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Neurology > Neuropathology > Flashcards

Neuropathology Flashcards

1
Q

Presentation of Brain tumours

A
  • Often asymptomatic esp. when small

- Signs and symptoms of raised ICP

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2
Q

What is raised intracranial pressure

A

Anything that takes up additional space within the skull will increase the pressure in the intracranial space

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3
Q

Causes of raised ICP

A

Brain tumours
Intracranial haemorrhage
Idiopathic intracranial hypertension
Abscesses or infection

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4
Q

Concerning features of a head ache that should prompt further investigation

A 
Constant
Nocturnal
Worse on waking
Worse on coughing, straining or bending forward
Vomiting
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5
Q

Features of Raised ICP

A 
Altered mental state
Visual field defects
Seizures (particularly focal)
Unilateral ptosis
Third and sixth nerve palsies
Papilloedema (on fundoscopy)
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6
Q

What is papilloedema

A
  • swelling of the optic disc secondary to raised intracranial pressure
  • can be seen on fundoscopy
  • CSF under high pressure flows into the optic nerve sheath.
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7
Q

What can be seen on fundoscopy in raised ICP

A

Blurring of the optic disc margin
Elevated optic disc (look for the way the retinal vessels flow across the disc to see the elevation)
Loss of venous pulsation
Engorged retinal veins
Haemorrhages around optic disc
Paton’s lines which are creases in the retina around the optic disc

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8
Q

Which cancers commonly metastasise to the brain

A

Lung
Breast
Colorectal
Prostate

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9
Q

What are Gliomas

A

Gliomas are tumours of the glial cells in the brain or spinal cord. There are three types to remember ( most to least malignant):

Astrocytoma (glioblastoma multiforme is the most common)
Oligodendroglioma
Ependymoma

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10
Q

What are meningiomas

A

Meningiomas are tumours growing from the cells of the meninges in the brain and spinal cord. They are usually benign, however they take up space and this mass effect can lead to raised intracranial pressure and neurological symptoms.

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11
Q

What is the treatment of pituitary tumours

A

Trans-sphenoidal surgery
Radiotherapy
Bromocriptine to block prolactin secreting tumours
Somatostatin analogues (e.g. ocreotide) to block growth hormone secreting tumours

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12
Q

Pituitary tumours have the potential to cause hormone deficiencies (hypopituitarism) or to release excessive hormones leading to:

A

Acromegaly
Hyperprolactinaemia
Cushing’s disease
Thyrotoxicosis

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13
Q

What is the key features of pituitary tumours

A
  • Tend to be benign.
  • Can press on the optic chiasm causing: bitemporal hemianopia. This causes loss of the outer half of the visual fields in both eyes.
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14
Q

What are Acoustic Neuroma (AKA Vestibular Schwannoma)

A
  • Tumours of the Schwann cells surrounding the auditory nerve that innervates the inner ear.
  • occur around the “cerebellopontine angle”
  • growing but eventually grow large enough to produce symptoms and become dangerous.
  • usually unilateral
  • Bilateral: associated with neurofibromatosis type 2.
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15
Q

What are the common symptoms of Acoustic Neuroma (AKA Vestibular Schwannoma)

A

Hearing loss
Tinnitus
Balance problems
Facial nerve palsy

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16
Q

What is neuropathic pain

A

abnormal functioning of the sensory nerves delivering abnormal and painful signals to the brain.

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17
Q

What is the presentation of neuropathic pain

A 
Burning
Tingling
Pins and needles
Electric shocks
Loss of sensation to touch of the affected area
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18
Q

What causes neuropathic pain

A

Postherpetic neuralgia from shingles is in the distribution of a dermatome and usually on the trunk
Nerve damage from surgery
Multiple sclerosis
Diabetic neuralgia typically affects the feet
Trigeminal neuralgia
Complex Regional Pain Syndrome (CRPS)

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19
Q

What assessment tool is used to assess whether pain is neuropathic in natures

A

DN4 questionnaire

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20
Q

What is the firt line management of neuropathic pain

A 
Amitriptyline:  tricyclic antidepressant
Duloxetine: SNRI antidepressant
Gabapentin: anticonvulsant
Pregabalin: anticonvulsant
Only use one at a time
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21
Q

