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MRCP PACES revision > Neurology > Flashcards

Neurology Flashcards

1
Q

Clinical Features of Myotonic Dystrophy

A 
Myotonia (and percussion myotonia)
Frontalis/Temporalis wasting
Bilateral ptosis
Distal myopathy
Reduced reflexes
Dysarthria/nasal speech
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2
Q

Systems involved in Myotonic dystrophy

A

Ocular: Cataracts
GI: Dysmotility (dysphagia, delayed gastric emptying)
Endocrine: Diabetes, Gynaecomastia, Testicular atrophy
Cardiovascular: Cardiomyopathy, Conduction defects
Respiratory: Sleep apnoea, recurrent LRTIs/aspirations
Other: slow wean from general anaesthetic

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3
Q

Investigations in myotonic dystrophy

A

EMG: repetetive action potentials after a single stimulus
Annual ECG
Diabetes screening (HbA1c, blood sugars)
Slit lamp (cataracts)
Lung function testing

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4
Q

Management of Myotonic dystrophy

A

Non-medical:

  • MDT approach: physiotherapy, SALT, OT
  • Genetic counselling and prenatal diagnosis
  • Anaesthetic risk counselling

Medical

  • Phenytoin for myotonia
  • Diabetes Screening
  • Surveillance for:
  • -Cataracts
  • -Respiratory failure
  • -Cardiomyopathy/conduction defects
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5
Q

Genetics of myotonic dystrophy

A

Mutation in DMPK gene (chromosome 9) - affects ion channels
Autosomal dominant
Trinucleotide repeat pattern (CTG)
Anticipation

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6
Q

Causes of myopathy

A

Congenital:

  • Muscular Dystrophies
  • –Axial: Duchenne; Becker; Limb Girdle
  • –Craniofascial: Fascioscapulohumeral; Oculopharyngeal
  • –Distal myopathies
  • Myotonic dystrophy
  • Congenital myopathies (nemaline, central core)
  • Muscle ion channel disorders
  • Metabolic myopathies (glycogen, fatty acid disorders)
  • Mitochondrial cytopathies

Acquired

  • Inflammatory
  • –Polymyositis
  • –Dermatomyositis
  • –assoc with CTD
  • Inclusion body myositis
  • Drug induced (statins most common)
  • Metabolic
  • Endocrine
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7
Q

Drug Induced Myopathy

A 
Statins
Fibrates
Antimicrobials: rifampicin, sulfonamides, zidovudine
ACEi
Cochicine
Penicillamine
Corticosteroids
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8
Q

Inheritance of Myopathies

A

X-linked

  • Duchenne
  • Becker
  • Emery Dreifuss

AD

  • Myotonic dystrophy
  • Facioscapulohumeral dystrophy
  • Limb-girdle
  • Oculopharyngeal
  • Periodic paralyses

AR

  • Limb girdle
  • Metabolic myopathies
  • Mitochondrial
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9
Q

Patterns of weakness in muscle disorders

A

Distal>proximal

  • Inclusion body (forearm flexors, quads)
  • FSHD
  • Myotonic dystrophy

Proximal>distal

  • Becker/DMD
  • Limb girdle
  • Inflammatory/endocrine
  • Drug induced

Face with external ocular muscles involved

  • Graves myopathy
  • Mitochondrial disorder (ptosis, deafness, retinitis pigmentosa)
  • MG

Calf pseudohypetrophy
-Becker & Duchenne

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10
Q

Myopathy investigations

A

Bloods

  • CK
  • FBC, U&E, LFT, ESR/CRP, bone profile, BM
  • TFT, ANA, ANCA, RF

Imaging

  • CXR
  • Echocardiogram
  • Malignancy screening - CT TAP +- PET

Functional

  • ECG
  • EMG
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11
Q

Features of Dermatomyositis

A 
Acute/subacute symmetrical proximal weakness
Dermatological: 
-Heliotrope rash - Violaceous eyelids
-Rash on V of chest/upper back
-Gottron's papules
-Nailbed capillary

Respiratory
-Lung fibrosis

Cardiac
-Myopericaridtis

Joints/soft tissues

  • Arthropathy (large, symmetrical)
  • Raynaud’s

Kidneys

  • Nephrotic syndrome
  • MSGN
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12
Q

What are the types of motor neurone disease?

