Hypertrophic pachymeningoencephalitis associated with temporal giant cell arteritis

We report the rare case of an 80-year-old male patient with hypertrophic pachymeningoencephalitis that may be associated with temporal arteritis. The patient presented to our neurological department with a 2-week history of latent paresis and ataxia affecting his right hand. He had been diagnosed with temporal arteritis 12 years earlier. Brain MRI showed an enhancement of the left-sided frontoparietal meninges with oedema of the adjacent tissue of the precentral and postcentral cortex. A leptomeningeal biopsy was performed. An autoimmune-mediated immunoglobulin G4-associated hypertrophic pachymeningoencephalitis was diagnosed. The patient received a high-dose corticosteroid therapy and his symptoms gradually improved. Our results suggest that hypertrophic pachymeningoencephalitis may occur as a complication of giant cell arteritis and may cause central neurological deficits by cerebral perifocal oedema.

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Fatal Aortic Dissection in a Patient with Giant Cell Arteritis: A Case Report and Review of the Literature

Case Reports in Vascular Medicine ◽

10.1155/2013/590721 ◽

2013 ◽

Vol 2013 ◽

pp. 1-4 ◽

Cited By ~ 2

Author(s):

Anjeli K. Nayar ◽

Michael Casciello ◽

Jennifer N. Slim ◽

Ahmad M. Slim

Keyword(s):

Aortic Dissection ◽

Giant Cell Arteritis ◽

Giant Cell ◽

Temporal Arteritis ◽

Acute Aortic Dissection ◽

Vascular Wall ◽

Clinical Implications ◽

Review Of The Literature ◽

History Of ◽

Prompt Diagnosis

Giant cell arteritis may lead to catastrophic, large-vessel complications from chronic vascular wall inflammation without prompt diagnosis and treatment. We describe a rare case of acute aortic dissection without preceding aneurysm secondary to histologically confirmed giant cell arteritis (GCA) in an 85-year-old female with a four-year history of polymyalgia rheumatica and temporal arteritis diagnosed per biopsy six months prior to presentation. The literature is reviewed and the clinical implications of this case are discussed.

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Giant Cell Arteritis: From Neurologist’s Perspective

10.5772/intechopen.97163 ◽

2021 ◽

Author(s):

Ravish Rajiv Keni ◽

M. Sowmya ◽

Sreekanta Swamy

Keyword(s):

Giant Cell Arteritis ◽

Giant Cell ◽

Visual Loss ◽

Multidisciplinary Care ◽

The Elderly ◽

External Carotid Artery ◽

Laboratory Evaluation ◽

High Dose ◽

Granulomatous Vasculitis ◽

Dose Corticosteroid

Giant cell arteritis (GCA) is a granulomatous vasculitis affecting large- and medium-sized arteries in the elderly and potentially causes visual loss. In an elderly patient presenting with acute pain in the distribution of the external carotid artery (e.g., headache, scalp tenderness); polymyalgia rhematica; or acute/transient visual loss or diplopia; a possibility of GCA should be considered in one of the differential diagnosis. Urgent laboratory evaluation (e.g., ESR, CRP, platelet count), followed immediately by empiric high-dose corticosteroid therapy is warranted in patients suspected of having GCA. Although ultrasound techniques are sensitive for the diagnosis of GCA, TAB remains the best confirmatory test. Patients with GCA often require long durations of steroid therapy and steroid-related complications are common. Multidisciplinary care and the use of steroid-sparing regimens are warranted in case of relapse.

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Progressive and Fatal Brainstem Stroke in Systemic Giant Cell Arteritis

Neurology Clinical Practice ◽

10.1212/cpj.0000000000001083 ◽

2021 ◽

pp. 10.1212/CPJ.0000000000001083

Author(s):

Valentina Poretto ◽

Silvio Piffer ◽

Valeria Bignamini ◽

Enzo Tranquillini ◽

Davide Donner ◽

...

