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Yr 3 MedEd > Chronic Neuro > Flashcards

Chronic Neuro Flashcards

1
Q

What is multiple sclerosis?
Give 4 symptoms of multiple sclerosis.
What 2 populations is higher prevalence found in?

A
  • Multifocal, demyelinating disease of CNS. Oligodendroglial and axonal pathology.
  • optic neuritis
  • motor weakness
  • Sensory disturbances
  • Fatigue
    Can have seizures and psychiatric disturbances.

Higher prevalence in white people of Nordic origin.
Latitude effect; prevalent at higher altitudes.

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2
Q

Give 2 fundoscopy findings in multiple sclerosis.

Give 2 other examination findings in MS.

A
  • Retro-bulbar neuritis. Optic neuritis with papilitis.
  • Intention tremor, ataxia, dysmetria.
  • Lhermitte’s sign: electric shock radiating down back triggered by neck flexion.
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3
Q

Give 5 risk factors for MS.

A
  • Nordic/ white origin, high altitude
  • Female
  • Low vit D areas
  • Other autoimmune condition
  • HLA DLRB1*15
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4
Q

What are the 3 types of MS and which is the most common?

A
  • Relapsing-remitting (RRMS), most common 80-85%.
  • Secondary progressive (SPMS). Can follow RRMS.
  • Primary progressive (PPMS).
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5
Q

Give 3 key features of MS diagnosis.

What is important imaging investigation for MS?

A
  • Absence of alternative diagnosis
  • Dissemination in time
  • Dissemination in space.
  • MRI is important. During acute attack, inflammation causes breakdown of BBB, allowing GAD (contrast agent) to penetrate CNS. GAD enhances active lesion.
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6
Q

What is positive CSF test for MS?

A

Oligoclonal bands not matched to serum.

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7
Q

Give 4 symptoms of Myasthenia Gravis.

A
  • Ptosis
  • Diplopia
  • Dysarthria
  • Dysphagia
    +/- SOB.
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8
Q

What occurs at the neuromuscular junction in myasthenia gravis?
What may blood antibody tests find?

Give two associated conditions in Mysathenia gravis.
What does EMG demonstrate in MG.

A
  • Antibodies target receptors for Acetylcholine. Prevents contraction.
  • Anti-ach, anti-MuSK or seronegative.
  • Thymic hyperplasia
  • Thymoma
  • Action potentials gradually decrease over time.
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9
Q

What is Lambert-Eaton myasthenic syndrome?
Give 4 ways LEMS may present.

Give 2 clinical signs.

A
  • Weakness where muscles improve with use. Antibodies to voltage gated calcium channel preventing pre-synaptic release of Ach.
  • Difficulty walking
  • Weakness in upper arms and shoulders.
  • Similar symptoms to myasthenia gravis.
  • Autonomic: dry mouth, constipation, incontinence.

Hyporeflexia and muscle function improvement following use

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10
Q

Give 2 conditions associated with Lambert Eaton Myasthenia syndrome.

Give important investigation for LEMS patient.

A
  • Small cell lung cancer: cancer producing antibodies to VGCC or collateral damage in auto-immune attack against cancer.
  • Autoimmune disease

LEMS patient should have CT/ MRI chest for cancer detection.

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11
Q

Give 3 symptoms of motor neurone disease (aka ALS, Lou Gehrig’s disease, Charcot’s disease)

Which 3 functions are spared?

A
  • Progressive muscle weakness
  • Dysphagia
  • Shortness of breath.
  • Sparing of oculomotor, sensory and autonomic function.
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12
Q 
Does MND show UMN or LMN signs?
Identify the presence of the following signs in Upper and Lower motor neuron lesions:
- Weakness
- Atrophy/ wasting
- Reflexes
- Fasciculations
- Tone
- Extensor response
A

MND shows both.

  • Weakness: both
  • Atrophy: LMN
  • Reflexes: increased in UMN
  • Fasciculations: LMN
  • Tone: Increased in UMN
  • Extensor response: present in UMN, absent in LMN.
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13
Q

Give two locations of muscle atrophy in MND.

What protein marker is present in motor neurones in MND?
What is seen on EMG for motor neurone disease?

A
  • Thenar muscle wasting
  • Tongue muscle wasting- bulbar onset i.e. medulla oblongata onset.
  • Ubiquitin. Ubiquinated proteins are marked for degradation by proteasome.
  • Fibirillations and fasciculations
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14
Q

What is the triad of symptoms for Parkinsonism?

What histopathology finding is specific for Parkinson’s disease?

