Neurology

Neurology and O&G

Meige's syndrome vs Meig's syndrome

Meige's syndrome (see video below)
A duet of:
1. blepharospasm
2. oromandibular dystonia (OMD)

Meig's syndrome:
A triad consisting of:
1. pleural effusion
2. ascites
3. benign ovarian tumour

Hippus: athetosis of the pupillary muscles (see below_
Hippus may be normal (physiological) but it may also be pathological, usually due to aconite poisoning (aconitum is a plant genus, a buttercup i think. not good with flowers)

Mind Matters

After charanya and dom left (Mr. 8.30 and yingx left early for some conference) the emergency department, i was left alone to survive the boredom till 5pm. Thats when the driver arrives and when i can go home.

For those who don't know me well:
(i) i can drive just not licensed to do so (yet)
(ii) i refuse to pay extra for cab. the fares rise faster than flour

So yea as i battled the sheer mind-numbing inaction (not a single P1 patient) busying myself going through random X rays, Lab reports and attempting to take a decent history and physical examination from any willing patients (most are nice), this lady got wheeled in from the p3 area.

Middle aged Chinese lady with a history of depression, lets call her Miss X. I know patient confidentiality is very important and i wholly agree. As such, her background will only be mentioned briefly:
- lives with her bf and 2 children
- bf's a drug addict
- she gives tuition to 'get by'

Anyway I didn't spot her at first (was busy finding that elusive vein in this very cachetic but very friendly elderly lady) until a police officer from clementi station asked me if i knew a patient by the name of Miss X who came in for valium overdose. Faced with such a challenging question, and to hide my lack of familiarity with the patients currently in P2, i played the 'bail' card:

"sorry sir, i'm a medical student". HAHA always works.

So lets now discuss: Depression and Mania (dont worry i won't throw the GSMIV at you)

SAGE + CAPS + L (12 in all)
- Sleep disturbances
- Anhedonia
- Guilt
- Energy levels low
- Cognitive loss, Concentration loss
- Appetite loss, asocial (social withdrawal)
- Psychomotor retardation
- Suicidal, somatic complaints
- Low Mood
"Think of a Robot (doesnt need to eat, doesnt need to sleep, can't enjoy things) which malfunction (poor performance status)"

MANIA: DIG + FAST (9 in all)
- Distractibility
- Indiscretion, irritable
- Grandiosity
- Flight of ideas, familiarity
- Activity
- Sleep
- Talkative (Pressure of speech)

Quick Assessment of Suicide Risks: SAD PERSONS SCORE, a 10 item assessment scale
- Sex (male)
- Age (<19>45)
- Depression or hopelessness (SAGE + CAPS + L)
- Previous attempt or psychiatric care
- Excess alcohol or drug abuse
- rational thinking loss: psychosis, organic brain disease
- Separated, divorced, widowed
- Organized plan/serious attempt
- No social support
- Sickness, chronic disease

<6: low risk
6-8: moderate risk
9-10: high risk!

Walking

There are 2 phases in walking (gait cycle)
1. Stance phase (60%)
2. Swing phase (40%)

Stance phase:
- Heel strike
- flat foot
- heel rise
- Toes off

Swing phase

The Owl Winks

Adorable isnt he?
The winking owl.

Not so adorable if you spot one on the AP X ray.

Spinal metastases.

The owl's eye is the pedicle of the spinal vertebrae.

With spinal metastasis, the pedical is obliterated by the invasive growth, resulting in its 'disappearance' and the winking owl sign. The intervertebral disc, however, is usually spared.

With further progression, the vertebral body may collapse and become wedge-shaped or flattened (Vertebra plana).

A radioisotope scan will be helpful.

Most malignant growths will show up as warm lesions on the scan, whereas myeloma will charactertistically be cold.

Description of the X ray above:

- osteosclerotic spinal metastasis 2* prostatic Ca

Common osteolytic bone mets:

- Lung

- Breast

- Thyroid

- Large bowel (Colorectal)

Common Osteosclerotic bone mets:

- lung

- breasts

- prostate

Side note:

In Paget's disease (TB bone): usually affects 2 adjacent vertebrae with narrowing of joint space.

