MRCP PACES A Flashcards

Recognize and interpret key clinical signs, examination findings, and underlying diagnoses across cardiovascular, neurological, respiratory, and abdominal systems in MRCP PACES-style bedside assessments. (250 cards)

2
Q

What are you looking for from the end of the bed when performing an abdominal examination?

A
  • Jaundice
  • Tense ascites
  • Caput medusae
  • Tattoos
  • Nutritional status
  • Scars (surgery, peritoneal dialysis, central venous line, tunnelled line)
  • Spider naevi (5+)
  • Medications around bedside
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3
Q

What signs might you see in the hands when performing an abdominal examination?

A
  • Thin skin (steroid use)
  • Bruising (steroid use, coagulopathy)
  • Dupuytren’s contracture
  • Palmar erythema
  • Leukonychia (hypoalbuminaemia)
  • Koilonychia (severe IDA)
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4
Q

What might a fine tremor be suggestive of during an abdominal examination?

A
  • Alcohol withdrawal
  • Tacrolimus toxicity (if evidence of liver transplant such as Mercedes-Benz scar)
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5
Q

What signs might you see in the face during an abdominal examination?

A
  • Parotid swelling
  • Jaundice
  • Angular cheilitis/stomatitis
  • Conjunctival pallor
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6
Q

What signs might you see in the mouth during an abdominal examination?

A
  • Ulceration (associated Crohn’s disease)
  • Dentition
  • Glossitis (iron and B12 deficiency)
  • Candidiasis
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7
Q

What is enlargement of Virchow’s node suggestive of?

A

Gastric malignancy

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8
Q

If you see that patient has a fistula on abdominal examination, what should you check for?

A
  • Is there a thrill? (i.e. is it functional)
  • Has it recently been needled? (i.e. they are currently on haemodialysis)
  • Check whether there are any renal transplant scars
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9
Q

What are some GI causes of pedal oedema?

A

Hypoalbuminaemia

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10
Q

List some inherited cystic kidney conditions.

A
  • PKD
  • Von-Hippel Lindau syndrome
  • Tuberous sclerosis
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11
Q

What system is used to classify renal cysts in polycystic kidney disease based on contrast-enhanced CT findings?

A

Bosniak System

  • Bosniak 1: Simple Cyst
  • Bosniak 4: Malignant
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12
Q

What is the prevalence of ADPKD?

A
  • 1 in 1000
  • Accounts for 10% of renal replacement patients in the UK
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13
Q

How common are simple renal cysts?

A
  • 2% of those aged 50 years
  • 20% of elderly people
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14
Q

What might you see on general inspection in a respiratory examination?

A
  • Scars
  • Chest wall asymmetry
  • Cyanosis
  • Increased work of breathing
  • Audible wheeze
  • Inhalers
  • Portable oxygen
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15
Q

What are you looking for in the hands of a patient whilst performing a respiratory examination?

A
  • Tar staining
  • Peripheral cyanosis
  • Rheumatoid arthritis (ILD)
  • Thickening of skin (scleroderma)
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16
Q

What are the respiratory causes of clubbing?

A
  • Suppurative lung disease (cystic fibrosis and bronchiectasis)
  • Lung cancer
  • Interstitial lung disease (IPF)
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17
Q

What might a fine tremor be suggestive of in a respiratory examination?

A
  • Salbutamol overuse
  • Tacrolimus toxicity (lung transplant)
  • CO2 retention
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18
Q

Why is an elevated JVP significant in a respiratory examination?

A

Indicates possible cor pulmonale.

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19
Q

What is the hepatojugular reflex suggestive of?

A

Right sided heart failure

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20
Q

What might abnormal chest expansion be suggestive of?

A
  • Symmetrically Decreased: Stiff lungs (interstitial lung disease)
  • Hyperinflated Lungs: COPD, severe asthma
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21
Q

What do you look for on inspection in a cardiovascular examination?

A
  • Oxygen tank
  • Metallic clicks
  • Vein harvesting scars
  • Midline sternotomy scar
  • Lateral thoracotomy scars
  • Previous chest drain insertion scars
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22
Q

What might you see in the hands on a cardiovascular examination?

A
  • Warmth
  • Tendon xanthomata
  • Peripheral cyanosis
  • Clubbing
  • Ecchymosis (warfarin)
  • Palmar erythema
  • Janeway lesions
  • Osler’s nodes (painful)
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23
Q

What are you looking for in the face when performing a cardiovascular examination?

A
  • Malar flush (mitral stenosis)
  • Xanthelasma
  • Corneal arcus
  • Conjunctival pallor or haemorrhage
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24
Q

What signs are you looking for in the mouth during a cardiovascular examination?

A
  • Central cyanosis
  • High arched palate
  • Problems with dentition
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25
Q

What might cause a regularly irregular pulse?

