C.N.S. Normal anatomy, congenital anomalies Flashcards

1
Q

The brain Develops from the _______

A

neural tube

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2
Q

Brain is Subdivided into:

○_____brain
○______brain
○____brain

A

Fore

Mid

Hind

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3
Q

Primary brain vesicles

____encephalon (forebrain)
_____encephalon (midbrain)
_________encephalon (hindbrain)

A

Prosencephalon
Mesencephalon
Rhombencephalon

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4
Q

Secondary brain vesicles
____encephalon
____encephalon
___encephalon
____encephalon
____encephalon

A

Telencephalon
Diencephalon
Mesencephalon
Metencephalon
Myelencephalon

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5
Q

Prosencephalon gives you _________ secondary vesicle

Mesencephalon gives you _________ secondary vesicle

Rhombencephalon gives you _________ secondary vesicle

A

Telencephalon; Diencephalon

Mesencephalon

Metencephalon; Myelencephalon

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6
Q

Prosencephalon gives you _________ Adult neural canal regions

Mesencephalon gives you _________ Adult neural canal regions

Rhombencephalon gives you _________ Adult neural canal regions

A

Lateral ventricles; Third ventricle

Cerebral aqueduct

Fourth ventricle; Central canal

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7
Q

CNS congenital abnormalities

This refers to a morphological abnormality of the CNS that dates to the embryonic or fetal period, regardless of the mechanism of its origin.

_______ are most common

Accounts for between ____ to ——% of cases that occur in Nigeria (studies from Enugu, Jos, Kano, Lagos)

A

Neural tube defects (NTD)

60 to 90

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8
Q

Neural tube defects
Anencephaly

○Total (____) or partial (____) _______ of the brain with absence of the _________ and _______

A

holo

mero

absence

cranial vault and covering skin.

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9
Q

Neural tube defects: Anencephaly

○Results from failure of ________________________________

A

closure of the anterior portion of the embryonic neural tube.

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10
Q

Anencephaly is compatible with life

T/F

A

F

Stillbirths or die immediately after birth

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11
Q

Aetiology of anencephaly

○_____ and _____ deficiency

○Genetic defects: Inactivation of _____________

○Environmental: ______,________

A

Folate & Vit B12

NUAK2 kinase (anencephaly)

Maternal smoking, radiation,

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12
Q

Craniorachischisis
○is basically ________ combined with a _________________________________

○Failure of ____________________

A

Anencephaly

contiguous bony defect of the spine and meninges.

closure of the anterior neuropore.

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13
Q

Craniorachischisis is compatible with life

T/F

A

F

Stillborn foetus

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14
Q

Neural tube defects: Iniencephaly
○Failure of ___________

○Characterised by ________ defect, _________ of the ______ vertebrae and _______________ on the cervical spine.

A

closure of neural tube

occipital bone

spinal bifida ; cervical

retroflexion of the head

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15
Q

Neural tube defects: Iniencephaly

Associated with Trisomies ———— as well as lack of ____________, drugs e.g. _______, and ________.

A

18 & 13

folic acid supplementation

vinblastine; obesity

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16
Q

Iniencephaly is compatible with life

T/F

A

Results in miscarriage or stillborn

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17
Q

Neural tube defects: Encephalocele

○Characterised by a _______________ or _____________ skin covered lesion protruding through a __________________ as a result of the failure of the ___________________________________

A

pedunculated or sessile cystic,

defect in the cranium

surface ectoderm to separate from the neuroectoderm.

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18
Q

Encephalocele

May be classified based on content/site

Content:

Meninges (________)

meninges and brain tissue (________)

brain (_________)

A

Meninges (meningocele)

meninges and brain tissue (meningoencephalocele)

brain (encephalocele)

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19
Q

Encephalocele

May be classified based on content/site

Location: list 6

A

frontal, nasofrontal, occipital, parietal,orbital, nasal

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20
Q

Encephalocele

Diagnosis:______ (pre- and post-natal)

Treatment:_______.

A

imaging

surgery

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21
Q

Spina Bifida

Incomplete embryologic closure of the _____________

A

vertebral arches

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22
Q

Most common NTD is ???