Thoracic outlet syndrome: a syndrome of many words

interscalene triangle/scalene fissue
scaleotracheal fossa
costoclavicular triangle
subcoracoid triangle deep to the tendon of pectoralis minor

Scalenus anticus syndrome
hyperabduction syndrome
cervical rib syndrome
costoclavicular syndrome

adson's test
costoclavicular maneouvre

Paget Schroetter disease (venous TOS)
Gilliat Sumner hands (neurogenic TOS)
- atrophy of abductor pollicis brevis, hypothenar eminences and hypothenar
- sensory deficits of the ulnar nerve distribution
- expected as ulnar nerve originate from lower levels of the brachial plexi

Adson's maneuvre
Wrights test
Roo's test
Costoclavicular maneuvre

Side notes on anatomy:

Thenar muscles: 3 muscles: abductor pollicis brevis, opponens pollicis, flexor pollicis brevis, all mediam nerve
Adductor pollicis is innervated by the ulnar nerve

Hypothenar muscles: 3 muscles: flexor digiti minimi, abductor digiti minimi, opponens digiti minimi

Anatomical snuff box: extensor pollicis longus, extensor pollicis brevis, abductor pollicis longus, radial artery, scaphoid bone, radial styloid

All about elephantine blisters

What is a bullae?

- a fluid filled blister greater than 5cm in diameter

- fluid = blood, serum, pus

What is Bullous Pemphigoid (BP)?

Bullous pemphigoid is an autoimmune disease, commonly seen in the older age groups, where IgGs are produced against hemidesmosomal proteins in the subepidermal layer. They typically present with tense blisters which are either itchy or painful, commonly sparing the mucous membranes. The prognosis is more benign than its cousin (pemphigus vulgaris) and is usually Nikolsky's negative.

What is Pemphigus vulgaris (PV)?

Pemphigus vulgaris (PV) is an autoimmune disease where autoimmune antibodies IgGs are produced against the desmoglein protein. Targetting the desmosomes in the intraepidermal layer, PV generally affects the younger age groups, involves the mucous, and forms painful flaccid bullae which are Nikolsky's +ve. Prognosis is usually worse than BP.

USMLE question

A 77-year-old man has multiple, painful blisters located on his face, neck, and torso. The patient complains that he cannot stop itching them. Which additional finding would suggest a diagnosis of Pemphigus Vulgaris rather than Bullous Pemphigoid?

A. Denuded skin and flaccid blisters
B. Fever and anorexia
C. Intact skin and tense blisters
D. Mouth Lesions
E. Posthealing hyperpigmentation

The answer turned out to be A. This is true because in pemphigus vulgaris, the autoimmune disorder where immunoglobulins (IgG) are produced against proteins called desmoglein. Targetted at the desmosomes at the epidermal layer, it creates a region of weakness allowing for the skin to be denuded (assuming what they meant was the Nikolsky's test).

However, is that the only answer? I respectfully disagree. I think D is also correct. Pemphigus vulgaris characteristically involves the mucous membranes (that includes the oral cavity) while bullous pemphigoid usually spares them.

Hottie99 posted:
"mouth ulcerations are associated with pemphigus vulgarius, and the oral mucosa is spared in bullous pemphigoid, that is another difference btw the 2 options, so therefore D can't be right, the question is describing pemphigus vulgaris, not bullous pemphigoid"

hottie99 didnt quite comprehend the question. utter bollocks about D can't be right

So in conclusion:
I believe both A and D may be right. but A is more right than D cos as high as 30% of people with bullous pemphigoid may have mouth lesions. so i guess NIKOLSKY WINS!

Anyway we saw a 55 year old Chinese woman with a past medical history of uterine cancer at age 35 (huh?!) presenting with generalized abdominal pain.

