A jaundiced 35 year old with late quadriparesis


MRI T2/FLAIR on day 17

A 36 year old alcoholic man was admitted to hospital with obtunding, jaundice and cachexia. In addition to deranged LFTs, anaemia and thrombocytopenia, all thought to be related to cirrhosis, he had Creat 194 (eGFR 36), Na 138, K 2.6. He was treated for alcohol withdrawal but was difficult to manage; for a few days he was intubated and ventilated. Lactulose was thought to blame for very profuse diarrhoea, and he developed significant electrolyte disturbance. Serum sodium rose to 158 over 30 hours at one point; then slowly improved with water hydration over several days. Potassium rose to 3.4 then dropped back to 2.4 before slowly returning to normal.

5 days later his conscious level reduced again and he developed spastic quadriplegia. An MRI scan later that day is shown above.

Question: what is the likely caused of this problem? Could anything have been done to prevent it?

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What the experts thought ...

This is osmotic demyelination (old name: Central Pontine Myelinolysis) but it is a very unusual example caused by rapid onset of hypernatraemia. Usually it is attributed to too-rapid correction of very low plasma [Na], but presumably the principle is the same. A few principles.

  • The hyponatraemia needs to be of some duration (not acute).
  • The delayed onset of symptoms, 2-6d after the peak change in sodium, is typical.
  • A ‘safe rate’ for correction of chronic hyponatraemia is said to be less than 8 mmol every 24h (up to 10 mmol quickly for severe acute symptoms). (UpToDate)(UoE login required).
  • Risk factors for its occurrence include alcohol abuse, liver disease, malnutrition, pre-existing brain disease. It is also much more likely if starting sodium is <120.

What happened? He is said to have made a good neurological recovery over a week or so. This is not always the case.

Further info

This case is adapted from Am J Kid Dis 68(5):xv-xvii (2016). The image is courtesy of Dr Lemuel Marquez Narcise, Radiopaedia.org, from the case rID: 44204

A breathless 20 year old with cough and fever

A 20 year old man complains of 3 weeks of cough and fever, and 4 days of oedema of his feet. On examination he is breathless.  There is marked engorgement of his neck veins, JVP higher than ears, ascites, 2+ oedema of his feet. P130/min irregularly irregular, of waxing and waning volume, apex rate 160. BP 95/80. Liver 4cm. There was dullness and reduced breath sounds at R lung base. His chest radiograph is shown.

ECG showed inverted T waves across anterolateral chest leads.

Questions

What is the likely diagnosis. What complication sounds imminent, and what would you do about it. What is the most likely cause?

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Show what happened next

He has a pericardial effusion with tamponade. 500 mls of bloody fluid were drained from the pericardium, with immediate improvement in breathlessness and blood pressure. 1000 ml of similar fluid was drained from the right pleural cavity. Microscopy did not show a cause.

The effusion recurred a week later, so a pericardial biopsy was undertaken. Don’t try this at home.

A week later the problem recurred. A biopsy was undertaken.

What might it show?

Show the outcome

The fluid again did not give a diagnosis, but the pericardial biopsy showed giant cell granulomas. A guinea pig was inoculated with the fluid, and 5 weeks later it had developed tuberculous lesions, confirming the diagnosis.

TB is a rare cause in UK practice. Viral causes are probably the most common cause of symptomatic acute pericarditis, followed by autoimmunity. However big effusions causing tamponade are probably most likely after cardiac surgery or myocardial injury (Dressler Syndrome), and in malignancy. Infections are a less common cause.

Further info

This case is from Sanghvi et al 1958, Sawai Man Singh Hospital and Medical College Rajasthan (Pericardial biopsy with Vim-Silverman needle, Archives of Internal Medicine 101:1147-10). Pushing a big biopsy needle towards the heart sounds hazardous, and probably fortunately the approach didn’t catch on. Guinea pig inoculation was a quicker, less demanding technique than TB cultures, though these had been developed in the 1930s.

Pericarditis from TB is rarely encountered in developed countries now, but still not rare worldwide. Drug resistance, and co-infection with HIV, are important issues. Surgery may be required in chronic examples, but is not always available in the areas of highest incidence.

Further further info

History of tuberculosis (Wikipedia) and Timeline of tuberculosis (Wikipedia) are both fascinating.

The image shows a modified Vim Silverman needle, the predecessor of the ‘Trucut’ cutting needle developed for liver biopsies, extended in the mid 1950s to renal biopsies (Renal biopsy becomes mainstream, 1954).