A
  1. Amyotrophic Lateral Sclerosis
    - -UMN and LMN
  2. Primary lateral sclerosis
    - -Predominantly UMN
    - -No wasting
  3. Progressive muscular atrophy
    - -Predominantly LMN
  4. Progressive bulbar palsy
    - -Bulbar onset
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13
Q

How would you manage a patient with MND?

A
  1. MDT approach:
    - Neurologist
    - MND nurse specialist
    - SALT
    - Physio
    - OT
    - Dietician/Nutrition team
    - Respiratory (NIV)
    - Palliative input
  2. Symptom management
    - Spasms (splints, baclofen)
    - Respiratory failure (NIV)
    - Drooling (anticholinergics)
    - Pain (analgesia)
    - Poor swallow (enteral feeding, SALT)
  3. Medical
    - Riluzole
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14
Q

How would you investigate for MND?

A
  1. Predominantly a clinical diagnosis
    - El escorial criteria
    - 4 body sections
    - Definite diagnosis is UMN & LMN in 3 sections
  2. EMG
  3. MRI brain/spinal cord
    - r/o other causes
  4. Bloods
    - TSH, HIV, lyme serology
    - CK can be raised
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15
Q

Examination findings in MND

A
  1. UMN and LMN signs
  2. Normal sensory/co-ordination signs
  3. Wasting
  4. Fasciculations
  5. Reduced power with variable tone
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16
Q

What is the clinical course of MS?

A
  1. Relapsing Remitting
    - Becomes secondary progressive
  2. Primary progressive
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17
Q

How do you diagnose MS?

A

McDonald Criteria
1. ≥2 attacks and ≥2 objective lesions

2. ≥2 attacks and 1 objective lesion
With dissemination in space:
a) oligoclonal bands
b) ≥2 MRI lesions
c) Further attack involving different site
  1. 1 attack and ≥2 objective clinical lesion
    With dissemination in time:
    a) MRI
    b) Second attack
  2. 1 attack and 1 objective lesion
    a) DIS shown by MRI/positive CSF
    b) DIT shown by MRI, second attack
  3. Insidious neurological progression suggestive of primary progressive MS
    a) CSF positive
    AND
    b) DIS shows by MRI evidence of 9 brain lesions, 2 spinal cord lesions, or mixture of brain/spinal OR positive visual evoked potential with 4-8 MRI lesions

c) DIT shown by MRI or continued progression over a year

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18
Q

Risk factors for MS

A
  1. Female x2 risk
  2. EBV
  3. Smoking
  4. Latitude
  5. Vitamin D deficiency
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19
Q

What is a clinically isolated syndrome, and how do you manage it?

A
  1. The initial episode of demyelination
  2. Investigations
    - Bloods (ANA, ANCA, ENA, RF, complement, ESR, aquaporin 4, antiphospholipid)
    - MRI - If positive:
    - –LP
    - –Visual evoked potential
  3. Consider steroids
  4. Education
    - 15% get MS if MRI negative
    - 80% get MS if MRI positive
20
Q

How do you investigate for MS?

A
  1. MRI
    - Gadolinium shows lesions
  2. LP
    - oligoclonal bands
  3. Visual evoked potentials
    - -unilateral delay
  4. Bloods
    - ANA, ANCA, ENA, dsDNA
    - ESR, B12, complement
    - NMO-IgG (devic’s disease)
21
Q

What are the treatment options in MS?

A

MDT approach

Relapses: 1g methylpred for 3 days

1st line DMARDS:

  • IFN Beta analogues
  • -avonex
  • -betaferon
  • -rebif
  • Glatrimir acetate
  • Nataluzimab
  • Fingolimod

Aggressive disease

  • Cyclophosphamide
  • Rituzimab/IVIG/PEX
22
Q

What are the cardinal features of parkinsonism?

A
  1. Tremor
  2. Rigidity
  3. Bradykinesia
  4. Postural instability
23
Q

What are the causes of parkinsonism?

A

Congenital

  • -Familial forms (<5%)
  • -Wilson disease

Acquired:
–Idiopathic PD

Secondary parkinsonism

  1. Drug induced
    - -Antidopaminergics (metoclopramdie, neuroleptcis, valproate)
  2. Neurodegenerative disorders
    - -Lewy Body dementia
    - -Alzheimer’s
    - -Normal pressure hydrocephalus
  3. Vascular parkinsonsim
  4. Head trauma (pugilist)
  5. Infection (encephalitis, prion disease, HIV)
  6. Paraneoplastic
  7. MPTP, Carbon monoxide
24
Q

What is the pathophysiology of parkinson disease?