A

Bigeminy and trigeminy

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26
What might a **collapsing pulse** be suggestive of?
* Aortic regurgitation * Patent ductus arteriosus * Hyperdynamic circulation
27
How can the **apex beat** be interpreted?
* **Displaced or thrusting**: Mitral regurgitation * **Undisplaced or heaving**: Aortic stenosis, LVH * **Tapping**: Mitral stenosis
28
What are you looking for upon inspection of the **lower limbs** in a **neurological examination**?
* Wasting * Fasciculations
29
What is the **difference** of **rigidity vs spasticity**?
There is increased tone regardless of speed in **rigidity**, whereas the degree of increased tone increases with speed in **spasticity**.
30
What are the four different types of **eye movements**?
* **Fast Saccades**: Moving rapidly between two fixed points that are a distance apart * **Smooth Pursuit**: Following a slowly moving object with smooth movements * **Vergence**: Focusing on an object close to the face, * **Vestibulo-ocular**: Being able to adjust the eyes to maintain focus on a fixed object in the context of involuntary head movement
31
What is the **accommodation response**?
Pupils constrict and eyes converge when suddenly focusing on a nearby object.
32
What is **athetosis** suggestive of?
Damage to the basal ganglia.
33
What is **pseudoathetosis**?
Writhing movements caused by a **proprioceptive defect** rather than due to damage to the basal ganglia.
34
What should a **cranial nerve palsy** in the context of **polycystic kidney disease** make you suspicious of?
Intracranial aneurysm
35
What are the two mutations that can cause **ADPKD**?
* PKD1 on Chromosome 16 (85%) * PKD2 on Chromosome 4
36
What are some **differences** between **PKD1 and PKD2**?
**PKD1** is more severe and tends to lead to end stage kidney disease at a younger age. It is also MORE common.
37
How does **polycystic kidney disease** present?
* Abdominal pain * Hypertension * Blood test abnormalities * Urinalysis abnormalities * Haematuria * UTI * Intracranial aneurysms * Genetic testing for family members
38
What are the main presenting **features** of **Von Hippel Lindau syndrome**?
* Headache * Ataxia * Dizziness * Weakness * Loss of vision * Hypertension
39
What are the main **associations** of **Von Hippel Lindau syndrome**?
* Renal cell carcinoma * Phaeochromocytoma * Spinocerebellar haemangioblastomas * Retinal angiomas
40
What are the main features of **tuberous sclerosis**?
* Epilepsy * Learning disability * Autism * Hamartomas * Renal cysts * Angiomyolipomas (renal) * Shagreen patches * Ash-leaf spots * Adenoma sebaceum
41
What are the main features of **Alport syndrome**?
* Deafness (bilateral and sensorineural) * Persistent microscopic haematuria * Proteinuria and CKD * Ocular abnormalities (e.g. lenticonus) * Leiomyomas
42
How is **ADPKD** diagnosed?
* Imaging (Ultrasound, CT, MRI) * Genetic testing * Ravine/Pei diagnostic criteria
43
How is **autosomal dominant polycystic kidney disease** (**ADPKD**) managed?
**CONSERVATIVE** * Low salt diet **MEDICAL** * Antihypertensives (RAAS blockade) * Tolvaptan limits cyst development **SURGICAL** * Remove problematic cysts and reduce mass effects * Renal replacement therapy
44
What proportion of patients with **ADPKD** have **extra-renal manifestations**?
* 70% Liver Cysts * 10% Pancreatic Cysts * 5% Berry Aneurysms * Mitral valve prolapse
45
How does **PBC** tend to present?
* Fatigue * Pruritus * Obstructive jaundice
46
What are some clinical features of **PBC**?
* Hepatosplenomegaly * Clubbing * Xanthelasma * Excoriation marks (from pruritus) * Jaundice
47
What are the main causes of **chronic liver disease**?
* Alcoholic liver disease * Non-alcoholic fatty liver disease * Viral hepatitis * Autoimmune hepatitis * Drug-induced liver disease * Infiltrative disease (e.g. Wilson's disease, haemochromatosis)
48
Which antibody is associated with **PBC**?
Antimitochondrial (M2) antibody ## Footnote Also associated with raised ALP and IgM.
49
How is **primary biliary cholangitis** (**PBC**) managed?
**MEDICAL** * Ursodeoxycholic Acid or Obeticholic Acid * Cholestyramine for pruritus **SURGICAL** * Liver transplant (for intractable pruritus and end stage disease) * Transplant has a good prognosis and low rate of recurrence
50
What are the main causes of **chronic kidney disease**?
* Diabetes * Hypertension * Glomerulonephritis * Polycystic Kidney Disease * Chronic Pyelonephritis * Reflux Nephropathy
51
What are the advantages of **renal transplant** over **dialysis**?
* Better quality of life * Increased survival (though generally fitter patients are chosen for transplant) * More cost-effective in the long-term
52
When should a patient be worked up for **renal transplant**?
Ideally as they progress towards end stage renal failure but before starting dialysis.
53
Which diseases are particularly at risk of recurrence in **renal transplantation**?
* Focal segmental glomerulosclerosis * Amyloidosis * IgA Nephropathy * Haemolytic uraemic syndrome
54
What are some **contraindications** for **renal transplantation**?
* Current malignancy * Severe poorly controlled comorbidity (e.g. COPD, heart failure) * Active infection (e.g. viral hepatitis) * Active substance misuse * Uncontrolled psychiatric disease * History of medication non-compliance
55
What is **hyperacute rejection** in the context of a **renal transplant**?
* Due to presence of antibodies against donor kidney * Kidney swells and becomes dusky within minutes of revascularisation * Transplant nephrectomy required * Very rare
56
What is **acute cell-mediated rejection** in the context of a **renal transplant**?
* Happens between 2 weeks to 6 months of transplantation * Characterised by mononuclear cell infiltration in the interstitium * Treated with high-dose steroids (often reversible)
57
What is **acute antibody-mediated rejection** in the context of a **renal transplant**?
Three of the four following criteria: * Graft dysfunction * Histological evidence of tissue injury * Positive staining for C4d * Presence of donor-specific antibodies
58
What is **chronic transplant rejection**?
Gradual decrease in kidney function that becomes apparent 3 months after transplant.
59
What specific **work up** needs to be done ahead of **renal transplantation**?
* ABO and HLA typing * Virology (hep B, hep C and CMV) * Urinalysis and culture * Cardiovascular assessment
60
What are some of the effects of **steroid** use for **immunosuppression**?