What are alternative methods to manage neuropathic pain

A 
- Tramadol ONLY as a rescue for short term control of flares
Capsaicin cream (chilli pepper cream) for localised areas of pain
Physiotherapy to maintain strength
Psychological input to help with understanding and coping
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22
Q

What is complex regional pain syndrome

A
  • Abnormal nerve functioning causing neuropathic pain and abnormal sensations
  • isolated to one limb
  • Often it is triggered by an injury to the area
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23
Q

what are the key features of complex regional pain syndrome

A
  • very painful and hypersensitive
  • Intermittently swell
  • change colour
  • change temperature
  • flush with blood
  • abnormal sweating.
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24
Q

What is the management of complex regional pain syndrome

A
  • pain specialist

- similar to other neuropathic pain.

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25
Q

What is neurofibromatosis

A
  • genetic condition that causes nerve tumours (neuromas) to develop throughout the nervous system
  • Tumours are benign, but cause neurological and structural problems
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26
Q

Which gene is associated with neurofibromatosis type 1

A
  • NF1 gene located on Cr 17
  • codes for a protein called neurofibromin, which is a tumour suppressor protein
  • Autosomal dominant inheritance
27
Q

What is the diagnostic criteria for NF1 (require 2/7)

A

C – Café-au-lait spots (6 or more) measuring ≥ 5mm in children or ≥ 15mm in adults
R – Relative with NF1
A – Axillary or inguinal freckles
BB – Bony dysplasia such as Bowing of a long bone or sphenoid wing dysplasia
I – Iris hamartomas (Lisch nodules) (2 or more) are yellow brown spots on the iris
N – Neurofibromas (2 or more) or 1 plexiform neurofibroma
G – Glioma of the optic nerve

28
Q

What investigations should be done to look for neurofibramatosis

A
  • genetic testing
  • X rays: bone pain/lesions
  • CT/MRI to look at brain and spinal cord
29
Q

What is the management of neurofibromatosis 1

A
  • no treatment of the underlying disease process or to prevent the development of neurofibromas
  • control symptoms
  • monitor the disease
  • treat complications.
30
Q

Complications of Neurofibromatosis 1

A

Migraines
Epilepsy
Renal artery stenosis causing hypertension
Learning and behavioural problems (e.g. ADHD)
Scoliosis of the spine
Vision loss (secondary to optic nerve gliomas)
Malignant peripheral nerve sheath tumours
Gastrointestinal stromal tumour (a type of sarcoma)
Brain tumours
Spinal cord tumours with associated neurology (e.g. paraplegia)
Increased risk of cancer (e.g. breast cancer)
Leukaemia

31
Q

What gene is involved in neurofibromatosis 2

A
  • Found Cr 22
  • codes for a protein called merlin, which is a tumour suppressor protein particularly important in Schwann cells
  • Cause Schwannomas
  • Autosomal dominant
32
Q

What ttype of tumour is associated with neurofibromatosis 2

A 
acoustic neuroma (if bilateral almost always NF2
Schwannomas can occur in brain in spinal cord
33
Q

What are the key features of acoustic neuromas

A

Hearing loss
Tinnitus
Balance problems

34
Q

What is the management of tumours associated with neurofibromatosis 2

A

Surgery can be used to resect tumours although there is a risk or permanent nerve damage.

35
Q

What is guillian barre syndrome

A

“acute paralytic polyneuropathy” that affects the peripheral nervous system. It causes acute, symmetrical, ascending weakness and can also cause sensory symptoms

36
Q

What causes GBS

A

Often because of infection:

  • Campylobacter jejuni
  • Cytomegalovirus
  • Epstein-Barr virus
37
Q

What is the presentation of GBS

A
  • Symmetrical ascending weakness (starting at the feet and moving up body)
  • Reduced reflexes
  • peripheral loss of sensation or neuropathic pain
  • may progress to the cranial nerves and cause facial nerve weakness
38
Q

What is the clinical course of GBS

A
  • Symptoms start within 4 weeks of the preceding infection
  • symptoms start in the feet and progresses upward.
  • peak within 2-4 weeks, then there is a recovery period that can last months to years.
39
Q

How is GBS diagnosed

A
  • Clinically
  • Nerve conduction studies (reduced signal through the nerves)
  • Lumbar puncture for CSF (raised protein with a normal cell count and glucose)
40
Q

