PACES 2 Flashcards
Purpose of NIHSS and MRS score
Both are disability scores to see whether pt have symptoms severe enough to benefit from intervention
DD of cerebellar syndrome
Ipsilateral
- stroke
- SOL
- demyelination (MS)
Bilateral
- alcohol cerebellar syndrome
- drug (antiepileptics, phenytoin)
- genetic (spinocerebellar ataxia, friedrech ataxia)
- MSA
- paraneoplastic
- infectious (VZV Cerebellitis)
DD Parkinsonism
Normal dopamine
- drug induced
- vascular Parkinsonism
- metabolic like alcohol
- Wilson
- NPH
Dopamine reduced
- idiopathic PD
- lewy body dementia
- MSA
- PSP
Name 5 MS mimics
Vasculitis - SLE, sjogren, behcet, sarcoidosis
Vascular - antiphospholipid, stroke, Fabry
Mitochondrial - MELAS
Infection - Lyme, HIV encephalitis, syphillis, PML
Metabolic - b12 deficiency
MRI finding in MS
Periventricular white matter lesions
Investigation for MS
Visual evoked potential
CSF unmatched oligoclonal band in serum VS CSF
MRI spine and brain showing new T2 lesion 30 days after clinical onset or a second clinical episode after a CIS (clinical isolated syndrome)
Bloods to exclude MS mimic - ESR, ANA, ANCA, dsDNA, ENA, antiphospholipid for AI cause + B12 level, treponemal serology etc
MS vs Neuromyelitis optica 3 differences
NMO is more severe
NMO has more extensive spinal cord lesion normally extending over 3 vertebral segments
NMO has anti aquaporin 4 antibodies
Rating scale for disability in MS and significance
Expanded disability status scale (EDSS)
Help guide disease modifying therapy use e.g. max EDSS 6.5 for initiating beta interferon or glatiramer acetate
Criteria for starting DMT in MS
Relapsing remitting MS with active disease defined by 2 significant relapses in last 2 years, 1 disabling relapse in last year or active MRI scan with new lesion that has developed over the last year
Common reasons for stopping Beta interferon in MS?
Flu like sx, autoimmune hepatitis, depression, treatment failure due to development of neutralising antibodies
Miller Fischer syndrome cause, 3 features and diagnosis
Autoimmune, post infectious
Ophthalmoplegia, ataxia, loss of lower limb reflexes
Anti GQ1b ganglioside antibody
Inflammatory myelitis
Predominantly sensory or motor symptoms?
Sensory
Cord compression
Predominantly sensory or motor symptoms?
Motor
Loss of pain and temp sensation spinal cord causes?
Syringomyelia
Anterior cord infarction
Loss of fine touch and proprioception sensation spinal cord causes?
B12 deficiency
HIV associated vacuolar myelopathy
Neurosyphillis
Friedreich ataxia
Marked spasticity out of keeping with degree of weakness causes
MND
Hereditary spastic paraparesis
Tropical spastic paraparesis
Early bladder involvement in predominantly motor syndrome
Compressive myelopathy
Dysarthria DD
Bulbar (LMN) - MG, MND, GBS, myopathy
Pseudobulbar (UMN) - brainstem stroke, MND, MS
Mixed UMN and LMN DD
Dual pathology - cervical spondylopathy + peripheral neuropathy - check glove and stocking neuropathy and finger prick signs
Cervical spondylomyelopathy from cervical spine degeneration - has sensory level, UMN below lesion and LMN at level of lesion
MND
Syringomyelia - cape like distribution pain and temp loss, normal sensation in lower limb. Wasting and weakness hand muscle. Brisk lower limb reflexes and extensor plantar.
Cauda equina
Subacute combined degeneration of cord - loss of proprioception (sensory ataxia) hence Romberg test positive, brisk knee jerk, absent ankle jerk. Extensor plantar. Mainly painful sensory neuropathy and lower limb weakness. Check for signs of pernicious anaemia
DD for absent ankle jerk and extensor plantar
MND
Fredreich ataxia
Subacute combined degeneration of cord
Syringomyelia
Tumour involving conus and cauda
Neurosyphillis
Name 10 causes of splenomegaly
Infective - malaria, EBV, CMV, leishmaniasis, salmonella
Infiltrative - SLE, amyloidosis, sarcoidosis, hereditary spherocytosis
Inflammation
Haematological - CLL, CML, myelofibrosis, lymphoma
Rheumatological - adult onset stills disease, felty syndrome
Liver disease
Causes of ascites
Cirrhosis
Cancer
CCF
CKD
Pancreatitis
Chylous ascites
Hypoalbuminaemia
Peritoneal dialysis associated ascites
IVC obstruction
Pericarditis
Budd chiari
Portal vein thrombus
Advanced hypothyroidism
Liver transplant criteria
UKMELD SCORE
KINGS COLLEGE CRITERIA - paracetamol overdose
Immunosuppressant clinical signs
Tacrolimus - fine tremor, hair loss
Ciclosporin - gum hypertrophy, renal toxicity, hypertension, hirsutism
