1. Hippel-Lindau disease Retinal angiomatosis Cerebellar angioma In adult cerebellar signs and ICH Sometimes angiomas of other organs Therapy - surgery Autosomally dominant

2. Hippel-Lindau disease

3. Neurofibromatosis (Morbus Recklinghausen) Neurofibroms – schwanomas At periferal nerves – periferal paresis At roots – root´s sy, spreading to spinal chanel – compresion of the spinal cord Autosomal dominant Chromosome 17, 22

4. Neurofibromatosis (Morbus Recklinghausen) Intracranial – n.VIII., II., epileptic seizures, ICH Risk of malignity Neurofibromas at skin Changes at skin – café-aulait

5. Neurofibromatosis (Morbus Recklinghausen)

7. Ataxia teleangiectasia Louis-Barov sy Cerebellar ataxia Later teleangiektasie – conjuctives, skin Pat.-anat. – degeneration of Purkyne cells and granular cells Gen ATM also risk of cancer

8. Hydrocephalus Enlargment of external and internal space of CSF CSF – origin in plexus chorioideus lateral ventricles, III., IV. ventricle To cisterna cerebellomedularis (magna), resorption through pacchion´s granulations to sinus sagitalis superior and blood circulation

9. Hydrocephalus

10. Obstructive – tumors of III th. ventricle, atresion, stenosis, gliosis, malformation of aqueduc Sylvii, tumors of fossa posterior, obstruction of foramen Magendi, Luschkae, malformations Communicant – obliteration of cysterns (inflammation), obliteration of subarachnoideal space after bleeding, subdural haematoma

11. Hydrocephalus „e vacuo“ – loss of brain tissue Clinical feature Big cranium, knocking on the head – sound of crack pot, eyes – sign of sunset

12. Hydrocephalus

13. Therapy of hydrocephalus

14. Basilar impression

15. Klippel-Feil