PACES Station 3 Flashcards
1
Q
Presenting Cardiac Cases
A
No stigmata of endocarditis
Pulse rate is…, regular/irregular, volume, character
Venous pressure
On examination of chest…
2
Q
Clinical Features of Aortic Stenosis
A
Slow rising pulse, low volume (severe)
Venous pressure not elevated
Undisplayed heaving apex
Soft second heart sound
Fourth heart sound (not in AF), forced atrial contraction
Ejection systolic murmur, louder in expiration and radiating to carotids
3
Q
Causes of aortic stenosis
A
Common:
- bicuspid aortic valve
- degenerative calcification
- rheumatic valve disease
- congenital
Rare:
- Infective endocarditis
- Hyperuricaemia
- Pagets disease of bone
4
Q
Differential Diagnosis for Ejection Systolic Murmur
A
Aortic stenosis
HOCM
Supravalvular aortic stenosis (Williams syndrome)
5
Q
Classifying severity in Aortic Stenosis
A
Aortic valve area:
- normal 3-4cm2
- mild >1.5cm
- moderate 1-1.5cm2
- severe <1cm
Pressure gradient:
- encorporates aortic valve area, cardiac output and heart rate
- severe is mean gradient >50mmHg
6
Q
What is aortic sclerosis?
A
Mild thickening/calcification of trileaflet aortic valve
Absence of outflow obstruction
Affects 1/4 over 65s
Progressive and overtime leads to stenosis
Inc risk of MI and cardiovascular mortality
Atherosclerosis as common aetiology
7
Q
Clinically differentiating aortic stenosis and sclerosis
A
Normal pulse volume and character in sclerosis
Localised murmur without radiation
8
Q
Causes of reversed splitting of second heart sound
A
Severe aortic stenosis LBBB HOCM PDA WPW B
9
Q
Complications of aortic stenosis
A
LVF Sudden death Pulmonary HTN Arrhythmia - AF and VT Heart block IE Systemic emboli Haemolytic anaemia
10
Q
Management of asymptomatic patient
A
Ask to report symptoms of angina, palpitations, syncope and breathless
Regular screening
If become symptomatic may warrant valve replacement
11
Q
Mechanism of Angina in AS
A
May have normal angio
but hypertrophied left ventricle has decreased blood flow reserve so sensitive to ischaemic injury
12
Q
Mechanism of Syncope in AS
A
low cardiac output state
arrhythmia
13
Q
Investigation of AS
A
ECG: LVH, left atrial hypertrophy (bifid Pwaves in II), LAD, conduction abnormalities
CXR: rib notching (sign of coarctation), calcification of valve, cardiomegaly (late), prominent pulmonary arteries (pHTN)
Echo: LV function, aortic valve area
Angio: exclude coronary artery disease as cause of symptoms, all being considered for replacement need
14
Q
Indications for Aortic Valve Replacement in AS
A
Symptomatic severe >50mmHg
Asymptomatic moderate/severe undergoing other cardiac surgery e.g. bypass, other valve
Asymptomatic Severe AS plus any of the following: - LV dysfunction - abnormal BP response to exercise - VT Valve area <0.6
15
Q
Williams Syndrome
A
Rare genetic condition: elfin facies, cardiovascular anomalies, mental retardation, sensorineural deficit, high calcium
Cardiac anomalies include supravalvular aortic stenosis, pulmonary stenosis, MR
Deletion in elastin gene chr 7
16
Q
Role of statins in AS
A
Atherosclerotic process
Use of statins to slow progression
However recent study (SALTIRE) did not show statins to significantly slow progression calcific aortic stenosis
17
Q
Clinical Features of Mitral Stenosis
A
Irregular low volume pulse (often in AF)
Elevated JVP
Malar flush (low cardiac output state)
Undisplayed tapping apex
Loud first heart sound (if mobile and pliable leaflets)
Opening snap and mid diastolic rumbling murmur heard best in expiration in left lateral position
Features of CCF
Early diastolic murmur at left sternal edge - graham steell murmur of pulmonary regard
18
Q
Causes of Mitral Stenosis
A
Rheumatic fever Congenital RA SLE Carcinoid syndrome Fabrys disease
19
Q
Causes of mid-diastolic rumbling murmur
A
Left atrial mass (myxoma)
Left atrial thrombus
Severe MR (inc flow across valve)
20
Q
Pathophysiology of Mitral Stenosis
A
Normal area 4-6cm2
<2.5cm2 impedes free flow over blood and leads to increased left atrial pressure
Critical stenosis if <1cm2 leads to pulmonary congestion and mimics left ventricular failure but with preserved LV contractility
21
Q
Clinical Markers of Severe Mitral Stenosis
A
Early opening snap Increased length of murmur Signs of Pulmonary HTN Signs of Pulmonary congestion Graham-Steel murmur (PR) Low pulse pressure
22
Q
Complications of mitral stenosis
A
Left atrial enlargement AF + left atrial thrombus Pulmonary HTN Pulmonary oedema RHF
23
Q
Differential Diagnosis for Malar Flush
A
Mitral stenosis Hypothyroidism Cold weather Carcinoid syndrome SLE SS Irradiation Polycythaemia
24
Q
Management of Mitral Stenosis
A
Asymptomatic in sinus - endocarditis prophylaxis, regular follow up with echo
Manage AF
Symptomatic - diuretics, as surgery worsens then for surgery
Surgery: closed/open commisurotomy, mitral valve replacement
Indications - pulmonary HTN, haemoptysis, recurrent thromboembolic events despite anticoag
25
Criteria for Valvuloplasty in Mitral Stenosis
Mobile valve
Minimal calcification
No MR
No left atrial thrombus
26
Cause of Hoarse Voice in Mitral Stenosis
Enlarged left atria compresses recurrent laryngeal nerve leading to vocal cord paralysis
(Ortners syndrome)
OR
Hypothyroidism from amiodarone used to treat AF
27
Clinical Features of Prosthetic Aortic Valve
```
No features of endocarditis
Prosthetic click after carotid pulse
Midline sternotomy scar, check for any venous harvest scars re:CABG
Ejection systolic murmur
Anaemia?
