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Neurology Flashcards

1
Q

Causes of cerebellar syndrome patter?

A
  • Rapid onset symptoms:
    o Stroke – haemorrhagic or ischaemic
  • Relapsing and remitting symptoms:
    o MS which can affect cerebellum
  • Gradual:
    o Alcohol excess – careful social history is important
  • Rarer causes:
    o Inherited ataxias
    o Paraneoplastic degeneration
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2
Q

Broad management of cerebellar syndrome?

A
  • Depends on the cause but will always be MDT – help pt retain function and independence
  • Physiotherapy
    o Help with mobility and preserving strength
  • Occupational therapy
    o Adaptations to the home and mobility aids
    o Are adaptations in the workplace needed to continue occupation
  • Medications:
    o Look at medications too – are there any medications which could be contributing to or making worse their dizziness/unsteadiness
  • Lifestyle:
    o Avoid alcohol as even if alcohol is not the cause this will exacerbate symptoms
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3
Q

Treatment of neuropathic pain?

A

o 1st line TCAs – SE’s drowsiness or dry mouth
o 2nd line Anti epileptics such as gabapentin or pregabalin
o Duloxetine SNRI
o Can try topical capsaicin

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4
Q

Patter for causes of polyneuropathy?

A

o Most common = diabetes
o Other metabolic causes:
 Hypothyroidism
 Vitamin deficiency: B1, B6, B12
o Toxic causes:
 Chemo
 Abx
 Other medications
 Alcohol overuse – would be predominantly sensory too
o Inflammatory conditions:
 Classically CIDP
 SLE
 Rheumatoid arthritis
- Sjorgens syndrome
o Paraneoplastic causes:
 Solid tumour such as lung
 Haematological malignancy: paraproteinaemia

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5
Q

What simple bedside/blood tests are useful in polyneuropathy?

A

o Opthalmoscopy ?diabetic changes
o Urine glucose
o Bedside CBG
- Other investigations:
o FBC (macrocytic anaemia), U&E to check urea level, LFTs ?alcohol use, TFTs, B12 level
o Autoimmune screen: ESR
o Ig and serum electrophoresis
o HbA1c

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6
Q

What role do neurophysiological studies have in investigating polyneuropathy?

A

o Nerve conduction studies can help determine if demyelinating or axonal
 Demyelinating more likely to be associated with inflammatory/immune mediated and would need specialist investigations
o Nerve conduction studies can help us to work out if length dependent or non length dependent
 Things which are not length dependent are more likely to be inflammatory in nature

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7
Q

What is the 3 main steps in management of diabetic neuropathy?

A

o Tight glycaemic control
o Physio
o Regular podiatry for foot care

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8
Q

What would be the initial investigations in stroke?

A
  • Acutely:
    o Urgent CT brain – if no contraindications on Hx and exam and no haemorrhage on CT then could be a candidate for thrombolysis
  • CBG to exclude stroke mimetics
  • Baseline blood tests: (Screening infection) – FBC, U&E, LFT, ESR, TFTs, clotting, lipids
  • Bedside swallow assessment and ECG
  • Chronic/sub acute:
    o MR brain – characterise changes
    o Then look for aetiology:
     ECG
     Carotid doppler
  • ?Evidence of stenosis, look at degree and consider if suitable for endarterectomy therapy
     24h ECG or 72h holter monitor for ?pAF
     Echocardiogram – structural heart defects
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9
Q

What are the finishing points if you think patient has had a stroke?

A

o Full history
o UL and LL PNS exam
 Looking for increased tone, reduced power, hyper-reflexia and clonus
o Bed side tests – BP, pulse (?AF), HS (?valvular pathology), carotid bruit suggestive of bruit and check for glycosuria

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10
Q

What are the causes of spastic paraparesis? (6 categories)

A

o Compressive causes
 Disc herniation
 Tumours – intramedullary/extramedullary, primary/secondary
 Spinal stenosis
o Vascular
 Infarcts
o Autoimmune/inflammatory causes (causing a transverse myelitis)
 Multiple sclerosis
 Lupus
 Sarcoid
o Infectious
 HIV
 Varicella
If travel history then HTLV1 (tropical spastic paraparesis)
o Nutritional
 Vitamin deficiencies such as B12
 Copper deficiency
o Rarer causes
 Hereditary spastic paraparesis

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11
Q

In addition to spastic paraparesis what important signs should you look for in MS?

