Ramsay Hunt Syndrome: A Herpes Zoster Infection

Ramsay Hunt syndrome is a condition characterized by a lower motor neurone facial palsy on one side of the face, often caused by herpes zoster infection of the geniculate ganglion. While a vesicular eruption in the external auditory canal, cranial integument, and oropharynx is typically present, it may not appear immediately or at all. The eighth cranial nerve may also be affected, leading to deafness and vertigo. The virus can be detected through exudates collected from the skin of the pinna and analyzed through PCR. Treatment with a combination of prednisolone and acyclovir is effective if started within seven days of symptom onset. Left-sided acoustic neuroma is unlikely due to the absence of hearing loss and tinnitus, while herpes simplex infection leads to cold sores rather than facial palsy. Atypical migraine may cause paralysis, but the absence of headache and other symptoms rules it out as a diagnosis.

The probable root cause of this condition is an atrial septal defect, which is often discovered incidentally in later life through the detection of RBBB. In infancy, a murmur may not be detectable, leading to a delayed diagnosis. Symptoms typically arise later in life as the right atrium enlarged, resulting in decreased cardiac efficiency. A fixed-split S2 is a typical finding in affected individuals.

Understanding Atrial Septal Defects

Atrial septal defects (ASDs) are a type of congenital heart defect that can be found in adulthood. They are associated with a high mortality rate, with 50% of patients dying by the age of 50. There are two types of ASDs: ostium secundum and ostium primum. Ostium secundum is the most common type, accounting for 70% of all ASDs.

ASDs can be identified by certain features, such as an ejection systolic murmur and fixed splitting of S2. They can also lead to embolisms passing from the venous system to the left side of the heart, which can cause a stroke.

Ostium secundum ASDs are often associated with Holt-Oram syndrome, which is characterized by tri-phalangeal thumbs. On an ECG, ostium secundum ASDs are typically identified by RBBB with RAD.

Ostium primum ASDs, on the other hand, present earlier than ostium secundum defects and are often associated with abnormal AV valves. On an ECG, they are typically identified by RBBB with LAD and a prolonged PR interval.

Understanding the different types of ASDs and their associated features can help with early identification and treatment, potentially improving outcomes for patients.

To diagnose SIADH in a euvolaemic patient with hyponatraemia and low serum osmolality, the criteria include Na < 135, serum osmolality <271, and urinary osmolality >100. Therefore, it is important to confirm elevated urinary osmolality. While a morning cortisol test can help diagnose Addison’s disease, a random serum cortisol test is not very useful. An echocardiogram may be necessary to rule out heart failure as a cause of hyponatraemia in the presence of fluid overload, but it is unlikely to be needed if there is no peripheral oedema, raised JVP, or pulmonary oedema.

SIADH is a condition where the body retains too much water, leading to low sodium levels in the blood. This can be caused by various factors such as malignancy (particularly small cell lung cancer), neurological conditions like stroke or meningitis, infections like tuberculosis or pneumonia, certain drugs like sulfonylureas and SSRIs, and other factors like positive end-expiratory pressure and porphyrias. Treatment involves slowly correcting the sodium levels, restricting fluid intake, and using medications like demeclocycline or ADH receptor antagonists. It is important to correct the sodium levels slowly to avoid complications like central pontine myelinolysis.

Importance of Continuous Chest Compressions in Cardiac Arrest

Current UK resuscitation guidelines highlight the significance of minimizing interruptions in chest compressions during a cardiac arrest. It is recommended to restart chest compressions immediately after each shock, before any other action is taken. The rhythm assessment and pulse check should be conducted after two minutes of continuous chest compressions. The guidelines also suggest a single shock strategy.

In advanced life support, adrenaline should be administered every 3-5 minutes, and amiodarone should be given after three shocks. Additionally, brief periods of echo (10 seconds) are now supported during an arrest situation, but it should be performed at the end of two minutes of compressions.

Therefore, the most appropriate immediate step in a cardiac arrest situation is to administer one shock and immediately restart chest compressions. This approach ensures that the patient receives continuous chest compressions, which is crucial for their survival.