```
Any features of heart failure to suggest valve is functioning well
Any complications of anticoagulation visible
NG Double click suggests -Starr-Edwards ball and cage prosthesis
28
Indications for Aortic Valve Replacement
Severe AS gradient >50mmHg and symptomatic
Asymptomatic AS moderate/severe but needs CABG anyway
Severe AS with LV dysfunction, abnormal BP in exercise, VT, valve area <0.6cm2
Symptomatic severe AR
Asymptomatic AR undergoing surgery anyway
AR with severe LV dysfunction (EF<50%)
Other:
IE failed medical therapy
Enlarging aortic root diameter
Acute severe AR - ruptured valsalva aneurysm
29
Different types of prosthetic valve
Mechanical
-starr-edwards ball and cage - high incidence of haemolysis
Medtronic-Hall - tilting disc valve
Xenografts
-porcine or pericardial
Cadaveric
30
Indications for Bioprosthetic Valve over Metallic
Where anticoag is contraindicated
Shorter life expectancy
>70 years as rate of degeneration slower in this age group
31
Complications of prosthetic valve
```
Thromboembolism
Anticoag complications
Valve dysfunction
Endocarditis
Haemolysis
```
32
Causes of anaemia in patient with prosthetic valve
Blood loss secondary to anticoag
Haemolysis
Endocarditis
33
Clinical Features of Prosthetic Mitral Valve
```
No stigmata of endocarditis
Pulse features
Anaemia
Prosthetic click coincides with first heart sound (with carotid pulse) or 2 clicks (Starr-Edwards)
Midline sternotomy scar
Any features of pulmonary HTN
```
34
Indications for Mitral Valve Replacement
Stenosis with pulmonary congestion, pHTN, haemoptysis or recurrent thromboembolic events
Regurg with LV dysfunction, EF<60%
35
Clinical Features of Mitral Regurg
```
No features of endocarditis
Pulse blah blah
Displaced and thrusting apex
Pan systolic murmur radiating to axilla
Any signs of pulmonary HTN/congestion
- if present then loud pulmonary component of second heart sound
```
36
Causes of MR
```
Rheumatic fever
Mitral valve prolapse
IE
LV dilatation (functional MR)
Marfans
Ehlers-Dalos
OI
RA
SLE
Cardiomyopathy
```
37
Mechanism of Functional MR
In LV dysfunction get dilatation of mitral valve annulus and lateral displacement of papillary muscles
38
Clinical Signs of Severe MR
```
Features of pulmonary HTN
Features of pulmonary congestion
Displaced apex
Third heart sound
Soft first heart sound
Precordial thrill
```
39
Differential Diagnosis for Pansystolic Murmur
Mitral regurgitation
Tricuspid regurgitation - pulsatile liver, no radiation to axilla, louder left sternal edge
VSD - heard throughout precordium, smaller defect = louder murmur
40
Management of MR
Asymptomatic
- annual echo
- endocarditis prophylaxis
Manage AF
Manage HF - diuretics, ACEi, beta blocker
Indications for surgery:
NYHA III despite optimum medical therapy
EF falls below 60% consider
41
Clinical Features of Aortic Regurgitation
```
No features of endocarditis
Large volume collapsing pulse
Wide pulse pressure
JVP not elevated
Displaced, thrusting apex
Early diastolic murmur at left sternal edge loudest with patient leaning forward in expiration
```
Corrigans sign - visible carotid pulsation
Quinkes sign - capillary pulsation in fingernails
De Mussets sign - head nodding with each heart beat
42
Causes of AR
```
Bicuspid aortic valve
HTN
Rheumatic fever
Aortitis - Takayasus, ank spond, Reiters, psoriatic arthropathy
RA
SLE
CTD: marfans, ehlers-danlos
```
Acute causes: dissection, IE, ruptured sinus of valsalva aneurysm
43
Clinical Signs of Severe AR
```
Wide pulse pressure
Long duration murmur
Third heart sound
Austin-Flint murmur
Signs of pHTN
Signs of LVF
```
44
What is an Austin-Flint Murmur
Low frequency mid-diastolic murmur heart at apex caused by aortic regarg jet on anterior mitral valve leaflet (mimics MS)
45
Causes of collapsing/bounding pulse
```
AR
Anaemia
Fever
Pregnancy
Thyrotoxicosis
PDA
AV fistula
```
46
Role of vasodilators in AR
reduce systolic BP as HTN will increase wall stress
| ACEi/nifedipine recommended for symptomatic patients
47
Clinical Features of Mitral Valve Prolapse
Late systolic crescendo-decresendo murmur loudest at left sternal edge
Hx: palpitations, atypical chest pains, fatigue, dyspnoea, anxiety
48
Causes of Mitral Valve Prolapse
Primary
Secondary to :
Marfans - high palate, arachnodactyly, pectus excavatum
Ehlers-Dalos - blue sclera, hyper extensible skin, purpura/poor skin healing
Pseudoxanthoma elasticum - pluck chicken skin
OI: blue sclera, hearing aids
PCKD: scars, RRT
SLE
49
Complications of Mitral Valve Prolapse
```
Stroke
Chordal rupture
Endocarditic
Arrhythmia (long QT)
Sudden death
```
50
Pathophysiology of mitral valve prolapse
Collagen dissolution results in myxomatous degeneration. Stretching of leaflets and chordae tendinae
51
Differential diagnosis for Mitral Valve Prolapse Murmur
AS
PS
HOCM
Trivial MR
52
Management of MVP
Reassure asymptomatic
Endocarditis prophylaxis in those with audible click/murmur
Treat arrhythmia
Treat atypical chest pain with simple analgesia and beta blockers
53
Clinical Features of Pulmonary HTN
```
Raised JVP with prominent a wave and giant v wave
Pulse ? AF, ? slow due to rate control
Undisplayed apex
Parasternal heave
Loud pulmonary component of S2
Pan systolic murmur if functional TR
Sacral and pedal oedema
```
54
Definition of Pulmonary HTN
Mean pulmonary artery systolic pressure of >25mmHg at rest
55
Causes of Secondary Pulmonary HTN
1. Increased pulmonary venous pressure:
- LV dysfunction
- Mitral/aortic valve disease
2. Decreased area of pulmonary vascular bed
- PE
- ILD
- Collagen vascular disease
3. Chronic Hypoxia
- COPD
- ILD
- OSA
- Neuromusclar disease
4. Left to right shunt
- ASD/VSD
56
Primary Pulmonary HTN
Rare disease effecting children and young adults with pHTN without demonstrable cause
1/10 are familial
Associated with CTD and HIV
57
Investigations for Pulmonary HTN
```
ECG for evidence of Right heart strain/left
ABG