What is the management of GBS

A
  • IV immunoglobulins
  • Plasma exchange
  • Supportive care
  • VTE prophylaxis
    Respiratory failure:
  • intubation, ventilation
  • admission to the intensive care unit.
41
Q

What is the prognosis of GBS

A

80% will fully recover
15% will be left with some neurological disability
5% will die

42
Q

What are seizures

A
  • transient episodes of abnormal electrical activity in the brain
  • many different types of seizures.
43
Q

How do you diagnose epilepsy

A
  • clinical diagnosis
  • MRI to look for structural causes
  • (EEG
  • Rule out other pathology eg. Heart
44
Q

What is a Generalised Tonic-Clonic Seizures

A
  • loss of consciousness and tonic (muscle tensing) and clonic (muscle jerking) episodes
  • Typically tonic before clonic
  • may be associated tongue biting, incontinence, groaning and irregular breathing.
  • prolonged post-ictal period
45
Q

What is the treatment for generalised tonic-clonic syndrome

A

First line: sodium valproate

Second line: lamotrigine or carbamazepine

46
Q

What are focal seizures

A
  • Focal seizures start in temporal lobes

- They affect hearing, speech, memory and emotion

47
Q

What features may you see in a focal seizure

A
  • Hallucinations
  • Memory flashbacks
  • Déjà vu
  • Doing strange things on autopilot
48
Q

What is the management of a focal seizure

A

First line: carbamazepine or lamotrigine
Second line: sodium valproate or levetiracetam
( reverse of tonic clonic)

49
Q

What are absence seizures

A
  • Typically in children
  • blank, stares into space and then abruptly returns to norma
  • Unaware of surroundings and won’t respond during the episode
  • Last 10-20 seconds
50
Q

What is the management of absence seizures

A
  • > 90% grow out

- First line: sodium valproate or ethosuximide

51
Q

What are atonic seizures

A
  • “drop attacks”. They are characterised by brief lapses in muscle tone
  • Last <3 minutes
  • typically being in children
52
Q

What may atonic seizures suggest

A

Lennox-Gastaut syndrome

53
Q

What is the management of atonic seizures

A

First line: sodium valproate

Second line: lamotrigine

54
Q

What are myoclonic seizures

A
  • sudden brief muscle contractions, like a sudden “jump”
  • Patient usually remains awake
  • typically happen in children as part of juvenile myoclonic epilepsy
55
Q

What is the management of myoclonic seizures

A

First line: sodium valproate

Other options: lamotrigine, levetiracetam or topiramate

56
Q

What are infantile spasms: ‘West syndrome”

A
  • rare (1 in 4000) disorder starting in infancy: 6 months
  • characterised by clusters of full body spasms
  • poor prognosis: 1/3 die by age 25, however 1/3 are seizure free
57
Q

What is the management of infantile spasms/west syndrome

A

Prednisolone

Vigabatrin

58
Q

How does sodium valproate work?

A

increasing the activity of GABA, which has a relaxing effect on the brain.

59
Q

What are the side effects of sodium valproate

A
  • Teratogenic so patients need careful advice about contraception (avoid in woman of child bearing age unless no alternatives)
  • Liver damage and hepatitis
  • Hair loss
  • Tremor
60
Q

What are the main side effects of carbamazepine

A

Agranulocytosis
Aplastic anaemia
Induces the P450 system so there are many drug interactions

61
Q

What are the key side effects of phenytoin

A 
Folate and vitamin D deficiency
Megaloblastic anaemia (folate deficiency)
Osteomalacia (vitamin D deficiency)
62
Q

What is status epilepticus

A
  • medical emergency

- seizures lasting more than 5 minutes or more than 3 seizures in one hour.

63
Q

What is the management of status epilepticus

A

Secure the airway
Give high-concentration oxygen
Assess cardiac and respiratory function
Check blood glucose levels
Gain intravenous access (insert a cannula)
IV lorazepam 4mg, repeated after 10 minutes if the seizure continues
If seizures persist: IV phenobarbital or phenytoin

64
Q

What are the medical options for status epilepticus in the community

A

Buccal midazolam

Rectal diazepam

Neurology (9 decks)

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