Answer To Recent Case

Here are the answers to the recent case-- I am sorry if it is overly long to read but I hope it will cast some light on this difficult scenario.

Question 1: Please make a problem list soley from the history and physical examination findings.

Problem list

• Unsteadiness
  
• Slurred speech
  
• Periperal numbness (on history and physical)
  
• Diplopia
  
• Meningoencephalitis
  
• Seizures
  
• Medications- Phenobarbital / Phenytoin / Sodium Valproate / Flunitrazepam / Metoclopramide
  
• Fine tremor
  
• Coarse Facies
  
• Alopecia
  
• Cerebellar signs
  
• Absent reflexes

Question 2: What other specific question(s) would you like to learn from the history that might help you with this diagnosis?

The drug history is very important here and the drug doses and frequency should be investigated in addition to searching for hidden alcoholism.

The fact that the patient is happily married does not exclude her from being infected with a sexually transmitted infection by her partner and hence, a discussion with the partner might be in order especially if an unusual infectious aetiology were to be found e.g. neurosyphilis.

Moreover, the fact that the patient works in the 'fashion industry' is very non-specific. For example, she might make jewellery!! She might be using Lead!! Lead can cause cerebellar disease too ! Hence, a more in-depth questioning about her occupation would be in order here.

Question 3: What particular feature of the oral examination might give you a further clue regarding the cause of this problem.

Given the fact that the patient takes Phenytoin, one chronic side effect is gum (gingival) hypertrophy. This might give the clinician a clue regarding the cause of some of the symptoms and signs.

Question 4: What is the diagnosis?

The fact that the patient has severe cerebellar signs, peripheral neuropathy, a resting tremor, coarse facies, alopecia and gum hypertrophy, a diagnosis of acute on chronic phenytoin toxicity would be top of the list.

However, when one thinks of cerebellar dysfunction, one needs to consider various other causes such as:

Acquired

Drugs- Phenytoin, flunitrazepam (can cause drowsiness, slurred speech, tremor), Phenobarbital (known to cause ataxia), Na valproate (known to cause tremor, ataxia, speech disorder.

Salicylates, aminoglycosides, sedatives ( e.g. benzodiazepines), chemotherapy agents e.g. fluorouracil, cytarabine

Infection- Viral encephalitis, HIV, CJD, abscess

Trauma

Connective Tissue- SLE, Sjogren’s disease, Hashimoto’s thyroiditis, Auto-Ab associated Cerebellar Ataxia

Neoplastic- primary ( e.g. meningioma, haemangioblastoma) or secondary brain tumour (melanoma, lung, breast, thyroid etc), and Primary CNS Lymphoma. Cerebellar pontine angle tumour e.g. acoustic neuroma associated with neurofibromatosis type 2.

Paraneoplastic cerebellar dysfunction can occur with Breast Ca, Lung Ca (mainly SCC), Ovarian Ca, Uterine Ca and Cervical Ca.

Haematovascular- posterior circulation stroke (infarction or bleed), dissection.

Immune- Multiple sclerosis, Guillain-Barre Syndrome (Miller-Fisher variant)

Metabolic- Alcoholic cerebellar degeneration, Lead poisoning.

Granulomatous- sarcoidosis

Degenerative- olivopontocerebllar disease (may be part of a multi-system atrophy)

Congenital- Friederich’s ataxia- cerebellar degeneration, peripheral neuropathy, spinal degeneration, pes cavus, autosomal recessive, onset age 20; 1:50,000 prevalence (one of the several causes of absent reflexes but extensor plantar response). There are several other more rare congenital ataxias.

Question 5: Which one laboratory test would give you the diagnosis?

The primary lab test to be done is the serum phenytoin level. This correlates well with symptoms when there is toxicity.

Phenytoin is a well established drug used for various conditions including seizures and dysrhythmias (although the latter used less commonly these days). It can be given intravenously or orally.

Normal range for phenytoin in Japan is 10-20mg/L.

Above 20mg/L, nystagmus is common. Above 30mg/L, tremor, ataxia and slurred speech occur. When levels exceed 40mg/L, lethargy, confusion and unconsciousness occur. There have only been a handful of patient cases who have survived with levels >100mg/L.

Phenytoin is protein bound and hence, in patients with low albumin levels or renal failure, patients may experience toxicity at lower serum concentrations.

Phenytoin exhibits dose-dependent elimination kinetics, and toxicity can occur easily in patients on chronic therapy especially when there are drug interactions or slight alterations of daily dose. As the phenytoin level reaches therapeutic range, the liver elimination of the drug becomes saturated and therefore becomes Zero-Order in Kinetics. Hence, the half-life increases as serum levels rise e.g. T1/2 = 26 hours at 10mg/L, 40 hours at 20mg/L and 60 hours at 40mg/L respectively.

There is no specific antidote available for treatment of phenytoin toxicity whether iatrogenic or intentional and treatment is supportive e.g. charcoal for elimination (if orally administered) and maintaining airway, breathing and circulation.

In this case, the patient was also taking several other drugs and hence, drug-drug interactions must be considered here and include:

Phenytoin-Valproic Acid interaction- valproate displaces phenytoin from protein binding causing toxicity

Phenobarbital - Valproate Interaction- valproate reduced metabolism of Phenobarbital and can result in toxicity. One case report of decreased valproate effect through increased metabolism.

Phenobarbital – Flunitrazepam interaction- decreased effect of benzodiazepine through increased metabolism induced by phenobarbital. Increased risk of respiratory failure with combination of benzodiazepine plus phenobarbital (one case report 1986).

Benzodiazepine – Phenytoin interaction - decreased effect of benzodiazepine through increased degradation via liver enzyme induction. Possible toxicity when phenytoin and benzodiazepine combined although mechanism not established.

Benzodizepine – Valproate interaction – possible benzodiazepine toxicity by displaced protein binding by valproate and also possible decreased detoxification by the liver (glucuronidation).

Hence, there are several potential drug interactions at work in this patient. Other drugs being used to control the seizures may result in phenytoin toxicity, and toxicity or reduced activity of other drugs.

I have no doubt that drug-drug interactions contributed to the acute on chronic toxicity. However, another part of the history found out later was that the patient had poor daily compliance and she took sometimes more medication than prescribed in order to make up for lost doses.

The usual daily dose is up to 300mg of phenytoin and only several cases has there been doses up to 500mg/day. This patient was apparently taking doses far in excess of the recommended daily dose.

Other clues to the diagnosis of phenytoin chronic administration were the mild leucopenia and thrombocytopaenia which are also well described. The CT and MRI scan of the cerebellum were both entirely normal.

Case Response From Dr Masami Matumura from Kanazawa Medical University. As usual, Dr Matsumura provided an excellent breakdown of the case, and is as follows;

Question 1:Please make a problem list soley from the history and physical examination findings.

#1 Ataxia

She showed unsteadiness when walking, gait- wide based, staggering in nature, heel to toe test positive, heel to shin test positive R>L, finger to nose test positive bilaterally, dysdiadochokinesis positive bilaterally, nystagmus, slurred speech, and fine resting tremor.

#2 Polyneuropathy

She had numbness of her arms and legs, mildly reduced light touch, and no reflexes.

#3 Diplopia

#4 Pinky-red cheeks

#5 Coarse skin

#6 Alopecia.