A

Degeneration of dopamine neurones in substantia
–lewy bodies
–alpha synuclein protein aggregates
Reduced dopaminergic transmission –> projects to basal ganglia –> initiates movement
Other neurotransmitters (ACh, 5-HT, NA) - non movement manifestiations of PD

25
Q

What are the clinical features of bradykinesia in PD?

A

Head

  • Mask like facies
  • Reduced blinking

Arms/trunk

  • Reduced arm swing
  • Micrographia
  • Reduced gesturing
  • Poor rhythmic movements (cutting food, fingers to thumb, foot tap)

Legs

  • Festinating gait
  • Turn en bloc
26
Q

What are the clinical features of rigidity in PD?

A

Cogwheel rigidity
Lead pipe rigidty
Increases if distracting monouevre in other limb

27
Q

What are the clinical features of tremor in PD?

A

Pill rolling tremor (85%)

  • 3-5Hx
  • Initially unilateral - can be trunk, legs, lips
  • Not head
  • At rest and with movement
  • worse with distraction (e.g. couting from 100)
28
Q

What are the clinical features of postural instability in PD?

A

Posture: fixed flexion of neck/trunk
Balance loss: loss of righting reflex
Gait: festinating

29
Q

What are the alternative features of PD? (I.E not bradykinesia, rigidity and tremor)

A
  1. Autonomic
    - -Postural hypotension
    - -Constipation
    - -urinary urgency/frequncy
    - -Sialorhoea (drooling)
  2. Neuropsychiatric
    - -Punding (mechnical objects, repetitive behaviours)
    - -Depression
    - -Impulse control (gambling, sexual, shopping,escalating use of L-dopa)
    - -Dementia
  3. Sleep disturbance
    - -Unable to turn in bed
    - -Resless legs
    - -REM sleep disturbance
30
Q

What are the Parkinson Plus syndromes?

A
  1. Supranuclear palsy (SNP)
    - -Loss of superior gaze & voluntary downward gaze
    - -Early falls/postural instability, pseudobulbar dysarthria
  2. Multiple system atrophy (MSA or Shy-Drager syndrome)
    - -Parkinsonism
    - -Cerebellar features
    - -Corticospinal tract signs (++reflexes)
    - -Autonomic features ++ (bladder, postural BP)
  3. Corticobasal degeneration (CBD)
    - -Clumsy limb - often rigid arm
    - -Alien limb syndrome
    - -Cognitive impairment early
    - -Dystonia, dysphagia, dysarthria
31
Q

How do you diagnose parkinson disease?

A
  1. Identify parkinsonism
  2. Apply exclusion criteria
  3. Elicit postive supporting criteria
32
Q

What are the exclusion criteria for idiopathic PD in parkinsonian patients?

A
  1. Repeat strokes
  2. Repeat head injury
  3. Drugs known to cause parkinsonism
  4. Encephalitis
  5. > 1 Family members affected
  6. Sustained remission
  7. No L-dopa response
  8. Unilateral after 3y
  9. exposure to neurotoxin
    10 Hydrocephalus
33
Q

What are supporting features for Idiopathic PD?

A

> 2 of:

  1. Unilateral onset
  2. persistent asymmetry
  3. Rest tremor
  4. Progressive
  5. L-dopa response >5y and chorea after 10y
34
Q

How do you investigate suspected PD?

A

Main aim is to avoid incorrect diagnosis

  1. CT head/MRI
  2. DaT scan
    - -To distinguish PD vs Essential tremor
    - -Functional imaging of dopaminergic system
    - -SPECT - FP-Cit tracer - binds to DA transporters
    - -Loss of “tail of the comma”
35
Q

How do you manage Parkinson disease?

A

MDT approach

  • -Physio
  • -OT
  • -Nutrition/dieteician team
  • -Palliative
  • -PD CNS
  • -Neurologist

Education
Pharmacological therapy

36
Q

What are the Pharmacological options in PD?

A

Drugs are symptomatic, not prognostic
Start when symptoms intefering with daily life

  1. Dopamine Agonists (enhance dopamine effects)
    - -Ropinerole
    - -Pramipexole
    - -Rotigotine (patch)
  2. L-dopa therapy (& decarboxylase inhibitor)
    - -Madopar
    - -Sinemet
  3. COMT/MAOIs (prevent dopamin breakdown)
    - -Entacapone
    - -Seligiline/rasigiline

General Approach

  1. Young/fit/mild disease
    - -Dopamine agonist/MAOb first
    - -Then L-dopa & COMT
  2. Older, severe, comorbid
    - -L-dopa first
    - -Then MAOi/COMT
  3. Managing wearing off of Ldopa
    - -MR preparations of l-dopa
    - -COMT
    - -DBS
    - -Duodopa
    - -Apromorphine
37
Q

Name some Dopamine agonists and their main effects/side effects?

