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
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
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
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
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
6
Q
Parkinsonism Viva pathophysiology
A
Destruction of dopaminergic neurones in pars compacta of substantia nigra.
- β-amyloid plaques
- Neurofibrillary tangles: hyperphosphorlated tau
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
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
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.
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
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
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
- Full neuro exam
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
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
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
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
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
18
Q
Cerebellar syndrome viva Lateral medullary syndrome (wallenberg’s)
DANVAH
A
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
20
Q
Cerebellar syndrome viva CPA Tx Ddx
A
- Vestibular Schwannoma
- Meningioma
- Cerebellar astrocytoma
- Metastases
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
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
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
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
25
UMN signs Ex
*Inspection/gait/UMN signs/completion*
**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
26
UMN Viva _Bilateral_ LL: spastic paresis
## Footnote
*common/other/mixed UMN and LMN*
**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
27
UMN Viva unilateral LL
## Footnote
*hemisphere and hemicord*
**Hemisphere → Spastic Hemiparesis**
* Stroke
* MS
* SOL
* CP
**Hemicord → Spastic Hemiparesis or Monoparesis**
* MS
* Cord Compression
28
UMN Viva Hx
* MS: tingling, eye problems, ataxia, other weakness
* Cord compression: back pain, fever, wt. loss
* Trauma
* FH
29
UMN Viva Ix; *Imaging & Causes: MS, Compression, SCDC*
**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
30
UMN: Mx (supportive)
Supportive
* **MDT**: GP, neurologist, radiologist, neurosurgeon, specialist nurses, physio, OT
* Orthoses
* Mobility aids
* Urinary: ICSC
* Contractures: baclofen, botulinum injection, physio
31
Cord compression
## Footnote
*Features, causes, Ix, Mx*
**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
32
Cauda equina lesion
## Footnote
* pain/weakness/sensation/sphincters*
* Causes*
* 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
33
Anterior spinal artery/Beck's syndrome
## Footnote
*what/causes/effect*
* 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
34
Human T-lymphotropic virus-1
* 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
35
Syringomyelia
*Characteristics, causes, cardinal signs, other signs*
**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”
2. Wasting/weakness of hands ± Claw hand
3. Loss of reflexes in upper limb
4. 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
36
Acute stroke
## Footnote
*definition and pathogenesis*
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%
37
Stoke clinical features
## Footnote
*Bamford classification (/Oxford Stroke Classification)*
**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
38
Acute stroke Ddx
* Head injury
* ↑ or ↓ glucose
* SOL
* Infection
* Drugs: e.g. opiate OD
39
Acute stroke Mx
**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**
40
Absolute CI to thrombolysis
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
41
Non-acute stroke;
## Footnote
*'work up'/Ix*
**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
42