#7 Viral meningoencephalitis

#8 Post-meningitis seizures treated with medication

Interestingly, she has two neuro-anatomical disorders, ataxia and polyneuropathy.

I have to pick up all possibilities in this case.

Differential diagnosis:

• Vascular: Cerebellar infarction, Subdural hematoma
  
• Infection: Cerebellar abscess, HIV, Acute viral cerebellitis, Lyme disease, Syphilis, Lepromatous leprosy,
  
• Neoplastic: Paraneoplastic syndrome, Cerebellar glioma, Cerebellar metastasis
  
• Collagen (autoimmune): Systemic lupus erythematosus, Sarcoidosis, Sjogren’s syndrome, Dermatomyositis, Primary biliary cirrhosis, Guillain-Barre syndrome, Post infection syndrome
  
• Toxic/Metabolic: Phenobarbital, Sodium valproate, Metoclopramide, Lead, Aluminum, Hypothyroidism, Vitamin B12 neuropathy, Diabetes
  
• Trauma/Degenerative: Spinocerebellar atrophy
  
• Iatrogenic: Phenobarbital, Sodium valproate, Metoclopramide
  
• Idiopathic: Amyloidosis, Multiple sclerosis
  
• Congenital: Fabry’s disease, Friedreich’s ataxia, Porphyria

She could not describe onset of symptoms. I assume these conditions were acute course or subacute course. If it was true, infection, neoplastic, collagen, toxic, and metabolic causes must be considered.

Question 2: What other specific question(s) would you like to learn from the history that might help you with this diagnosis?

I would ask her whether she has episodes of skin rash after sun exposure or not. And I would ask her change of voice and feeling cold or not.

Question 3: What particular feature of the oral examination might give you a further clue regarding the cause of this problem?

I seek oral ulcers!

Question 4: What is the diagnosis?

Systemic lupus erythematosus (SLE) is highly suspected in this case. She had two neuro-anatomical disorders, pinky-red cheeks, coarse skin, and alopecia.

Second candidate is autoimmune thyroiditis. She is Japanese woman.

Interestingly, both SLE and Hashimoto’s thyroiditis can be associated in several cases.

I can’t explain the cause of diplopia. Opthalmopathy is not described.

Metoclopramide is often prescribed in many patients. This drug can cause extrapyramidal sign. Tremor might be caused by metoclopramide in this case.

Question 5: Which one laboratory test would give you the diagnosis?

Serum antinuclear antibodies.

If it is possible, I would order CBC, FT4, and TSH.

Comments from B:

Dr Matusmura raises some very interesting points with his case interpretation. SLE is still a possibility. However, the pink cheeks on this patient did not involve the bridge of her nose, which the classical ‘Butterfly Rash’ of SLE does. Moreover, skin rashes are known to be a manifestation of phenytoin administration as well.

Alopecia is indeed a feature of SLE although it is not a pathognomic feature and can be found in phenytoin toxicity too—as can hirsuitism!

However, interestingly, phenytoin has been associated with inducing SLE in several patients although this tends to be a discoid-type rather than systemic.

Lastly, when the phenytoin dose was decreased, the symptoms began to resolve, and if this were purely SLE, one might not expect such a rapid improvement.

In order to diagnose SLE, the must be several criteria met and they include the following:

1) Malar rash

2) Discoid rash

3) Photosensitivity

4) Oral ulcers

5) Arthritis

6) Serositis- pleurisy or pericarditis

7) Renal disorder- persistent proteinuria >0.5g/24h or cellular casts

8) Neurological Disorder- seizures or psychosis (having excluded other causes first)

9) Haematologic disorders

• Haemolytic anaemia or
  
• Leucopaenia <4.0x109/litre>
  
• Lymphopaenia <1.5x109/l>
  
• Thrombocytopaenia
  

10) Immunological disorders-

• Raised anti-DNA antibodies
  
• Anti-Sm antibody
  
• Positive anti-phospholipid antibodies
  

11) Raised titre of Anti-nuclear antibody

SLE can be diagnosed if 4 or more of the above criteria are met either simultaneously or serially over time.

(1982 revised criteria of the American Rheumatologu Association [now American College of Rheumatology] for the diagnosis of SLE)

From the above criteria, at most, the patient may have only had two criteria for suspecting SLE, those being the haematological disorders and the possible Malar rash. Cerebellar dysfunction although well recongised in SLE is not one of the diagnostic criteria. Unfortunately, antibodies for SLE were not performed in this patient.

Hence, it is still possible that phenytoin may have induced some features of SLE, and checking autoantibodies would be certainly worthwhile.

Finally, the haematological disorders in phenytoin toxicity are related to depleted folic acid and hence, checking this blood level and giving replenishment would be one way to correct the problem.

I think this paper from 25 years ago sums up what needs to be said:

1: Acta Neurol Scand Suppl. 1983;97:49-67.


Side effects of phenobarbital and phenytoin during long-term treatment of epilepsy.

Iivanainen M, Savolainen H.

Phenobarbital and phenytoin have good antiepileptic effect, but clinically significant untoward effects occur during their long-term use. Phenobarbital may cause hyperactivity, behavioral problems, sedation, and even dementia; these effects are dose related to some extent. Side effects of phenytoin include sedation, a cerebellar syndrome, phenytoin encephalopathy, psychosis, locomotor dysfunction, hyperkinesia, megaloblastic anemia, decreased serum folate level, decreased bone mineral content, liver disease, IgA deficiency, gingival hyperplasia, and a lupus-like hypersensitivity syndrome. Especially susceptible to the neurotoxic effects of phenytoin are epileptic children with severe brain damage who are on multiple drugs. In those children, balance disturbance may develop and be followed by gradual loss of locomotion. Among 131 mentally retarded epileptic patients, phenytoin intoxication occurred in 73 (56%), of whom 18 experienced persistent loss of locomotion. There is experimental evidence that the toxic action of phenytoin lies at the cellular level, predominantly in the cerebellum. Many experts avoid the long-term use of phenytoin because of its insidious and potentially dangerous side effects.

Thank you very much Dr Matsumura.

Another response was from Professor Lefor of Jichi Medical University who made an excellent attempt to answer this very difficult case. Professor Lefor’s way of working up this case is also very thorough and indeed, the diagnosis for this case does fall into that ‘other’ group. His reponse is below:

1.My problem list includes:

a. Unsteady gait

b. Diplopia x2y

c. Sensory changes

d. Slurred speech

e. Hx seizures in the distant past, on meds

f. PE showed no bruits, nystagmus, slurred speech, tremor, decr sensation and wide gait

2. What other questions?

I would want to know more about the time course of onset of these various symptoms.

3. Oral Exam: I am not sure what to look for

4. What is the diagnosis?

This is a tough case. There is a wide variety of disparate neurologic findings here. It is difficult to put it all together. The major etiologies would include vascular, infectious, tumor, and other. I doubt it is vascular given her age, physical findings and onset. Infectious is possible, but the time course does not support this. Tumor is certainly possible, but the wide variety of symptoms makes it hard to assign to a mass lesion. My number one is in “Other” category, and I believe that this is most consistent with some global neurologic disorder such as Multiple sclerosis which affects a wide area of the CNS as a de-myelinating disease. She is a little bit old for the usual patient (in the USA the onset is rarely over age 40), but this disease, or some other de-myelinating condition seems most probable given the wide range of structures affected as evidenced by her symptoms.

