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Table of Contents    
Year : 2021  |  Volume : 69  |  Issue : 5  |  Page : 1432-1433

A Case of Atypical Cogan's Syndrome Associated with Reversible Cerebral Vasoconstriction Syndrome

Department of Neurology, Aster Medcity, Kothad, Kochi, Kerala, India

Date of Submission31-Aug-2019
Date of Decision09-Jan-2020
Date of Acceptance11-Jan-2020
Date of Web Publication30-Oct-2021

Correspondence Address:
Boby V Maramattom
Department of Neurology, Aster Medcity, Kothad, Kochi - 682 023, Kerala
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0028-3886.329590

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How to cite this article:
Talish M, Thomas J, Maramattom BV. A Case of Atypical Cogan's Syndrome Associated with Reversible Cerebral Vasoconstriction Syndrome. Neurol India 2021;69:1432-3

How to cite this URL:
Talish M, Thomas J, Maramattom BV. A Case of Atypical Cogan's Syndrome Associated with Reversible Cerebral Vasoconstriction Syndrome. Neurol India [serial online] 2021 [cited 2021 Dec 3];69:1432-3. Available from:


Cogan's syndrome is an autoimmune disorder characterized by vestibulo-auditory, ocular and systemic manifestations.[1],[2],[3] The time between the onset of ocular and audio-vestibular symptoms is often <2 years.[3],[4] In Atypical Cogan syndrome (ACS), other ocular symptoms such as scleritis, episcleritis, choroiditis, papillitis, retinitis, optic neuritis, central retinal artery occlusion and systemic symptoms may coexist.[1],[2] [Table 1] In ACS, the time frame between the ocular and audio-vestibular symptom onset can be >2 years.[3],[4],[5] We report the first case of Atypical Cogan's syndrome (ACS) associated with thunderclap headache and Reversible cerebral vasoconstriction syndrome (RCVS). A 30-year-old man, a known case of ACS (Bilateral Anterior Uveitis and Bilateral Sensorineural deafness), was admitted with sudden onset severe post-coital occipital headache. He had three similar self-resolving episodes in the last 2 weeks; at that time CT, MRI/MRA/MRV and CSF studies were normal. He had no focal neurological findings. Repeat CT and CSF exam was also normal. He was started on Nimodipine 60 mg 4 hourly with a presumptive diagnosis of subarachnoid haemorrhage. A direct catheter angiography (DSA) on day 2 showed spasm in the right anterior cerebral artery (ACA) [Figure 1]. He improved gradually and was discharged on Nimodipine 60 mg 4 hourly for 21 days. A repeat DSA 6 weeks later was normal [Figure 2]. A diagnosis of RCVS with ACS was made.
Figure 1: Arrow depicts short segment spasm of ACA

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Figure 2: Post-treatment DSA showing resolution of ACA spasm [arrow]

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Table 1: Systemic involvement in patients with ACS[1],[4]

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The ACS is a disease of young white adults and autoimmune origin with autoantibodies against inner ear antigens, anti-neutrophil cytoplasmic antibodies, and anti-endothelial cell antibodies.[1],[5] Vasculitic features have been noted in 21% of ACS.[4] Our patient was initially thought to have CNS vasculitis, a known complication of this disease. However, the angiographic reversal of spasm, thunderclap headache and response to Nimodipine suggested the diagnosis of RCVS.

Reversible cerebral vasoconstriction syndromes generally present with an acute severe thunderclap headache with or without focal neurological deficits and with diffuse segmental narrowing of the cerebral arteries that is reversible within 3 months.[6],[7],[8] It may occur spontaneously, but several factors including illicit drugs, vasopressor medications and the post-partum state have also been implicated [Table 2]. The incidence of RCVS is estimated to be 8%-45% in patients presenting with a thunderclap headache without any evidence other cause for the headache.[7] The differential diagnoses of RCVS include subarachnoid haemorrhage, Primary angiitis of the CNS, CNS vasculitis, and cervical artery dissection.
Table 2: Diagnostic criteria for RCVS[9]

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RCVS generally has a relatively benign course compared to CNS vasculitis. However, about 5%-10% of RCVS patients are left with permanent neurological damage.[8] In a patient presenting with RCVS, the use of calcium channel blockers is recommended.[7],[9] Our patient with ACS had an angiographic resolution of cerebral arterial spasm [Figure 2] and good clinical improvement with Nimodipine. This is the first case report exhibiting an association between ACS and RCVS. ACS should be added to the list of conditions that present with RCVS.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

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Conflicts of interest

There are no conflicts of interest.

  References Top

Grasland A, Pouchot J, Hachulla E, Blétry O, Papo T, Vinceneux P; Study Group for Cogan's syndrome. Typical and atypical Cogan's syndrome: 32 cases and review of the literature. Rheumatology 2004;43:1007-15.  Back to cited text no. 1
Migliori G, Battisti E, Pari M, Vitelli N, Cingolani C. A shifty diagnosis: Cogan's syndrome: A case report and review of the literature. Acta Otorhinolaryngol Ital 2009;29:108-13.  Back to cited text no. 2
Sevgi DD, Sobrin L, Papaliodis GN. Cogan syndrome with severe medium and large vessel vasculitis. Digit J Ophthalmol 2015;22:32-4.  Back to cited text no. 3
Masiak A, Waśkowska A, Lipowski P, Smoleńska Ż, Przewoźny T, Zdrojewski Z. Diagnostic difficulties of Cogan syndrome. J Clin Case Rep 2016;6:691.  Back to cited text no. 4
Singh P, Gupta M, Lehl SS, Singh K. Cogan's syndrome: Achievement of complete resolution of auditory deficit with steroids. BMJ Case Rep 2013;bcr2013008951.  Back to cited text no. 5
Saini M, Jeerakathil T, Butcher K. Reversible cerebral vasoconstriction syndrome. Neurol India 2009;57:63-5.  Back to cited text no. 6
[PUBMED]  [Full text]  
Kim T, Ahn S, Sohn CH, Seo DW, Kim WY. Reversible cerebral vasoconstriction syndrome at the emergency department. Clin Exp Emerg Med 2015;2:203-9.  Back to cited text no. 7
Cappelen-Smith C, Calic Z, Cordato D. Reversible cerebral vasoconstriction syndrome: Recognition and treatment. Curr Treat Options Neurol 2017;19:21.  Back to cited text no. 8
Calabrese LH, Dodick DW, Schwedt TJ, Singhal AB. Narrative review: Reversible cerebral vasoconstriction syndromes. Ann Intern Med 2007;146:34-44.  Back to cited text no. 9


  [Figure 1], [Figure 2]

  [Table 1], [Table 2]


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