Steroid - thin skin, Cushingnoid, bruises, cataract, infection, diabetes, hypertension
Skin cancer
Liver transplant complications (name 5)
- Infection
- Graft failure
- Graft rejection
- Graft leak
- Haemorrhage
- Disease relapse
Indication for liver transplant (name 5)
Refractory ascites despite TIPSS
HCC
Acute liver failure
HRS
intractable pruritus
Recurrent cholangitis
Polycystic liver disease
Contraindication for liver transplant
- Ongoing alcohol use
- Metastatic liver cancer
- Age >65
- IVDU
- Significant psychiatric problem
Name 5 most common causes of ESRD
Diabetes
HTN
Chronic glomerulonephritis
ADPKD
Obstructive uropathy
Unexplained CKD
Chronic pyelonephritis
Immunosuppressant for kidney transplant name 4 classes
Calcineurin inhibitor - tacrolimus, ciclosporin
Antiproliferative agent - mycophenolate mofetil
mTOR inhibitor - sirolimus
Steroids
Complications from immunosuppressant name 6
- Cancer (SCC, kaposi sarcoma, lymphoma, cervical)
- Diabetes
- Hypertension
- Hyperlipidaemia,
- CMV infection
- BK virus infection
Causes of bronchiectasis (name 4 categories)
Congenital
- CF
- Kartageners
Infective
- TB, measles, diphtheria
Immune overactivity
- RA, ABPA, IBD
Immune underactivity
- Hypogammaglobinaemia
- CVID
Hereditary sphereocytosis inheritance and chromosome affected
Autosomal dominant
Chromosome 8
Investigation for hereditary spherocytosis
- FBC, blood smear, blood film
- EMA binding test or osmotic fragility test
- Haptoglobin, reticulocyte, coombs test
- Split bilirubin
- USS abdomen for spleen and gallstones
Hereditary spherocytosis complication
- Aplastic crisis
- Anaemia
- Gallstones
Hereditary spherocytosis management
Vaccination for encapsulated organism - meningococcal, pneumococcal
Folic acid
Splenectomy
Blood transfusion
Criteria for simultaneous kidney and liver transplant
ESRD + poorly controlled diabetes (usually type 1)
Reason for simultaneous kidney and pancreas transplant
- Prevent diabetic nephropathy
- Better QOL with reduced dialysis
- Better long term outcome than isolated kidney transplant
Name 5 causes of chronic pancreatitis
Genetic - PRSS1, SPINK1, CFTR
Autoimmune - IgG4
Alcohol
Gallstones
Trauma
Indications for aortic valve replacement in aortic stenosis
- Symptomatic severe AS (angina, syncope, dyspnoea)
Signs of severe AS clinically
Slow rising pulse
Symptomatic
Quiet S2
LVH
Decompensation
Signs of severe AS on echo
Valve area <1cm2
Peak gradient >60mmhg or mean gradient >40mmhg
DD for aortic stenosis (name 4)
HOCM
Aortic sclerosis
Mitral regurgitation
Post infarction VSD
Criteria for surgery for aortic stenosis
All symptomatic
Asymptomatic + LVEF <45%, LVH, VT, or sx on exertion
Criteria for TAVI
Age >75
Frail
Not fit for surgery
Contraindications to TAVI
Bad CAD
Complications of TAVI (name 4)
Pacemaker
Vascular access complication
Annular rupture
CAD if block coronary artery
Aortic regurgitation criteria for surgery
All moderate to severe symptomatic
Asymptomatic + LV dilatation, aortic root dilatation or RF >50%
Mitral regurgitation causes (name 5)
Age related degeneration
Mitral valve prolapse
Infection (IE, Rheumatic)
Papillary muscle rupture
Connective tissue disorder
Criteria for MV repair in mitral regurg
Asymptomatic + EF 30-50% + ESLV dimension >40mm
Chronic severe MR + new AF or PH
Mitral stenosis causes name 3
Acquired
- rheumatic
- degenerative calcification
- infective endocarditis
Signs of severe mitral stenosis
Malar flush
AF
HF
pHTN
Loud S1
Chronic severe MR + new AF or PH
True
Mitral stenosis causes (name 3)
Acquired:
- rheumatic
- degenerative calcification
- infective endocarditis
Signs of severe mitral stenosis on echo
Valve area <1cm2, gradient >10mmhg
Pulmonary stenosis causes (name 5)
Congenital:
- Noonan’s
- Turner’s
- William
- Tetralogy of fallot (PS, VSD, overriding aorta, RVH)
Acquired:
- Carcinoid
Causes of peripheral neuropathy
Metabolic:
- Diabetes
- Hypothyroidism
- Uraemia
- Vit B1, B6, B12 deficiency
Toxic:
- Chemotherapy
- Antibiotics
- Alcohol - sensory predominant
Inflammatory:
- CIDP
- Sarcoidosis
- RA
- HIV
- Lyme
Neoplastic:
- lung cancer
- multiple myeloma or MGUS
Investigation for peripheral neuropathy
FBC - macrocytic anaemia
u&E - for urea
LFT
Vit B12
ESR
Serum electrophoresis
Hba1c
NCS - find out if demyelinating or axonal, also find out if length dependent in nature which is more likely peripheral neuropathy
EMG
Charcot Marie tooth is demyelinating or axonal?