5. What is the one test: For MS, there is no one absolute test, but MRI is quite good and is the one test I would get. MRI would also rule out a brain tumor, which is also a possible dx, and must be fully ruled out quickly to avoid “missing it”.

Thanks for letting me participate

Alan Lefor

Comment from Dr B： Professor Lefor’s consideration of MS is very thought provoking especially as diplopia can be a symptom of MS. Moreover, the onset of cerebellar dysfunction can also occur in MS and neurological deficits occuring in various places over time is one feature of this disorder. Indeed, exacerbations of MS with acute deterioration can occur although this is usually related to an underlying infection which there is no history of in this case. Moreover, the generalised loss of peripheral tendon reflexes is not a usual presentation of MS. However, with an acute deterioration, one might expect the loss of reflexes e.g. in a single limb, and for them to return in time and become hyper-reflexic signifying a previous upper motor neurone lesion.

However, the pinky cheeks, coarse face, alopecia etc do not fit with MS or with a tumour and hence, a metabolic or autoimmune condition would be considerably higher up on the list. However, things such as tumours and MS should always be ruled out in such conditions as has been described above.

Thank you very much Prof Lefor.

Learning Points From This Case

If a detailed history is taken, if it is a common disorder, the cause will usually become obvious quickly.

Taking a thorough drug history, drug doses and frequency is very important. One must not just re-prescribe the drugs just because that is what the previous physician did or that is what the patient says they are taking. You must check the correct doses if you do not already know.

In this case, the drug doses and frequencies were purposely omitted in order to draw your attention to the fact that they were missing. They should never be over-looked or omitted from the history and young physicians should always be asking about drugs the patient takes. Drug doses and frequencies must be checked to avoid toxicity. This case demonstrates this example very clearly.

Checking drug-drug interactions is also essential. I would suggest obtaining a handbook on drugs that deals with this or perhaps one for PDA e.g. Epocrates (epocrates.com [free]) or the Handbook of Adverse Drug Interactions (skyscape.com).

Generally, as physicians we all have to know an awful lot about drugs but it is something that can cure or curse a patient but we must know when that happens. The only way is learn throughly about drugs and especially about commonly used ones in daily practise. One textbook that I used as a student was Rang & Dale's Pharmacology with an updated edition just last year. You can never know too much about a drug.

Common Things Are Common and 'When You Hear Hoofbeats, Don’t Expect To See A Zebra'~~(Theodore E. Woodward M.D) are very important ideas. Obviously, things such as drugs, alcohol, stroke and infection are high up on any list for cerebellar diseases. These should be excluded initially and if ruled out, investigation for other causes such as MS, SLE and even Friederich’s ataxia which lie somewhere further down the investigative road.

Usually, if you prioritise your differential diagnosis according to the history and physical plus specific lab and radiological tests, the answer will soon become apparent.

But remember this, the answer usually lies somewhere within the history but you have to elicit the right answers to find it.

Please consider and have a good week!

Finally, thank you to Dr T and his team for showing me such a great case.

A new case-- time to put your thinking caps on

Dear Bloggers

Today I have another great case for you supplied by a distant hospital in Japan. The case has been anonymised to safeguard patient confidentiality.

Presenting complaint: unsteadiness when walking

The patient was a 47 year old female working for a busy fashion design company. She developed unsteadiness when walking at work. She was uncertain whether it was of sudden onset but she remembered she did not have this problem when on the bus and then train that she took daily to her job.The unsteadiness was not associated with dizziness, new visual disturbance, nausea, vomiting or hearing loss and she denied motor symptoms. She denied vertigo and dizziness on standing.

She did however admit to the following symptoms including:

1) Diplopia- a two year history that had not got worse and was corrected by her prescription spectacles; this occurred mainly when she looked upwards.

2) Numbness of her arms and legs- this had occurred several days before the onset of the unsteadiness and was throughout the entire length of her limbs. She did not have any facial numbness according to her account.

3) Slurred speech- this occurred around the same time as the onset of unsteadiness.

Previous medical history included

• Viral meningoencephalitis (20 years before)
• Post-meningitis seizures treated with medication; no seizures for many years.
• Glaucoma

Medications included

• Phenobarbital
• Sodium valproate
• Phenytoin
• Flunitrazepam
• Metoclopramide

No known drug allergies (NKDA)

She was otherwise fit and healthy and was a hard working mother of two children and was happily married. She was fully independent and was a non-drinker and had never smoked. There was no family history of cardiac, respiratory, abdominal or CNS disease.

Pertinent Negatives

She denied the following:

GEN- no fever, no sweats, noshakes/chills, no constitutional symptons

HEAD- no sinus pain, no otalgia and no throat pain.

CVS- no palpitiations, no chest pain, no dyspnoea

RESP- no recent cold, no cough, no sputum, no haemoptysis

ABDO- no nausea, no vomiting, no jaundice, no pain, no constipation or diarrhea. Normal appetite and no weight loss.

MUSC- no weakness, no muscle pain, no joint pains or swelling

UROGEN- normal menstrual periods, no menorrhagic, no abnormal vaginal discharge, no urethral pain, no urgency, no frequency and no haematuria. No new sexual partner—not using barrier contraception.

CNS- no sudden onset headache, no hx of migraines, no blurred vision, no loss of vision, no swallowing problem, no hearing disturbance, no facial numbness.

PNS- no falls, no collapse, no uni- / bi-lateral weakness, no loss of bladder or bowel control. No back pain.

Endocrine- No polyuria, no polydipsia, no disturbance of concentration, no increased or decreased appetitie, no lactation, no recent increase or decrease in weight / size of hands or feet, no Hx of hypertension.

Skin- she denied any new skin problems

On examination

The patient was alert and looked otherwise well. She was afebrile, BP 120/80mmHg, Pulse 80/min regular, Resp rate 14/min regular, O2 sats 98% breathing ambient room air.

General- pinky-red cheeks, coarse skin and evidence of alopecia.

No jaundice, anaemia, clubbing, cyanosis, oedema or lymphadenopathy (JACCOL)

CVS- Hands warm and well perfused. Pulse good volume. JVP not elevated. Heart sounds 1 & 2 normal with no added sounds or murmurs. No carotid bruits. No peripheral oedema.

RESP- Trachea central and no tracheal tug. Expansion normal. Percussion note normal. Normal vesicular breath sounds.

ABDO- Soft, flat, non-tender, no masses. Bowel sounds normal. Genital and rectal examinations not performed.

Breast exam- normal.

CNS

I normal sense of smell

II normal visual fields. Pupils equal and reactive to light and consensual reflex normal.

III / IV / VI – Normal range of movement but Nystagmus bilaterally (mild) and some vertical nystagmus on vertical gaze.

V- motor and sensory normal.

VII / VIII - normal

IX / X / XI / XII - individual nerve testing normal.

Mild slurring of speech noticed.

PNS

Fine resting tremor- not ‘pill rolling’ type.

Tone- normal throughout

Power- normal throughout

Reflexes- absent throughout

Sensation- mildly reduced light touch throughout; other modalities not tested

Coordination- finger to nose test positive bilaterally. Dysdiadochokinesis positive bilaterally. Heel to shin test positive R>L.

Babinski sign negative bilaterally

Gait- wide based, staggering in nature (ataxic) and heel to toe test positive.

Question 1: Please make a problem list soley from the history and physical examination findings.

