Tongue Stiffness as Presentation of Antiphospholipid Syndrome: A Case Report.

Hana Rajevac,Kelly Steed

doi:10.7759/cureus.8584

Abstract

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.

Highlights

Antiphospholipid syndrome (APS), known as Hughes syndrome, is an autoimmune disease that can cause arterial, venous, or small-vessel thrombosis
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
We report a case of atypical APS which manifested with three months of intermittent tongue stiffness, initially assumed to represent either isolated cranial nerve palsy or an early manifestation of multiple sclerosis until the diagnosis of APS was suspected

Summary

Introduction

Antiphospholipid syndrome (APS), known as Hughes syndrome, is an autoimmune disease that can cause arterial, venous, or small-vessel thrombosis. The main laboratory feature of APS is the presence of antiphospholipid antibodies (APLA) such as anticardiolipin, Lupus anticoagulant, anti-beta 2 glycoprotein I, and have been shown to enhance activation of platelets, endothelial cells and monocytes, causing an overproduction of tissue factor and thromboxane A2, as well an excessive activation of the complement factors. Inappropriate initiation of this pro inflammatory and prothrombotic cascade can result in diffuse thrombosis of and/or well-defined obstetrical manifestations [1,2,3]. The purpose of this report is to raise awareness for rare extra-criteria presentations of APS, and discuss a differential diagnosis

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