Teaching the Nurses Abdominal Examination

Dear Bloggers

Last night was the second in my series of lectures of teaching the various elements of clinical examination to the nursing staff.

The topic of last night's meeting was the abdominal examination.

It was necessary to explain that much of what leads to differential diagnosis of abdominal disease is based on the history.

For example, with abdominal pain, was the onset acute or slow onset, the location, the quality, the severity, radiation of the pain, relieving factors, precipitating factors and exacerbating factors, etc... All these elements can be important to give a particular diagnosis more weight than other disease processes.

One of the nurses raised an excellent question with regards to why acute appendicitis typically begins as central abdominal pain and then becomes localised in the right iliac fossa. It was then necessary to explain about the difference of the nerve supply to the bowel compared to that of the abdominal wall and distension of a viscus resulting in poorly localised pain compared to direct stimulation of the parietal peritoneum resulting in localising pain respectively.

In this 2-hour session, it was not possible to cover all the elements of the history and physical examination of the abdomen, but a number of serious conditions and their clinical manifestations were covered e.g. pancreatitis with Cullen's and Grey-Turner's signs, bowel obstruction, acute cholecystitis and so on.

From the photo above, it can be seen that the 'simulated patient' and other nursing staff were asked to perform the clinical examination for encephalopathy, known as hepatic flapping tremor or asterixis.

Thanks to Yuka-san for such great translating to the nursing staff !

Have a good day.... :-)

Ways to Identify Jugular Venous Distension

Dear Bloggers

I often get asked the question about how to identify the internal jugular vein for measuring the Jugular Venous Distension (pressure).

It is sometimes not so easy to be able to identify by just positioning the patient.

Interestingly, in some US based examination texts, they mention that the patient should be positioned at 30 degrees. This concept is contrary to the traditonal way of checking the JVP which is at 45 degrees-- the UK standard.

Hence, with the patient at 45 degrees, they should turn their head to the left hand side thereby exposing the two heads of the sternocleidomastoid muscles (sternal head and clavicular head). The internal jugular vein should be seen to run between these two heads in an upward and anteroposterior incline.

The normal JVP is no more than 4cm H2O. This is measured in a vertical direction from the manubriosternal joint to the maximum height of the jugular venous pulse.

An estimated height is adequate because the most important thing is to identify if it is raised or normal i.e. just visible.

There are several different wave forms to the JVP, which although are described in the traditional physical examination textbooks, in everyday practise, only a few of them are clinically helpful.

If one is unable to idenfity the JVP by position at 45 degrees alone, then if the patient has no abdominal pain, try pressing on the liver and watch for a rise in the JVP. This is the hepatojugular reflex.

Moreover, the JVP has a double pulse compared to the carotid that has just one.

By positioning the patient vertically, the JVP should disappear (unless severe venous obstruction) whereas the carotid pulse does not. Conversely, by lying the patient flat, the JVP should then increase.

The JVP may sometimes be missed by the inexperienced eye particularly if the patient has a large neck with subcutaneous fascia. One other sign is to look for movement of the earlobe. With severely elevated JVP, with the large upstroke of blood in the internal jugular vein, it causes the earlobe, beneath which it runs, to move!

Why is the JVP important at all??

JVP is important because with the right clinical history, it may aid in the diagnosis of heart failure, atrial fibrillation, tricuspid regurgitation, cor pulmonale, PE, tamponade, iatrogenic fluid overload etc... Although it is relatively non-specific for the above conditions, it nevertheless gives the physician some idea that there is pathology occuring that requires further investigation.

For example, in a patient with slowly increasing breathlessness, dyspnoea on exertion and at rest, plus the complaint of bilateral leg swelling, the physical examination looking for the raised JVP and lung crackles would make one consider at least the common diagnosis of heart failure.

Conversely, sudden on onset of dypnoea, chest pain, haemoptysis, unilateral leg pain with an examination of a tachycardia, raised JVP and a clear chest would make most competent physicians consider a large pulmonary embolism.

I hope this helps!

Why Are The Hands So Important?

Dear Bloggers

Today I want to go over why I think the physical examination of the hands is such an important part of the general physical examination.

Without a good look at the hands, one can sometimes miss the diagnosis that might otherwise take a long time to make via other means.

After standing at the end of the bed to observe the general appearance of the patient, I advise to then look at the hands.

Check the nails-- these are like the looking glass into the body. Digital Clubbing is a great sign to find and can help to narrow your differential diagnosis significantly. Please see my previous posting on this topic of clubbing here.

One can also see the arrest of nail growth referred to a Beau's Lines, which are horizontal indentations in the nails. One can accurately date the onset of serious illness by measuring the distance in millimeters from the Beau line to the edge of the nail fold. The nail grows approximately 0.1 mm / day. Hence, for example, a distance of 5mm would equate to onset of illness dating back 50 days or just under 2 months.

Muehrcke's Lines are paired white lines without indentation that are seen in the nail in patients with hypoalbuminaemia, post-chemotherapy etc.

Splinter haemorrhages, which are outlined in my previous blog entry linked above, are a fascinating phenomenon which may indicate infective endocarditis. They are not a pathognomic feature because they can also be caused by trauma to the nail. Hence, if you see them, don't just consider I.E. Ask the patient if they do gardening or some other hobby or employment that exposes them to nail trauma. However, more than 6 is significant for considering I.E.