Non-acute stroke
## Footnote
*Secondary prevention*
* 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%
43
Non-acute stroke
## Footnote
*rehabilitation (MENDS)*
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
44
Non-acute stroke;
## Footnote
*prognosis@1yr*
* 10% recurrence
* PACS
* 20% mortality
* 1/3 of survivors independent
* 2/3 of survivors dependent
* TACS is much worse
* 60% mortality
* 5% independence
45
MS definition and dignosis
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
46
MS *epidemiology*
Lifetime risk: 1/1000
Age: mean @ onset = 30yrs
Sex: F\>M = 3:1
Race: rarer in blacks
47
MS pathophysiology
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))
48
MS *classifications*
* Relapsing-remitting: 80%
* Secondary progressive
* Primary progressive: 10%
* Progressive relapsing
49
MS Examination Presentation (TEAM)
Tingliing Eye; optic neuritis (vCentral vision + eye movement pain)
Ataxia + other cerebellar signs
Motor; usually spastic parapesis
50
MS Features
## Footnote
*(sensory, motor, eyes, cerebellum, GI, GU, Lhermiutte's)*
* 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
51
MS; Optic neuritis
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
52
MS
## Footnote
*INO / ataxic nystagmus / conjugate gaze palsy*
* Disruption of MLF connecting CN6 to CN3
* Weak adduction of ipsilateral eye
* Nystagmus of contralateral eye
* Convergence preserved
53
MS Viva Ix
* 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
54
MS viva
## Footnote
*Ddx*
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
55
MS viva
## Footnote
*Mx acute attack*
Methylpred 1g IV/PO /24h for 3d
* Doesn’t influence long-term outcome
* ↓ duration and severity of attacks
56
MS viva
## Footnote
*Mx Prevent relapse*
* 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
57
MS viva
## Footnote
*Mx Symptoms: Fatigue Depression Pain Spasticity Urgency / frequency ED Tremor*
Fatigue: modafinil
Depression: SSRI (citalopram)
Pain: amitryptylline, gabapentin
Spasticity: physio, baclofen, dantrolene, botulinum
Urgency / frequency: oxybutynin, tolterodine
ED: sildenafil
Tremor: clonazepam
58
MS viva
## Footnote
*poor prognosis*
* Older female
* Motor signs @ onset
* Many relapses early on
* Many MRI lesions
59
MND Examination
## Footnote
*I TPR S Completion*
* 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
60
MND viva
## Footnote
*Ddx*
* Cervical cord compression → myelopathy
* Brainstem lesions
* Polio: asymmetrical LMN paralysis
* Mixed UMN and LMN Signs
* MND
* Ataxia, Friedrich’s
* SCDC: B12
* Taboparesis
61
Viva
## Footnote
*mixedUMN/LMN signs Ddx*
**Mixed UMN and LMN Signs (MAST)**
* MND
* Ataxia, Friedrich’s
* SCDC: B12
* Taboparesis
62
MND Viva
## Footnote
*Ix*
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
MND viva
## Footnote
*Mx*
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
MND viva Prognosis
Most die w/i 3yrs: Bronchopneumonia and respiratory failure
Worse prog: elderly, female, bulbar involvement
65
MND viva
## Footnote
*classification*
* 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
LMN signs
* Wasting
* Fasciculation
* Hypotonia
* Hyporeflexia
67
LMN
C*auses:*
1. *Bilateral, Symmetrical and **Distal***
2. *Bilateral, Symmetrical and **Proximal***
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
LMN viva
## Footnote
*Unilateral causes*
Isolated to single limb + no sensory signs
* Old polio
Localised to group of muscles c¯ same supply
* Segmental: nerve roots, plexus
* Peripheral: mononeuropathy
69
LMN Ddx
## Footnote
*Hand wasting*
**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
LMN
## Footnote
*Ix **proximal** myopathy*
* 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
LMN
## Footnote
*Ix Mononeuropathy*
* Bloods
* DM: glucose, HbA1c
* B12, folate
* Vasculitis: ESR, ANA, ANCA