CXR: prominent vasculature, oligaemic lung fields, may have large heart or evidence of lung pathology suggesting underlying cause
Echo
CTPA/VQ to exclude clot
Lung functions
HRCT - ILD
Right and left heart catheterisation
```
58
Treatment of secondary pulmonary HTN
```
Treat underlying cause
Diuretics for congestive symptoms
LTOT
Anticoag if VTE
Vasodilator therapy - prostacyclins
```
59
Treatment of Primary Pulmonary HTN
Diuretics for congestive symptoms to reduce preload to right heart
Anticoag
Vasodilators - nifedipine, prostacyclin analogues
Surgery - atrial septostomy (create right to left shunt)
Transplant
60
Clinical Features of VSD
Parasternal thrill
Pan systolic murmur all over chest, loudest at left sternal edge
Absence of pulmonary HTN or LV enlargement suggests it is haemodynamically insignificant
61
Causes of VSD
Congenital:
Maternal factors - diabetes, PKU, alcohol consumption
Aneuploid syndromes eg trisomy 21, digeorge deletion 22
Acquired:
Post MI
Iatrogenic
62
Classification of VSD
Perimembranous - most common
Supra-cristal, infundibular - rare
Muscular
Posterior
63
Complications of VSD
```
IE
Pulmonary HTN
Aortic regurgitation
LV dysfunction
Eisenmengers
Arrhythmia
```
64
Mechanism of spontaneous closure of VSD
Hypertrophy of muscular septum
Formation of fibrous tissue
Sub aortic tags
65
Investigations of VSD
CXR - large defect cardiomegaly and features of pulmonary HTN
ECG - large defect LV hypertrophy
Echo - location, size and direction of shunt, function and pressures
66
Management of VSD
Small - reassure, prophylaxis, encourage living normal life
Larger - prophylaxis, diuretics for symptoms, treat LV dysfunction, closure if no contraindications
Contraindications to VSD closure - severe irreversible pulmonary HTN
67
Clinical Features of ASD
Fixed splitting of second heart sound
Ejection systolic murmur at upper left sternal edge
Absence of pulmonary HTN suggests haemodynamically insignificant shunt
68
Types of ASD
Ostium secundum
Ostium primum
Sinus venosus ASD
Coronary sinus ASD
69
Mechanism of widely split S2 in ASD
Left to right shunt
| Increased right heart volume so slower for P2 to close
70
Complications of ASD
```
Arrhythmia
Pulmonary HTN
Eisenmengers
IE
Paradoxical embolism
```
71
Clinical Features of Coarctation of Aorta
Developed upper torso compared to lower torso
Pulse differential?? left diminished in correction proximal to left subclavian artery (pre ductal - infantile)
Vigorous carotid pulsation
Bruit and thrills over collaterals of scapula
Ejection systolic murmur
Left thoracotomy scar if repaired
72
Different types of coarctation
Infantile - pre ductal - proximal to origin of left subclavian, heart failure in infancy
Adult - post ductal, presents between 15-30 years of age
73
Cardiac conditions associated with coarctation
```
Bicuspid aortic valve
PDA
VSD
Mitral valve anomaly
Transposition of Great Vessels
Hypo plastic left heart
```
74
Non-cardiac associations with coarctation
Turners syndrome
Berry aneurysm
Haemangiomas
Renal abnormalities
75
Where do collaterals arise in long standing coarctations
Internal mammary to external iliac
| Spinal and intercostal arteries to descending aorta (rib notching)
76
Complications of coarctation
HTN
Endocarditis
Hypoplastic limbs depending on site and severity
LV dysfunction
77
Clinical Features of PDA
```
No features of endocarditis
Large volume collapsing pulse
Parasternal heave
Left subclavian thrill
Continous machinery murmur in left subclavicular area
```
IF features of pulmonary HTN/congestion then haemodynamically significant
Also comment on any cyanosis/clubbing
78
Pathophysiology of PDA
Derived from 6th aortic arch
As fetes diverts blood from pulmonary artery into systemic circulation, promoted by prostaglandin E2
Functional closure 15hrs after birth due to breathing and increased pO2
79
Causes of PDA
```
Prematurity
Low birth weight
Maternal NSAID use (prostaglandins antagonist)
Maternal rubella
High altitude
Fetal alcohol syndrome
Maternal phenytoin
```
80
Main complications of PDA
```
LV dysfunction
IE
Pulmonary HTN
Eisenmengers
Ductal aneurysm/rupture
```
81
Clinical Features of Pulmonary Stenosis
Ejection systolic murmur with prominent a wave in JVP (not elevated), parasternal heave
Louder in inspiration (unlike aortic stenosis)
Signs of right heart failure without pulmonary congestion
82
Causes of Pulmonary Stenosis
Congenital
Rheumatic
Carcinoid
Noonans
Congenital rubella - supravalvular
TOF - subvalvular
83
Grading pulmonary stenosis
Area
Severe <0.5cm2
Transvalvular gradient
severe >80mmHg
84
Noonans Syndrome
Autosomal dominant/sporadic
Male phenotypic form of Turners but karyotype XX/XY
MSK: short stature, webbed neck, wide spaced nipples
Cardiac: pulmonary stenosis, Hypertrophic cardiomyopathy, ASD/VSD
Facies: triangular, ptosis, strabismus, high nasal bridge
Haem: vWF, coag defects
Small genitalia, undescended testes
85
Watsons Syndrome
Noonans + Neurofibromatosis
86
Clinical Features of Tetralogy of FAllot
```
Central cyanosis
Clubbing
Diminished left radial pulse
Smaller left arm
Thoracotomy scar
Parasternal heave
Loud ESM (pulm)+ early diastolic murmur (aortic)
```
87
What are the components of TOF
VSD
Pulmonary infundibular stenosis
Right ventricular hypertrophy
Overriding aorta
88
Complications of TOF
```
Cyanotic spells
Endocarditis
RHF
Polycythaemia
Paradoxical emboli
Cerebral abscess
```
89
Causes associated with TOF
Catch22 digeorge
Fetal alcohol syndrome
Maternal PKU
90
Black-Taussig shunt
Palliative procedure used in TOF (not common anymore)
Anastomosis of subclavian artery and pulmonary artery thus bypassing right ventricular outflow obstruction
Would have thoracotomy scar and absent left radial pulse, otherwise healed
91
Clinical Features of Eisenmengers
Central cyanosis and pulmonary HTN!!!
Clubbing
Features of underlying defect e.g. VSD, ASD, PDA
92
What happens to VSD murmur in Eisenmenges
As right to left shunt develops, pressure equalises and murmur disappears.
