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PACES Hawthorne > Neurology > Flashcards

Neurology Flashcards

1
Q

Parkinsonism Examination

(inspection/arms/eyes/extras/completion)

A

Gait: slow initiation, shuffling, festination, absent arm swing, stooped posture, hurried steps, retropulstion

Inspection: Hypominia (vFacial expression)

  • Extrapyramidal posture
  • Asymmetrical resting tremor: 5Hz (exacerbated by counting backwards)
    • Ask pt to hold hands out in front of them, Fingers spread (Parkinsonian T-improves, BET-worsens)

Arms:

  • Bradykinesia (touch thumb to each finger in turn as quickly as possible)
  • Tone:
    • cogwheel rigidity (‘shake’ hand)
    • lead pipe rigidity (flex and extend elbow, whilst tapping knee with other hand (synkinesis))
  • Power: n
  • Reflexes: n
  • Co-ordination: n (abnormal in MSA)

Eyes: Movement: nystagmus (MSA) + Vertical gaze palsy (progressive supranuclear palsy) + Saccades

Extra:

  • Glabellar tap- Ask pt to fx eyes on a point on the wall “I am going to tap on your forehead” Tap repeatedly between their eyes with your index Finger Look for failure of attenuation of the blink response
  • Speech: state name and date of birth, listen for slow monotonous speech,
  • Write (name and address-micrographia), draw spiral
  • Functional: do up a button/handle coins/motion of turning a tap BP (supine and erect)

Complete:

  • Hx
  • Full neuro examination
  • MMSE
  • Drug chart
  • Abdo; Hepatomegaly + CLD
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2
Q

Parkinsonism Viva Causes

A
  • Idiopathic PD
    • Parkinson Plus Syndromes
    • Progressive supranuclear palsy
    • MSA
    • Lewy Body Dementia
    • Corticobasilar degeneration
  • Multiple infarcts in the substantia nigra
  • Wilson’s disease
  • Drugs: neuroleptics and metoclopramide
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3
Q

Parkinsonism Viva Hx

A
  • Symptoms: tremor, rigidity, akinesia
  • Autonomic
    • Postural hypotension
    • Urinary problems, constipation
    • Hypersalivation
  • ADLs
    • Handwriting, buttons, shoe-laces
    • Getting in and out of a car
  • Sleep
    • Turning in bed
    • Insomnia
    • Daytime sleepiness
  • Complications
    • Depression
    • Drug SEs: esp. motor fluctuations
  • Cause
    • Sudden onset
    • Eye or balance problems
    • Visual hallucinations, ↓ memory
    • DH
    • FH
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4
Q

Parkinsonism Viva Ix

A

Bloods
 Caeruloplasmin: ↓ in Wilson’s
Imaging
 CT / MRI: exclude vascular cause
 DaTscan

  • Ioflupane I123 injection
  • Binds to dopaminergic neurones and allows visualisation of substantial nigra
  • Can exclude other causes of tremor: e.g. BET
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5
Q

Parkinsonism Viva Mx

A
  • General
    • MDT: neurologist, PD nurse, physio, OT, social worker, GP and carers
    • Assess disability e.g. UPDRS: Unified Parkinson’s Disease Rating Scale
    • Physiotherapy: postural exercises
    • Depression screening
  • Specific
    • L-DOPA + Carbidopa or benserazide
    • Da agonists: ropinerole, pramipexole
    • Apomorphine: SC rescue drug
    • MOA-B inhibitors: rasagiline
    • COMT inhibitors: tolcapone
    • Amantidine
    • Anti-muscarinics: procyclidine
  • Adjuncts
    • Domperidone: nausea
    • Quetiapine: psychosis
    • Citalopram: depression
  • Other
    • Deep brain stimulation
    • Basal ganglia disruption
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6
Q

Parkinsonism Viva pathophysiology

A

Destruction of dopaminergic neurones in pars compacta of substantia nigra.

  • β-amyloid plaques
  • Neurofibrillary tangles: hyperphosphorlated tau
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7
Q

Parkinsonism Viva features

(TRAPPS PD)

A

Asymmetric onset: side of onset remains worst

  • Tremor: ↑ by stress, ↓ by sleep
  • Rigidity: lead-pipe, cog-wheel
  • Akinesia: slow initiation, difficulty c¯ repetitive movement, micrographia, monotonous voice, mask-like face
  • Postural instability: stooped gait c¯ festination
  • Postural hypotension: + other autonomic dysfunction
  • Sleep disorders: insomnia, EDS, OSA, RBD
  • Psychosis: esp. visual hallucinations
  • Depression / Dementia / Drug SEs
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8
Q

Parkinsonism Viva autonomic dysfuctions

A

Combined effects of drugs and neurodegeneration

  • Postural hypotension
  • Constipation
  • Hypersalivation → dribbling (↓ ability to swallow saliva)
  • Urgency, frequency, nocturia
  • ED
  • Hyperhidrosis
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9
Q

Parkinsonism Viva L-Dopa Drug SEs

(DOPAMINE)

A
  • Dyskinesia
  • On-Off phenomena = Motor fluctuations
  • Psychosis
  • ABP↓
  • Mouth dryness
  • Insomnia
  • N/V
  • EDS (excessive daytime sleepiness)
  • *End-of-dose**: deterioration as dose wears off w progressively shorter benefit.
  • *On-Off effect**: unpredictable fluctuations in motor performance unrelated to timing of dose.
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10
Q

Parkinsonism Viva PLUS syndromes

A

PSP: +postural instab + speech disturb (?psudobulbar palsy) + palsy vertical gaze

MSA: Autonomic + cerebellar and pyramidal + rigidity>tremor

LBD: fluctuating cognition + visual hallucinations

Corticobasilar degeneration: aphasia, dysarthria, apraxia + akinetic rigidity + asterognosis + alien limb

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11
Q

Parkinsonism Viva: Differential of Tremor

A
  • Resting: parkinsonism
  • Intention: cerebellar
  • Postural = Worse c¯ arms outstretched
    • BET
    • Endocrine: ↑T4
    • Alcohol withdrawal
    • Toxins: β-agonists
    • Sympathetic: anxiety

Benign Essential Tremor

  • AD
  • Occur with movement and worse c¯ anxiety, caffeine
  • Doesn’t occur c¯ sleep
  • Better c¯ EtOH
  • Treat with beta blockers
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12
Q

Cerebellar syndrome examination

Gait, arms, DANISH, completion

A
  • Inspect:
    • bruising/scars (recurrent falls)
    • Symmetry, muscle wasting, fasciculations (LMN lesion)
  • daNiSh:
    • Nystagmus (&rapid saccades)
    • Slurred speech:
      • baby hipopotamous-slurring
      • Read something aloud- stacatto speech)
  • ULEx:
    • Tone: Hypotonia
    • Power: low power may pause pseudo co-ordination problems
    • Coordination:
      • Rebound (dysmetria), bound test. Ask pt to put arms out straight in front, palms down and close eyes“Keep your arms in that position”Push each arm down in turn ~10cm then release itWatch for arm bouncing back up to beyond original position
    • Finger nose test: past pointing, dysmetria, intention tremor
    • -Dysdiadokinesis: ataxia
  • ?LL:
    • (Tone hypotonia)
    • (Power (?reduced))
    • (Co-ordination:
      • foot tapping (dysdiadokinesis)
      • Heel-shin (intention tremor, dysmetria)
  • Posture:
    • Truncal ataxia: Assess stability sitting: Sit on side of bed+Ask to cross arms in front and sit still. If stable: assess stability standing, feet together and arms by sides
    • Romberg’s test (positive in patients with sensory ataxia, negative in cerebellar ataxia nb low specificity and in cerebellar disease often unsteady with eyes open)
  • Gait: wide-based, unsteadiness with lateral veering, irregular steps, heel-toe (v.difficult if cerebellar lesion)
  • Completion:
    • Full neuro exam
      • CN; brainstem stroke/MS/CPA lesion
      • Peripheral NS; MS
    • Signs of CLD: wilsons
    • Drug chart: phenytoin
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13
Q

