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Medicine > Neurology > Flashcards

Neurology Flashcards

1
Q

Inheritance pattern of Huntington’s disease

A

Autosomal Dominant

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2
Q

Contents of the cavernous sinus

A 
OTOMCAT:
OTOM = lateral wall
CA = within sinus, joining to T
Occulomotor n
Trochlear n
Ophthalmic division of CNV
Maxillary division of CNV
internal Carotid artery
Abducent n
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3
Q

Ramsay Hunt syndrome

A

Herpes zoster otitis - reactivation of VZV in geniculate ganglion

Ipsilateral facial paralysis, ear pain and vesicles in auditory canal

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4
Q

Ipsilateral facial paralysis, ear pain and vesicles in auditory canal

A

Ramsay Hunt syndrome

VZV reactivation in geniculate ganglion

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5
Q

Cause of myasthenia gravis

A

Antibodies to anticholine receptor at post synaptic membrane

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6
Q

Regulators of cerebral blood flow

A

Partial pressure of CO2 and O2

  • hypercapnia increases flow
  • hypoxia increases flow
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7
Q

Definition of Parkinson’s disease

A

Progressive neurodegenerative disorder characterised by rigidity, tremor, postural instability and bradykinesia due to a loss of dopamine in the neostriatal pathway

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8
Q

Pathophysiology of Parkinson’s disease

A

Abnormal aggregation of alpha synuclein (Lewy body constituent)
Loss of pigmented dopaminergic neurons in substantia nigra pars compacta of midbrain
-loss of DA in neostriatal pathway (esp putamen)

60% of these neurons have degenerated before clinical features develop

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9
Q

Clinical features of Parkinson’s disease

A

Tremor (resting)
Rigidity (cogwheel/leadpipe)
Akinesia/bradykinesia
Postural instability

+/- autonomic features (bowel, bladder, orthostatic dizziness)
+/- anosmia
+/- fatigue and nonspecific discomfort
+/- neuropsychiatric (anxiety, depression, sleep disruption)
Insidious onset

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10
Q

Parkinsonian gait features (9)

A 
Hesitation in starting
Shuffling
Freezing
Propulsion
Retropulsion
Reduced arm swing
Festination (short, accelerating steps)
Difficulty stoping
Difficulty turning (multi point turn)
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11
Q

Pharmacological management options for PD

A 
Dopamine replacement
Dopamine D2 receptor agonists
Combination dopaminergic/antiCh
COMT inhibitors
MAOB inhibitors
ACh inhibitors
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12
Q

Dopamine replacement therapy in PD

A

Levo-dopa crosses BBB, converted to DA within CNS by dopa decarboxylase (DDC)

DDC inhibition to prevent L-dopa being converted in periphery
(carbidopa, benserazide) - cannot cross BBB

Standard treatment = L-dopa + peripheral DDC inhibitor

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13
Q

DDC inhibitors

A

Carbidopa, beserazide

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14
Q

Adverse effects of L-dopa

A

Nausea, vomiting
Postural hypotension
Dyskinesia
Hallucination

In long term, shorter duration of benefit and reduced efficacy as disease progresses

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15
Q

Benefits of L-dopa

A

Improves tremor, bradykinesia and rigidity

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16
Q

Types of dopamine D2 receptor agonists

A 
Non-ergot derivatives:
- pramipexole
Ergot-derivatives:
- cabergoline
- pergolide
- bromocriptine
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17
Q

Mechanism of dopamine D2 agonists in Parkinson’s disease

A

mimics action of DA at D2 receptors in striatum

less marked benefit than L-dopa

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18
Q

Benefits of D2 agonists in PD

A

Longer duration of action than L-dopa
Can be first-line in younger patients to delay L-dopa use OR in combination with L-dopa in late stage disease when need additional response

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19
Q

Adverse effects of DA D2 agonists

A

Nausea, vomiting
Postural hypotension
More likely to cause hallucinations and confusion than L-dopa

ergot-derivatives lead to fibrosis (especially heart valve disease)

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20
Q

Combined preparations for Parkinson’s

A 
Sinemet = L-dopa + carbidopa
Madopar = L-dopa + benserazide
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21
Q

Example of a COMT inibitor

A

Entacapone

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22
Q

Mechanism of COMT inhibitors

A

(catechol-O-methyltransferase - responsible for breakdown of catecholamines)
Inhibition leads to reduced peripheral breakdown of L-dopa, increasing amount delivered to CNS