Type 1 = demyelinating
Type 2 = axonal
Management of Charcot Marie tooth
- No disease modifying therapy available
- Multidisciplinary team
- Orthotics
- OT to help home set up
Charcot Marie tooth features
Wasting of thenar/hypothenar eminences
Distal lower limb wasting
Peripheral sensorimotor neuropathy
Cerebellar ataxia management
MDT
- physio exercise
- OT home adaptions and mobility aids
Lifestyle advice
- look at occupational risk factors
- look at meds to see if affect cerebellum or dizziness
- advice reduce alcohol
Differential for pure motor weakness
MND
ALS
KENNEDY DISEASE (LMN)
Multifocal neuropathy with conduction block (LMN)
Spinal muscular atrophy (LMN)
Investigation for MND
EMG - check for fasiculation, fibrillation in absence of sensory signs
If multifocal neuropathy with conduction block then will find demyelinating disease with conduction block
MRI spine
MND features
Asymmetrical
Rapid
Fasiculation
Muscle wasting
Behavioural disturbance like frontal temporal dementia
Management of MND
Riluzole small increase in survival
MDT
- Physio, OT
- SALT, to consider alternative feeding, assess swallowing, communication aid
Neuro
Screen weight appetite and swallowing
Screen respiratory function (early morning blood gas, FVC) as some may get resp weakness. Cough assist device
Screen cognitive function for FTD
Name the types of myelopathy
Compressive:
- tumour
- disc herniation
Autoimmune or inflammatory:
- MS, NMO
- SLE
- Sarcoidosis
Infectious:
- HIV
- Varicella
- Lymes disease
- Syphillis
Nutritional:
- B12
Genetic:
- hereditary spastic paraparesis
Dorsal column sensation?
Fine touch
Vibration
Proprioception
Anterior column sensation?
Pain (pin prick)
Temperature
MND subtypes
UMN features:
- Primary lateral sclerosis
Mixed
- ALS
- PRogressive bulbar palsy
LMN
- Progressive muscular Atrophy
Kennedy disease (x-linked spinal bulbar muscular atrophy) features
Perioral fasiculation
LMN predominant
X linked, associated with androgen insensitivity
Slow progression
Features of Cerebellar syndrome
- head tremor
- truncal ataxia
- jumpy vision / oscillopsia
- scanning dysarthria
- nystagmus in all directions
How to differentiate spinocerebellar syndrome from cerebellar syndrome?
Spinocerebellar syndrome has UMN signs like upgoing plantar and increased tone or reflexes
How to differentiate pure cerebellar syndrome with alcohol associated cerebellar syndrome?
Alcohol related would present with some degree of peripheral neuropathy
Name 6 types of muscular dystrophy
Duchenne (childhood) MD
Beckers (childhood) MD
Ocular pharyngeal MD
Myotonic MD
Facioscapulohumeral muscular dystrophy
Limb girdle muscular dystrophy (inherited) – shoulder and pelvic girdle – raised CK and EMG myopathic changes + positive genetic test
Spinal muscular atrophy – mainly LMN
Spinal bulbar muscular atrophy (Kennedy’s disease) – perioral fasiculation + LMN disease only unlike MND
Neurological findings for friedreich’s ataxia
motor, sensory, UMN, LMN and cerebellar signs
Features of spastic paraparesis
increased tone, brisk reflexes, upgoing plantars, weakness and reduced sensation
DD for spastic paraparesis/myelopathy
Hyperacute + backpain = compressive pathology, such as an intravertebral disc herniation
Hyperacute without back pain = vascular cause, such as a spinal stroke, could also be the cause; however, I would not expect the patient to present with such spasticity in the acute phase
Days = inflammatory (multiple sclerosis) or infective pathology (VZV)
Months = metabolic causes such as Vitamin B12 deficiency, copper deficiency other infective causes e.g. HIV. If the patient has travel history, things such as HTLV1 infection could also be considered
Years = slowly growing tumours, neurodegenerative conditions such as primary lateral sclerosis
Positive family history = hereditary spastic paraparesis
Post polio syndrome neurological findings
isolated LMN signs + cramp, weakness
Pes cavus differential
Long standing neuropathy e.g. charcot marie tooth, polio neuropathy, Friederich ataxia , muscular dystrophy
Absent reflex neurological syndromes
LMN disease
Sensorineuropathy
DD for LMN findings only
Hereditary
- Spinal muscular atrophy
- Hereditary motor neuropathies
Sporadic
- MND
Immune mediated
- CIDP
- MND
Infectious
- Post polio syndrome
Name the causes of interstitial lung disease
Idiopathic
Idiopathic fibrosis (most common)
Sarcoidosis (2nd most common)
Cryptogenic organising pneumonia
Connective tissue disorder
SLE
AS (apical)
RA (upper zone)
Dermatomyositis
Systemic sclerosis
Mixed CTD
Occupational
Asbestosis
Silicosis
Organic exposure
Bird (most common EEA)
Hay
Mold
Medication
Amiodarone
Nitrofurantoin
Methotrexate
Name the signs pointing towards idiopathic pulmonary fibrosis instead of other types of pulmonary fibrosis
clubbing, age <45
Name 5 investigation for suspected interstitial lung disease
Spirometry + transfer factor – if FVC <80% then need referral to tertiary care centre to consider antifibrotic therapy
Blood test for CTD (ANA, dsDNA, ANCA, rheumatoid factor)
ACE level if suspect sarcoidosis
Allergy testing may be performed for example avian precipitins if bird fancier’s lung suspected.
CXR, HRCT
Echo for pul HTN
Blood gas
bronchoscopy with BAL, transbronchial lung biopsy or surgical lujng biopsy if indicated
How is diagnosis of ILD made?
History + spirometry + radiology
Biopsy not needed
Need ILD MDT for a decision about diagnosis and to understand the underlying aetiology
Management of IPF?
Refer for consideration for lung transplant
Pulmonary rehab, supportive treatments where necessary such as oxygen.