A

o Optic neuropathy
 RAPD and pale optic disc for optic neuritis
o Internuclear ophthalmoplegia (lesion in the medial longitudinal fasiculus)
 Failure of one eye to adduct and nystagmus on abduction of the other eye

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12
Q

What investigations are important in MS?

A

o MR cervicothoracic spine – transverses myelitis
o MR brain – lesions such as periventricular lesions
o LP – normal cell count but oligoclonal bands (if present very indicative of MS)

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13
Q

When a patient is started on high dose steroids (eg. in MS) what should they be counselled on?

A

 Warn about effects on sleep
 Personality changes even mania
 Monitor blood glucose
 GI ulceration
 AVN of the hip

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14
Q

What clinical signs would you see in the LMN examination in Charcot Marie Tooth?

A
  • Inspection:
    o Muscle wasting particularly in the distal muscle groups
    o High arched feet
    o Flaccid tone
  • Reflexes:
    o Can’t elicit even with potentiation
  • Coordination:
    o Intact
  • Sensation:
    o Reduced distally
  • Gait
    o High stepping gait with bilateral foot drop
  • Rombergs:
    o Can’t stand still together
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15
Q

What is the management of Charcot Marie Tooth and why is diagnosis important?

A

o No current disease modifying treatments
o Involves the MDT - Physiotherapists, orthotics, OT team to keep people ambulant
o Ankle foot orthoses are used to correct foot drop
- Diagnosis important as it’s a genetic condition, allows patient to plan and understands what is affecting them, genetic counselling will play a role

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16
Q

Give a brief description of Charcot Marie Tooth disease?

A

It is an autosomal dominant hereditary condition affecting sensory and motor lower motor neurons. There are multiple subtypes and many different mutations.

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17
Q

Clinical diagnosis of MND is based on what findings?

A

o Combined UMN and LMN signs
o LMN signs: wasting, fasciculations
o UMN signs: hypertonia, brisk reflexes (Because of the combination may not see brisk reflexes might just see normal reflexes)
o Also need to demonstrate abnormalities in multiple different segments, specifically
 Bulbar segment
 Cervical segment
 Thoracic segment
 Lumbar segment

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18
Q

What other neurological conditions are associated with MND?

A

Fronto-temporal dementia - so ask about behavioural change and cognitive difficulties

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19
Q

What role do genetics play in MND?

A

some cases are familial and associated predominantly with the C9orf72 hexonucleotide repeat expansion mutation

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20
Q

What is the management of MND?

A

o Patient centered and entire MDT
o Drug treatment with Riluzole can improve survival but the effect is modest
o Specialist nurses, OTs (need for adaptations in home, work place and with transportation) and physios may have more practical benefit
o Regular screening for complications is a massive part of management
o SALT – help with communication and appropriate diet to avoid aspiration
o Dietician support – whether PEG feeding is needed
o Regular monitoring of respiratory function – FVC and early morning ABG
 Neuromuscular respiratory failure can be ameliorated with NIV

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21
Q

What are 3 differentials for isolated motor weakness and how can they be differentiated from MND?

A

o X linked spinobulbar muscular atrophy aka Kennedys disease – also affects LMN only
o Spinal muscular atrophy but this only affects lower motor neurons
o Multifocal motor neuropathy with conduction block – this is an acquired autoimmune mediated neuropathy that is responsive to treatment, get weakness without sensory loss and LMN signs only. Get demyelinating features and a conduction block

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22
Q

What findings are seen on EMG in MND?

A

o EMG – fibrillations, fasciculations and an absence of sensory signs

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23
Q

If a patient has weakness what are the 4 questions you need to answer to describe it?

A

Assymmetric or symmetric
Spastic versus flaccid
With or without sensory signs (and which ones)
Where in particular is the weakness (can be generalised/more proximal/distal or particular muscle groups)

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24
Q

What is the primary investigation required in suspected myelopathy and how urgently should this be organised?

A
  • MRI spine following full clinical examination to localise lesion
  • Urgency of the imaging is determined by speed of onset of symptoms and severity
  • Acute onset or rapidly progressive requires same day MRI as you are trying to rule out SCC which requires urgent surgical decompression or metastasis which requires same day radiotherapy
  • In slower onset cases an OP MRI would be appropriate
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25
Q

What points in the history can point towards the underlying aetiology of myelopathy?