Short-half life therefore administered with each dose of L-dopa

Used in late disease with “wearing off” phenomenon of L-dopa

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23
Q

Adverse effects of COMT inhibitors

A

dyskinesia
Nausea and vomiting
Dry mouth
Diarrhoea

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24
Q

Example of a MAOB inhibitor

A

selegiline

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25
Q

Mechanism of MAOB inhibitors in PD

A

inhibits MAOB - reduces breakdown of dopamine - prolongs effect of L-dopa

Used in late stage disease (wearing off phenomenon)

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26
Q

Adverse effects of MAOB inhibitors

A

Insomnia

Exaggeration of L-dopa side effects

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27
Q

ACh inhibition in PD

A

Benzotropine (e.g.)
blocks muscarinic receptors - improvement of tremor and rigidity (little effect on bradykinesia)

Mainly used in drug induced parkinsonism

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28
Q

Adverse effects of benzotropine

A 
Drowsiness
Confusion
Restlessness
Dry mouth
Blurred vision
Urinary retention
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29
Q

Drugs to consider adding to patient with PD suffering from “wearing off” phenomenon of L-dopa

A 
MAOB inhibitor (selegiline)
OR
ACh inhibitor (benzotropine)
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30
Q

Symptoms of PD likely to respond or not to deep brain stimulation

A

Gait changes and freezing (especially if respond well to medications)

Severe balance problems less likely to respond to DBS

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31
Q

Patients in which to consider deep brain stimulation

A

Significant motor fluctuations difficult to control with drug therapy

Patients with early PD, who have been responding well to drugs, but having increasing difficulties threatening social/work

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32
Q

Definition of multiple sclerosis

A

A chronic autoimmune demyelinating disease of the CNS characterised by subacute neurological impairment correlated with CNS lesions separated in time and space that cannot be explained by another disease

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33
Q

Patterns of MS

A

Relapsing-Remitting (80%): relapses followed by (near)complete recovery… 50-80% will later transition to secondary progressive

Secondary progressive: progression of disability with few or no relapses

Primary progressive (20%): progression from onset of disease, typically without relapses

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34
Q

Pathophysiology of MS

A

autoimmune destruction of myelin sheaths in the CNS

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35
Q

Effect of pregnancy on MS

A

Child birth is likely to trigger a relapse/flare-up

breastfeeding offers some protection to relapse

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36
Q

Presentations of MS

A

Optic neuritis (visual blurring +/- pain)
Weakness or sensory disturbances
Incoordination, dysarthria and intention tremor
Trigeminal neuralgia
Bladder or bowel symptoms (urgency or incontinence)

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37
Q

Diagnosis of MS

A

MRI brain and spinal cord

  • more than 3 lesions greater than 6mm diameter
  • oval shaped
  • located in periventricular area, corpus callosum and posterior fossa
  • gadalonium-enhancing lesions indicate new attacks
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38
Q

Lumbar puncture in MS

A

Non-specific, rarely used if MRI positive

  • lymphocytic raised WCC
  • oligoclonal bands
  • raised IgG/albumin index
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39
Q

Primary and secondary prevention of MS

A

Vitamin D - reduces risk of development in susceptible individuals (e.g. family history) by 70% and reduces relapse

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40
Q

Management of acute exacerbation for MS

A

high-dose IV methlyprednisolone 5 days

(followed by oral corticosteroids if optic neuritis)

Plasmapheresis sometimes indicated (ask neurologist)

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41
Q

Disease modifying therapy in MS

A

First line: IFN beta OR galatiramer
Second-line natalizumab
Fingolimod

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42
Q

Causes of resting tremor

A

Parkinson disease/syndromes
Midbrain (rubral) tremor
Wilson’s disease
Severe essential tremor

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43
Q

Causes of postural-action tremor

A 
Enhanced physiological tremor
Essential tremor
Primary writing tremor
Extrapyradimal disorders (PD, Wilson's, dystonia)
Cerebellar disease
Peripheral neuropathy
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44
Q

Causes of intention tremor

A

(Cerebellar outflow)

  • Cerebellar disease
  • Multiple sclerosis
  • Midbrain stroke
  • Midbrain trauma
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45
Q

Causes of stroke

A

Ischaemic - 90%

  • Cardioembolic - 30%
  • Artery-artery embolism (atherosclerotic plaques)
  • In situ thrombosis

Haemorrhagic (10%) - SAH or ICH

  • hypertensive small vessel disease
  • amyloid angiopathy
  • congenital vascular malformations (young people)