A murmur in VSD and eisenmengers is likely functional TR
93
Hypertrophic Cardiomyopathy
```
Jerky pulse with double carotid impulse
Prominent a waves
Double apical impulse, undisplaced, heaving
Reversed splitting S2
4th heart sound
Pansystolic/ESM (LV outflow obstruction)
```
94
Difference between Hypertrophic Cardiomyopathy and HOCM
HCM common, only with evidence of LV outflow gradient is HOCM
95
Genetics of HCM
Autosomal dominant, 50% familial.
| Over 200 distinct mutations identified, multiple chromosmes
96
Complications of HCM
```
Heart failure (diastolic in early)
AF
Arrhythmia
Sudden death
Angina
Endocarditis
```
97
Markers of Poor Prognosis in HCM
```
History of syncope
Family hx of sudden death
Non sustained VT on Holter
Marked LV wall thickness
Drop in BP during exercise
```
98
reasons for Angina in HCM despite normal angio
Increased muscle mass
Decreased capillarity density
Abnormal intramural coronal arteries
Increased myocardial O2 consumption
99
Investigations of HCM
ECG: LV hypertrophy, LAD, LA dilatation - bifid p waves in V1-V2, Q was in II, III and avF, ST/T wave changes, AF
CXR: cardiomegaly in late, large left atrial appending and double right heart border
Echo: LV hypertrophy, asymmetry septal hypertrophy, dynamic LV outflow obstruction, diastolic/systolic dysfunction
Exercise treadmill: ischaemia, inducible arrhythmia
Holter; arrythmias
100
Conditions associated with HCM
WPW
| Friedrichs ataxia
101
Management of HCM
Endocarditis prophylaxis, education, screening
Without outflow obstruction:
- no therapy if asymptomatic
- if symptoms then beta blocker or verapamil, diuretics, ACEi, treat AF, ICD for ventricular arrthmias
```
With outflow obstruction
All get beta blockers
Treat AF, diuretics
ICD
Alcohol septal ablation - controlled septal MI by injecting alcohol into septal arteries to reduce obstruction, alternative to surgical myomectomy. - risk of VSD/heart block.
```
102
Clinical Features of Parkinsons Disease
Expressionless facies (hypomimia)
Bradkinesia - ask to open and close hands repeatedly or tap foot
Reduced blink rate
Soft speech (hypophonia)
Blepharoclonus - eye lid tremor when eyes gently closed
Tremor 4-6Hz, resting, pill rolling often asymmetric, enhanced by mental activity
Lead pipe rigidity - inc tone throughout passive movement
Cog wheeling
Forward stooping posture with lowered head and flexed knees and elbows
Shuffling small steps gait with reduced arm swing
May have loss of postural reflexes
Look for features of parkinson plus incl gaze palsy
TO end: measure postural BP, MMTS, handwriting and assess skin (seb dermatitis)
103
What is Parkinsonism?
Movement disorder characterised by bradykinesia and at least 1 of tremor, rigidity and postural instability
104
Causes of Parkinsonism
```
Idiopathic
Drugs: valproate, metoclopramide, prochlorperazine
Tumour of basal ganglia
Lewy Body Dementia
Dementia pugilistica
Normal pressure hydrocephalus
Wilsons
Anoxic brain injury
Parkinsons plus:
-MSA, PSP, Corticobasal degeneration
```
105
Aetiology of Idiopathic Parkinsons Disease
Neurodegenerative condition caused by disruption of dopaminergic neurotransmission in basal ganglia. Loss of production in substantia nigra and presents of cytoplasmic inclusions (lewy bodies) also seen in LBD and Alzheimers
106
Distinguishing between PD and Parkinsonism
PD - asymmetric at first, progressive, good response to levodopa
Parkinsonism - rapid progression, symmetrical and responds badly to levodopa
107
Parkinsons Plus: Multiple Systems Atrophy
Parkinsonism with autonomic failure, cerebellar dysfunction and pyramidal signs.
108
Parkinson's Plus: Progressive Supranuclear Palsy
```
Abnormal vertical then horizontal gaze
Prominent falls
Speech and swelling difficulties
Frontal symptoms - apathy, cognitive decline, depression
More axial than limb rigidity
```
109
Parkinsons Plus: Corticobasal degeneration
High cortical abnormalities - limb apraxia and sensory loss - (alien limb)
Myoclonus
Dementia
110
Differentiating tremor of parkinson vs BET
Essential tremor - symmetrical, worse with voluntary movements, accompanied by voice or head tremor, large writing
PD - asymmetrical, postural component but prominent tremor at rest, micrographia
111
Management of Parkinsons Disease
Watch and wait till symptoms need treating
1. Dopaminergics - levodopa, Dopamine agonist (Bromocriptine), MAO-B inhibitors (Selegilline)
Cause motor fluctuations, dyskinesias
2. Anticholinergics
3. COMT inhibitors (entacapone)
decrease GI breakdown of levodopa thus reducing off time and increasing on time
4. Subcut apomorphine
112
Postural hypotension in PD
Can be due to disease process and pharmacotherapy - can use fludrocortisone / midodrine
113
Surgical therapies available in PD
Thalmotomy
Pallidotomy
Subthalmotomy
Deep brain stimulation - will have something like a pacemaker
114
Clinical Features of Charcot-Marie-Tooth Disease (lower limb)
Lower limbs:
- distal wasting (especially anterolateral muscle compartment leading to inverted champagne bottle)
- check for scars to suggest traumatic cause of foot drop
- Palpable popliteal nerve
- bilateral pes Casus and clawing of foot (high arch doesn't flatten when weight bear)
- Symmetrical distal weakness and bilateral foot drop (check shoes for signs of orthotics/scuff marks)
- Absent deep tendon and planters
- Stocking sensory loss (later on)
- High stepping gait
- Rombergs positive as sensory ataxia
Motor+sensory neuropathy with pes cavus and or palpable nerves = CMT
115
Clinical Features of C-M-T Upper Limb
Distal wasting of upper limbs
Wasting of small muscle hands (guttering)
Hyperextension of MCPs and flexion at IPs resulting in claw hand
Absent deep tendon reflexes
Symmetrical glove distribution of sensory loss
May have postural tremor
116
Pathogenesis of Pes Cavus in CMT
Weakening of anterior tibialis and perineal muscles. Posterior muscles antagonised and pull harder. Imbalance results in deformity
117
Causes of Pes Cavus
Unilateral:
- malunion of previous fracture
- burns
- poliomyelitis
- spinal trauma/tumour
Bilateral:
- CMT
- Freidrichs ataxia
- Spinal muscular atrophy
- CP
- Spinal cord tumours
118
Different types of CMT (HSMN)
Hereditary sensory motor neuropathies. A heterogenous group with multiple underlying genetic mutations
1 - demyelination, autosomal dominant, palpable nerves
2 - axonal degeneration
3 - demyelinating, infantile onset and more severe
119
Why are pain and temperature usually unaffected in CMT
- carried by unmyelinated fibres and most CMT is process of demyelination
120
Histology of nerves in CMT
Onion bulb appearance
As a result of demyelination, schwann cells proliferate and form concentric arrays of remyelination. Get build up of thick layers of abnormal myelin
121
Management of CMT
```
Symptomatic
MDT
Patient education and advice
Exercise to maintain function - physic
Walking aids
Orthotics for foot drop
OT
Analgesia for neuropathic pain
Orthopaedic surgery
```
122
Clinical Features of MND
Dysarthria
Nasal quality speech
Wasted flaccid tongue with prominent fasciculations
Palatal paralysis
Normal eye movements (oculomotor nerves not involved)
Upper limbs: proximal and distal wasting with fasciculations, hypertonia and hyperreflexia, normal sensation
Lower limbs: as above + angle clonus and bilateral extensor plantars
To finish assess respiratory function
123
What is MND?