A

o Acute onset minutes
 Vascular causes
o Acute but over a day
 Trauma/disc herniation
o Subacute – days to weeks
 Autoimmune causes
o Weeks to months:
 Nutritional deficiencies or malignancy
o Chronic
 Genetic causes
o Ask about family history, systemic enquiry which may point towards nutritional deficiencies or malignancy

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26
Q

What is Kennedy’s disease?

A
  • Motor system disorder
  • Only LMN affected
  • Peri-oral fasciculations are almost pathognomonic
  • X linked recessive disorder
  • Associated with androgen insensitivity
  • Has a very slow rate of progression which significantly distinguishes it from sporadic ALS/MND
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27
Q

Peri-oral fasciculations and pathognomic of what condition?

A

Kennedy’s disease (X linked spinobulbar muscular atrophy)

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28
Q

What are the 4 main anterior horn cell disease?

A

Amyotrophic lateral sclerosis (MND)
Kennedy’s disease
Spinal muscular atrophy
Polio (but this is not progressive like the others)

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29
Q

What is pes cavus and what does it reflect?

A

High arched palate. Reflects chronic neuromuscular disease, usually one that started in childhood, eg. polio, charcot marie tooth or friedreichs ataxia

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30
Q

What clinical signs are seen in polio?

A
  • May see lots of scars – describe these as ‘lots of scars suggesting previous surgery’
  • Walking aids including splints
  • Wasting, dramatic weakness, areflexia and limb shortening
  • Likely asymmetric
  • Pes cavus – suggests a chronic neuromuscular disease, usually one that started in childhood
    (Note don’t usually have sensory signs)
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31
Q

What is post polio syndrome and how is it diagnosed and treated?

A
  • New and progressive weakness in a patient with signs of old polio
  • Poorly understood syndrome
  • Patients develop symptoms of progressive weakness in the previously affected areas but can also get significant issues with pain, cramping and fatigue
  • Diagnosis rests on history
  • But often need to investigate further to exclude concurrent or alternative pathologies – likely includes structural imaging and nerve conduction studies
  • No specific treatment
  • Management is supportive
    o Careful physiotherapy – cautious as overexercise of affected muscle groups has been associated with a worse prognosis
    o Occupational therapy and orthotics
    o Neuro-psychologists can also have a role to help pt come to terms with diagnosis
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32
Q

What are the 2 most common inflammatory/immune mediated polyneuropathies?

A

Acute onset: Guillain Barre syndrome
Chronic: Chronic inflammatory demyelinating polyneuropathies

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33
Q

In a patient with mixed motor sensory polyneuropathy what 3 red flags would point you towards an immune mediated polyneuropathy rather than the more common (DM/hypothyroid/alcohol)?

A

o Non length dependency: eg. proximal weakness but intact distally
o Marked asymmetry
o Marked sensory ataxia (always think sensory neuropathies)

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34
Q

What is the ward based management of GBS and what therapies may be considered if severe?

A
  • Ward based management:
    o Acute setting ABCDE manner and resuscitate appropriately
    o Majority can be managed with supportive care on the ward
    o Monitoring of respiratory function with FVC (much better than PEFR)
     If FVC <1.5L then need to consider evidence of ventilatory failure, consider ABG and discussion with critical care as to need for ventilation
    o In patients with severe disease (eg. Unable to walk) therapies such as plasma exchange or IV immunoglobulin may be required
    o VTE risk – appropriate thromboprophylaxis
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35
Q

What is the overall management for CIDP?

A
  • MDT approach
  • Regular neurology review to see if any supportive treatments are required such as for neuropathic pain or as to whether disease modifying therapies such as plasma exchange, IVIG, steroids or immunosuppression are required
  • Physiotherapy, OT and orthotics team
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36
Q

How would you investigate a suspected inflammatory (aka immune mediated) polyneuropathy?

A
  • Routine blood tests: FBC, U&E, LFTs, CRP and ESR
  • Further blood tests which should be done in peripheral neuropathies: HbA1c, TFTs and Vit B12 and virology
  • Paraneoplastic screen can be done if suspected
  • Neurophysiological studies such as EMG and nerve conduction studies can help ascertain if axonal or demyelinating pathology which can help guide diagnosis
  • CSF examination: in CIDP or GBS would expect raised protein but normal cell countH
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37
Q

How can the timing of onset of symptoms in spastic paraparesis help to point to the diagnosis?