* Thin skin * Easy bruising * Cushingoid appearance * Diabetes mellitus
61
What are some physical features of **immunosuppressive therapy**?
* **Ciclosporin**: Gingival hyperplasia * **Tacrolimus**: Tremor * **Steroids**: Cushingoid, easy bruising
62
What are some **complications** of **immunosuppression**?
* New-onset diabetes after transplantation * Cancer (especially squamous cell carcinoma and non-Hodgkin lymphoma) * Opportunistic infections (BK, CMV)
63
What are some **prognostic factors** for **renal transplants**?
* Primary diagnosis * Previous episodes of rejection * Total ischaemic time * Donor factors (e.g. age)
64
Define **end stage renal failure**.
eGFR < 15 mL/min
65
What are the features of **decompensation** of **chronic liver disease**?
* Jaundice * Asterixis * Ascites
66
What are some causes of **hepatomegaly**?
* Cirrhosis * Congestive cardiac failure * Carcinoma * Viral hepatitis * Immune (PBC, PSC, autoimmune hepatitis) * Infiltrative (haemochromatosis, amyloidosis, Wilson's disease)
67
What are the main **investigations** you would request in a patient with **hepatomegaly**?
* FBC * U&E * LFT * Clotting * Glucose * Ferritin * Viral hepatitis screen * Caeruloplasmin * Autoantibodies * Ultrasound * Ascitic Tap * Fibroscan (extent of liver fibrosis)
68
What are the best markers of **liver synthetic function**?
* Albumin * Clotting (PT)
69
How can **AST: ALT ratio** be interpreted?
* > 2 is suggestive of alcoholic liver disease * Very high in ischaemic hepatitis
70
What are the different **parameters** that can be analysed on an **ascitic tap** and why would you request them?
* Cell count (SBP) * Gram stain * Amylase/lipase (in pancreatitis) * Cytology (cancer) * SAAG (11 g/L) * Glucose (low in infection)
71
What should all patients with **cirrhosis** have done?
**Endoscopy** to check for varices.
72
Which tests are useful in the diagnosis of **chronic pancreatitis**?
* Faecal elastase * Albumin * Vitamin D * Magnesium
73
How is **chronic pancreatitis** treated?
* Creon * Pain management * PPI
74
What are some causes of increased **SAAG**?
* Cirrhosis * Alcoholic hepatitis * Budd-Chiari syndrome * Heart failure
75
What are some causes of decreased **SAAG**?
* Nephrotic syndrome * Pancreatitis * Malignancy * Peritonitis
76
How is **ascites** managed?
* Treat cause * Drain * Diuretics (often spironolactone then furosemide) * Prophylactic antibiotics
77
What are some differentials for **abdominal distension**?
* Fluid (ascites) * Flatus * Foetus (pregnancy) * Obesity * Tumour
78
What are some reasons for a **nephrectomy** in patients with **ADPKD**?
* Debulking to provide room for transplant * Renal cell carcinoma * Recurrent urinary tract infections * Chronic pain * Excessive bleeding
79
How does **autosomal recessive polycystic kidney disease** differ from **ADPKD**?
It causes **end stage renal impairment** in childhood (much less common).
80
Why is **peritoneal dialysis** avoided in patients with **ADPKD**?
* Mechanical difficulty performing peritoneal dialysis if abdomen contains bulky kidneys * Increased risk of cyst infection
81
What would you request to complete the **examination** after performing an **abdominal examination**?
* Observations * Examine external hernial orifices and external genitalia * Perform a DRE
82
What are the two forms of **peritoneal dialysis**?
* Continuous ambulatory peritoneal dialysis * Automated peritoneal dialysis
83
When is **dialysis** recommended by **NICE**?
* eGFR < 5-7 mL/min if asymptomatic * Impacted by symptoms of uraemia on a daily basis
84
What are some **symptoms** that patients on **dialysis** may experience?
* Breathlessness * Fatigue * Pruritus * Loss of appetite * Abdominal cramps * Depression
85
What are the **acute indications** for **dialysis**?
* Acidosis * Pulmonary oedema * Hyperkalaemia * Uraemic complications
86
What are the most common **immunosuppression regimens** used in **renal transplants**?
* Calcineurin inhibitor (e.g. tacrolimus) * Antimetabolite (e.g. MMF) * Steroid
87
After how long does **continuous ambulatory peritoneal dialysis** stop working?
3-6 years
88
How long does a **fistula** take to mature?
* 4-6 weeks * N.B. you want to achieve a flow rate of 250-400 mL/min into the dialyser
89
What are some **complications** of **peritoneal dialysis**?
* Bacterial peritonitis * Ultrafiltration failure * Encapsulating peritoneal sclerosis * Hernia * Fluid leak
90
What are the main **complications** of **haemodialysis**?
* Hypotension * Infection (e.g. line-related) * Venous stenosis * Air embolus * Cardiovascular disease * Dialysis-related amyloidosis * Pseudogout
91
What are the main features of **graft rejection** after a **renal transplant**?
* Fluid retention * High blood pressure * Rapidly rising creatinine
92
How does **CMV infection** manifest in **transplant patients**?
* Myocarditis * Encephalitis * Retinitis * Renal dysfunction ## Footnote Treated with ganciclovir.
93
What are some factors that favour the use of **haemodialysis** rather than **peritoneal dialysis**?
* Previous peritonitis * Severe malnutrition * Catabolic states (e.g. intercurrent illness) * Chronic severe respiratory disease (may compromise respiratory function)
94
What are the causes of **splenomegaly**?
* Infiltration * Increased function * Abnormal flow * Immune hyperplasia * Disordered immunoregulation ## Footnote **Infiltration**: Myelo and lymphoproliferative disorders, Lymphoma, Amyloidosis, Sarcoidosis, Gaucher's disease, Lipid storage disease **Increased function**: Spherocytosis, Thalassemia, Early sickle cell **Abnormal flow**: Cirrhosis, Hepatic or portal vein obstruction **Immune hyperplasia**: Tropical: chronic malaria, visceral leishmaniasis, brucellosis, Glandular fever, Infectious hepatitis **Disordered immunoregulation**: Felty's syndrome, SLE
95
What other **features** should you look for on examination in a patient with **splenomegaly**?
* Hepatomegaly and ascites: cirrhosis * Jaundice: haemolytic anaemia, liver impairment * Anaemia * Lymphadenopathy: lymphoma, infection (e.g. EBV)
96
What are the causes of **massive splenomegaly**?
* Chronic myeloid leukaemia * Myelofibrosis * Gaucher's disease * Chronic malaria * Kala azar
97
What **investigations** should you request in a patient with **splenomegaly**?
* FBC and film * If suspicion of haematological malignancy: CT CAP, bone marrow aspirate/trephine, lymph node biopsy * If suspicion of malaria: thick and thin films
98
