1. Congenital and perinatal disorders of brain
Genetic disorders
During pregnancy

2. Cerebral palsy
Neurodevlopmental nonprogressive lesion of the motor development of the child durineg prenatal, perinatal or postnatal damage of the brain
Affected - perception, cognition, communication, behaviour, epilepsy, musculosceletal problems

3. Cerebral palsy Prevalence: 2 – 3/1000 The most risk children –
Premature babies — particularly those who weigh less than 3.3 pounds (1,510 grams)

4. Cerebral palsy 1. Prematur babies
2. Intrauterine infections (toxoplazmosis, rubeola, cytomegalovirus, herpes, etc....),
3. Developmental anomalies
4. Intrauterine intoxication (alkohol, opiats, kokain, Pb, ....)

5. Cerebral palsy 5. Fetal hypotrophy – chronic intrauterine
hypoxy and malnutrition (mother´s
hypertension, mother´s malnutrition,..)
6. Intracranial bleeding
Unmaturated children – periventricular
Maturated children – hemispheral – hemispheres, thalamus, brainstem, cerebellum

6. Cerebral palsy 7. hypoxic-ischemic damage periventricular leukomalacia
Maturated - subcortical or parasagital leukomalacia

7. Cerebral palsy Forms: Spasticc – 65%
Dyskinetic-dystonicá (extrapyramidal) – 20%
Ataktic – 5%
Mixed forms

8. Cerebral palsy Spastic form
Central motoneuron
Diparetic – 30%
Hemiparetic – 30%
Kvadruspastic – 5% - mental retardation, epilepsy

9. Diplegia spastica

10. Cerebral palsy Dyskinetic-dystonic form
Hyperkinetic – 20%
Dystonic – tonic contraction of extremity or all of the body
Bazálne ganglia
No mental retardation

11. Cerebral palsy Ataktic form
cerebellum
hypotonic syndrom, apathy, psychomotiric retardation

12. Cerebral palsy 25% improvement 50% improvent after physiotherapy –
Physiotherapy –Bobath and Vojta

13. Malformations and genetic abnormalities of brain

14. Dandy-Walker syndróm Cystic enlargment of 4th ventricle, hydrocephalus
Atresion of foramen Luschkae and Magendii, dysgenesis of cerebellar vermis

15. Arnold-Chiari malformation
Part of brainstem and cerebellar tonsils are below the level of foramen magnum
Sometimes hydrocephalus, spina bifida
Clinical feature – in adult age, bilateral pyramidal signs, lesions of cranial nerves, brainstem syndromes, syringomyely

16. Arnold-Chiari malformation

17. Arnold-Chiari malformation

18. Microcephalus Mental retardation Loss of hearing Spinocerebellar signs
Risc for malignity

19. Meningoencephalocele, meningomyelocele
Development of neuronal tube – 19th day of devolopment of faetus, occlusion of cranial and spinal end in the end of the 1st month
Meninges and parts – from mesenchyma

20. Meningoencephalocele, meningomyelocele
Non connestions of bone spina bifida occulta (risc – low level of fol acid)
Herniation of meninges through defect in bone – meningocele
Brain and spine tissue in herniation – encefalo(myelo)meningocele
Part of ventricles in herniation – encefalo(myelo)meningocystocele

24. Meningoencephalocele, meningomyelocele
Neuronal tube is open, not covered– open anencephalus
Clinical feature – depends on localisation and severity
Spinal cord lesions - paraparesis - paraplegia below the pathology of spinal cord incontinentia
Brainn lesions – depends on localisation

25. Meningoencephalocele, meningomyelocele
therapy
Surgery
Brain – meningocele and encefalomeningocele,
Spinal cord – good prognosis - operation during 1st day

28. Meningoencephalocele, meningomyelocele
alfafetoprotein (child albumin in mother´s blood)
Malformation in 1 from 15 pregnancy with high levels
in 1 from pregnancy with normal levels

29. Phakomatoses
Group of hereditary diseases affecting he skin and other organs as well as the brain

30. Tuberous sclerosis (Bourneville)
Mental deficit
Skin signs (adenoma sebaceum)
Brain lesions, lesions of kidney, heart, retina
Epileptic seizures
Intracranial calcifications
Autosomal dominant, chromosome 9

31. Tuberous sclerosis (Bourneville)
Mental deficit – first 2 years
Depigmental lesions of skin
Naevus Pringle (adenoms) – in 1st year
Calcifications on X-ray - first 2 years
Epi seazures - first 2 years
Retina
Brain lesions

32. Tuberous sclerosis (Bourneville)

33. Tuberous sclerosis (Bourneville)
Adenoma sebaceum

34. Encefalofacial angiomatosis (Sturge-Weber)
Cutaneous haematoma in face
Seizures
Mental retardation
Hemiparesis
X-ray – kalcifications at the convexity hemisfér (kalcifikácie v of hemisfers P-O
Autosomaly dominant

35. Encefalofacial angiomatosis (Sturge-Weber)

36. Encefalofacial angiomatosis (Sturge-Weber)