A

o Acute onset minutes
 Vascular causes but don’t expect spasticity so quickly
o Acute but over a day
 Trauma/disc herniation
o Subacute – days to weeks
 Autoimmune causes
o Weeks to months:
 Nutritional deficiencies or malignancy
o Chronic
 Genetic causes
o Ask about family history, systemic enquiry which may point towards nutritional deficiencies or malignancy

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38
Q

How would a diagnosis of hereditary spastic paraparesis be made?

A
  • Diagnosis normally made from history
  • Slowly progressive picture of walking difficulties and other affected family members
  • Can confirm diagnosis with genetic testing often using next generation sequencing
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39
Q

In addition to MRI what other investigations may be performed in spastic paraparesis?

A
  • Check vitamin B12 and copper levels as deficiencies of these would be reversible causes
  • If family history then genetic screening for conditions such as hereditary spastic paraparesis and referral to the genetic counselling service
40
Q

Give some examples of types of muscular dystrophy (inc. inheritance pattern and rough characteristic features):

A
  • Duchenne’s muscular dystrophy (X linked)
    o Early symptoms (1-3y), proximal LL muscles, speech delay, behavioural or LD, may need wheelchair in teens, life expectancy about 30
  • Becker’s muscular dystrophy (X linked)
    o Similar to Duchenne but milder (have some functioning dystrophin), tend to live to 40-50
  • Oculopharyngeal muscular dystrophy (AD)
    o Presents in 5th decade, ptosis, eyes, swallowing and speech, life expectancy unaffected
  • Myotonic dystrophy (AD)
  • Fascio-scapulohumeral muscular dystrophy (AD)
    o Face and scapula and thoracic regions, more common late teenage years, may affect body asymmetrically, doesn’t shorten life expectancy
  • Limb girdle muscular dystrophy (some AD, some AR)
    o Can present later (like in this case)
41
Q

What features/signs might muscular dystrophy present with (so you need to enquire about)?

A
  • Difficulty walking
  • Weakness
  • Respiratory problems
  • Cardiomyopathy
  • Waddling gait
  • Gower’s sign (weird way of getting up)
42
Q

How would you investigate suspected muscular dystrophy?

A
  • Hx and examination
  • Blood tests:
    o Creatinine phosphokinase (specific to intracellular leakage and not affected by other things), 3 elevated levels 1 months apart = diagnostic (but levels may decrease with muscle mass
  • Genetic testing
  • Electromyography
  • US
  • Muscle biopsy = definitive
43
Q

What is the general approach to management of muscular dystrophy?

A
  • MDT approach to reach needs of patient
  • PT for muscle strengthening and flexibility
  • Physical walking aids/braces
  • Low impact exercise
  • OT – adaptations in the home or work place
  • Pharmacological options:
    o Steroids can slow muscle weakening
    o Creatinine supplements
    o Ataluren (for children with Duchenne over 5 who can still walk)
    o Cardiac meds if have cardiomyopathy
  • Surgical:
    o Can be some options depending on type
    o Pacemaker may be needed
    o Gastrosotomy or PEG feeding
44
Q

What is the general approach to management of idiopathic parkinson’s disease and what medications can be used?

A
  • MDT approach and holistic tailored to patient
  • OT – modifications to help in home/help with eating/ADLs
  • PT – help improve mobility, can have problems with sleep and difficulty turning at night (which can lead to low mood as sleep deprived) so physios can give exercises to help turn at night which can significantly improve QoL
  • Mood management - therapies and anti depressant medications
  • Dietician
  • SALT
  • Specific medications:
    o Levodopa combined with peripheral decarboxylase inhibitors (to reduce peripheral conversion of levodopa to dopamine which helps reduced SEs of N&V)
    o Dopamine agonists
    o Dopamine breakdown inhibitors (monoamine oxidase B inhibitors and catechol-O-mehtyl transfer inhibitors)
45
Q

What are the possible causes of Parkinsonism?

