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Year 3 - Neurology > Week 4 > Flashcards

Week 4 Flashcards

1
Q

What proportion of sleep in young adults is non-REM? At what point of the night is this type of sleep mostly seen?

A

3/4s of sleep in young adults is non-REM

This type of sleep is most commonly seen at the beginning of the night (in contrast to REM which is more seen at the end of the night)

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2
Q

How does Confusional arousal (type of parasomnia) present?

A

Increasing sleep disturbances

Shouting out during sleep and unusual behaviours

Vivid dreams

No significant history

Typically occurs in the first 3rd of the night

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3
Q

Between REM and non-REM sleep, which is more dispensable for adults?

How does the proportion of REM vs non-REM sleep vary over an individual’s lifetime?

A

REM sleep - many common drugs (including tricyclics) suppress REM without any effect being seen

As we age, the amount of REM sleep we have reduces

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4
Q

What is the diagnostic tool used to determine if an individual is suffering from narcolepsy?

A

Multiple Sleep Latency Test

Consists of 4x25 minute naps scheduled 2 hours apart

EEG, muscle activity and eye movements are all recorded

Measures sleep latency - time from the start of a daytime nap to the first signs of sleepiness

(Can also do overnight polysomnography or LP to measure CSF hypocretin)

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5
Q

What process is the most important histopathological indicator of CNS injury, regardless of the cause?

A

Gliosis - hyperplasia and hypertrophy of astrocytes

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6
Q

Of the anterior, middle and posterior cerebral arteries, which is most commonly affected by thrombotic events? Which parts of the brain does this supply?

A

The middle cerebral artery is most commonly affected

Supplies portions of the frontal, temporal and parietal lobes

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7
Q

What is MS?

Are men or women more commonly affected? At what ages typically?

A

MS is an inflammatory demyelinating disorder of the CNS

More commonly affects women (3:1) and initial presentation is typically in the 30s and 40s

Plaques are disseminated in time and place

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8
Q

What are some of the systems affected by MS?

A

Pyramidal dysfunction

Optic system (optic neuritis)

Sensory symptoms (ranging from loss to increased response, as well as altered sensations)

Urinary tract dysfunction

Cerebellar and brainstem features - ataxia, tremors, facial weakness, nystagmus etc.

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9
Q

The effects of MS can be seen in the eyes of some patients - how does this present?

What screening test can be used initially?

A

Optic neuritis develops (painful vision loss)

Colour vision is the first thing to be lost, so Ishihara plates are used to screen

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10
Q

What are the various clinical courses that MS might take?

A

Primary progressive - continually getting gradually worse

Progressive relapsing - continually getting worse, but with some spikes that then return back to the baseline

Secondary progressive

Relapse remitting

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11
Q

What diagnostic criteria is used for MS? What does it state?

A

McDonald Criteria

2 or more attacks suggestive of demyelination

Objective clinical evidence of 2 or more lesions on MRI

Objective clinical evidence of 1 lesion with reasonable historical evidence of a prior attack

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12
Q

What diagnostic investigations are used in a case where MS is suspected?

A

Clinical history and examination - paying close attention to the timing between attacks and spacing of where lesions may be

MRI

CSF sampling - shows an increase in immunoglobulin profiles and protein levels, but the telltale sign is oligoclonal IgG bands seen in PCR (however someone can still have MS in the absence of these bands, and the presence of bands is not specific for MS)

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13
Q

MS treatment - acute relapses and disease-modifying drugs

A

Acute relapses - short course of steroids for severe relapses. Speed up recovery but do not alter long term outcome

DMDs - first line is interferon-ß or glatiramer acetate (decreases relapse rate by 1/3rd and severity of relapse rate by 1/2, however carries a risk of malignancy due to immunosuppression). Second line is monoclonal antibodies (considered in patients with severe relapse-remitting MS) or fingolimod (immunoomodulating drug)

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14
Q

What are the 4 groups of nerve fibre?

