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Year 4 - Special Senses > Neurology - Other > Flashcards

Neurology - Other Flashcards

1
Q

What is developmental delay?

A

A significant lag in a child’s physical, cognitive, behavioural, emotional, or social development, relative to established growth milestones.

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2
Q

Red flags of development:

a) smile

b) sit unsupported

c) walk

A

a) doesn’t smile at 10 weeks old

b) cannot sit unsupported at 12 months old

c) cannot walk at 18 months old

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3
Q

What does hand preference before 12 months indicate?

A

Cerebral palsy

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4
Q

What are some causes of speech and language problems?

A
  • congenital deafness
  • environmental deprivation
  • general development delay
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5
Q

Causes of developmental delay.

A
  • ASD
  • cerebral palsy
  • fragile X syndrome
  • Down syndrome
  • fetal alcohol spectrum disorder (FASD)
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6
Q

What is chorea?

A

Involuntary, rapid, jerky movements which often move from one part of the body to another.

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7
Q

Pathophysiology of chorea.

A

Damage to the basal ganglia, in particular the caudate nucleus.

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8
Q

Causes of chorea.

A
  • Huntington’s disease
  • Wilson’s disease
  • SLE
  • anti-phospholipid syndrome
  • antipsychotics
  • pregnancy
  • thyrotoxicosis
  • carbon monoxide poisoning
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9
Q

What is hemiballism?

A

Involuntary, sudden, jerking movements of the proximal limb contralateral to the side of the lesion.

NB: A type of chorea.

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10
Q

Pathophysiology of hemiballism.

A

Damage to the subthalamic nucleus.

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11
Q

Treatment of hemiballismus.

A

Antidopaminergic agents (e.g. haloperidol) are the mainstay of treatment.

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12
Q

Genetics of Huntington’s disease.

A

Autosomal dominant repeat expansion of CAG.

As it is a trinucleotide expansion, the phenomenon of anticipation may be seen (ie. disease presents at an earlier age in successive generations).

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13
Q

Pathophysiology of Huntington’s disease.

A

Degeneration of cholinergic and GABAergic neurons in the striatum of the basal ganglia.

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14
Q

Features of Huntington’s disease.

A

Typically develop after 35 years of age:
- chorea
- personality changes (e.g. irritability, apathy, depression)
- intellectual impairment
- dystonia
- saccadic eye movements

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15
Q

What is an oculogyric crisis?

A

The spasmodic movement of the eyeballs into a fixed position, usually upwards

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16
Q

Causes of oculogyric crisis.

A
  • antipsychotics
  • metoclopramide
  • Parkinson’s disease
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17
Q

Management of oculogyric crisis.

A
  • cessation of causative medication
  • IV antimuscarinic (procyclidine)
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18
Q

Clinical features of restless leg syndrome.

A
  • uncontrollable urge to move legs
  • paraesthesia
  • movements during sleep
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19
Q

Causes of restless leg syndrome.

A
  • genetics
  • iron deficiency anaemia
  • uraemia
  • diabetes mellitus
  • pregnancy
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20
Q

Management of restless legs syndrome.

A

Simple measures: walking, stretching, massaging affected limbs.

Treat any iron deficiency.

First line: dopamine agonists (e.g. ropinirole).

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21
Q

What is Wilson’s disease?

A

Autosomal recessive disorder causing abnormal copper deposition in the tissues.

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22
Q

Features of Wilson’s disease.

A

Symptom onset from 10-25 years:
- hepatitis and cirrhosis
- chorea
- dementia
- parkinsonism
- Kayser-Fleischer rings
- renal tubular acidosis
- haemolysis
- blue nails

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23
Q

Investigations of Wilson’s disease.

A
  • reduced serum caeruloplasmin
  • reduced total serum copper
  • increased free serum copper
  • increased urinary copper excretion
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24
Q

Management of Wilson’s disease.

A

First line (current): penicillaemine - chelates copper.

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25
Q

What is insomnia?

A

The difficulty initiating or maintaining sleep, or early-morning waking that leads to dissatisfaction with sleep quality or quantity.

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26
Q

Causes of acute insomnia.

A

Often related to a life event and resolves without treatment.

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27
Q

Presentation of insomnia.

A
  • fatigue
  • decreased periods of sleep
  • decreased daytime functioning
  • poor concentration / attention

Often the partner’s rest will also suffer.

NB: Important to identify the cause of insomnia, as management can differ.

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28
Q

Risk factors for insomnia.

A
  • increased age
  • unemployment
  • alcohol / substance abuse
  • stimulant usage
  • corticosteroids
  • chronic pain
  • poor sleep hygiene
  • psychiatric illness (e.g. anxiety, depression, mania, PTSD)
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29
Q

Investigating insomnia.