A
  • Idiopathic Parkinson’s disease
  • Drug induced – metoclopramide, methyldopa, sodium valproate
  • Post encephalitis
  • Wilson’s disease
  • Parkinsons plus syndromes (neurodegenerative conditions which have features of Parkinson’s disease but have an extra distinguishing feature such extra-pyramidal, cerebellar, ocular signs) – also may have poor response to levodopa or early dementia
    o Progressive Supranuclear Palsy most common (think if early falls)
    o Multi system atrophy syndromes – characterised by varying features of Parkinsonism plus cerebellar signs, autonomic instability and pyramidal signs (think this if these are prominent)
    o Diffuse Lewy bodies disease – poor cognition
    o Corticobasal generation – higher cerebral dysfunction
46
Q

How is a diagnosis of idiopathic parkinson’s disease made?

A

Investigation/diagnosis of Parkinson’s disease:
- Predominantly clinical (2 out of 4 cardinal signs positive inc. bradykinesia)
- Dopamine transporter SPECT imaging – reduced and asymmetrical uptake of striatal dopamine biomarkers
- CT/MRI to rule out alternative diagnoses

47
Q

What are the 4 cardinal symptoms of parkinsonism?

A
  1. Tremor
  2. Rigidity
  3. Bradykinesia
  4. Postural instability
48
Q

What features point more towards a diagnosis of idiopathic Parkinson’s disease rather than other causes of Parkinsonism?

A
  • Unilateral onset and persistent asymmetry
  • Resting tremor
  • Good response to levodopa
  • Levodopa induced dyskinesia
  • Long disease course (>10y)
49
Q

What is the basic pathology of Parkinson’s disease?

A
  • Neurodegenerative disease characterised by degeneration of substantia nigra dopaminergic neurons
50
Q

How is a basic swallow assessment performed?

A
  • Especially if suggestion of language problems on assessment
  • If a patient has R sided weakness (suggesting a L sided lesion) it is worth thinking about as in most patients language is left hemisphere lateralised
  • Basic speech assessment:
    o Comprehension (deficit in this affects rest of assessment), give simple instructions then progress to more complex commands
     Do 1, 2 then 3 step commands (eg. Open your eyes, touch your R ear with left hand etc)
    o Spontaneous speech eg. Ask what was for breakfast
    o Naming (hold up a pen and ask to name)
     Word retrieval difficulties and hesitancy
    o Speech production
     Well articulated or dysphonic – ‘baby hippopotamus’ or ’42 west register street’
51
Q

What is the basic management of migraine?

A

o Acute
 Simple analgesia (para/NSAID) and antiemetic
 Avoidance of opioids
 SC/intranasal or PO triptans is next step
o Prophylaxis
 For pts who have multiple per month o chronic migraine
* Propranolol and Topiramate
* Ensure no analgesia overuse
o Lifestyle measures: exercise, good sleep hygiene
 Avoidance of triggers
 Withdrawal or reducing in caffeine
 Good fluid intake
 Good sleep hygiene

52
Q

What are the acute and chronic causes of chorea?

A
  • Acute onset
    o Stroke (classically asymmetrical) – lesion of subthalamic nucleus causing hemiballismus
    o Hyperglycaemia (do a CBG)
  • Chronic
    o Polycythaemia
    o Post infection – Syndenham’s chorea but less common now
    o SLE
    o Huntington’s - ask about family Hx
53
Q

How would you investigate Chorea?

A
  • Thorough history
    o Acute – CTH, CBG
  • Symptoms associated with SLE
    o SLE – dsDNA, ANA, anti phospholipid Ab
  • Family history
    o Genetic testing in form of blood test but normally need referral to clinical genetics team to help with this
54
Q

What is the management approach to Huntington’s disease?

A
  • Trinucleotide repeat disorder which shows the phenomenon of anticipation
  • Management:
    o No disease modifying therapies exist currently
    o Specialist multi disciplinary
    o Nuerologist identifying treatments which can be treated
    o Neuropsychiatrist – associated mood disorders
    o Dieticians – have an increased basal metabolic rate with the involuntary movements
    o OT – adaptations to the home and work place to be able to maintain independence
    o PT – help maintain strenght
    o Close links with research facilities as the field moves towards clinical trials for potential disease modifying treatments
55
Q

What clinical features and tests can help distinguish between a NMJ disorder and a myopathy in a patient with weakness but no sensory or UMN signs?

A

Typical of NMJ:
- Fatigable weakness, ptosis, ocular paresis
Neurophysiological studies, EMG:- In NMJ disorders – repetitive stimulation shows decrement in action potentials
- Myopathy – characteristic changes on EMG

56
Q

How would you investigate a suspected myasthenia gravis?