Cerebellar syndrome viva Causes

DAISIES

A
  • Demyelination
  • Alcohol
  • Infarct: brainstem stroke
  • SOL: e.g. schwannoma + other CPA tumours
  • Inherited: Wilson’s, Friedrich’s, Ataxia Telangiectasia, VHL
  • Epilepsy medications: phenytoin
  • System atrophy, multiple
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14
Q

Cerebellar syndrome viva nystagmus: cerebellar Vs vestibular origin

A
  • Cerebellar Cause
    • Fast phase towards lesion
    • Maximal looking towards lesion
  • Vestibular Cause
    • Fast phase away from lesion
    • Maximal looking away from lesion
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15
Q

Cerebellar syndrome viva Hx

A
  • MS: paraesthesia, visual problems, muscle weakness
  • Alcohol consumption
  • Infarct: onset, stroke risk factors
  • Schwannoma: hearing loss, vertigo, tinnitus, ↑ICP
  • FH
  • DH
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16
Q

Cerebellar syndrome viva Ix

A
  • ECG: Arrhythmia
  • Bloods
    • EtOH: FBC, U+E, LFT
    • Thrombophilia: clotting
    • Wilson’s: ↓ caeruloplasmin
  • CSF: Oligoclonal bands
  • Imaging: MRI is best to visualise the posterior cranial fossa
  • CPA lesion: pure tone audiometry
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17
Q

Cerebellar syndrome viva Mx

A
  • General
    • MDT: GP, neurologist, radiologist, neurosurgeon, specialist nurses, physio, OT
    • CV Risk
    • ↓ EtOH
  • Specific
    • MS: methylprednisolone
    • EtOH: Pabrinex, tapering course of chlordiazepoxide
    • Infarct: consider thrombolysis
    • Schwannoma: gamma-knife, surgery
    • Wilson’s: penicillamine
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18
Q

Cerebellar syndrome viva Lateral medullary syndrome (wallenberg’s)

DANVAH

A
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19
Q

Cerebellar syndrome viva Vestibular schwannoma

A

Pathophysiology

  • Benign, slow-growing tumour of superior vestibular nerve
  • SOL → CPA syndrome: 80% of CPA tumours
  • Assoc. c¯ NF2

Presentation

  • Unilat SNHL, tinnitus ± vertigo
  • ↑ICP: headache
  • Ipsilateral CN 5, 6, 7 and 8 palsies and cerebellar signs
    • Facial anaesthesia + absent corneal reflex
    • LR palsy
    • LMN facial nerve palsy
    • SNHL
    • DANISH
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20
Q

Cerebellar syndrome viva CPA Tx Ddx

A
  • Vestibular Schwannoma
  • Meningioma
  • Cerebellar astrocytoma
  • Metastases
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21
Q

Cerebellar syndrome viva Von Hippel-Lindau Syndrome features

A
  • Renal cysts
  • Bilateral renal cell carcinoma
  • Haemangioblastomas
    • Often in cerebellum → cerebellar signs
  • Phaeochromocytoma
  • Islet cell tumours
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22
Q

Cerebellar syndrome viva Friedrich’s ataxia Pathophysiology and features

A
  • Pathophysiology
    • Auto recessive mitochondrial disorder
    • Progressive degeneration of
      • Dorsal column
      • Spinocerebellar tracts and cerebellar cells
      • Corticospinal tracts
    • Onset in teenage years
    • Assoc. c¯ HOCM and mild dementia
  • Main Features
    • Pes cavus
    • Bilateral cerebellar ataxia
    • Leg wasting + areflexia but extensor plantars
    • Loss of vibration and proprioception
  • Additional Features
    • High-arched palate
    • Optic atrophy and retinitis pigmentosa
    • HOCM: ESM + 4th heart sound
    • DM in 10%: dip the urine
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23
Q

Ataxia Telangiectasia Viva

A
  • Autosomal recessive
  • Defect in DNA repair
  • Onset in childhood / early adult

Features

  • Progressive ataxia
  • Telangiectasia: conjunctivae, eyes, nose, skin creases
  • Defective cell-mediated immunity and Ab production
  • Infections
  • Lymphoproliferative disease
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24
Q

Cerebellar syndrome viva wilsons features (CLANK)

A
  • AR mutation of ATP7B gene on Chr13

Features: CLANK

  • Cornea: Keiser-Fleischer rings
  • Liver: CLD
  • Arthritis
  • Neuro: parkinsonism, ataxia, psychiatric problems
  • Kidney: Fanconi’s syn
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25
Q

UMN signs Ex

Inspection/gait/UMN signs/completion

A

Inspection

  • Walking aids
  • May have disuse atrophy and contractures
  • Limb position
    • Leg: extended, internally rotated c¯ foot plantar flexed
    • Arm: flexed, internally rotated, supinated

Gait

  • Unilateral → circumducting
  • Bilateral → scissoring

UMN Signs

  • ↑ tone
  • Pyramidal distribution of weakness
    • Leg: extensors stronger than flexors
    • Arm: flexors stronger than extensors
  • Hyper-reflexion
  • Extensor plantars

Sensation:

  • Examine for sensory level: suggests cord lesion

Completion

  • CN: evidence of MS
  • Cerebellum: evidence of MS
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26
Q

UMN Viva Bilateral LL: spastic paresis

common/other/mixed UMN and LMN

A

Common

  • MS
  • Cord compression
  • Cord Trauma
  • CP

Other

  • Familial spastic paraparesis
  • Vascular: e.g. aortic dissection → Beck’s syndrome
  • Infection: HTLV-1
  • Tumour: ependymoma
  • Syringomyelia

Mixed UMN and LMN Features

  • MND
  • Ataxia, Friedrich’s
  • SCDC: B12
  • Taboparesis
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27
Q

UMN Viva unilateral LL

hemisphere and hemicord

A

Hemisphere → Spastic Hemiparesis

  • Stroke
  • MS
  • SOL
  • CP

Hemicord → Spastic Hemiparesis or Monoparesis

  • MS
  • Cord Compression
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28
Q

UMN Viva Hx

A
  • MS: tingling, eye problems, ataxia, other weakness
  • Cord compression: back pain, fever, wt. loss
  • Trauma
  • FH
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29
Q

UMN Viva Ix; Imaging & Causes: MS, Compression, SCDC

A

MRI: Cord and brain

Further Ix Depend on Cause

  • MS
    • LP: oligoclonal bands
    • Abs: MBP, NMO
    • Evoked potentials
  • Compression
    • FBC: infection
    • CXR: malignancy
    • DRE
  • SCDC
    • B12 level
    • Pernicious anaemia Abs: IF, parietal cell
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30
Q

UMN: Mx (supportive)

A

Supportive

  • MDT: GP, neurologist, radiologist, neurosurgeon, specialist nurses, physio, OT
  • Orthoses
  • Mobility aids
  • Urinary: ICSC
  • Contractures: baclofen, botulinum injection, physio
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31
Q

Cord compression

Features, causes, Ix, Mx

A

Key Features

  • Pain
    • Local, deep
    • Radicular
  • Weakness
    • LMN @ level
    • UMN below level
  • Sensory level
  • Sphincter disturbance

Causes

  • Trauma: vertebral #
  • Infection: epidural abscess, TB
  • Malignancy: breast, thyroid, bronchus, kidney, prostate
  • Disc prolapse: above L1/2

Ix

  • MRI is definitive modality
  • CXR for primaries

Mx

  • This is a neurosurgical emergency
  • Malignancy
    • Dexamethasone IV
    • Consider chemo, radio and decompressive laminectomy
  • Abscess: abx and surgical decompression
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32
Q