Haemodynamic (hypovolaemic)
- circulatory failure (hypotension, cardiac arrest)

Cerebral vein thrombosis - inc. pressure into brain due to congestion - swollen - haemorrhage

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46
Q

Investigations to perform in stroke

A

General:

  • CXR
  • ECG ?AF
  • CBE - ?hypercoagulable state
  • ESR - ? vascultis
CT
MRI
Diffusion weighted MRI or FLAIR
Cardiac imaging (TOE)
Imaging of cerebral vessels (Carotid doppler, CTA, MRA, catheter angiogram)
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47
Q

CT findings in stroke

A

Not very sensitive in first few hours

  • Normally should see “cortical ribbon (line running around brain between gyri) - lost early after stroke
  • Loss of grey-white differentiation

Infarction = hypodense (DARK)
Acute haemorrhage = hyperdense (WHITE)

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48
Q

What is a FLAIR scan and what is it’s role?

A

Fluid attenuation inversion recovery MRI
Shows all cerebral damage (post-traumatic, scarring, demyelination etc.)
Non specific
Does not differentiate acute from chronic ischaemia

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49
Q

Role of diffusion weighted imaging in stroke

A

MRI scan
Measures acute cytotoxic oedema
Circulation of water particules through an area of damage - accumulated in acute damage (appears bright on scan!)
Is able to differentiate acute from chronic damage

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50
Q

Imaging for lacunar or brainstem infarcts

A

MRI - much more sensitive than CT

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51
Q

General supportive care following a stroke

A
  1. Fever
    - hyperthermia increases infarct size
    - treat fever over 37.5 with antipyretics
  2. Blood pressure
    - treat only if diastolic 120+ or systolic 220+ (180+ if using thrombolysis)
    - oral captopril or sublingual GTN
    - Avoid nifedipine
  3. Hyperglycaemia
    - associated with poorer outcome
    - monitor BGLs, consider insulin if 10mmol/L+
  4. Admission to stroke unit!
    - reduces risk of death and disability
    - medical expertise, nursing expertise, allied health expertise
52
Q

4 interventions proven to improve outcome after acute ischaemic stroke

A
  1. Manage in stroke unit
  2. Give aspirin within 48h of ischaemic stroke
  3. IV alteplase (thrombolysis)
    - treat within 4.5h of symptom onset
    - risk of haemorrhagic transformation (6% will be asymptomatic, some fatal)
  4. Hemicraniectomy
53
Q

Contraindications of thrombolysis with alteplase in patient suffering ischaemic stroke

A
  • Over 4.5h since onset of symptoms
  • severe HTN (over 180 systolic)
  • Recent surgery
  • anticoagulation
54
Q

Indications for hemicraniectomy following acute ischaemic stroke

A
  • under 60y
  • infarction over 50% MCA territory
  • Able to perform procedure within 48h of symptom onset
  • deterioration in conscious state from time of admission
55
Q

Primary prevention of stroke

A

Blood pressure detection and management!!!

AF - detection and management (CHADS) - 2+ - warfarin or NOACs

Lifestyle: smoking, hyperlipidaemia

Early detection and investigation of patients experiencing TIAs

56
Q

Secondary prevention of stroke

A
  • *15% of ischaemic strokes are preceded by a TIA, highest risk in first days - week after initial TIA**
  • start anti-platelet therapy (aspirin 100mg/day)
  • Cerebral imaging (exclude differentials)
  • establish ASAP if eligible for carotid endarterectomy (within 14 days)
  • treat even normotensive patients with ACEi
  • Statin regardless of cholesterol
  • ABCD2 score to determine risk of stroke
57
Q

ABCD2 score

A

Estimated the risk of a stroke after a TIA

  • Age over 60
  • Blood pressure over 140/90
  • Clinical features (weakness = 2, speech only = 1)
  • Duration of symptoms (10-59min = 1, 60+ = 2)
  • Diabetes history

Over 4 = high risk - investigate within 24h
Less than 4, investigate within 72h

58
Q

Clinical features of intracerebral haemorrhage

A 
Neck stiffness
Seizures
Diastolic BP over 110
Vomiting
Headache
Focal neurological changes
59
Q

Differentiating intracerebral haemorrhage from subarachnoid haemorrhage

A

ICH forms “blobs” of bleeding

SAH forms lines etc. following meninges - not a large pool of blood

60
Q

Management of intracerebral haemorrhage

A

Supportive care

  • maintain airway
  • oxygenation
  • prevention of secondary complications

Medical care:
- IV recombinant factor VIIa (Novo7) within 4h (reduced volume of haemorrhage and surrounding oedema, no improvement in clinical outcomes)