A
  • Most patients seropositive so first line is acetylcholine receptor Ab, if this is negative then can do anti MuSK (anti muscle specific kinase) Ab
  • If seronegative then neurophysiological studies
    o Single fibre EMG can demonstrate characteristic changes of an NMJ disorder and have a diagnosis of seronegative Myasthenia gravis
    CT chest in everyone ?thymoma - resect if present
57
Q

What is the management of myasthenia gravis?

A

Myasthenic crisis (increasing weakness and double vision, haemodynamic instability and reduced chest expansion, can be triggered by infection or any stressor)
- Approach A-E and resuscitate as necessary
- IP care and monitoring
- Monitor FVC early, escalation to ITU
- IVIG or plasma exchange may be required
Subacute presentation
- HX and exam
- Neurophysiological studies
- Antibody testing
- CT chest for thymoma in everyone – if present resect
- Pyridostigmine helps with symptoms but doesn’t modify disease
- Then modify disease with immune suppression with initially steroids
o When first start steroids can have a ‘steroid dip’ where you get worsening of symptoms so in people with already severe symptoms may need IP admission for initial steroid titration so can monitor closely for worsening symptoms, if done as an OP start at a low dose and titrate slowly
- Then titration of steroid sparing agents
- If resistant to these treatments then specialist may consider IVIG

58
Q

What is the inheritance pattern of Friedrich’s ataxia?

A
  • Commonest genetic ataxia
  • Trinucleotide repeat disorder, autosomal recessive inheritance, mutation in frataxin gene
59
Q

Which parts of the nervous system are affected in Friedrich’s ataxia and what signs do you therefore get?

A
  • Affects spinocerebellar tracts, pyramidal tracts, dorsal columns, cerebellum and medulla (UMN, LMN, sensory ataxia and cerebellar ataxia signs)
60
Q

How would you investigate suspected Friedrich’s ataxia?

A
  • Confirm diagnosis by genetic testing
  • Structural imaging brain and spinal cord to rule out alternative or co-existing pathology
  • Vitamin B12 and Vitamin E as deficiencies can produce similar clinical signs
61
Q

How would you approach management of Friedrich’s ataxia and what complications do you monitor for as part of that?

A
  • MDT management
  • Cardiomyopathy and arrythmias common cause of mortality – regular echo and ECG, if symptomatic with palpitations then also use ambulatory ECGs for exercise provoked arrythmias. Involvement of cardio needed – may include implantable devices
  • Can develop impaired glucose tolerance – monitor and treat when develops
  • Can also develop optic atrophy and hearing loss
  • Supportive care as no DMARDs
  • PT, OT (assessment home, workplace and transportation to ensure appropriate) , orthotics, speech therapy
62
Q

What can help distinguish between Friedrich’s ataxia and Spinocerebellar ataxias?

A
  • Spinocerebellar ataxia (AD inheritance, most are trinucleotide repeat disorders, UMN and extrapyramidal signs, peripheral neuropathy, ophthalmoplegia) but FA tends to have a sooner onset and people lose ability to walk by their 20s, most spinocerebellar ataxia, onset of symptoms after age of 18
63
Q

How would you distinguish between ocular myasthenia and generalised myasthenia and why is it an important distinction?

A
  • If someone presented with just ocular symptoms would need to monitor for 2y to see if developed generalised symptoms as if they are going to develop them it will happen in this space of time. If don’t develop within those 2 years you can be more confidence of diagnosis of ocular myasthenia
  • Important distinction as ocular myasthenia is often milder and often only requires pyridostigmine
64
Q

How can you distinguish classically between a surgical and medical third nerve palsy and what would be the respective causes?

A

Surgical causes classically also affect the peripheral parasympathetic fibres leading to a dilated pupil and may be painful, in medical causes the pupil is classically not affected
Surgical causes include: Posterior communicating artery aneurysm, SOL, raised IC pressure with cerebral uncal herniation, cavernous sinus pathology (IV,V,VI may also be affected in this case)
Medical causes include: mononeuritis multiplex (eg. DM), midbrain infarction (Weber’s syndrome), midbrain demyelination (MS) and migraine

65
Q

What is Weber’s syndrome?

A

3rd nerve palsy with contralateral hemiparesis - suggests a lesion of cerebral peduncle

66
Q

What are the features of Wallenburgs syndrome and where is the lesion?

