system: a systematic literature review

Table 1 Characteristics of patients with neurologic Rosai–Dorfman–Destombes disease (N = 224)

Characteristic	All Patients		Adult		Pediatric		p value
Characteristic	N	%	N	%	N	%	p value
Age (years)	37.6	2–79	43.8	18–79	11	2–17	< 0.0001
Sex
Male	149	66.5	127	69.8	22	52.4	0.03
Female	75	33.5	55	30.2	20	47.6	0.03
Presenting symptom(s)
Headache	101	45.1	85	46.7	16	38.1	0.31
Seizure	55	24.6	50	27.5	5	11.9	0.03
Focal finding	73	32.6	62	34.1	11	26.2	0.33
Cranial nerve deficit	26	11.6	19	10.4	7	16.7	0.29
Ocular/visual symptoms	72	32.1	54	29.7	18	42.9	0.10
Cerebellar symptoms	25	11.2	17	9.3	8	19	0.10
Cognitive decline/dementia	13	5.8	10	5.5	3	7.1	0.71
Other symptoms
Sinus/nasal symptoms	4	1.8	4	2.2	0	0	1.00
Superficial mass	3	1.3	2	1.1	1	2.4	0.47
Hormonal abnormalities	4	1.8	1	0.5	3	7.1	0.02
Lymphadenopathy	1	0.5	1	0.5	0	0	1.00
Site(s)
Calvarium	6	2.7	5	2.8	1	2.4	1.00
Dura	111	49.6	101	55.5	10	23.8	0.0002
Spine: dural cord	37	16.5	28	15.4	9	21.4	0.34
Parenchyma	21	9.4	16	8.8	5	11.9	0.56
Orbit	17	7.6	7	3.9	10	23.8	0.0001
Multiple sites	32	14.3	25	13.7	7	16.7	0.62

^*p value comparing variables across adult and pediatric populations. T-test for age and Chisquared or Fisher's test as appropriate for other variables

ISSN: 1750-1172