Progressive disorder characterised by neuronal loss at all levels of motor system
Physical signs therefore include upper and lower motor neurones
4 different types
- Amyotrophic lateral sclerosis - both upper and lower
- Progressive muscular atrophy - predominantly lower
- Primary lateral sclerosis - predominantly upper
- Progressive bulbar palsy - predominantly bulbar or pseudobulbar
124
Pathophysiology of MND
Unclear. Theories include
Excitotoxicity hypothesis: amino acid neuromodulators such as glutamate become toxic at supra physiological concentrations. These trigger calcium influx stimulating cascade resulting in free radical formation and neuronal death
Free radical hypothesis:
125
Diseases that mimic MND
UMN & LMN:
- syringomyelia (also may have horners)
- cervical myelopathy - no bulbar signs
UMN: spinal tumours, HIV myelopathy
LMN:
- chronic inflammatory demyelinating polyneuropathy
- Spinal muscular atrophy
- Old polio
126
Prognosis in MND
Worse in older age at onset, female, bulbar onset
Mean age onset 60
Mean survival 3-5years from onset
127
Management of MND
MDT - supportive
- Symptom control, QOL
- planning for palliative care early
- anticholinergics for drooling
- dietary advice/PEG feeding
- SALT for communication difficulties
- Quinine/diazepam for fasciculations and cramps
- Baclofen and physic for spasticity
- Consider NIV fro respiratory support
Riluzole - disease modifying effects, 3 month increased survival, doesn't help symptoms
128
Clinical Features of Cerebellar Syndrome
Ataxic on side of lesion
Ipsilateral dysdiadochokinesia, dysmetria and intention tremor which worsens closer to target
Rebound phenomenon - failure of displaced arm to find original position when eyes closed
Impaired heel shin test
Hypotonia
Nystagmus with fast component to side of lesion
Broad based gait with tendency to fall to side
Slurred speech with staccato character
All features ipsilateral
Features of underlying cause
MS - internuclear ophthalmoplegia
Alcohol - liver disease, peripheral neuropathy
Friedrichs ataxia - pes Casus
Paraneoplastic - bilateral signs, clubbing, horners, anti Hu (SLCL), anti Yo (gynae and breast)
Truncal features if vermis, limb ataxia if hemisphere
129
Causes of Cerebellar Syndrome
```
Demyelination (MS)
Alcohol cerebellar degeneration
Posterior fossa SOL
Brainstem lesion
Friedrichs ataxia
Ataxia telangiectasia
Hypothyroidism
VitE Deficiency
von-Hippel lindau
Arnold-chiari malformations
Drugs - phenytoin, carbamazepine, lithium, chemo
```
130
Drugs causing cerebellar disturbance
Phenytoin
Carbamazepine
Lithium
Chemo agents
131
Clinical Features of Myotonic Dystrophy
Myopathic facies - lifeless, lean and expressionless
Frontotemporal balding
Ptosis
Cataracts
Wasting of facial muscles, temporals, master, small muscles of hands
Difficulty opening eyes from firm closure
Distal wasting and weakness with impaired fine movement
Foot drop
Depressed deep tendon reflexes
High stepping gait
Other features you would like to look for:
- Diabetes: skin prick, urinalysis, fundoscopy
- Cardiomyopathy: signs of heart failure
- Valvular heart disease: MVP
- Arrythmias: AF
- Conduction defects: do they have pacemaker
- Hypogonadism
- Goitre: nodular thyroid enlargement
May have family hx of respiratory complications following anaesthetic
132
What is the genetic basis of myotonic dystrophy
Autosomal dominant
CTG repeat expansion in myosin protein kinase gene on chr 19
Expansion and anticipation
133
Cardiovascular Complications of Myotonic Dystrophy
```
ECG: prolonged PR, lon QTC, widened QRS
Arrhythmia: AF, SVTs, VT
Conduction defect: heart block
MVP
Cardiomyopathy
```
134
GI Complications of Myotonic Dystrophy
```
Diabetes
Dysphagia
Reflux
Hypo motility
Bacterial overgrowth
Malabsorption
Megacolon
```
135
Respiratory Complications of Myotonic Dystrophy
Hypoventilation
Respiratory failure following anaesthesia
Pneumonia
136
Drugs which can help in Myotonia
Phenytoin
Quinine
Procainamide (may worsen cardiac conduction)
137
Causes of distal wasting and weakness
Myotonic Dystrophy
HMSN
Inclusion body myositis
138
Common Causes of Sensori-motor neuropathy
```
Alcohol
Diabetes
Hypothyroidism
Uraemia
Sarcoidosis
Vasculitis
Paraneoplastic
GBS
Drugs: Cisplatin, amiodarone, allopurinol, vincristine
```
139
Causes of Sensory Neuropathy
```
Alcohol
Diabetes
Hypothyroidism
Uraemia
Sarcoidosis
Vasculitis
B12 deficiency
Drugs; isoniazid, metronidazole, flecainide
```
140
Small fibre neuropathy
Subtype of peripheral neuropathy where cold, pain and autonomic sensory nerves are involved buy large fibre vibration sense and proprioception are preserved
141
Main causes of mono neuritis multiplex
```
Diabetes
Polyarteritis nodosa
Churn-Strauss
Wegners
SLE
Sjogrens
Sarcoidosis
Lymphoma
Amyloidosis
```
142
Investigations for patient with peripheral neuropathy
FBC, ESR, U&E, LFT, Glucose, TFTs, B12, folate, serum protein electrophoresis, autoimmune profile
Urine: glucose and pence-jones
Other: nerve conduction studies, CSF may be indicated, nerve biopsy, genetic testing
143
Clinical Features of Common Peroneal Nerve Palsy
High stepping gait
Foot drop
Wasting of anterolateral compartment of calf
Weakness of ankle dorsiflexion and eversion
