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Anatomic Pathology > Congenital CNS Pathology > Flashcards

Congenital CNS Pathology Flashcards

1
Q

Factors involved in the development of CNS congenital disorders include:?

A
  • Maternal and fetal infections
  • Drugs
  • Anoxia
  • Ischemia
  • Genetic
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2
Q

The most common developmental CNS abnormality is ?

A

Neural Tube defects
- results from defective closure of the neural tube
- occurs at the two ends of the nueroaxis

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3
Q

What deficiency is implicated in Neural tube defects ?

A

Folate deficiency

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4
Q

Anencephaly is the ?

A

Absence of cranial vault, incompatible with life

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5
Q

NTD affecting the spinal cord include ?

A
  • Myelocele
  • meningomyelocele
  • Meningocele
  • Spinal bifida occulta
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6
Q

Spinal bifida occulta is ?

A

A bony defect of the vertebral arch

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7
Q

Meningocele is ?

A
  • Bony defect with the outpouching of the meninges.
  • Protrusion of the meninges (filled with csf) through a defect in the skull or spine
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8
Q

Meningomyelocele is?

A

Defective formation of the bony arch with cystic outpouching of the meninges, spinal cord and spinal roots

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9
Q

Myelocele is?

A

Defective bony arch with complete exposure of the spinal cord

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10
Q

The most consistent defect in neural tube defects is ?

A

Defective formation of the bony arch

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11
Q

Complications of NTD?

A

Paraplegia and urinary incontinence from birth

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12
Q

Mention 2 posterior fossa anomalies ?

A
  • Arnold-Chiari malformations (1& 2)
  • Dandy-Walker malformation
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13
Q

Typical features of Type 1 Arnold chiari malformations ?

A
  • Mostly asymptomatic
  • Downward displacement of the cerebellar tonsils
  • could involve syringomyelia
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14
Q

Typical features of type 2 Arnold chiari malformations

A
  • Most often symptomatic
  • Faulty cranio-spinal junction resulting in small posterior fossa
  • there’s Downward displacement of the cerebellar vermis and tonsils
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15
Q

Complications of type 2 A.chiari malformation?

A
  • Compression of the 4th ventricle
  • compresses aqueduct and medulla
  • Obstructive hydrocephalus
  • Frequent lumbar meningomyelocele
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16
Q

The acronym TAS for type 1 Arnold chiari malformation represents what?

A

T - Tonsil herniation (cerebellar tonsils)
A - Asymptomatic
S - Syringomyelia

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17
Q

The acronym MAD for type 2 Arnold chiari malformation represents what?

A

M - Meningomyelocele (Lumbar)
A - Aqueductal and medulla compression
D - Dangerous symptoms (Hydrocephalus, paralysis)

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18
Q

The development of a fluid filled cavity within the spinal cord is called?

A

Syringomyelia

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19
Q

What can you remember about Syringomyelia ?

A
  • Ependymal-lined, CSF filled channel that forms parallel to and connected with the central canal
  • 90% of cases associated with Arnold-chiari type 1
  • 10% post traumatic or intraspinal tumors
  • Syrinx enlarged progressively and destroys the spinal parenchyma
  • symptoms: paralysis and loss of sensory functions
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20
Q

Hydromyelia is?

A
  • an abnormal widening of the central canal of the spinal cord.
  • Dilation of the central canal
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21
Q

What part of the spine is most commonly affected by syringomyelia?

A

The cervical region

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22
Q

Mention 5 neural tube defects?

A

Meningomyelocele
Anencephaly
Spinal bifida occulta
Encephalocele
Myelocele
Meningocele

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23
Q

Forebrain anomalies include ?

A
  • Microcephaly- Small head size
  • Lissencephaly - Absence of convolutions (folds) in the cerebral cortex
  • Agyria - Absence of gyri on the surface of the cerebral cortex (complete lissencephaly)
  • Megalencephaly - a condition in which an infant or child has a large, heavy, and potentially malfunctioning brain.
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24
Q

Forebrain anomalies also include?

A
  • Polymicrogyria - multiple small gyri (microgyri) creating excessive folding of the brain… CMV is a cause
  • Neuronal heterotopias; brain malformations resulting from deficits of neuronal migration
  • Holoprosencephaly; failure of the prosencephalon (the embryonic forebrain) to sufficiently divide into double lobes of cerebral hemispheres
  • Agenesis of the corpus callosum
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25
Q

CNS traumas can result in three categories of injury namely?

