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Nine-gaze eye positions. Range of motion in cardinal position demonstrates impairment of downward, lateral and medial gaze in the left eye, and of lateral gaze in the right eye.
Source publication
To report a case of third, fourth, and six cranial nerve palsies with antiphospholipid syndrome (APS).
Medical records of a 16-year-old female diagnosed with idiopathic intracranial hypertension (IIH) in primary APS were reviewed.
A 16-year-old female presented with headache and diplopia. Ocular examinations revealed marked bilateral disc edema. Sh...
Context in source publication
Context 1
... examinations showed a visual acuity of 20/20 in both eyes, a mid dilated pupil of the left eye, and marked bilateral disc edema with peripapillary hemorrhage. She was unable to depress, adduct, and abduct the left eye and had limited abduction of the right eye (Fig. 1). Visual field examination showed peripheral constriction and enlargement of the blind spot in both ...
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Patient: Female, 15
Final Diagnosis: Varicella Zoster aseptic meningitis
Symptoms: —
Medication: —
Clinical Procedure: Lumber punctur
Specialty: Infectious Diseases
Objective:
Unusual clinical course
Background:
Neurologic complications can occur with varicella zoster virus (VZV) infection, usually after vesicular exanthem. A review of the...
Citations
... Ocular motility disorders due to extraocular muscle nerve palsies have been reported in APS patients in the settings of idiopathic intracranial hypertension and cerebral sinus thrombosis [49,50]. Superior ophthalmic vein thrombosis is another pathology that can also cause ophthalmoplegia as well as proptosis in APS patients [51]. ...
Antiphospholipid syndrome (APS) is an autoimmune disorder associated with obstetrical complications, thrombotic complications involving both arteries and veins, and non-thrombotic manifestations affecting multiple other systems presenting in various clinical forms. Diagnosis requires the presence of antiphospholipid antibodies. The exact pathogenesis of APS is not fully known. However, it has recently been shown that activation of different types of cells by antiphospholipid antibodies plays an important role in thrombosis formation. Ocular involvement is one of the important clinical manifestations of APS and can vary in presentations. Therefore, as an ophthalmologist, it is crucial to be familiar with the ocular findings of APS to prevent further complications that can develop. Furthermore, the ongoing identification of new and specific factors contributing to the pathogenesis of APS may provide new therapeutic options in the management of the disease in the future.
... 103 Shin et al. described a single case of multiple cranial nerve palsies associated with papilledema secondary to idiopathic intracranial hypertension in an APS patient. 104 Ali et al. 105 reported a rare case of APS presenting as papilledema and sixth nerve palsy in the right eye due to superior sagittal sinus thrombosis, which ceased after anticoagulation and acetazolamide therapy. Champion et al. 106 described a 24-year-old female who presented with sudden onset of painless diplopia and ptosis in her left eye, with isolated incomplete pupil-sparing left oculomotor nerve palsy. ...
Objective
There is a broad spectrum of eye involvement in antiphospholipid syndrome (APS). The majority of descriptions are presented as case reports that include mostly APS patients secondary to systemic lupus erythematosus (SLE), with no compelling evidence in primary APS (PAPS). This study aimed to describe ocular manifestations in our well-defined PAPS cohort (APS-Rio) and then perform a systematic literature review (SLR) of ocular manifestations in patients with APS or positivity to aPL without SLE.
Methods
We retrospectively analyzed PAPS patients followed at our outpatient clinics. All patients fulfilled Sydney APS classification criteria (2006). We evaluated them for ocular symptoms and previous ocular diagnoses. Antiphospholipid antibodies and clinical APS manifestations were compared between patients with and without ocular manifestations. For the SLR, electronic databases were searched up to November 2019.
Results
We studied 105 PAPS patients; 90.5% were female and 56.2% were Caucasian. We found ocular manifestations in 37.1% of our cohort. Thrombosis was the main criteria manifestation (95.2%) and lupus anticoagulant was the most prevalent antibody. Ophthalmologic diagnoses were present in 7 patients, with 5 having retinal vessels thromboses. Amaurosis fugax was the leading complaint, present in 30 patients. In the univariate analysis, amaurosis fugax was related to livedo (p = 0.005), Raynaud’s phenomenon (p = 0.048) and the presence of anticardiolipin antibody (≥40 GPL/MPL) (p = 0.041). Hemianopia was associated with arterial hypertension (p = 0.049). In the multivariate analysis, the only association found was between livedo and amaurosis fugax (OR 4.09, 95%CI 1.5–11.11, p = 0.006). Our SLR incorporated 96 articles of ocular manifestations in patients with PAPS or positivity to aPL without SLE. Ocular findings varied from 5 to 88%, including anterior and posterior segments, orbital and neuro-ophthalmologic changes.