What is **hereditary spherocytosis** and how is it diagnosed?
* **Autosomal dominant** defect in the cell wall of red cells * **Diagnosis**: EMA binding test (or osmotic fragility)
99
What would you see on the **blood film** of a patient with **hereditary spherocytosis**?
* Small round red cells that have lost their central pallor * Increased reticulocytes
100
Which causes of **splenomegaly** are more likely in **YOUNG people**?
* Spherocytosis * Thalassemia * Early sickle cell * Tropical: chronic malaria, visceral leishmaniasis, brucellosis * Glandular fever * Lymphoma
101
Why is it important to look inside the **top lip** of a patient when doing an **abdominal examination**?
To check for **gingival hyperplasia** (ciclosporin).
102
Which **scars** are associated with **liver transplant**?
* Mercedes-Benz (bilateral rooftop with sternal extension) * Makuuchi (inverted J)
103
What **signs** would suggest that a **liver transplant** is not working properly?
* Ascites * Asterixis * Jaundice
104
Which **features** of **chronic liver disease** are likely to persist post-transplantation?
* Gynaecomastia * Dupuytren's contracture * Splenomegaly (may change)
105
Is the size of a **transplanted liver** significant?
Difficult to interpret because it may be suggestive of a **size mismatch** between the donor and the recipient.
106
What are some **physical signs** of **post-transplant immunosuppression**?
* Scars from excision of SCCs * Tremor (tacrolimus) * Gingival hypertrophy (ciclosporin) * Steroid (Cushingoid, diabetes mellitus cap glucose testing and insulin injections, easy bruising)
107
What **clinical features** may be suggestive of an underlying diagnosis of **haemochromatosis**?
* Venesection scars * Tanned appearance * Hepatosplenomegaly
108
What are the main **indications** for **liver transplantation** in the UK?
* Cirrhosis * Hepatocellular carcinoma * Acute fulminant liver failure (e.g. paracetamol overdose)
109
Define **acute liver failure**.
Multisystem disorder characterised by severe impairment of liver function with **encephalopathy** within 8 weeks of the onset of symptoms and no recognised underlying chronic liver disease.
110
Which **variant syndromes** are considered for **liver transplantation**?
* Diuretic-resistant ascites * Chronic hepatic encephalopathy * Intractable pruritus * Hepatopulmonary syndrome * Polycystic liver disease * Recurrent cholangitis
111
What are some **contraindications** for **liver transplantation**?
* IV drug use * Ongoing alcohol excess * Significant medical or psychiatric comorbidities * This is all discussed by a specialist MDT
112
Which **scoring criteria** are used to assess patient's need for **liver transplantation**?
* UKELD (based on INR, creatinine, bilirubin and sodium) * A score of 49 or more is considered for elective liver transplantation
113
Which **scoring system** is used to triage **acute alcoholic hepatitis**?
* Maddrey's Score * Glasgow Alcoholic Hepatitis Score
114
What are the main causes of **ascites**?
* Vascular * Low albumin * Peritoneal * Miscellaneous ## Footnote **Vascular**: Portal hypertension in liver disease, Budd-Chiari syndrome, Congestive cardiac failure, Constrictive pericarditis **Low albumin**: Cirrhosis, Nephrotic syndrome, Protein-losing enteropathy **Peritoneal**: Meig syndrome, TB, Malignancy (e.g. ovarian) **Miscellaneous**: Pancreatitis, Hyperthyroidism (advanced)
115
Which **investigations** should you request in a patient with **ascites**?
* **Bloods**: FBC, Clotting, LFT, viral serology, autoantibody screen, ferritin, caeruloplasmin, AFP * **Imaging**: Ultrasound, CXR (congestive cardiac failure) * **Other**: Ascitic Tap
116
What are some **indications** for a **transjugular liver biopsy**?
* Deranged coagulation * Massive ascites
117
What causes **hereditary haemochromatosis**?
**Autosomal recessive** condition with variable penetrance. Caused by mutation in the HFE gene (increases intestinal iron absorption).
118
How does **hereditary haemochromatosis** present?
* Screening relatives * Lethargy * Arthralgia * Skin pigmentation * Hepatomegaly * Chronic liver disease * Diabetes mellitus * Erectile dysfunction
119
How do **men and women** differ in the presentation of **haemochromatosis**?
Women tend to present later in life due to the protective effect of menstruation.
120
Which **investigations** are most important in the diagnosis of **hereditary haemochromatosis**?
* Transferrin saturation (raised) * Ferritin (raised) * HFE genotyping * Liver biopsy (not essential but can help assess stage of disease) * Urinalysis and HbA1c for diabetes mellitus * ECG for AF * AFP for HCC * Liver ultrasound for cirrhosis
121
How is **hereditary haemochromatosis** managed?
* Venesection (3-4 times per year) * Alternative: iron chelation * Avoid food containing iron or vitamin C * Avoid alcohol * Transplant (if cirrhotic)
122
How should patients with **hereditary haemochromatosis** be monitored?
* Regular FBC, transferrin saturation and ferritin monitoring * Monitor HbA1c * If cirrhotic, regular ultrasound and AFP monitoring
123
What is the **prognosis** in **hereditary haemochromatosis**?
If **non-diabetic** and **non-cirrhotic**, venesection returns life expectancy to normal.
124
What are the main **complications** of **hereditary haemochromatosis**?
* Cirrhosis (and HCC) * Diabetes mellitus * Cardiomyopathy
125
What are the main aspects of managing **hepatic encephalopathy**?
* Lactulose (aiming for at least 2 bowel movements per day) * Rifaximin ## Footnote Identify and treat any precipitants (e.g. bleed).
126
How should an **upper GI bleed** be managed?
* A to E assessment and appropriate resuscitation with IV fluids and/or blood products * Prophylactic antibiotics (e.g. ceftriaxone) * Terlipressin * Calculate Glasgow-Blatchford score and discuss with on call endoscopist * EMERGENCY: Sengstaken-Blakemore tube
127
How does **spherocytosis** present?
* Anaemia * Jaundice (including neonatal) * Splenomegaly * Screening of first degree relatives
128
What are some of the **complications** of **hereditary spherocytosis**?
* Aplastic crisis * Severe anaemia * Gallstones
129
What mutation causes **hereditary spherocytosis**?
5 possible mutations with the most common (ANK1) being on chromosome 8.
130
What is the outcome of **splenectomy** in moderate-to-severe cases of **hereditary spherocytosis**?
Virtually eliminates haemolysis
131
How can **intercurrent infections** affect people with **hereditary spherocytosis**?