A

Lateral medulla supplied by posterior inferior cerebellar artery
Features:
- Cerebellar symptoms
- Ipsilateral Horner’s syndrome
- Ipsilateral facial pain and temp loss
- Ipsilateral bulbar weakness
- Contralateral body pain and temp loss

67
Q

What is the typical history of bell’s palsy?

A

Isolated 7th nerve palsy, coming on over hours to days, may be preceded by hyperacusis, altered taste or post auricular pain, tends to recover within a couple of months

68
Q

How do you treat Bell’s palsy?

A

High dose steroids (tends to be if started within 1/52) and eye lubricants and tape to protect for exposure keratitis. Inconclusive evidence base for use of antivirals but would use if evidence of herpes zoster in ear canal

69
Q

What are the possible causes of a 7th nerve palsy?

A

Anatomical causes anywhere along the route (pons, cerebellopontine angle, internal auditory meatus, parotid gland) - SOL, bony compression, trauma or vascular malformation
Infective: herpes zoster, lyme’s disease, HIV (think of the latter 2 especially in bilateral)
Infiltrative: sarcoidosis, amyloidosis
Inflammatory: Guillain barre syndrome, miller fisher syndrome, sjogren’s syndrome
Ischaemia

70
Q

How would you investigate a 7th nerve palsy?

A

FBC and CRP, HbA1c
Otoscopy
Urine dip for glucose
Special tests: Serology (HIV, Lyme), autoimmune screen
Might consider LP
MRI if concerning features for IC lesion

71
Q

What are the features of Horner’s syndrome?

A
  1. Partial ptosis: affects upper and lower lid
  2. Apparent enophthalmos: Due to above
  3. Miosis
  4. Anhidrosis and flushing
    - First order (brain to spinal cord): Ipsilateral body
    - Second order (spinal cord to neck): Ipsilateral face
    - Third order (neck to pace): Absent or just above the brow
72
Q

Causes of Horner’s syndrome?

A

Congenital: birth injury to sympathetic chain (Klumpke’s) or hereditary
First order: Stroke (Wallenburgs), SOL, demyelination (MS), syringomyelia - unlikely to be isolated Horner’s as would get other neurological signs with it
Second order: trauma, pancoast tumour, schwannoma
Third order: trauma, carotid artery dissection, venous sinus thrombosis, cluster headaches, migraines

73
Q

What is the most worrying diagnosis if a patient has neck/face pain at onset of Horner’s?

A

Carotid artery dissection - need urgent CT or MR angiography

74
Q

How would you investigate a Horner’s syndrome?

A

Dependent on cause
Imaging and choice would depend on other signs/symptoms but could include:
MRI brain (first order neuron)
MRA and/or carotid doppler (2nd and 3rd)
MRI cavernous sinus and orbits (3rd order)

75
Q

Which nerves run through the cavernous sinus?

A

III, IV, VI, Va,Vb (essentially eye movements and facial sensation) and also the ICA

76
Q

Which nerves would be affected in a cerebellopontine angle tumour?

A

V, VII, VIII and cerebellar signs

77
Q

What nerves would be affected by a lesion affecting midbrain?

A

III and IV

78
Q

What nerves would be affected by a lesion affecting the pons?

A

V, VI, VII and VIII

79
Q

What nerves would be affected by a lesion affecting the medulla?

A

IX, X, XI and XII

80
Q

What are the different types of stroke according to the Bamford classification?

A

Total anterior stroke (worst prognosis): All 3 of homonymous hemianopia, motor-sensory deficit affecting 2/3 of face, arm, leg 3) Some evidence of higher dysfunction (dysphasia, neglect, asteroagnosis, reduced GCS)
Partial anterior stroke: 2 out of 3 things for PACS present
Posterior circulation stroke: Cranial nerve deficit with contralateral motor-sensory deficit, conjugate eye movement disorder, bilateral motor-sensory deficit, cerebellar dysfunction or isolated homonymous hemianopia.
Lacunar stroke: Discrete symptoms affecting 2/3 of face, arm or leg (pure motor, pure sensory, both or ataxi hemipareses)

81
Q

Complications of spina bifida?

A
  • Leg spasticity
  • Development of neuropathic bladder
  • Hydrocephalus – needs VP shunt
  • Arnold Chiari malformation
  • Recurrent meningitis
  • Syrinxes
82
Q

Risk factors for development of spina bifida?

