CNS patho Flashcards

1
Q

clinical presentations of patho of CNS

A
  • raised ICP
  • localising signs - sudden (hemorrhage), gradual (tumour)
  • neurodegenerative stage: cognitive/motor impairment
  • demyelinating diseases (problem w/ the myelin sheath)
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2
Q

CNS diseases

A

V: cerebrovascular disease, intracranial hemorrhage
I: infections
T: raised ICP from trauma
A: demyelinating disease
M: alcoholic encephalopathy, storage diseases
I: idiopathic
N: neoplastic (tumour)

C: congenital malformations
D: neurodegeneration

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3
Q

hydrocephalus

A

increase in CSF volume due to disturbances of formation/flow/absorption
- non-communicating: physical obstruction (tumour/mass, meningitis causing scarring)
- communicating: problem w/ venous drainage, defective absorption
subarachnoid hemorrhage -> block arachnoid villi in subarachnoid space
meningitis

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4
Q

cerebral herniation

A
  • subfalcine herniation: below falx cerebri
    can cause hemorrhage and necrosis -> compress cerebral artery -> infarction
    clinically silent
  • uncal herniation: medial temporal lobe
    loss of consciousness
    compress CN3 -> pupil fixed and dilated
    can cause displacement of the brainstem -> stretch the vessels -> hemorrhage
  • tonsillar herniation: through foramen magnum
    coning: compress pons and medulla -> affect respi and cardiac fn
    presents w/ neck stiffness
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5
Q

causes of cerebrovascular accidents

A
  • ischemia
  • hemorrhage

increases risk:

  • HTN -> lacunar infarcts; HT encephalopathy; HT intracerebral hemorrhage
  • DM
  • atherosclerosis
  • TIA (transient ischemic attack) - temp cerebrovascular insufficiency
  • atrial fibrillation
  • vascular malformations
  • coagulopathy
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6
Q

ischemia

A
  • thrombosis (pale infarct)
  • embolism (red hemorrhagic infarct)
    hemorrhagic infarct CANNOT use thrombolytics

micro appearance - liquefactive necrosis

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7
Q

hypertensive cerebrovascular disease (hemorrhagic)

A

effects:
- lacunar infarcts
multiple infarcts in basal ganglia/ white matter/ brainstem
- HT encephalopathy: diffuse cerebral dysfunction
can cause cerebral herniation (cause of the increased pressure)
- HT intracerebral hemorrhage: caused by atherosclerosis/ hyaline arteriolosclerosis/ aneurysms

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8
Q

hemorrhage within brain tissue

A
  • intraparenchymal - petechial hemorrhages (dislodged fat embolism, malaria, vasculitis, HT encephalopathy)
  • intraventricular

causes:

  • HTN
  • amyloid angiopathy
  • venous sinus thrombosis
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9
Q

hemorrhage outside tissue

A
  • subarachnoid: ruptured berry aneurysms (red) (high mortality)/ vascular malformations
  • subdural: tearing of bridging veins, can be by trauma. acute has high mortality - need urgent decompression, chronic good prognosis
  • epidural: caused by trauma: affects MMA
    lucid intervals, loss of consciousness, sudden deterioration
    immediate decompression surgery
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10
Q

CNS tumours presentation

A

raised ICP -> compression/herniation
headaches
seizures
focal neurological deficits

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11
Q

types of tumours

A

classification by age
children
- medulloblastoma
- ependymoma (ventricles/anywhere with CSF fluid)
- germ cell tumour (midline)
- pilocytic astrocytoma (arise from astrocytes)
adults: likely metastasis

site:

  • meninges: meningioma
  • parenchyma: neurons (neuroblastoma, medulloblastoma); glial cells (gliomas: astrocytoma/ oligodendroglioma/ ependomyoma)
  • ventricles: choroid plexus tumour, ependymoma
  • midline: pituitary tumour, germ cell tumour
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12
Q

meningioma

A

uniform ovoid cells
psammoma bodies
nuclear inclusions

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13
Q

astrocytoma (glial cells in parenchyma)

A
  • pilocytic astrocytoma (low grade) = hairy cell
    IDH1 mutant better prognosis than wild type
    in children
  • glioblastoma multiforme (high grade)
    aggressive
    ‘butterfly tumour’
    palisading necrosis (in a row)
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14
Q

oligodendroglioma (glial cells in parenchyma)

A

affects cerebral cortex
mutation in the IDH1 gene and deletion of 1p and 19q
uniform round cells w/ fried egg appearance