Koilonychia (spoon nails) is the famous sign of iron deficiency anemia. However, never forget to look at the corners of the mouth for red, painful areas which is known as angular cheilosis. The tongue may be affected by atrophic glossitis (inflamed). Problems with swallowing should alert you to the rare post-cricoid web which can become cancerous. The presence of the latter with IDA is referred to in the UK as the Plummer-Vinson Syndrome and in the USA as the Patterson-Kelly-Brown Syndrome. Both identical syndromes were described at approximately the same time in the two English speaking continents!

Looking at the nail folds is very important. Sometimes, nail fold infarcts can be visualised as is the case in rheumatiod vasculitis and several other vasculitic disorders. Moreover, if one looks closely at the nailfold proper, seeing capillary loops may signify systemic lupus erythematosis (SLE)! Yes, just from looking at the nailfold!

As I documented last week, Quinke sign can be found in the nail in the area of the interface between the white and red areas of the nail. It can also be found in the skin-- ? B−sign :-)

Nails can also be affected by psoriasis and take on several features including:

• Subungual hyperkeratosis (thickening of the nail)
• Onycholysis (lifting off of the nail)
• Nail pitting (looks like the indentations on the surface of a thimble)
• Nail ridging

In the UK exams, they may sometimes just show the doctor the patient's finger nails and ask for the diagnosis without showing the patient's skin. Psoriasis would be the right answer.

Looking at the tips of the fingers can reveal the tender Osler Nodes (small lymph nodes) of I.E.

One might also be able to identify the skin colour changes of Raynaud's Phenomenon.

Tightening of the skin of the fingers is a sign of possible systemic sclerosis.

Of course checking for diffuse synovial swelling, joint swelling, joint pain and deformation is important and part of the rheumatological examination and diagnoses of RA, osteoarthritis, gout, etc, can be made in addition to the unusual Complex Regional Pain Syndrome which can mimick rheumatic disease.

Looking at the palm of the hand may reveal thickening and shortening of the 4th and 5th tendons that one sees with Dupytren's contracture, the cause of which is usually due to alcoholic liver disease, although other causes include

• Use of heavy, vibrating machinery e.g. drilling tools
• Peyronie's disease
• AIDS
• Epilepsy (due to drug treatment)
  

The palm may also reveal Janeway lesions of I.E., and hence, this is another very important place to observe for signs of this serious infection.

Palmar erythema (red palms) is another helpful sign as it may signify one for the following

• Liver disease
• Thyrotoxicosis
• Rheumatoid arthritiis
• Pregnancy (the distended abdomen usually gives you a better idea :-) )

Checking the lines in the palms may reveal hyperpigmentation which is consistent with increased output of ACTH e.g. Addison's disease, ACTH secreting tumour; obviously, this depends upon the race of the patient as it is easier to identify in lighter skinned individuals.

Ask the patient extend their arms and fully extend the palms with open fingers to look for the sign of asterixis (flapping tremor) that one can observe in

• Hepatic encephalopathy
• CO2 retention
• Uraemia

Checking for muscle loss in the hand e.g. thenar eminence, may give you a clue about median nerve impairment whether it be an entrapment in the carpal tunnel (check Phalen's and Tinel's tests for that) or higher up in the arm / cervical area.

Checking the muscles on the dorsum of the hand (Dorsal interossei) can signify a motor neuropathy, myopathy, or malnutrition if bilateral and diffuse. However, be warned, unilateral dorsal interosseus muscle wasting in a smoker can signify the presence of a Pancoast tumour.

Other neurological signs can be found in the hand which include the fine tremor of thyrotoxicosis, the pill-rolling tremor and cog-wheel rigidity of the wrist in Parkinson's disease. Hoffman's Sign, flicking the nail of the middle finger in a downward motion leads to the flexion of the index finger and thumb, which signifies an upper motor neurone lesion.

The last and widely neglected part of the physical examination is taking the radial pulse. This is truly essential. One must assess the heart rate, rhythm, and volume quality. The first two are relatively easy to understand. The third essential element requires experience in understanding the normal pulse and being able to understand the abnormal.

The patient with a high bounding pulse which collapses may well have aortic regurgitation or a vascular shunt e.g. portocaval anastomosis in liver failure, dialysis shunt. On the other hand, the pulse may be slow to rise which signifies advanced aortic stenosis.
A jerky pulse may signify hypertrophic cardiomyopathy.
The low volume pulse can be found in patients with poor cardiac output, hypovolaemia e.g. vascoconstriction, and is described as thready.

Hence, as can be appreciated, there is a vast amount of information that can be gleaned from examining the hands.

For a more in depth explanation and to see pictures of the examples I have given above, please see a good physical examination book. I would highly recommend MacLeod's Clinical Examination, which has 424 pages with detailed colour illustrations and is published by Churchill-Livingston which is obtainable from Amazon or other good book sellers. I used an earlier edition for aiding my learning of physical examination as a student, and I consider it as an excellent book for this medical art. In my opinion, it is better than the various 'famous' USA physical examination books that are promoted in Japan.

Hence, please start looking at the hands. You will be amazed what you may find.

Hokkaido and beverages to boot!

Dear Bloggers

I recently returned to Hokkaido to teach the junior residents in an excellent local hospital.

Most of the residents I have worked closely with previously and it was a delight to be able to impart some further knowledge based on hearing the histories and seeing the physical examination of various patients.

