Hippel-Lindau disease

• Retinal angiomatosis

• Cerebellar angioma

• In adult cerebellar signs and ICH

• Sometimes angiomas of other organs

• Therapy - surgery

• Autosomally dominant

Hippel-Lindau disease

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