Surgical therapy:

  • evacuate intracerebral haematoma - no better than medical therapy
  • Aim to remove cause (e.g. AV malformation) and prevent hydrocephalus
61
Q

Causes of subarachnoid haemorrhage

A

Most common = trauma
Most common spontaneous cause = ruptured saccular aneurysm

Other causes: AV malformations, bleeding into pre-existing tumours, vasculitis, cerebral artery dissection

62
Q

Clinical features of subarachnoid haemorrhage

A

Thunderclap headache (97%)

  • worst headache of life
  • lateralised toward the side of the haemorrhage in 30%
  • only 10% presenting with this headache will have SAH
  • ONLY symptoms in 30% of SAH patients
Loss of consciousness
Seizures - poor prognosis
Focal neurological deficits
Photophobia/meningism
Vomiting
Sudden death (10-15% before reaching hospital)

Sympathetic storm

  • raised BP
  • Neurogenic pulmonary oedema
  • arrhythmias/arrest (3%)
  • ECG changes (may resemble acute MI) + increased troponin
63
Q

Acute v subacute haemorrhage on CT

A

Acute = hyperdense (white)

Subacute (over5d) = isodense to brain (around the same colour as surrounding tissue)

64
Q

Imaging in acute CNS bleed

A

CT more sensitive acutely, MRI for sensitive few days later

65
Q

Investigations in subarachnoid haemorrhage

A 
CT head
Lumbar puncture
- raised opening pressure
- raised RBC, not diminishing from tube 1-4
- Xanthochromia (yellowish/pink colour)

CT angiography brain
- determine location, accessibility and shape of aneurysm
- ?coil-able
CBE, INR, coag studies etc.

66
Q

Management of subarachnoid haemorrhage

A
  1. Prevent re-bleeding
    - clipping or coiling (cannot coil if aneurysm has broad base, only used if saccular)
  2. Identify/treat hydrocephalus
    - over 10-15mmHg, may have Cushing’s triad
    - Hyperventilation
    - elevate head
    - osmotherapy (IV mannitol)
    - diuretics
    - hypothermia
    - Novo 7
  3. Identify/treat vasospasm
    - vasodilators (nimodipine)
    - HHH therapy: induce hypervolaemia, hyperdilution, hypertension (if aneurysm is secured)
    - balloon or chemical angioplasty (local Ca release via catheter)
67
Q

Complications of subarachnoid haemorrhage

A 
Re-bleed
Communicating hydrocephalus
Cerebral oedema
Seizures
Cerebral vasospasm
- most common cause of late death (post day 7)
68
Q

Causes of extradural haemorrhage

A 
Trauma (fall, assault, MVA, sports)
Complications of neurosurgery
Coagulopathy
Vascular malformations
Bleed is USUALLY arterial (can be venous or mixed)
69
Q

Extradural haemorrhage on imaging

A

CT:

Lens/lentiform shape that does not cross suture lines (dura is welded at suture lines)

70
Q

Clinical features of extradural haemorrhage

A 
Headache - may be gradual and present weeks after traumatic event
Vomiting
Drowsiness
Lucid intervals between LOC
Reduced GCS
Confusion
Speech difficulties
Seizures
Focal neurologic deficits
Cushing's triad (raised ICP)
71
Q

Management of extradural haemorrhage

A

ABC principles
C-spine protection if unconscious
Treatment of underlying coagulopathy/correction of medication induced anticoagulation
Immediate neurosurg consultation once confirmed (for evacuation of haematoma)
Mannitol to reduce ICP while being transferred if acute deterioration

72
Q

Definition of subdural haemorrhage

A

A collection of blood or blood products between the arachnoid mater and the dura mater of the brain. May be acute or chronic

73
Q

Cause of subdural haemorrhage

A

Shearing and trauma of a bridging VEIN between brain parenchyma and dura mater
(slow bleed therefore presentation can be delayed by days to weeks)

Trauma
Antithrombotic therapy
Brain atrophy secondary to age, alcoholism (incr. stretch of bridging vessels)

74
Q

investigations in subdural haemorrhage

A

Coagulation studies
CBE: PLT
LFT (esp if history of alcoholism)
CT: crescent shape hyperdensity, extends over suture lines

75
Q

Management of of subdural haemorrhage

A

Correction of underlying coagulopathy
Majority of awake patients can be managed without surgery