Below are some photos of the residents and nursing staff attending the rounds.

Later in the evening, the hospital team and myself dined out locally to try some cuisine in an old converted red brick warehouse which housed a famous German-style beer restaurant. You can see the red brick behind ! Ahhh, reminds me of England..... As can be seen, when drinking from a glass boot, when one wants to get the last drop of beer, it can prove quite a challenge.

Here is the original boot of beer. It was my first time to see such a glass....!

One the other hand, for nostalgia purposes, I was duty bound to have my own country's brew of the famous Guiness, otherwise known as Black Gold :-) or Genius!

Following such a lovely gathering of like minded doctors and the free flow of beverages, we went to a famous Mountain to observe what is regarded as one of the most beautiful night views in the world. I would agree whole heartedly. What a great night !

The next day, up bright and breezy, albeit slightly worse for wear, it was time to teach the residents about how to use the traditional opthalomoscope.

Here, the 1st year doctor is looking for the 'red reflex'

Here the doctor is trying to identify the position of the optic disc.

Opthalmoscopy is best performed in a darkened room with the patient's pupils dilated with tropicamide

In this patient, the doctor was able to identify bilateral dense opacities in the lenses consistent with cataracts.

I am looking forwards to going back again.... :-)

Quinke Sign in the Skin !!!!

Dear Bloggers

Sorry for keeping you waiting for new updates this week.

Here is a patient from with ankylosing spondylitis who was noticed to have pulsating carotid arteries in the neck.

Examination revealed a high upstroke volume of the radial pulse followed by a collapsing quality. The carotid pulsation was the classical Corrigan Sign.

Cardiac examination revealed a low grade systolic murmur at the aortic area with radiation to the carotid arteries in the neck. There was no audible diastolic murmur.

Femoral bruits were heard and the popliteal arteries were also palpable (in normal individuals it is unusual to feel the popliteal arteries unless hyperdynamic circulation or popliteal aneurysms are present).

As can be seen from the video below, Quinke Sign is strongly positive. Usually, one sees Quinke sign in the nail bed as a change in the redness that surrounds the white part of the nail near to the nail fold. It is a bit like watching the 'tide come in and out' in the nail. However, in this patient, the regurgitation was so strong, this change in blood flow pattern could be seen in the skin !!!

This patient had suspected Aortic Regurgitation. 

Ankylosing spondylitis is associated with aortic root dilatation and may precipitate aortic regurgitation. Other causes of aortic dilatation include:

• Marfan's Syndrome
• Reiter's Syndrome
• Atherosclerosis
• Syphilis (aortitis)
• Hypertension

Causes of Aortic Regurgitation not associated with dilatation include:

• Rheumatic heart disease
• Infective Endocarditis
• Trauma
• Bicuspid valves
• Disproportionate cusps

As a first year doctor in the UK, I first found Quinke sign in an elderly lady with accelerated hypertension who had a wide-pulse pressure. On the ward round the next morning I mentioned to the consultant that the patient had Quinke sign. The consultant boastfully said that he had never seen it-- until he indeed checked the patient and confirmed that it was indeed Quinke sign!!

Just because you may have not seen a sign does not mean that it does not exist anymore or is somehow obsolete. I have seen Quinke sign over a dozen times or more in my career by just spending the time to look for the sign when considering the diagnosis of Aortic Regurgitation.

Things to think about at the bedside to suspect the diagnosis include:

1. Wide Pulse Pressure
2. Collapsing pulse and Water Hammer pulse
3. Quinke Sign
4. Corrigan Sign 
5. De Mussett's Sign (head nodding with the cardiac cycle)
6. Femoral Bruits 'Pistol Shot'-type
7. Popliteal pulses being palpable
8. Aortic ejection systolic flow murmur
9. Diastolic flow murmur

Have a good day!

Headache and Sensory Changes - History Reveals All !!

Dear Bloggers

This next case has been anonymised for safeguarding patient confidentiality.

In a recent case at a distant hospital in Japan, a young patient was admitted with a headache and sensory changes down the left side of her body.

The headache had occurred in the morning and had woken the patient from sleep. She had severe pain and was unable to stand. She then noticed that she had sensory changes down the left side of her body although her limb movements appeared to be unimpaired. 

She mentioned to the junior resident that she had no previous medical history of note and was taking no regular medications.

There was no history of thrombotic diathesis, no symptoms of SLE, no pregnancy or consumption of oestrogens and no smoking history. She had no subjective fever and had no preceding infective symptoms. She complained of no neck stiffness or photophobia. She had not had any preceding sexual intercourse prior to the onset of symptoms (n.b. sexual intercourse is a classic history before SAH). There was  no chest pain or interscapular pain. She complained of no bone / vertebral pain and the headache was not worsened by subjective head or neck movement.

On examination (when seen by the resident), there were focal problems on general physical examination.

Neurological examination revealed left sided cerebellar signs of slowed irregular movement when performing finger-to-nose examination, mild nystagmus to the left side and dysarthria. There was sensory loss including modalities of light touch and nociception on the same side.

There was no sensory loss involving the face, and cranial nerves were otherwise normal.

The differential diagnoses before cranial imaging included:

1. Stroke (infarction or bleed) causing a Wallenberg Syndrome (Lateral Medullary Infarction)
   
2. Encephalitis
   
3. Meningitis
   
4. Transverse Myelitis
   

After several hours, the patient had had several studies including CSF examination and a cranial MRI scan.

