Subacute sclerosing panencephalitis presenting as severe depression in an adult.

Subacute sclerosing panencephalitis (SSPE) is a fatal disorder that occurs as a rare complication of childhood measles. Symptoms typically manifest between the ages of 5 and 15. While the incidence of SSPE is declining globally, it is still prevalent in regions where measles remains common and vaccination rates are low due to poverty and lack of health education. Diagnosing SSPE can be challenging, particularly when patients exhibit unusual symptoms. A thorough clinical evaluation, including vaccination history, physical examination, electroencephalogram (EEG) and Cerebrospinal fluid (CSF) analysis, can help in making a diagnosis. We present the case of a young woman in her early 20s who initially experienced depressive symptoms, followed by myoclonus, dementia and visual impairment. The patient was ultimately diagnosed with SSPE based on characteristic EEG findings, neuroimaging results, CSF analysis and elevated serum measles antibody levels.

Long-acting porcine ACTH stimulated salivary cortisol reduces the overdiagnosis of adrenal insufficiency compared to serum cortisol in cirrhosis liver.

BACKGROUND: There are no reliable methods in clinical practice to diagnose adrenal insufficiency (AI) in patients with cirrhosis owing to variable cortisol-binding protein levels. This leads to unreliable results in ACTH stimulated serum cortisol test. We aimed to estimate the long-acting porcine (LA)ACTH-stimulated serum and salivary cortisol levels of patients at different stages of cirrhosis using second generation electrochemiluminescence and to determine the prevalence of true adrenal insufficiency in these patients. DESIGN, PATIENTS AND MEASUREMENTS: We included 135 noncritical patients with cirrhosis (45 each from CHILD A, B and C) and 45 healthy controls. Serum and salivary samples were collected at baseline in the morning and at 1 and 2 h after LA-ACTH injection. RESULTS: In healthy subjects, the 2.5th centile of 2 h ACTH stimulated serum and salivary cortisol were 19.8 and 0.97 µg/dL, which were used as cut-offs for defining AI based on serum and saliva respectively. The median (interquartile-range) 2-h stimulated salivary cortisol in Child A, B, C categories and controls were 1.36(1.23-2.38), 1.46(1.18-2.22), 1.72(1.2-2.2) and 2.12(1.42-2.72) µg/dL respectively. Six subjects (4.4%) were diagnosed to have AI based on stimulated salivary cortisol cut-off, whereas 39 (28.9%) cirrhosis subjects had inadequately stimulated serum cortisol. Three patients (symptomatic) required steroid replacement therapy. Hypoalbuminemia was identified as a major risk factor for the misdiagnosis of adrenal insufficiency by serum cortisol-based testing. CONCLUSIONS: Long-acting porcine ACTH stimulated salivary cortisol reduces the overdiagnosis of adrenal insufficiency compared to serum cortisol in cirrhosis liver. Stimulated salivary cortisol is a promising investigation for evaluation of adrenal function in cirrhosis and more studies are required for its further validation before clinical use.

Diabetic striatopathy in type 1 diabetes mellitus: a rare conundrum.

Diabetic striatopathy is a clinicoradiological syndrome characterised by acute hyperkinetic movement disorder in the form of hemichorea-hemiballism with basal ganglia abnormalities in neuroimaging. The hallmark basal ganglia abnormalities appear as hyperdensities in CT brain and hyperintensities in MRI brain, which could mislead the clinician towards an erroneous diagnosis of cerebral haemorrhage. It is classically described in elderly patients with type 2 diabetes mellitus, and its occurrence in type 1 diabetes is extremely rare. This case report entails the clinical details of a young man in his 20s with type 1 diabetes mellitus who had uncontrolled blood glucose levels and presented with a recent onset of abnormal movements in his left upper and lower limbs. The semiology, biochemistry and radiological investigation findings and treatment are detailed. A clear understanding of the condition could lead to an early diagnosis, spare the patient unnecessary investigations and improve treatment outcomes.

Isolated muscular sarcoidosis presenting as hypercalcaemic renal failure.

The case report describes the details of a man in his 40s admitted for evaluation of renal failure. Biochemical testing revealed parathyroid hormone (PTH) -independent hypercalcaemia. The evaluations for the usual causes such as malignancies, granulomatous diseases, multiple myeloma and vitamin D toxicity were negative. Fluorodeoxyglucose Positron Emission Tomography (FDG-PET) scan identified diffuse uptake in the muscles, and the subsequent muscle biopsy showed non-caseating granulomas suggestive of granulomatous myositis, possibly sarcoidosis, in view of raised ACE levels and the absence of other causes. The patient showed a dramatic response to glucocorticoids, with prompt relief of symptoms and normalisation of serum calcium and creatinine. The case highlights the importance of considering sarcoid myositis in the evaluation of hypercalcaemia and the need for prompt initiation of glucocorticoid therapy to achieve favourable outcomes. The successful use of FDG-PET in diagnosing PTH-independent hypercalcaemia suggests its potential as a valuable tool in the diagnostic algorithm for this condition.