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@ARTICLE{Zimmermann:154279,
author = {Zimmermann, Milan and Deininger, Natalie and Willikens,
Sophia and Haack, Tobias B and Grundmann-Hauser, Kathrin and
Streubel, Berthold and Schreiber, Melanie and Lerche, Holger
and Grimm, Alexander},
title = {{T}etraparesis and sensorimotor axonal polyneuropathy due
to co-occurrence of {P}ompe disease and hereditary {ATTR}
amyloidosis.},
journal = {Neurological sciences},
volume = {42},
number = {4},
issn = {1590-3478},
address = {Milano},
publisher = {Springer},
reportid = {DZNE-2021-00133},
pages = {1523 - 1525},
year = {2021},
note = {ISSN 1590-3478 not unique: **3 hits**.},
abstract = {Hereditary transthyretin amyloidosis with polyneuropathy
(hATTRPN) is an autosomal dominant multi-organ disorder
manifesting in the third to fifth decade with the key
clinical features of distal and painful sensory loss of the
lower limbs and autonomic dysregulation. Motor neuropathy
and cardiomyopathy evolve in the course of the disease.
Pompe disease is an autosomal recessive disease leading to
decreased levels of lysosomal enzyme acid α-glucosidase and
proximal muscle weakness. We report the clinical features
and diagnostic workup in the rare case of a patient with
ATTR amyloidosis and late-onset Pompe disease, both
genetically confirmed.We performed a detailed clinical
assessment, exome sequencing, and biochemical
measurements.The patient presented with a distal, painful
hypaesthesia of both legs, a cardiomyopathy, and a muscle
weakness in the form of a girdle-type pattern of the arms
and legs at the beginning and a spreading to distal muscle
groups in the course of disease.This study highlights the
importance of searching for co-occurrence of rare
monogenetic neuromuscular diseases, especially in cases in
which all clinical features can be readily explained by a
single gene defect.},
keywords = {Aged / Aged, 80 and over / Amyloid Neuropathies, Familial:
complications / Amyloid Neuropathies, Familial: genetics /
Cardiomyopathies / Glycogen Storage Disease Type II:
complications / Glycogen Storage Disease Type II: genetics /
Humans / Male / Middle Aged / Polyneuropathies / Prealbumin
/ Amyloidosis (Other) / Axonal degeneration (Other) /
Polyneuropathy (Other) / Pompe disease (Other) /
Transthyretin (Other)},
cin = {Tübingen common},
ddc = {610},
cid = {I:(DE-2719)6000018},
pnm = {352 - Disease Mechanisms (POF4-352)},
pid = {G:(DE-HGF)POF4-352},
typ = {PUB:(DE-HGF)16},
pubmed = {pmid:33188503},
pmc = {pmc:PMC7955998},
doi = {10.1007/s10072-020-04896-3},
url = {https://pub.dzne.de/record/154279},
}