My mom recently had a bleeding stroke. The underlying cause is presumed to be amyloid angiopathy.
Her recent MRI showed "multiple punctate foci of dephasing artifacts within a small region of subarachnoid space along the left frontal lobe.... which may indicate leptomeningeal amyloidosis (LA)."
Information on leptomeningeal amyloidosis seems to all about hereditary amyloidosis, but i can't tell if its always hereditary, or is that just one form of LA?
Amyloidosis is deposition of amyloid or insoluble protein in extracellular space. This can affect any body organ including brain. This may or may not be hereditary. The hereditary amyloidoses are inherited through autosomal dominant traits, which usually involve the transthyretin (TTR) gene. This usually presents as a systemic form of amylodosis where amyloid is deposited in various organs of the body, and not selectively in brain.
Leptomeningeal amyloidosis is a rare variation of hereditary TTR amyloidosis, in which amyloid deposition occurs preferentially in the central nervous system, typically involving the pia arachnoid and subpial area, and walls of leptomeningeal vessels. Some patients may experience visual symptoms due to deposition of amyloid in the vitreous space of the eye. This is called oculoleptomeningeal amyloidosis.
So, to answer your question, yes, this is rare form of hereditary amyloidosis.
I sincerely hope you will find this information useful. Good Luck and take care!
That doesn't really answer my question. I understand that leptomeningial amyloidosis describes the location of the amyloid deposits and that there is a hereditary form where this occurs. The question is do leptomengial amyloidosis ever occur in other forms?
Yes, it can be part of a systemic amyloid deposit, but if amyloid deposits are exclusively in brain and the above mentioned areas ( pia arachnoid and subpial area, and walls of leptomeningeal vessels) then it is hereditary, unless proved otherwise.
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