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Phase 3a - Geriatrics and Neurology > Neurodegenerative Diseases + Movement Disorders > Flashcards

Neurodegenerative Diseases + Movement Disorders Flashcards

1
Q

Sx of parkinsonism, including:
* Triad
* Non-motor features

A

Triad:
1. Bradykinesia
2. Asymmetric “pill-rolling” tremor
3. Lead pipe rigidity

Non-motor:
* Olfactory loss
* Sleep disorders
* Psychiatric (depression, anxiety, etc)
* Autonomic dysfunction - constipation, postural hypotension

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2
Q

Ix for Parkinson’s?

A
  • Clinical diagnosis: +ve response to tx trials - failure to respond to 1-1.5g levodopa excludes diagnosis of parkinsonism
  • Other: MRI head
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3
Q

Tx of parkinsons

A

1st line: Levodopa + decarboxylase inhibitor (cobeneldopa)
S.E. hypotension, restlessness, GI upset

2nd line:
* COMT inhibitirs
* DA agonists

Levodopa - dopamine replacement agent

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4
Q

Complications of Parkinson’s?

A

Depression and anxiety

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5
Q

What is benign essential tremor?

A

Cdtn associated with older age characterised by a fine tremor affecting all voluntary muscles

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6
Q

Sx of essential tremor?

A

Fine tremor (6-12 Hz)
Symmetrical
More prominent with voluntary movement
Worse when tired, stressed or after caffeine
Improved by alcohol
Absent during sleep

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7
Q

Mx of essential tremor?

A

Meds used to improve sx:
* Propranolol
* Primidone

Primidone is a barbiturate anti-epileptic medication

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8
Q

What is MS?

A

Chronic autoimmune disease ivolving CNS which is marked by degenration of the insulating covers of nerve cells in the brain and spinal cord leading to demyelination and axonal loss.

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9
Q

Causes of MS

A

Unknown but may be influenced by:
EBV
Low Vit D
Smoking
Obesity

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10
Q

Sx of multiple sclerosis

A

Sx progress over more than 24hrs. Then last days to wks and then improve:
* Sensory disease (numbness, paraesthesia, trigeminal neuralgia, Lhermitte’s sign)
* Focal weakness (urinary incontinence, horner syndrome, limb paralysis)
* Optic neuritis (loss of central vision + painful eye movement)
* Internuclear opthalmoplegia (diplopia and nystagmus)
* Subacute cerbellar ataxia
* Uhtoff’s phenomenon

  • Optic neuritis = demyelination of the optic nerve
  • Lhermitte’s sign describes an electric shock sensation that travels down the spine and into the limbs when flexing the neck.
  • Uhtoff’s - worsening of sx following inc. in temp i.e. hot bath
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11
Q

What are the different patterns of MS?

A
  1. Relapsing-remitting: MC - episodic flare ups (days/mths) followed by remission periods
  2. Secondary progressive: Starts as relapsing-remitting then progresses to no periods of remission + worse sx
  3. Primary progressive: Sx worse from onset with no periods of remission
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12
Q

What are the imaging findings for MS and what are the results of a lumbar puncture?

A

MRI brain + spine - Dawson’s fingers (periventricular plaques)
Lumbar puncture - oligoclonal bands in CSF

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13
Q

Which criteria is used for diagnosis of MS?

A

McDonald criteria

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14
Q

Describe acute and chronic tx of MS

A

Acute:
* 1st line - IV methylprednisolone (reduce relapse duration)
* Plasma exchange (if does not respond to steroids)

Chronic:
* 1st line - Beta-interferon (DMARD) and glatiramer
* Biologics (natalizumab)

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15
Q

What is Huntington’s disease?

A

Genetic disorder that causes progressive breakdown of nerve cells in the brain. It is a trinucleotide repeat disorder.

Sx usually begin aged 30-50 yrs.

Trinucleotide repeats refer to repetitions of a sequence of three nucleotides (e.g., CAGCAGCAGCAGCAG).

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16
Q

What kind of genetic inheritance is Huntington’s disease? Where does the mutation occur and on which gene?

A

Autosomal dominant. Genetic mutation in HTT (huntingtin) gene on chromosome 4.

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17
Q

What is meant by anticipation in relation to Huntington’s disease?

A

Huntington’s displays something called genetic anticipation. Anticipation is a feature of trinucleotide repeat disorders, where successive generations have more repeats in the gene, resulting in:

Earlier age of onset
Increased severity of disease

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18
Q

Sx of Huntington’s disease?

A

Triad:
1. Choreoathetosis - involuntary, random, irregular and abnormal body movements, dystonia, rigidity, dysphagia and dysarthria
2. Cognitive impairment - dementia and memory problems
3. Psychiatric abnormalities - depression, irritability and sometimes psychosis

Choreoathetosis is a movement disorder that causes involuntary twitching or writhing.

19
Q

Ix for Huntington’s?

A

MRI/CT brain - striatial volume loss
Genetic testing

20
Q

Mx of Huntington’s and prognosis?

A
  • Chorea mx - tetrabenazine
  • Depression mx - SSRIs
  • Extensive counselling

Prognosis = Poor. Life expectance is around 10-20 years after the onset of symptoms. Death is often due to aspiration pneumonia. Suicide is also a common cause of death.