Nintedanib or pirfenidone can be considered in patients with a forced vital capacity between 50-80% predicted to slow disease progression.
Management of NSIP
Immunosuppressive treatment may be started for patients with moderate to severe disease e.g. oral or IV glucocorticoids and steroid sparing immunosuppressive agents such as azathioprine or mycophenolate mofetil although other agents may be trialled for refractory disease.
When to use steroids for ILD?
If CT shows groundglass changes suggesting inflammatory component
Management of bronchiectasis
Physiotherapy chest
Prophylactic abx – low dose azithromycin 2 times a week
Salbutamol inhalers if significant wheeze or breathlessness
DD for double thoracotomy scar with normal breath sounds
o Bilateral apical pleurectomy
o Bullectomy
o Pulmonary TB surgical management (in older patient)
Pulmonary DD for clubbing
o Interstitial lung disease
o Chronic suppurative lung disease, bronchiectasis CF, lung abscess
DD for fine bibasal crackles
o CCF
o Interstitial fibrosis
o Pneumonia (but would clear with cough)
DD for double lung transplant
CF (most common)
Bronchiectasis
Pulmonary hypertension
Idiopathic pulmonary fibrosis
DD for single lung transplant
“Dry” lung conditions
o Pulmonary fibrosis (single lung transplant)
o COPD (single lung transplant)
When to refer for lung transplant in ILD?
Usual interstitial pneumonia or fibrotic NSIP (non-specific usual interstitial pneumonia) who have no contraindications should be considered for referral regardless of their lung function.
Non-fibrotic/cellular NSIP should be referred if their forced vital capacity < 80% or their transfer factor is < 40%, or any oxygen requirement or symptomatic dyspnoea. This does not mean that these patients will be immediately listed, however they can complete a full and timely transplant assessment and be listed if shown to be rapidly deteriorating for example > 10% drop in FVC within six months
Eligibility for lung transplant?
Meet 3 criteria
- They should have a > 50% risk death from lung disease within two years if transplant is not performed.
- They should have a > 80% likelihood of surviving at least 90 days post-transplant.
- They should have > 80% likelihood of a 5-year post-transplant
Contraindication to lung transplant
o Malignancy within last 5 years
o High or low BMI
o Smoker
o Illicit drug
o Psychiatric condition that stop them from taking meds or coming to regular appointments
o Smoking or dependence
Complications of lung transplant
o Hyperacute rejection (common)
o Opportunistic infection
o Bronchiolitis obliterans (chronic rejection and terminal event)
o Malignancy (post transplant lymphoproliferative disorder)
Indications for video assisted thoracoscopy
o Wedge resection or segmentectomy of solidary pulmonary nodule
o Lung biopsy
o Lobectomy
o Decortication
o Bullectomy
o Tx for recurrent pneumothoraces
Benefit of VATS
Small incision = less pain, less healing time, less hospital stay, less infection
Indications for lobectomy
Lung ca
Aspergilloma
TB
Lung absecess
Investigation for fitness for lobectomy
FEV1 >1.5L
o Full lung fuction test with transfer factor assessment
o Cardiopulmonary testing
Investigation for fitness for pneumonectomy
FEV >2L
o Full lung fuction test with transfer factor assessment
o Cardiopulmonary testing
DD for wheeze
o COPD
o Asthma
o Bronchiectasis
o Obliterative bronchiolitis (due to viral infection, certain pollutants or stem cell transplantation as GVHD or after lung transplant as GVHD)
Inheritance and chromosome and gene for CF
- Autosomal recessive, chromosome 7, CFTR gene
Management of CF
Dietician, OT, physio, SALT, psychologist, social workers
Regular chest physio for postural drainage and to clear mucus
Nebulised antimucolytics (recombinant DNAse and hypertonic saline)
Nebulised antibiotics
Azithromycin prophylaxis
Creon tablets
Nutritional supplement by PEG tube
o CFTR modulating therapies such as ivacaftor and lumacaftor/ivacaftor have resulted in a marked improvement in lung function and exacerbation frequency in eligible patients who carry specific mutations that respond to these medications
Most common bacteria in CF in adults and children
pseudomona in adult
Staph aureus in children
Bacteria in CF that will be contraindicated for lung transplantation?
o Burkholderia cenocepacia, mycobacterium abscessus
Things to look out for on examination of someone with suspected pulmonary fibrosis
Indications for pneumectomy
o NSCLC (PET scan if potentially radically treatable)
o Localised bronchiectasis
o malignant nodules
o Lung abscess, treated either with a lobectomy or a wedge resection
o Tuberculosis
o Lung trauma with significant damage to the lung
Why pneumonectomy and not lobectomy
- Lung ca involving both the upper and lower lobe or that the tumour was particularly central and excision of just one lobe would not have been possible
- significant bronchiectasis on the left side requiring pneumonectomy
Bronchiectasis investigation
- FBC, U&E, LFT, CRP
- HIV, immunoglobulin, pneumococcal serology
- Aspergillus precipitins
- CF (for all age <40)
- Sputum analysis (general, fungal and mycobacterium cultures)
- Spirometry
- CXR, HRCT
- Referral to specialist centre for nasal brush biopsy if suspected primary ciliary dyskinesia
Signs to look out for in suspected lung cancer
- Clubbing
- SVC obstruction
- Radiotherapy tattoo
- Hoarse voice (from recurrent laryngeal nerve compression)
- Horner’s syndrome
- Hypercalcaemia, SIADH, LEMS
- Pulmonary osteoarthropathy or wasting of the small muscles of the hand
Also look for recurrence - cachexia, anaemia, neck lymphadenopathy, clubbing
Investigation for suspected lung cancer
- Bronchoscopy guided biopsy
- Endobronchial USS biopsy (to sample mediastinal lymph nodes)
- CT guided percutaneous biopsy
- Lymph node biopsy
- Spirometry to see fitness for surgery
What is light criteria for exudate?
pleural fluid is exudative if one of the following applies:
pleural fluid: serum ratio is > 0.5
pleural fluid: serum LDH ratio is > 0.6
pleural fluid is more than two-thirds limit of normal serum LDH
a pH < 7.1 also suggests an exudate
Reason for low glucose on pleural aspirate?
infection, malignancy and oesophageal rupture.