A

Separated based on their degree of myelination (most myelinated first)

Large motor fibres (A alpha)

Large sensory fibres (A alpha/beta)

Small sensory fibres (A delta and C)

Autonomic fibres (A delta and C)

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15
Q

What function(s) do large motor fibres allow for?

How would damage to these nerves present itself?

A

Allow for muscle control

Damage would result in weakness, unsteadiness and wasting. Reflexes would be absent

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16
Q

What function(s) do large sensory fibres allow for?

How would damage to these nerves present itself?

A

Allow for touch, vibration and position perception

Damage to these fibres would present with numbness, parasthaesia and unsteadiness

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17
Q

What function(s) do small sensory fibres allow for?

How would damage to these nerves present itself?

A

Allow for warm and cold perception, and pain

Damage to these fibres would present with pain and dysesthesia (abnormal pain/sensation when touched)

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18
Q

What function(s) do autonomic fibres allow for?

How would damage to these nerves present itself?

A

Allow for heart rate, BP, sweating, gastrointestinal and urinary function

Damage to these fibres would present with dizziness (postural hypotension), impotence and nausea and vomiting

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19
Q

What is the difference between a neuropathy and a radiculopathy?

A

Neuropathy - affects the nerves

Radiculopathy - affects nerve roots

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20
Q

What investigation is used to investigate demyelinating neuropathies?

A

Nerve Conduction Studies

Important in the diagnosis of, for example, GBS

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21
Q

Give an example of one acute and one chronic demyelinating neuropathy

A

Acute - GBS

Chronic - Chronic Inflammatory Demyelinating Polyradiculopathy (CIDP, considered to be the chronic counterpart to GBS). Also Hereditary sensory motor neuropathy (formerly known as Charcot-Marie-Tooth disease)

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22
Q

At what age does hydrocephalus cease to cause cranial enlargement and instead cause increased ICP?

A

At the age of 2 (after the sutures of the infant skull have closed)

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23
Q

Bacterial meningitis - what is the most common cause in the following age groups…

  • neonates
  • infants and children
  • adolescents and young adults
  • older adults or children
  • older adults
A

Neonates - E.coli

Infants and children - H.influenzae

Adolescents and young adults - N.meningitides

Older adults or children - S.pneumoniae

Older adults - L.monocytogenes

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24
Q

What are the components of the Glasgow Coma Scale?

A

Eye opening (4)

  • spontaneous
  • to command
  • to pain
  • none

Verbal (5)

  • orientated
  • confused
  • inappropriate words
  • incomprehensible sounds
  • none

Motor (6)