Cauda equina lesion

  • pain/weakness/sensation/sphincters*
  • Causes*
A
  • Pain
    • Back pain
    • Radicular pain down legs
  • Weakness
    • Bilateral flaccid, areflexic lower limb weakness
  • Sensation
    • Saddle anaesthesia
  • Sphincters
    • Incontinence / retention of faeces / urine
    • Poor anal tone

Causes:

  • Trauma: vertebral #
  • Infection: epidural abscess, TB
  • Malignancy: breast, thyroid, bronchus, kidney, prostate
  • Disc prolapse: below L1/2
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33
Q

Anterior spinal artery/Beck’s syndrome

what/causes/effect

A
  • Infarction of spinal cord in distribution of anterior spinal artery: ventral 2/3 of cord.
  • Causes: Aortic aneurysm dissection or repair
  • Effects
    • Para- / quadri-paresis
    • Impaired pain and temperature sensation
    • Preserved touch and proprioception
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34
Q

Human T-lymphotropic virus-1

A
  • Retrovirus
  • ↑ prevalence in Japan and Caribbean

Features

  • Adult T cell leukaemia / lymphoma
  • Tropical spastic paraplegia / HTLV myelopathy
    • Slowly progressing spastic paraplegia
    • Sensory loss and paraesthesia
    • Bladder dysfunction
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35
Q

Syringomyelia

Characteristics, causes, cardinal signs, other signs

A

Characteristics

  • Syrinx: tubular cavity in central canal of the cord.
  • Symptoms may be static for yrs but then worsen fast
    • e.g. on coughing, sneezing as ↑ pressure → extension
  • Commonly located in cervical cord
  • Syrinx expands ventrally affecting:
    • Decussating spinothalamic neurones
    • Anterior horn cells
    • Corticospinal tracts

Causes

  • Blocked CSF circulation c¯ ↓ flow from posterior fossa
    • Arnold-Chiari malformation (cerebellum herniates through foramen magnum)
    • Masses
  • Spina bifida
  • 2O to cord trauma, myelitis, cord tumours and AVMs
  • *Cardinal Signs**
    1. Dissociated Sensory Loss
  • Loss of pain and temperature → scars from burns
  • Preserved touch, proprioception and vibration.
  • Root distribution reflects syrinx location
    • Usually upper limbs and chest: “cape”
  1. Wasting/weakness of hands ± Claw hand
  2. Loss of reflexes in upper limb
  3. Charcot joints: shoulder and elbow
    * *Other Signs**
  • UMN weakness in lower limbs c¯ extensor plantars
  • Horner’s syndrome
  • Syringobulbia: cerebellar and lower CN signs
  • Kyphoscoliosis
  • *Ix**: MRI spine
  • *Surgery:** Decompression at the foramen magnum for Chiari mal
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36
Q

Acute stroke

definition and pathogenesis

A

Definition

  • Rapid onset focal neurological deficit of vascular origin lasting >24hrs.

Pathogenesis

  • Ischaemic: 80%
  • Atheroma: large or small vessel
  • Embolism: cardiac or atherothromboembolism
  • Haemorrhagic: 20%
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37
Q

Stoke clinical features

Bamford classification (/Oxford Stroke Classification)

A

TACS: carotid / MCA and ACA territory

  • Hemiparesis and/or hemisensory deficit
  • Homonymous hemianopia
  • Higher cortical dysfunction
    • Dominant: aphasia
    • Non-dominant: neglect, apraxia

PACS: carotid / MCA and ACA territory

  • 2/3 of TACS criteria, usually
  • Homonymous hemianopia
  • Higher cortical dysfunction

POCS: vertebrobasilar territory
Any of:

  • Cerebellar syndrome
  • Brainstem syndrome
  • Homonymous hemianopia

LACS: infarct around basal ganglia, internal capsule, thalamus and pons

  • Pure motor: post. limb of internal capsule
  • Pure sensory: post. thalamus (VPL)
  • Mixed sensorimotor: internal capsule
  • Dysarthria / clumsy hand
  • Ataxic hemiparesis: ant. limb of internal capsule
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38
Q

Acute stroke Ddx

A
  • Head injury
  • ↑ or ↓ glucose
  • SOL
  • Infection
  • Drugs: e.g. opiate OD
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39
Q

Acute stroke Mx

A

Resuscitate

  • Ensure patent airway: consider NGT
  • NBM until swallowing assessed by SALT
  • Don’t overhydrate: risk of cerebral oedema
  • BM: exclude hypoglycaemia

Monitor

  • Glucose: 4-11mM: sliding scale if DM
  • BP: Rx of HTN can → ↓ cerebral perfusion
  • Neuro obs

Bloods

  • FBC: infection (sepsis may → stroke)
  • U+E: electrolyte disturbances may mimic stroke
  • Glucose: exclude hypoglycaemia
  • Clotting: ↑ or ↓ INR may indicate cause

Imaging

  • Urgent CT/MRI
  • Diffusion-weighted MRI is most sensitive for acute infarct
  • CT will exclude primary haemorrhage

Medical

  • Consider thrombolysis if 18-80yrs and <4.5hrs since onset of symptoms
    • Alteplase (rh-tPA)
    • → ↓ death and dependency (OR 0.64)
    • CT 24h post-thrombolysis to look for haemorrhage
  • Aspirin 300mg PO/PR once haemorrhagic stroke excluded ± PPI
    • Clopidogrel if aspirin sensitive

Surgery

  • Neurosurgical opinion if intracranial haemorrhage
  • May coil bleeding aneurysms
  • Decompressive hemicraniectomy for some forms of MCA infarction.

Stroke Unit

  • Specialist nursing and physio
  • Early mobilisation
  • DVT prophylaxis
  • *Secondary Prevention**
  • *Rehabilitation**
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40
Q

Absolute CI to thrombolysis

A

Contraindications to thrombolysis

  • active internal bleeding
  • recent haemorrhage, trauma or surgery (including dental extraction)
  • coagulation and bleeding disorders
  • intracranial neoplasm
  • stroke < 3 months
  • aortic dissection
  • recent head injury
  • pregnancy
  • severe hypertension

Side-effects

  • haemorrhage
  • hypotension - more common with streptokinase
  • allergic reactions may occur with streptokinase
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41
Q

Non-acute stroke;

‘work up’/Ix

A

ECG ± 24hr Tape

  • Arrhythmia
  • Old ischaemia

Bloods

  • FBC: ↑ or ↓ Hb
  • U+E: association c¯ renovascular disease
  • Glucose: exclude DM
  • Lipids: CV risk
  • Clotting and thrombophilia screen
  • Vasculitis: ESR, ANA

Thrombophilia Screen

  • FBC, clotting, fibrinogen concentration
  • APC resistance / F5 Leiden
  • Lupus anticoagulant
  • Anti-cardiolipin Abs
  • Assays for protein C and S and AT3 activity
  • PCR for prothrombin gene mutation

Imaging

  • CXR
    • Cardiomegaly 2O to HTN
    • Aspiration
  • Carotid doppler
  • Echo
    • Mural thrombus
    • Regional wall motion abnormality
    • ASD, VSD: paradoxical emboli
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42
Q

Non-acute stroke

Secondary prevention

A
  • Risk factor control e.g. Start a statin after 48h
  • Aspirin / clopi 300mg for 2wks after stroke then either
    • Clopidogrel 75mg OD (preferred option)
    • Aspirin 75mg OD + dipyridamole MR 200mg BD
  • Warfarin/DOAC instead of aspirin/clopidogrel if
    • Cardioembolic stroke or chronic AF
    • Start from 2wks post-stroke (INR 2-3)
    • Don’t use aspirin and warfarin together.
  • Carotid endarterectomy if good recovery + ipsilat stenosis ≥70%
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43
Q

Non-acute stroke

rehabilitation (MENDS)

