Reference, number and age of patients
Headache
Nausea/vomiting
Papilloedema
Cranial nerves palsy
Ataxia/unsteadiness
Cognitive dysfunctiona
Decreased consciousness/coma
Visual field defects
Baskin and Wilson [13]
n = 74
38 <18 years
50%
–
–
–
–
–
–
72%
Hetelekidis et al. [19]
n = 61
all <21 years
77%
43%
29%
20%
18%
–
3%
–
Weiner et al. [10]
n = 56
26 <19 years
7% adults
15% children
–
–
8% children
–
20% adults
8% children
–
60% adults
54% children
Duff et al. [14]
n = 121
32 ≤16 years
74%
21%
10%
2%
–
10%
29%
62%
Van Effenterre and Boch [17]
n = 122
29 <16 years
53%
–
16%
3%
–
13%
8%
79%
Karavitaki et al. [11]
n = 119
41 <16 years
56% adults
78% children
26% adults
54% children
6% adults
29% children
9% adults
27% children
3% adults
7% children
17% adults
10% children
4% adults
10% children
60% adults
46% children
Gautier et al. [15]
n = 171
65 <18 years
61% adults
61% children
–
–
–
–
–
–
–
Nielsen et al. [16]
n = 189
39 <15 years
59% adults
71.8% children
17% adults
51.3% children
–
7% adults
13% children
–
13% adults
13% children
3% adults
3% children
–
Range
7–81%
21–54%
6–29%
2–27%
3–18%
8–17%
3–29%
46–79%
Reference, number and age of patients
Decreased visual acuity or visual deterioration
Growth failure b
Failure of sexual development b
Hypogonadism (adults)
Poor energy
Somnolence
Anorexia/poor weight gain or weight loss
Obesity or weight gain
Polyuria/polydipsia
Baskin and Wilson [13]
n = 74
38 <18 years
–
93%
–
85%
–
–
–
–
23%
Hetelekidis et al. [19]
n = 61
all <21 years
–
25%
–
–
–
–
–
–
13%
Weiner et al. [10]
n = 56
26 <19 years
47% adults
50% children
8%
4%
10%
–
–
–
4% children
3% adults
Duff et al. [14]
n = 121
32 ≤16 years
62%
17%
14%
–
23%
–
–
8%
12%
Van Effenterre and Boch [17]
n = 122
29 <16 years
80%
45%
–
40%
–
–
–
–
18%
Karavitaki et al. [11]
n = 119
41 <16 years
40% adults
39% children
32%
24%
28%
32% adults
22% children
10% adults
5% children
8% adults
20% children
13% adults
5% children
15% adults
15% children
Gautier et al. [15]
n = 171
55 <18 years
71% adults
75% children
–
–
–
–
–
–
–
12% adults
14% children
Nielsen et al. [16]
n = 189
39 <15 years
65% adults
49% children
–
–
–
47% adults
31% children
–
10% adults
10% children
17% adults
8% children
19% adults
10% children
Range
39–80%
8–93%
4–24%
10–85%
22–47%
5–10%
8–20%
4–17%
3–23%
Other less common or rare features at presentation include motor disorders, as hemi- or monoparesis [11, 12, 17], seizures [12], psychiatric symptoms, as emotional lability, hallucinations, paranoic delusions [11, 12, 14, 23], autonomic disturbances [12], precocious puberty [23, 24], syndrome of inappropriate secretion of antidiuretic hormone [25], chemical meningitis due to spontaneous cyst rupture [26], hearing loss [27], anosmia [12], nasal obstruction [28], epistaxis [29], photophobia [30], emaciation [31], Weber’s syndrome [32] and Wallenberg’s syndrome (signs due to occlusion of the posterior inferior cerebellar artery) [33].
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