Supportive therapy should be given alongside active treatment for this patient, as there is a low chance of spontaneous remission. However, supportive therapy alone is not appropriate. Although the patient recently traveled from a TB-endemic area, there is no indication of TB infection that requires anti-tuberculous medication. While the patient has risk factors for renal artery stenosis, the biopsy findings and presentation do not support this as the cause of renal function decline. Additionally, the kidneys are of normal size and have equal peak flow velocities on doppler. Therefore, revascularization is not the correct treatment. There is no indication of urgent need for hemodialysis in this patient.

Understanding Focal Segmental Glomerulosclerosis

Focal segmental glomerulosclerosis (FSGS) is a type of kidney disease that often leads to nephrotic syndrome and chronic kidney disease. It is commonly diagnosed in young adults and can be caused by various factors such as HIV, heroin, Alport’s syndrome, and sickle-cell. In some cases, it may also be idiopathic or secondary to other renal pathologies like IgA nephropathy or reflux nephropathy.

To diagnose FSGS, a renal biopsy is usually performed, which shows focal and segmental sclerosis and hyalinosis on light microscopy and effacement of foot processes on electron microscopy. If left untreated, FSGS has a low chance of spontaneous remission, with less than 10% of cases experiencing it.

Management of FSGS typically involves the use of steroids and immunosuppressants. However, it is important to note that FSGS has a high recurrence rate in renal transplants, making it a challenging condition to manage.

The appropriate management for primary hyperaldosteronism caused by bilateral adrenal hyperplasia is spironolactone, a mineralocorticoid receptor antagonist. Chemotherapy is not the correct answer as it is not the first-line treatment for this condition, but may be considered for disseminated malignancy caused by an adrenal carcinoma. IV hydrocortisone is not indicated as the patient’s cortisol levels are normal and there are no signs of hypoadrenalism. Radiotherapy is also not the correct answer as it is typically used for unilateral mass-like lesions, rather than bilateral diffuse enlargement.

Primary hyperaldosteronism is a condition characterized by hypertension, hypokalaemia, and alkalosis. It was previously believed that adrenal adenoma, also known as Conn’s syndrome, was the most common cause of this condition. However, recent studies have shown that bilateral idiopathic adrenal hyperplasia is responsible for up to 70% of cases. It is important to differentiate between the two causes as it determines the appropriate treatment. Adrenal carcinoma is an extremely rare cause of primary hyperaldosteronism.

To diagnose primary hyperaldosteronism, the 2016 Endocrine Society recommends a plasma aldosterone/renin ratio as the first-line investigation. This test should show high aldosterone levels alongside low renin levels due to negative feedback from sodium retention caused by aldosterone. If the results are positive, a high-resolution CT abdomen and adrenal vein sampling are used to differentiate between unilateral and bilateral sources of aldosterone excess. If the CT is normal, adrenal venous sampling (AVS) can be used to distinguish between unilateral adenoma and bilateral hyperplasia.

The management of primary hyperaldosteronism depends on the underlying cause. Adrenal adenoma is treated with surgery, while bilateral adrenocortical hyperplasia is managed with an aldosterone antagonist such as spironolactone. It is important to accurately diagnose and manage primary hyperaldosteronism to prevent complications such as cardiovascular disease and stroke.

AKI that occurs after angiography can be attributed to either contrast-induced nephropathy or cholesterol emboli. The most common cause is contrast-induced nephropathy, but if there is an elevated eosinophil count and skin rash (livedo reticularis), it is highly indicative of cholesterol emboli.

While acute interstitial nephritis can cause eosinophilia, it is not associated with contrast. ANCA-associated vasculitis, such as Churg Strauss, can cause a rash and eosinophilia, but there are no other symptoms like anemia or systemic upset that match this condition.

The elevated creatine kinase level suggests muscle injury, which aligns with an embolic phenomenon. However, the CK level is not high enough to diagnose rhabdomyolysis, which typically requires a level above 5000.

