Table 1

Clinical characteristics of NF2 patients in four studies

CharacteristicKanter et al14 Evans et al15 Parry et al19 Mautner et al20
No of cases731206348
No of families17753244
Sporadic cases0451744
Mean age at onset (y)20 (of 59)222017
Intracranial meningiomas (%)18454958
Spinal tumours (%)NA266790
Skin tumours (%)32 (of 73)68 (of 100)6764
>10 skin tumours (%)NA10 (of 100)NANA
Café au lait macules (%)42 (of 31)43 (of 100)47NA
Cataract (%)NA38 (of 90)8162
Intracranial astrocytoma (%)NA4.11.6NA
Ependymoma (%)NA2.53.26
Optic sheath meningioma (%)1-150 NA4.14.88
  • 1-150 In Mautner et al,20 the frequency of optic nerve sheath tumours is for all histological types (that is, schwannomas and meningiomas).