A

Diagnosis is primarily made through patient interview.

Sleep diaries may aid diagnosis.

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30
Q

Short term management of insomnia.

A
  • identify potential causes
  • sleep hygiene measures
  • advise the person not to drive while sleepy
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31
Q

Long-term management of insomina.

A

If daytime sleepiness is SEVERE, can consider Z drug prescription (e.g. zopiclone).

Consider referral for cognitive behavioural therapy if not fixed within 2 weeks.

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32
Q

What is polymyalgia rheumatica?

A

Underlying cause is not understood.

PMR is relatively common and seen in older people, characterised by proximal muscle stiffness and raised inflammatory markers.

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33
Q

Features of PMR.

A
  • age >60 years
  • rapid onset (<1 month)
  • morning stiffness in proximal limb muscles*
  • polyarthralgia

*weakness is NOT a symptom of PMR.

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34
Q

Investigations of PMR.

A
  • raised ESR
  • CK normal
  • EMG normal
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35
Q

Treatment of PMR.

A
  • prednisolone

pts typically respond dramatically to steroids - failure to do so should prompt consideration of an alternative diagnosis.

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36
Q

What is motor neuron disease?

A

A neurological condition of unknown cause which can present as both upper and lower motor neuron signs.

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37
Q

Pathophysiology of motor neurone disease.

A

Progressive degeneration of the upper and lower motor neurones, resulting in motor deficit.

NB: sensory neurones are spared; sensory deficit should suggest an alternate diagnosis.

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38
Q

Lower motor neurone signs.

A
  • hypotonia
  • hyporeflexia
  • rapid muscle wasting
  • weakness and paralysis
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39
Q

Upper motor neuron signs.

A
  • hypertonia
  • hyperreflexia
  • muscle mass maintained
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40
Q

Features of amyotrophic lateral sclerosis (MND).

A
  • LMN signs in arms
  • UMN signs in legs
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41
Q

Features of primary lateral sclerosis (MND).

A
  • UMN signs only in arms and legs
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42
Q

Features of progressive. muscular atrophy (MND).

A
  • LMN signs only in arms and legs
  • affects distal muscles before proximal
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43
Q

Features of progressive bulbar palsy (MND).

A
  • palsy of tongue
  • palsy of mastication muscles
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44
Q

Which type of MND is most common?

A

Amyotrophic lateral sclerosis (50%)

45
Q

Which type of MND carries the best prognosis?

A

Progressive muscular atrophy

46
Q

Which type of MND carries the worst prognosis?

A

Progressive bulbar palsy

Loss of function of brainstem motor nuclei - eventual cardiorespiratory compromise.

47
Q

Medical management of motor neuron disease.

Give MOA.

A

Riluzole

Prevents stimulation of glutamate receptors.

Prolongs life by around 3 months.

48
Q

Non-pharmacological management of motor neuron disease.

A

Respiratory care - BIPAP at night gives survival benefit of around 7 months.

Nutrition - PEG to support nutrition.

49
Q

Prognosis of motor neuron disease.

A

50% of patients die within 3 years.

Poor prognosis.

50
Q

What is multiple sclerosis?

A

Chronic cell-mediated autoimmune disorder characterised by demyelination in the central nervous system.

51
Q

Causes of multiple sclerosis.

A
  • multiple genes
  • EBV
  • low vitamin D
  • smoking
  • obesity
52
Q

What is the most common subtype of multiple sclerosis?

A

Relapsing-remitting type.

Acute attacks (lasting 1-2 months) followed by periods of remission as re-myelination occurs.

Lesions vary in location, meaning the affected sites and symptoms change over time.

53
Q

What is secondary progressive multiple sclerosis?

A

When relapsing-remitting patients deteriorate, they develop permanent neurological signs and symptoms between relapses.

This is due to an inability to remyelinate axons in the CNS.

Most patients with relapsing-remitting disease go on to develop secondary progressive disease within 15 years of diagnosis.

54
Q

Features of multiple sclerosis.

A
  • optic neuritis (most common)
  • pins and needles
  • numbness
  • trigeminal neuralgia
  • spastic weakness in legs
  • ataxia
  • tremor
  • urinary incontinence
  • sexual dysfunction
  • intellectual deterioration
55
Q

What is optic neuritis?

A

Demyelination of the optic nerve presenting with unilateral reduced vision.

56
Q

Optic neuritis

a) symptoms

b) signs

A

a) enlarged central blind spot; pain with eye movement; impaired colour vision;

b) relative afferent pupillary defect

57
Q

What is relative afferent pupillary defect?

A

Direct pupillary reflex: reduced pupillary constriction in affected eye.

Consensual pupillary reflex: normal pupillary constriction in affected eye.

