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
Q

Spina bifida : Severity of defect

Spina bifida ______

Spina bifida _________

___________/_____________

A

occulta

cystica

Myelocele/syringomyelocele

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26
Q

Spina bifida : Severity of defect

Spina bifida occulta: just a ——————-, ____________________

Spina bifida cystica: ______ of the _____________ and or __________________.

Myelocele/syringomyelocele: defective _____________ leading to ________

A

vertebral defect; does not affect spinal cord and meninges

herniation; meninges (dura & arachnoid); spinal cord (meningo-/myelomeningocele)

closure of the spinal canal; CSF leakage

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27
Q

Myelomeningocele and myelocele are associated with neurologic deficits including _______,________, and ______

A

bladder and bowel dysfunction, paraplegia.

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28
Q

__________ and _______ are associated with neurologic deficits including bladder and bowel dysfunction, paraplegia.

A

Myelomeningocele and myelocele

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29
Q

Spina bifida

Diagnosis is by imaging (may be confused with _________________).

Increased levels of _____ in maternal blood in certain cases

Amenable to _________

A

sacrococcygeal teratomas

AFP

surgery

30
Q

Hydrocephalus
The __________ of ________________ in the ventricular system.

A

accumulation

excessive cerebrospinal fluid

31
Q

Hydrocephalus

This usually results in the _______ of the ________ and increased ——————

A

dilatation

ventricles

intracranial pressure.

32
Q

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)

A

3

Africa and Latin America

North America

33
Q

Hydrocephalus (Classification)

________ hydrocephalus
________ hydrocephalus

A

Primary

Secondary

34
Q

Hydrocephalus (Classification)

Primary hydrocephalus: characterised by _______________ alongside ___________

A

increased volume of CSF

increased intracranial pressure.

35
Q

Hydrocephalus (Classification)

Primary hydrocephalus:

It can be divided into:
○_____________
○________________

A

Communicating

Non-communicating

36
Q

Secondary hydrocephalus: caused by _____________ with resultant compensatory ___________. In this case, there is ______________________________

A

reduction in neural tissue

increase in CSF volume

no increase in intracranial pressure.

37
Q

CSF production

Produced by the _________ in the ________________

_____ -_______ mls in volume

A

choroid plexus

lateral, 3rd, 4th ventricles

120 - 150

38
Q

Primary Hydrocephalus
Aetiology

○________ to CSF flow
○_____________ of CSF
○__________________ of CSF

A

Obstruction

Increased production

Reduced absorption

39
Q

Non-communicating Hydrocephalus

Characterised by ———- to CSF flow at the level of the _____________,____________,_____________ or __________________

A

obstructions

intraventricular foramen, 3rd ventricle, aqueduct of sylvius or the foramen of Lushcka and Magendie.

40
Q

Non-communicating Hydrocephalus

Aetiology
○ Congenital :

______ of the aqueduct

___________ malformation

Intra-uterine _______

Progressive _______ of the aqueduct and ____________

____________ syndrome

A

Stenosis

Arnold-Chiari

meningitis

gliosis; arachnoid cysts

Dandy-Walker

41
Q

Non-communicating Hydrocephalus

Aetiology

○ Acquired:

________
__________
___________________

A

Tumours
Haemorrhage
Inflammatory lesions

42
Q

Non-communicating Hydrocephalus

Aetiology

○ Acquired:
Tumours e.g. ____________,_________

Haemorrhage: ————,———-,————-

Inflammatory lesions: _________,_________

A

choroid plexus papillomas, ependymoma

Epidural, subdural, intraventricular

Meningitis, cerebral abscess

43
Q

Communicating Hydrocephalus

CSF _________ within the _________ and the ___________.

A

freely flows

dilated ventricles

spinal canal

44
Q

Communicating Hydrocephalus

Aetiology:
○Overproduction of CSF e.g. _________
○Impaired reabsorption e.g. following ______,___________ etc
○Congenital absence of _________

A

choroid plexus papilloma

meningitis, subdural haemorrhage

arachnoid villi

45
Q

Secondary hydrocephalus

•__________ hydrocephalus

•Hydrocephalus ______

A

Normal pressure

ex-vacuo

46
Q

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 _________

A

Slow

tissues; does not increase.

