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Tongue Stiffness as Presentation of Antiphospholipid Syndrome: A Case Report

Antiphospholipid syndrome (APS) is a systemic autoimmune disorder with marked thrombotic and inflammatory features driven by the presence of antiphospholipid antibodies (APLA). Here, we report a case of APS with a rare, atypical manifestation and discuss a differential diagnosis. A 53-year-old male...

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Autores principales: Rajevac, Hana, Steed, Kelly
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Cureus 2020
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7358906/
https://www.ncbi.nlm.nih.gov/pubmed/32670719
http://dx.doi.org/10.7759/cureus.8584
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author Rajevac, Hana
Steed, Kelly
author_facet Rajevac, Hana
Steed, Kelly
author_sort Rajevac, Hana
collection PubMed
description Antiphospholipid syndrome (APS) is a systemic autoimmune disorder with marked thrombotic and inflammatory features driven by the presence of antiphospholipid antibodies (APLA). Here, we report a case of APS with a rare, atypical manifestation and discuss a differential diagnosis. A 53-year-old male without significant past medical history presented with new onset of episodic tongue stiffness and dysarthria which lasted for about a minute over a period of three months. This was associated with intermittent right retro-orbital sharp pain radiating to the parietal area. He also reported swelling and stiffness of the third and fourth right proximal interphalangeal (PIP) joints lasting throughout the day. A physical exam revealed tongue fasciculations. As the MRI showed patchy white matter hyperintensities neurology initially suspected multiple sclerosis. However, cerebrospinal fluid (CSF) analysis including neuromyelitis optica (NMO) antibodies and oligoclonal antibodies was negative. Rheumatological work up was remarkable for positive antinuclear antibodies (ANA); anticardiolipin antibodies and lupus anticoagulant were positive 12 weeks apart. This, alongside with stable white matter changes on imaging was suspicious for an extra-criteria manifestation of antiphospholipid antibody syndrome. The most commonly described neurological manifestations of APS are headache, transient ischemic attack (TIA), and stroke. Tongue stiffness as an initial symptom is quite unusual and, to the best of our knowledge has not been reported in medical literature. In patients with isolated neurological findings of unclear etiology, an autoimmune disease such as APS should be considered, and appropriate diagnostic work up should not be postponed. Unfortunately, positive laboratory markers can have a wide differential diagnostic panel. In addition, APS may mimic many diseases both in clinical presentation and MRI findings thus making the correct diagnosis challenging. However, studies show that, unlike multiple sclerosis (MS), white matter changes in APS remain static during the course of the disease. Identification of atypical presentations of APS is critical as prompt and correct medical management can improve patients’ quality of life and clinical outcomes.
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spelling pubmed-73589062020-07-14 Tongue Stiffness as Presentation of Antiphospholipid Syndrome: A Case Report Rajevac, Hana Steed, Kelly Cureus Internal Medicine Antiphospholipid syndrome (APS) is a systemic autoimmune disorder with marked thrombotic and inflammatory features driven by the presence of antiphospholipid antibodies (APLA). Here, we report a case of APS with a rare, atypical manifestation and discuss a differential diagnosis. A 53-year-old male without significant past medical history presented with new onset of episodic tongue stiffness and dysarthria which lasted for about a minute over a period of three months. This was associated with intermittent right retro-orbital sharp pain radiating to the parietal area. He also reported swelling and stiffness of the third and fourth right proximal interphalangeal (PIP) joints lasting throughout the day. A physical exam revealed tongue fasciculations. As the MRI showed patchy white matter hyperintensities neurology initially suspected multiple sclerosis. However, cerebrospinal fluid (CSF) analysis including neuromyelitis optica (NMO) antibodies and oligoclonal antibodies was negative. Rheumatological work up was remarkable for positive antinuclear antibodies (ANA); anticardiolipin antibodies and lupus anticoagulant were positive 12 weeks apart. This, alongside with stable white matter changes on imaging was suspicious for an extra-criteria manifestation of antiphospholipid antibody syndrome. The most commonly described neurological manifestations of APS are headache, transient ischemic attack (TIA), and stroke. Tongue stiffness as an initial symptom is quite unusual and, to the best of our knowledge has not been reported in medical literature. In patients with isolated neurological findings of unclear etiology, an autoimmune disease such as APS should be considered, and appropriate diagnostic work up should not be postponed. Unfortunately, positive laboratory markers can have a wide differential diagnostic panel. In addition, APS may mimic many diseases both in clinical presentation and MRI findings thus making the correct diagnosis challenging. However, studies show that, unlike multiple sclerosis (MS), white matter changes in APS remain static during the course of the disease. Identification of atypical presentations of APS is critical as prompt and correct medical management can improve patients’ quality of life and clinical outcomes. Cureus 2020-06-12 /pmc/articles/PMC7358906/ /pubmed/32670719 http://dx.doi.org/10.7759/cureus.8584 Text en Copyright © 2020, Rajevac et al. http://creativecommons.org/licenses/by/3.0/ This is an open access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.
spellingShingle Internal Medicine
Rajevac, Hana
Steed, Kelly
Tongue Stiffness as Presentation of Antiphospholipid Syndrome: A Case Report
title Tongue Stiffness as Presentation of Antiphospholipid Syndrome: A Case Report
title_full Tongue Stiffness as Presentation of Antiphospholipid Syndrome: A Case Report
title_fullStr Tongue Stiffness as Presentation of Antiphospholipid Syndrome: A Case Report
title_full_unstemmed Tongue Stiffness as Presentation of Antiphospholipid Syndrome: A Case Report
title_short Tongue Stiffness as Presentation of Antiphospholipid Syndrome: A Case Report
title_sort tongue stiffness as presentation of antiphospholipid syndrome: a case report
topic Internal Medicine
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7358906/
https://www.ncbi.nlm.nih.gov/pubmed/32670719
http://dx.doi.org/10.7759/cureus.8584
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