* EMG + nerve conduction
72
LMN
## Footnote
*Ix radiculopathy/plexopathy*
MRI
73
LMN
## Footnote
*Ix peripheral polyneuropathy*
* 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
Peipheral polyneuropathy
## Footnote
*Examination sensory/motor/completion*
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
Peripheral polyneuropathy
*Ddx (*causes*) mainly sensory vs mainly motor*
* 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
Peripheral polyneuropathy
## Footnote
*Mx (general and specific)*
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
Peripheral polyneuropathy
*Hx*
* Time-course
* Precise symptoms
* Ataxia: B12
* Painful dysesthesia: EtOH, DM
* Assoc. events
* D&V: GBS
* ↓wt: Ca
* Arthralgia: connective tissue
* Travel, EtOH, drugs
78
Diabetic neuropathy
## Footnote
* Examination of lower limbs*
* (Inspection/motor/sensory/completion)*
* 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
Diabetic neuropathy viva
## Footnote
*Hx*
* Pain: esp. @ night
* Glycaemic control
* Complications of insulin
* Other micro- and macro-vascular complications
80
Diabetic neuropathy viva
## Footnote
*pathophysiology*
* Metabolic*: glycosylation, ROS, sorbitol accumulation
* Ischaemia*: loss of vasa nervorum
81
Diabetic neuropathy viva
## Footnote
*Ix*
* Urine: glucose, ACR
* Blood
* Glucose
* HbA1c
* U+E
82
Diabetic neuropathy viva
## Footnote
*Mx*
* MDT: GP, endocrinologist, neurologist, DNS
* Good glycaemic control
* Amitriptyline, Gabapentin
* Capsaicin cream
83
Diabetic Autonomic neuropathy
## Footnote
*Sx*
* **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
Charcot-Marie-Tooth syndrome
## Footnote
*(AKA) Define and pathophysiology*
Peroneal Muscular Atrophy
*Hereditary Motor and Sensory Neuropathy*
Pathophysiology; Group of inherited motor and sensory neuropathies
85
Charcot-Marie-Tooth syndrome Viva
## Footnote
* Examination*
* (Inspection, motor, sensory)*
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
HMSN1 and HMSN2
## Footnote
*(herediatry motor sensory neuropathy)*
* HMSN1
* Commonest form
* Demyelinating
* AD mutation in the peripheral myelin protein 22 gene
* HMSN2
* Second commonest form
* Axonal degeneration
* Autosomal dominant
87
Charcot-Marie-Tooth syndrome Viva
## Footnote
*Ix*
* Nerve conduction studies
* HMSN1: *demyelination* → ↓ conduction *velocity*
* HMSN2: *axonal* degeneration → ↓ *amplitude*
* Genetic testing
* HMSN1: Peripheral myelin protein 22 (PMP22) gene
88
Charcot-Marie-Tooth syndrome Viva
## Footnote
*Mx*
* MDT: GP, neurologist, specialist nurses, physio, OT
* Foot care and careful shoe choice
* Orthoses: e.g. ankle braces
89
Myasthenia Gravis
*Ex*
(inspection, eyes, facial movements, voice, limbs, completion)
* 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
Myasthenia Gravis Viva
## Footnote
*Ix*
* 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
Myasthenia Gravis Viva
## Footnote
* associations*
* \<50, female // \>50, male*
**\<50, female** - AI disease: DM, RA, Graves, SLE
**\>50, male** - Thymoma
92
Myasthenia Gravis Viva
## Footnote
*Mx acute and chronic*
**Acute**
* Plasmapheresis or IVIg
* Monitor FVC: consider ventilation
**Chronic**
* Pyridostigmine
* Immunosuppression: steroids and azathioprine
* Thymectomy: benefit even if no thymoma
93
LEMS
* Abs vs. VGCC
* Often paraneoplastic: e.g. SCLC
* Lower limb girdle weakness
* Weakness *improves* on repetitive testing
94
DDx *Bilateral Ptosis*
* MG
* Myotonic dystrophy
* Congenital
* Senile
* Bilateral Horner’s (rare)
95
GBS
## Footnote
*Pathophysiology*
*...is an acute polyneuropathy*
Molecular *mimicry*: Abs x-react c¯ ganglioside
Bacteria: C. jejuni, mycoplasma
Viruses: CMV, EBV
96
GBS
## Footnote
* Classification*
* (AIDP/MF)*
***AIDP***: acute autoimmune demyelinating polyneuropathy
***Miller-Fisher:*** ophthalmoplegia + ataxia + areflexia
97
