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OMS - 2 Neuro Final > CIS II NPH and PSP > Flashcards

CIS II NPH and PSP Flashcards

1
Q

PSP is due to aggregation of ______

A

4R tau (3 isoforms that have 4 microtubule binding domains due to alternative splicings of 2,3, & 10 → 6 isoforms

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2
Q

Tau is encoded for by _______ gene.

A

MAPT

(etiology of PSP)

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3
Q

If MAPT/mRNA includes _____ it is a 4R tau → PSP

A

E10

(4 microtubule binding domains)

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4
Q

_____ hapylotype on chromo. 17q is over-expressed in patients w/PSP

A

H1

(this increases 4R tau→ increasing risk of aggregation)

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5
Q

Increase H1 haplotype leads to increased _______ expression.

A

exon 10 → 4R tau

(PSP)

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6
Q

4R tau increases risk of ______ aggregation

A

tau

(increased risk of PSP)

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7
Q

Environmental influences in causing PSP

A

toxins in Annona fruit

(fruit consumed in Caribbean island Guadeloupe)

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8
Q

Annona fruits are toxic due to ______ (2)

A
  1. inhibits mitochondrial complex 1
  2. increase 4R tau mRNA in neurons
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9
Q

Why is tau aggregation bad (2)?

A
  1. it can’t stabilize microtubules → impairs axonal transport & metabolism
  2. activates UPR → apoptosis

(this tau pathology can spread from one neuron to another like prion diz)

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10
Q

The globose neurofibrillary tangles consist of ______, which gives the neurons an oval to round appearance.

A

whorled filaments composed of aggregated 4R tau

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11
Q
A

globose neurofibrillary tangles (PSP)

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12
Q

4R tau aggregates in neurons as well as ______

A

glial cells (i.e. astrocytes)

(leads to globos neurofibrillary tangles)

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13
Q

Tufted astrocytes found in the gray matter are especially characteristic of _______.

A

PSP

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14
Q
A

tufted astrocytes

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15
Q
A

globose neurofibrillary tangles - tau positive

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16
Q

PSP has atrophy of ______ (4).

A
  1. midbrain
  2. globus pallidus
  3. superior cerebellar peduncles
  4. cerebral cortex

(degreased pigmentation of substantia nigra & locus coeruleus → parkinsonian sx)

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17
Q

What is a very characteristic feature of PSP (histopathology)

A

tufted astrocytes

(as well as globus neurofibrillary tangles)

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18
Q

_____% of patients have normal pressure hydrocephalus

A

1.4% patients > 65 yo

(insidious onset usually > 40)

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19
Q

2 Associated diz w/Normal pressure hydrocephalus

A
  1. cerebrovascular diz
  2. ALZ
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20
Q

Secondary causes of Normal pressure hydrocephalus (3)

A
  1. SAH
  2. TBI
  3. Meningitis

(in this order)

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21
Q

What other conditions have

A

0000

22
Q

______ is NOT attributable ventricular enlargement seen in normal pressure hydrocephalus

A

cerebral atrophy

23
Q

Tx for normal pressure hydrocephalus (2)

A
  1. shunt
  2. serial LPs

(ventriculoperitoneal or lumboperitoneal)

24
Q

Non-communicating hydrocephalus

A

obstruction of CSF flow through ventricles → CSF pressure increased

(normal pressure hydrocephalus has too much CSF)

25
Q

3 conditions that cause non-communicatting hydrocephalus

A
  1. intraventricular cyst
  2. tumor
  3. ependymal granulation
26
Q

Communicating hydrocephalus is due to _______.

A

impaired resorption of CSF

(CSF pressure is normal)

27
Q

_______% of NPH are idiopathic

A

50

28
Q

_______ act like one-way valve for CSF

A

arachnoid villi (aka granulations)

29
Q

4 secondary causes of NPH

A
  1. subarachnoid hemorrhage
  2. meningitis
  3. TBI
  4. CNS tumor
30
Q

As a subarachnoid hemorrhage heals it can lead to normal pressure hydrocephalus due to

A

→ fibrosis → obliterates arachnoid granulations → impairs CSF reabsorption

(similar situation w/meningitis & TBI)

31
Q

hyperproteinarachia

A

increased protein in CSF

(seen in CNS tumors due to release; this clogs the subarachnoid granulations → NPH)

32
Q

3 idiopathic NPH causes

A
  1. increased central venous pressure
  2. arteriosclerosis
  3. congenital hydrocephalus
33
Q

how does increased central venous pressure lead to idiopathic NPH

A

central vein pressure increases pressure up to the dural venous sinuses → impaired CSF reabsorption

34
Q

How does arteriosclerosis lead to NPH?

A

chronic ischemia of leptomeninges → leptomeningeal fibrosis

35
Q

4 causes of central venous pressure increase

A
  1. OSA
  2. heart failure
  3. pulmonary disease
  4. incompetent internal jugular valves

(increased pressure in the R side of heart)

36
Q

decompensated congenital hydrocephalus leads to _______ later in life

A

idiopathic normal pressure hydrocephalus due to cranial sutures not closing

37
Q

NPH histopathologic findings include (3)

A
  1. arteriosclerosis w/scattered micro-infarcts in periventricular white matter
  2. demyelination in subcortical white matter
  3. leptomeningeal fibrosis (due to ischemia; aka arachnoid fibrosis)
38
Q

MC degenerative forms of atypical Parkinsonism

A

progressive supranuclear palsy (PSP)

39
Q

Population affected by PSP

A

Male > 62 yo

(1 in 100,000)

40
Q

Hallmark sign of progressive supranuclear palsy (PSP)

A

supranuclear opthalmoparesis: vertical gaze problems

41
Q

Prognosis of PSP

A

death w/in 6-12 years

42
Q

Sx of PSP (5)

(other than hallmark: supranuclear opthalmoparesis - vertical gaze problems)

A
  1. dysphagia
  2. dysarthria
  3. pseudobulbar palsy
  4. abnormal postural reflexes
  5. frontal cognitive abnormalities
43
Q

PSP treatment

A

symptom management

(PT/OT, dietician, cognitive therapy)

44
Q

PSP finding of hummingbird sign indicates ______

A

midbrain atrophy

45
Q

How can you tell PSP apart from Parkinson Disease (2)?

(both have rigidity)

A
  1. resting tremor rare in PSP
  2. poor response to L-DOPA
46
Q

gold standard for diagnosing PSP

A

neuropathology

47
Q

Classic triad of normal pressure hydrocephalus

A
  1. apraxia (“magnetic gait”)
  2. urinary incontinence (indifference)
  3. cognitive problems (frontal & subcortical)

(similar to Parkinson)

48
Q

both Parkinson disease and normal pressure hydrocephalus have ______ (3 symptoms).

A
  1. gait problems
  2. hypokinesia
  3. extrapyramidal symptoms
49
Q

What helps to distinguish between PSP, normal pressure hydrocephalus and Parkinson disease?

A

L-DOPA only helps Parkinson Disease

50
Q
A

Left: Coronal section of normal brain

Right: Coronal section of brain from patient with Normal Pressure Hydrocephalus

51
Q
A

Left: Section of brain showing normal leptomeninges (arrow) and underlying cerebral cortex.

Right: Leptomeningeal fibrosis: thickening of the pia-arachnoid (arrow), exhibiting dense fibrosis with a scattering of lymphocytes. The subarachnoid space has been obliterated. Immediately beneath the pia is a broad band of glial fibers (*), and the underlying molecular layer shows reactive gliosis.

OMS - 2 Neuro Final (36 decks)

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