The patient was then seen by a senior physician who wanted more history!!

The patient first mentioned the headache. The senior physician asked the patient to explain the quality of the pain. The patient replied that it was a throbbing type pain. Such throbbing is generally inconsistent with any of the above causes. 

The next question was whether the pain was unilateral or bilateral. The patient mentioned that it was predominantly unilateral.

The physician then asked if the patient had a 'history of migraines'. The patient said-- YES!

The next question was- 'Is this the same pain as with previous migraine attacks?' -- YES!

Why the patient had not mentioned migraine to the resident doctor is anyone's guess, but it is clear that the diagnosis of migraine had not be entertained because the quality of the headache had not initially been appreciated. Pain in the head is not simply pain. Pain has many different qualities e.g throbbing, lancinating, pressure-type, superficial, deep etc. However, in a young patient, throbbing pain would make one consider migraine whereas in an elderly patient, temporal arteritis might be the cause.

Some patients do not remember their medical history in detail and may have infrequent migraine episodes such that they do not think to mention it to the doctor. The fact that the patient had a headache should always make the physician consider whether migraine is a possible cause. 

Basilar-Type Migraine can cause headache and peripheral sensory problems such as this.

The patient recanted the previous migrainous episodes and there had always be a visual aura. However, on this occasion the patient had awoken with the symptoms and hence, no aura had occurred. However, migraines classically can occur upon waking and may even wake the patient from sleep!

On physical examination, the cerebellar signs were still mildly present but the sensory deficits had completely resolved which was not consistent with an infarction (unless it was TIA) , bleed or infective cause, but it was more consistent with basilar type migraine.

The MRI scan was normal as was the CSF examination.

The patient was given simple pain relief (acetaminophen) but not anti-migraine treatment, as in basilar-type migraine such abortive therapies can be associated with infarction and are best omitted. Symptoms entirely resolved and the patient was discharged home on the same day.

When asking about pain one needs to know several things which include:

• Location
  
• Quality
  
• Severity
  
• Onset (sudden or slow onset)
  
• Causative, relieving or exacerbating factors
  
• Radiation
  
• Duration
• Associated symptoms
  

There is a medical mnemonic to help with this as follows:

COLD RAP TAPE

•C- Character e.g. What is it like?

•O- Onset e.g. When did it start?

•L- Location e.g. Where did you notice it?

•D- Duration e.g. How long does it last?

•R- Relieving factor e.g. What makes it better?

•A- Aggravating factors e.g. What makes it worse?

•P- Precipitating factors e.g. What brings it on?

•T- Therapy e.g. What have you tried to make it better?

•A- Associated symptoms e.g. Do you have any other symptoms along with this?

•P- Past medical history e.g. Have you ever had anything like this before?

•E- Emotional impact e.g. what concerns do you have about this and how it may affect your life

The work up of the patient by the resident was entirely correct to rule out severe pathologies and I fully support that approach. If the patient were to have further episodes of Basilar-Type Migraine, the patient should not have further investigations unless there are unusual features e.g. neck stiffness, fever, persistent symptoms i.e. failure to resolve, because such investigations are unlikely to be revealing.

For further reading on this neurological condition, please see UpToDate or any good medical text.

As for the actual diagnosis, this was reached within 4 questions by taking more history.

Please consider asking about migraine when you see a patient. Enquire about visual symptoms, cluster-type headaches, worsening of headaches with neck movement, laterality of pain, pain quality, severity, associated symptoms etc, as listed above. These features will help you discriminate the likely cause and guide you with further tests and therapeutics.

Please consider....

Bedside Better Than MRI

Dear Bloggers

There is too much reliance on machinery to try and help us as physicians make a diagnosis. 

The next case, which as always has been anonymised, demonstrates that a simple history and bedside examination was all that was needed to establish a firm diagnosis.

A 70 year old female was admitted to a hospital in North Japan with dizziness on standing, weakness of her legs and blurring of her vision.

She had been otherwise well one week before admission and had been started on hypertensive medication by a local doctor for newly diagnosed hypertension. The patient had been taking the drug according to directions. However, she began to experience dizziness on standing and leg weakness. All symptoms resolved when she was lying flat at night time. She denied collapse or loss of consciousness and there was no history of chest pain. However, when she stood up, she did experience a rapid heart beat but without chest discomfort.

There was no previous medical history of note.

She was a non-smoker and only drank occasional alcohol. She lived with her husband in a ground floor apartment.

On examination the patient looked well and was alert and fully conversant.

General: Afebrile. No jaundice, anaemia, clubbing, cyanosis, oedema or lymphadenopathy (JACCOL)

CVS: Pulse 72 regular, BP 190/90 (lying), JVP not elevated, Heart sounds S1 normal, loud S2. No added sounds or murmurs. No carotid bruits. No peripheral oedema.

RESP: RR 16/min, Sats 98% breathing ambient room air, trachea central, expansion  normal, percussion resonant bilaterally, auscaultation normal vesicular breath sounds.

ABDO: Soft, flat, non-tender, no rebound or guarding. No organomegaly or masses. Bowel sounds normal. Rectal exam normal and no faecal occult blood.

CNS- Cranial nerves II-XII within normal limits.