Particularly low glucose levels (< 1.6 mmol/L) are seen in effusions due to rheumatoid arthritis.
Indication for drainage in pleural effusion?
If pH <7.2, frank pus, culture positive
Management of pleural effusion in lung ca?
Pleural catheter long term
Pleurodesis (medically with talc or via thoracoscopy)
Signs of yellow nail syndrome
Lymphodema (lower limb)
Thick dystrophic nail with fungal infection
Pleural effusion
Bronchiectasis
Contraindication to renal transplant
Active or recent malignancy
Active vasculitis
Issue with donor matching
Deep seated infection
Give 6 examples of congenital heart disease
pulmonary atresia
tricuspid atresia,
pulmonary stenosis
Eisenmenger syndrome
Ebstein’s anomaly
transposition of the great arteries
TOF
Examples of scars visible in a fixed TOF
Lateral thoracotomy scar for a Blalock-Taussig Shunt, which is done by plumbing the left subclavian artery into the pulmonary artery, distal to the pulmonary stenosis to improve =blood flow to the lungs.
Midline sternotomy scar for repair of the ventricular septal defect or pulmonary valve replacement
What is pneumonectomy space filled by?
Initially air then becomes filled with gelatinous material
Causes of apical fibrosis
Berryliosis
Radiation
EEA
ABPA/AS
Silicosis/sarcoidosis
TB
Causes of basal fibrosis
Radiation
UIP (RA)
Bronchiectasis
Aspiration
Asbestosis
Complications of bronchiectasis
Corpulence
Secondary amyloidosis (dip urine for protein)
Massive haemoptysis (mycotic aneurysm)
Side effect of TB drug
Rifampicin - orange urine and contact lens, hepatitis, reduced effectiveness of COCP
Isoniazid - peripheral neuropathy and hepatitis
Pyrazinamide - hepatitis
Ethanbutol - optic neuritis and hepatitis
Causes of COPD
Smoking, industrial dust
A1AT deficiency
Severity of COPD
Based on FEV1 vs predicted
Mild = >80% predicted
Moderate = 50-80%
Severe = 30-50%
Very severe = <30%
LTOT criteria for COPD
PO2 < 7.3kPa on air
Or
PO2 <8kPa on air with evidence of decompensation
- corpulmonale
- secondary polycythaemia
Benefit of LTOT in COPD
Improve average survival by 9 months
How long to wear LTOT per day?
At least 16h a day
Dystrophia myotonica inheritance and chromosome
Autosomal dominant
Type 1 = DMPK gene on chromosome 19
Type 2 = ZNF9 gene on chromosome 3
Dystophia myotonica features
Cataract
Ptosis
Myotonia (slowly relaxing hand grip)
Frontal balding
Cardiomyopathy
Dysphagia
Muscle wasting
Areflexia
DD ptosis
Unilateral
- Stroke
- Third nerve palsy
- Horner’s syndrome
Bilateral
- Myotonic dystrophy
- MG
- Congenital
- oculopharyngeal muscular dystrophy
- chronic progressive external ophthalmoplegia (CPEO) (associated with Kearns-Sayre syndrome)
- syringomyelia.
