Congenital and perinatal disorders of brain

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Congenital and perinatal disorders of brain

• Genetic disorders
• During pregnancy

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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

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Cerebral palsy

• Prevalence 2 3/1000
• The most risk children
• Premature babies particularly those who weigh
  less than 3.3 pounds (1,510 grams)

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Cerebral palsy

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

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Cerebral palsy

• 5. Fetal hypotrophy chronic intrauterine
• hypoxy and malnutrition (mothers
• hypertension, mothers malnutrition,..)
• 6. Intracranial bleeding
• Unmaturated children periventricular
• Maturated children hemispheral hemispheres,
  thalamus, brainstem, cerebellum

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Cerebral palsy

• 7. hypoxic-ischemic damage
• periventricular leukomalacia
• Maturated - subcortical or parasagital
  leukomalacia

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Cerebral palsy

• Forms
• Spasticc 65
• Dyskinetic-dystonicá (extrapyramidal) 20
• Ataktic 5
• Mixed forms

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Cerebral palsySpastic form

• Central motoneuron
• Diparetic 30
• Hemiparetic 30
• Kvadruspastic 5 - mental retardation, epilepsy

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Diplegia spastica
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Cerebral palsyDyskinetic-dystonic form

• Hyperkinetic 20
• Dystonic tonic contraction of extremity or all
  of the body
• Bazálne ganglia
• No mental retardation

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Cerebral palsyAtaktic form

• cerebellum
• hypotonic syndrom, apathy, psychomotiric
  retardation

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Cerebral palsy

• 25 improvement
• 50 improvent after physiotherapy
• Physiotherapy Bobath and Vojta

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Malformations and genetic abnormalities of brain
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Dandy-Walker syndróm

• Cystic enlargment of 4th ventricle, hydrocephalus
• Atresion of foramen Luschkae and Magendii,
  dysgenesis of cerebellar vermis

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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

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Arnold-Chiari malformation
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Arnold-Chiari malformation
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Microcephalus

• Mental retardation
• Loss of hearing
• Spinocerebellar signs
• Risc for malignity

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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

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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

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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

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Meningoencephalocele, meningomyelocele

• therapy
• Surgery
• Brain meningocele and encefalomeningocele,
• Spinal cord good prognosis - operation during
  1st day

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Meningoencephalocele, meningomyelocele

• alfafetoprotein (child albumin in mothers blood)
• Malformation in 1 from 15 pregnancy with high
  levels
• in 1 from 10 000 pregnancy with normal levels

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Phakomatoses

• Group of hereditary diseases affecting he skin
  and other organs as well as the brain

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Tuberous sclerosis (Bourneville)

• Mental deficit
• Skin signs (adenoma sebaceum)
• Brain lesions, lesions of kidney, heart, retina
• Epileptic seizures
• Intracranial calcifications
• Autosomal dominant, chromosome 9

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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

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Tuberous sclerosis (Bourneville)
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Tuberous sclerosis (Bourneville)
Adenoma sebaceum
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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

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Encefalofacial angiomatosis (Sturge-Weber)
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Encefalofacial angiomatosis (Sturge-Weber)