A

Spina bifida

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23
Q

Spina bifida: Classification

○Anatomical: _______ ,______,_______

A

Cervical, Thoracic, Lumbosacral

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24
Q

most common Anatomical location for Spina bifida is ??

A

Lumbosacral

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25
Spina bifida : Severity of defect Spina bifida ______ Spina bifida _________ ___________/_____________
occulta cystica Myelocele/syringomyelocele
26
Spina bifida : Severity of defect Spina bifida occulta: just a ——————-, ____________________ Spina bifida cystica: ______ of the _____________ and or __________________. Myelocele/syringomyelocele: defective _____________ leading to ________
vertebral defect; does not affect spinal cord and meninges herniation; meninges (dura & arachnoid); spinal cord (meningo-/myelomeningocele) closure of the spinal canal; CSF leakage
27
Myelomeningocele and myelocele are associated with neurologic deficits including _______,________, and ______
bladder and bowel dysfunction, paraplegia.
28
__________ and _______ are associated with neurologic deficits including bladder and bowel dysfunction, paraplegia.
Myelomeningocele and myelocele
29
Spina bifida Diagnosis is by imaging (may be confused with _________________). Increased levels of _____ in maternal blood in certain cases Amenable to _________
sacrococcygeal teratomas AFP surgery
30
Hydrocephalus The __________ of ________________ in the ventricular system.
accumulation excessive cerebrospinal fluid
31
Hydrocephalus This usually results in the _______ of the ________ and increased ——————
dilatation ventricles intracranial pressure.
32
The incidence of congenital hydrocephalus in the U.S. is ___/1000 live births. Globally, the incidence is higher in ______ and _______ (145 and 316/100,000 live births). It is lowest in _______ (68/100000 live births)
3 Africa and Latin America North America
33
Hydrocephalus (Classification) ________ hydrocephalus ________ hydrocephalus
Primary Secondary
34
Hydrocephalus (Classification) Primary hydrocephalus: characterised by _______________ alongside ___________
increased volume of CSF increased intracranial pressure.
35
Hydrocephalus (Classification) Primary hydrocephalus: It can be divided into: ○_____________ ○________________
Communicating Non-communicating
36
Secondary hydrocephalus: caused by _____________ with resultant compensatory ___________. In this case, there is ______________________________
reduction in neural tissue increase in CSF volume no increase in intracranial pressure.
37
CSF production Produced by the _________ in the ________________ _____ -_______ mls in volume
choroid plexus lateral, 3rd, 4th ventricles 120 - 150
38
Primary Hydrocephalus Aetiology ○________ to CSF flow ○_____________ of CSF ○__________________ of CSF
Obstruction Increased production Reduced absorption
39
Non-communicating Hydrocephalus Characterised by ———- to CSF flow at the level of the _____________,____________,_____________ or __________________
obstructions intraventricular foramen, 3rd ventricle, aqueduct of sylvius or the foramen of Lushcka and Magendie.
40
Non-communicating Hydrocephalus Aetiology ○ Congenital : ______ of the aqueduct ___________ malformation Intra-uterine _______ Progressive _______ of the aqueduct and ____________ ____________ syndrome
Stenosis Arnold-Chiari meningitis gliosis; arachnoid cysts Dandy-Walker
41
Non-communicating Hydrocephalus Aetiology ○ Acquired: ________ __________ ___________________
Tumours Haemorrhage Inflammatory lesions
42
Non-communicating Hydrocephalus Aetiology ○ Acquired: Tumours e.g. ____________,_________ Haemorrhage: ————,———-,————- Inflammatory lesions: _________,_________
choroid plexus papillomas, ependymoma Epidural, subdural, intraventricular Meningitis, cerebral abscess
43
Communicating Hydrocephalus CSF _________ within the _________ and the ___________.
freely flows dilated ventricles spinal canal
44
Communicating Hydrocephalus Aetiology: ○Overproduction of CSF e.g. _________ ○Impaired reabsorption e.g. following ______,___________ etc ○Congenital absence of _________
