neuro diseases/syndromes Flashcards

1
Q

where is the hippocampus

A

medial temporal lobe

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2
Q

types of declarative and non-declarative memory

A

declarative - semantic and episodic

non-declarative - skills, priming, basic associative learning, reflexes

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3
Q

what specific ion channel is involved in learning and memory

A

NR1 - key glutamate receptor

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4
Q

what is catatonia

A

motor immobility as evidenced by catalepsy or stupor

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5
Q

what do psychiatric illnesses affect

A

neurons, astrocytes and microglia leading to altered molecular pathways

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6
Q

epilepsy is characterized by…

A

recurrent unprovoked epileptic seizures

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7
Q

what causes seizures

A

paroxysmal excessive, synchronous, abnormal firing of populations of neurons in the brain - where it disrupts normal neuronal processing

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8
Q

why does someone get epilepsy

A
  • genetic = inherited the predisposition
  • structural/metabolic = something has happened to the brain as a result of injury
  • unknown
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9
Q

what defines a partial (focal) seizure

A

arise in a limited number of cortical neurons within one hemisphere

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10
Q

what defines a generalized seizure

A

appear to arise simultaneously in both hemispheres

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11
Q

which cause of epilepsy usually responds well to medication and which type doesnt

A

responds well - genetic

doesnt respond well - structural

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12
Q

what causes the abnormal firing of neurons that causes seizures

A

a disturbance in the balance between inhibition and excitation of cortical neurons and neuronal networks (either increases or decreases in neuronal inhibition or excitation)

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13
Q

what can cause the disturbance in the excitatory-inhibitory balance causing seizures

A
  • loss of inhibitory neurons
  • gain of excitatory neurons
  • aberrhant sprouting
  • change in intrinsic neuronal cellular excitability –> change NT in the circuit
  • alterations in synpatic transmission
  • alterations in the extra-neuronal environment (glia)
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14
Q

what is the typical characteristic pathological pattern of medial temporal sclerosis

A
  • cell loss in CA1, CA3 and dentate hilus regions
  • mossy fibre sprouting
  • glosis
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15
Q

why is medial temporal sclerosis so important

A
  • commonest pathology in adults with partial epilepsy
  • most refractory to medial drug therapy
  • good prognosis with epilepsy surgery
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16
Q

what are the MRI signs of focal cortical dysplasia

A
  • focal thickening of the cerebral cortex
  • blurring of the grey/white interface
  • gyral abnormalities
  • may be associated with a region of increased T2 signal
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17
Q

what causes preiventricular nodular heterotopia

A

abnormal neuronal migration

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18
Q

what is the most common type of tumour to cause seizures

A

gliomas

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19
Q

what type of vascular lesions can cause seizures

A

cavernomas

arteriovenous malformations

20
Q

what is focal encephalomalacia

A

focal lesion resulting from previous destructive insule

21
Q

definition of cellular senescence

A

cells lose the ability to divide

22
Q

what is organismal senscence

A

the ageing of whole organism

23
Q

what is the difference between “successful” and “usual” ageing

A

successful - changes due solely to the ageing process, UNAFFECTED BY DISEASE, ENVIRONMENTAL OR LIFESTYLE FACTORS

usual - changes associated with THE COMBINED EFFECTS of the ageing process, disease and adverse environment and lifestyle factors

24
Q

definition of frailty

A

diminished ability to carry out the important practical and social activities of daily living

25
Q

why do we want to prevent someone becoming frail

A

they are unable to integrate responses in the face of stress and therefore are at risk for adverse health outcomes such as geriatric syndromes

26
Q

what are the “biomarkers” of frailty

A

associated with a low grade chronic activation of the immune system

  • cytokines (IL-6 and TNF-alpha)
  • CRP
27
Q

what is sarcopenia

A

ageing skeletal muscle due to altered CNS and PNS innervation, altered hormonal status, inflammatory effects and altered caloric, protein intake and disuse

28
Q

when does the decline in skeletal muscle start to occur

A

begins in the 3rd decade

29
Q

which muscle fibres are more affected by sarcopenia

A

type 2 fibres

proximal and distal muscles affected to the same amount

30
Q

what are the clinical signs of frailty

A
slowness
weakness
weight loss
low activity
fatigue
31
Q

What are the Fried’s criteria for frailty

A
  • unintentional weight loss
  • weakness
  • exhaustion
  • slow walking speed
  • low physical activity
    (pre frail = 1-2, frail = 3 or more)
32
Q

definition of co-morbility

A

concurrent presence of 2 or more chronic diseases

33
Q

definition of a disability

A

physical or mental impairment that limits 1 or more ADL

34
Q

what are the geriatric syndromes

A
incontinence
immobility
impaired balance
impaired cognition
iatrogenic illness
35
Q

What are the protein products leading to Alzeihmer’s disease

A

alpha-beta amyloid

Neurofibrillary tangles

36
Q

Which chromosomes are involved in early onset AD

A

21, 14 and 1

37
Q

sporadic mutation of which chromosome leads to sporadic AD

A

19

38
Q

what are the 4 types of AD

A

Amnestic (temporal)
Visuospatial (R>L)
Aphasic (L>R)
Frontal

39
Q

What is the precursor protein to amyloid plaques and how does it become amyloid plaques

A

APP
Cleaved by beta and gamma secretases which changes the conformation into beta sheets which is insoluble. This screws up neuronal signalling and ultimately leads to degredation

40
Q

How does gamma secretase cleave APP

A

Introduces a water molecule into the transmembrane domain

41
Q

Where are amyloid plaques deposited

A

On the membrane of extra and Intra synaptic sites

42
Q

How do we currently diagnose AD

A

Measure the Amyloid peptide in the CSF and do a PET scan

43
Q

What is the approximate time frame from normal to full blown AD

A

30 years

44
Q

What is Creutzfeldt-Jakob disease

A

A rapidly progressing disease due to an infectious toxic protein in the brain

45
Q

What causes mad cow disease

A

Abnormal Prion protein that is resistant to proteolytic degredation

46
Q

Why are people not allowed to give blood if you have lived in Britain

A

Because mad cow disease goes into the blood and therefore can be passed on

47
Q

Which protein is associated with Parkinson’s disease

A

Alpha-synuclein