PNS- tone, power, reflexes, coordination, sensation all within normal limits.

The resident was uncertain of the cause of the symptoms and the patient underwent several tests.

ECG- revealed slight sinusoidal T wave abnormalities in the lateral chest leads but there was no ST elevation or depression.

Lab Data- revealed elevated liver function with AST and ALT being 3x normal. The bilirubin and ALP were normal. All other blood results were normal including the CBC, Renal function and Cardiac enzymes (CK and Troponin-T).

CXR- was within normal limits.

CT head scan was performed which revealed only age-related cerebral atrophy.

MRI head scan again revealed no pathological problem.

In this case, the history is extremely important. The patient had started a new anti-hypertensive drug and had begun to develop what appeared to be postural-related symptoms which resolved on lying flat.

Unfortunately, the history had not been fully appreciated by the resident. Despite a normal neurological examination, the patient still underwent cranial scanning, twice, which was unnecessary.

An astute physician reviewed the patient at the bedside and obtained further history which again supported the likely diagnosis of postural hypotension. A simple bedside test was performed which gave the diagnosis.

Initially, the patient was laid flat and the blood pressure was 193/92mmHg. The patient was then elevated to about 50 degrees sitting and at one minute the blood pressure was rechecked and was 189/87mmHg and there were no symptoms. At two minutes with still no symptoms present, the blood pressure was 180/79mmHg. By three minutes, the patient was developing blurred vision and the blood pressure was 178/77mmHg. It was decided to stand the patient with her being held on either side by a physician and with a third physician checking the blood pressure. Full resuscitation equipment was available in case of collapse.

On standing, the patient developed the full set of dizziness symptoms but was fully conversant with no loss of consciousness. The blood pressure at one minute was 150/69mmHg. Pulse rate had not risen appreciably.

On lying the patient flat, the symptoms completely resolved.

Hence, this patient had confirmed postural (orthostatic) hypotension likely to be drug-induced.

A blood pressure drop of more than 20 mmHg of the systolic and / or 10 mmHg of the diastolic (20/10 mmHg) are consistent with the diagnosis of postural hypotension.

Other problems to consider are autonomic failure (pure autonomic failure, diabetes, multisystem atrophy, Parkinson's disease), hypovolaemia (e.g. dehydration, haemorrhage etc), adrenal failure, pituitary failure, baroreceptor dysfunction etc.

However, with such a strong history of starting a new drug and developing postural hypotension, the drug was the likely offender and in addition, it may well have been the cause of the mild liver dysfunction.

In this case, a simple bedside test would have sufficed to make the diagnosis. Cranial scanning was not necessary especially as the thorough neurological examination was within normal parameters.

You may be saying to yourself, 'Hang on a minute, the blood pressure is still high but the patient has hypotensive symptoms?!' Yes, that is correct. This patient despite not having what we would all regard as true hypotension nevertheless has a sufficient drop in both systolic and diastolic blood pressure on standing to cause symptoms. This is a relative hypotension and is still relevant and should not be ignored just because the systolic blood pressure remains above 100mmHg !

Normal Blood Pressure Homeostasis

When initially standing up, approximately 0.5-1.0 litres of venous blood pools in the capacitance vessels. Baroreceptors sense a drop in blood pressure and result in reflex vasoconstriction, increased heart rate and return of venous blood to the heart. These effects are immediate and without appreciable symptoms to a normal individual. Longer  term changes include increased renin output to increase angiotensin II and aldosterone to increase vessel tone and sodium reabsorption respectively. Other changes include the increased output of ADH which leads to vasoconstriction and increased water absorption from the kidneys.

Pathological Causes

Hypovolaemia- in states of severe hypovolaemia e.g. bleeding, approximately 3o% of the blood volume can be lost before postural blood pressure drops can be seen. This is especially relevant in young patients. Hence, asking questions about bleeding and searching for possible unseen causes e.g. abrutio placenta, GI haemorrhage are mandatory.

Drugs- the elderly are very sensitive to drugs. Even seemingly innocuous drugs can have adverse side effects such thiazide diuretics, tricyclic antidepressants, beta-blockers, neuroleptic medications. As I always say, take a decent drug history and look up the side-effects (if you don't already know them) and check for drug-drug interactions!

Adrenal Failure- steroids are required to allow the vascular smooth muscle to be responsive to circulating catecholamines to maintain vascular tone. Hypocortisolaemia leads to vascular collapse and hence, patients with no obvious hypovolaemia and no offending drugs, should have this easily treatable condition ruled out. Other possible signs might be a low sodium and high potassium in addition to fasting hypoglycaemia. However, patients with a normal K level can still have hypocortisolaemia.

Pituitary Failure- Absence of ACTH leads to a similar problem of hypocortisolaemia. Whereas adrenal failure is associated with raised ACTH levels and hyperpigmentation, panhypopituitarism, and hence low ACTH output, is not. Checking anterior pituitary hormones would be advisable and should be part of the work-up for patients in whom the immediate cause of postural hypotension is not clear.

Other conditions are associated with autonomic failure and include diabetes mellitus, and hence, checking a fasting glucose and a 75 gram oral glucose tolerance test would be appropriate. HbA1c is not part of the British or American Diabetes Association guidelines for making the diagnosis of diabetes as it can be misleading.

