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Neurology - Sem 3 > Case 1 - Spina bifida > Flashcards

Case 1 - Spina bifida Flashcards

1
Q
  1. What were the findings of the Black report?
  2. What did the whitehall 2 study find?
  3. What are the 3 explanations for social inequality and health outcomes?
A
  1. Likelihood of a person becoming ill is strongly linked to the social and economic heirarchy
  2. There is a strong correlation between health outcomes and socioeconomic status
  3. Natural & Cultural & materialist explanations
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2
Q
  1. What does the natural/social selection explanation say?
  2. What does the cultural explanation say?
  3. What does the materialist explanation say?
A
  1. The poor are more likely to have problems which increase their poverty or ill health
  2. Poor people have poor diets, and habits which then leads to unhealthy lives.
  3. People are unhealthy or hazardous due to societies organisation
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3
Q
  1. At what age is a person presumed to have capacity?
  2. At the age of 16 what can a person do?
  3. If serious treatment is needed, which guidelines are used?
  4. What do the Fraser guidelines state? (3)
  5. What does the gillick ruling apply to?
  6. How many parents’ consent is adequate?
  7. What are the rights of a child to confidentiality?
A
  1. 18 yrs old
  2. Consent to non-life threatening treatment
  3. Fraser guidelines
  4. Assess the maturity and understanding of the individual, they should be able to understand the complexity of the decision and know and understand the consequences of their decision
  5. Contraception and sexual health advice
  6. 1 parent is sufficient
  7. Same as an adult
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4
Q
  1. What is the relationship between folic acid and spina bifida (SB)?
  2. What is the function of folic acid in the body?
  3. What is folic acid converted into? Where is this done?
  4. What is this intermediate then converted into?
  5. When must a mother take folic acid, in order to decrease the risk of SB? Why?
  6. Where does the embryo take folate from if the mother is deficient?
A
  1. FA can prevent spina bifida
  2. Used to make pyrimadines, and purines, & Methylation & Production of NT’s
  3. Converted into dihydrofolate, in the gut
  4. THF
  5. Before conception, as during neural tube development the embryo is not connected to the mother so her stores wont affect it
  6. Liver
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5
Q
  1. Which part of the neural tube fails to close in ancephaly?
  2. Which part of the neural tube fails to close in SB?
  3. What are the 4 causes of neurodevelopmental defects?
  4. What is Cranial Rachiasisis?
  5. What is Lissencephally?
  6. What is Microcephaly?
A
  1. Top
  2. Bottom
  3. Genetic, Metabolic disorders (Phenylketonuria), Environmental (Alcohol, Nutritional deficiencies), or complications of infection etc.
  4. Open neural tube
  5. Smooth brain, with no convolutions. Cells dont stop migrating to the surface of the brain
  6. Small brain, due to failure to generate migration into the developing brain
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6
Q
  1. What is Holodposencephally?
  2. Why are brain tumours more common in babies?
  3. What is Ancephaly?
  4. What is Encephalocele?
  5. What is Hydrocephalus?
  6. What is the treatment?
A
  1. Failure of the brain to split on the midline, incompatible with life when severe.
  2. Because there is rapid growth, so more chances for mistakes
  3. Brain and meninges stick out from back of the babies head.
  4. Collection of too much CSF in the brain, rise in pressure in the cranium
  5. Shunt into the brain to drain the CSF, must remain for the life of the patient
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7
Q
  1. What are the 3 types of Spina bifida?
  2. Which region is most commonly affected?
A
  1. Spina bifida occulta, Meningocele, Myelomeningeocele
  2. Lumbosaccral region
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8
Q
  1. What is Spina Bifida Occulta?
  2. What causes the hair growth on the skin outside?
  3. How is it treated?
A
  1. Opening in vertebrae of spinal cord, NO damage to spinal cord
  2. Growth factors in the CSF
  3. Surgery to repair the opening
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9
Q
  1. What is Meningocele?
  2. How common is it?
  3. What is found in the sac?
A
  1. Spine is open and meningocele (Sac) protrudes
  2. Rarest type
  3. Meninges and spinal fluid + NO neural tissue
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10
Q
  1. What is a myelomeningocele?
  2. What does the sac contain?
  3. What is the biggest risk if it is open?
  4. What are the possible results?
  5. What is there an increased risk of?
A
  1. Opening in spinal cord
  2. Meninges and spinal nerves
  3. Infection
  4. Legs can be left paralysed, due to nerves being exposed, can also affect bowel movements
  5. Risk of tethered cord syndrome later in life
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11
Q
  1. What are the environmental factors causing SB? (4)
  2. What pharmacological agents can cause SB?
  3. What genetic factors can cause SB? (3)
A
  1. Folic acid deficiency, Low parity, Low socioeconomic status, and diabetes & Obesity
  2. Sodium Valproate
  3. Trisomy 13, 18, MTHFR gene
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12
Q
  1. What is looked at in blood samples when diagnosing SB?
  2. Why would there be increased AFP?
  3. What is the lemon sign?
  4. How does it happen?
  5. When does it disappear?
  6. What causes it to regain its shape later? (2)
A
  1. High Alpha fetoprotein levels
  2. Leaks into the maternal blood as the neural tube is open
  3. Shape of fetal skull, as the frontal bones appear concave or flattened (Like top of lemon)
  4. Decrease in intraspinal pressure, so brain shifts down – decrease in intracranial pressure so there cranium implodes
  5. 24 weeks
  6. Increase strength in bones or Hydrocephalus (Increases pressure again)
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13
Q
  1. When are the first pregnancy ultrasound scans taken?
  2. When are the 2nd scans taken? For what reason?
A
  1. 8-14 weeks
  2. 18-21 weeks, Anomaly scan
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14
Q
  1. What are the other signs of SB?
  2. What is the banana sign?
  3. What is it associated with?
  4. When does it disappear?
  5. Where is it seen?
  6. What is lost?
A
  1. Ventriculomegaly, Microcephaly
  2. Cerebellum wrapped around the brainstem, spinal cord tethering
  3. Chiari 2 malformation
  4. 24 weeks
  5. Posterior cranial fossa
  6. Cisterna magna
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15
Q

