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Neurology > Neurodegenerative conditions > Flashcards

Neurodegenerative conditions Flashcards

1
Q

What are the symptoms of AD?

A

Progressive, global impairment

  • cognition
  • visuo-spatial skills
  • memory
  • verbal skills
  • planning

Later: mood changes, depression, psychosis, behaviour changes, agnosis

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2
Q

What are the symptoms of vascular dementia?

A

Cumulative effect of many small strokes
Sudden onset
Evidence of arteriopathy - high BP, past strokes, focal neurology

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3
Q

What are the symptoms of Lewy Body dementia?

A

Fluctuating cognitive state
Hallucinations (visual)
Parkisonism

Don’t give anti-psychotics

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4
Q

What are the symptoms of fronto-temporal dementia?

A

Behaviour/personality change
- disinhibition, hyperorality, emotional unconcern

Memory and spatial orientation maintained until later stages

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5
Q

What is Pick’s disease?

A

Fronto-temporal dementia with Pick inclusion bodies (spherical clusters of tau laden neurons)

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6
Q

What is the pathology of dementia/AD?

A

APOE - main gene

  • beta amyloid plaques
  • tau tangles
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7
Q

What is the medical management of dementia/AD?

A

AChE-I

  • Donepexil
  • Rivastigmine
  • Galantamine

Anti-glutamatergic (NMDA antagonist)
- Memantine

Anti-psychotics

  • give for psychosis/extreme agitation
  • DO NOT GIVE: PD, AD, Lewy body, vascular

Also refer to a memory service
BP control

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8
Q

What is the diagnostic triad of Parkinson’s?

A

Extrapyrimidal:

  • tremor (at rest, ‘pill-rolling’)
  • hypertonia (cogwheel rigidity)
  • bradykinesia (slow movement initiation, decreasing movement amplitude)
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9
Q

What are the prodromal symptoms of PD?

A

Depression

Agnosia - loss of sense of smell

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10
Q

What are the symptoms of PD?

A 
Autonomic dysfunction 
- postural hypoTN, constipation, urinary frequency
Sleep disturbance 
Loss of smell 
Neuropsychiatric dysfunction 
- depression
- dementia
-  psychosis
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11
Q

What are some PD plus syndromes?

A

Progressive supernuclear palsy
- postural instability, vertical gaze palsy, falls, speech and swallowing problems

Multiple system atrophy
- early autonomic features, postural hypoTN, cerebellar and pyrimidal signs, rigidity > tremor

Cortico-basal degeneration
- akinetic rigidity in one limb, cortical sensory loss, apraxia

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12
Q

How is PD managed?

A

Levodopa (in combination with a DDC)
- co-beneldopa
- co-careldopa
Can cause dyskinesia, dystonia, vomiting

Dopamine agonists

  • Ropinirole, pramipexole
  • Rotigotine patches
  • Bromocriptine/cabergoline less favoured
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13
Q

What medical management can be added in later stage PD?

A

Amantadine (for drug induced dyskinesias)

Anticholinergics - benzhexol
MAO-B I - rasagiline, selegiline
COMT I - entacapone, tolcapone

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14
Q

What is the pathology of MS?

A

Inflammatory plaques of demyelination in the CNS

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15
Q

What are the symptoms of MS?

A

UL optic neuritis - pain when moving eyes and loss of central vision, diplopia, hemianopia
Pins and needles, loss of vibration sense, trigeminal neuralgia
Spastic weakness
ED, anorgasmia, urinary retention/incontinence
Trunk and limb ataxia, intention tremor, scanning speech
Cognitive decline

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16
Q

What might investigations show in MS?

A

MRI - demyelination
CSF - oligoclonal bands of IgG (not present in serum)
Delayed evoked potentials

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17
Q

How is MS managed?

A

Disease modifying drugs (relapsing remitting) - di-methyl fumarate (relapsing remitting), alemtuzumab (T cells)
Methylprednisolone (for a relapse)
Spasticity - gabapentin, baclofen
Tremor - botulinum
Urinary urgency/frequency - self-catheterisation, tolteridine
Fatigue - amantadine, CBT, exercise

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18
Q

What is the pathology of MND?

A

Loss of neurons in motor cortex, CN nuclei and anterior horn cells

NO sensory/sphincter problems

19
Q

What are patterns of MND?