choroid plexus papilloma meningitis, subdural haemorrhage arachnoid villi
45
Secondary hydrocephalus •__________ hydrocephalus •Hydrocephalus ______
Normal pressure ex-vacuo
46
Secondary hydrocephalus •Normal pressure hydrocephalus: ○(Slow or Rapid?) but persistent increase in CSF. ○The ______ around the ventricles compensate and the fluid pressure in the head _________
Slow tissues; does not increase.
47
Secondary hydrocephalus •Normal pressure hydrocephalus: ○May result from head trauma, infections, post-surgery or be insidious ○Characterised by problems with ________,_________, and —————
walking, bladder control and difficulty with reasoning
48
Clinical features of hydrocephalus Infants: (Small or Large?) head (slow or rapid?) increase in head size bulging ________ _________ vomiting problems with ______ eyes fixed _________ (“________”) seizures.
Large ; rapid anterior fontanelle projectile; feeding downwards; sun setting
49
Clinical features of hydrocephalus Older children/young to middle aged adults: headache, _________ nausea and vomiting difficulty in __________ poor ————— loss of ______ control _______ loss
blurred vision maintaining balance coordination bladder memory
50
Clinical features of hydrocephalus Older adults: ________ problems (____ feeling ____), ——— impairment and _________ loss of _________ poor __________________
Walking; feet; stuck mental ; dementia bladder control coordination and balance
51
Investigations of hydrocephalus Imaging: Ultrasound, MRI, CT Scan ○ ______ ventricles, loss of ____________ and sites of obstruction Neurological examination
Enlarged sulcal gyral pattern
52
Treatment of hydrocephalus Surgery: ○______ ○________ ———- _______ ○Choroid villi ________ ○__________ / removal of tumour
○Shunts ○Endoscopic third ventriculostomy ○Choroid villi cauterisation ○Debulking / removal of tumour
53
Treatment of hydrocephalus Drugs: ______ and _______ are useful in reducing CSF production Physiotherapists, speech therapists etc
furosemide and acetazolamide
54
____________ is world spina bifida and hydrocephalus day
October 25th
55
Neural Tube Begins as neural _____ to neural _____ which “closes” to form neural ____ Process complete by ________ gestation
plate fold Tube 28 weeks
56
Neural Tube ___________ = cranial and caudal open ends of tube (First or Last?) structures to close
Neuropores Last
57
Neural Tube Defects: Risk Factors Low ———- intake during pregnancy _________ drugs, Especially _______ and ________
folic acid Antiepileptic valproic acid ; carbamazepine
58
Neural Tube Defects Risk Factors Folate in pregnancy Normal-risk mothers: _____ mcg/day High-risk mothers: ___mg/day
400 4
59
ANENCEPHALY Ultrasound: ____ calvaria _____-like appearance of fetus
Open Frog
60
ANENCEPHALY Mother will have ______hydramnios Why?
poly Baby can’t swallow amniotic fluid normally
61
Least common neural tube defect is ????
ENCEPHALOCOELE
62
ENCEPHALOCOELE Most common site:________ bone
occipital
63
SPINA BIFIDA: Clinical Features May damage ____________ nerves _____/______ dysfunction _______ symptoms Treatment: surgery
spinal cord Motor / sensory Urologic
64
Spina Bifida Occulta Often has skin abnormalities: ____,______ Usually (asymptomatic or symptomatic?)
dimple, tuft of hair asymptomatic
65
Least common type of spina bifida is ?????
Meningocele
66
Meningocele Usually ( asymptomatic or symptomatic?) (Good or Bad?) prognosis
asymptomatic Good
67
Dandy-Walker Malformation ______ or ———— of the _____________ (Enlarged or Shrunken?) _______ ventricle
Hypoplasia or agenesis; cerebellar vermis Enlarged; 4th
68
Dandy-Walker Malformation ______ of ——- ventricle  hydrocephalus Imaging findings: Small ________ Dilated ________
Cysts; 4th cerebellum 4th ventricle
69
Dandy-Walker Malformation Often detected by _______ in utero
ultrasound
70
Dandy-Walker Malformation Many, many associated symptoms/conditions: ____,______, or ______defects ____________________
Heart, facial or limb Spinal bifida
71
Dandy-Walker Malformation Affected children _________ Delayed ________ ________ dysfunction (crawling, walking) Ataxia
Hydrocephalus development Motor
72
NPH(normal pressure hydrocephalus) PATIENTS ARE _______,________, and ————
WET, WOBBLY & WACKY