Multi-System Atrophy (MSA) which includes the famous Shy-Drager and Parkinsonism etc should be considered but such abnormalities of cerebellar dysfunction, tremor, bradykinesia and cog-wheel rigidity should be unmasked during the physical examination (if performed correctly!) 

Autonomic failure can be examined for by doing the postural blood pressure testing and checking compensatory changes in heart rate. If the heart rate fails to increase by more than 10  beats per minute during the postural test, then this suggests autonomic failure. Heart rate increase > 100 / min suggests hypovolaemia. If symptoms develop without hypotension, then this suggests Postural Orthostatic Tachycardia Syndrome (POTS); this is usually evident in younger patients.

Investigations for autonomic failure include the measuring of the R-R interval on ECG with the patient doing slow breathing. The ratio of the R-R interval of expiration to inspiration should be more than 1.15. If less, it suggests autonomic failure.

Baroreceptor dysfunction paradoxically occurs in patients with hypertension which thereby predisposes elderly patients to orthostatic hypotension. The commonest cause in the elderly is decreased baroreceptor responsiveness plus impaired arterial constriction.

Treatment

1. This involved removing / treating the underlying cause
2. Postural manoeuvres such as getting out of bed slowly in the morning, taking sufficient fluid and salt in the diet (as long as their is no hypertension or heart failure), using compression stockings to improve venous return, addition of fludrocortisone to mimimise symptoms in those individuals with sufficient salt intake, midodrine to cause vasoconstriction and non-selective beta-blocker Propranolol which leads to uninhibited alpha-vasoconstrictive effects.

Lessons to be Learned

• Avoid cranial scanning unless it is absolutely necessary; just because you can scan someone does not mean that you have to or that you should. A thorough history and examination may be sufficient to give all the necessary information. Only if there is a neurological abnormality e.g. upper motor neurone signs, should the patient be scanned.

• Patients with a history of postural related symptoms should have a bedside measurement of blood pressure. An initial lying blood pressure should be performed and then repeating the measurements at 1, 2 and 3 minutes in the sitting position (if the patient cannot stand) or standing. If the patient is stood up, they should be supported in case of potential collapse with full resuscitation equipment being available. Blood pressure measurements should then be performed at 1, 2 and 3 minutes.  

• A blood pressure drop of more than 20 mmHg of the systolic and / or 10 mmHg of the diastolic (20/10 mmHg) is consistent with the diagnosis of postural hypotension.

 In this case, there was a hint of dropping blood pressure at 3 minutes when sitting and this  was confirmed after just 1 minutes of standing.

• Patients without a supportive drug history and in whom there is no clear cut cause, should have a rectal examination to check for gastrointestinal haemorrhage. Consider other potential causes in the elderly as listed above.

Have a great week !

One of the most difficult cases yet-- the answers!!!

Dear Bloggers

Thank you for waiting for the answers to this very challenging case. The doctors involved here thought of a restricted differential diagnosis from the beginning having considered various underlying causes, they undertook selective testing and had the confirmation of the eventual diagnoses within one week of the patient being admitted to the hospital. 

No CT scan was ever performed to make the diagnosis.

Questions: 

1) From the history and physical examination, please make a problem list.

• Fever
  
  
• Cough
  
  
• Fatigue & Malaise & Appetite Loss
  
  
• Numbness and pain in the right hand
  
  
• Pain in the proximal lower limbs and buttock pain
  
  
• Previous left sided weakness
  
  
• Bilateral chest pain on pressing the chest
  
  
• Left proximal pain on lateral aspect of leg
  
  
• Painful springing of the posterior pelvis
  
  
• Weakness of long and short flexors of the hand, sensory loss affecting the distribution of median and ulnar nerves in right hand. Weakness of adductor muscles of the hand.
  
  
• Arthralgia of MCPs and PIPs of right hand.
  
  
• Antalgic gait with dragging of the left foot
  
  

2) What are the possible differential diagnoses in this case?

This case is somewhat difficult and has a wide set of differential diagnoses. The fact that the patient had a fever for 6 weeks and he did not respond to antibiotic treatment makes a simple infection unlikely. The fact that the patient was reviewed several times at a clinic, and one would hope that basic investigations were performed, makes this a likely Fever of Unknown Origin (FUO)

Infection

Bacterial

• Bacterial endocarditis-  this can cause a generalised vasculitis, fever, malaise, fatigue etc
  
• Osteomyelitis (this would not explain the neurological impairment)
• Tuberculosis (this would not explain the neurological symptoms). If this patient had been living in a high risk area for leprosy (mycobacterium leprae), it might explain the neuropathy but not the cough and chest pain. 
  

Viral

• Parvovirus B19 can cause an upper respiratory infection, constitutional symptoms and joint involvement but cannot explain the neurological impairment. Other viruses that are
   known to cause neuropathies include Varicella zoster (zoster sine herpete) and HIV.
  
  

Neoplastic

• Primary bronchogenic carcinoma with a paraneoplastic neuropathy
  
• Metastatic disease ( affecting chest, bone and causing a paraneoplastic neuropathy
  
• Multiple myeloma (causing bone pain, amyloid induced neuropathy and immune
   suppression with subsequent infection)
  
  

Haematologic

• Lymphoma / Leukaemia with paraneoplastic neuropathy
  

Connective Tissue Disease

• Systemic Lupus Erythematosis
  
• Rheumatoid vasculitis
  
• Wegener’s Granulomatosis (fever, malaise, cough, neuropathy, arthralgia)
  
• Microscopic Polyangiitis (fever, malaise, neuropathy, arthralgia)
  
• Polyarteritis nodosum

Immune

• Guillain-Barre Syndrome – less likely as usually begins in feet and spreads proximally with bilateral weakness.
  