Question 2: What other specific question(s) would you like to learn from the history that might help you with this diagnosis?

Question 3: What particular feature of the oral examination might give you a further clue regarding the cause of this problem?

Question 4: What is the diagnosis?

Question 5: Which one laboratory test would give you the diagnosis?

Tendon Hammers and Patient's Feet

Dear Bloggers

Today I would like to advise on what I consider to be the correct use of a Tendon Hammer to perform the Babinski sign for determining upper motor neurone lesions.

Essentially, the end tips of most of the modern hammers are far too sharp and can be very uncomfortable to use on a patient's foot especially when there are a team of doctors and students all having a try to practise their skills to elicit the sign.

Unless the end of the tendon hammer is blunted it is therefore not appropriate to use the sharp tip for eliciting the Babinski sign. Please try and use another type of blunted instrument.

In order to understand just how uncomfortable it is to perform Babinski sign on a patient with a sharp tipped tendon hammer I suggest you take off your own shoes and socks and get a colleague to perform it on you. It is not a pleasant experience. Never do to a patient what you would not have done to yourself!

Use of the tip of the tendon hammer by doctors in the UK MRCP examinations can lead to failing that section of the examination. Never put your patient through unnecessary discomfort. Things that can be used are 'orange' sticks, your thumb (although not in an formal examination!), a blunt tipped tongue depressor. The former and latter can be disposed of although I would recommend washing your thumb rather than trying to dispose of it :-p

Just to remind you of the correct technique, ensure that the toes are neutrally positioned e.g. not pointing up or down. Use the blunt instrument to excite the lateral border of the foot starting at the heal and running caudally towards the fifth metatarsal head followed by a medial turn across the foot towards the remaining metatarsal heads in the direction of the great toe.

A negative sign is visualised by the toes plantar flexing [true flexion]. A positive sign is visualised by the toes moving into dorsiflexion [plantar extension]. The most sensitive aspect is visualising the movement of the Great Toe because it may be the only toe that moves.

Sometimes the excitation of the plantar elicits no response at all and it can be due to peripheral neuropathy or myopathy although it may also be seen in early stroke or spinal cord injuries. Drugs also temporarily influence the Babinski response and include sedative drugs such as propofol, general anaesthetics etc.

Please consider....

Antibiotics and Evidence versus Eminence Based Medicine

Dear Bloggers

I hope that you are having an enjoyable Golden Week. The weather is not particularly golden and more like 'lead'- grey with it being overcast :-(

Today I would like to focus on the duration of antibiotic usage for treatment of pneumonia. The 'recommended' duration of intravenous antibiotic use is widely accepted to be a week to 10 days or sometimes longer. From one colleague I had heard that intravenous antibiotics are routinely given for infections resulting in positive blood cultures for a standard of two weeks even if the patient has recovered, despite the underlying cause. In many of the traditional textbooks and handbooks covering the use of antibiotics, there is the standard use of 7-10 days of antibiotic use in pneumonia. However, where is the evidence for this widely accepted dogma??

A team from the Netherlands attempted to investigate whether an abbreviated three-day course of intravenous amoxicillin was inferior to an 8-day course of amoxicillin consisting of 3 days of initial intravenous therapy followed by 5 days of orally administered amoxicillin for use in mild to moderate-severe community acquired pneumonia.

Patients were included in the study if they had symptoms and signs of pneumonia plus radiological evidence of the diagnosis. They all received high-dose intravenous amoxicillin for 3-days after which, if there was seen to be improvement e.g. feeling better, reduced sputum, fever reduction etc, the patients either received placebo or high dose oral antibiotics for a further 5 days.

The results were astonishing in that a 3-day course of intravenous amoxicillin was observed not to be inferior to an 8-day course of amoxicillin for the treatment of mild to moderate-severe community acquired pneumonia.

As 60-80% of admitted patients have mild to moderate-severe pneumonia [pneumonia severity score <110], it is therefore conceivable that most hospitalized patients with CAP could be treated with high dose intravenous amoxicillin for just 3 days.

The majority of bacterial isolates were the usual culprits of Streptococcus pneumoniae followed by Haemophilus influenza.

The study had less than 200 patients and the 3-day-treatment-placebo group had worse symptoms than the 8-day-treatment group on admission but the overall cure outcome in the 3-day group was statistically non-inferior to the 8-day group.

It seems logical that antibiotics when given either orally or intravenously for specific infections e.g. CAP, uncomplicated UTI, should be done so at high-dose and for a short period of time if there are no complicating factors. The idea behind this is that the Minimum Inhibitory Concentration can be achieved as the serum level is high and with a short duration of administration reduces the exposure of bacteria to prolonged periods of antibiotics which is known to promote antibiotic resistance.

Moreover, the longer that antibiotics are continued, the more side-effects that one gets such as drug-associated fevers, drug induced rashes and antibiotic associated colitis e.g. Clostridium difficile infection.

Short course antibiotics lead to reduced expense on antibiotics, reduced hospital stay and reduced exposure to hospital acquired infections.

Patients should not be kept in hospital just because of the widely accepted dogma that 10-days of antibiotics have to be administered. There needs to be a re-evaluation of the current thinking so that evidence is used when treating patients rather than eminence based opinion.

One thing I have noticed commonly is the use of low dose prolonged courses of antibiotics. I cannot see the use for such prolonged courses especially when side effects are increasingly common. In the BMJ study, the three-day course was associated with no observed side-effects as opposed to the 8-day course which was associated with side-effects. Moreover, if low-dose antibiotics are used, then the MIC cannot always be effectively achieved and would also promote resistance and failure to achieve a clinical and/or bacteriological cure.

To rely on rigidly fixed doses for use of antibiotics is not logical because patients have all different weights, volumes of distribution depending on body fat, and they may have underlying renal or liver disease which ultimately affects the final dose that can be used. Just consider for one moment the use of weight per kilogram per day of antibiotic administration akin to childrens dosing.

The following examples may surprise you of just how much antibiotic is recommended. Obviously, doses and duration of antibiotics depends on the underlying infection, the patient’s immunity, renal and hepatic disease plus local bacteriological resistance factors.

For example, the maximum allowed oral dose of amoxicillin in Japan is 250mg three times daily. However, if one uses the paediatric dosing according to world standards e.g. 40mg/kg/day and if for example an adult patient is 40kg, the total daily dose required is 1600mg. Divided into thrice daily dosing, this equates to over 500mg per dose [533mg to be precise]. Hence, the dose allowed to be prescribed is half of what is recommended to be given to adult patients according to world standards.

As a consequence, with this single example, it may to some extent explain why some community acquired pneumonias and urinary tract infections are only partially treated in the community thereby requiring hospital admission for higher dose intravenous antibiotic administration.

A further example is the use of clarithromycin-- maximum daily dose in Japan is 200mg twice daily. However, if one uses the pediatric world dosing standard which is 7.5mg/kg/day every 12 hours, in a 40kg patient, the dose would be 300mg per dose. Obviously, for larger patients the dose would be higher. In fact, the recommended world dose for adults is 500mg twice daily.

One other final example is the aversion to use Benzylpenicillin (Penicillin G) in pneumococcal pneumonia for fear of not covering the Penicillin Resistant Streptococcus Pneumoniae [PRSP]. This aversion is unfounded because pneumococcal pneumonia can be effectively treated with this well established antibiotic even for PRSP so long as it is used at high-dose e.g. 2 mega units, six times daily [total of 12,000,000 units daily].