A

Rehabilitation: Must occur on a dedicated stroke unit
MENDS

  • MDT: physio, SALT, dietician, OT, specialist nurses, neurologist, family
  • Eating
    • Screen swallowing: refer to specialist
      • NG/PEG if unable to take oral nutrition
    • screen for malnutrition (MUST tool)
      • Supplements if necessary
  • Neurorehab: physio and speech therapy
    • Botulinum can help spasticity
  • DVT Prophylaxis
  • Sores: must be avoided @ all costs
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44
Q

Non-acute stroke;

prognosis@1yr

A
  • 10% recurrence
  • PACS
    • 20% mortality
    • 1/3 of survivors independent
    • 2/3 of survivors dependent
  • TACS is much worse
    • 60% mortality
    • 5% independence
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45
Q

MS definition and dignosis

A

A chronic inflammatory condition of the CNS characterised by multiple plaques of demyelination disseminated in time and space

Diagnosis is CLINICAL: Demonstration of lesions disseminated in time and space May use McDonald Criteria

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46
Q

MS epidemiology

A

Lifetime risk: 1/1000

Age: mean @ onset = 30yrs

Sex: F>M = 3:1

Race: rarer in blacks

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47
Q

MS pathophysiology

A

CD4 cell-mediated destruction of oligodendrocytes → demyelination and eventual neuronal death. Initial viral inflamation primes humoral Ab responses vs. MBP. Plaques of demyelination are hallmark

(Aetiology: Genetic (HLA-DRB1), environmental, viral (EBV))

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48
Q

MS classifications

A
  • Relapsing-remitting: 80%
  • Secondary progressive
  • Primary progressive: 10%
  • Progressive relapsing
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49
Q

MS Examination Presentation (TEAM)

A

Tingliing Eye; optic neuritis (vCentral vision + eye movement pain)

Ataxia + other cerebellar signs

Motor; usually spastic parapesis

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50
Q

MS Features

(sensory, motor, eyes, cerebellum, GI, GU, Lhermiutte’s)

A
  • Sensory:
    • Dys/paraesthesia
    • ↓ vibration sense
    • Trigeminal neuralgia
  • Motor:
    • Spastic weakness
    • Transverse myelitis
  • Eye:
    • Diplopia
    • Visual phenomena
    • Bilateral INO
    • Optic neuritis → atrophy
  • Cerebellum:
    • Trunk and limb ataxia
    • Scanning dysarthria
    • Falls
  • GI:
    • Swallowing disorders
    • Constipation
  • Sexual/GU:
    • ED + anorgasmia
    • Retention
    • Incontinence
  • Lhermitte’s Sign
    • Neck flexion → electric shocks in trunk/limbs
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51
Q

MS; Optic neuritis

A

pain on eye movement, rapid ↓ central vision

Uhthoff’s: vision ↓ c¯ heat: hot bath, hot meal, exercise

o/e: ↓ acuity, ↓ colour vision, white disc, central scotoma, RAPD

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52
Q

MS

INO / ataxic nystagmus / conjugate gaze palsy

A
  • Disruption of MLF connecting CN6 to CN3
  • Weak adduction of ipsilateral eye
  • Nystagmus of contralateral eye
  • Convergence preserved
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53
Q

MS Viva Ix

A
  • MRI: Gd-enhancing or T2 hyper-intense plaques
    • Gd-enhancement = active inflammation
    • Typically located in periventricular white matter
  • LP: IgG oligoclonal bands (not present in serum)
  • Abs
    • Anti-MBP
    • NMO-IgG: highly specific for Devic’s syn.
  • Evoked potentials: delayed auditory, visual and sensory
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54
Q

MS viva

Ddx

A

Inflammatory conditions may mimic MS plaques:

  • CNS sarcoidosis
  • SLE
  • Devic’s: Neuromyelitis optica (NMO)
    • MS variant c¯ transverse myelitis and optic atrophy
    • Distinguished by presence of NMO-IgG Abs
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55
Q

MS viva

Mx acute attack

A

Methylpred 1g IV/PO /24h for 3d

  • Doesn’t influence long-term outcome
  • ↓ duration and severity of attacks
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56
Q

MS viva

Mx Prevent relapse

A
  • Preventing Relapse: Disease Modifying
    • IFN-β: ↓ relapses by 30% in RRMS MS
    • Glatiramer: similar efficacy to IFN-β
  • Preventing Relapse: Biologicals
    • Natalizumab: anti-VLA-4 Ab
      • ↓ Relapses by 2/3 in RRMS
    • Alemtuzumab (Campath): anti-CD52
      • 2nd line in RRMS
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57
Q

MS viva

Mx Symptoms: Fatigue Depression Pain Spasticity Urgency / frequency ED Tremor

A

Fatigue: modafinil

Depression: SSRI (citalopram)

Pain: amitryptylline, gabapentin

Spasticity: physio, baclofen, dantrolene, botulinum

Urgency / frequency: oxybutynin, tolterodine

ED: sildenafil

Tremor: clonazepam

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58
Q

MS viva

poor prognosis

A
  • Older female
  • Motor signs @ onset
  • Many relapses early on
  • Many MRI lesions
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59
Q

MND Examination

I TPR S Completion

A
  • Inspection
    • Wasting and fasciculation
      • Esp. tongue fasciculation
  • Tone
    • Spastic
  • Power
    • Weak
  • Reflexes
    • Absent and/or brisk
      • E.g. absent knee jerks c¯ extensor plantars
  • Sensation: NORMAL
  • Completion
    • Speech
      • Bulbar: nasal
      • Pseudobulbar: hot-potato
    • Jaw-jerk
      • Bulbar: absent
      • Pseudo-bulbar: brisk
  • Eye movements: MND does not involve the eyes
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60
Q

MND viva

Ddx

A
  • Cervical cord compression → myelopathy
  • Brainstem lesions
  • Polio: asymmetrical LMN paralysis
  • Mixed UMN and LMN Signs
    • MND
    • Ataxia, Friedrich’s
    • SCDC: B12
    • Taboparesis
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61
Q

Viva

mixedUMN/LMN signs Ddx

A

Mixed UMN and LMN Signs (MAST)

  • MND
  • Ataxia, Friedrich’s
  • SCDC: B12
  • Taboparesis
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62
Q

MND Viva

Ix

A

Ix

  • Brain/cord MRI: exclude structural cause
  • Cervical cord compression → myelopathy
  • Brainstem lesions
  • EMG: fasciculation
  • LP: exclude inflammatory cause

Diagnostic Criteria

  • Revised El Escorial Criteria
63
Q

MND viva

Mx

A

General

  • MDT: neurologist, physio, OT, dietician, specialist nurse, GP, family
  • Discussion of end-of-life decisions
    • E.g. Advanced directive
    • DNAR

Specific

  • Riluzole: antiglutamatergic that prolongs life by ~3mo

Supportive

  • Drooling: amitriptyline
  • Dysphagia: NG or PEG feeding
  • Respiratory failure: NIV
  • Pain: analgesic ladder
  • Spasticity: baclofen, botulinum
64
Q

MND viva Prognosis

A

Most die w/i 3yrs: Bronchopneumonia and respiratory failure

Worse prog: elderly, female, bulbar involvement

65
Q

MND viva

classification

A
  • Amyotrophic Lateral Sclerosis: 50%
    • Corticospinal tracts → UMN and LMN signs + fasciculation
  • Progressive Bulbar Palsy: 10%
    • Only affects CN 9-12 → bulbar palsy
  • Progressive Muscular Atrophy: 10%
    • Anterior horn cell lesion → LMN signs only
    • Distal to proximal
    • Better prognosis cf. ALS
  • Primary Lateral Sclerosis: 30%
    • Loss of Betz cells in motor cortex → mainly UMN signs
    • Marked spastic leg weakness and pseudobulbar palsy
    • No cognitive decline
66
Q