Surgical evacuation indications:

  • Acute: over 10mm with midline shift over 5mm
  • Chronic: mass effect, clear change in neuro examination from baseline, enlargement of haematoma size
76
Q

Normal pressure hydrocephalus clinical triad

A

Syndrome of enlarged lateral ventricles in elderly patients with triad of:

  1. A gait apraxia
  2. dementia
  3. Urinary incontinence
77
Q

Types of brain herniations

A

SUPRATENTORIAL:
Uncal/transtentorial:
- medial temporal lobe from middle into posterior fossa across the tentorial opening
- CNIII palsy, PCA infarct

Central:
- diencephalon and parts of temporal lobes squeezed through a notch in tentorium cerebelli

Cingulate/subflacine (most common)
- cingulate gyrus to contralateral hemisphere under falx cerebri

Transcalvarial/external
- brain squeezes through a fracture or surgical site in the skull

INFRATENTORIAL
Upward:
- midbrain moves from posterior fossa through tentorial notch
Cerebellar tonsillar:
- displacement of cerebellar tonsils into foramen magnum
- neck stiffness, cardiorespiratory arrest

78
Q

Classifications of peripheral neuropathies

A
  1. Axonal degeneration
    - reduced amplitude on nerve conduction studies +/- reduced conduction velocity
  2. demyelination
    - reduced internodal conduction, reduced conduction velocity in affected segment
  3. neuronopathies
79
Q

Axonal degeneration causes

A 
Systemic illness:
- DM
- sepsis/critical illness
- uraemia
- Vit B12 deficiency
- HIV
Drugs/toxins:
- Amiodarone
- Disulfuram
- Phenytoin
- Arsenic

Secondary demyelination can occur making nerve conduction studies difficult to interpret

80
Q

Types of demyelination neuropathies

A
  1. uniform - all segments in all nerves (e.g. hereditary and sensory type 1 disorders)
  2. multifocal -some segments in some nerves but not all (e.g. acute/chronic inflammation)
  3. Monofocal - 1 nerve, 1 site (e.g. carpal tunnel, usually compressive syndromes)
81
Q

Causes of demyelination

A 
Systemic illness
- DM
- chronic liver disease
- mulitple myeloma
Drugs/toxins:
- amiodarone
- diphtheria toxin
82
Q

Definition of Guillain-Barre syndrome

A

Acute immune-medicated peripheral polyneuropathy

83
Q

Clinical features of Guillain-Barre syndrome

A

Progressive, symmetrical muscle weakness + areflexia
Usually begins in legs and moves upwards
+/- paraesthesia in hands and feet
Dysautonomia in 70% (tachycardia, urinary retention, alternating BP, orthostatic hypotension, bradycardia, arrhythmias, loss of sweating)

84
Q

Laboratory features of Guillain-Barre syndrome

A

Lumbar puncture (CSF)

  • increased protein
  • normal WCC

Neurophysiology studies (electromyogram or NCS)

  • acute polyneuropathy
  • predominantly demyelinating features

Glycolipid antibodies

85
Q

Natural history of Guillain Barre syndrome

A

Usually lasts a few weeks, symptoms then improve slowly over weeks to months
Most recover with no long-term weakness
2% will develop relapsing weakness of chronic inflammatory demyelinating polyneuropathy

86
Q

management of Guillain Barre Syndrome

A

Supportive care:
- O2, intubation/ventilation as indicated
- autonomic function monitoring
- bowel and bladder care
- pain control (CBZ or gabapentin)
- Rehab
Disease modifying treatment:
- if non-ambulatory presenting within 4w of symptom onset
- Plasma exchange (removes circulating antibodies, complement and soluble biological response modifiersd)
- IVIG (if more severe clinical disease, usually preferred treatment based on administration and availability)
NO ROLE OF CORTICOSTEROIDS

87
Q

Complications of plasma exchange

A

Hypotension
Sepsis
Problems with IV access

88
Q

Complications of IVIG

A

Aseptic meningitis
Rash
Acute renal failure

89
Q

Poor prognostic factors for Guillain-Barre syndrome

A
  • older age
  • rapid onset (less than 7d) prior to presentation
  • Severe weakness on admission
  • need for ventilator support
  • preceding diarrhoeal illness
90
Q