A recent example from another hospital was a female patient with a typical uncomplicated community acquired pneumococcal pneumonia who was treated with the high-dose Penicillin G regimen and who had antibiotics stopped after just 4 days because of complete resolution of symptoms. She was discharged on day 5 with early outpatient follow-up. Penicillin G does work ! :-)

Finally, in the Netherlands 3-day pneumonia treatment study mentioned above, the intravenous dose is not mentioned but the 5-day oral course was 750mg three times daily-- hence, high dose!

This study demonstrates that for specific case definitions e.g. mild to moderate-severe community acquired pneumonia, the use of a three day course of intravenous amoxicillin is not inferior to three days of intravenous therapy followed by five days of high dose oral amoxicillin [total of eight days treatment].

If you are interested in reading this fascinating research it can be read for free on www.bmj.com.

The reference is:

el Moussaoui et al. The effectiveness of discontinuing antibiotic treatment after three days versus eight days in mild to moderate-severe community acquired pneumonia: randomised, double blind study. BMJ. Vol 333, 30 Sep 2006.

Please Consider....

Ethics in Medicine

Dear Bloggers

I sometimes like to do an article based on ethics. Medicine is not just about trying to make a diagnosis and treating everything that is a problem. Medicine is not just a science project that can be controlled like a laboratory experiment. Medicine is a mix of science, personal interactions, subjective and objective opinions from the doctor, the patient and the family with the abundance of feelings all intermingled.

Ethics is a very important part of medicine and is used perhaps unknowingly all the time when making decisions on how to proceed with patient care.

I have observed that the ethical dilemmas are different in Japan compared to the UK. For example, sometimes patients in Japan do not have the finances to be able to pay for expensive treatments and so on occasion, families will make the decision for the patient which may sometimes lead to an alteration of therapy which may not be what the medical staff would advise or think is appropriate. In the UK, taxation pays completely for the medical system and use of medical services is free at source, and hence, treatments are started if there is a clinical need and the patients and their families do not consider the financial aspects as is done in Japan. However, in the UK, I have said that treatments may be started if there is a clinical need. This is where ethics comes into play because although certain treatments are available, all patients are different, and just because certain investigations and treatments can be done it does not necessarily mean that it is appropriate to do such things either because of the patient's refusal or because of severity of illness, reduced life expectancy, poor quality of life etc...

There is often the saying here of 'All or Nothing' treatment. Either you do everything for the patient or do nothing. I have to say that I really do not agree with such a concept to apply to every patient scenario. Life is not always so black and white-- in fact, life is different shades of grey.

For example, should an elderly patient with a pre-morbid history of severe debilitating chronic disease with profound sepsis and fulminant multiorgan failure with a poor quality of life receive expensive extra-corporeal Polymixin therapy, haemodialysis and receive cardiopulmonary resuscitation when such interventions are very unlikely to cause the patient to survive and moreover, would be unlikely to alter the underlying pathology and importantly, cause more unneccessary discomfort?

There are doctors who might say yes. There would be many who would however say no. This is where ethics comes into the situation. Not everything is so clear cut.

Although haemodialysis removes the toxins from the blood, in the presence of DIC, the use of heparin during dialysis in the treatment of renal failure can worsen bleeding! Haemodialysis is a renal replacement intervention but it does not make the kidneys recover any better! It is purely supportive whilst waiting for the kidney to hopefully recover. However, fulminant renal failure might not recover leaving a patient with an additional chronic diseases and worsening quality of life left on thrice weekly haemodialysis which the patient and their family might not be able to afford. Such therapy might prolong life but might be seen by the patient as inhumane and intolerable. From my observations over the years, doctors frequently forget to consider the end outcome when starting major intervential therapies such as haemodialysis or intubation with ventilation therapy in such patients e.g. terminal lung cancer with respiratory failure.

The great anf famous textbooks show us how to think about symptoms and signs of disease, how to investigate and treat but never about the ethical issues and appropriateness of treatment. For this, the only real textbook is experiencing these situations yourself in the hustle and bustle of daily hospital life. Yes, there are many ethics books available with excellent examples of dealing with difficult issues and I would highly recommend reading such books.

Continuing on, patients with severe sepsis have a very high mortality [over 50% ] in various studies despite optimal treatment. Hence, in such a severe situation, even if there is eventual cardiopulmonary arrest, is it appropriate to perform cardiopulmonary resuscitation? One has to think about the overall outcome. If cardiopulmonary resus is successful, will that stop it from happening again? Will it make the other organs recover? The answer is usually no. One has to consider factors such as cerebral damage of CPR, the potential chronic heart disease resulting from ischaemia of the cardiac arrest, the likelihood of success and I come back to the point once again of Quality of Life.

Many patients with severe chronic disease have a shortened life expectancy and poor quality of life. In the UK, patients are asked directly about whether they would want to be resuscitated rather than asking the family. The family memebers are not the patient and in the UK, they are regarded as separate entities and therefore are not able to make such life and death decisions about the patient unless the patient is a child. On occasion, UK patients will directly state that they do not want to be resuscitated despite the contrary opinion from their relatives. Obviously, if the patient is not confused and not suffering from a psychiatric illness, and is considering such an opinion in a logical, clear way, the doctors have to respect the decision of the patient over that of the relatives. The patient is put first.

However, in Japan, the wishes of the family sometimes over-ride the wishes of the patient and sometimes, the patient is not directly consulted. There is the fear of family members that such discussions might make the patient's mental condition worse and that they will somehow 'die from shock' or that the patient would somehow not want to know their fate. From my experience of direct talking to patients in the UK, I have never experienced such spontaneous death from a discussion about the patient's condition directly with the patient and hence, such thoughts are somewhat unfounded in my opinion. Moreover, doctors siding with family members to avoid informing the patient of the diagnosis is again unfounded. As I have mentioned in a previous blog dealing with ethics, by informing the patient allows family members to be entirely honest with eachother in the final days, weeks or months of terminal illness which has the benefit of bringing family members closer together.

Hence, when we are considering whether to institute a treatment, we need to consider not only the medical problem in front of our eyes, but also the social factors and particularly Quality of Life issues. Doing knee-jerk reactional therapy with the 'Do All Or Nothing' approach is ignoring the Ethical issues which might be in direct conflict of the wishes and the interests of the patient.

Another concern I have about family oriented decisions rather than patient oriented decisions is when family members have a potential vested financial interest to gain from the patient's death. Hence, it is my belief that medical personel should be making the decisions when it comes down to investigating and treating the patient albeit still taking into account the opinion and feelings of the family. However, medical personel can look at the patient and their problems in an independent, unfettered manner to decide just what therapies are appropriate. In doing so, it removes a serious conflict of interest that might potentially exist between the patient and the family members interests.

Palliative care is another area which needs much development in Japan and it is entrenched with ethical issues. I have written on Palliative Care in the past and I see this as a very important arm of medicine. Patients should not be put through pointless operations for cancer that have no hope for prolonging life when the other intervention would be controlling pain and other symptoms to make life as happy and tolerable as possible during the final stages of illness. Using a palliative approach is Not a Failure of the Doctors! Sometimes, diseases can no longer be controlled by 'modern' medicine and pursuing treatments which do not alter the final outcome and cause more pain and suffering is not putting the patients first. Although as medical people it makes us feel better that we have tried our best and shows the family that we are really trying to do everything, the patient can get lost in the mix and it might not be the best for them!