Keyword(s):

Giant Cell Arteritis ◽

Giant Cell ◽

Temporal Artery ◽

Facial Artery ◽

High Dose ◽

Temporal Artery Biopsy ◽

Halo Sign ◽

Pet Ct ◽

Brainstem Stroke ◽

History Of

A 74-year-old woman presented with acute worsening of six-months long history of vertigo and postural instability, with MRI evidence of cerebellar and brainstem acute infarcts. Extensive neurovascular assessment revealed a severe vascular damage with multiple stenoses and occlusions along vertebrobasilar axis (figure 1). Duplex ultrasonography showed hypoechoic halo sign along facial artery, while PET-CT highlighted increased [18F]-FDG uptake along vertebral and other larger arteries, thus allowing a diagnosis of giant cell arteritis (figure 2).1,2 Despite prompt treatment with high-dose steroids and tocilizumab, which probably made uninformative a subsequent temporal artery biopsy (figure 2), patient died of reported disability after strokes.

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Temporal Artery Vascular Diseases

Journal of Clinical Medicine ◽

10.3390/jcm11010275 ◽

2022 ◽

Vol 11 (1) ◽

pp. 275

Author(s):

Hélène Greigert ◽

André Ramon ◽

Georges Tarris ◽

Laurent Martin ◽

Bernard Bonnotte ◽

...

Keyword(s):

Giant Cell Arteritis ◽

Giant Cell ◽

Temporal Arteritis ◽

Checkpoint Inhibitors ◽

Vascular Diseases ◽

Temporal Artery ◽

Immunoglobulin G4 ◽

Varicella Zoster ◽

Related Disease ◽

Anca Associated Vasculitis

In the presence of temporal arteritis, clinicians often refer to the diagnosis of giant cell arteritis (GCA). However, differential diagnoses should also be evoked because other types of vascular diseases, vasculitis or not, may affect the temporal artery. Among vasculitis, Anti-neutrophil cytoplasmic antibodies (ANCA)-associated vasculitis is probably the most common, and typically affects the peri-adventitial small vessel of the temporal artery and sometimes mimics giant cell arteritis, however, other symptoms are frequently associated and more specific of ANCA-associated vasculitis prompt a search for ANCA. The Immunoglobulin G4-related disease (IgG4-RD) can cause temporal arteritis as well. Some infections can also affect the temporal artery, primarily an infection caused by the varicella-zoster virus (VZV), which has an arterial tropism that may play a role in triggering giant cell arteritis. Drugs, mainly checkpoint inhibitors that are used to treat cancer, can also trigger giant cell arteritis. Furthermore, the temporal artery can be affected by diseases other than vasculitis such as atherosclerosis, calcyphilaxis, aneurysm, or arteriovenous fistula. In this review, these different diseases affecting the temporal artery are described.

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Normal inflammatory markers in giant cell arteritis with long-standing cranial and symptomatic large-vessel involvement

BMJ Case Reports ◽

10.1136/bcr-2021-242602 ◽

2021 ◽

Vol 14 (6) ◽

pp. e242602

Author(s):

Alicia Rodriguez-Pla ◽

Sailendra G Naidu ◽

Yasmeen M Butt ◽

Victor J Davila

Keyword(s):

Giant Cell Arteritis ◽

Giant Cell ◽

Inflammatory Markers ◽

Care Physician ◽

Large Vessel ◽

High Dose ◽

Surgical Revascularisation ◽

History Of ◽

Surgery Department ◽

Vessel Involvement

We report the case of a 78-year-old woman who presented with cardiovascular risk factors and a history of an atypical transient ischaemic attack. She was referred by her primary care physician to the vascular surgery department at our institution for evaluation of progressive weakness, fatigue, arm claudication and difficulty assessing the blood pressure in her right arm. She was being considered for surgical revascularisation, but a careful history and review of her imaging studies raised suspicion for vasculitis, despite her normal inflammatory markers. She was eventually diagnosed with biopsy-proven giant cell arteritis with diffuse large-vessel involvement. Her symptoms improved with high-dose glucocorticoids.

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Neurosyphilis in a suspected case of giant cell arteritis

BMJ Case Reports ◽

10.1136/bcr-2021-242733 ◽

2021 ◽

Vol 14 (9) ◽

pp. e242733

Author(s):

Annalisa Montebello ◽

Daniela Harmsworth ◽

Paul John Cassar ◽

Sandro Vella

Keyword(s):

