Transcript Vrodené a perinatálne získané ochorenia mozgu

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