A
  • Skull fractures
  • Parenchyma injury
  • Vascular injury
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26
Q

Injury to the head may be ? And may cause ?

A
  • Penetrating or blunt
  • Open or closed injury
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27
Q

The magnitude, distribution and consequence of traumatic brain lesions depends on ?

A
  • The shape of the object causing the injury
  • The force of impact
  • Whether the head is in motion at the time of injury
  • Anatomical location of the lesion
  • Limited capacity of the brain for functional repair
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28
Q

Injury of brain parenchyma may be silent in ? Severely disabling in? Or fatal in?

A

Silent - Frontal lobe injury
Severely disabling - spinal cord injury
Fatal - Brainstem injury

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29
Q

Trauma to the CNS (Cranium cranial cavity and brain) can cause ?

A
  • Skull fracture
  • Brain concussions
  • Contusions
  • Diffuse axonal injury
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30
Q

Trauma to the CNS (Blood vessel rupture) can cause

A
  • Epidural hemorrhage
  • Subdural hemorrhage
  • Subarachnoid hemorrhage
  • Intraparenchymal hemorrhage
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31
Q

Types of cerebral herniations that can result from CNS Trauma;

A

Subfalcian herniation (cingulate gyrus)
Transtentorial herniation (uncal)
Cerebellar tonsillar herniation

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32
Q

Most common cause of cerebral palsy is?

A

Perinatal brain injury

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33
Q

What is the germinal matrix?

A

A highly cellular and highly vascularized region in the brain out from which cells migrate during brain development.

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34
Q

What causes germinal matrix hemorrhage?

A

The blood vessels of the germinal matrix are weak walled and fragile and predisposed to hemorrhage.

Common is infants born before 32 weeks gestation

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35
Q

Periventricular leukomalacia?

A

PL is an injury to the white matter in the brain. White matter softens and dies around the lateral ventricles, leaving fluid-filled cysts.

PL is a white matter lesion in premature infants that results from hypotension, ischemia, and coagulation necrosis at the watershed zones of deep penetrating arteries of the middle cerebral artery.

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36
Q

Multi-cystic encephalopathy ?

A
  • Occurs following prenatal or perinatal hypoxia-ischemia
  • multiple brain infarcts occurring early in pregnancy
  • varying sized cystic lesions in the brain encountered in developing fetuses or infants.
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37
Q

Cerebral palsy is defined as?

A

Non-progressive motor deficit related to prenatal and perinatal neurological insults

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38
Q

The major risk factor for cerebral palsy is ?

A

Prematurity

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39
Q

Clinical manifestations of cerebral palsy ?

A
  • Dystonia - Involuntary muscle contractions
  • Spasticity - abnormal muscle tightness due to prolonged muscle contraction
  • Ataxia - Impaired balance or coordination
  • Athetosis - slow, involuntary, and writhing movements of the limbs, face, and other muscle groups
  • Paresis - Reduction in muscle strength with a limited range of voluntary movement.
40
Q

Ischemic infarcts occurring within the periventricular white mater is called?

A

Periventricular leukomalacia

41
Q

Ischemic infarcts occurring within the cerebral hemisphere

A

Multi-cystic encephalopathy

42
Q

Intraparenchymal hemorrhage occurring within the germinal matrix often occurs between?

A

The thalamus and caudate nucleus and can extend into the ventricular system

43
Q

Ulegyria ?

A

Thin, gliotic gyri due to perinatal cortical ischemia

44
Q

Status marmoratus reflects ?

A

Ischemic neuronal loss and gliosis in the basal ganglia and thalamus associated with irregular myelin formation

45
Q

When bone shifts into the cranial vault by more than its thickness is called?

A

Displaced fracture

46
Q

Accidental falls tend to involve which part of the skull?

A

The occiput

There’s secondary basal skull involvement, lower CN or cervicomedullary symptoms and csf discharge and/or meningitis

47
Q

Trauma occurring as a consequence of syncope tends to involve the?

A

The frontal skull

Diastatic fracture - occur when there is a separation of the cranial sutures, most commonly with the lambdoid suture

48
Q

Concussion is caused by?

