Objective    To summarize the imaging features and diagnosis of neurocutaneous syndrome.     Methods    Imaging data of 36 patients with neurocutaneous syndrome

proved by operation and clinical symptoms

were retrospectively analyzed.     Results    ① Of all 17 cases of tuberous sclerosis complex (TSC)

subependymal nodular calcification lesions were found in 12 cases on precontrast CT image

patchy calcification lesions in brain were found in 3 cases

  iso- or slight hyper-density nodules protruded into ventricle and around ventricle were found in 2 cases

and accompanied astrocytoma was found in 1 case. After using co

ntrast media

nodules without calcification and astrocytoma were moderately enhanced. These nodules appeared as isodensity signal on precontrast T

1

WI

were markedly enhanced on postcontrast MRI.  ② All 7 cases of neurofibromatosis in the report displayed abnormal skull and spinal canal

concave change of vertebral. Enlarged dura spinal sac were found on CT and  MRI

partly stretching to nerve root. Three cases of bilateral vestibular schwannomas

2 of optic nerve glioma

and 1 meningioma were found. ③ Gyriform cortical calcification lesions were found in 6 patients with Sturge-Weber syndrome on plain film of skull and CT image of brain. In these lesions

notorious strip

nodular and markedly enhanced vessels were also found after intravenous shot of contrast agents. ④ Among 6 cases of von Hipple Linda

3 cases appeared as large cyst with mural nodule in cerebelum

  2 cases as heterogeneous density solid mass in supratentor. Mural nodule and solid mass had marked enhancement on enhanced CT image. The other case showed iso-density signal on T

1

WI

heterogeneous hyper-density signal on T

2

WI and marked enhancement on post-contrast T

1

WI in spinal cord.  Of the 6 cases

one case accompanied with hemorrhage

1 with retinal angioma

3 with multi cyst of liver and kidney

1 with syringomyelia.     Conclusion    Diagnosis of neurocutaneous syndrome can be clearly made by using optimal imaging examination

analyzing all kinds of imaging features and associating typical clinical syndrome.