  • obeys commands
  • localises to pain
  • flexes to pain
  • decorticate (abnormal flexion)
  • decerebrate (abnormal extension)
  • none
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25
How might the brain herniate in the event of raised ICP? What structures might be compressed?
**Subfalcine herniation** - may compress the anterior cerebral artery **Tentorial herniation** - may compress the hippocampus, ipsilateral CNIII resulting in pupillary dilatation and impaired ocular movements **Cerebellar herniation** - may compress brainstem resulting in Cushing's Triad
26
What are some of the clinical signs of an upper motor neurone lesion?
**Increased** tone No/late atrophy Fasciculations are absent **Reflexes are brisk** **Plantars are upgoing** **Clonus is present**
27
What are some of the clinical signs of an lower motor neurone lesion?
**Decreased** tone **Atrophy is seen early** **Fasciculations are present** **Reflexes are decreased/absent** Plantars are downgoing Clonus is absent
28
How might pyramidal dysfunction in someone with MS present?
Increase in tone Spasticity of muscles Weakness of muscles Upper limbs might show spasticity in flexion Lower limbs might show spasticity in extension and weakness in the knee and hip
29
What eye pathologies may be seen in someone with MS?
**Optic neuritis** (painful vision loss) - colour goes first and this can be seen with Ishihara plates Patients may also develop a RAPD
30
What sensory pathologies may be seen in someone with MS?
Pain Parasthaesia Dorsal column loss (vibration, fine touch and proprioception) Numbness Trigeminal neuralgia - pain caused by stimulation of the areas supplied by the trigeminal nerve
31
What cerebellar and brainstem dysfunctions may be seen in someone with MS?
Cerebellar * Ataxia * Intention tremor * Past pointing * Nystagmus * Dysdiadokinesis * Dysarthria Brainstem * Diplopia (CN VI palsy) * Facial weakness (CN VII palsy)
32
What is internuclear ophthalmoplegia?
INO is a disorder of conjugate lateral gaze - affected eye shows **impairment of ADDuction** When an attempt is made to contralaterally adduct the affected eye, it does so minimally if at all, while the normal eye does so as expected by with **nystagmus**This also leads to **horizontal diplopia** Disorder is caused by injury/dysfunction of the **medial longitudinal fasciculus** - heavily myelinated tract. In young people with INO, MS is typically the cause
33
What urinary tract pathologies may be seen in someone with MS?
Frequency and urgency Nocturia Urge incontinence Retention
34
Lots of things can mimic MS, which is why there is a scoring criteria for its diagnosis! What is the criteria called? What conditions might mimic MS?
Scoring criteria - **McDonald Criteria** Differentials - vasculitis, granulomatous disorders, vascular disease, structural lesion, infection, metabolic disorders
35
A few investigations can be carried out to determine whether or not someone has MS. What investigation in particular appears to be +ve in at least 90% of cases?
CSF sampling - **oligoclonal IgG bands** NB - their absence doesn't exclude MS!
36
How would you manage an acute presentation of MS if it was... - mild - moderate - severe?
Mild - symptomatic treatment Moderate - oral steroids Severe - admit/IV steroids (methylprednisolone)
37
What treatments are there to aid with spasticity?
Physiotherapy Oral medications - **baclofen, tizanidine** **Botulinum toxin** injections to relax the affected muscles Intrathecal baclofen (administered directly into CSF - end of life/palliative stage)
38
What treatments are there to aid with the sensory symptoms caused by MS?
Anticonvulsants - gabapentin, pregabalin Antidepressants - amitryptiline TENS (transcutaenous electrical nerve stimulation) machines Acupuncture Lignocaine infusion
39
What disease modifying therapies are there for treating MS?
First line - **Interferon-ß**, Glitiramer Acetate (**Copaxone**), **Tecfedira** (first line in RR MS) Second line - mAbs, Fingolimond (immunomodulator) Third line - mitoxantrone
40
What is the first line disease modifying drug for treating people with Relapse-Remitting MS?
Tecfidera - oral agent 44% reduction in relapse rate
41
What type of drug is Fingolimod? What reduction in MS relapse rate is seen?
Immunomodulator, second line medication in MS Modulates **sphingosine 1-phosphate (S1P)** Reduction in relapse rate of \>50% and has a significant effect on disease progression
42
In what circumstances are monoclonal antibodies used to treat MS? Examples?