* Increase rate of haemolysis * e.g. EBV can increase spleen size and worsen haemolysis
132
What is the main **complication** of **hereditary spherocytosis**?
Pigment gallstones
133
What are the relevant **examinations** to perform in a patient with suspected **ankylosing spondylitis**?
* Listen for aortic regurgitation * Listen for pulmonary fibrosis * Assess chest expansion * Assess Achilles' tendons * Assess the axial spine
134
What are the **motor functions** of the **ulnar nerve**?
**Anterior Forearm** * Flexor carpi ulnaris (flex and adduct hand) * Flexor digitorum profundus medial half (flexed ring and little finger at DIP) **Hand** * Most intrinsic hand muscles (hypothenar muscles, medial lumbricals, Adductor pollicis, palmar and dorsal interossei)
135
What is **Froment's sign**?
* Test for ULNAR nerve palsy * Flexion of thumb at interphalangeal joint when pulling paper away
136
What are the **sensory components** of the **ulnar nerve**?
* Medial half of palm and dorsum of hand * Medial 1 and a half fingers ## Footnote There are 3 branches: palmar, superficial and dorsal.
137
What are the main **motor features** of **ulnar nerve damage** at the elbow?
* Flexion of wrist accompanied by abduction * Weakness of finger abduction and adduction (due to interossei) * Weakness of 4th and 5th digits (due to medial 2 lumbricals and hypothenar muscles) * Weakness of thumb adduction * Positive Froment sign
138
What are the main **sensory features** of **ulnar nerve damage**?
* Loss of sensation of medial half of palm and dorsum of hand * Loss of sensation over medial 1 and a half digits
139
What are the main **motor features** of **ulnar nerve damage** at the wrist?
* Weakness of finger abduction and adduction * Weakness of 4th and 5th digits (due to medial lumbricals and hypothenar muscles) * Weakness of thumb adduction * Froment's sign positive * Wasting of hypothenar eminence
140
How does the **sensory deficit** in **ulnar nerve palsy** at the wrist level differ from the elbow?
Dorsal branch is usually unaffected in **ulnar nerve palsy** at the wrist.
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What are the main **differences** between **ulnar nerve damage** at the wrist and at the elbow?
* Normal sensation over dorsal area * More severe clawing (due to preserved innervation of flexor digitorum profundus) * Normal wrist flexion without abduction
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What is the **ulnar paradox**?
* A lesion at the elbow produces a milder deformity than a lesion at the wrist. * This is because a lesion at the elbow also paralyses flexor digitorum profundus (this causes weakness of finger flexion to balance the weakness of finger extension caused by paralysis of the lumbricals).
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What are the causes of **ulnar nerve injury** at the wrist and elbow?
* **Wrist**: Lacerations to the anterior wrist * **Elbow**: Trauma (e.g. supracondylar fracture), surgery
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Which **muscles** are innervated by **C8/T1** via the MEDIAN nerve?
* Lateral 2 lumbricals * Opponens pollicis * Abductor pollicis brevis * Flexor pollicis brevis
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Which **investigation** could you request in a patient with a suspected **ulnar nerve palsy**?
* EMG * MRI of the neck should be considered if concerns about cervical radiculopathy
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How is **neuropathic pain** managed?
* Tricyclic antidepressant (e.g. amitriptyline) * Then move on to pregabalin and gabapentin * Alternative: SNRI (duloxetine) * Non-Tablet: Capsaicin cream
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What are the main causes of **peripheral neuropathy**?
Diabetes mellitus * **Metabolic**: uraemia, hyperthyroidism, vitamin B1/B6/B12 deficiencies * **Toxic**: chemotherapy (e.g. vincristine, cisplatin), antibiotics (e.g. isoniazid), alcohol excess * **Inflammatory**: CIDP, sarcoidosis, ANCA-positive vasculitis, rheumatoid arthritis * **Malignant**: paraneoplastic syndromes (e.g. lung cancer, myeloma)
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Which **tests** should be done in a patient with **peripheral neuropathy**?
* **Bedside**: urinalysis, BM, fundoscopy (diabetic retinopathy) * **Bloods**: HbA1c, U&E, FBC (macrocytosis), B12, LFT (alcohol), TFT, ESR (chronic inflammation), serum electrophoresis * **Other**: Nerve conduction studies
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Why are **nerve conduction studies** useful in **peripheral neuropathy**?
* Distinguish demyelinating vs axonal neuropathy * Determine whether the pattern is length-dependent vs non–length dependent ## Footnote Demyelinating, non–length dependent neuropathies are more likely to be immune-mediated (e.g. CIDP).
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What are some aspects of managing **peripheral neuropathy**?
* Treat cause (e.g. tight glycaemic control) * Podiatry for foot care * Physiotherapy (if gait issues)
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Which **investigations** should be requested in suspected **Charcot-Marie-Tooth disease**?
* Nerve conduction studies (severe and uniformly reduced responses) * Genetic testing (autosomal dominant) ## Footnote Nerve conduction studies will also tell you whether it is axonal or demyelinating in nature which helps identify the type of CMT.
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How is **Charcot-Marie-Tooth disease** managed?
* No disease-modifying treatments * Genetic counselling for family members * OT/PT * Orthotics
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How is a **homonymous hemianopia** caused by an **occipital stroke** different from that caused by an MCA stroke?
**Occipital stroke** has **macular sparing**.
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Which **investigations** should be requested to identify potential causes in a patient with a **stroke**?
* Echocardiogram * Carotid Doppler * Ambulatory ECG Monitoring
155
List some causes of a **spastic paraparesis**.
* Spinal trauma * Disc prolapse * Multiple sclerosis * Motor neurone disease * Cerebral palsy * Spinal infarct
156
What are the main **investigations** to request in suspected **multiple sclerosis**?
* MRI (Brain and Spine) * CSF (oligoclonal bands)
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How is **multiple sclerosis** managed?