Triggers commonly identified for Guillain-Barre syndrome

A 
Campylobacter
HIV
CMV
Influenza-like illness
EBV

Immunisation (esp flu and meningococcal)
Trauma
Bone marrow transplant

91
Q

Management of migraines

A
  • Avoid identified triggers
    ACUTE ATTACK
  • simple analgesics (paracetamol, NSAIDs, aspirin)
    + triptans (serotoning agonists, inhibit release of vasoactive peptides - promote vasoconstriction and block pain pathways in the brainstem)

PROPHYLACTIC TREATMENT

  • indicated if more than 1/week or symptomatic treatments ineffective or contraindicated
  • Anti-HTN (Propanolol, Ca-channel blockers, ACEi)
  • Antidepressants (TCAs)
  • AEDs (valproate, topiramate)
  • Domperidone (motillium)
  • Gabapentin
92
Q

Definition of motor neurone disease

A

Progressive, incurable degenerative disorder of motor neurons of the CNS related to excitotoxicity due to elevated levels of glutamate
Most commonly sporadic but can be familial in 5-10%

93
Q

Clinical features of motor neuron disease

A

INITIAL PRESENTATION:

  • Asymmetric limb weakness is most common presentation (commonly foot drop and hand weakness)
  • bulbar segment onset (dysarthria or dysphagia)
  • respiratory muscle weakness
  • generalised weakness in limbs and bulbar muscles

COGNITIVE - frontotemporal dementia

AUTONOMIC

  • constipation
  • dysphagia
  • early satiety and bloating
  • urinary urgency
  • diaphoresis

Progression:

  • variable rate
  • symptoms initially spread within the segment of onset then to other regions in relatively predictable pattern (e.g. unilateral arm onset - contralateral arm - ipsilateral leg - contralateral leg - bulbar muscles)
94
Q

Life threatening features of motor neuron disease

A

Neuromuscular respiratory failure

  • diaphragm generally spared, therefore easier to breathe while sitting upright
  • often experience nocturnal hypoventilation if lying flat

Progressive dysphagia

  • aspiration of foods, liquids or secretions - pneumonia
  • malnutrition and dehydration
  • minimise risk early with PEG feeding
95
Q

Good prognostic factors in motor neuron disease

A
  1. Riluzole
    - glutamate antagonist - red. excitation
    - increase survival by 3-6m
  2. Early PEG feeding
    - reduces risk of aspiration pneumonia
    - improves nutrition, general health
  3. Non-invasive ventilation
    - prevents respiratory failure overnight especially and improves drowsiness etc. during the day
  4. Referral to a multi-disciplinary MND clinic
96
Q

Features of frontotemporal dementia

A

Medial syndrome:

  • apathy
  • lack of energy
  • introverted
  • reduction in speech, potentially progressing to mutism

Lateral syndrome:

  • loss of empathy, especially towards spouse (usually on non-dominant side of the brain)
  • disinhibition
  • lack of judgement/insight - impulsivity
  • Wrong use of words
  • perseveration of motor movements and speech

Most cases originate from the R frontal lobe

97
Q

Loss of MMSE points per year with dementia

A

3-4

If declining slower, probably just age related

98
Q

Brainstem 4 rules of 4s

A
  1. There are 4 structures in the midline, beginning with M
    - motor pathway (Corticospinal tract)
    - medial lemniscus
    - medial longitudinal fasculus
    - motor nuclei (3, 4, 6, 12)
  2. There are 4 structures to the Side (lateral) beginning with S
    - Spinothalamic tract
    - Spinocerebellar tract
    - Sensory nucleus of CN V
    - Sympathetic pathway
  3. There are 4 CN above the pons, 4 CN in the pons and 4 CN in the medulla
  4. The 4 motor nuclei that are in the midline are dose that divide into 12, others are lateral brainstem (i.e. 3, 4, 6, 12)
99
Q

Clinical features of medial brainstem syndromes

A

Motor nuclei - ipsilateral loss of CN affected (3 = dow’n n’ out, 4, 6, 12 = deviation of tongue towards side of lesion)
Motor pathway - contralateral weakness of arm or leg
Medial lemniscus - contralateral loss of vibration and proprioception affecting arm and leg
Medial longitudinal fasciculus - failure of adduction of ipsilateral eye + nystagmus of contralateral eye as it looks laterally

100
Q

Clinical features of lateral brain stem syndromes

A

Spinocerebellar - ipsilateral ataxia of arm and leg
Spinothalamic - contralateral alteration of pain and temperature affecting arm and leg
Sensory nucleus of CNV - ipsilateral alteration of pain and temperature on the face in distribution of CNV
Sympathetic pathway - ipsilateral horner’s syndrome