A
  • Bradykinesia
  • Rigidity
  • Resting tremor.
  • Lack of dopaminergic neurones in substantia nigra
    On naked eye examination, lack of neuromelanin due to inactivity- slide 48.
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15
Q

What is the pathophysiology of Parkinson’s?

Give 2 risk factors.

A
  • Alpha-synuclein deposition in dopaminergic neurones (in nitgrostriatal pathway) due to misfolding of the protein.
  • Packed in cell as Lewy bodies

Male gender and living in countryside.

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16
Q

When is the relative onset of Parkinson’s dementia?
How does Parkinson’s differ from Lewy body dementia?
Give 2 features of Parkinson’s dementia.

A
  • Dementia occurs many years after onset of motor symptoms.
  • Lewy body dementia develops cognitive symptoms without motor symptoms.
  • Visuospatial dysfunction- can’t draw a clock
  • Hallucinations: of little people or animals.
17
Q

What are the 5 As of alzheimer’s dementia.

What happens to the brain shape in late stage Alzheimers?

A
  • Amnesia
  • Agnosia
  • Anomia
  • Aphasia
  • Apraxia
  • Global cortical atrophy.
18
Q

What is the Alzheimer’s pathophysiology relating to Amyloid plaques?

A
  • Amyloid precursor protein broken down by Beta and Gamma secretase (instead of alpha and gamma secretase) producing Amyloid Beta, resistant to degradation, accumulates as amyloid plaques outside cell. Disrupts neuronal communication.
19
Q

What is the Alzheimers pathophysiology relating to Tau tangles

A
  • Tau protein (part of neuronal microtubule) phosphorylated by Amyloid Beta. Tau dissociates from microtubule.
  • Causes Tau tangles and a weakened microfilament.
  • Reduction in neuronal function and apoptosis.
  • Reduce in cortical Ach.
20
Q

Give 3 genetic risk factors for Alzheimers

Give 4 other risk factors for Alzheimers

A
  • APOE (Apolipoprotein E): cholesterol transport protein involved in Beta amyloid clearance.
  • Autosomal dominant Alzheimer disease
  • Down syndrome.
  • Trauma: acute head injury can cause beta amyloid deposits.
  • Female
  • Vascular risk factors: DM, BP, dyslipidaemia
  • age >65: prevalence doubles every 5 years from 65 to 85
21
Q

What is seen in CSF in Alzheimer’s?

What is required for definitive diagnosis.

A
  • Tau and beta amyloid in CSF

- Brain tissue histology required for definitive diagnosis.

22
Q

Describe 2 features of Vascular dementia presentation.

A
  • Sudden onset

- Stepwise deterioration.

23
Q

In Fronto-temporal dementia (Pick’s disease) give 4 presenting features.
What are Pick bodies?

A
  • Personality change
  • Overeating, preference for sweet foods
  • Emotional blunting.
  • Disinhibition.

Pick bodies are hyperphosphorylated tau protein.

24
Q

What is the CAN triangle of Wernicke’s encephalopathy.
What is it caused by?
Give 2 signs of Wernicke’s.

A
  • Confusion
  • Ataxia
  • Nystagmus and other eye signs
  • Lack of Thiamine (Vit B1).
  • Alcoholism
  • Malnourished
25
Q

Give 2 blood investigations with Wernicke’s.
Give 2 other investigations.
What is immediate medication for Wernicke’s?

A
  • Serum albumin to measure liver and renal function
  • Serum Vit B1.
  • ECG, CT scan
  • Pabrinex (B1)
26
Q

Give 3 characteristic features of Korsakoff’s.

A
  • Chronic
  • Alert
  • Amnesia (anterograde and retrograde) and confabulation.
27
Q

Give 4 motor symptoms of Huntington’s disease

Give 4 cognitive symptoms of Huntington’s disease.

A

Motor:

  • Athetosis- writhing movements of hands
  • Chorea
  • Ataxia
  • Dysphagia

Cognitive:

  • Lack of concentration
  • Depression
  • Dementia
  • Personality changes, aggression.
28
Q

Which gene is mutated in Huntington’s disease?
What is the inheritance pattern?
What genetic change is seen in nucleotide sequence?
What part of the brain is the degeneration located.

A
  • Huntingtin gene (HTT)
  • Autosomal dominant. A small number of cases are sporadic.
  • CAG trinucleotide repeats. Forms abnormal protein.
  • Death of spiny GABAergic (inhibitory) neurons in Caudate and Putamen.
29
Q

What investigation is needed for Huntington’s disease?

What is a positive result?

A
  • CAG repeat testing.

- More than 40 repeats is positive result, individual will develop Huntington’s.

Yr 3 MedEd (31 decks)

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