One has to ask what is best? Doing everything to make us and the family feel better at the expense of the patient's feeling both mentally and physically or putting the patient first and using ethics based ideology to decide on whether the patient really wants such treatment and whether it will lead to a favourable outcome. Hence, palliative care is sometimes better than 'full active' treatment as pain, agitation, dyspnoea etc can be controlled through symptomatic measures and medications.

When I started off in medicine I thought that medicine was just about saving people from dying of illnesses. Yes, that principle I still hold true and is the driving force for my love of medicine. However, I learned over the years, that not all patients can be saved from the natural course of their illness and having a humane passage out of this world by using appropriate palliative interventions rather than futile active treatment, is sometimes better than doing everything and still losing the battle with the patient suffering more as a result.

Please remember about ethical issues and think about your patient as a whole being, rather than just a system disease based approach. Please consider....

Have a nice day....

Answer To Recent Case- Acute Weakness and Sensory Impairment

Dear Bloggers

Here is the answer to the recent case.


Question 1: From history and examination, list the problems with this patient

• Rapidly progressive weakness of the lower limbs with numbness
• Ascending sensory level to T10
• Bowel and bladder disturbance
• Loss of muscle power, deep tendon reflexes and Babinski absent in lower limbs
• Incomplete presence of light touch and vibratory sense; absent nociception and thermoreception
  
• Loss of anal tone and 'wink' sign
• Hypertension
• Smoker
• Mildly expansile aorta
  

Question 2: What immediate test or tests would you perform and why?

From the acute history of weakness and numbness progressing over minutes, loss of bowel and bladder function with progression over several more hours is consistent with a acute spinal cord condition either from external compression or impairment of blood supply or a transverse myelitis.
The patient was otherwise well and was afebrile on admission, making a viral infection less likely. Moreover, there was no history of previous back problems e.g. disc herniation. The fact that the patient is a smoker and hypertension are cardiovascular risk factors and with this history, it is consistent with an acute spinal cord infarction.

The immediate test would be do perform an MRI to rule in or out a spinal cord compression. An abdominal ultrasound or CT to look for an aortic aneurysm would also be prudent if there was a history of abdominal pain and/or an abdominal aorta consistent with aneurysmal change. An ECG should be performed to look for atrial fibrillation which might contribute to systemic embolisation. Echocardiogram would also be worthwhile to rule out a cardiac source of embolism e.g. intra-atrial thrombus, vegetation from endocarditis.

Question 3: What anatomical problem is this from the history and physical examination?

The anatomical problem is two-fold. Firstly, the blood supply to the spinal cord has been impaired and this has led to infarction. However, from the physical examination, the nociception and thermoreception plus motor are predominantly impaired whereas a modicum of light touch and vibratory sense are maintained.

In view that the anterolateral cord contains the descending long tracts (corticospinal tracts) and the ascending spinothalamic tracts (pain and temperature), it would appear that the anterolateral cord is most affected with less of the dorsal cord affected which convey senses such as light touch and vibratory sense. However, strangely, the joint position sense was impaired which also runs up the dorsal columns. This cannot be easily explained unless the patient already had an existing peripheral neuropathy prior to the onset of this disorder e.g. diabetes mellitus.

In view that there is a single anterior spinal artery supplying the anterior cord and two posterior spinal arteries, this problem is consistent with an anterior spinal artery occlusion and anterolateral cord infarction.

Question 4: What is the current evidence based treatment for such a condition?

There have been many proposed therapies including anti-platelet treatment, anti-coagulation, thrombolytic therapy. This condition is relatively rare and so being able to perform a randomised control trial is difficult. Hence, most treatments are based on a case by case basis.
However, therapy is similar to that of cerebral infarction.

To date, there has been only one published case of using thrombolysis in a patient with this problem, although I have heard of another case of spinal cord infarction where thrombolysis was also used [unpublished] with good results.


Tex Heart Inst J. 2007;34(1):134-5.

Acute spinal cord ischemia during aortography treated with intravenous thrombolytic therapy.
Restrepo L, Guttin JF.

In the above case, the infarction occurred after aortography rather than de novo without an inciting cause.

In this case, anti-platelet therapy was commenced without modification of the patient symptoms and following this, heparin was commenced. The patient did not receive rTPA.

There was a minimal response to therapy with only a slight improvement in sensation but no improvement in motor response. It is therefore not certain whether the mild improvement was related to the treatment or just the natural course of the condition.

The intial MRI scan showed some osteoarthritis high up in the neck but not considered to be the cause of the symptoms when reviewed by the neurosurgeons. The cord proper looked normal.
A repeat MRI taken after several days again looked normal.

CSF examination was not performed.

ECG showed sinus rhythm and the echocardiogram revealed no thrombus. Abdominal echo revealed no evidence of an abdominal aortic aneurysm.

Arteritis was also considered but the ESR was within the normal range for age and sex.

Several doctors have kindly responded to the case and include Professor Alan Lefor, Professor of Surgery, Jichi medical school, Japan gives a surgeons view of the history and physical followed by the kind Dr Masami Matsumura of Kanazawa University School of Medicine with an indepth and excellent break down of the case into its essential elements. Thanks to both these esteemed doctors for their opinions here.

Professor Alan Lefor:

To me, a case of acute paralysis and anal sphincter loss of tone, is an acute spinal cord problem such as trauma (no history here), spinal cord ischemia (like a dissection, but in the absence of pain might not be as high on the list, although she does have hypertension) or spinal cord compression (like a tumor, hematoma).

My workup would start with a CT scan to evaluate the aorta and spinal canal looking for dissection, aneurysm, and spinal canal mass.

Rx for a dissection would be htn control.

Dr Masami Matsumura:

Questions
1) From history and examination, list the problems with this patient.

Problem list:
#1 Numbness of the buttocks
#2 Rapid onset lower limb weakness, then paralysis of the lower limbs
#3 Reduced muscle tone
#4 Reflexes knee (L2-4) absent, ankle (S1) absent
#5 Light touch- decreased in lower limbs up to the umbilicus (Th10) region
#6 Vibratory sense (posterior column) was intact on the pelvic brim (S3) and right knee (L4) but absent further down
#7 Nociception and thermoreception were absent.
#8 Reduced anal tone (S2-4)
#9 Bowel and bladder disturbance.
#10 Absence of joint position sense (posterior column)
#11 Hypertension
#12 Smoking history
#13 Aorta mildly expansile

Assessment:
At the beginning, this patient is 70 years old woman who has history of HTN and smoking. I would use following system for thinking differential diagnosis in view of weakness. I would differentiate neoplastic or degenerative diseases first.