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15
Q

neuronal tumour

A

neuroblastoma
supratentorial (above tentorial cerebelli)
rare, poor prognosis
affects children

medulloblastoma - better prognosis w/ treatment, more common
infratentorial
aggressive, spread via CSF
micro: sheets of small cells, high n/c, mitosis, “carrot shaped nuclei”, rosettes (canals)
treatment: surgery and radiotherapy
- loss of 17p

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16
Q

ependymoma (ventricle and tumour of glial cells in parenchyma)

A

young patients
can cause hydrocephalus -> raised ICP
micro: perivascular pseudorosettes and true rosettes (canals)

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17
Q

choroid plexus tumour

A
  • papilloma
  • carcinoma
    can cause hydrocephalus -> raised ICP
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18
Q

midline tumour

A

pituitary tumour: can compress optic chiasma -> visual field defects (bitemporal hemianopia)
- endocrine: adenoma (more common), carcinoma
- embryonal remnant: craniopharyngioma (benign)
—> micro: cysts lined by sse, calcification
good prognosis but possible recurrence

germ cell tumour ** (when there is a midline tumour, think GC tumour 1st)
affects children & males more
- teratoma: benign/malignant
- germinoma

pineal gland tumour

  • pineocytoma
  • pineoblastoma (high grade)
19
Q

lymphoma

A

B cell non-hodgkin lymphoma

affects immunosuppressed pts, esp AIDS pts

20
Q

peripheral nerve sheath tumours

A

benign:
- schwannoma: attached to nerve, well circumscribed & encapsulated
micro: spindled cells, nuclear palisading (arises from neural cells), hyalinised vessels
- neurofibroma: cutaneous (skin/nervous system), non-encapsulated
autosomal dominant
-> NF1 (neurofibromatosis)
can affect skin, CN8, spinal cord nerve roots. optic nerve lioma, hermartomas nodules
renal/musculoskeletal abnormalities
potential malignant transformation of neurofibromas
-> NF2
clinical diagnosis:
- bilateral vestibular schwannoma/
- 1st deg relative w/ NF2 + unilateral
- multiple meningiomas + unilateral
-> tuberous sclerosis
TSC1 and 2 gene alterations
presentation:
(brain) cortical tubers, hermartoma
(skin) lesions: thickened, elevated, pebbly skin on the lower back - contains a lot of fibrous tissue + lumps of fibrous tissue growth on nail bed
(lung) lesions
(renal) angiomyolipoma
-> von hippel-lindau disease (tumour in many organs)
hemangioblastoma (retinal, cerebellar)
cysts (renal, pancreatic, adrenal)
RCC
pheochromocytoma

malignant:
- neurofibromatosis

21
Q

CNS infections possible routes

A
  • trauma
  • blood borne: septicemia, infective emboli
  • peripheral nerves: rabies, HSV
  • sinusitis, otitis media, dental caries, facial infection (affecting venous sinuses)
22
Q

bacterial infection

A

can cause:

  • extradural abscess
  • meningitis
  • brain parenchyma abscess formation (localised)
23
Q

fungal infection

A

can cause:

  • meningitis
  • brain parenchyma abscess formation (localised)
24
Q

viral infection

A

can cause:

  • meningitis
  • encephalitis
  • meningoencephalitis
25
Q

prion disease (misfolded proteins)

A

can cause:

- encephalitis

26
Q

meningitis

A

clinical presentation:

  • headache
  • fever
  • neck stiffness, kernig’s sign (stiffness of leg doesnt allow extension of knee)
  • photophobia

bacterial:

  • neonates: e.coli, listeria
  • infants: h.influenza
  • adolescent: n.menigitidis
  • adults: s.pneumonia
27
Q

encephalitis

A

clinical presentation: altered mental state

usually caused by viral infection:

  • acute: HSV, measles, HIV, CMV, rubella
  • delayed: VZV, measles
  • prion disease

HSV, rabies -> affect neuron/glial cells
polio, enterovirus -> motor neurons
VZV -> DRG
HIV -> microglia

28
Q

parenchyma abscess

A

clinical presentation:

  • swinging fever
  • raised ICP -> vomitting/ fluctuating conscious level
  • localising signs

causes:

  • single abscess: local source (otitis media/ sinusitis/ trauma)
  • multiple: from septic emboli of infective endocarditis
  • organisms (bacteria): strep, s.aureus, e.coli
29
Q

subdural empyema (pus)

A

from skull/air infection

cause: thrombophlebitis (clotting of blood) of bridging veins -> infarction

30
Q

extradural abscess

A

cause: local - sinusitis

if it infects the spinal cord -> cord compression (emergency)