Weakness of dorsiflexion of first toe
Preserved ankle jerk
Loss of sensation over lateral calf and dorsum of foot
Look for scars to suggest traumatic cause
144
Clinical Features of L5 root lesion
High stepping gait with foot drop
Weakness of hip abduction, dorsiflexion of first toe
Ankle inversion also involved (spared in peroneal nerve)
Loss of sensation to lateral calf, dorsum of foot and lateral thigh
Don't forget to examine back for scars
145
What is the course of the common peroneal nerve
Arises as terminal branch of sciatic on posterior aspect of distal thigh
Winds around neck of fibula then splits into superficial and deep
-Superficial supplies peroneus longus and braves and sensation to anterior lower leg and dorsum of foot
-Deep supplies tibialis anterior, extensor hallacus longus and sensation to 1st web of foot
146
Causes of flaccid foot drop
```
Muscle - myotonic dystrophy, trauma
NMJ - myasthenia gravis
LMN - MND, causes of motor neuropathy
Cauda equina tumour
L5 disc prolaps, neurofibroma
Common peroneal nerve palsy - often external compression/trauma
Sciatic nerve palsy
```
147
Management of Foot Drop
Treat any underlying cause
Physio
Orthotics
148
Clinical Features of Friedrichs Ataxia
Pes cavus
Kyphoscoliosis
High arched palate
Gait ataxia is common Pc
Cerebellar features: DANISH bilateral
Distal wasting of limbs in pyramidal distribution
loss of proprioception and JPS (degeneration of posterior columns)
Hearing aids/foot orthoses
```
Other features:
Diabetes in 10%
Optic atrophy - look at disc
Hypertrophic cardiomyopathy in 50% - double jerky apex, systolic murmur
Sensorineural deafness
```
149
Genetics of Friedrichs Ataxia
Autosomal recessive, chr 9
| Trinucleotide GAA expansion of protein frataxin involved in mitochondrial iron regulation. Shows anticipation
150
Cardiac abnormalities in Friedrichs Ataxia
Arrhythmia
Conduction defects
Hypertrophic cardiomyopathy
Myocardial fibrosis
151
Multiple Sclerosis Sites
Optic nerve - atrophy and RAPD
Brainstem - internuclear ophthalmoplegia, facial sensor loss and weakness, tremor
Cerebellum - ataxia, dysarthria
Spinal cord (corticospinal) - pyramidal weakness
Spinal cord (dorsal columns) - loss of vibrations and proprioception
152
Examining the Eyes of Patient with MS
Internuclear ophthalmoplegia - impaired ipsilately adduction, flicking of other eye in abduction
Pale optic disc - atrophy
RAPD - loss of direct pupil response but consensual is intact
Central scotoma on red hat pin field testing
Reduced acuity
May also have cerebellar signs, pseudoathetosis etc
153
Examining Upper/Lower Limbs in MS
Pyramidal weakness
Hypertonia, hyperreflexia
Dysdiadochokinesia, impaired finger nose testing and intention tremor
Pseudoathetosis when eyes closed
Rebound phenomenon
Impaired vibration and proprioception
Slurred speech, nystagmus etc if you have time
154
Pathophysiology of MS
Idiopathic Demyelinating Disorder of CNS characterised by demyelinating plaques separated in both space and time. Inflammatory and autoimmune
155
Different Categories of MS
Relapsing remitting
Secondary progressive
Primary progressive
Progressive relapsing
156
What is Lhermittes sign?
Neck flexion results in rapid tingling and electric shows down spine into arms and legs. Indicates dorsal column involvement of c-spine.
Seen in - MS, cervical myelopathy, SACDC
157
What is Internuclear Ophthalmoplegia?
Lesion in medial longitudinal fasciculus
Connects ipsilateral CNIII (medial rectus, adductor) to contralateral CNIV (lateral rectus, abductor)
Results in impaired ipsilateral adduction and nystagmus on abduction of contralateral eye
Causes: MS, brainstem lesions, Wernickes, SLE, miller fisher
158
Investigations useful in confirming MS
T2 weighted MRI - periventricular white matter lesions
CSF if diagnosis is uncertain - oligoclonal bands - raised intrathecal IgG, lymphocytosis
Oligoclonal bands also seen in neurosarcoidosis, CNS lymphoma, SLE, GBS
159
Management of MS
General: education, PT/OT, walking aids, visual aids
Acute attacks: IV methyl pred, plasma exchange
Prevention of relapses:
- Beta interferon: reduced relapse in 33% but can cause flu like symptoms/myalgia
- --licences for relapse remitting form after 2 relapses in 2 years
- Glatirimer - competes with autoantibody
- Mitoxantrone
- Natalizumab
Symptomatic treatment
- Spasticity - baclofen
- Depression
- Fatigue - amantadine, SSRIs
- Pain - neuropathic
- Bladder dysfunction - intermittent self cath, anticholinergics
160
Clinical Features of Cervical Myelopathy
Upper Limbs: fasciculations, absent biceps, brisk triceps jerk, sensory loss in C5-C7 dermatome, wasting in C5-C7 dermatome,
Difficulty opening and closing grip in succession
Pseudoathetosis of fingers
Lower Limbs: spastic paraparesis with dorsal column signs
Ddx of friedrichs, MS, SACDC
161
Pathophysiology of Cervical Myelopathy
Canal stenosis encroaches on cord leading to lower signs in arms proceeding to upper signs below level
Spondylosis most common but can occur in tumours or following trauma
162
Cerebellar nystagmus
Horizontal nystagmus
| Fast component towards side of lesion
163
Vestibular nystagmus
Horizontal nystagmus
Fast component away from side of lesion.
