Blackout at Rest and Slurring in a Man Afraid of COVID-19

Ankit Raiyani, MBBS, MD, DNB (Hematology)

Disclosures

March 03, 2021

Discussion

Systemic amyloidosis is a rare disease that occurs when an abnormal protein called amyloid builds up in vital organs and interferes with their normal function. Amyloid is not normally found in the body, but it can be formed from more than 30 different types of protein.[1]

Deposition of amyloid can occur under these conditions[1]:

Amyloid fibril can be deposited in any soft tissue. The most commonly affected organs in systemic amyloidosis are the heart, kidneys, liver, skin, and nervous system.[1]

The degree of involvement varies among these organs, so each patient may have a unique set of symptoms. A high degree of clinical suspicion is required for a timely diagnosis, particularly in the early stages of the disease.

The two major types of systemic amyloidosis are the AL type and the AA type. The most frequent type of amyloidosis in high-income countries is AL amyloidosis.[1] AL amyloidosis may or may not be associated with a concurrent plasma cell dyscrasia, such as multiple myeloma or monoclonal gammopathy of unknown significance. AA amyloidosis may occur with chronic inflammatory conditions (eg, rheumatoid arthritis) or chronic infections (eg, tuberculosis).

In the patient in this case, recurrent cardiac arrhythmias, low voltage on an ECG, congestive heart failure, and diastolic dysfunction can be explained by amyloid deposition in the heart. Edema, periorbital swelling, and hypoproteinemia may be due to nephrotic-range proteinuria, which results from renal deposition of amyloid fibrils. Frequent loose bowel movements and postural hypotension may be caused by autonomic nervous system involvement. Macroglossia and the resultant slurring of speech may be attributed to amyloid deposition in the tongue. Easy bruising may be due to cutaneous involvement and/or acquired clotting factor X deficiency.

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