31
Q

diagnostic method for infections

A
  • imaging: localise the infection
  • sterile samples: CSF/blood
  • EEG: detect prion disease
32
Q

HSV infection

A

affect neuron/glial cells

  • temporal lobe abscess think HSV
    clinical: alteration in mood/memory/behavior
    micro: necrotising, perivascular lymphocytic infiltrates, intranuclear inclusions
33
Q

rabies

A

affect neuron/glial cells
edema, congestion
location: basal ganglia, midbrain, 4th ventricle, spinal cord, DRG
micro:
(hippocampus) cytoplasmic oval eosinophilic inclusion
(cerebellum) purkinje cells

symptoms:
early - (nonspecific) malaise, fever, headache
advanced - CNS excitability: painful touch, violent motor response, spasms, convulsions

34
Q

HIV infection

A

cause menigitis, encephalitis
micro: multinucleated giant cell, microglial nodules (elongated nuclei), perivascular cuffing

if it infects spinal cord: vacuolar myelopathy (holes in myelin sheath) of posterior column

presentation: progressive painless leg weakness/stiffness; sensory loss; imbalance

35
Q

neurosyphilis

A

affects
(brain) neuronal loss -> dementia
(meninges) granulomatous meningitis, subintimal vessel thickening
(spinal cord) demyelination of post cord -> damage skin and joints

36
Q

fungal infection

A

affects immunocompromised pts

  • candida
  • Cryptococcus neoformans

cause
vasculitis and thrombosis -> infarction, hemorrhage
meningitis (crypt)
parenchymal invasion

37
Q

parasitic infection

A

protozoa:

  • malaria
  • toxoplasma
  • amoeba
  • trypanosoma

helminths

  • echinococcus
  • taenia solium
38
Q

prion disease pathogenesis

A

usually genetic cause, affects males more
can also be transmissible - contaminated transplant tissue/ food

spread of misfolded proteins -> causes spongiform encephalopathy -> neuronal death

CJD:
rapidly progressive dementia; abnormal muscular coordination (jerks, myoclonus); personality changes (impaired memory, depression)
variant CJD
affects young adults
slower progress; prominent behavioral changes

micro: spongiform transformation (cerebral cortex, deep grey matter)
amyloid deposits and plaques

39
Q

CNS malformations

A

causes

  • prenatal insult: folate def/ radiation/ infections/ drugs
  • genetic

types:
- neural tube defects: neural tube fail to close
folate def -> spina bifida (spine) (causes LL weakness/paralysis; bladder and bowel dysfunction, UTIs; clubbed feet, hip dilocation)

(brain) anencephaly (born w/o parts of brain/skull cause forebrain development got disrupted);
encephalocele (outpouching of membrane into skin)

  • forebrain anomalies
    polymicrogyria: seizures, mental retardation, hemi/quad paresis (paralysis)
    microencephaly: difficulty swallowing, seizures, psychomotor retardation
  • post fossa anomalies
    1. Arnold chiari (chirai type 2) malformation*: displacement of cerebellar tonsils through foramen magnum -> headaches, muscle weakness, fatigue
    severe: brainstem damage
    2. dandy walker syndrome: enlarged post fossa -> enlargement of skull
    raised ICP - vomitting, irritability
    poor coordination of eye and facial muscles
    affects females & infants (slow motor development) more
40
Q

Alzheimer’s disease

A

pathology:
mutation of chromosome 21 (amyloid precursor protein) -> production and deposition of beta amyloid peptides (in hippocampus, amygdala, neocortex) -> neuronal damage
frontal and parietal lobes affected
+ brain atrophy

micro: amyloid deposits; intracellular tau protein

41
Q

parkinson disease

A
pathology:
loss of nerve cells from midbrain -> reduced dopamine
lewy bodies (contain a-synuclein gene)

gross: substantia nigra (basal ganglia) becomes pale

42
Q

metabolic/toxic diseases

A
vit B1 (thiamine) def = wernicke encephalopathy:
  thiamine def -> damage medial thalamus and mammillary bodies -> cerebral atrophy 
vit B12 def = degeneration of spinal cord

storage diseases: niemann pick, tay-sachs disease

hepatic encephalopathy

CO poisoning: diffuse cortical necrosis

43
Q

alcohol affecting CNS

A
  • fetal alcohol syndrome: growth retardation, cerebral malformation of baby
  • acute intoxication -> respiratory depression
  • chronic:
    cerebral cortical atrophy
    cerebellar atrophy
    wernicke encephalopathy (cause of b1 def) -> korsakoff psychosis