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No Abstract available.
We present the neuropathological findings in a 75-year-old man who had the clinical diagnosis of amyloidosis restricted to the heart, which was confirmed by biopsy. The patient died of cardiac insufficiency in the context of arrhythmia. General autopsy revealed amyloid deposits in the heart and additionally in the lung, kidney, thyroid gland, esophagus, pancreas, liver, spleen, periumbilical fat tissue, and rectum.
In the brain, prominent amyloid deposits were restricted to the vessel walls of the choroid plexus (Figure 1A). There were no deposits in the meninges, CNS parenchyma, or the nerve roots of brainstem. Amyloid deposits were intensely congophilic (Figure 1B, C), birefringent under polarized light, and thioflavin-positive (Figure 1D, arrow). Amyloid deposits were immunoreactive for α- and κ-light chain (Figure 1E), but negative for transthyretin (Figure 1F), amlyoid A, βA4-amyloid, and β2-microglobulin.
In generalized amyloidoses, amyloid deposits in the CNS have been found in regions were the blood brain barrier is insufficient. This is the case in the choroid plexus, infundibulum, pineal gland, area postrema (representing circumventricular organs), ganglion Gasseri, and dura mater [1, 2], and suggests a hematogenic pattern of spread . Other regions of the brain, such as leptomeninges and brain parenchyma, are devoid of these amyloid deposits, in contrast to what is observed in classical βA4-amyloidosis such as Alzheimer’s disease.
The authors report no conflict of interest.