On physical examination:
- jaundices (scleral icterus)
- tender hepatomegaly (unable to assess size as the ED doctors were busy assessing her and that she's reluctant to be examined due to complaints of tenderness on palpation)
- no shifting dullness

FAST (focussed assessment with sonography in trauma) showed no fluid in morrison's pouch and splenorenal angle.

This got us puzzled. So what are the causes of tender and painful hepatomegaly?
- We HAV Cardiac failure
1. Weil's disease (Leptospira. Biphasic symptoms: non specific 1st phase, hepatosplenomegaly with nephritis in the 2nd phase. Transmission: urine and even semen of infected animals like pigs and rats)
2. Hepatoma (HCC whcih could have ruptured)
3. Actinomycosis, amoebiasis, abscess
4. viral hepatitis
5. cardiac failure

During my 3 weeks of emergency medicine elective, I noticed a lot of alcoholics (coincidentally most are middle aged to elderly Indian gentlemen and ironically, some have Muslim names [disclaimer: this is a personal observation. I do not intend to discriminate against Indians and Muslims. I, instead, wish to remind the medics to consider alcohol intoxication in people from such demographic groups presenting with altered mental state, but also keeping in mind that Indians have higher risks of coronary and cerebrovascular events.

Anyway, we have certain biological markers useful in evaluating and managing an alcoholic patient.

1. GGT: gamma glutamyl transferase: rises significantly after heavy alcohol consumption for 1-2 months. requires abstinence of about one month before GGT normalizes. This is thus useful for assessing long term abstinence
2. CDT: carbohydrate deficient transferrin: like GGT, its level rises after heavy alcohol consumption, but earlier than GGT at half a month. In addition, unlike GGT, elevated CDT is more specific than elevated GGT for heavy alcohol consumption as other hepatic conditions may result in raised GGT.
3. MCV: MCV raised after heavy alcohol consumption for 1 to 2 months
4. FAEE: fatty acid ethyl esters:
5. ?thrombocytopaenia?

The Significance of Liver Function Test Results

ALP: alkaline phosphatae:
-in a child, ALP 2x adult URL (growing bones)
-in a teenager, ALP 3x adult URL (growing bones)
-older people have increased URL (osteoporesis/osteolytic mechanisms)
-Pregnant women have higher ALP due to placental production
-in normal non-pregnant people: main sources of ALP: liver and bone
-Significance of raised ALP: can’t tell if its bone or liver, best to tie in with clinical symptoms and other tests
- Raised GGT and ALP with hyperbilirubinaemia shows biliary blockage
- In the case of liver, ALP is present in the biliary epithelium

GGT: gamma glutamyltransferase
- GGT is higher in men as prostate and testes also secretes GGT
- Raised GGT and ALP = likely cholestatic condition (i.e. GGT's function is to investigate cause of raised ALP as that of biliary or extrabiliary =)
- Raised GGT = acute or chronic alcohol toxicity
- GGT as monitoring marker for long term alcohol abstinence: a month of abstinence = GGT returns to normal. This may be complicated by other liver pathologies yielding false positives.

AST and ALT:
- ALT is more specific for liver
- AST is also found in cardiac and skeletal muscles, and RBCs
- AMI and skeletal muscle diseases may result in raised AST
- elevated LDH also shows liver damage. however LDH, if you can recall, is one of the first marker enzymes for AMI

5NT: 5' nucleotidase
- the use of 5' nucleotidase is similar to that of GGT
- they serve to investigate the cause of raised ALP as that of biliary or extrabiliary

Advanced Stuff:
Discussion of AST/ALT ratio

Scenario 1: AST/ALT ratio >2, ALT
- AST/ALT ratio >2 is characteristically seen in people with alcoholic liver disease
- this relative low ALT is due to deficiency in pyridoxine 5 phosphate (P5P)
- in addition: raised GGT, MCV and Platelet count

Scenario 2: AST/ALT ratio >2, ALT > 500IU/L
- it suggests a diagnosis other than alcoholic liver disease

Scenario 3: AST/ALT <1.o
- viral hepatitis, especially hepatitis C
- the AST/ALT ratio may subsequently rise due to cirrhotic changes

AMYLASE!!!