Identify:

  • Lemon sign
  • Banana sign
  • Ventriculomegaly
A
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16
Q
  1. What are Chiari malformations?
  2. What is a primary chiari malformation?
  3. What is a secondary chiari malformation?
A
  1. Structural defect in the cerebellum
  2. Caused by structural defect in brain and spinal cord during fetal development
  3. CM caused when CSF is drained excessively, or due to injury, or infection
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17
Q
  1. What is a type I CM?
  2. What is a type type II CM? What is it also called?
  3. What is a type III CM?
  4. What is a type IV CM?
A
  1. The cerebellar tonils enter the foramen magnum, can be acquired
  2. Cerebellar tonsils and brainstem tissue protrude through FM, Arnold Chiari malformation. (Vermis herniation)
  3. Whole cerebellum and brainstem protrude through to spinal cord, and 4th ventricle.
  4. Incomplete or underdeveloped cerebellum (Hypoplasia)
18
Q
  1. What are the symptoms of a CM?
  2. What is syringomyelia?
  3. What is tethered cord syndrome?
A
  1. Neck pain, balance and vision problems, headaches
  2. CSF filled cyst within the spinal cord central canal, destroying it
  3. When spinal cord is attached to bony spine, and there is abnormal stretching leads to nerve damage
19
Q
  1. What is an X-ray used for?
  2. What is a CT scan used for?
A
  1. Identify bone abnormalities
  2. Identify hydrocephalus, and bony abnormalities
20
Q
  1. What are the treatments for CM? (2)
  2. What is posterior fossa decompression surgery?
  3. What is a spinal laminectomy?
A
  1. Spinal laminectomy, Posterior fossa decompression surgery
  2. Creates more space in cerebellum, relieves spinal column pressure
  3. Removal of the lamina to increase size of spinal canal, release pressure
21
Q
  1. How many pregnancies does myelomeningocele affect?
  2. Is it more likely in males or females?
A
  1. 1 in 2000
  2. Females 1.2 to 1
22
Q

Define:

  • Hypomania
  • Mania
  • Depression
A
  • Less severe elevations of mood, usually doesnt require medical attention
  • Severe elevated mood with psychotic symptoms
  • Feeling down
23
Q
  1. What is Bipolar 1 disorder?
  2. What is Bipolar 2 disorder?
  3. What is the average age of onset?
  4. Is it more common in men or women?
A
  1. Episodes of mania & Depression
  2. Depressive episode with 1 episode of hypomania
  3. 25 years old
  4. Women 2:1
24
Q
  1. What are the 3 treatment methods for mania?
  2. What are 3 examples of antipsychotics?
  3. What is the treatment for depression?
  4. Why mustnt antipsychotics be given to pregnant, or women who could get pregnant?
A
  1. CBT & Antipsychotics & Benzodiazepines
  2. Lithium, Valproate, Carbamazepine
  3. SSRI’s - Antidepressants
  4. It has teratogenic effects
25
Q
  1. Which genes control cerebral cortex development?
  2. Where do glial cells begin? What causes them to migrate to the top of the cortical areas?
  3. Which cells in the embryo become the peripheral nervous system? (PNS)
A
  1. Emx & Otx genes
  2. Bottom, Migration signals in the CSF
  3. Neural crest cells
26
Q
  1. What usually pumps CSF away from the ventricles?
A
  1. Arachnoid villi
27
Q

What do the following form in the embryo?