A

Examples

  • Oral
  • –Ropinerole
  • –Pramipexole
  • Transdermal
  • –Rotigotine
  • Subcutaneous
  • –Apormorphine

Advantages - spare L-dopa
Disadvantages - dopaminergic side effects, hypersomnolence, impulse control
Not for dementia patients

Ergot derived (fibrosis - lung/retroperitoneal/cardiac)

  • Cabergoline
  • Pergolide
38
Q

What are COMT inhibitors and MAO inhibitors?

A

Good agents to prevent wearing off effect in L-dopa use

COMT (catechol-O-methyltransferase inhibitors)

  • Entacapone
  • Increase half life of l-dopa, reduces dose requirement
  • Disadvantages: dopamine side effects, orange urine, diarrhoea, dyskinesias

MAO type b inhibitors

  • Rasagiline
  • Seligiline
  • Possible neuroprotective role
  • side effects: dry mouth, headache, urinary, depresison
39
Q

Features of Horner Syndrome

A

Ptosis
Constricted pupil
Anhydrosis

40
Q

What are the causes of Horner Syndrome

A

A lesion anywhere along the sympathetic outflow tract from hypothalamus to the eye

1st order neuron (hypothalamus –> spinal cord)

  • CVA (ischaemia/bleed)
  • Tumour
  • Inflammatory (vasculitis, granulomatous)
  • Demyelination
  • Syringomyelia

2nd Order Neuron (spinal cord to sympathetic chain)
-Tumour in neck/mediastinum (e.g. pancoast, thyroid/mediastinum)

3rd order neuron (chain –> carotid –> middle fossa)

  • Trauma
  • carotid dissection/aneurysm
41
Q

Causes of extensor plantar reflex with absent ankle reflexes

A
  1. Subacute combined degeneration of the cord (B12 deficiency)
  2. Motor neuron disease
  3. Friedreich’s ataxia
  4. Tabes Dorsalis (syphilis)
    (5) . Dual pathology e.g. spastic paraparesis & peripheral neuropathy
42
Q

What is the cerebellopontine syndrome and what causes it?

A
  1. CN V, VII and VIII
    - can get gaze palsies/cerebellar involvement if large lesion
  2. Causes
    - Middle fossa tumours (acoustic neuroma, cholesteatoma, meningioma etc)
    - Vascular - vertebrobasilar artery enlargement
    - Meningeal infection (TB, syphilis)
43
Q

What are the examination findings in syringomyelia?

A
  1. Dissociated sensory loss
    - Cape distribution spinothalamic (pain/temperature)
    - Spared dorsal columns (vibration/position)
  2. Bilateral weakness/wasting and loss of upper limb reflexes
  3. Other findings
    - Lower limb weakness/UMN signs if severe
    - Horner Syndrome
    - Charcot Joint
    - Kyphoscoliosis
44
Q

Causes of syringomyelia

A
  1. Chiari malformation
  2. Intrinsic cord tumour
  3. Idiopathic
45
Q

What are the examination findings in spinal cord lesions?

A
  • Spastic paraparesis
  • -Increased tone
  • -Pyrmaidal weakness (Legs flexor >extensor)
  • -Hyper-reflexia/upgoing plantars
  • -Look for sensory level (above L1)
  • Urinary catheter
  • Spinal surgical scars
46
Q

Causes of Spinal Cord lesions

A
  1. Compressive
    - -Trauma
    - -Spondylopathy/prolapse
    - -Tumour
2. Non compressive
Hereditary
--HSP (hereditary spinal paraparesis)
Non-hereditary
--Vascular (bleed/ischaemia)
--Inflammatory
--Demyelinating
--Infective
--Metabolic
47
Q

What are the driving restrictions for patients with loss of consciousness?

A

Unrestricted
-Clear vaso-vagal

4 weeks
-Syncope with no clear cause

6 Months:

  • Seizure
  • > 1 episode in 6 months
  • Occured at wheel, significant injury, sitting/lying down

12 months
-Epilepsy diagnosed

MRCP PACES revision (8 decks)

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