Toxins / Metabolic

• Alcohol (common cause of peripheral neuropathy but usually not so profound) 
  
• Vitamin B6 / B12 deficiency
  
• Lead poisoning

3) What tests would you undertake to investigate this patient's problem including both simple and advanced tests?

Various tests should be performed and include:

• CBC, BUN, Creatinine, Na, K, Liver function, Coagulation, ESR
  
• Urine analysis
  
• Blood, urine and sputum culture
  
• Blood smear
  
• Serum electrophoresis and Bence Jones protein
  
• Autoimmune screen: RhF, ANA, ANCA (MPO and PR3), complement
  
• Thyroid hormones
  
• Xrays of hands, chest lumbar spine and pelvis
  
• Bone marrow examination
  
• Isotopic bone scan
  
• Nerve conduction and electromyographic (EMG) studies 
  

4) Give your top three diagnoses.

• Malignancy e.g. multiple myeloma, metastatic disease
  
• Infection Bacterial endocarditis / osteomyelitis / UTI with disseminated infection
  
• Autoimmune disease e.g. Wegener’s Granulomatosis, Microscopic polyangiitis, polyarteritis nodosum
  

In this case,  the patient underwent several tests. On admission, his haemoglobin was 8.1 with a normal MCV. BUN  and Creatinine were abnormal at 28.5 and 1.4 respectively with normal sodium and potassium levels. Liver function was normal.

Calcium was elevated at 11.6 (corrected).

Urine examination revealed >100 wbc/hpf, 30-49 red cells/hpf, 1+ protein, no evidence of casts and 3+ bacteria.

Initial urine analysis therefore suggested a possible UTI.

From the history, physical examination and basic blood tests, multiple myeloma, bacterial endocarditis, metastatic disease and autoimmune disease were possible diagnoses.

The chest X-ray is below:

 

In view of the chest pain and raised calcium, an isotope bone scan was performed which shows some ‘hot spots’ at the costochondral junctions. The pelvic views show increased uptake at the sacroiliac joints suggesting a possible sacroiliitis.

Abdominal ultrasonography revealed bilateral renal stones only. No other focal abnormality was identified.

Lower limb doppler scans ruled out deep venous thrombosis.

The PTH result was 102 (10-65) suggesting a primary hyperparathyroidism. PTHrP was negative. Vitamin D level was mildly low at 19 with normal above 20.

Neck ultrasonography revealed a  4x4mm mass in the right upper pole of the thyroid.

ESR >100mm/hr

ANA was negative with a positive RhF of 114 (<15).

PR3-ANCA <10

Serum electrophoresis and Bence Jones protein studies refuted the diagnosis of Multiple Myeloma.

MPO-ANCA 546 (<20)

IL2-receptor 1758 (190-650)

Thyroid tests: T3 1.7 (decreased), T4 1.1 (0.8-1.9) TSH 0.8 (within normal limits)

Blood and urine cultures negative

Transthoracic echocardiography revealed no vegetation

MRI scan of the lower vertebral column revealed no metastatic disease or any area consistent with multiple myeloma.

This patient had non-specific symptoms of malaise, fatigue and appetite loss. The fact that both motor and sensory modalities were affected in one hand affecting the distribution of two peripheral nerves i.e. the ulnar and median nerves, makes one consider a distal motor-sensory polyneuropathy; with the addition of arthralgia makes a connective tissue disease much higher as the cause. 

Wegener’s granulomatosis and microscopic polyangiitis would be regarded as the main two diagnoses here, as both can cause neuropathy and are associated with a rise in the serum ANCA. However, 90% of Wegener’s patients have PR3 ANCA versus 10% who have MPO ANCA positivity. The history of a non productive cough makes one consider Wegener’s Granulomatosis which affects both upper and lower respiratory tracts. However, there were none of the very classical upper respiratory features of the disease which can affect the nose, ear etc.

50% of patients with microscopic polyangiitis develop pulmonary disease and this is usually a pulmonary fibrosis.

The arthralgia is a non-specific symptom and can be present in infective, malignant and connective tissue disease but with the above history makes it more likely to be a connective tissue cause.

The majority of patients with microscopic polyangiitis have MPO-ANCA serum positivity and therefore, it was considered to be the most likely underlying diagnosis. Both Wegener’s Granulomatosis and Microscopic polyangiitis can cause renal impairment and hence, a renal biopsy was performed to investigate the underlying cause. The fact that the patient had no casts in the urine but high WBCs and RBCs plus bacteria suggested a UTI rather than a glomerulonephritis-- a lesson learned!

The fact that the patient had lateral leg pain with no joint or nerve stretch signs either suggests a localized myopathy, bone pain or a localized peripheral neuropathy such as a meralgia paraesthetica. However, the bone scan did not reveal bone uptake making a myopathy or neuropathy more likely.

The painful buttocks were likely to be a sacroiliitis as noted by the physical examination and the abnormal uptake in the sacroiliac joints on the bone scan. However, Xray of the sacroiliac joints revealed no obvious abnormality.