A
  • Folate deficiency
  • Family history of neural tube defects
  • Medications:
    o Anti epileptics such as sodium valproate
    o Methotrexate
  • Diabetes (esp poorly controlled)
83
Q

Differentials for bulbar weakness?

A
  • Botulism (pupillary muscles involved and progresses fast
  • Miller Fisher syndrome
  • Guillan barre syndrome
  • Lambert Eaton Myasthenic syndrome (SCLC, symptoms worse in morning and improve as days goes on – repetitive stimulation results in improved potentials and voltage gated calcium channel Ab present)
  • Myasthenia gravis (worse at end of day and pupils never involved)
84
Q

Differentials for ocular symptoms of double vision and ptosis?

A
  • Thyroid ophthalmopathy – CT of orbits and TFTs
  • Brainstem and cranial motor weakness
  • Myotonic dystrophy
85
Q

Steroid side effects?

A
  • Cushingoid appearance
  • Skin thinning easy bruising
  • Adrenal failure
  • CVD
  • DM
86
Q

What broadly is Sturge Weber syndrome?

A

Congenital condition
Port wine stain (in distribution of some or all of trigeminal nerve)
Vascular eye abnormalities
Glaucoma
Leptomeningeal angiomas
Often get seizures
Often intellectual disability

87
Q

What are the differentials for bilateral gradual visual loss?

A
  • Cataracts (can also affect night time problems) – can occur prematurely in diabetics or steroid users
  • Glaucoma
  • Diabetic retinopathy (do a CBG in them all)
  • Macular degeneration
  • Vitamin A deficiency rare – might get in CF
  • Retinitis pigmentosa (night time visual problems then progressive to tunnel vision)
  • Papilloedema – IIH
  • Leber’s optic neuropathy
  • Rare causes of optic neuropathy
88
Q

What is the management of retinitis pigmentosa?

A
  • Diagnosis by ophthalmology
  • Referral to genetic counsellor
  • No treatment atm
  • Supportive – OT
  • DVLA guidelines on driving
  • Progressive condition – most people registered blind by middle age
89
Q

What are the associations of retinitis pigmentosa and what other symptoms point towards a syndrome rather than isolated retinitis pigmentosa?

A
  • Associations (but can occur in isolation)
    o Think if evidence of cerebellar signs or peripheral neuropathy
    o Kearns-Sayre syndrome
    o Usher syndrome – with sensorineural deafness
    o Alstroms syndrome
    o Refsums syndrome – with muscle weakness and ataxia
90
Q

What is the inheritance of retinitis pigmentosa?

A

AD, AR or X linked, sometimes de novo

91
Q

Post stroke complications?

A
  • Thromboembolic disease – so pneumatic mechanical compressions devices to prevent DVT
  • Further stroke – ischaemic or haemorrhagic
  • In large strokes can get swelling requiring craniectomy
  • Seizures - acute and long term
  • Aspiration pneumonia – needs SALT assessment as risk of swallow deterioration
  • Spasticity in long term – ongoing physio and OT, sometimes medical treatment for this
  • High risk falls – reduced mobility, confusion, reduced cognition
  • Increased risk urinary and faecal incontinence
92
Q

Important secondary prevention in stroke?

A
  • Appropriate antiplatelet agents
  • Managed BP
  • Cholesterol – lifestyle modifications, increased fruit and veg, statins
  • Reduced salt intake
  • Reduced alcohol intake
  • Stop smoking
  • Identify and manage diabetes
93
Q

Causes of CVA in the young?

A
  • Anti phospholipid syndrome – need to ask about pregnancies and miscarriages
  • PFO
  • Extracranial dissection – trauma or hyper extension injuries
  • Embolic cause: cardiac myxoma, valvular disease, atrial fibrillation, subacute endocarditis
  • Vasculitis
  • Substance abuse – cocaine and methamphetamine
  • Rarer - Metabolic problems and mitochondrial conditions
94
Q

Management of anti phospholipid syndrome and complications?

A
  • Management: anticoagulation with warfarin
  • Cx: livedo reticularis, thrombocytopenia, thrombophlebitis, DVT, PE, recurrent fetal loss
95
Q

Investigations in anti phospholipid syndrome?

A

ENA, ANA, ds DNA, complement, anti cardiolipin Ab and anti phospholipid Ab and lupus anticoagulant and clotting screen

PACES (7 decks)

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