Giant Cell Arteritis ◽

Giant Cell ◽

Venereal Disease ◽

High Dose ◽

Suspected Case ◽

Venereal Disease Research Laboratory ◽

Disease Research ◽

History Of ◽

Visual Blurring ◽

Syphilis Serology

A 67-year-old man had a few month history of deteriorating visual acuity. He had originally presented to ophthalmology with right-sided visual blurring. This subsequently progressed to involve the left eye. At this point, he was empirically treated with high-dose glucocorticoids, both orally and intravenously, with the suspicion that giant cell arteritis was causing acute visual deterioration of his left eye. Unfortunately, his symptoms did not improve. During an admission to hospital for a pneumonia, he underwent further investigations for this bilateral visual loss. He was diagnosed with left neuroretinitis and right vitritis. A thorough workup revealed positive syphilis serology and cerebrospinal fluid was positive on venereal disease research laboratory testing. He was diagnosed and treated for neurosyphilis with intravenous benzylpenicillin 4 million units 4 hourly for 14 days. His left-sided vision improved but he still suffers from severe visual impairment in his right eye.

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Arteritic Ischemic Optic Neuropathy

Neuro-Ophthalmology ◽

10.1093/med/9780190603953.003.0002 ◽

2019 ◽

pp. 9-14

Author(s):

Matthew J. Thurtell ◽

Robert L. Tomsak

Keyword(s):

Giant Cell Arteritis ◽

Giant Cell ◽

Clinical Features ◽

Optic Neuropathy ◽

Vision Loss ◽

Medical Emergency ◽

Management Approach ◽

High Dose ◽

Ischemic Optic Neuropathy ◽

Dose Corticosteroid

Arteritic ischemic optic neuropathy occurs in the setting of giant cell arteritis and often produces devastating irreversible vision loss. It is a medical emergency because there is a high risk of fellow eye involvement if high-dose corticosteroid treatment is not initiated in a timely fashion. In this chapter, we begin by reviewing the clinical features of anterior and posterior ischemic optic neuropathy. We next review the clinical features of arteritic ischemic optic neuropathy. We then discuss the clinical features and workup for giant cell arteritis. Lastly, we review the initial and long-term management approach for ischemic optic neuropathy occuring in the setting of giant cell arteritis.

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Polymyalgia Rheumatica - a Disease of the Elderly

Revista de Chimie ◽

10.37358/rc.18.1.6063 ◽

2018 ◽

Vol 69 (1) ◽

pp. 152-154

Author(s):

Vasilica Cristescu ◽

Aurelia Romila ◽

Luana Andreea Macovei

Keyword(s):

Giant Cell Arteritis ◽

Giant Cell ◽

Polymyalgia Rheumatica ◽

Temporal Arteritis ◽

Viral Infections ◽

Clinical Manifestations ◽

The Elderly ◽

Clinical Aspects ◽

Immune Disorder ◽

Markers Of Inflammation

Polymyalgia rheumatica is a disease that occurs mostly in the elderly and is rarely seen in patients less than 50 years of age. Polymyalgia rheumatica is a vasculitis, which manifests itself as an inflammatory disease of the vascular wall that can affect any type of blood vessel, regardless of its size. It has been considered a form of giant cell arteritis, involving primarily large and medium arteries and to a lesser extent the arterioles. Clinical manifestations are caused by the generic pathogenic process and depend on the characteristics of the damaged organ. PMR is a senescence-related immune disorder. It has been defined as a stand-alone condition and a syndrome referred to as rheumatic polyarteritis with manifestations of giant cell arteritis (especially in cases of Horton�s disease and temporal arteritis) which are commonly associated with polymyalgia. The clinical presentation is clearly dominated by the painful girdle syndrome, with a feeling of general discomfort. Polymyalgia and temporal arteritis may coexist or be consecutive to each other in the same patient, as in most of our patients. The present study describes 3 cases of polymyalgia rheumatica, admitted to the Clinic of Rheumatology of Sf. Apostol Andrei Hospital, Galati. The cases were compared with the literature. Two clinical aspects (polymyalgia rheumatica and/or Horton�s disease) and the relationship between them were also considered. Polymyalgia rheumatica is currently thought to have a multifactorial etiology, in which the following factors play a role: genetic factors or hereditary predisposition (some individuals are more prone to this disease), immune factors and viral infections (triggers of the disease). Other risk factors of polymyalgia rheumatica include age over 50 years and the association with giant cell arteritis. The characteristic feature of the disease is girdle pain, with intense stiffness of at least one hour�s duration. Markers of inflammation, erythrocyte sedimentation rate and C-reactive protein are almost always increased at the onset of the disease. Diseases that can mimic the clinical picture of polymyalgia rheumatica are neoplasia, infections, metabolic disorders of the bone and endocrine diseases.

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