Nystagmus suppressed by visual fixation and adapts to continued gaze
164
CN III Palsy (oculomotor)
Ptosis
Divergent strabismus at rest
Eye fixed in down and out position
Fixed dilated pupil - means parasymp involved usually from extrinsic compression (surgical rather than medical)
165
CN IV Palsy (trochlear)
Adducted eye can't look down
| Diplopia worse on looking down and away on affected side
166
CN VI Palsy (
Convergent strabismus and impaired abduction
| Diplopia maximal on gaze to that side
167
Differential for opthalmoplegis which doesn't fit into specifically III, IV or VI
```
Myopathy
Muscular dystrophy
Myasthenia
Myositis
Trauma
```
168
Main structures in cavernous sinus
CNs III, IV, VI, Va and Vb
Sympathetic carotid plexus
Carotid artery
169
Causes of cavernous sinus syndrome
```
Thrombosis
Aneurysm
Fistula
Tumour - primary or secondary
Trauma
Inflammation - Herpes zoster, TB, sarcoid, wegners
```
170
Clinical features of cavernous sinus lesion
```
Unilateral III, IV and VI nerve palsies
Sensory loss in Va and Vb
Loss of corneal reflex
Painful ophthalmoplegia
Proptosis
Visual loss
Papilloedema
```
171
Clinical Features of Myasthenia Gravis
Variable ptosis accentuated by sustained upward gaze, improves following eye closure
Furrowing of forehead
Variable strabismus and diplopia
Bilateral facial muscle weakness, jaw weakness
Weak voice with nasal twang
Proximal weakness with fatiguability
Look for evidence of steroid use, thymectomy scar and other autoimmune conditions e.g. DM, RA, SLE, pernicious anaemia
172
Pathophysiology of myasthenia gravis
Autoimmune disorder characterised by antibodies against nicotinic acetyl choline receptors on post-synaptic membrane of NMJ
Can be generalised or ocular
173
Drugs which exacerbate myasthenia gravis
```
Amino glycosides
Macrolides
Beta blockers
Phenytoin
Lignocaine
```
174
Difference between MG and lambert-eaton
Lambert-Eaton assoc with SCLC, antibodies against pre-synaptic Ca channels, more limb than eye involvement
175
Causes of False positive nAChR antibodies
```
First degree relative with myasthenia
MND
Lambert-eaton
Autoimmune hepatitis
PBC
SLE
Penicillamine therapy
```
176
Treatment options for Myasthenia Gravis
Acetyl cholinesterase inhibitors - symptomatic benefit not DMARD, can cause N&V, cramps, salivation
Immunosuppressive - steroids then azathioprine, cyclosporin. NB steroids may make weakness worse in first 10 days
Plasma exchange/IVIG
Thymectomy - MG occurs as a result of loss of tolerance to auto antigens. Thymic abnormalities common in these patients and can lead to remission. Always consider if have thymoma or young with thymic hyperplasia
177
Clinical Features of Pancoast Syndrome
Horners
C8-T1 weakness (hand)
C8-T1 dermatomal weakness
Clubbing and tar staining (lung Ca)
178
In a patient with wasting of hand(s) always look for
```
Horners
Neurofibromas
Tongue fasciculations
Clubbing (Horners syndrome)
Pes Cavus (HSMN, Friedrichs, old polio)
inverted champagne legs
Spastic paraparesis (syringomyelia, cervical myelopathy, MND
```
179
Innervation and action of hand muscles
All C8-T1 nerve root
Thenar muscles median
Interossei and digiti minimi ulnar
180
Clinical Features of Syringomyelia
Kyphoscoliosis - median motor nuclei involvement which supplies paraspinal muscles
Surgical scars
LMN signs in upper limbs
UMN signs in lower limbs
Dissociated sensory loss - loss of pain and temp with preservation of JPS and proprioception
181
What is syringomyelia?
development of fluid filled tubular cavity in central area of spinal cord. Most often develops in cervical or thoracic spinal cord
Causes include:
- CSF blockage from Arnold-Chiari malformation
- Spinal cord injury
- Intramedullary spinal tumour
- Idiopathic
182
How to explain the clinical signs seen in syringomyelia?
At level of syrinx
- anterior horn cell affected thus damaging LMN
-decussating spinothalamic tracts affected through length of syrinx
Below level - corticospinal tracts affected at level thus UMN signs below level
183
Causes of Dissociated Sensory Loss
Syringomyelia
Anterior spinal artery occlusion
Small fibre neuropathies
184
Clinical Features of Ulnar Nerve Palsy
Wasting and weakness of small muscles of hand with sparing of thenar eminence
Extension deformity at MCP
Clawing of ulnar aspect of hand
Weakness of finger ab and adduction, weakness of thumb adduction only
reduced sensation on 5th finger and ulnar aspect of 4th
Most commonly caused by lesion at the elbow
185
Causes of Ulnar Nerve Palsy
At elbow - fracture, arthritis, fibrous arch compression
At wrist - ganglion, tumour, fracture, aberrant artery, entrapment in guyons canal
186
Clinical Features of Radial Nerve Palsy
Motor: wrist drop, weak extensors of wrist and MCPs, weak elbow flexion
Triceps intact
Sensory loss over first dorsal interosseous space
If lesion higher at humerus then also forearm sensory loss
If no sensory loss then likely pure motor branch
187
Course of Radial Nerve
Posterior branch of brachial plexus
| Wraps around humerus in spiral grove then divides into sensory and motor
188
Causes of Radial Nerve Palsy
Axilla - trauma, compression
Spiral groove - trauma, saturday night palsy
Proximal forearm - trauma, sublux of radius, elbow synovitis, ganglion, tumour
wrist - trauma, compression
189
Clinical Features of Median Nerve Palsy
Wasting of thenar eminence
Weakness of thumb
Sensory loss over palmar aspect of first 3 fingers
If spared over thenar eminence likely carpel tunnel (if not spared then lesion higher as palmar cutaneous branch involved)
Tinels
Phalens
Consider underlying cause: acromegaly, RA, trauma, hypothyroidism, amyloidosis, gout, DM, pagets
190
Relative Afferent Pupillary Defect - Optic Atropy
Can have normal direct and consensual reflexes when tested separately
During swinging torch test, one appears dilated and the other constricted because the direct reflex of affected eye is weaker
Causes: optic neuritis/optic atrophy causes, retinal causes including central retinal artery/vein occlusion, severe ischaemic diabetic retinopathy
191
Argyll Robertson pupils
Both pupils small and irregular accommodate but don't react to light