Conclusion
There is little evidence on ocular manifestations in PAPS. We described an association between livedo and amaurosis fugax. Prospective studies are needed to promote the best treatment and avoid blindness in PAPS patients.
... Vasculitis may manifest as anterior uveitis, episcleritis (Figure 10), scleritis ( Figure 11) or retinal vasculitis causing retinal artery occlusion ( Figure 12). APS may also be associated with cranial nerve III (Genevay, Hayem et al. 2002) (Figure 13), cranial nerve IV (Shin and Lee 2006) and cranial nerve VI (Shin and Lee 2006) palsies, in addition to ischaemic optic neuropathy (Giorgi and Balacco Gabrieli 1999), and optic neuritis (Giorgi and Balacco Gabrieli 1999). Patients with retinal vaso-occlusion usually present with pain, unilateral or bilateral decreased visual acuity, visual field defect or amaurosis fugax. ...
... Vasculitis may manifest as anterior uveitis, episcleritis (Figure 10), scleritis ( Figure 11) or retinal vasculitis causing retinal artery occlusion ( Figure 12). APS may also be associated with cranial nerve III (Genevay, Hayem et al. 2002) (Figure 13), cranial nerve IV (Shin and Lee 2006) and cranial nerve VI (Shin and Lee 2006) palsies, in addition to ischaemic optic neuropathy (Giorgi and Balacco Gabrieli 1999), and optic neuritis (Giorgi and Balacco Gabrieli 1999). Patients with retinal vaso-occlusion usually present with pain, unilateral or bilateral decreased visual acuity, visual field defect or amaurosis fugax. ...
Se describe el caso de un paciente con púrpura trombocitopénica idiopática refractario al tratamiento, quien ingresó por cefalea, exotropía del ojo derecho, ptosis palpebral, trombocitopenia severa, requiriendo transfusión. Se realizó resonancia cerebral contrastada que evidenció hemorragia subaracnoidea en el opérculo frontal derecho, con perfil infeccioso, carencial, de autoinmunidad y vasculítico negativos, con perfil para síndrome antifosfolípido positivo. Se consideró posible mecanismo fisiopatológico, trombosis de la vasa nervorum, lo que favoreció la parálisis incompleta del tercer par craneal. Se inició anticoagulación con warfarina, se ajustó terapia inmunomoduladora de enfermedad de base, con evolución favorable del paciente.
A 53-year-old immunocompromised woman developed acute left eye blindness and paraparesis suspected to be due to neuromyelitis optica (NMO). During treatment for NMO, right eye blindness and progressive multiple cranial neuropathies developed. Cerebrospinal fluid polymerase chain reaction (PCR) revealed Varicella zoster virus (VZV). This case emphasizes the importance of considering VZV in individuals, particularly the immunocompromised, presenting with a constellation of neurological signs and symptoms, even in the absence of rash.
Multiple cranial nerve paresis (MCNP) can occur due to some syndromes, systemic
diseases, extracranial and intracranial pathologies. The paresies including cranial
nerves III, IV, V, VI, and VII are eye-associated MCNP. The common causes of eyeassociated MCNP often include cavernous sinus syndrome, superior orbital fissure
syndrome, orbital apex syndrome, and cerebellopontine angle syndrome. Clinical
approach to MCNP includes the careful examination for generalized limitation in
various gaze positions, proptosis, decreased corneal and facial sensation, conjunctival
injection, ptosis, anisocoria, and cerebellar signs. In this review, we aim to briefly
remind the main causes of MCNP associated with the eye.
To report a case of monocular elevation deficiency as the presenting manifestation of systemic lupus erythematosus (SLE).
To report the clinical course and results of two cases of anterior segment manifestations associated with systemic lupus erythematosus (SLE).
To investigate the clinical characteristics of retinopathy associated with systemic lupus erythematosus (SLE) and its risk factors.
Antiphospholipid syndrome (APS) is an autoimmune disease characterised by a heterogenous group of antibodies directed against negatively charged phospholipids including antiphospholipid antibodies (aPL), anticardiolipin antibodies (aCL) and β-2 glycoprotein I (aβ-2-GP1). The major features of this disorder include arterial and venous thrombosis and recurrent fetal loss. The vasculature of the eye is frequently involved and may be the presenting manifestation. A diagnosis of APS should be considered in a young patients without traditional thromboembolic risk factors presenting with ocular vaso-occlusive disease. Management of these patients involves a team-approach with a haematologist/oncologist or rheumatologist to manage the coagulation status of these patients to prevent further systemic vascular occlusions.