Features of tuberous sclerosis
Angiofibromata
Shagreen patch
Periungal fibroma
Ash leaf macules
Polycystic kidney disease
Renal angiomyolipomata
Seizures
Causes of wasting of hand muscles
Anterior horn cell
- MND
- Syringomyelia
- Cervical cord compression
- Polio
Brachial plexus
- cervical rib
- pancoast tumour
- trauma
Peripheral nerve
- peripheral neuropathy
- combined median and ulnar nerve lesion
Muscle
- disuse atrophy like RA
Features of neurofibromatosis
Cafe au lait spots 6 or more >15mm diamter
Lisch nodule (melanocytic haematoma of iris)
Interstitial lung disease
Enlarged palpable nerve
Type 1 = phaechromocytoma
Type 2 = bilateral acoustic neuroma and sensorineural deafness
Causes of third nerve palsy (pupil sparing)
Medical causes
- Mononeuritis multiplex (DM)
Midbrain infarction e.g. Webers
Midbrain demyelination (MS)
Migraine
Causes of complete third nerve palsy (dilated pupil)
Surgical causes
- Communicating artery aneurysm
- Cavernous sinus pathology (thrombosis, tumour, fistula)
- Cerebral uncus herniation
Optic atrophy causes
Pressure e.g. tumour, glaucoma, pagets
Ataxia (friedreich)
LEbers
Dietary (b12)
Degenrative e.g. retinitis pigmentosa
Ischaemia (Central retinal artery occlusion)
Syphillis and other infections CMV, toxoplasmosis
Cyanide and other toxins e.g. alcohol, lead, tobacco
Sclerosis (MS)
Age related macular degeneration features
Loss of vision
Wet = neovascularisation, drusen
Dry = non-neovascular, atrophic and non exudative
Management of age related macular degeneration
Opthalmology referral
Wet AMD = intravitreal injections of anti VEGF
Retinitis pigmentosa features
Gradual loss of peripheral vision
Retinitis pigmentosa causes and associations
- Congenital (AR ingeritance)
- Post-inflammatory retinitis
Associations
- Friedreich ataxia
- Refsum disease (deafness, cerebellar ataxia, peripheral neuropathy, cardiomyopathy, ichyosis)
- Kearns Sayre syndrome (ataxic, ptosis, opthalmoplegia, permanent pacemaker, deafness)
- Lawrence moon bardet biedl syndrome (deafness, polydactyly, short stature, learning disability)
- Usher syndrome )congenital with neurosensory deafness)
Causes of tunnel vision
Papilledema
Glaucoma
Chorioretinitis
Migraine
Hysteria
Retinitis pigmentosa
Causes of retinal artery occlusion
- Embolic e.g. AF, carotid plague rupture, cardiac mural thrombus - tx aspirin, anticogulation and endaterectomy
- GCA - tx high dose steroids
Causes of retinal vein occlusion
Hypertensin
Hyperglycaemia
Hyperviscosity syndrome e.g. waldenstrom macroglobulinaemia or MM
Glaucoma
Features of hypertensive retinopathy
Grade 1 = silver wiring
Grade 2 = + AV nipping
Grade 3 = + cotton wool spots (superficial and overlies vessels) and flame haemorrhages
grade 4 = + papilloedema
Features of diabetic retinopathy
Pre-proliferative = no neovascularisation
Proliferative = neovascularisation + cotton wool spots + papilloedema
Causes of bilateral high stepping gait
Peripheral neuropathy
- Charcot Marie Tooth disease
- CIDP
- Leprosy
Neuromuscular:
- inclusion body myositis
- motor neurone disease
- dystrophia myotonica
other = cauda equina syndrome.
DD for leg rash in diabetic patient
- Necrobiosis lipoidica diabeticorum
- Diabetic dermopathy
- Granuloma annulare
- Leg ulcers
- Vitiligo
- Eruptive xanthomata
DD for leg ulcers
- Arterial ulcer
- Venous ulcer
- Neuropathic ulcer
- Infective (syphillis, cutaneous leishmaniasis)
- Vasculitic (RA)
- Neoplastic (SCC)
- Haematological (SCA)
Erythema nodosum causes
- Pregnancy
- COCP
- Sarcoidosis (parotid swelling)
- Strep infection
- TB
- IBD
- Idiopathic
- Lymphoma
DD for hyperextensible joint and skin
- Pseudoxanthoma elasticum (AR)
- Ehler danlos (AD)
Difference between pseudoxanthoma elasticum and ehler danlos
- PXE has associated with premature CVD
- PXE has plucked chicken skin appearance especially in neck and axilla
- PXE is AR inheritance - ABCC6 gene in chromosome 16; Ehler danlos is classically Is COL5A1 gene
Methotrexate SE and monitoring?
Myelosuppression
Hepatitis
Pneumonitis
Lung fibrosis
Monitor FBC and LFT weekly until stabilised then every 2-3 months
Baseline CXR
Hydroxychloroquine SE and monitoring
Bulls eye retinopathy
Annual ophthalmology review
Sulfasalazine SE and monitoring
G6PD deficiency haemolysis
Stained contact lenses
Oligospermia
SJS
Lung fibrosis
Myelosuppression
FBC
Raynauds (sensitivity to cold due to vasospams) type
Primary
- Age <30, thumb sparing, low BMI
Secondary
- Due to other underlying cause
- Drugs (beta blocker), cytotoxic chemo
- Systemic sclerosis
Systemic sclerosis features
Limited (more vascular features)
- Calcinosis
- Raynauds
- Esophageal dysmotility (GORD) - very common
- Sclerodactyly (tight joints, sausage digits from inflammation)
- Talengiectasia
- Anti-centromere antibodies
Diffuse cutaneous (more systemic features) - 50% survival in 5 years
- Pulmonary arterial hypertension
- Arrhythmia
- Pulmonary fibrosis
- Anti SCL70 antibodies
Shared features
- Joint pain (overlap syndrome with RA)
- Muscle pain (overlap with polymyositis)
- Gastrointestinal dysmotility + malabsorption + diarrhoea
- Renal artery stenosis
- Glomerulonephritis
- Dry mouth
Ankylosing spondylitis examination test
- Neck movement
- Gait (fixed neck on rotation, question mark posture)
- Occiput wall distance >5cm
- Schobers test modified
- ROM of spine
- Check for other manifestations
- Aortic regurgitation
- Aoical lung fibrosis
- Anterior uveitis
- AB nodal heart block
- Arthritis
Marfan syndrome examination
- Arachnodactylyl (long fingers) - test iwth wrapping thumb and little finger around wrist
- Hyperextensible joints - test with thumb touching ipsilateral wrist and thumb adduction over pam
- High arch palate
- Increased arm span and height ratio
- Check dislocated lens
- Check pectus carinatum
- Check signs of aortic regurgitation/coarctation/MVP
Marfan syndrome inheritance and gene
Firbillin gene chromosome 15, autosomal dominant
Marfan syndrome management
- Annual TTE surveillance aortic root size
- Beta blocker and ACE-i to slow aortic root dilatation
- Screen family member
Management of TIA
- If within 7 days, then need urgent specialist appointment within 24h
- If >7d, then need specialist appointment within 1 week.
