Working Diagnosis:
Stroke due to Focal Cerebral Arteriopathy

Treatment:
The patient was administered IV Tenecteplase and a cerebral angiogram was completed without thrombectomy. The patient was started on aspirin and IV steroids with transition to an oral steroid taper.

Outcome:
He was transferred to an inpatient rehab facility where he developed spasticity and neuropathic pain for which he was started on Baclofen and Pregabalin. His left-sided strength improved from a 0/5 to 3-5/5 in major muscle groups. He progressed from mobilizing with a manual wheelchair to ambulating with a left forearm crutch and an ankle-foot orthosis (AFO).

The patient is currently not safe to play able-bodied soccer given left-sided weakness and inattention. He will undergo intensive outpatient therapies with hopeful return of further strength and functional gains. Therapists will provide him with resources to engage in adaptive sporting activities once appropriate. He plans to help coach his younger brother's soccer team to remain involved in the sport.

Author's Comments:
Focal Cerebral Arteriopathy is defined as unilateral intracranial arteriopathy of the anterior circulation. It accounts for approximately 20% of pediatric stroke cases. The definitive diagnosis is made by various vessel imaging modalities. Clinical trials are underway to determine how effective steroids are in treating this condition. This case highlights the importance of maintaining a high differential diagnosis for the collapsed athlete and/or those displaying a focal neurologic deficit. It further emphasizes the difficulties associated with experiencing a career-ending injury when the athlete's identity largely relies on their sport participation.

Editor's Comments:
This case highlights the importance of having an emergency action plan and practicing it. If an athlete collapses, don't panic. Know your ABCs (airway, breathing, circulation), have someone get the AED and call 911. Assume in-exercise collapse is cardiac until proven otherwise. Check vital signs and consider a rectal temperature if heat stroke or hypothermia are considerations.

In this case the authors recognized focal neurologic deficits, quickly stabilized the cervical spine, and had the athlete transported to the emergency department. Imaging findings led to the diagnosis of an ischemic stroke caused by Focal Cerebral Arteriopathy.

Many cases of Focal Cerebral Arteriopathy are thought to be inflammatory or related to an infectious process (especially Varicella-Zoster). Though in this case inflammatory markers (CRP, ESR) and tests for the two most commonly implicated viruses (Varicella-Zoster and Herpes Simplex) were negative. Additionally and unfortunately, there is a high risk of recurrent stroke in these patients (up to 25% in studies).

References:
McKenna MC, Fanning N, Cronin S. Focal Cerebral Arteriopathy in Young Adult Patients With Stroke. Stroke. 2020 May;51(5):1596-1599. doi: 10.1161/STROKEAHA.119.028343. Epub 2020 Mar 26. PMID: 32212896.

Oesch G, Perez FA, Wainwright MS, Shaw DWW, Amlie-Lefond C. Focal Cerebral Arteriopathy of Childhood: Clinical and Imaging Correlates. Stroke. 2021 Jul;52(7):2258-2265. doi: 10.1161/STROKEAHA.120.031880. Epub 2021 May 27. PMID: 34039030.

Park Y, Fullerton HJ, Elm JJ. A pragmatic, adaptive clinical trial design for a rare disease: The FOcal Cerebral Arteriopathy Steroid (FOCAS) trial. Contemp Clin Trials. 2019 Nov;86:105852. doi: 10.1016/j.cct.2019.105852. Epub 2019 Oct 13. PMID: 31614215; PMCID: PMC6857809.

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