Used in **highly active relapsing remitting MS** that have a high disease activity despite treatment with Interferon Examples - **Tysabri** (targets integrins), **Lemtrada** (targets CD52), **Ocrelizumab** (targets B cells, used in early primary progressive MS and delays progress by 25%)
43
What is pseudoathetosis? What pathway might it indicate has been disrupted?
Abnormal writhing movements, usually fingers, caused by a failure of joint position sense (proprioception) Indicates disruption of the proprioceptive pathway from nerve to parietal cortex
44
**Conscious** proprioception is communicated by the _____ pathway **Unconscious** proprioception is communicated by the _____ pathway
Conscious = **DCML** Unconscious = **spinocerebellar** (dorsal and ventral)
45
What nerve would you expect to be affected in a patient presenting with foot drop and a high stepping gait?
**Common peroneal nerve**
46
What is the most common peripheral mononeuropathy?
Carpal tunnel syndrome May cause wasting of the muscles in the **thenar eminence**
47
The mechanism of injury in 90% of peripheral neuropathies is axonal loss/peripheral nerve demyelination
Axonal loss
48
Name a) an acute demyelinating neuropathy and b) a chronic demyelinating neuropathy
a) acute (days to weeks) - **Guillaine-Barre Syndrome** b) chronic (months to years) - chronic inflammatory demyelinating polyradiculopathy (CIDP), **hereditary sensory motor neuropathy** (formerly Charcot-Marie-Tooth disease)
49
In a patient that presented with champagne bottle deformities of the legs, clawing of the hands and high arched feet, what diagnosis might you suspect? How is this condition inherited?
**Charcot-Marie-Tooth** (hereditary sensory motor neuropathy) Loads of mutations, can be AD, AR, X-linked Genetic testing is available for the most common mutations e.g. **CMT1a**
50
What is the result of hydrocephalus if it occurs a) under 2 years of age and b) over 2 years of age?
a) under 2 years - **cranial enlargement** b) over 2 years - **raised ICP**
51
What cell type is most commonly the source of primary intracranial tumours in adults? How does grade affect prognosis?
**Astrocytomas** (specifically **glioblastomas**) (astrocytes provide structural and metabolic support for the brain, regulate ion concentration in the extracellular space, modulate synaptic transmissions and transmitter uptake and release) **Lower grade** astrocytomas (I and II) tend to present in **younger** patients - **better prognosis** **Higher grade** (III and IV) tend to present in **older** patients - prognosis is **"dismal"**
52
What is the most common type of primary brain cancer in children? What neighbouring structure do they typically invade?
**Medulloblastoma** Arise from the cerebellum and invade the **4th ventricle**
53
In relation to the tentorium cerebelli, where do brain tumours classically present in a) adults and b) children?
a) **adult** brain tumours classically present **above** the tentorium cerebelli b) **child** brain tumours classically present **below** the tentorium cerebelli
54
Name some bacterial causes of meningitis Which are most commonly seen in each age group?
(**E. coli** - seen in Neonates) **H. influenzae** - seen in infants and children **N. meningitides** - seen in adolescents and young adults **S. pneumoniae** - seen in older adults/children **L. monocytogenes** - seen in older adults (over 60) or IC/IS
55
Initial management of bacterial meningitis - what antibiotics do you give? What about if you want to cover Listeria spp. (i.e. your patient is over 60 or IC/IS, including alcohol dependency and diabetes)?
**1. Ceftriaxone IV 2g bd** (or if penicillin allergy, **Chloramphenicol**) **2. PLUS Dexamethasone IV 10mg qds** - if pneumococcal cause has been confirmed, continue for 4 days. If other cause, stop dexamethasone **If Listeria suspected ADD Amoxicillin IV 2g every 4 hours** (or if penicillin allergy, **Co-trimoxazole**)
56
If you knew that a patient being treated for suspected Listeria bacterial meningitis had recently travelled to a country with a high rate of penicillin resistance, what medication would you give instead?
Instead of amoxicillin, give **Vancomycin or Rifampicin** Countries with high penicillin resistance - Canada, China, Croatia, Pakistan, Poland, Spain, Mexico, Italy, USA, Greece, Turkey
57
Patient has been initially treated with antibiotics for bacterial meningitis, but no causative organism has been found... How long should they be treated?
Treat for **10 days until patient is clinically recovered**
58