"did you know that the organ that secretes most amylaze isnt the pancreas, but the salivary gland! (about 6:4)"

"only 75% of acute pancreatitis had hyperamylasaemia"

About amylaze
It is secreted mainly by pancreas and saliva (significantly large amounts)
2 main types of amylaze:
- P type from pancreas
- S type from salivary glands
(S type is also found in tears, breast milk, sweat, testes, fallopian tube etc. but Salivary gland = major contributor)

Clearance of amylaze:
- Renal: nephrectomy patients have 50% higher amylaze levels
- Extrarenal: currrently unknown, probably hepatic: people with hepatic necrosis have raised amylase (both S and P type)too

Causes of hyperamylasaemia can be subdivided into:
1. pancreatitis/parotitis
2. decreased metabolic clearance (liver/renal pathologies)

Discussion of common causes:
Pancreatitis:
- Causes: I GET SMASHED: idiopathic, mumps, autoimmune, scorpion, ERCP, drugs (SAND) and duodenal ulcers
- History: epigastric pain radiating to the back, relieved on sitting up and leaning forward and severe N and V
- S/S: Steatorrhea, Cullen's sign, Grey-Turner's sign
- Test: lipase, amylaze raised 4x on day of presentation (electrophoresis shows P type). Imaging (CT, A U/S)
interesting notes:
(i) 3-4x increased in amylaze 4 hours after ERCP = predictive of post procedural pancreatitis
(ii) 3x increased amylaze with biliary colic = passage of stones through bile duct

Prognostication: RANSON's criteria: GA LAW at presentation, C Hobbs (calvin and hobbs) at 48 hours
- Glucose
- Age >55
- Lipase
- AST
- WBC

- calcium

- Ht

- O2 arterial

- BUN

- Base deficit

- Sequestration of fluid

Other causes of raised amylase:
1. salivary disease
2. decreased metabolic clearance (hepatic and renal disease) - both S and P are increased
3. Macroamylasaemia (amylze binds to larger molecules to form complexes, prolonging its Thalf and reducing renal excretion)
4. Intestinal disease: P amylase is raised as intestinal diseases result in increased absorption of amylaze. in the case of perforated viscus, there is increased reabsorption of amylase via peritoneal lining.
5. Gynaecological causes
6. AAA (p type)
7. pneumonia (S type)

how to differentiate acute pancreatitis from the others?
- chronic hyperamylasaemia is almost never acute pancreatitis
- ACR = (amy[urine] x creat[serum])/(amy[serum] x creat [urine]) x 100. an ACR > 5% = pancreatitis or DKA

Sail sign on lateral elbow Xray vs sail sign on CXR:



1. Sail sign on lateral elbow X ray:

- fat pad in joint capsule, outside synovium

- fat pad usually hidden in olecranon and coronoid fossa

- intraarticular injury --> intraarticular haemorrhage --> lifts the fat pad into view resulting in sail sign



2. Sail sign on CXR:

- left lower lobe collapse

Lactic acidosis: Its more than JUST lactic acidosis

Summary of Lactic Acidosis:
Major sites of Lactate production includes the skin, muscles, brain and RBCs (Placenta in pregnant women)
In the absence of oxygen (or in the case of RBC), energy is derived from glycolysis of glucose
Lactate is produced in this process with 2 ATP
(pyruvate to lactate with NADH --> NAD+. NAD+ regeneration is important for further glycolysis)
With the reestablishment of oxygen, lactate is oxidized into water and carbon dioxide or undergoes gluconeogenesis (only liver and kidney have enzymes for gluconeogenesis)
Liver (60%) and kidneys (30%) are important metabolizers of lactate
50% are converted to glucose (gluconeogenesis) and 50% metabolized to water and carbon dioxide in the citric acid cycle

If the oxidation rxn is absent, lactate accumulates resulting in lactic acidosis:
- the balance of blood lactate depends on release into bloodstream and hepatorenal uptake
- these mechanisms maintain blood lactate at 1 mmol/L
- renal threshold for lactate is around 5-6mmol/L, i.e. if blood lactate levels exceed 5-6 mmol/L ==> lactate will be excreted in the urine