* Acute Relapse: High dose IV steroids (methylprednisolone) * Disease-Modifying Treatments (beta interferons, glatiramer acetate, natalizumab) * Manage complications (baclofen for spasticity, psychological support, PT/OT, neuropathic pain medications, laxatives for constipation)
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What are the different **types** of **multiple sclerosis**?
* Relapsing-remitting (90%) * Secondary progressive * Primary progressive ## Footnote 25% of RRMS becomes SPMS within 6 years.
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What are some **features** that you would see in **cerebellar ataxia** that you would not see in sensory ataxia?
* Nystagmus * Dysarthria
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What are the main **features** of **sensory ataxia**?
* Impaired joint position and vibration sense * Pseudoathetosis * Finger nose test is ok with eyes open but struggle with eyes closed
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What are the main causes of **sensory ataxia**?
* **Central**: Dorsal column damage * **Peripheral**: diabetes mellitus, alcohol excess, uraemia, vitamin B1/6/12 deficiency
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What is the most common cause of both central and peripheral **sensory ataxia**?
B12 deficiency
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Which other **neurodegenerative condition** is often comorbid in the context of **ALS**?
Frontotemporal dementia
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What are the four types of **motor neuron disease**?
* Amyotrophic lateral sclerosis * Progressive bulbar palsy * Progressive muscular atrophy * Primary lateral sclerosis
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What are some **differentials** for **motor neuron disease**?
* Myopathy * Stroke * Guillain-Barre syndrome * Myasthenia gravis * Spinal cord tumours
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Which **investigations** are useful in suspected **motor neuron disease**?
* EMG (look for fasciculations and fibrillations) * Nerve conduction studies
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How is **motor neuron disease** managed?
* Riluzole (small prognostic benefit) * OT/PT * SALT * Dietician (nutritional plans) * Addressing ventilatory issues (e.g. NIV)
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What is **Kennedy disease**?
**X-linked spinobulbar muscular atrophy** * CAG repeat associated lower motor neuron disease that presents with progressive weakness and wasting of limb and bulbar muscles * Associated with gynaecomastia and subfertility * Perioral fasciculations
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What is the **prognosis** for **MND**?
* Varies depending on type * 15-20% 5-year survival
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What is **myelopathy**?
Injury to the spinal cord causing neurological symptoms.
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How is a **myelopathy** investigated?
* Urgent MRI (if acute) * May need urgent neurosurgical discussion
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What are some causes of **myelopathy**?
**Acute** * Disc prolapse * Infarction * Epidural abscess **Subacute** * Infection (e.g. mycoplasma induced transverse myelitis) **Chronic** * B12 deficiency * Inflammatory (e.g. MS) * Spinal cord tumour * Hereditary spastic paraparesis
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What are the main **clinical features** of **Kennedy's disease**?
* Lower motor neuron only * Progressive weakness and wasting of limb and bulbar muscles * Slow rate of progression * Perioral Fasciculations * Gynaecomastia * Subfertility
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What are a **head tremor** and **truncal ataxia** associated with?
Cerebellar ataxia
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What are the main causes of **cerebellar ataxia**?
* **Acute**: haemorrhage, stroke, infection (varicella), autoimmune * **Chronic**: alcohol excess, nutritional deficiency (vitamin E)
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What are some **classical features** of **polio**?
* Limb wasting and weakness * Limb shortening * Areflexia * Pes cavus
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Which conditions are associated with **pes cavus**?
* Charcot-Marie-Tooth disease * Polio * Friedreich's Ataxia
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What are the main features of **post-polio syndrome**?
* Progressive weakness in previously affected areas * Pain * Cramping * Fatigue
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What is **ataxia**?
Group of conditions that are characterised by disorders of **coordination, balance and speech**.
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What simple **test** can you use to distinguish **sensory ataxia** from cerebellar ataxia?
**Sensory ataxia** gets much worse with the eyes closed (e.g. not being able to do finger to nose with eyes closed).
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Which unusual **neurological features** should raise suspicion of an **immune-mediated polyneuropathy**?
* Non-length dependent (e.g. primarily proximal weakness) * Marked asymmetry in neurological features * Prominent sensory ataxia
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What are the most common acute and chronic **immune-mediated neuropathies**?
* **Acute**: Guillain-Barre syndrome * **Chronic**: Chronic Inflammatory Demyelinating Polyneuropathy
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How is **Guillain-Barre syndrome** managed?
* Monitor FVC as evidence of respiratory compromise would warrant ABG and discussion with ICU (if FVC < 20 mL/kg) * IVIG and plasma exchange
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What is the **prognosis** of **Guillain-Barre syndrome**?
* Most people make a full recovery though this could take months * 5% mortality rate
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Which **investigations** should be requested in suspected **Guillain-Barre syndrome**?
* CSF Analysis (high protein) * Nerve conduction studies (evidence of demyelination) * FVC
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What are the main **clinical features** of **Guillain-Barre syndrome**?
* Rapidly evolving ascending pattern of weakness * Variable sensory loss * Flaccid muscle tone * Hyporeflexia * Post-infection (most commonly Campylobacter jejuni)