Patient has been initially treated with antibiotics for bacterial meningitis and meningococcus has been determined to be the cause. How long should they be treated for?
**5 days Ceftriaxone** (or extend to 7 if not recovered) ## Footnote **STOP Dexamethasone**
59
Patient has been initially treated with antibiotics for bacterial meningitis and pneumococcus has been determined to be the cause. How long should they be treated for?
**10 days Ceftriaxone** (extend up to 14 days) **CONTINUE Dexamethasone for 4 days**
60
Patient has been initially treated with antibiotics for bacterial meningitis and penicillin/cephalosporin-resistant pneumococcus has been determined to be the cause. How long should they be treated for?
**14 days Ceftriaxone + Vancomycin** **CONTINUE Dexamethasone for 4 days** NB - Vancomycin monotherapy not recommended due to concerns with CSF penetration
61
Patient has been initially treated with antibiotics for bacterial meningitis and Listeria has been determined to be the cause. How long should they be treated for?
**At least 21 days of Amoxicillin** **STOP Dexamethasone**
62
What is the histological difference between inactive and active MS plaques?
Inactive - **gliosis predominates** (non-specific reactive change of glial cells in response to damage to the CNS) Active - **ongoing demyelination**
63
Men/Women are more commonly affected by Alzheimer's Disease What are the features of this condition?
**Women** are more commonly affected (2:1) Causes alterations in mood and behaviour due to cortical atrophy (typically in the **frontal, temporal and parietal lobes** - occipital-sparing) Presents with **progressive deterioration**, memory loss and aphasia
64
What histological changes are seen in Alzheimer's Disease?
**Neurofibrillary tangles** containing **Tau** (**intracellular**) Neurytic plaques with **alpha and beta Amyloid** centres (**extracellular**)
65
Amyloid angiopathy is a feature of which neurological condition? In this condition, polymerised beta pleated sheets are formed by alpha and beta amyloid - what is this stained with?
Amyloid angiopathy - **Alzheimer's Disease** Stains with **Congo Red** (this stain is used for any form of amyloidosis)
66
Presenilin 1 (PSEN1) is found on chromosome \_\_\_\_ Presenilin 2 (PSEN2) is found on chromosome \_\_\_\_ Amyloid Precursor Protein (APP) is found on chromosome \_\_\_\_. Mutations affecting this chromosome also cause what other condition, in which early Alzheimer's is commonly seen?
PSEN1 - **chromosome 14** PSEN2 - **chromosome 1** APP - **chromosome 21**. Also involved in **Down's Syndrome** in which early onset Alzheimer's is seen
67
What form of dementia is associated with Parkinson's Disease?
**Dementia with Lewy Bodies**
68
What % of people with Parkinson's Disease will go on to develop Dementia? What % of people with LBD will develop Parkinson's Disease?
Only **5-****10%** of people with Parkinson's Disease will develop LBD However, **all people with LBD will develop Parkinson's**
69
What is this image? What protein abnormally clumps in neurones to form this? What condition is it associated with?
**Lewy Body** Formed by the abnormal accumulation of **alpha-synuclein** protein in the neurones Associated with **Lewy Body Dementia** (and **Parkinson's**) - both
70
How does Dementia with Lewy Bodies present clinically? What might be the first sign seen?
Presents with **FLUCTUATING changes in behaviour, cognition** and **movement** NB - memory loss is not always present early on in the disease **Dementia steadily worsens over time.** May also see visual hallucinations, marked fluctuation in attention/alertness, slowness of movement/rigidity (Parkinsonism) Core feature seen early on is that individuals lose normal muscle paralysis during REM sleep (**REM sleep behaviour disorder**), meaning that they **act out their dreams**
71
What family of medications can be used to treat the cognitive symptoms of dementia (memory and learning deficits), and are also used to increase neuromuscular transmission in myasthenia gravis? Examples of drugs in this family?
**Acetylcholinesterase inhibitors** Examples - **rivastigmine, neostigmine, pyridostigmine**
72
What medication can be used to aid sleep-related problems in someone with Lewy Body Dementia?
**Melatonin**
73
What condition is Sydenham's Chorea associated with?
Rheumatic fever
74
What type of meningitis would the following make you think of? - normal opening pressure, raised lymphocytes and normal glucose - cloudy, raised opening pressure, neutrophils, raised protein and lowered glucose - opaque and clears when allowed to settle, raised opening pressure, lymphocytes, raised protein and lowered glucose