47
Q

Secondary hydrocephalus

•Normal pressure hydrocephalus:

○May result from head trauma, infections, post-surgery or be insidious
○Characterised by problems with ________,_________, and —————

A

walking, bladder control and difficulty with reasoning

48
Q

Clinical features of hydrocephalus

Infants:

(Small or Large?) head

(slow or rapid?) increase in head size

bulging ________

_________ vomiting

problems with ______

eyes fixed _________ (“________”)

seizures.

A

Large ; rapid

anterior fontanelle

projectile; feeding

downwards; sun setting

49
Q

Clinical features of hydrocephalus

Older children/young to middle aged adults:

headache, _________

nausea and vomiting

difficulty in __________

poor —————

loss of ______ control

_______ loss

A

blurred vision

maintaining balance

coordination

bladder

memory

50
Q

Clinical features of hydrocephalus

Older adults:

________ problems (____ feeling ____),

——— impairment and _________

loss of _________

poor __________________

A

Walking; feet; stuck

mental ; dementia

bladder control

coordination and balance

51
Q

Investigations of hydrocephalus

Imaging: Ultrasound, MRI, CT Scan
○ ______ ventricles, loss of ____________ and sites of obstruction

Neurological examination

A

Enlarged

sulcal gyral pattern

52
Q

Treatment of hydrocephalus

Surgery:
○______
○________ ———- _______
○Choroid villi ________
○__________ / removal of tumour

A

○Shunts
○Endoscopic third ventriculostomy ○Choroid villi cauterisation ○Debulking / removal of tumour

53
Q

Treatment of hydrocephalus

Drugs: ______ and _______ are useful in reducing CSF production

Physiotherapists, speech therapists etc

A

furosemide and acetazolamide

54
Q

____________ is world spina bifida and hydrocephalus day

A

October 25th

55
Q

Neural Tube

Begins as neural _____ to neural _____ which “closes” to form neural ____

Process complete by ________ gestation

A

plate

fold

Tube

28 weeks

56
Q

Neural Tube

___________ = cranial and caudal open ends of tube

(First or Last?) structures to close

A

Neuropores

Last

57
Q

Neural Tube Defects: Risk Factors

Low ———- intake during pregnancy

_________ drugs, Especially _______ and ________

A

folic acid

Antiepileptic

valproic acid ; carbamazepine

58
Q

Neural Tube Defects
Risk Factors

Folate in pregnancy

Normal-risk mothers: _____ mcg/day

High-risk mothers: ___mg/day

A

400

4

59
Q

ANENCEPHALY

Ultrasound:

____ calvaria

_____-like appearance of fetus

A

Open

Frog

60
Q

ANENCEPHALY

Mother will have ______hydramnios

Why?

A

poly

Baby can’t swallow amniotic fluid normally

61
Q

Least common neural tube defect is ????

A

ENCEPHALOCOELE

62
Q

ENCEPHALOCOELE

Most common site:________ bone

A

occipital

63
Q

SPINA BIFIDA: Clinical Features

May damage ____________ nerves

_____/______ dysfunction

_______ symptoms

Treatment: surgery

A

spinal cord

Motor / sensory

Urologic

64
Q

Spina Bifida Occulta

Often has skin abnormalities: ____,______

Usually (asymptomatic or symptomatic?)

A

dimple, tuft of hair

asymptomatic

65
Q

Least common type of spina bifida is ?????

A

Meningocele

66
Q

Meningocele

Usually ( asymptomatic or symptomatic?)

(Good or Bad?) prognosis

A

asymptomatic

Good

67
Q

Dandy-Walker Malformation

______ or ———— of the _____________

(Enlarged or Shrunken?) _______ ventricle

A

Hypoplasia or agenesis; cerebellar vermis

Enlarged; 4th

68
Q

Dandy-Walker Malformation

______ of ——- ventricle  hydrocephalus

Imaging findings:
Small ________
Dilated ________

A

Cysts; 4th

cerebellum

4th ventricle

69
Q

Dandy-Walker Malformation

Often detected by _______ in utero

A

ultrasound

70
Q

Dandy-Walker Malformation

Many, many associated symptoms/conditions:

____,______, or ______defects

____________________

A

Heart, facial or limb

Spinal bifida

71
Q

Dandy-Walker Malformation

Affected children

_________
Delayed ________
________ dysfunction (crawling, walking) Ataxia

A

Hydrocephalus

development

Motor

72
Q

NPH(normal pressure hydrocephalus) PATIENTS ARE _______,________, and ————

A

WET, WOBBLY & WACKY