LMN signs

A
  • Wasting
  • Fasciculation
  • Hypotonia
  • Hyporeflexia
67
Q

LMN

Causes:

  1. Bilateral, Symmetrical and Distal
  2. Bilateral, Symmetrical and Proximal
A

Pathology anywhere from anterior horn to muscle itself

Bilateral, Symmetrical and Distal

  • Motor Peripheral Polyneuropathy
    • sensory disturbance = Mixed Peripheral Polyneuropathy

Differential

  • HMSN: Hereditary motor sensory neuropathy
  • Paraneoplastic
  • Lead poisoning
  • Acute:
    • GBS
    • Botulism

Bilateral, Symmetrical and Proximal

  • Proximal myopathy

Differential

  • Inherited: muscular dystophy
  • Inflammation
    • Polymyositis
    • Dermatomyositis
  • Endocrine
    • Cushing’s Syndrome
    • Acromegaly
    • Thyrotoxicosis
    • Osteomalacia
    • Diabetic Amyotrophy
  • Drugs: alcohol, statins, steroids
  • Malignancy: paraneoplastic
68
Q

LMN viva

Unilateral causes

A

Isolated to single limb + no sensory signs

  • Old polio

Localised to group of muscles c¯ same supply

  • Segmental: nerve roots, plexus
  • Peripheral: mononeuropathy
69
Q

LMN Ddx

Hand wasting

A

Anterior horn: syringomyelia, MND, polio

Roots (C8 T1): spondylosis

Brachial Plexus

  • Compression: cervical rib
  • Avulsion: Klumpke’s palsy

Neuropathy

  • Generalised: HMSN
  • Mononeuritis multiplex: DM
  • Compressive mononeuropathy

Muscle

  • Disuse: RA
  • Distal myopathy: myotonic dystrophy
70
Q

LMN

Ix proximal myopathy

A
  • Bloods
    • DM: glucose, HbA1c
    • Muscle damage: CK, ESR, AST, LDH
    • Endocrine: TSH, Ca, 9am cortisol, IGF-1
    • Abs: anti-Jo1
  • CXR: paraneoplastic
  • EMG
  • Genetic analysis
  • Muscle biopsy
71
Q

LMN

Ix Mononeuropathy

A
  • Bloods
    • DM: glucose, HbA1c
    • B12, folate
    • Vasculitis: ESR, ANA, ANCA
  • EMG + nerve conduction
72
Q

LMN

Ix radiculopathy/plexopathy

73
Q

LMN

Ix peripheral polyneuropathy

A
  • Dipstick: glucose
  • Bloods
    • DM: Glucose, HBA1c
    • EtOH: FBC ± Film, LFTs, GGT
    • CRF: U+E
    • B12, folate
    • Vasculitis: ESR, ANA, ANCA
    • Thyroid disease: TFTs
  • Imaging
    • CXR: exclude paraneoplastic phenomena
  • Other
    • Nerve conduction studies
      • Demyelination → ↓ conduction speed
      • Axonal degeneration → ↓ conduction amplitude
    • Electromyography
    • Genetic: PMP22 gene in CMT
    • Nerve biopsy
74
Q

Peipheral polyneuropathy

Examination sensory/motor/completion

A

Sensory

  • Bilateral, symmetrical
  • Glove and stocking distribution: length dependent
  • ↓ tendon reflexes: loss of ankle jerks in DM
  • Signs of trauma or joint deformity (Charcot’s joints)
  • Loss of proprioception → +ve Romberg’s

Motor

  • Bilateral, symmetrical
  • LMN weakness
    • Wasting and fasciculation
    • ↓ tone
    • Hyporeflexia

Completion

  • Drug chart
  • Dipstick: glucose
  • Gait + Romberg’s test
  • CN
75
Q

Peripheral polyneuropathy

Ddx (causes) mainly sensory vs mainly motor

A
  • Mainly Sensory
    • DM
    • Alcohol
    • B12 deficiency
    • CRF and Ca (paraneoplastic)
    • Vasculitis
    • Drugs: e.g. isoniazid, vincristine
  • Mainly Motor
    • HMSN / CMT: Hereditary motor sensory neuropathy
    • Paraneoplastic: Ca lung, RCC
    • Lead poisoning
    • Acute: GBS and botulism
76
Q

Peripheral polyneuropathy

Mx (general and specific)

A

General

  • MDT: GP, neurologist, specialist nurses, physio, OT
  • Foot care and careful shoe choice
  • Splinting joints can prevent contractures

Specific

  • Optimise glycaemic control: DCCT, UKPDS trials
  • Replace nutritional deficiencies
  • Avoid EtOH or other precipitants
  • Vasculitis: steroids and other immunosuppressants
  • Neuropathic pain: amitriptyline, gabapentin
  • GBS: IVIg
77
Q

Peripheral polyneuropathy

Hx

A
  • Time-course
  • Precise symptoms
    • Ataxia: B12
    • Painful dysesthesia: EtOH, DM
  • Assoc. events
    • D&V: GBS
    • ↓wt: Ca
    • Arthralgia: connective tissue
  • Travel, EtOH, drugs
78
Q

Diabetic neuropathy

  • Examination of lower limbs*
  • (Inspection/motor/sensory/completion)*
A
  • Inspection
    • Evidence of finger pricks from BM monitoring
    • Peripheral vascular disease
    • Charcot joints
  • Motor
    • Bilateral loss of ankle jerks
      • 2O to sensory neuropathy
    • Mononeuritis multiplex
      • Foot drop
  • Sensory
    • Distal sensory loss in stocking distribution
  • Completion
    • Examine the fundi
    • Examine the upper limbs and cranial nerves
      • Sensory neuropathy
      • Mononeuritis multiplex
        • CN3
        • CN6
        • Ulnar nerve
    • Urine dip: glucose, proteinuria
79
Q

Diabetic neuropathy viva

Hx

A
  • Pain: esp. @ night
  • Glycaemic control
  • Complications of insulin
  • Other micro- and macro-vascular complications
80
Q

Diabetic neuropathy viva

pathophysiology

A
  • Metabolic*: glycosylation, ROS, sorbitol accumulation
  • Ischaemia*: loss of vasa nervorum
81
Q

Diabetic neuropathy viva

Ix

A
  • Urine: glucose, ACR
  • Blood
    • Glucose
    • HbA1c
    • U+E
82
Q

Diabetic neuropathy viva

Mx

A
  • MDT: GP, endocrinologist, neurologist, DNS
  • Good glycaemic control
  • Amitriptyline, Gabapentin
  • Capsaicin cream
83
Q

Diabetic Autonomic neuropathy

Sx

A
  • Postural hypotension – Rx: fludrocortisone
  • Gastroparesis → early satiety, GORD, bloating options include metoclopramide, domperidone or erythromycin (prokinetic agents
  • Diarrhoea – Rx: codeine phosphate
  • Urinary retention
  • ED
84
Q

Charcot-Marie-Tooth syndrome

(AKA) Define and pathophysiology

A

Peroneal Muscular Atrophy

Hereditary Motor and Sensory Neuropathy

Pathophysiology; Group of inherited motor and sensory neuropathies

85
Q

Charcot-Marie-Tooth syndrome Viva

  • Examination*
  • (Inspection, motor, sensory)*
A

Inspection

  • Pes cavus
  • Symmetrical distal muscle wasting
    • → claw hand
    • → champagne bottle leg
  • Thickened nerves: esp. common peroneal around fibula

Motor

  • High-stepping gait: foot drop
  • Weak foot and toe dorsiflexion
  • Absent ankle jerks

Sensory

  • Variable loss of sensation in a stocking distribution

History: Family Hx?

