Abstract Details

Title A case report of Granulomatous angiitis associated with Cogan syndrome.

Topic Autoimmune Neurology

Presentation(s) P12 - Poster Session 12 (5:30 PM-6:30 PM)

Poster/Presentation
Number 9-002

Background Cogan's syndrome is a rare autoimmune disorder typically characterized by Non-syphilitic interstitial keratitis and progressive audiovestibular symptoms. Systemic manifestations are present in less than 5%. Various neurological symptoms have been reported but mechanisms and diagnostic criteria are not clearly defined.

Objective To describe a case of CNS vasculitis related to Cogan syndrome with emphasis on the importance of early recognition of the condition based on clinical features, and the critical role of tissue biopsy to make a definitive diagnosis.

Design/Methods case report

Results 53-year-old man with history of rheumatoid arthritis, Cogan syndrome being treated with methotrexate and renal cell carcinoma in remission presented with two months of waxing and waning episodes of severe headache, seizures, word-finding difficulty, confusion, memory changes and stroke like symptoms. Infectious and metabolic workup was unremarkable. Paraneoplastic antibodies in serum and CSF were negative. Neuroimaging showed areas of diffusion restriction in bilateral basal ganglia and frontal lobes concerning for embolic infarcts, but conventional angiograms didn’t reveal any evidence of vasculitis. Due to worsening symptoms, brain biopsy was done which was consistent with granulomatous angiitis. The patient was started on pulse steroids and Rituximab which resulted in significant improvement in symptoms.

Conclusions Though neurological symptoms in Cogan syndrome are rare, delay in treatment can be devastating in this potentially treatable condition. Nervous system involvement in Cogan syndrome may present itself with varied symptomatology. Therefore, in patients presenting with new onset neurological symptoms in the context of visual impairment and sensorineural hearing loss, one should have a high index of suspicion for Cogan’s syndrome related angiitis. Tissue biopsy is of utmost importance to differentiate this condition from other subacute encephalopathies and other non-vasculitic vascular processes. Also, there is no clear guidelines on choosing appropriate immunosuppressant so establishing clear treatment guidelines is more important.

Authors/Disclosures
Praveen Ramani, MBBS (Arkansas Children's Hospital.) PRESENTER	Dr. Ramani has nothing to disclose.
Hira Zafar, MBBS (Uams)	Dr. Zafar has nothing to disclose.
Nayana Prabhu, MD (UNIVERSITY OF ARKANSAS FOR MEDICAL SCIENCES)	Dr. Prabhu has nothing to disclose.
Salman Zahoor, MBBS (UNIVERSITY OF ARKANSAS FOR MEDICAL SCIENCES)	Dr. Zahoor has nothing to disclose.
Sisira Yadala, MD, FAAN	Dr. Yadala has nothing to disclose.