187
List some causes of **transverse myelitis**.
* **Inflammation**: MS, neuromyelitis optica, sarcoidosis, lupus * **Infection**: VZV, Mycoplasma, TB, HTLV1
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What are the main **clinical features** of a **spastic paraparesis**?
* Hypertonia * Brisk reflexes * Upgoing plantars * Weakness * Reduced sensation
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How can you test **bradykinesia**?
Finger snapping and rapid rotation of the wrists
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In someone with **Parkinsonian features**, which other examination steps would you take to assess whether **Parkinson's plus syndromes** are a possibility?
* Eye Movement (PSP) * Nystagmus (cerebellar signs are associated with MSA) * LSBP (autonomic dysfunction in MSA)
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What are some early **non-motor features** of **Parkinson's disease**?
* Anosmia * REM sleep disorders * Constipation
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What are some other **non-motor features** of **Parkinson's disease**?
* Depression * Insomnia * Pain * Autonomic disturbance
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What are the **cardinal features** of **Parkinson's disease**?
* Tremor * Rigidity * Bradykinesia * Gait instability
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List some other causes of **Parkinsonism** other than **Idiopathic Parkinson's Disease**.
* Progressive supranuclear palsy * Multiple system atrophy * Dementia with Lewy bodies * Vascular Parkinsonism * Drug-induced Parkinsonism
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How is **idiopathic Parkinson's disease** diagnosed?
* Largely a clinical diagnosis * MRI and SPECT imaging may be considered
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How is **Parkinson's disease** managed?
* **Pharmacological**: Dopaminergic therapy (Levodopa, Dopamine Agonists), MAO-B inhibitors (selegiline), COMT inhibitors (entacapone), amantadine * **Surgical**: Deep brain stimulation * **Non-medical**: PT/OT, Speech and Language Therapist (SALT)
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How do you assess **speech**?
1. Comprehension (1-, 2 and 3-step commands) 2. Repetition (baby hippopotamus) 3. Naming (e.g. pen) 4. Spontaneous speech (normal articulation, phonological errors, word retrieval difficulty, hesitancy, anomia)
198
Which **hemisphere** is dominant in **language**?
Left
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What are the main features of **posterior circulation strokes**?
* Cranial nerve involvement * Disorder of conjugate eye movements * Cerebellar signs * Homonymous hemianopia * Cortical blindness * Unilateral or bilateral motor or sensory deficit
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What are the main features of **total anterior circulation strokes**?
* Hemiplegia (contralateral) * Homonymous hemianopia (contralateral) * Aphasia or visuospatial disturbance * Hemisensory deficit (contralateral)
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What are the features of a **partial anterior circulation stroke**?
Two of the following need to be present for a diagnosis of a PACS: * Unilateral weakness (and/or sensory deficit) of the face, arm and leg * Homonymous hemianopia * Higher cerebral dysfunction (dysphasia, visuospatial disorder)
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Which **features** on **neurological examination** are associated with **raised intracranial pressure**?
* 6th cranial nerve palsy * Papilloedema * Visual field defect
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What **features** of a history would make you think of an **immune-mediated cause** of a polyneuropathy?
* Waxing and waning * Abrupt onset * Starting in the hands, trunk or face (non-length dependent) * Prominent sensory ataxia (worse in the dark/eyes closed) * Features of associated conditions (inflammatory arthropathy, sicca syndrome)
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Which additional things should you do when examining a patient with a suspected **movement disorder**?
* Assess for tremor in different positions (e.g. hands in front of face) * Finger snapping (bradykinesia) * Eye movement testing
205
What are the main causes of **chorea**?
* **Acute**: Stroke (asymmetric), hypoglycaemic * **Chronic**: Sydenham's chorea (rheumatic fever), SLE, Huntington's disease
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What is **Huntington's disease** caused by?
Autosomal dominant trinucleotide repeat disorder (CAG) on chromosome 4.
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How is **Huntington's disease** investigated?
* Largely a clinical diagnosis (presence of a family history helps) * Genetic testing (blood) * MRI and CT can show loss of striatal volume
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How is **Huntington's disease** managed?
* MDT approach * Genetic counselling * OT/PT * Clinical trials
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If you think that **reflexes** may be brisk, what can you do to check whether it is **pathologically brisk**?
* Finger jerks * Hoffman sign
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What is a positive **Hoffmann sign** suggestive of?
Corticospinal tract dysfunction in the cervical segments of spinal cord.
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What pattern of **weakness** is seen in **myopathies** and in neuromuscular junction disorders?
Proximal weakness
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Which additional **features** of an examination should be performed if **myasthenia gravis** is suspected?
* Fatiguable ptosis * Oculoparesis
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Which **investigations** should be requested in suspected **myasthenia gravis**?
* **Bedside**: Ice test * **Bloods**: ACh receptor antibodies, MuSK antibodies, TFT * **Other**: single-fibre EMG, edrophonium test, CT/MRI to check for thymoma
214
How is **myasthenia gravis** treated?
* **Cholinesterase inhibitor** (e.g. pyridostigmine) * **Steroids** (immunosuppression) * **Steroid-sparing agents** (e.g. azathioprine) * **Myasthenic Crisis**: IVIG and Plasmapheresis (and ventilatory support) * **Surgical**: Thymectomy
215
What causes **Friedreich's ataxia**?