58
Q

Diagnosis of mutliple sclerosis.

A

Clinical diagnosis over time - demonstration of lesions disseminated in time and space.

Investigations may support the diagnosis:
- MRI scan (demonstrate typical lesions)
- lumbar puncture (oligoclonal bands in CSF)

59
Q

Management of acute MS relapse.

A

High dose steroids - usually responsive to a course of steroids.

60
Q

Relapse reduction in MS.

A

Disease modifying drugs (DMARDs) - monoclonal antibodies that appear to help slow / prevent demyelination.

61
Q

How is fatigue associated with MS managed?

A

Exclude other causes (e.g. anaemia, thyroid, depression).

NICE recommend a trial of amantadine; other options include mindfulness and CBT.

62
Q

How is spasticity associated with MS managed?

A

Baclofen or Gabapentin

Physiotherapy is important.

63
Q

How is bladder dysfunction associated with MS managed?

A

Ultrasound to assess residual volume.

If significant residual volume: intermittent self-catheterisation.

If no significant residual volume: anticholinergics may improve urinary frequency.

64
Q

Complications of MS.

A
  • fatigue
  • neurological deficity
  • incontinence
  • retention
65
Q

Inheritance pattern for Duchenne muscular dystrophy.

A

X-linked recessive

66
Q

Pathophysiology of Duchenne muscular dystrophy.

A

Inherited mutation in dystrophin genes, required for normal muscular function.

67
Q

Features of Duchenne muscular dystrophy.

A
  • progressive proximal muscle weakness from age 5 years
  • calf pseudohypertrophy
  • intellectual impairment
  • Gower’s sign (see image)
68
Q

Investigations of Duchenne muscular dystrophy.

A
  • raised CK
  • genetic testing
69
Q

Prognosis of Duchenne muscular dystrophy.

A
  • most children cannot walk by the age of 12 years
  • patients survive to around the age of 25-30 years
  • associated with dilated cardiomyopathy
70
Q

What is myotonic dystrophy?

A

An inherited myopathy affecting skeletal, cardiac and smooth muscle.

71
Q

Inheritance of myotonic dystrophy.

A

Autosomal dominant trinucleotide repeat.

DM1 caused by DMPK mutation.

DM2 caused by ZNF9 mutation.

72
Q

Features of myotonic dystrophy.

A

Onset around 20-30 years:
- tonic muscle spasms
- weakness of arms and legs
- testicular atrophy
- heart block
- dysphagia

73
Q

Clinical features to differentiated DM1 and DM2.

A

DM1: distal weakness more prominent.

DM2: proximal weakness more prominent.

74
Q

What is myasthenia gravis?

A

An autoimmune disorder resulting in insufficient functioning acetylcholine receptors.

75
Q

Features of myasthenia gravis.

A
  • fatigability (ie. muscles become progressively weaker during periods of activity, and slowly improve after periods of rest)
  • diplopia
  • proximal muscle weakness
  • ptosis
  • dysphagia
76
Q

Associations of myasthenia gravis.

A

Thymoma - thymus gland tumours.

Other autoimmune disorders:
- pernicious anaemia
- autoimmune thyroid disorder
- rheumatoid arthritis
- SLE

77
Q

Investigations of myasthenia gravis.

A

Antibody test to look for:
- AChR antibodies (~80%)
- MuSK antibodies
- LRP4 antibodies

CT or MRI of thymus to look for thymoma.

78
Q

Management of myasthenia gravis.

A

Long-acting acetylcholinesterase inhibitors (e.g. pyridostigmine).

As the disease progresses, immunosuppression with steroids may become necessary.

79
Q

How to elicit fatiguability in muscles (myasthenia gravis).

A
  • repeated blinking exacerbates ptosis
  • prolonged upward gaze induces diplopia
  • repeated abduction of one arm results in weakness

NB: also check for thymectomy scar.

80
Q

What is a myasthenic crisis?

A

Another illness, such as respiratory infection, causes an acute worsening of symptoms.

Respiratory muscle weakness can lead to respiratory failure - patients may need non-invasive ventilation or mechanical ventilation.

81
Q

Treatment of myasthenic crisis.

A
  • plasmapheresis
  • IV immunoglobulins
82
Q

Which drugs may exacerbate myasthenia?

A
  • penicillamine
  • quinidine
  • beta blockers
  • lithium
  • phenytoin
  • gentamicin
  • macrolides
  • quinolones
  • tetracyclines
83
Q

Differential diagnoses of a tremor.

A
  • benign essential tremor
  • Parkinson’s disease
  • multiple sclerosis
  • Huntington’s chorea
  • hyperthyroidism
  • fever
  • dopamine antagonists (e.g. antipsychotics)
84
Q

Presentation of brain tumours.

