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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 Neurofibromatosis (Morbus Recklinghausen) • Intracranial – n.VIII., II., epileptic seizures, ICH • Risk of malignity • Neurofibromas at skin • Changes at skin – café-aulait Neurofibromatosis (Morbus Recklinghausen) Neurofibromatosis (Morbus Recklinghausen) 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 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 Hydrocephalus 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 Hydrocephalus • „e vacuo“ – loss of brain tissue • Clinical feature • Big cranium, knocking on the head – sound of crack pot, eyes – sign of sunset Hydrocephalus Therapy of hydrocephalus Basilar impression Klippel-Feil