86
Q

HMSN1 and HMSN2

(herediatry motor sensory neuropathy)

A
  • HMSN1
    • Commonest form
    • Demyelinating
    • AD mutation in the peripheral myelin protein 22 gene
  • HMSN2
    • Second commonest form
    • Axonal degeneration
    • Autosomal dominant
87
Q

Charcot-Marie-Tooth syndrome Viva

Ix

A
  • Nerve conduction studies
    • HMSN1: demyelination → ↓ conduction velocity
    • HMSN2: axonal degeneration → ↓ amplitude
  • Genetic testing
    • HMSN1: Peripheral myelin protein 22 (PMP22) gene
88
Q

Charcot-Marie-Tooth syndrome Viva

Mx

A
  • MDT: GP, neurologist, specialist nurses, physio, OT
  • Foot care and careful shoe choice
  • Orthoses: e.g. ankle braces
89
Q

Myasthenia Gravis

Ex

(inspection, eyes, facial movements, voice, limbs, completion)

A
  • Inspect
    • Thymectomy scar
  • Eyes
    • Bilateral ptosis: worse on sustained upward gaze
    • Complex ophthalmoplegia
  • Facial Movements
    • Myasthenic snarl on smiling
  • Voice
    • Nasal
    • Deterioration: ask pt. to count to 50
  • Limbs
    • Fatiguability: repeatedly flap arm
  • Completion
    • Assess respiratory muscle function: spirometry (FVC)
90
Q

Myasthenia Gravis Viva

Ix

A
  • Abs: Anti-AChR, Anti-MuSK
  • EMG: ↓ response to titanic train of impulses
  • Tensilon test: improvement c¯ edrophonium (anticholinesterase)
  • TFTs: Graves in 5%
  • CT mediastinum: thymoma in 10%
91
Q

Myasthenia Gravis Viva

  • associations*
  • <50, female // >50, male*
A

<50, female - AI disease: DM, RA, Graves, SLE

>50, male - Thymoma

92
Q

Myasthenia Gravis Viva

Mx acute and chronic

A

Acute

  • Plasmapheresis or IVIg
  • Monitor FVC: consider ventilation

Chronic

  • Pyridostigmine
  • Immunosuppression: steroids and azathioprine
  • Thymectomy: benefit even if no thymoma
93
Q

LEMS

A
  • Abs vs. VGCC
  • Often paraneoplastic: e.g. SCLC
  • Lower limb girdle weakness
  • Weakness improves on repetitive testing
94
Q

DDx Bilateral Ptosis

A
  • MG
  • Myotonic dystrophy
  • Congenital
  • Senile
  • Bilateral Horner’s (rare)
95
Q

GBS

Pathophysiology

A

…is an acute polyneuropathy

Molecular mimicry: Abs x-react c¯ ganglioside

Bacteria: C. jejuni, mycoplasma

Viruses: CMV, EBV

96
Q

GBS

  • Classification*
  • (AIDP/MF)*
A

AIDP: acute autoimmune demyelinating polyneuropathy

Miller-Fisher: ophthalmoplegia + ataxia + areflexia

97
Q

GBS

features

A
  • Symmetrical ascending flaccid paralysis
  • Sensory disturbance: paraesthesia
  • Autoimmune neuropathy: labile BP
98
Q

GBS

Ix

A
  • Evidence of infection: e.g. stool MC+S
  • Anti-ganglioside Abs
  • LP: ↑↑ CSF protein
  • Nerve conduction studies: demyelination
99
Q

GBS

Mx

A
  • Supportive
    • Airway / ventilation: ITU if FVC < 1.5L
    • Analgesia: NSAIDs, gabapentin
    • Autonomic: may need inotropes, catheter
    • Antithrombotic: TEDS, LMWH
  • Immunosuppression
    • IVIg
    • Plasma exchange
  • Physiotherapy
    • Prevent flexion contractures
100
Q

GBS

Prognosis

A
  • 85% complete recovery
  • 10% unable to walk alone at 1yr
  • 5% mortality
101
Q

Facial nerve palsy

  • Examination*
  • (Inspection & weakness &UMN vs LMN)*
A

Inspection

  • Unilateral facial droop
  • Absent nasolabial fold
  • ± absent forehead creases
  • ? scar or parotid mass
  • Ear rash

Weakness

  • Raising eyebrows: frontalis
  • Screwing up eyes: orbicularis oculi
  • Bell’s sign: eyeball rolls back on closure
  • Smiling: orbicularis oris

UMN or LMN?

  • UMN → sparing of frontalis and orbicularis oculi
  • Due to bilateral cortical representation
102
Q

Facial nerve palsy

Examination:Other CN involvement

A

B. other CN involvement seen in 8% of Bell’s Palsy

  • Pons → Millard-Gubler Syndrome
    • CN6 nucleus → ipsilateral lateral rectus palsy
    • CN7 nucleus → ipsilateral LMN facial palsy
    • Corticospinal tracts → contralateral hemiparesis
  • CPA: ipsilateral CN 5, 6, 7 and 8 palsies and cerebellar signs
    • C5 Facial anaesthesia + absent corneal reflex
    • C6 LR palsy
    • C7 LMN facial nerve palsy
    • C8 SNHL
    • Cerebellar DANISH

Auditory Canal → CN8

103
Q

Facial Nerve palsy

  • Ex*
  • completion UMN and LMN*
A

If UMN: likely stroke

  • Examine limbs for ipsilateral spasticity
  • Examine visual fields: ipsilateral homonymous hemianopia

If LMN: likely Bell’s Palsy

  • Look in ears
  • Examine PNS, CN and cerebellar function
  • Test taste
104
Q

Facial nerve palsy

(unilateral) causes

A
  • 75% Idiopathic Bell’s Palsy
  • Supranuclear: vascular, MS, SOL
  • Pontine: vascular, MS, SOL
  • CPA: vestibular Schwannoma, meningioma, 2O
  • Intra-temporal: Ramsay Hunt, Cholesteatoma, trauma
  • Infra-temporal: Parotid tumour, trauma
  • Systemic
    • Neuropathy: DM, Lyme, Sarcoidosis
    • Pseudopalsy: MG
105
Q

Facial nerve palsy

bilateral causes

A
  • Bilateral Bell’s
  • Sarcoidosis
  • GBS
  • Lyme
  • Pseudopalsy: MG, Myotonic dystrophy
106
Q

Facial nerve palsy viva

Hx Symptoms and causes and aguesia/hyperacusis

A
  • Symptoms
    • Eye dryness
    • Drooling
    • ↓ taste: ageusia
    • Hyperacusis
  • Cause
    • Onset: rapid in Bell’s
    • Rash or external ear pain
    • Hx of DM
    • SOL: headache, nausea
    • Other CN: vertigo, tinnitus, diplopia
    • Limb weakness
    • Rash, fever
  • Aguesia and Hyperacusis
    • Chorda tympani and nerve to stapedius arise just distal to geniculate ganglion w/i the temporal bone.
    • Loss of these functions indicates a proximal lesion
    • Common in Ramsay Hunt: VZV @ geniculate ganglion
107
Q

Facial nerve palsy Viva

Ix

A

Urine Dip: glucose
Bloods

  • DM: glucose, HbA1c
  • Serology: VZV and Lyme
  • Abs: anti-ACh receptor

Imaging

  • MRI posterior cranial fossa

Other

  • Pure tone audiometry
  • LP: exclude infection
  • Nerve conduction studies
    • May predict delayed recovery when performed @ 2wks.
108
Q

Facial nerve palsy

  • Mx*
  • (bells)*
A
  • Prednisolone w/i 72hrs
  • Valaciclovir if VZV suspected
  • Protect eye
    • Dark glasses
    • Artificial tears
    • Tape closed @ night
109
Q

Facial nerve palsy

complications

A

Aberrant Neural Connections e.g…

Synkinesis: e.g. blinking causes up-turning of mouth

Crocodile tears: eating stimulates unilateral lacrimation, not salivation

110
Q

FN palsy

Prognosis

A
  • Incomplete paralysis: recovers completely w/i wks
  • Complete: 80% get full recovery
    • Remainder have delayed recovery or permanent neurological / cosmetic abnormalities
111
Q