GBS
## Footnote
*features*
* Symmetrical ascending flaccid paralysis
* Sensory disturbance: paraesthesia
* Autoimmune neuropathy: labile BP
98
GBS
## Footnote
*Ix*
* Evidence of infection: e.g. stool MC+S
* *_Anti-ganglioside Abs_*
* LP: ↑↑ CSF protein
* Nerve conduction studies: demyelination
99
GBS
## Footnote
*Mx*
* 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
GBS
## Footnote
*Prognosis*
* 85% complete recovery
* 10% unable to walk alone at 1yr
* 5% mortality
101
Facial nerve palsy
## Footnote
* Examination*
* (Inspection & weakness &UMN vs LMN)*
**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
Facial nerve palsy
## Footnote
*Examination:Other CN involvement*
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
Facial Nerve palsy
## Footnote
* Ex*
* completion UMN and LMN*
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_, **_C_**N and _cerebellar_ function
* Test _taste_
104
Facial nerve palsy
## Footnote
*(unilateral) causes*
* 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
Facial nerve palsy
## Footnote
*bilateral causes*
* Bilateral Bell’s
* Sarcoidosis
* GBS
* Lyme
* Pseudopalsy: MG, Myotonic dystrophy
106
Facial nerve palsy viva
## Footnote
*Hx Symptoms and causes and aguesia/hyperacusis*
* 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
Facial nerve palsy Viva
## Footnote
*Ix*
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
Facial nerve palsy
## Footnote
* Mx*
* (bells)*
* Prednisolone w/i 72hrs
* Valaciclovir if VZV suspected
* Protect eye
* Dark glasses
* Artificial tears
* Tape closed @ night
109
Facial nerve palsy
## Footnote
*complications*
Aberrant Neural Connections e.g...
Synkinesis: e.g. blinking causes up-turning of mouth
Crocodile tears: eating stimulates unilateral lacrimation, not salivation
110
FN palsy
## Footnote
*Prognosis*
* Incomplete paralysis: recovers completely w/i wks
* Complete: 80% get full recovery
* Remainder have delayed recovery or permanent neurological / cosmetic abnormalities
111
FN palsy: *Bell's*
* 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
FN palsy: Ramsay Hunt
* 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
FN palsy: *Cholesteatoma*
* 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
FN Palsy: Lyme disease
* Borellia burgdorferi
* Early Local: Erythema migrans + systemic malaise
* Late Disseminated
* CN palsy: esp. _facial palsy_
* Polyneuropathy
* Meningoencephalitis
* Arthritis
* Myocarditis
* Heart block
115
Facial anaesthesia
## Footnote
*Examination*
* ↓ 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
Facial anaesthesia
## Footnote
* Ddx*
* (Supranuclear/nuclear/peripheral mononeuropathy)*
**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
Horner's
## Footnote
* Examination*
* (Face/Neck/hands/completion)*
**Face: PEAS**
* **P**tosis: partial (superior tarsal muscle)
* **E**nophthalmos
* **A**nhydrosis
* **S**mall 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
Horner's
## Footnote
*Differential: central, pre-ganglionic post-ganglionic*
**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
Oculomotor nerve palsy features
* 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
Oculomotor nerve palsy
## Footnote
*Med vs surgical (pathophysiology)*
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
Oculomotor nerve palsy
## Footnote
*Med vs surgical Ddx*
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
Holmes-Adie (Myotonic) Pupil
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
Argyll Robertson Pupil
## Footnote
*(features/completion/causes)*
**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
RAPD / Marcus Gunn Pupil
## Footnote
*features*
Features
- Minor constriction to direct light
- Dilatation on moving light from normal to abnormal eye.