Normal opening pressure, raised lymphocytes and normal glucose - **viral meningitis** Cloudy, raised opening pressure, neutrophils, raised protein and lowered glucose - **bacterial meningitis** Opaque and clears when allowed to settle, raised opening pressure, lymphocytes, raised protein and lowered glucose - **TB meningitis**
75
What type of dementia can a MMSE _not_ aid in diagnosing? What kind of dementia can be diagnosed using a SPECT scan?
**Frontotemporal dementia** can't be diagnosed with MMSE - memory isn't impaired SPECT scanning can be used to diagnose **vascular dementia**
76
Lamotrigine can be used first line to treat what presentation? What is a (rare) side effect of this medication?
Lamotrigine (anticonvulsant) can be used first line for treating **seizures** Rarely can cause **Steven-Johnson syndrome**
77
What medication is used first line to treat focal seizures?
**Carbamazepine** (and **Lamotrigine**)
78
What medication can be given to treat severe migraines and cluster headaches? What medications should it be used in combination with? Which medication should it NOT be used in combination with?
**Sumatriptan** (serotonin, 5-HT1 receptor agonist) is used to treat severe migraines and cluster headaches Use in combination with an **anti-emetic** and a **NSAID** Do NOT use in combination with the **oral contraceptive pill** Also beware, may precipitate serotonin syndrome
79
What's the diagnosis? How's it treated? - severe unilateral headache - possible ipsilateral autonomic features e.g .Horner's syndrome, nasal congestion etc. - lasts 15 mins-3 hours, occurring between 1 and 8 times a day
Diagnosis - **cluster headache** Treatment - **high flow O2** and sub. cut. **sumatriptan**
80
What's the pathogen? Bacterial meningitis affecting neonates, elderly and pregnant women. Culture shows gram +ve rods
**Listeria** - give **amoxicillin**
81
Infection with what pathogen is associated with Guillain-Barre Syndrome?
Campylobacter
82
What medications are used to treat the following in MS - spasticity - fatigue
Spasticity - **Baclofen** (GABA inhibitor) Fatigue - **Amantidine** (dopamine agonist)
83
What's the diagnosis? - weakness seen at the beginning of the day and improves with exercise - mainly affects lower limbs and larger muscle groups - associated with small cell lung cancer
**Lambert Eaton Syndrome**
84
What is the main type of symptom seen in someone with frontotemporal dementia?
**Behavioural** symptoms (loss of executive functions)
85
Lewy Bodies specifically target what part of the dopaminergic pathway?
The **pars compacta** of the **nigrostriatal pathway**
86
What medication is used first line to treat Parkinson's disease? What other medications can be used?
**Levodopa** is first line Can also use **monoamine oxidase B inhibitors** and **dopamine agonists**
87
Drugs that can cause Parkinsonism?
Typical antipsychotics - **chlorpromazine, haloperidol** Anticonvulsants - **Lithium, Sodium valproate** Antiemetics - **Metoclopramide**
88
What is the difference between Parkinson's Disease Dementia and Lewy Body Dementia?
If dementia started at least **1 year after** **the onset of Parkinson's**, this is termed Parkinson's Disease Dementia Dementia/cognitive dysfunction occurring **prior to Parkinson's** = Lewy Body Dementia
89
What is the difference between a tremor seen in Parkinson's and an essential tremor? How might an essential tremor be improved?
Parkinson's tremor is seen at rest (people often try to hide it - asking them to count down from 100 can exacerbate it) Essential tremor is a movement disorder characterised by a postural or action tremor Often improves **after alcohol**, and may be helped by **beta-blockers** (propranolol)
90
What are intention tremors caused by? What is the most common cause?
Caused by **lesions of the cerebellum** Most common cause is **MS**
91
What are the following signs? - Hoffman's sign - Lhermitte's sign
Hoffman's sign - hold patient's hand and **flick nail of their middle finger**. If **index finger and thumb contract simultaneously** = positive sign and indicates central nervous disease Lhermitte's sign - **sudden, sharp electrical shock feelings** felt in the limbs upon moving the head in certain directions
92
What nerve roots make up the following nerve plexuses? - brachial - lumbar - cervical
Brachial - **C5-T1** Lumbar - **L1-S4** Cervical - **CN XII, C3-C5**

Year 3 - Neurology (7 decks)

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