Vascular: possible, infarction of spinal cord, arterio-venous malformation
Infection: less likely,spinal epidural abscess,Lyme disease, syphilis
Neoplastic: possible, vertebral or spinal cord tumor, metastasis ofbreast cancer, lung cancer,gastrointestinal cancers,or renal cell carcinoma, non-Hodgkin's lymphoma, plasmacytoma, multiple myeloma, orparaneoplastic syndrome
Collagen: possible, sarcoidosis, polymyositis, vasculitis, Guillan-Barre syndrome, SLE (very rare)
Toxic/Metabolic: less likely, periodic paralysis, hypokalemia
Trauma/Degenerative: possible,intervertebral disk disease or spinal stenosis,
Congenital: less likely
Iatrogenic: less likely
Idiopathic: less likely,amyloidosis

Cauda equina syndrome is likely in this case from problem #1 to #10.
#1 Numbness of the buttocks
#2 Rapid onset lower limb weakness, then paralysis of the lower limbs
#3 Reduced muscle tone
#4 Reflexes knee (L2-4) absent, ankle (S1) absent
#5 Light touch- decreased in lower limbs up to the umbilicus (Th10) region
#6 Vibratory sense was intact on the pelvic brim (S3) and right knee (L4) but absent further down
#7 Nociception and thermoreception were absent.
#8 Reduced anal tone (S2-4)
#9 Bowel and bladder disturbance
#10 Absence of joint position sense (posterior column)

Cauda equina syndrome is included in concept of epidural spinal cord compression (ESCC).

UpToDate 16.1 describes as follows:
Neopalastic ESCC is a common complication of cancer that causes pain and sometimes irreversible loss of neurologic function. In adults, the tip of the spinal cord usually lies at the L1 vertebral level; below this level, the lumbosacral nerve roots form the cauda equina, which floats in cerebrospinal fluid. With cauda equina lesions, the weakness is associated with depressed deep tendon reflexes in the legs.

My first question is as follows:
Can we interpret problem #5 Light touch- decreased in lower limbs up to the umbilicus (Th10) region as a finding of cauda equina syndrome?
UpToDate 16.1 describes as follows:
When a spinal sensory level is present, it is typically one to five levels below the actual level of cord compression. Saddle sensory loss is commonly present in cauda equina lesions, while lesions above the cauda equina frequently result in sparing of sacral dermatomes to pinprick.

Cauda equina syndrome can cause decreased light touch in lower limbs up to the umbilicus (Th10) region.

My second question is as follows:
Why she doesn't have back pain?

UpToDate 16.1 describes as follows:
Pain is usually the first symptom of ESCC, being present in 83 to 95 percent of patients at the time of diagnosis.
This patient didn’t complain back pain. But 5 to 17 percent of ESCC cases don’t disclose back pain.

Third my question is as follows:
What is the cause of her cauda equina syndrome?

UpToDate 16.1 describes as follows:
The three most common are prostate cancer, breast cancer, and lung cancer, each of which accounts for about 20 percent of cases.

This patient is woman and smoker.Vertebral or spinal cord tumor, metastasis of breast cancer, lung cancer, gastrointestinal cancers, or renal cell carcinoma, non-Hodgkin's lymphoma, plasmacytoma, or multiple myeloma must be differentiated.

Problem #13 Aorta mildly expansile is probably caused by #11 Hypertension.

Questions
2) What immediate test or tests would you perform and why?
MR imaging

UpToDate 16.1 describes as follows:
MR imaging offers several potential advantages over myelography.
It produces anatomically faithful images of the spinal cord and intramedullary pathology and is even more sensitive than radionuclide bone scans at identifying bony metastases. It can image the entire thecal sac regardless of whether a spinal subarachnoid block is present. It is not contraindicated with large brain metastases, thrombocytopenia, or coagulopathy. It spares the patient a lumbar puncture.

Questions
3) What anatomical problem is this from the history and physical examination?
From the history and physical examination, cauda equina syndrome is likely. Neoplastic disease or degenerative disease including spinal canal stenosis might be the cause of L1 or L2 vertebral level compression.

Questions
4) What is the current evidence based treatment for such a condition?
Administration of corticosteroids is first strategy. Next surgical management with anterior decompression should be considered. If diagnosis is neplastic, radiotherapy or chemotherapy should be planned.

UpToDate 16.1 describes as follows:
Management include the administration ofcorticosteroids(dexamethasone ), followed either by surgery and/or radiotherapy (RT). Aggressive surgical management with anterior decompression appears to give better results than RT alone in patients who are candidates for surgical intervention. Compared to RT alone, surgery followed by RT is associated with better neurologic outcomes. Chemotherapy may be beneficial in patients with chemosensitive tumors.

Interestingly, both Dr Matsumura and Professor Lefor listed spinal cord ischaemia / infarction as part of their differential diagnoses from the history and physical examinations. This is the most important thing.

The case by itself without the advanced imaging studies is very difficult to make an accurate diagnosis and in this case, MRI did not show an obvious mass lesion or even the usual cord infarction pattern. However, with the history of rapid onset, paralysis and sensory loss affecting specific sensory modalities, and a non-diagnostic MRI scan, spinal cord infarction was still considered the most likely.

This was later confirmed by a most excellent neurologist.

Summary

In summary, spinal cord ischaemia is rare accounting for <1% of all strokes. Most spinal cord ishaemia occurs due to abdominal surgery. The spinal cord is supplied by one anterior spinal artery and two posterior spinal arteries. Superiorly the vessels originate from the vertebral arteries and they receive feeding radicular arteries from many levels with many coming from the intercostal arteries.
The Adamkiewicz artery is most commonly affected. Studies have shown that the most probable cause of infarcts is related to mechanical stress affecting the radicular arteries which can extend for several levels. Vertebral body infarcts can also occur and are associated with cord infarction and can be another sign of radicular artery occlusion.

There is an association of spinal cord ischaemia and spinal disease and in such chronic compressive diseases there is impairment of blood flow which can impair collateral blood supplies putting at risk the compensatory mechanisms in the event of ischaemia.

Transient ischaemic attacks affecting the spinal cord can also occur.

Clinical Features Associated with Various Types of Spinal Cord Ischaemia

Anterior spinal artery territory- bilateral motor impairment with spinothalamic impairment

Posterior Spinal artery territory- bilateral motor impairment with dorsal column sensory impairment

Complete [transverse] infarct- Bilateral motor impairment with total sensory loss at and below the area of the ischaemia.

Unilateral infarcts produce a hemiparesis, in the case of anterior impairment, a contralateral spinothalamic deficit and with a posterior impairment, a unilateral dorsal column impairment.

[please review the neuroanatomy of the various sensory pathways-- spinothalamic neurones cross over at the level of entry and traverse to the contralateral side and ascend in the lateral spinothalamic tracts whereas the dorsal column input ascends directly from the site of entry and cross over in the brainstem region. The routes of these two pathways explain why there is a difference in the sensory impairment within the spinal cord and different findings on physical examination].

There are no clear guidelines for treatment as there have been no prospective clinical studies and various anti-thrombotic / anti-coagulation measures have been tried in previous cases. In my opinion, a prospective study seems unlikely to be able provide a clear answer because of the paucity of such strokes.

A Classic Sign-- the Spider Naevus

Dear Bloggers

I apologise for not writing this week. I have been very busy indeed!! I was in Hokkaido teaching junior doctors the art of history taking and physical examination.

Here is a video that was taken some time ago of a patient with chronic liver disease from alcohol excess, which shows the classical sign of a spider naevus / vascular spider.

As can be seen, when the lesion is pressed, the blood is pushed out and when the finger pressure is released, the vascular lesion fills from the centre and spreads outwards. There is a central red area which is the feeding vessel. This is classical !!

Spider naeva are commonly found in chronic liver disease, pregnancy and in patients using oestrogen steroids. They are induced by the high level of oestrogens.

As for the answer to the recent case, I have deferred this until next week. Sorry ! :-0

Have a great weekend!