Cholesterol embolisation is a condition where cholesterol deposits break off and can lead to renal disease. This condition is commonly seen as a result of vascular surgery or angiography, but can also occur due to severe atherosclerosis, especially in large arteries like the aorta. Symptoms of cholesterol embolisation include eosinophilia, purpura, renal failure, and livedo reticularis.

Differential Diagnosis for a Febrile Illness with Signs of Consolidation and Meningitis

When a patient presents with a febrile illness and signs of consolidation in the lung, the most common cause is community-acquired pneumonia, often caused by Streptococcus pneumoniae. However, if the patient develops signs of bacterial meningitis and a deteriorating level of consciousness, further investigation is necessary. Herpes labialis is also commonly associated with S. pneumoniae infection.

Other potential causes of meningitis include Cryptococcus, which is more common in immunocompromised patients, and Listeria monocytogenes, which primarily affects pregnant women and the elderly. Staphylococcus aureus pneumonia is typically seen in conjunction with influenza infection.

Mycoplasma pneumoniae is a rarer cause of community-acquired pneumonia, but it can cause more severe changes on chest X-ray than would be expected based on the physical exam. In cases where meningitis is suspected, a CT scan should be performed to rule out raised intracerebral pressure and assess the risks of lumbar puncture. This can help confirm a diagnosis of S. pneumoniae meningitis and rule out potential herpes encephalitis.

There are four types of congenital adrenal hyperplasia (CAH) that can be differentiated based on their hormone profiles. The first type, 21-hydroxylase deficiency, is the most common and results in low cortisol and aldosterone levels, as well as elevated androgens. The second type, 11-beta hydroxylase deficiency, leads to elevated blood pressure due to some aldosterone activity from 11-deoxycortisol, as well as raised androgens. The third type, 17-alpha hydroxylase deficiency, results in raised aldosterone but low androgens. The fourth type, 3-beta steroid dehydrogenase, leads to low levels of both aldosterone and androgens.

The short synacthen test can be useful in cases where 17OH-progesterone levels are only slightly elevated. If levels are very high, it can be diagnostic for CAH. Measuring 17 OH-progesterone at 0 and 60 minutes after ACTH administration can cause elevated responses in patients with CAH, with levels exceeding 35 nmol/L.

Congenital adrenal hyperplasia is a group of genetic disorders that affect the production of adrenal steroids. These disorders are inherited in an autosomal recessive manner. The low levels of cortisol in response to this condition cause the anterior pituitary gland to secrete high levels of ACTH. This, in turn, stimulates the production of adrenal androgens that can cause virilization in female infants. The most common cause of congenital adrenal hyperplasia is 21-hydroxylase deficiency, accounting for 90% of cases. 11-beta hydroxylase deficiency is responsible for 5% of cases, while 17-hydroxylase deficiency is very rare.

In many countries, newborns are screened for congenital adrenal hyperplasia by measuring the serum concentration of 17-hydroxyprogesterone (17 OHP). However, this screening is not yet done in the UK. To confirm the diagnosis, ACTH stimulation testing is used. This involves administering synthetic ACTH and measuring the levels of cortisol and other adrenal steroids in the blood before and after the administration. This test helps to determine the type and severity of the condition.

Diagnosis of Ascending Cholangitis and Differential Diagnosis of Extrahepatic Duct Dilatation

Ascending cholangitis is the most likely diagnosis in a patient with extrahepatic duct dilatation and evidence of obstruction on ultrasound scanning. While gallstones may not be visualized on ultrasound, MRCP is superior for diagnosing and locating them with a sensitivity of around 93%. ERCP and sphincterotomy may be necessary in the acute setting if sepsis does not resolve with antibiotics. Cholecystitis is an unlikely explanation for extrahepatic duct dilatation, and a cholangiocarcinoma at the porta hepatis would not explain it either. While a tumour at the ampulla of Vater or in the head of the pancreas could cause distal biliary obstruction and ascending cholangitis, they are more likely to present with obstructive jaundice, weight loss, and dull abdominal pain.