Common causes:

1. Medication: Phenformin (like metformin, it is a biguanide and causes weight loss. It is no longer in use due to lactic acidosis being its S/E) and anti-retrovirals (mitochondrial toxicity). Catecholamines and Cocaine cause lactic acidosis by increasing lactate production.
2. poisons: CO, cyanide
3. Diabetic ketoacidosis
4. Liver disease
5. Convulsions, severe exercise

Typically, they present with:
1. abdominal pain and vomiting
2. rapid and deep breathing (Kussmauls?)

Sounds like GE isnt it?

Definition:
Normal lactate ~ 1mmol/L
Hyperlactaemia ~2-5mmol/L
Severe lactic acidosis >5mmol/L (thus lactate in urine?)

Classification of Lactic acidosis:
Lactic acidosis is divided into types A and B
They differ with regards to adequacy of tissue oxygen delivery

Type A: decreased perfusion or oxygenation (anaerobic metabolism), mitochondria assumed to be normal and intact, most common clinical situation. Examples"
- severe respiratory failure
- anaemia (poor oxygen delivery)
- CO poisoning
- hypoperfusion of tissues (e.g. hypovolaemia(e.g. DKA) hypotension)
- convulsions and sprinting (anaerobic muscular activity)
- mesenteric ischaemia (compromised regional oxygen delivery)

Type B:
- B1: underlying diseases (e.g. Liver disease, renal disease, lymphoma, leukaemia, DKA, AIDS)
- B2: intoxication, medication (e.g. phenformin, anti-retroviral, catecholamines, cocaine, cyanide)
- B3: inborn errors of metabolism

Special attention is paid in the discussion of liver's role:
- the liver has immense capacities for lactate metabolism
- pathologies like sepsis, hypovolaemia, hypotension, cirrhosis, hypothermia result in decreased hepatic capacity for lactate metabolism


Notes:
Causes of HAGMA:
- uraemic acidosis
- lactic acidosis
- ketoacidosis

Why bicarbonate does not work in lactic acidosis:
- metabolic acidosis (intracellular) inhibits PFK, the RDS enzyme in glycolysis
- on administration of IV bicarb, there is intracellular metabolic alkalosis, as such removes the inhibition of PFK resulting in further lactate production

Mirriam asked me a question with regards to sodium levels in people with congestive cardiac failurei did some reading and thought the following info may be useful for those doing Gen med. The question Mirriam asked was:"in decompensated congestive cardiac failure, is the sodium status high or low?" The answer will likely be that the sodium levels are low especially in the case of decompensated cardiac failure. This answer seems to be contradictory to what we've learnt:CCF --> decreased renal perfusion --> activation of Renin-Angiotensin-Aldosterone pathway --> Aldosterone induces Na+ retention (and subsequent fluid retention) with consequent loss of K+However, other mechanisms:1. CCF --> atrial stretch --> ANP --> sodium loss 2. RAAS/peripheral baroreceptors --> posterior pituitary gland secretes ADH --> favours water retention alone3. Increase Sympathetic Nervous System Activity --> favours water retention alone These favours a significantly increased level of water retention relative to sodium retention.The end result is the patient being in a state of hyponatremia. However, the story does not end here. Hyponatremia's significance as a predictor of mortality is frequently ignored by physicians. Its a pity because recent studies by the American College of Cardiology found out that:1. 1/4 of patients with severe heart failure have hyponatremia2. people with CCF with hyponatremia are 2 times more likely to die than those without hyponatremia in a period of 60 days Perhaps we should consider ditching this ignorance as our patients will certainly benefit since:1. Sodium levels can be easily obtained with U/E/Cr2. Low sodium levels are potentially treatable (no not by fluid loading them with more normal saline, but there are drugs like Tolvaptan which may be available in the future) Anw thats my 2 cents' worthThank you for your kind attention =) Youjiang