- All would need carotid Doppler and endaterectomy within 2 week
Tx with 300mg aspirin for 2 weeks then followed by clopidogrel 75mg
If AF and meet CHADSVASC then DOAC instead of aspirin
Investigation for TIA?
- Bloods to check RF such as FBC, U&E, LFT, Clotting, ESR, TFT, glucose, lipid profile, HBA1C
- Carotid Doppler
- ECG +/- 24h tape
- Echo to look for valvular lesion
- CTH if taking anticoagulation
- MRI head guided by specialist
Causes of hypertension
- Essential hypertension (95%)
- Secondary hypertension (5%)
- Endocrine (6); hyperthyroid, hyperparathyroid, cushing, conn, acromegaly, phaeochromocytoma
- Renal; - glomerulonephritis, ADPKD, renal artery stenosis,
Causes of dysphagia
- Liquid and solid
- Oesophageal spasm
- Achalasia (progressive)
- Oesophageal dysmotility (systemic sclerosis) - Gradual solid then liquid
- Oesophageal ca
- Pyloric stricture
- Gastric stricture
- Oesophageal webbing (plummer vinson) - Painful swallowing
- Oesophagitis (eosinophilic)
- GORD - Neuromuscular
- Stroke
- Myopathy
- MND
- MG
Treatment for methotrexate toxicity
Folinic acid
How to diagnose rheumatoid arthritis?
ACR/EULAR classification criteria (>6 points is diagnostic)
- Joint involvement
- Serology
- ESR/CRP
- Duration > 6 week
Name 5 extra-articular manifestation of RA
Haem - aneamia, splenomegaly (Felty)
Skin - nodules, pyoderma gangrrenosum
Eye - scleritis, episcleritis
Cardio - pericarditis, valvular disease, MI from accelerated atherosclerosis, HF
Resp - pulmonary fibrosis (lower zone), pleural effusion, lung nodules
Renal - amyloidosis
Neuro - peripheral neuropathy, mononeuritis multiplex, compression neuropathy (cervical myelopathy, ulnar neuropathy, carpal tunnel)
X ray finding for RA and OA
RA
- Juxtaarticular osteopenia
- Joint erosions
OA
- Osteophytes
- Subarticular sclerosis
Both:
- Loss of joint space
- Subchondral cyst
SLE 10 signs and symptoms
Neurology
- CN lesion
- Mononeuritis multiplex
- Transverse myelitis
Face
- Malar rash (comes and goes)
- Alopecia
- Discoid rash
Mouth
- Oral ulcers
Cardio
- Valvular incompetence
- Endocarditis
Resp
- Pleuritis
- Pleural effusion
- Pulmonary fibrosis
Renal
- Glomerulonephritis (HTN, haematuria)
- Nephrotic syndrome
Haematological
- Anaemia
- Thombosis
Rheumatological
- Secondary sjogren
Investigation for SLE
FBC (anaemia)
Coombs test (if haemolytic anaemia)
Reticulocyte count
U&E (renal fn)
ESR (chronic inflammation), CRP (active inflammation)
Autoimmune antibodies e.g. ANA (usually very high), dsDNA, ENA
Complement (low C4 and low C3)
Antiphospholipid antibodies
CK (myositis)
Urine dip and urinalysis (blood and protein)
CXR
CTPA (if suspect PE)
Echocardiogram
Skin biopsy (immune complex deposition at dermal epidermal junction)
Renal biopsy to check for lupus nephritis
SLE diagnosis
ACR criteria (4 or more)
- Malar rash
- Discoid rash
- Photosensitivity rash
- oral ulcers
- Non erosive arthritis
- Serositis
- Renal disorder
- Neurological disorder
- haematologicla disorder
- ANA
- other autoantibodies e.g. anti-SM, antidsDNA, antiphospholipid
Management of SLE
Long term steroids
Hydroxychloroquine
Immunosuppressants luke IV cyclophosphamide or azathioprine or methotrexate
Biologics like rituximab
IVIG if serious flares
Side effects of cyclophosphamide
Immunosupression (need check FBC, need oral septrin for prophylaxis)
Bladder Ca
Haemorrhagic cystitis (but rare now due to use of oral mesna)
Infertility
Teratogenic
Alopecia
DD for sudden monocular complete visual loss
Vitreous haemorrhage
Central retinal artery occlusion
Central retinal vein occlusion
Ischaemic optic neuropathy
Trauma
DD for sudden bilateral visual loss
Pituitary apoplexy (bilateral temportal hemianopia)
Stroke (bilateral hemianopia)
Haemorrhagic wet age related macular degenration
DD for sudden monocular partial visual loss
Vitreous haemorrhage
Branch retinal artery occlusion
Branch retinal vein occlusion
DD for absent red reflex
Vitreous haemorrhage
Cataract
DD for gradual onset visual loss
Retinitis pigmentosa
Cataract
ARMD
Diabetic maculopathy
Glaucoma
Optic atrophy
Causes for horner’s syndrome
1st order = brainstem/spinal cord - stroke, tumour, demyelination, syringomyelia, tumourm trauma
2nd order = lung apex/neck - pancoast tumour, cervical node tumour, surgery, trauma, common carotid artery dissection
3rd order = internal carotid artery/carvenous sinus/orbit - dissection, thrombus, tumour
Congenital
Symptoms to ask for CTD screening
Rashes and association with sunlight (SLE)