A

Change in the momentum of the head (head impact against a rigid surface)

49
Q

Clinical presentation of concussion?

A
  • Loss of consciousness and reflexes
  • temp respiratory arrest
  • amnesia of the event
  • post concussive neuropsychiatric syndromes
50
Q

Chronic traumatic encephalopathy (CLE)?

A

Associated with repeated head injuries and blows to the head.

Cognitive impairment with distinct pathological findings

51
Q

Contussions are caused by?

A

Impact of parts of brain against inner Calvarial surfaces

There’s bruising to the brain that results from vessel and tissue disruption

52
Q

Site of injury of contusion?

A

Crests of orbital gyri in frontal and temporal lobes

  • Coup - site of injury
  • Contrecoup - site diametrically opposite to injury
  • They develop when the head is mobile at the time of impact
53
Q

Signs of Acute contusions;

A

Hemorrhage of the brain tissue in a wedge shaped area

areas of hemorrhage and tissue disruption

54
Q

Signs of subacute contusion?

A

Necrosis and liquefaction of brain

55
Q

What are Remote contusions?

A

Depressed area of the cortex with yellow discoloration (Plaque Jaune)

56
Q

Diffuse axonal injury is caused by ?

A
  • Injury to the white matter due to acceleration and deceleration
  • Damage to the axons at nodes of ranvier
  • impairment of axoplasmic flow
57
Q

Diffuse axonal injury has a predilection for which areas of the brain?

A
  • Corpus callosum
  • Periventricular white matter
  • Hippocampus
  • cerebral and cerebellar peduncles
58
Q

Clinical manifestation of diffuse axonal injury?

A

Coma after trauma without evidence of direct parenchymal injuries

59
Q

Histopathology of Diffuse axonal injury ?

A
  • Axonal swellings appreciable in the white matter
  • Focal hemorrhage
  • Subsequently replaced by degenerated fibers and gliosis (fibrosis in the Brain)
60
Q

Lesion to the thoracic segment of the spinal cord or below results in?

A

Paraplegia (paralysis in left and right legs)

61
Q

Lesions to the cervical segments of the spinal cord results in ?

A

Tetraplegia (paralysis in all 4 limbs)
(C1-C8)

62
Q

Lesions above C4 results in?

A

Respiratory arrest due to paralysis of the diaphragm

63
Q

Acute and chronic finding of spinal cord injury ?

A

Acute - Hemorrhage, necrosis, white matter axonal swellings
Chronic - necrotic lesions become cystic and gliotic

64
Q

Epidural hematomas result from?

A

The rupture of Dural arteries, most commonly middle meninges artery

  • Usually associated with skull fracture
65
Q

Complication of epidural hematoma?

A

Leads to cerebral herniation (sulfacrine mostly) if not promptly evacuated

66
Q

Classical clinical presentation of epidural hematomas ?

A

Lucid intervals - Talk and die syndrome

67
Q

Whats the underlying mechanism behind subdural hemorrhage?

A

It results from tearing of the bridging veins

bridging veins drain the venous blood from the cortical surface, through the subarachnoid and subdural spaces and into the superior sagittal sinus

68
Q

The most susceptible category of people to subdural hemorrhage are ?

A

Geriatric patients with cerebral atrophy even after minor trauma

69
Q

Clinical manifestations of subdural hemorrhage?

A
  • Headache - Slowly progressive and non-localizing
  • Drowsiness and confusion - within 48 hours of injury
  • Focal neurological deficit
  • Dementia
70
Q

Why does chronic subdural hematoma occur?

A

Subdural hematoma can cause recurrent bleeding due to hemorrhage from thin-walled vessels of granulation tissue

Granulation tissue forms during the healing process of a wound, containing fibroblasts and new blood vessels which are thin walled

71
Q

There’s accumulation of what type of blood in epidural hematoma and subdural hematoma ?

A
  • Epidural hematoma - Arterial blood accumulates between the dura and the skull
  • Subdural hematoma - Venous blood accumulates between the dura and the arachnoid matter
72
Q

Sequelae of brain trauma ?

A
  • epilepsy
  • meningioma
  • infectious disease
  • psychiatric disorders
  • Post traumatic hydrocephalus
  • Post traumatic dementia (Dementia pulgilistica)
73
Q

How does post traumatic hydrocephalus occurs?