+ Cranial neuropathies of lateral CN in affected area (pons v medulla)

101
Q

Vascular supply to the brainstem

A

Paramedian branches - medial structures

Long circumferential branches - lateral structures
(Superior cerebellar artery, anterior inferior cerebellar artery and posterior inferior cerebellar artery)

Lateral medullary syndromes can also be secondary to unilateral vertebral occlusion

102
Q

Features of lacunar/subcortical strokes

A

PURE motor hemiparesis or PURE sensory loss affecting contralteral arm and leg EQUALLY

is NOT associated with:

  • dysphagia
  • visual field loss
  • visual inattention
103
Q

Features of anterior cerebral artery stroke

A

Leg weakness more than arm
Personality change
Oculomotor palsy
Urinary incontinence

104
Q

Features of middle cerebral artery stroke

A 
Arm weakness more than leg
Dysphasia
Dyspraxias
Agnosia (inability to recognise things)
Neglect
Poor two-point discrimination
Dysgraphaesthesia
105
Q

Features of posterior cerebral artery stroke

A

Cortical blindness = contralateral homonymous hemianopia with normal fundoscopy and normal pupil reaction to light
Can also have subcortical signs to confuse things

106
Q

Features and cause of Gerstmann’s syndrome

A 
RAAF (like the air force)
right-left confusion
Agraphia
Acalculia
Finger agnosia

Caused by infarct to DOMINANT parietal lobe

107
Q

Features of vertebrobasilar territory ischaemia

A

Bilateral weakness or sensory disturbance
Diplopia
Vertigo
+/- nausea, vomiting and inability to stand

108
Q

Risk factors for brainstem infarcts

A 
SYSTEMIC HYPERTENSION
Diabetes mellitus
Smoking
Inc. age
High LDL and cholesterol
Chiropractic manipulation
109
Q

Aetiology of lateral v medial medullary infarcts

A 
Lateral medullary syndrome:
- atherothrombosis 58%
- arterial dissection 31%
- cardioembolic 10.3%
Tend to be more associated with younger patients with head trauma

Medial medullary syndrome:

  • atherothrombosis 87%
  • arterial dissection 12.5%
110
Q

Investigations in brainstem infarcts

A

Must have a high index of clinical suspicion, as very small infarcts which may not be visible on MRI can cause significant clinical symptoms!

MRI misses 37% and 13% of medial and lateral syndromes respectively
Therefore MRI does not change management in a classical medullary presentation, but excludes other causes

Poor visibility of posterior circulation on CT due to the skull

111
Q

Definition of myasthenia gravis

A

An autoimmune disorder in which antibodies form against acetylcholine nicotinic postsynaptic receptors at the neuromuscular junction of skeletal muscles, causing weakness of those muscles involved

112
Q

Epidemiology of myasthenia gravis (incidence, who does it affect, risk factors)

A

1-2/100,000 per year
Occurs at any age
Bimodal distribution: early peak in teens-20s with female predominance, late peak in 50-70s with male predominance

Neonatal myasthenia gravis is transient form in neonates as a result of transplacental passage of maternal antibodies interfering with neuromuscular junction

May be associated with other AI disorders (e.g. RA, SLE, scleroderma)

113
Q

Pathophysiology of mysasthenia gravis

A

Anti-acetylcholine receptor autoantibodies attack AChR, reduce number of receptors over time

Autoantibodies thought to originate in hyperplastic germinal centres in the thymus

  • 60-70% have thymic hyperplasia
  • 10-12% have thymoma
114
Q

Presenting symptoms of myasthenia gravis

A

FLUCTUATING SKELETAL MUSCLE WEAKNESS

  • specific muscles, not generalised muscle fatigue
  • most commonly worse in late day/evening or after exercise

Occular (over 50%):

  • ptosis and or diplopia
  • may switch from one eye to the other etc
  • PUPILS ALWAYS SPARED

Bulbar (15%):

  • fatigable chewing occurring half-way through meal
  • dysarthria (nasal or hypophonic, worsens with prolonged speech)
  • dysphagia
  • nasal regurgitation of liquids (palatal weakness)

Proximal limb weakness alone (5%)
- arms more than legs

115
Q

Clinical signs in myasthenia gravis

A

Curtain sign: ptosis increases by holding up the opposite eyelid with examiner’s finger

Myasthenic sneer: mid-lip rises when try to smile but outer corners of mouth fail to move

Orbicularis oculi weakness - prying eyes open on forced closure

Dropped head syndrome: weight of head overcomes neck extensors, especially late in day

