1. 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

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

3. Neurofibromatosis (Morbus Recklinghausen)

4. Neurofibromatosis (Morbus Recklinghausen)

5. 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

6. 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

7. Hydrocephalus

8. Hydrocephalus
Obstructive – tumors of IIIth. 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

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

10. Hydrocephalus

11. Therapy of hydrocephalus

12. Basilar impression

13. Klippel-Feil