FN palsy: Bell’s

A
  • 75% of Facial Palsies
  • Dx of exclusion
  • Inflammatory oedema → compression of CNVII in narrow facial canal
  • Probably of viral origin (HSV1)

Features

  • Sudden onset
  • Complete LMN facial palsy
  • Ageusia: corda tympani
  • Hyperacusis: stapedius
  • Assoc. c¯ other CN involvement in 8%
112
Q

FN palsy: Ramsay Hunt

A
  • American neurologist James Ramsay Hunt in 1907
  • Reactivation of VZV in geniculate ganglion of CNVII

Features

  • Preceding ear pain or stiff neck
  • Vesicular rash in auditory canal ± TM, pinna, tongue, hard palate (no rash = zoster sine herpete)
  • Ipsilateral facial weakness, ageusia and hyperacusis
  • May affect CN8 → vertigo, tinnitus, deafness

Mx

  • If Dx suspected give valaciclovir and prednisolone w/i first 72h

Prognosis

  • Rxed w/i 72h: 75% full recovery
  • Otherwise: 1/3 full recovery, 1/3 partial, 1/3 poor
113
Q

FN palsy: Cholesteatoma

A
  • Locally destructive expansion of stratified squamous epithelium within the middle ear.
  • Usually 2O to attic perforation in chronic suppurative OM

Presentation

  • Foul smelling white discharge
  • Vertigo, deafness, headache, pain, facial paralysis
  • Appears pearly white c¯ surrounding inflammation

Complications

  • Deafness (ossicle destruction)
  • Meningitis, cerebral abscess

Management

  • Surgery
114
Q

FN Palsy: Lyme disease

A
  • Borellia burgdorferi
  • Early Local: Erythema migrans + systemic malaise
  • Late Disseminated
    • CN palsy: esp. facial palsy
    • Polyneuropathy
    • Meningoencephalitis
    • Arthritis
    • Myocarditis
    • Heart block
115
Q

Facial anaesthesia

Examination

A
  • ↓ absent sensation in trigeminal distribution
    • Note modality
    • Note which trigeminal branch
  • Weak masseter and temporalis
  • Jaw jerk
    • Brisk: UMN
    • Absent: LMN
  • Loss of corneal reflex
116
Q

Facial anaesthesia

  • Ddx*
  • (Supranuclear/nuclear/peripheral mononeuropathy)*
A

Supranuclear

  • Demyelination
  • Infarct
  • SOL

Nuclear

  • CPA lesion: c¯ other CN palsies
  • Lateral medullary syndrome: loss of pain and temp

Peripheral: mononeuropathy

  • DM, sarcoid, vasculitis
  • Cavernous sinus
    • Ophthalmic and maxillary divisions
    • Bilateral
117
Q

Horner’s

  • Examination*
  • (Face/Neck/hands/completion)*
A

Face: PEAS

  • Ptosis: partial (superior tarsal muscle)
  • Enophthalmos
  • Anhydrosis
  • Small pupil: miosis
  • NO ophthalmoplegia

Neck Scars

  • Central lines
  • Carotid endarterectomy

Hands

  • Complete claw hand + intrinsic hand weakness
  • ↓ / absent sensation in T1

Completion: Cerebellum, CNs and PNS

118
Q

Horner’s

Differential: central, pre-ganglionic post-ganglionic

A

Central

  • MS
  • Wallenberg’s

Pre-ganglionic (neck)

  • Pancoast’s tumour: T1 nerve root lesion
  • Trauma: CVA insertion or carotid endarterectomy

Post-ganglionic

  • Cavernous sinus thrombosis
  • Usually 2O to spreading facial infection via the ophthalmic veins
  • CN 3, 4, 5, 6 palsies
119
Q

Oculomotor nerve palsy features

A
  • Complete ptosis: LPS
  • Eye points down and out: unopposed SOB and LR
  • Dilated pupil, doesn’t react to light: unless spared
  • Ophthalmoplegia and diplopia
120
Q

Oculomotor nerve palsy

Med vs surgical (pathophysiology)

A

Parasympathetic fibres originate in Edinger Westphal Nucleus and run on periphery of oculomotor nerve. Receive rich blood supply from external pial vessels. These fibres are affected late in ischaemic causes (medical) but early in compression (surgical).

121
Q

Oculomotor nerve palsy

Med vs surgical Ddx

A

Medical

  • Mononeuritis: e.g. DM
  • MS
  • Infarction in midbrain
  • Weber’s: CN3 palsy + contralateral hemiplegia
  • Migraine

Surgical

  • ↑ ICP: transtentorial herniation compresses uncus
  • Cavernous sinus thrombosis
  • Posterior communicating artery aneurysm: painful
122
Q

Holmes-Adie (Myotonic) Pupil

A

Dilated pupil that has no response to light and sluggish response to accommodation.

↓ or absent ankle and knee jerks

Benign condition, more common in young females

123
Q

Argyll Robertson Pupil

(features/completion/causes)

A

Features

  • Small, irregular pupils
  • Accommodate but doesn’t react to light
  • Atrophied and depigmented iris

Extras

  • Offer to look for sensory ataxia: tabes dorsalis
  • Dip the urine: glucose

Causes

  • Quaternary syphilis: RPR or TPHA
  • DM
124
Q

RAPD / Marcus Gunn Pupil

features

A

Features

  • Minor constriction to direct light
  • Dilatation on moving light from normal to abnormal eye.

‘Consensual response overrides direct’

125
Q

Features of Optic Atrophy

A
  • ↓ visual acuity
  • ↓ colour vision: esp. red desaturation
  • Central scotoma
  • Pale optic disc
  • RAPD
126
Q

Causes of RAPD

CAC VISION

A

Commonest: MS and glaucoma

Congenital

  • Leber’s hereditary optic neuropathy
    • Epi: mitochondrial, onset 20-30s
    • PC: attacks of acute visual loss, sequential in each eye ± ataxia and cardiac defects
  • HMSN / CMT
  • Friedrich’s ataxia
  • DIDMOAD

Alcohol and Other Toxins

  • Ethambutol
  • Lead
  • B12 deficiency

Compression

  • Neoplasia: optic glioma, pituitary adenoma
  • Glaucoma
  • Paget’s
  • Vascular*: DM, GCA or thromboembolic
  • *Inflammatory**: optic neuritis – MS, Devic’s, DM
  • *Sarcoid** / other granulomatous
  • *Infection**: herpes zoster, TB, syphilis
  • *Oedema**: papilloedema
  • *Neoplastic infiltration**: lymphoma, leukaemia
127
Q

Visual pathway

A
  • Retina
  • Optic nerve
  • Optic chiasm: nasal fibres decussate
  • Optic tract
  • LGN of thalamus
  • Optic radiation
    • Superior field: temporal
    • Inferior field: parietal
  • Visual cortex
128
Q

Visual Fields Ix

A

Perimetry

CT/ MRI brain

129
Q

Homonymous Hemianopia

Features and Extras and Hx

A
  • Retrochiasmatic
  • Greater defect = larger lesion or closer to chiasm
  • Contralateral

Extras

  • Examine for ipsilateral hemiparesis
  • Examine for cerebellar signs
  • Right: test for neglect
  • Left: test for aphasia

Hx

  • Speed of onset
  • Vascular risk factors
130
Q

Visual fields and stroke

MCA vs PCA

A

MCA Stroke

  • MCA supplies the optic radiation in the temporal and parietal lobes
  • Hemiparesis
  • Higher cortical dysfunction: neglect, aphasia

PCA Stroke

  • PCA supplies occipital lobe and visual cortex
  • Homonymous hemianopia c¯ macula sparing
    • Branch of MCA supplies part of visual cortex
  • No hemiparesis
  • May have cerebellar signs
131
Q