'Consensual response overrides direct'
125
Features of Optic Atrophy
* ↓ visual acuity
* ↓ colour vision: esp. red desaturation
* Central scotoma
* Pale optic disc
* RAPD
126
Causes of RAPD
## Footnote
*CAC VISION*
Commonest: ***MS*** and ***glaucoma***
**C**ongenital
* 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
**A**lcohol and Other Toxins
* Ethambutol
* Lead
* B12 deficiency
**C**ompression
* 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
Visual pathway
* Retina
* Optic nerve
* Optic chiasm: nasal fibres decussate
* Optic tract
* LGN of thalamus
* Optic radiation
* Superior field: temporal
* Inferior field: parietal
* Visual cortex
128
Visual Fields *Ix*
Perimetry
CT/ MRI brain
129
Homonymous Hemianopia
## Footnote
*Features and Extras and Hx*
* 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
Visual fields and stroke
## Footnote
*MCA vs PCA*
**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
Homonymous Hemianopia Causes
* Vascular: ischaemia or haemorrhage
* SOL: tumour, abscess
* Demyelination: MS
132
Bitemporal Hemianopia *'*
## Footnote
*Found on examination, what 'Extra' examinations should be considered*
Chiasmatic lesion
Extras:
* Take Hx and examine for features of endocrine disease
* Prolactinoma
* Acromegaly
* Cushing’s
133
Bitemporal Hemianopia
## Footnote
*causes*
* 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
Monocular Blindness
## Footnote
*How to examine*
*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
Ophthalmoplegia Eye
## Footnote
*examination: H and key elements*
* 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
Ophthalmoplegia: 3rd Nerve Palsy
* Complete ptosis
* Down and out in rest position
* ± dilated, non-reactive pupil
* Diplopia maximal: in and up
137
Ophthalmoplegia: 4th Nerve Palsy
* 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
Ophthalmoplegia: 6th Nerve Palsy
* Appear normal in resting position
* Failure to abduct
* Diplopia maximal in abduction
* Commonly a false localising sign of ↑ ICP: contralateral lesion
139
Causes of simple Ophthalmoplegia
**Central** NS: MS, vascular, SOL
**Peripheral** NS: DM (mononeuritis), compression, trauma
140
Ix of simple Ophthalmoplegia
* Urine dip: glucose
* Bloods: glucose + HbA1c
* Imaging: MRI brain
141
Internuclear Ophthalmoplegia Examination
* Failure of ipsilateral adduction
* Nystagmus in the contralateral abducting eye
* May be bilateral
* Convergence preserved
142
Internuclear Ophthalmoplegia Neurophysiology
* 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
Internuclear Ophthalmoplegia causes
* MS
* Infarct: ischaemic or haemorrhagic
* Syringomyelia
* Phenytoin toxicity
144
Complex Ophthalmoplegia define
Dx of exclusion
Ophthalmoplegia doesn’t fit a single pattern
145
Complex Ophthalmoplegia causes
* DM: Mononeuritis multiplex
* MS
* Myasthenia gravis
* Thyrotoxicosis
146
Complex Ophthalmoplegia Ix
* Urine dip: glucose
* Bloods
* DM: glucose + HbA1c
* TFT: ↓TSH
* MG: anti-AChR antibodies
* MRI Brain
* Plaques in the periventricular white matter
147
Hearing loss
## Footnote
*Examination*
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
Causes of Hearing loss conductive and SNHL
**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
Speech
## Footnote
*Examination quick screen and (test x3)*
**_Aims to Test_**
* Dys**phonia**: impaired production of voice **sounds**
* Dys**arthria**: impaired **articulation** of sound → words
* Dys**phasia**: 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
Dys**phonia** (sound)
* Voice: quiet or hoarse
* Cough: bovine
* Say Ahh: vocal cord tension
Dys**phasia** (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
Speech abnormality
## Footnote
*Dysarthria pseudo-bulbar/bulbar/cerebelar*
* 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
Speech abnormality
## Footnote
*Dysphonia*
* Hoarse voice
* Bovine cough
Cause
* Local cord pathology: laryngitis, tumour, nodule
* Recurrent laryngeal N. palsy
152
Speech abnormality
## Footnote
*Dysphasia*
* 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
What is polio
* 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
## Footnote
*Polio was eradicated by the 1988 WHO global polio eradication initiative; 37 cases in 2016, VACCINATION PROGRAM -\> Herd immunity*