The renal biopsy showed: crescentic glomerulonephritis with 50% of the nephrons being involved. Immune staining was consistent with the diagnosis of microscopic polyangiitis.

Likely diagnosis: Microscopic Polyangiitis 

Treatment with pulsed methylprednisolone was started and there was a dramatic drop in fever to the normal range with the patient feeling much better. However, the renal function deteriorated with a creatinine rise to over 3mg/dl in just one week prior to commencing this treatment. Moreover, despite pulse steroids, there was no immediate return of motor function in the affected upper extremity. This was not surprising in view of the prolonged duration it takes peripheral nerves to recover function. Electrophysiological studies were ordered to investigate the likely underlying inflammatory neuropathy.

The patient also underwent neck ultrasonography plus MIBG scanning of the neck.

The underlying diagnoses include:

1) Microscopic polyangiitis  presenting with

• Fever, malaise, fatigue and appetite loss
  
• Motor-sensory polyneuropathy
  
• Arthralgia
  
• Possible meralgia paraesthetica
  
• Anaemia of chronic disease 
• Crescentic glomerulonephritis 
• Positive MPO-ANCA

2) Primary Hyperparathyroidism presenting with 

• Hypercalcaemia
  
• Bilateral rib pain and increased uptake on bone scan
  
• Bilateral renal stones on ultrasound.
  
• Mass in upper pole of right lobe of the thyroid

3) Sick Euthyroid state

Professor Masami Matsumura, Department of Internal Medicine, Kanazawa University Graduate School of Medicine, has again kindly answered the case. 

His answer is frankly amazing and just from the history and examination!

"Thank you very much for showing challenging case! This case is difficult to diagnose, but challenging again.

This patient is previous healthy 61-year-old Japanese man. Patient first symptom was six-week history of cough. The patient thought that this was due to a common cold. Next fever appeared. From these information, I would point out possibilities of autoimmune, infection, and neoplastic diseases. Fatigue and malaise are not high yield symptoms. However, numbness and pain in the right hand, pain in the proximal lower limbs and buttock, and dragging of the left leg appeared. Mono-neuritis multiplex is highly suspected in this case. If physical examination showed findings of mono-neuritis multiplex, vasculitis is most likely.

Questions

1 From the history and physical examination, please make a problem list.

I listed problems as follows;

#1 Cough

#2 Fever

#3 Fatigue and Malaise

#4 Pain in the proximal lower limbs (L5, S1-2)

#5 Buttock pain (L5, S1-2)

#6 Dragging of the left leg

#7 Left sided weakness at age of 30 years

#8 Tachycardia

#9 Obesity, BMI 38.2

#10 Tenderness along the antero-lateral aspect of the ribs bilaterally.

#11 Reduced sensation and pain of the right hand (C6-8)

#12 Joint pain in the MCP joints of the index and middle finger, and several of the PIP joints, suspect polyarthritis

#13 Movements were reduced in extension and flexion of the fingers.

#14 Unable to grip paper between his thumb and index finger (pincer grip) or between the index and middle and the middle and ring finger (C8, T1)

#15 Pain over the sacro-iliac regions (L5, S1-2)

2 What are the possible differential diagnoses in this case?

This patient had cough, fever, and mono-neuritis multiplex.

Differential diagnoses are as follows. Dr. Tierney in SFVA taught this system. These eleven categories are great.

Vascular: Less likely

Infection: Chronic hepatitis, HIV, TB (TB is always differentiated in feverish patient in Japan), Leprosy

Neoplastic: Lymphoma, Paraneoplastic syndrome

Collagen (autoimmune): Microscopic polyangiitis, Polyarteritis nodosa, Goodpasture’s syndrome, Wegener’s granulomatosis, Churg-Straus ssyndrome, SLE, Sarcoidosis, Waldenström’s macroglobulinemia, Cryogloblinemia, Chronic inflammatory demyelinating polyradiculoneuropathy.

Toxic/Metabolic: Diabetes (less likely)

Trauma/Degenerative: Less likely

Iatrogenic: Less likely

Idiopathic: Amyloidosis

Congenital: Less likely

3 What tests would you undertake to investigate this patient's problem including both simple and advanced tests?

I highly suspect vasculitis in this case. In Japan, microscopic polyangiitis is not so rare. Wegener’s glanulomatosis is very rare in Japan. Churg-Strauss syndrome is also rare in any countries. Moreover, this patient doesn’t have history of asthma. Microscopic polyangiitis will involve lung and kidney. Measurement of creatinine, urinalysis, and chest x-ray are essential.

I would order CBC, AST, ALT, LDH, creatinine, Na, K, Cl, ESR, urinalysis, P-ANCA, and chest X-ray.

4 Give your top three differential diagnoses.

1 Microscopic polyangiitis

2 Polyarteritis nodosa

3 Chronic inflammatory demyelinating polyradiculoneuropathy"

Professor Matsumura, as always, thank you very much !

I think the above case and the expert breakdown into the essential elements by Professor Matsumura teaches us important lessons.

For example, autoimmune disease can present with motor-sensory weakness and moreover, a vasculitis can simulate what would initially appear to be a UTI !

As a matter of evidence based treatment, many patients with microscopic polyangiitis require both high dose steroids plus cyclophosphamide in order to settle the inflammation rather than just steroids alone.

This case was certainly a great challenge!