A

ALS - loss in motor cortex and anterior horns (UMN, LMN)
Bulbar palsy - loss in CN IX - XII (LMN in tongue)
Muscular atrophy - loss in anterior horn (LMN)
Primary lateral sclerosis - loss in motor cortex (UMN)

20
Q

What are the symptoms of MND?

A 
Stumbling, spastic gait
Foot drop 
Proximal myopathy 
Weak grip 
Shoulder abduction (hard to wash hair)

UMN and LMN signs

21
Q

What is the pathology of Myasthenia gravis?

A

Abs to nicotinic ACh receptors - post synaptic side

B and T cells involved

22
Q

What are the symptoms of MG?

A

Increasing/relapsing muscular fatigue
- extraocular -> bulbar -> face -> neck -> limbs -> trunk
Symptoms worsen with exercise, gent, pregnancy, tetraciclines, quinine, b-blockers

Ptosis, diplopia, myasthenic snarl, eye ‘peek’ sign, voice fading

Normal reflexes

23
Q

What do tests for MG show?

A

Abs - anti-AChR, MUSK abs

EMG - decremental muscle response to repeated stimulation

24
Q

How is MG managed?

A

Anticholinesterase - pyridostigmine
Prednisolone (relapses)
Osteoporosis prophylaxis

Thymectomy (even without thymoma)

25
Q

How is a myasthenic crisis managed?

A

Weakness of respiratory muscles

Treat cause, plasmapheresis (remove Ab), IVIg

26
Q

What is the pathology of Lambert Eaton?

A

AI - Ab to voltage-gated Ca channels on presynaptic membrane
Paraneoplastic - small cell lung cancer

27
Q

What are the symptoms of Lambert Eaton?

A 
Gait difficulty (before eye signs unlike MG)
Autonomic - dry mouth, constripation, impotence
Hyporeflexia and weakness - improves after exercise

Diplopia and respiratory muscle involvement are rare

28
Q

How is Lambert Eaton treated?

A

Pyridostigmine
3,4-diaminopyridine
IVIg

29
Q

What is the pathology of GBS?

A

Acute inflammatory demyelinating polyneuropathy

Occurs a few weeks after infection - Abs attack nerves

30
Q

What are the symptoms of GBS?

A

Rapid onset, proximal muscle involvement first, symmetrical

Sweating
High pulse
BP change
Arrythmias
CN involvement 
Mild sensory symptoms
31
Q

How is GBS managed?

A

IV Ig
Plasma exchange

Do not give steroids

Complete paralysis can still have complete recovery

32
Q

What is the pathology of Duchenne’s/Becker’s?

A

X-linked recessive

Dystrophin gene

33
Q

What are the symptoms of Duchenne’s/Becker’s?

A

Onset at 4yo
Clumsy walking
Difficulty standing
Respiratory failure

Later onset and better outcomes in Becker’s
Very high CK levels

34
Q

What is the pathology of myotonic dystrophy?

A

Autosomal dominant

Trinucleotide repeat expansions

35
Q

What are the symptoms of myotonic dystrophy?

A

Hand/foot drop
Weak SCM
Myotonia
Facial weakness and wasting

36
Q

What is the trinucleotide repeat in Huntingdon’s?

A

CAG

37
Q

What are the symptoms of Huntingdon’s?

A 
Middle age onset 
Depression 
Chorea 
Aggression 
Athetosis (abnormal muscle contraction)
38
Q

What are the signs of neurofibromatosis 1 (NF1)

A 
Cafe au lait spots
Freckles 
Dermal neurofibromas
Nodular neurofibromas
Lisch nodules
39
Q

What are the signs of NF2?

A

Bilateral acoustic neuromas (vestibular schwannomas)
Sensorineural hearing loss
Tinnitus
Vertigo

40
Q

What is the trinucleotide repeat in Friedrich’s ataxia?

A

GAA in FXN gene

41
Q

What is a Ddx of dementia?

A

Normal pressure hydrocephalus

  • wet (incontinent)
  • wacky (confused)
  • wobbly
42
Q

What is the trinucleotide repeat in Friedrich ataxia?

A

GAA

43
Q

What is the trinucleotide repeat in myotonic dystrophy?

A

CTG

44
Q

What is the trinucleotide repeat in fragile X syndrome?

A

CGG

Neurology (9 decks)

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