Homonymous Hemianopia Causes

A
  • Vascular: ischaemia or haemorrhage
  • SOL: tumour, abscess
  • Demyelination: MS
132
Q

Bitemporal Hemianopia ’

Found on examination, what ‘Extra’ examinations should be considered

A

Chiasmatic lesion

Extras:

  • Take Hx and examine for features of endocrine disease
    • Prolactinoma
    • Acromegaly
    • Cushing’s
133
Q

Bitemporal Hemianopia

causes

A
  • Pituitary tumour
    • Compresses from below → descending visual loss
  • Craniopharyngioma
    • Compresses from above → ascending visual loss
    • Benign suprasellar tumour originating from Rathke’s pouch.
    • Calcified as arises from odontogenic epithelium
134
Q

Monocular Blindness

How to examine

A

No vision in one eye

  • Check counting fingers, movement and light perception
  • Lesion proximal to optic chiasm
    • Eye itself: cornea, vitreous, retina
    • Optic nerve: i.e. optic neuropathy
135
Q

Ophthalmoplegia Eye

examination: H and key elements

A
  • Key Elements
    • Inspect the eye
      • Ptosis
      • Alignment
      • Pupil sizes
    • Ask pt. to tell you if they get double vision
    • Use H to test movements noting:
      • Ophthalmoplegia
      • Diplopia: do cover test
      • Nystagmus
    • Saccades: vertical and horizontal
  • Diplopia
    • Maximal in direction of pull of affected muscle
    • Cover test: outer image disappears c¯ affected eye
136
Q

Ophthalmoplegia: 3rd Nerve Palsy

A
  • Complete ptosis
  • Down and out in rest position
  • ± dilated, non-reactive pupil
  • Diplopia maximal: in and up
137
Q

Ophthalmoplegia: 4th Nerve Palsy

A
  • Slight head tilt: ocular torticollis
  • Appear normal in rest position
  • Failure to depress eye in adduction
  • Diplopia maximal down and in
  • Ask if pt. has trouble walking down stairs
138
Q

Ophthalmoplegia: 6th Nerve Palsy

A
  • Appear normal in resting position
  • Failure to abduct
  • Diplopia maximal in abduction
  • Commonly a false localising sign of ↑ ICP: contralateral lesion
139
Q

Causes of simple Ophthalmoplegia

A

Central NS: MS, vascular, SOL

Peripheral NS: DM (mononeuritis), compression, trauma

140
Q

Ix of simple Ophthalmoplegia

A
  • Urine dip: glucose
  • Bloods: glucose + HbA1c
  • Imaging: MRI brain
141
Q

Internuclear Ophthalmoplegia Examination

A
  • Failure of ipsilateral adduction
  • Nystagmus in the contralateral abducting eye
  • May be bilateral
  • Convergence preserved
142
Q

Internuclear Ophthalmoplegia Neurophysiology

A
  • To maintain convergent gaze, MLF yokes together the nuclei of CN3 and 6.
  • Pontine centre for lateral gaze initiates movement and outputs to CN3 nucleus and CN6 nucleus via the MLF
  • Failure of adduction is ipsilateral to MLF lesion
143
Q

Internuclear Ophthalmoplegia causes

A
  • MS
  • Infarct: ischaemic or haemorrhagic
  • Syringomyelia
  • Phenytoin toxicity
144
Q

Complex Ophthalmoplegia define

A

Dx of exclusion

Ophthalmoplegia doesn’t fit a single pattern

145
Q

Complex Ophthalmoplegia causes

A
  • DM: Mononeuritis multiplex
  • MS
  • Myasthenia gravis
  • Thyrotoxicosis
146
Q

Complex Ophthalmoplegia Ix

A
  • Urine dip: glucose
  • Bloods
    • DM: glucose + HbA1c
    • TFT: ↓TSH
    • MG: anti-AChR antibodies
  • MRI Brain
    • Plaques in the periventricular white matter
147
Q

Hearing loss

Examination

A

Examination: USE a 512Hz TUNING FORK

Weber’s Test

  • Normal: central
  • SNHL: lateralises to normal ear
  • Conductive: lateralises to abnormal ear

Rinne’s Test

  • Positive: AC > BC
  • Negative: BC > AC
    • True: conductive deafness
    • False: complete SNHL
148
Q

Causes of Hearing loss conductive and SNHL

A

Conductive

  • Impaired conduction anywhere between auricle and round window.
    • Canal obstruction: wax, FB
    • TM perforation: trauma, infection
    • Ossicle defects: otosclerosis, infection
    • Fluid in middle ear

SNHL

  • Defects of cochlea, cohlear N. or brain
    • Congenital
      • Alports: SNHL + haematuria
      • Jewell-Lange-Nielsen: SNHL + long QT
    • Acquired
      • Presbyacussis
      • Drugs: gentamicin, vancomycin
      • Infection: meningitis, measles
      • Tumour: vestibular schwannoma
149
Q

Speech

Examination quick screen and (test x3)

A

Aims to Test

  • Dysphonia: impaired production of voice sounds
  • Dysarthria: impaired articulation of sound → words
  • Dysphasia: impairment of language

Quick Screen

  • Ask: How did you get here today?
    • Listen to volume, rhythm, clarity and content
    • Any striking abnormality?

Dysarthria (articulation)

  • Repetition
    • Yellow lorry: test lingual sounds
    • Baby hippopotamus: labial sounds
    • The Leith police dismisseth us: multiple processes
  • Count to thirty: muscle fatigue in MG

Dysphonia (sound)

  • Voice: quiet or hoarse
  • Cough: bovine
  • Say Ahh: vocal cord tension

Dysphasia (language)

  • Name three objects: nominal dysphasia
  • Three stage command: receptive dysphasia
  • Avoid visual clues by instructing from behind
  • Repeat sentence: Today is Thursday
  • Tests for conductive dysphasia

Extras

  • Dominant parietal lobe lesions → dyslexia, dysgraphia and dyscalcula.
  • Read a paragraph: dyslexia
  • Write a sentence: dysgraphia
150
Q

Speech abnormality

Dysarthria pseudo-bulbar/bulbar/cerebelar

A
  • Lesion in tongue, lips, mouth or disruption of NM pathway*
  • *Pseudo-bulbar**
  • Bilateral UMN lesions → spastic dysarthria
  • Difficulty c¯ lingual sounds
    • “Hot Potato speech”
  • Brisk jaw jerk
  • Causes
    • CVA: e.g. bilateral internal capsule infarcts
    • MS
    • MND

Bulbar

  • Unilateral LMN weakness
  • Palatal weakness → nasal “Donald Duck” speech
  • Causes
    • Brainstem infarct
    • MND
    • GBS

Cerebellar: Slurred, drunken speech

151
Q

Speech abnormality

Dysphonia

A
  • Hoarse voice
  • Bovine cough

Cause

  • Local cord pathology: laryngitis, tumour, nodule
  • Recurrent laryngeal N. palsy
152
Q

Speech abnormality

Dysphasia

A
  • Expressive
    • Broca’s area: frontal lobe
    • Non-fluent speech
    • Comprehension intact
  • Receptive
    • Wernicke’s Area: temporal lobe
    • Fluent but meaningless speech
    • Comprehension impaired
  • Conductive
    • Damage to arcuate fasciculus connecting Broca’s and Wernicke’s areas.
    • Comprehension intact
    • Unable to repeat words or phrases
153
Q

What is polio

A
  • RNA virus
  • Affects anterior horn cells
  • Fever, sore throat, myalgia 0.1% develop paralytic polio
  • Asymmetric LMN paralysis
  • No sensory involvement
  • May be confined to upper or lower limbs or both
  • Respiratory muscle paralysis can → death

Polio was eradicated by the 1988 WHO global polio eradication initiative; 37 cases in 2016, VACCINATION PROGRAM -> Herd immunity

PACES Hawthorne (18 decks)

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