Horner Syndrome in Giant Cell Arteritis: Case Series and Review of the Literature.

Giant cell arteritis (GCA) is a systemic inflammatory vasculitis that affects medium- and large-sized arteries and can result in permanent vision loss. In rare instances, Horner syndrome has been noticed at the time of GCA diagnosis, although the mechanism of both diagnoses occurring at the same time is not entirely understood. We reviewed 53 charts of all patients diagnosed with biopsy-proven GCA in tertiary neuro-ophthalmology practice to find patients who presented with new onset of Horner syndrome at the time of GCA diagnosis. Two patients with biopsy-confirmed GCA who presented with concurrent Horner syndrome were found. Data on age, sex, and ophthalmic and neuroradiologic examination findings were collected. Patient 1 was a 67-year-old man who presented with new onset of vertical binocular diplopia and was diagnosed with right fourth cranial nerve palsy. He then developed left ptosis and miosis, and was diagnosed with Horner syndrome by pharmacologic testing. He also had persistently elevated inflammatory markers. Patient 2 was a 71-year-old man who presented with new onset of binocular vertical diplopia, bitemporal headaches, and jaw ache. Both of his inflammatory markers were elevated. On examination, he had left ptosis and myosis, and small comitant left hypertropia. The diagnosis of left Horner syndrome was confirmed on pharmacologic testing and left hypertropia was attributed to skew deviation. Both patients underwent temporal artery biopsy, which confirmed the diagnosis of GCA. Treatment with high dose of oral corticosteroids commenced, and vertical diplopia has completely resolved in both patients. Horner syndrome persisted in Patient 1 and resolved in Patient 2. MRI and MR angiography of the brain and neck were unrevealing in both patients. This case series describes 2 patients with new diagnosis of GCA and concurrent Horner syndrome, with new diagnosis of likely nuclear/fascicular fourth nerve palsy in one patient and skew deviation in the other. In both patients, vasculitis presumptively affected vertebral arteries and their branches supplying the first-order sympathetic neurons in the brainstem. Considering the severe complication of permanent vision loss in GCA, this diagnosis should be considered in older patients presenting with concurrent new onset of Horner syndrome.

Sverdlichenko I ，Lam C ，Donaldson L ，Margolin E ... -  《-》

Giant Cell Arteritis Presenting with Ptosis and Diplopia.

Hiraoka D ，Ishizaki J ，Horie K ，Matsumoto T ，Suemori K ，Takenaka K ，Hasegawa H ... -  《-》

Retrospective, Multicenter Comparison of the Clinical Presentation of Patients Presenting With Diplopia From Giant Cell Arteritis vs Other Causes.

Ross AG ，Jivraj I ，Rodriguez G ，Pistilli M ，Chen JJ ，Sergott RC ，Moster M ，Sheldon CA ，Liu GT ，Foroozan R ，Ko MW ，Francis CE ，Williams ZR ，Lee AG ，McClelland CM ，Shindler KS ，Yalamanchili S ，Osborne B ，Hedges TR 3rd ，Van Stavern GP ，Puckett E ，Rigi M ，García-Basterra I ，Tamhankar MA ... -  《-》

Characteristics and Prognosis of Binocular Diplopia in Patients With Giant Cell Arteritis.

Giant cell arteritis (GCA) is a large vessel vasculitis associated with a risk of permanent ophthalmologic complications. Data about diplopia prognosis in GCA are scarce. This study was designed to better characterize diplopia in newly diagnosed GCA patients. All consecutive patients diagnosed with GCA from January 2015 to April 2021 in a French tertiary ophthalmologic center were retrospectively reviewed. GCA diagnosis relied on a positive temporal artery biopsy or high-definition MRI. Among 111 patients diagnosed with GCA, 30 patients (27%) had diplopia. Characteristics of patients with diplopia were similar to other GCA patients. Diplopia resolved spontaneously in 6 patients (20%). Diplopia was attributed to cranial nerve palsy in 21/24 patients (88%), especially third (46%) and sixth cranial nerve (42%). Ocular ischemic lesions occurred in 11 of the 30 patients with diplopia (37%); 2 patients developed vision loss after initiation of corticosteroids. In the remaining 13 patients, diplopia resolved after treatment onset in 12 patients (92%) with a median delay of 10 days. Patients treated intravenously tended to have a quicker improvement than those treated orally, but with a similar resolution rate of diplopia at 1 month. Two patients had relapse of diplopia at 4 and 6 weeks after an initial treatment course of 24 and 18 months, respectively. Diplopia is a rare feature at GCA diagnosis, but should raise clinician suspicion for GCA when associated with cephalic symptoms and prompt the initiation of corticosteroids to prevent ocular ischemic complications.

Chazal T ，Clavel G ，Leturcq T ，Philibert M ，Lecler A ，Vignal-Clermont C ... -  《-》

Ischemic ocular manifestations of giant cell arteritis: A Canadian case series.

Diagnosing giant cell arteritis (GCA) can be challenging due to the variable clinical presentation and lack of systemic symptoms in approximately 20% of cases, which are termed occult GCA. GCA affects the ocular circulation in more than half of cases and can cause irreversible bilateral blindness if not rapidly recognized and treated. We compared clinical and laboratory findings of patients with occult and systemic temporal artery biopsy (TAB)-positive GCA with ocular manifestations. Retrospective chart review of patients with TAB-positive GCA with ocular manifestations seen at a tertiary neuro-ophthalmology practice between 2015 and 2020. GCA was occult in 14.3% (6/42) and systemic in 85.7% (36/42) of patients. Occult GCA affected older patients (average age 84.6 ± 5.3 vs 75.9 ± 8.2). There was no significant difference in ESR or CRP levels between two cohorts. In patients with occult GCA, 83.3% (5/6) presented with AION, and 16.7% (1/6) with isolated cranial nerve six palsy (CN6P). In the systemic GCA cohort, 47.2% (17) presented with AION, 5.6% (2) with CRAO, 2.8% (1) with PION, 2.8% (1) with isolated CWS, 2.8% (1) had both CRAO and AION, 2.8% (1) isolated cranial nerve 4 palsy (CN4P), 2.8% (1) isolated CN6P, 22.2% (8) had history of amaurosis fugax but normal examination, and 11.1% (4) had visual symptoms but a normal exam. Three of the above patients with AION also had CN4P. Of patients diagnosed with AION, presenting visual acuity in the affected eye(s) was significantly worse in patients with occult GCA. Involvement of multiple ocular circulations was seen in six patients in the systemic group but none in the occult group. Occult GCA occurs more commonly in older patients who most of the time present with AION. Levels of inflammatory markers were similar in the occult and systemic groups. Though ocular ischemic manifestations were similar in both cohorts, in systemic GCA involvement of multiple ocular circulations is likely more common GCA should always be considered in older patients who present with signs of retinal and/or optic nerve head ischemia (AION and/or CWS) and new onset of CNPs, especially in patients where more than one ocular arterial circulation is involved.

Issa M ，Donaldson L ，Jeeva-Patel T ，Margolin E ... -  《-》