Hippel-Lindau disease

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Hippel-Lindau disease

• Retinal angiomatosis
• Cerebellar angioma
• In adult cerebellar signs and ICH
• Sometimes angiomas of other organs
• Therapy - surgery
• Autosomally dominant

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Hippel-Lindau disease
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Neurofibromatosis (Morbus Recklinghausen)

• Neurofibroms schwanomas
• At periferal nerves periferal paresis
• At roots roots sy, spreading to spinal chanel
  compresion of the spinal cord
• Autosomal dominant
• Chromosome 17, 22

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Neurofibromatosis (Morbus Recklinghausen)

• Intracranial n.VIII., II., epileptic seizures,
  ICH
• Risk of malignity
• Neurofibromas at skin
• Changes at skin café-aulait

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Neurofibromatosis (Morbus Recklinghausen)
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Neurofibromatosis (Morbus Recklinghausen)
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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

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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 pacchions granulations to
  sinus sagitalis superior and blood circulation

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Hydrocephalus
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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

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Hydrocephalus

• e vacuo loss of brain tissue
• Clinical feature
• Big cranium, knocking on the head sound of
  crack pot, eyes sign of sunset

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Hydrocephalus
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Therapy of hydrocephalus
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Basilar impression
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Klippel-Feil