21
Q

What is Motor Neuron Disease (MND)?

A

A group of progressive neurological disorders that destory motor neurons (the cells that control voluntary muscle activity such as speaking, walking, breathing and swallowing.

22
Q

Sx of upper and lower motor neuron signs

A

UMN:
* Inc. tone or spasticity
* Brisk reflexes (hyperreflexia)
* Upgoing plantar reflex (+ve babinski)

LMN:
* Muscle wasting
* Reduced tone
* Fasiculations (twitches in the muscles)
* Reduced reflexes
* -ve babinski

23
Q

Ix for MND?

A

Clinical dx but can do:
* TFTs
* Protein electrophoresis
* MRI brain and spinal cord

24
Q

Tx for MND and prognosis?

A

Riluzole - extends life expectancy by 3 mths.

The prognosis for motor neuron disease is generally poor, with life expectancy from diagnosis usually less than 5 years. Most patients succumb to the respiratory complications of the disease.

25
Q

What is cerbellar dysfunction syndrome?

A

A cluster of clinical manifestations resulting from impaired cerbellum function - responsible for maintaining balance and movement coordination.

26
Q

What are the causes of cerebllar dysfunction?

Hint: mnemonic VITAMIN C

A

Vascular: Stroke
Infectious: Lyme disease, Cerebellar Abscess
Inflammatory: Multiple sclerosis.
Traumatic: Trauma to the posterior fossa.
Metabolic: Alcoholism.
Iatrogenic: Drugs (phenytoin and carbamazepine)
Neoplastic: Primary and secondary tumours
Congenital/Hereditary: Friedrich’s ataxia, and the spinocerebellar ataxias.

27
Q

Sx of cerebellar dysfunction?

Hint: mnemonic DANISH

A

Dysdiadochokinesia
Ataxia: A broad-based, unsteady gait
Nystagmus: Involuntary eye movements
Intention tremor: Finger-nose test
Slurred speech.
Hypotonia.

28
Q

Ix of cerebellar dysfunction

A

Neuroimaging
Lumbar puncture
Genetic testing

29
Q

Tx of cerebellar dysfunction

A

Tx of underlying cx + lifestyle modifications i.e. alcohol cessation

30
Q

What is a normal pressure hydrocephalus (NPH)?

A

Neurological disorder in which excess CSF accumulates in brain’s ventricles causing them to enlarge. However the CSF pressure often appears normal on LP hence the term NPH.

31
Q

What are causes of normal pressure hydrocephalus?

A

Cdtns that can block flow/absorption of CSF leading to accumulation i.e. subarachnoid hameorrhage, meningitis, head injury or surgical complication

32
Q

Sx of normal pressure hydrocephalus?

mnemonic - Wet, Wacky and Wobbly

A
  • Dementia - attention + memory disturbance
  • Magnetic gait - difficulty lifting feet off floor
  • Incontinence - mainly urinary incontinence but can be fecal
33
Q

Ix for NPH?

A
  • CT/MR imaging
  • Lumbar Puncture
34
Q

Mx of NPH?

A
  • Therapeutic LP
  • Ventriculoperitoneal shunt - insert a shunt to permanently redirect the excess CSF from the brain to the abdomen.
35
Q

What is cerebral palsy?

A

A group of permanent movement disorders that appear in early childhood due to damage to a child’s CNS particularly motor control. It’s non-progressive so does not worsen over time.

36
Q

Causes of cerebral palsy?

A

Antenatal:
* Hypoxic-ischaemic encephalopathy
* Infection

Postnatal:
* Meningitis
* Trauma
* Haemorrhage
* Meds toxicity

37
Q

Sx of cerebral palsy?

A

Spastic (damage to pyramidal pathways):
* Inc. tone + reflexes
* Flexed hip/elbow
* Scissor gait
* Possibly paralysed in one or more limbs

Dyskinetic (damage to basal ganglia pathways):
* Choreiform movements (i.e. jerking)
* Sx of parkinsonism

Ataxic (damage to cerebellar pathways):
* Uncoordinated movements
* Cerebellar lesion sx

38
Q

Ix for cerebral palsy?

A

MRI
Genetic testing - rule out ddx

39
Q

Mx of cerebral palsy?

A

Baclofen - mx muscle spasms
Botox
Physio
Speech and language therapists - support with swallowing

Ortho surgery for MSK deformities

40
Q

What is hypoxic ischaemic encephalopathy?

A

Cdtn characterised by brain damage resulting from antenatal hypoxia.

41
Q

What are some causes of poor oxygenation that can lead to hypoxic ischaemic encephalopathy in infants including a pre-partum, during delivery and post-partum cause?

A

Pre-partum: Placental abruption
During delivery: Cord compression
Post-partum: Prolonged resp arrest

42
Q

Sx of ischaemic hypoxic encephalopathy?

A

Mild - irritability, changes in behaviour
Severe - Hypotonia, poor responsiveness to stimuli, prolonged seizures

43
Q

Ix for hypoxic ischaemic encephalopathy?

A

EEG - seizure activity
Multiple MRI scans - brain injury extent

44
Q

Mx of hypoxic ischaemic encephalopathy?

A

Resp support - oxygen
Fluid balance
Seizure mx + support BP

Phase 3a - Geriatrics and Neurology (11 decks)

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