Autosomal recessive condition caused by GAA repeat expansion of the frataxin gene.
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What are the main **features** of **Friedreich's ataxia**?
* Progressive ataxia * Absence of deep tendon reflexes * Spasticity * Peripheral sensory neuropathy * Dysarthria
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What are some **non-neurological manifestations** of **Friedreich's ataxia**?
* **Heart**: cardiomyopathy, arrhythmias, heart failure * **MSK**: kyphoscoliosis * **Pancreas**: diabetes mellitus * **Eyes**: optic atrophy
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Which **investigations** should be requested in suspected **Friedreich's ataxia**?
* Genetic studies * Nerve conduction studies * ECG and echo * Vitamin B12 and vitamin E levels * MRI brain and spinal cord
219
How is **Friedreich's ataxia** managed?
* MDT approach * OT/PT * SALT * Orthotics * Cardiology (for cardiomyopathy) * Genetic counselling * Diabetes management
220
What is **seronegative myasthenia gravis**?
Clinical and neurophysiological evidence of **myasthenia gravis** but without antibodies.
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At what time within the onset of **myasthenia gravis** affecting the eyes would you expect the disease to become **generalised**?
* 2 years * If no generalisation at this point, they can be diagnosed with ocular myasthenia
222
What is the '**steroid dip**' seen in patients with **myasthenia gravis**?
Weakness gets worse when treatment is started. ## Footnote If severe weakness, they should be admitted when steroids are started.
223
How do you test for **fatiguable ptosis**?
Sustained upgaze
224
What is a **midline sternotomy scar** suggestive of?
* CABG (check for vein harvesting) * Valve replacement * Heart transplant
225
What are the common **indications** for **aortic valve replacement**?
* Severe symptomatic aortic stenosis or regurgitation * Infective endocarditis
226
What are the advantages and disadvantages of a **mechanical heart valve**?
**Advantage** * Longer-lasting * Durable (15-20 years) **Disadvantage** * Requires lifelong anticoagulation ## Footnote Bioprosthetic valves only last around 10 years.
227
What are the main causes of **aortic stenosis**?
* Senile calcification * Bicuspid aortic valve * Rheumatic heart disease * Lupus * Fabry's disease
228
What are some **signs of severity** in **aortic stenosis**?
* Quiet second heart sound * Long duration to the murmur * Low volume pulse * Forceful apex beat
229
What are the causes of an **ejection systolic murmur**?
* Aortic stenosis * Aortic sclerosis * HOCM * Pulmonary stenosis
230
Which **investigations** should be requested in suspected **aortic stenosis**?
* ECG (LVH by voltage criteria) * Echocardiogram * CXR (heart failure)
231
What are the **management options** for **severe aortic stenosis**?
* Metallic or Tissue Aortic Valve Replacement * TAVI
232
Which **medications** should be avoided in **severe aortic stenosis**?
* ACE inhibitors * Nitrates ## Footnote In severe aortic stenosis, the ventricle is unable to augment its stroke volume in the context of a reduction in preload. Afterload is fixed they are preload dependent..
233
What are some **echocardiographic features** of **severe aortic stenosis**?
* Peak velocity of > 4 m/s * Mean gradient > 40 mm Hg * Valve area < 1 cm^2
234
What are the **indications** for **mitral valve replacement**?
* Mitral stenosis * Mitral regurgitation * Infective endocarditis
235
In a young person with a **midline sternotomy scar**, what should you consider?
**Mitral valve REPAIR** (rather than replacement) ## Footnote This is preferred to mitral valve replacement.
236
What are the different **types** of **mitral valves**?
* Ball and cage * Single tilting disc * Bileaflet
237
Which **bacterium** causes **prosthetic valve infection** within 6 months of implantation?
Staphylococcus epidermidis
238
What is a **fixed split S2**?
It means that there is a discrepancy between the aortic and pulmonary valve closing leading to a double heart sound. Physiological splitting refers to splitting that only occurs during inspiration. Whereas fixed splitting is present during inspiration and expiration.
239
What are some causes of a **fixed split S2**?
* Pulmonary hypertension * Right heart failure * Atrial septal defect
240
What are some **features** of **pulmonary hypertension** noted on examination?
* Raised JVP * Right ventricular heave * Loud P2 (fixed split second heart sound) * Peripheral fluid overload
241
What are the main causes of a **pansystolic murmur**?
* Mitral regurgitation * Tricuspid regurgitation * VSD
242
What **clinical features** would be suggestive of **SEVERE mitral regurgitation**?
* Features of pulmonary hypertension (raised JVP, RV heave, loud P2) * Displaced apex/heave * Breathlessness and fluid overload
243
What is the **JVP** a reflection of?
Pressures within the right atrium
244
What are the **indications** for **mitral valve replacement**?
* Symptomatic mitral regurgitation * Pulmonary hypertension * Fluid overload * Asymptomatic with declining ejection fraction (< 50%) or LV dilatation (> 50 mm) * Acute mitral regurgitation following MI * Can also be considered in some people with severe MR but without the above where surgical risk is low and durable repair is likely
245
What are the potential causes of **mitral regurgitation**?
* Age-related mitral regurgitation * Mitral valve prolapse (including connective tissue disorders) * Papillary muscle rupture * Rheumatic fever * Infective endocarditis * Cardiomyopathy (ventricular dilation)
246
Why is a **urine dipstick** useful in the context of a **new heart murmur**?
Microscopic haematuria and proteinuria are associated with **infective endocarditis**.
247
Which **ECG features** might you see in **mitral valve disease**?
* P mitrale * AF
248
What causes an **S3 heart sound**?
* Rapid ventricular filling of a compliant left ventricle * S3 = KENTUCKY
249
What causes an **S4 heart sound**?
* Atria contracting against stiff ventricles * S4 = TENNESSEE
250
What are the main **differences** between an **S3** and split S2?
* S2 split is higher pitch * S2 is heart over pulmonary area, whereas S3 is heart at apex
251
Who should be recommended a **bioprosthetic valve**?
* 65 years + for mitral valves * 70+ years for aortic valves * Patients with high risk of haemorrhage (so not for anticoagulation) * Patients who are not compliant with medications * Young women of childbearing age