A
  • asymptomatic
  • progressive focal neurology
  • raised intracranial pressure
85
Q

What is the concern of an usual change in personality and behaviour?

A

Frontal lobe tumour - responsible for personality and higher-level decision making.

86
Q

Causes of intracranial hypertension.

A
  • brain tumours
  • intracranial haemorrhage
  • idiopathic
  • asbcess
  • infection
87
Q

Features of intracranial hypertension.

A
  • constant headache
  • worse on waking
  • worse on coughing
  • vomiting
  • papilloedema on fundoscopy
88
Q

What is papilloedema?

A

Swelling of the optic disk secondary to raised intracranial pressure.

The raised CSF pressure increases pressure around the optic nerve, causing the optic disc to bulge forwards.

89
Q

What are the glial cells?

A
  • astrocytes
  • oligodendrocytes
  • epdenymal cells
90
Q

What are gliomas?

A

Tumours of the glial cells in the brain or spinal cord.

Astrocytoma (most common)
Oligodendroglioma
Ependymoma

91
Q

What is a meningioma?

A

Tumours growing from the cells of the meninges.

Benign but have mass effect, leading to raised intracranial pressure and neurological symptoms.

92
Q

Cancers that most often spread to the brain.

A
  • lung
  • breast
  • renal cell carcinoma
  • melanoma
93
Q

What visual field defect is seen with pituitary tumours?

A

Bitemporal hemianopia, due to compression of the decussating nasal fibres in the optic chiasm.

94
Q

Management of pituitary tumours.

A
  • trans-sphenoidal surgery
  • radiotherapy
  • bromocriptine (blocks excess prolactin)
  • somatostatin analogues (blocks growth hormone)
95
Q

What is an acoustic neuroma?

A

A benign tumour of the Schwann cells surrounding the auditory nerve.

96
Q

Presentation of acoustic neuroma.

A
  • unilateral sensorineural hearing loss
  • unilateral tinnitus
  • dizziness / imbalance
  • sensation of fullness in ear
  • facial nerve palsy

NB: See image for relevant anatomy & link to symptoms.

97
Q

Management of vestibular schwannoma.

A
  • conservative
  • surgery
  • radiotherapy
98
Q

Management of brain tumours.

A
  • MRI scan
  • biopsy for histological diagnosis

MDT involvement:
- surgery
- chemotherapy
- radiotherapy
- palliative care

99
Q

Pathophysilogy of Lamberton-Eaton myasthenic syndrome?

A

Paraneoplastic NSCLC, producing autoantibodies targeting voltage-gated calcium channels in the presynaptic terminal, meaning less acetylcholine is released into the synaptic cleft.

This results in a weaker signal and reduced muscle contraction.

100
Q

Presentation of Lamberton-Eaton myasthenic syndrome.

A
  • proximal muscle weakness (ie. difficultly climbing stairs, standing from seat)
  • autonomic dysfunction (ie. dry mouth, blurred vision, impotence, dizziness)
  • hypo-/areflexia
101
Q

How can Lambert-Eaton myasthenic syndrome and myasthenia gravis be differentiated?

A

Myasthenia gravis: fatiguable muscle weakness.

Lambert-Eaton: muscle weakness improves on exercise.

102
Q

Management of Lambert-Eaton myasthenic syndrome.

A

Exclude underlying malignancy.

FIRST LINE: Amifampridine - blocks voltage gated potassium channels in the presynaptic terminal.

Consider: pyridostigmine (cholinesterase inhibitor); immunosuppressants; IV immunoglobulins; plasmapheresis.

103
Q

Pathophysiology of Charcot-Marie-Tooth disease.

A

Inherited disease affecting peripheral motor and sensory neurones.

104
Q

Features of Charcot-Marie-Tooth disease.

A
  • high foot arches
  • distal muscle wasting
  • lower leg weakness
  • weakness in hands
  • hyporeflexia
  • hypotonia
  • peripheral sensory loss
105
Q

What is peripheral neuropathy?

A

Reduced sensory and motor function in the peripheral nerves, typically affecting the feet and hands.

106
Q

Causes of peripheral neuropathy.

A

ABCDE mnemonic:

Alcohol
B12 deficiency
Cancer (e.g. myeloma) and CKD
Diabetes / Drugs
Every vasculitis

107
Q

Drugs that cause peripheral neuropathy.

A
  • isoniazid
  • amiodarone
  • leflunomide
  • cisplatin
108
Q

Management of Charcot-Marie-Tooth disease.

A

Supportive management:
- neurologists
- physiotherapists
- occupational therapists
- podiatrists
- analgesia
- othopaedic surgeons

Year 4 - Special Senses (17 decks)

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