  • Ectoderm?
  • Mesoderm?
  • Endoderm?
A
  • Skin and nervous system
  • Muscles and heart
  • Internal organs
28
Q
  1. What is gastrulation?
  2. What sends a signal to the neral plate?
  3. What does the Notochord secrete? What are they for?
  4. What are Morphogens? Give 2 examples?
A
  1. Where the mesoderm invaginats and the neural fold starts to form
  2. Mesoderm underneath it
  3. Molecules which aid differentiation and growth
  4. Secreted proteins which define identity of neurons. SHH & BMP
29
Q
  1. What part secretes BMP?
  2. What part secretes SHH?
  3. What molecules set up the AP axis?
  4. What are Homeotic genes?
A
  1. Mesoderm
  2. Notochord and floor of neural tube
  3. FGF or Retinoic acid
  4. Transcription factors that dictate differentiation
30
Q
  1. When does the neural plate form?
  2. When do the neural folds form?
  3. Which end does fusion of the folds start?
A
  1. Day 17
  2. Week 3
  3. Cervical end
31
Q
  1. What day does the cranial neuropore close?
  2. What day does the caudal neuropore close?
A
  1. 25 days
  2. 28 days
32
Q

Identify the following parts of the the embryo:

  • Mesoderm
  • Ectoderm
  • Endoderm
  • Notochord
  • Neural crest cells
  • Neural tube
  • Neural plate
33
Q
  1. What are the 3 parts of the neural tube?
  2. What are the 2 parts of the prosencephalon?
  3. What does the Telencephalon form?
  4. What does the Diencephalon form?
A
  1. Prosencephalon (Forebrain), Mesencephalon (Midbrain), Rhombencephalon (Hindbrain)
  2. Telencephalon, Diencephalon
  3. Cerebral hemispheres
  4. Thalamus & Hypothalamus
34
Q

Identify these parts of the embryonic NS:

  • Mesencephalon
  • Rhombencephalon
  • Telencephalon
  • 4th ventricle
35
Q
  1. What does the mesencephalon form?
  2. What are the 2 parts of the Rhombencephalon?
  3. What does the Metencephalon form?
  4. What does the Myelencephalon form?
A
  1. Tectum, tegmentum
  2. Metencephalon & Myelencephalon
  3. Pons and Cerebellum
  4. Medulla
36
Q
  1. Which layer forms the grey matter of the spinal cord?
  2. Which layer forms the white matter of the spinal cord?
  3. What are the ventral thickenings also called? What part of the spinal cord do they form?
  4. What are the dorsal thickenings also called? What part of the spinal cord do they form?
A
  1. Mantle layer
  2. Marginal layer
  3. Basal plates, ventral motor horn cells
  4. Alar plates, Dorsal sensory horn cells
37
Q
  1. What is the level of the spinal cord in the embryo?
  2. What is the level of the spinal cord at birth?
  3. What is the level of the spinal cord in an adult?
  4. Where does the dural sac extend to?
A
  1. Length of the vertebral column
  2. L3
  3. L2-L3
  4. S2
38
Q
  1. What cells form the glial cells?
  2. What is the breech presentation?
    3.
A
  1. Neurepithelial cells
  2. Breech presentation
39
Q
  1. What is the mechanism of action of Valproate?
  2. What are the 4 methods by which valproate is teratogenic?
A
  1. Potentiates inhibitory action of GABA by promoting GABA synthesis
  2. Drug accumulation, Oxidative stress, Folate antagonism, and Histone deacetylase inhibition
40
Q
  1. How does drug accumulation affect embryo’s?
  2. How does the drug lead to oxidative stress?
A
  1. Valproate crosses the placenta, dissociates from plasma proteins and cross into circulation of embryo
  2. Binds to antioxidants and prevents them removing oxidative species. Causes alterations in DNA/RNA
41
Q
  1. How does it cause folate antagonism?
  2. What happens when there is Histone deacetylase inhibition?
A
  1. Antagonism of folates metabolism which is essential for purine & pyramidine synthesis
  2. Inhibits deacetylase which is needed to decompress DNA strands for transcription to occur
42
Q

Identify the following:

  • Dorsal
  • Ventral
  • Caudal
  • Rostral
  • Anterior
  • Posterior

Neurology - Sem 3 (15 decks)

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