Losing hair (SLE)
Mouth ulcers (SLE)
Dry mouth and eye (Sjogren)
SOB (pulmonary fibrosis/PAH)
Difficulty swallowing (oesophageal dysmotility in SS)
Heartburn (SS)
Diarrhoea and weight loss (malabsorption in SS)
Painful and weak muscles (Myositis)
Difficulty rising from chair getting in and out of car (proximal myopathy)
DD for thrombocytopenia
Haem
- ITP (flu-like illness)
- HUS (diarrhoea + fever + AKI + shiga toxin Ecoli O157)
- TTP (neuro, fever, MIHA, AKI + ADAMST13)
- Leukaemia
Infection
- Sepsis
- HIV, EBV, CMV, viral hepatitis
Nutritional
- Folate
- B12
Medication
- Quinine
- Heparin (HIT)
Treatment for ITP
IV steroids if symptomatic and platelet <10
IVIG if active bleeding
Haematology referral
Types of syncope
Neuro-mediated AKA reflex (vasovagal, situational, carotid sinus syncope)
Prodromal = light headedness, clammy, sweaty, tunnelling vision
Orthostatic hypotension = drop BP but no cardioinhibitory effect
Primary or secondary autonomic failure
* Parkinsons disease
* Diabetes
Drug induced
Volume depletion
Cardiac syncope = AS, tachy or brady arrythmia, structural heart disease
Prodromal = NO PRODROME
FH of HCOM or sudden death
Vertebrobasilar insufficiency = changing lightbulb
Seizures
Prodromal = funny sight, smell, déjà vu
Causes: genetic, SOL, infection, metabolic (uraemic, alcohol), autoimmune encephalitis
Ix for syncope
Obs = Pulse, LSBP, glucose
12 lead ECG – LVH, HOCM, arrythmia, QT interval, brugada syndrome
Bloods = infection, anaemia, electrolytes
Imaging = CXR (heart disease), echocardiogram, CT head, EEG (look for predisposition to seizure),
Ambulatory ECG
24h vs 72h vs holter monitoring vs implanatable loop recorder
Tilt table testing
Vasovagal = BP drop <60 but HR maintained
Cardiac syncope = HR drop <40 for >10s, BP drop before HR fall
Mixed = HR drop but not <40, BP drop before HR fall
Orthostatic = drop in BP >20 systolic or diastolic >10 on standing
POTS = HR increase >30 or HR >120 after standing + symptoms
Name the motor action of each cervical nerve root starting C5
C5 - shoulder abduction
C6 - Elbow flexion
C7 - Elbow extension, Wrist flexion, wrist extension, finger extension
C8 - Finger flexion
T1 - index and ring finger abduction, thumb movement
How to distinguish each lesion in nerve branch (Radial, median, ulnar) from cervical nerve root radiculopathy?
Radial lesion = unable to extend finger due to damaged posterior interossesous nerve (motor branch of radial nerve); but will be able to flex and extend wrist (C7 intact)
Median lesion = unable to move thumb but can abduct finger (T1 intact)
Ulnar lesion - unable to abduct little finger but able to abduct first finger (intact T1). Sensation altered in medial palm but present in medial forearm (intact C8).
Name the motor action of each lumbar nerve root starting L2
L2 - hip flexion
L3/L4 - knee extension
L4 - ankle inversion
L5 - ankle inversion, ankle eversion and ankle dorsiflexion
S1 - ankle plantarflexion
How to distinguish each lesion in nerve branch (common peroneal nerve) from lumbar nerve root radiculopathy?
Common peroneal nerve palsy = unable to evert ankle and dorsiflex ankle but able to invert foot (intact L5)
Dukes criteria for infective endocarditis
2 major
- Typical organism on 2 blood culture
- Evidence on echo
5 minor
- Fever >38
- Vascular phenomenon - glomerulonephritis
- Embolic phenomenon - splinter haemorrhages
- Atypical organism on blood culture
- Risk factor like prosthetic valve
- Echo suggestive
Medication contraindicated in HOCM and AS
Vasodilators or medications that reduce preload like ACE-i
Echo diagnosis for pulmonary hypertension
Resting PASP >25
Exercise PASP >30
Types of Pulmonary hypertension
Group 1 = pulmonary arterial hypertension (idiopathic, vasculitis, CTD)
Group 2 = left heart disease
Group 3 = right heart disease
Group 4 = chronic VTE
Group 5 = Others
Reasons for having ICD
Primary prevention
- 4 week post MI
LVEF <35% + nonsustianed VT + positive EP study
OR LVEF <30% + QRS >120ms
- Familial high risk SCD e.g. LQTS, brugada, HCM
Secondary prevention
- Cardiac arrest due to VT/VF
OR hemodynamically compromising VT
OR VT with LVEF <35%
CRT indication
LVEF <35%
QRS >120ms
MYHA 2-4 on optimal medical therapy
Absent radial pulse DD
BT shunt
Acute
- Embolism
- Aortic dissection
- Trauma
Chronic
- Atherosclerosis
- Coarctation of aorta
- Takayasu arteritis
Coarctation of aorta associations
Cardiac
- VSD
- Bicuspid aortic valve
- PDA
Non cardiac
- Turners syndrome
- Berry aneurysm
