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

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

Mishaal Talish, Joe Thomas, Boby V Maramattom 
 Department of Neurology, Aster Medcity, Kothad, Kochi, Kerala, India

Correspondence Address:
Boby V Maramattom
Department of Neurology, Aster Medcity, Kothad, Kochi - 682 023, Kerala

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;69:1432-1433

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 2022 Jan 18 ];69:1432-1433
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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}{Figure 2}{Table 1}

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}

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.


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