A

Occurs when hemorrhage into the Subarachnoid space obstruct csf resorption

74
Q

Post traumatic dementia is also called ?

A

Dementia pulgilistica

75
Q

Post traumatic dementia clinical findings?

A

Consequence of repeated head trauma
- hydrocephalus
- corpus callosum thinning
- diffuse axonal injury
- amyloid plaques
- neurofibrillary tangles

76
Q

Define brain herniation?

A

Brain herniation is the displacement of brain issue past rigid dural folds (the falx and tentorium) or through an opening in the skull due to increased ICP

77
Q

Causes of increased ICP?

A
  • Space occupying lesion
  • Cerebral edema
  • Increased venous pressure obstruction of the CSF
78
Q

Space occupying lesions include ?

A

Brain tumor
Edema
Abscess
Contusions
Hematoma

79
Q

Symptoms of raised ICP?

A

Cushings reflex
Initial headache, vomiting and, nauseas
Blurry vision
Drowsiness
Altered mental status
Lethargy

80
Q

Cushings reflex include?

A
  • Hypertension (widened pulse pressure)
  • Bradycardia
  • cheyne stokes breathing (irregular respiration)
81
Q

The most common form of herniation is?

A

Sub-falcine (Cingulate) herniation

82
Q

What happens in subfalcine herniation ?

A

The cingulate gyrus is displaced underneath the falx cerebri, to the opposite side

83
Q

Clinical significance of cingulate herniation?

A

Compression of the anterior cerebral artery

84
Q

Compression of ACA may result in?

A
  • Ischemia in the frontal and parietal lobes
  • Contralateral leg weakness
  • Impaired/loss of consciousness
85
Q

What happens in Cerebellar tonsillar herniation ?

A
  • Displacement of the cerebellar tonsils through the foramen magnum
  • compression of the brain stem, RAS, cardiorespiratory arrest
86
Q

Decorticate posturing involves?

A
  • while your arms flex upward and hold tensely to your chest.
  • legs being held straight out and the toes being pointed downward
  • the head and neck being arched backward
87
Q

Brainstain compression features

A
  • Midbrain compression; Midsized unreactive pupils
  • Pontine hemorrhage: Pinpoint unreactive pupils
  • Abnormal posturing
  • coma
88
Q

What happens in Transtentorial (uncal) herniation?

A

Uncal of temporal lobe displaces over the free edge of the tentorium cerebelli

89
Q

Clinical significance of Uncal herniation?

A
  • Compression of the occulomotor nerve
  • compression of the Posterior cerebral artery
  • compression and rupture of the paramedian basilar artery branches (Duret hemorrhages)
90
Q

Clinical presentation of uncal herniation?

A

1) Ipsilateral oculomotor nerve (CN3) palsy
- Ipsilateral Fixed and dilated pupils
2) Paralysis of Oculomotor muscles later leads to
- ** Ipsilateral Ptosis**
- ipsilateral inferiorlateral gaze (down and out gaze)
3) compression of Ipsilateral PCA
- contralateral Homonymous hemianopsia with macular sparing
- contralateral hemiparesis/hemiplegia
4) compression of the contralateral cerebral peduncle against the tentorium
- Kernohan notch (Ipsilateral hemiparesis/hemiplegia)

91
Q

Duret hemorrhage occurs from the rupture is what artery?

A
  • Paramedian basilar artery branches
  • Very fatal
92
Q

What is cerebral edema??

A

Accumulation of intercellular fluid in the brain parenchyma

93
Q

Types of Cerebral edema?

A

1) Vasogenic Cerebral edema
2) Cytotoxic cerebral edema
3) Interstitial cerebral edema

94
Q

Vasogenic Cerebral edema is associated with what??

A
  • Increased vascular permeability leads to the focal or generalized accumulation of intercellular fluid
95
Q

Cytotoxic Cerebral edema

A
  • Increased intracellular fluid secondary to endothelial neuronal or glial injury (after anoxia or toxic metabolic disturbances)
96
Q

Interstitial cerebral edema?

A

Fluid from the ventricular system transudates across the ependymal lining secondary to increased ICP

Anatomic Pathology (12 decks)

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