Causes: neurosyphilis, diabetes, midbrain tumour/lesion, wernickes
192
Myotonic Pupil
Dilated pupil which reacts slowly
Benign condition seen in middle aged females
when accompanied by arreflexia known as Holmes-Adie syndrome
193
Causes of Miosis
```
Argyll Robertson pupil
Horners
Opiates
Organophosphate poisoning
infancy/advanced age
```
194
Clinical features of Horners Syndrome
Miosis
Partial ptosis (some innervation from III) which can be overcome with voluntary gaze
Normal eye movements
Ipsilateral anhidrosis if lesion central or pre-ganglionic
Look at neck: scars, masses, aneurysms
Hands: wasting, clubbing, nicotine staining, sensory loss etc to show pancoast tumour
Chest: apical lung signs, sternotomy scars, cervical rib
195
Causes of unilateral ptosis
III nerve palsy
Horners
MG
Congenital
196
Causes of bilateral ptosis
```
MG
Myotonic dystrophy
Bilateral horners - syringomyelia
Nuclear III nerve palsy
Congenital
```
197
Causes of Horners
Central (1st order neurone) - demyelination, brainstem tumour, basal meningitis
Peripheral (2nd order) - pan coast tumour, cervical rib, neurofibromatosis, LN, cardiothoracic surgery
Peripheral (3rd order) - internal carotid dissection, herpes zoster
198
Clinical Features of Cerebellopontine angle lesion
```
VI nerve palsy - unable to abduct eye
LMN weakness of face
Sensory loss in CN V
Wasting of jaw muscles (V)
Sensorineural hearing loss (unilateral)
May be involvement of IX-XII: hypophonia, neck muscle weakness, tongue deviation
```
199
Causes of Cerebellopontine angle lesion
```
Acoustic neuroma
Meningioma
Cholesteatoma
Haemangioblastoma
Granuloma
Metastases
```
200
Clinical Features of Hemiparesis
```
Flexural posturing
Dystonic
Pronator drift
Hypertonia
Hypereflexia
Reduced sensation
Circumducting gait
May be expressive dysphasia
Extensor plantars
```
Would also like to assess:
complete neuro exam, speech, visual fields, measure BP, assess pulse (AF)
201
Common causes of hemiplegia
```
CVA - ischaemic or haemorrhage
Tumour
Demyelination
Abscess
Post-Octal (Todds)
Functional
```
202
Why should pain/temp and JPS/vibration be tested?
If there is dissociation between two it suggests c-spine lesion not cerebral (Brown-Sequard)
203
Clinical Features of Chorea
Brief, irregular, non repetitive movements with flow and dancing quality
Accentuated by activity
Motor impersistence - unable to sustain posture, tongue protrusion or eyelid closure
Milkmaids grip
Look for features of underlying cause:
- Huntingtons: mental status/dementia
- Wilsons: K-F rings, stigmata of CLD
- SLE: rash, purpura
- Drugs
204
Causes of Chorea
Inherited
- Huntingtons
- Wilsons
- Benign familial chorea
- Spinocerebellar ataxia
Acquired
- Sydenhams chorea
- SLE
- Thyrotoxicosis
- Pregnancy
- OCP
- CVA
205
Tardive dyskinesia
Long term dopamine antagonists leads to up regulation and supersensitive to dopamine leading to exaggerated response
Drugs implicated include phenothiazines, metoclopramide
206
Features of Bulbar Palsy (flaccid dysarthria)
Speech nasal quality and lacks modulation
Weak voice, low volume
Slurring of consonants
Bilateral palatal weakness
Flaccid paralysis of tongue with fasciculations
Absent jaw jerk
207
Features of pseuobulbar palsy (spastic dysarthria)
```
High pitch slow speech which lacks modulation
Harsh vocal quality
Strained voice
Bilateral palatal weakness
Small, immobile, spastic tongue
Brisk jaw jerk
Emotional lability
```
208
What are causes of bulbar palsy
Higher Cranial nerve lesion
```
True bulbar palsy is LMN
-MND
-Brainstem ischaemia
Brainstem tumour
-Poliomyelitis
-GBS
```
Pseudo bulbar is UMN
- MND
- MS
- Brainstem tumour
209
Clinical Features of Old Poliomyelitis
```
Hypoplastic limb with distal wasting, weakness and hypotonia
Hyporeflexia
Absent plantar responses
Normal sensation
Pes Casus
```
LMN but may have contractures from immobility
look for walking aids, orthoses
No sensory involvement, otherwise consider HSMN
210
Poliovirus
Enterovirus
Single strand RNA virus surrounded by protein capsid
3 different strains
211
Pathophysiology of poliomyelitis
Faecal oral transmission or ingestion of contaminated water
1-3 week incubation period
only 5% get nervous system involvement
5 clinical syndromes:
1. Abortive poliomyeletis - mild pharyngitis
2. Non-paralytic poliomyelitis - features of meningism
3. Paralytic - flaccid limb paresis followed by atrophy, may be partial/complete recovery
4. Paralytic with bulbar involvement
5. Encephalitis - very rare, very high mortality
212
Differential for Acute Limb Paralysis
Virus: polio, enterovirus 71, tick paralysis
Bacteria: botulism, diphtheria, lyme
Neuropathy: GBS, acute intermittent porphyria
Spinal disease: acute transverse myelitis, spinal cord compression
213
Clinical Features of Facial Nerve Palsy
```
Facial weakness with no forehead sparing
No ophthalmoplegia
Normal sensation
Check no parotid enlargement (cause of peripheral palsy
Check for hearing defect
```
214
Causes of Bells LMN palsy
```
Idiopathic
Structural:
-brainstem demyelination/tumour
-cerebellopontine angle acoustic neuroma
-middle ear infection
-parotid infection/tumour
Mononeuritis multiplex
```
215
Investigations for Bells Palsy
Bloods: glucose, CRP, ESR, ACE, lyme serology etc if atypical presentation
Nerve conduction studies if concerned about GBS
LP if multiple cranial nerves etc
216
Treatment of Bells Palsy
High dose oral red for 5 days
Oral acyclovir sometimes given in first week if suggestion of ramsay-hunt
Corneal protection - check eye before steroids then lubrication/tape eye shut at night
217
Complications of Bells Palsy
Persistence
Corneal abrasion
Pain in distribution of nerve
Hemifacial spasm
218
Clinical Features of DMD
```
Young patient
Wheelchair bound
Generalised wasting and weakness predominantly affecting neck and proximal muscles
Contractures on lower limbs
Reduces reflexes
Pseudo hypertrophy of calves
Kyphoscoliosis
Inability to generate forceful cough
```
219
Genetics of DMD and Beckers Muscular Dystrophy
Both X linked recessive mutation in dystrophin gene
220
Riluzole
used to treat ALS
| Delays ventilator dependence & increase survival by 2-3 months
