Intracranial and systemic manifestations of familial leptomeningeal amyloidosis, as seen on CT and MRI

Intracranial and systemic manifestations of familial leptomeningeal amyloidosis, as seen on CT and MRI

Leptomeningeal amyloidosis is a subset of familial transthyretin amyloidosis, a family of diseases occurring in conjunction with multiple known mutations of the transthyretin gene. Though this is primarily a disease of the central nervous system, amyloid deposition is multisystemic. We describe a ca...

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Main Authors: Saralyn Beckius, BS, Kamran Shah, MD
Format: Article
Language:English
Published: Elsevier 2018-12-01
Series:Radiology Case Reports
Online Access:http://www.sciencedirect.com/science/article/pii/S1930043318302164
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spelling doaj-9a45786edf01421ebce530807b2bc6a92020-11-24T21:04:00ZengElsevierRadiology Case Reports1930-04332018-12-0113611791184Intracranial and systemic manifestations of familial leptomeningeal amyloidosis, as seen on CT and MRISaralyn Beckius, BS0Kamran Shah, MD1Wayne State University School of Medicine, 540 E Canfield St., Detroit, MI 48201, USA; Corresponding author.Department of Radiology, Wayne State University School of Medicine, 540 E Canfield St., Detroit, MI 48201, USA; Department of Radiology DMC Detroit Receiving Hospital 3L-8, 4201 St Antoine St., Detroit, MI 48201, USALeptomeningeal amyloidosis is a subset of familial transthyretin amyloidosis, a family of diseases occurring in conjunction with multiple known mutations of the transthyretin gene. Though this is primarily a disease of the central nervous system, amyloid deposition is multisystemic. We describe a case of a 61-year-old man with known central nervous system amyloidosis presenting to the emergency room with stroke-like symptoms, including left hemineglect, right gaze paresis, and left hemiplegia, atop baseline dementia. A noncontrast CT head demonstrated ventriculomegaly and no acute hemorrhage. Urinalysis indicated an underlying urinary tract infection, ultimately believed to have prompted a breakthrough seizure. Electroencephalogram revealed diffuse encephalopathy. Contrast-enhanced MRI demonstrated hallmarks of intracranial amyloid with no new infarct. Previously taken noncontrast CT neck and thorax demonstrated evidence of systemic disease. Keywords: Amyloid, MRI, CT, Deposition, Intracranial, Pulmonaryhttp://www.sciencedirect.com/science/article/pii/S1930043318302164
collection DOAJ
language English
format Article
sources DOAJ
author Saralyn Beckius, BS
Kamran Shah, MD
spellingShingle Saralyn Beckius, BS
Kamran Shah, MD
Intracranial and systemic manifestations of familial leptomeningeal amyloidosis, as seen on CT and MRI
Radiology Case Reports
author_facet Saralyn Beckius, BS
Kamran Shah, MD
author_sort Saralyn Beckius, BS
title Intracranial and systemic manifestations of familial leptomeningeal amyloidosis, as seen on CT and MRI
title_short Intracranial and systemic manifestations of familial leptomeningeal amyloidosis, as seen on CT and MRI
title_full Intracranial and systemic manifestations of familial leptomeningeal amyloidosis, as seen on CT and MRI
title_fullStr Intracranial and systemic manifestations of familial leptomeningeal amyloidosis, as seen on CT and MRI
title_full_unstemmed Intracranial and systemic manifestations of familial leptomeningeal amyloidosis, as seen on CT and MRI
title_sort intracranial and systemic manifestations of familial leptomeningeal amyloidosis, as seen on ct and mri
publisher Elsevier
series Radiology Case Reports
issn 1930-0433
publishDate 2018-12-01
description Leptomeningeal amyloidosis is a subset of familial transthyretin amyloidosis, a family of diseases occurring in conjunction with multiple known mutations of the transthyretin gene. Though this is primarily a disease of the central nervous system, amyloid deposition is multisystemic. We describe a case of a 61-year-old man with known central nervous system amyloidosis presenting to the emergency room with stroke-like symptoms, including left hemineglect, right gaze paresis, and left hemiplegia, atop baseline dementia. A noncontrast CT head demonstrated ventriculomegaly and no acute hemorrhage. Urinalysis indicated an underlying urinary tract infection, ultimately believed to have prompted a breakthrough seizure. Electroencephalogram revealed diffuse encephalopathy. Contrast-enhanced MRI demonstrated hallmarks of intracranial amyloid with no new infarct. Previously taken noncontrast CT neck and thorax demonstrated evidence of systemic disease. Keywords: Amyloid, MRI, CT, Deposition, Intracranial, Pulmonary
url http://www.sciencedirect.com/science/article/pii/S1930043318302164
work_keys_str_mv AT saralynbeckiusbs intracranialandsystemicmanifestationsoffamilialleptomeningealamyloidosisasseenonctandmri
AT kamranshahmd intracranialandsystemicmanifestationsoffamilialleptomeningealamyloidosisasseenonctandmri
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