116
Q

Investigations for myasthenia gravis

A

Bedside test:

  • Tensilon/Edrophonium test: readily reversible acetycholinesterase inhibitor prevents breakdown of ACh at NM junction - reduces weakness or ptosis in patients with myasthenia gravis
  • Ice pack test: cover eye for 1-2 minutes with ice pack and examine for improvement of ptosis when remove

Laboratory tests:

  • serologic tests for autoantibodies
  • repetitive nerve stimulation studies and single-fibre EMG
117
Q

Management of myasthenia gravis

A

Symptomatic treatment:

  • oral anticholinesterases (pryidostigmine, neostigmine)
  • 15-30 min onset of action, duration 3-4h
  • S/E cholinergic crisis (rare) but mimics worsening of myasthenia gravis

Chronic immunotherapies

  • if patients have significant symptoms on pyridostigmine
  • high dose glucocorticoids (preferred in child-bearing age patients)
  • AZA, mycophenolate mofetil, cyclosporine

Rapid immunotherapies:

  • Used in myasthenic crisis, preop before thymemctomy or other surgery, as a bridge to slower acting immunotherapies
  • Plasmapheresis or IVIG

Surgical:

  • thymectomy
  • mainstay if thymoma is present

Lifestyle:

  • avoid drugs which worsen MG
  • annual influenza vaccine if on immunosuppressive therapy
118
Q

Medications to avoid in myasthenia gravis

A 
Aminoglycoside antibiotics
Beta blockers
Procainamide
Quinidine
Quinine
Phenytoin
119
Q

Prognosis of myasthenia gravis

A

Symptoms start as transient early on
typically worsen and become more persistent
Progression peaks within 2-3y
Active phase 5-7y, stable phase, remission phase

120
Q

Myasthenic crisis

A

Life threatening condition characterised by neuromuscular respiratory failure
Severe bulbar weakness - dysphagia - aspiration often complicated respiratory failure

Typically experience generalised weakness as a warning

Treat with hospitalisation and rapid immunotherapy (IVIG or plasmapheresis)

121
Q

Definition of CJD

A

Creutzfeldt-Jakob disease is the most frequently occurring human prion disease, classified into 5 different types, each of which cause neurodegenerative disease which progress inexorably after a long incubation period

122
Q

Types of CJD

A

Sporadic: 85-95%

  • family history
  • personal history of psychosis
  • multiple surgical procedures
  • spent over 10y on a farm

Familial: 5-15%

Variant - much younger age
- related to mad cow disease

Iatrogenic: less than 1%
- following administration of cadaveric human pituitary hormones (GH and gonadotrophin), dual graft transplants, corneal orliver transplants, contaminated neurosurgical instruments

123
Q

Symptoms of CJD

A 
RAPIDLY PROGRESSIVE MENTAL DETERIORATION AND MYOCLONUS
Mental deterioration:
- dementia
- behavioural abnormalities
- deficits of higher cortical function
- concentration, memory and judgement difficulties are frequent early signs
- mood changes (apathy and depression)
- hypersomnia or sleep disturbances

Myoclonus - 90% of patients will develop at some point, especially provoked by startle

Extrapyramidal and cerebellar:
- hypokinesia
- nystagmus
- ataxia
Corticospinal tract involvement
- hyperreflexia
- extensor Babinski
- spasticity

Psych symptoms are more prominent in younger patients and clinical course is often more prolonged

CRANIAL AND PERIPHERAL NERVES ARE NOT INVOLVED. SENSORY ABNORMALITIES ARE RARE

124
Q

Diagnostic criteria for CJD

A 
Progressive dementia
AND
At least 2 of the following
- myoclonus
- visual or cerebellar disturbance
- pyramidal/extrapyramidal dysfunction
- akinetic mutism
AND 1  of:
- atypical EEG during an illness
- a positive 14-3-3 CSF assay with a clinical duration to death less than 2y
- MRI high signal abnormalities in caudate nucleus and/or putamen on DWI or FLAIR
AND
Routine investigations do not suggest alternative diagnosis
125
Q

Protein marker for CJD

A

14-3-3 in CSF fluid

126
Q

Prognosis of CJD

A

No effective treatment
Supportive care
Death usually occurs within 1 year of symptom onset
Median disease duration of 6 months

127
Q

Definition of syringomyelia

A

The formation of fluid-filled, gliosis-lined cavity (syrinx) in the spinal cord

Medicine (12 decks)

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