A Helpful Way to Identify Patient Deterioration Early!

Dear Bloggers

As physicians, we have all been called urgently to attend the patient who has been deteriorating over several days and who is peri-arrest.

An imaginary example of a typical patient vignette includes the orthopaedic patient with a hip fracture, recently operated and with worsening urine output, low fluid intake, increasingly rapid pulse and onset of confusion. Nursing staff have been charting the deterioration in parameters but were unaware of when to tell the surgical doctor because the doctor is always in the operating theatre treating trauma patients.

Another case vignette might be the patient admitted with general deterioration and weight loss with worsening breathlessness, but the junior doctor is on-call in the Emergency Department and has no time to come and see the patient and the nursing staff are unable to pinpoint the problem and have no idea how to help the patient except to give more oxygen.

These types of patient problems do occur in real life and unfortunately more often than one would perhaps envisage. From the two case vignettes, the underlying problem is not initially acute. There has been a chronic or subacute progression leading to deterioration in parameters of the patient thereby leading to a worsening state and hence, peri-arrest.

Sometimes the doctor does not pick up the problem either because of inexperience, infrequent visits to the patient, failure to adequately examine the patient rather than just the area of their specialty interest e.g. the broken hip, failure to ask the patient about new problems and symptoms, failure by nursing staff to adequately measure the daily observations, failure to report problems to medical staff because of inexperience or over work.

However, such problems cannot always be avoided in hospitals although it is possible to measure patient observations and enter them into a Scoring System which involves the patient's pulse, blood pressure, temperature, respiratory rate and AVPU score. The number generated by the parameters provides a total score which can then determine if the patient is at increased risk of deterioration and death.

Such a scoring system is referred to as an Early Warning System (EWS) and nursing staff can calculate this at the bedside and call the doctor in charge of the patient to attend the patient immediately for reassessment to see if changes are required to the patient's therapy to prevent further deterioration.

Many UK hospitals use such a system and it can lead to very appropriate changes in treatment.

Some hospitals have Intensive Therapy Unit 'Out-Reach' teams of highly experienced nurses that patrol the wards for such patients who are deteriorating with high EWS scores and they can make assessments and call the ITU specialists physicians for further help in addition to the Attending Physician so that an appropriate plan of action can be instituted such as moving the patient to the HCU / ICU for further treatment or the decision of appropriateness of active treatment versus palliative treatment, etc.

An interesting study using this EWS scoring system was published by the Oxford University Press several years ago in respect of all new acute admission on a Medical Assessment Unit with validation of a modified EWS. The results are interesting and show how changes were made to patients treatment in an attempt to reduce mortality and provide more appropriate care. The link to paper is here. Please read it and make up your own minds.

In several hospitals I have visited in Japan, I have not seen any equivalence to an EWS scoring system or an Out-Reach team. With many hospitals in Japan having an electronic patient system [Denshi Kalte], such a scoring system could be entered by the nurses with an immediate score produced for the patient with advice to the nurse whether a call to the doctor is warranted.

Such an idea is not new and not labour intensive and can help with patient management.

In the UK and USA amongst other western countries, there has been increasing training of specialist nurses in all specialties who can perform various important functions to aide patient care e.g. gastroenterology nurses, rheumatology nurses, dermatology nurses, Emergency specialist nurses, ITU specialist nurses etc... Such a common place nurse specialist is not so common place in Japan with there still being over-reliance on doctors to carry out almost all assessments and interventions when there is an unfortunate lack of doctors in various hospitals who cannot plug all the leaking holes in the wall.

With increased nursing and physician training, awareness of an Early Warning System and provision of a modicum of responsibility for nurses to intervene in patient treatment decisions, the care of acutely ill patients and deteriorating patients could in my opinion be improved and this is borne out by the evidence from several UK studies, one reference that I have also provided above.

If such a system could be implemented in all hospitals in Japan, there might conceivably be a reduction in sudden cardiac arrests and overall mortality as problems could be dealt with that much earlier.

This would certainly make an interesting study in Japan if anyone is interested by this idea!

Please consider!!!

Another interesting problem!

Dear Bloggers

This anonymised case is a warning for all of you out there when you see someone with leg weakness and numbness to appreciate how rapidly problems can progress. Please try and answer the case as best you can and you are welcome to leave your answers anonymously.

A 70 year old female who was otherwise well was admitted following a short history of

• numbness of the buttocks
• rapid onset lower limb weakness
  

The patient had got up for breakfast normally and was sitting eating her breakfast. She suddenly began to notice numbness of her buttock region and a 'heavy' feeling in her lower back. She decided to visit the toilet as she thought she might pass stool. In the toilet the sensation continued and after several minutes, the patient was unable to stable from the toilet seat because of leg weakness. Her family were concerned and called an ambulance and the patient was taken to the local hospital.

The patient denied any back pain. There was no history of vertebral disc problems, no chest pain or abdominal pain. There were no palpitations. The patient denied symptoms of diabetes such as excessive thirst, blurred vision etc. There was no history of being previously unwell and there were no symptoms consistent with infection or bleeding.

The patient was a known hypertensive and took atenolol 25mg once daily.

She had no drug allergies. She smoked 15 cigarettes per day for 40 years and she consumed small quantities of alcohol occasionally.

On examination

The patient seemed otherwise well. She was afebrile. Pulse 64/min regular. BP 140/80. RR- 16/min. Sats 98% on room air.

CVS: Heart sounds normal. Regular rhythm. No added sounds or murmurs. No peripheral oedema.

RESP: trachea central. Percussion resonant. Breath sounds vesicular.

ABDO: soft, non-tender, no hepatosplenomegaly, bowel sounds normal. No bruits. Aorta mildly expansile

CNS: Cranial nerves 2-12 normal.

PNS:

RUL LUL RLL LLL

Tone Normal Normal Normal Normal
Power 5/5 5/5 4+/5 4+/5
Reflexes
Biceps Normal Normal
Supinat Normal Normal
Triceps Normal Normal
Knee Normal Normal
Ankle Normal Normal

Sensation
Light touch- normal in upper limbs, decreased in lower limbs around ankles and buttocks.

Babinski sign was normal bilaterally.

Coordination of the lower limbs was not performed.

Vertebral examination was non-tender.

Over several hours the neurology worsened:

RUL LUL RLL LLL

Tone Normal Normal Reduced Reduced
Power 5/5 5/5 3/5 3/5
Reflexes
Biceps Normal Normal
Supinat Normal Normal
Triceps Normal Normal
Knee Absent Absent
Ankle Absent Absent

Babinski sign became negative bilaterally.

Sensation
Light touch- normal in upper limbs, decreased in lower limbs up to the umbilicus (T10) region. Vibratory sense was intact on the pelvic brim and right knee but absent further down. Nociception and thermoreception were absent. Joint position sense was not performed.

Anal tone was reduced with an absent anal 'wink' sign.

In fact, the weakness progressed further resulting in paralysis of the lower limbs and bowel and bladder disturbance. However, light touch and vibratory sense were maintained although nociception and thermoreception remained absent. Joint position sense was interestingly absent.

Questions

1) From history and examination, list the problems with this patient.

2) What immediate test or tests would you perform and why?

3) What anatomical problem is this from the history and physical examination?

4) What is the current evidence based treatment for such a condition?

The answer to this fascinating case will be published in the near future.