Carolinas Research Day 2021

New Onset Ischemic Cerebellar Infarction Secondary to Multi-vessel Occlusion in Sixteen-year-old Male: A Case Report Gretel Rodriguez, OMS-IV; Emily Medhus, OMS-IV; Hanna S. Sahhar, MD, FAAP, FACOP Edward Via College of Osteopathic Medicine-Carolinas Campus, Spartanburg, South Carolina

CBR- 6

Abstract

Conclusions

Case Presentation

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A 16-year-old Caucasian male presented to the emergency department with his grandmother, who stated that he had been sleepy, confused and agitated for the past hour. Per grandmother, the patient complained of “things spinning” and had an alleged fall after having taken a shower. There was concern for ingestion of illicit drugs or medications but due to the patient’s altered mental status we were not able to obtain that information. • Past Medical History: ADHD, borderline Autism, Pervasive Developmental Disorder • Medications: Escitalopram, Guanfacine, Methylphenidate HCl • Social History: Denies tobacco, alcohol, or illicit drug use • Immunizations are up to date Physical Exam: General: Patient sleepy but responsive to verbal stimuli Neurologic: Altered mental status, slurred speech, no neuro deficits at this time HEENT: Normocephalic, atraumatic, PERRL, neck is supple Respiratory: Clear to auscultation, no wheezing, rales, or rhonchi Cardiovascular: Regular rate and rhythm, no murmurs Hypercoagulable studies demonstrated mildly decreased activity levels of protein C and protein S at 46% (normal range 70% -180%) and 63% (normal range 70% -150%) respectively. The patient was admitted to the PICU for observation. ~12 hours later → patient began to show signs of neurologic decline including: left gaze deviation right-sided weakness (face, upper & lower extremities)

Ischemic strokes , when occurring in neonates, infants, and children, can often be difficult to diagnose. The presenting signs and symptoms may mimic normal child behavior and if dismissed as such, could have devastating consequences. For this reason, it is important to be extremely vigilant and perform a thorough neurological examination when a stroke is suspected, especially in the least expected population of pediatrics. In this case, we describe a pediatric patient with an obscure presentation, and outline our workup which led to the ultimate diagnosis of cerebellar infarct. Dizziness, confusion, and agitation were his presenting symptoms - according to his grandmother, who had accompanied him to the emergency department. Due to his altered mental status, obtaining a more detailed history was difficult. Initial neuroimaging studies were also unremarkable. To further evaluate and treat the patient, he was admitted to the pediatric intensive care unit (PICU). Shortly into his hospital stay, his condition progressed, revealing more definitive signs of neurological compromise. Emergent computerized tomography angiogram (CTA) of the head and neck showed multiple arterial occlusions. Our patient was successfully treated with thrombectomy and craniotomy and showed a promising recovery. Due to our patient’s age and lack of obvious medical comorbidities, we felt that it was imperative to further investigate any underlying causes. What caused this 16-year-old boy have a stroke? Stroke Defined An episode of focal neurological deficit with an acute onset that either lasts longer than 24 hours or less than 24 hours with evidence of stroke on neuroimaging. Epidemiology • The annual incidence of arterial ischemic stroke in infants and children: 0.6 – 7.9 per 100,000 • Males > females • The incidence has increased over the past few decades due to: ‣ Improved neuroimaging Risk Factors • Cardiac conditions, metabolic and hematologic conditions, vascular lesions, trauma, and illicit drug use Diagnosis • CT may be obtained initially, especially to rule out hemorrhage • MRI with diffusion weighted imaging and an MRA are recommended as the initial studies in children due to higher sensitivity for brain ischemia Treatment • Since no randomized controlled trials have been performed on the pediatric population, treatment protocols have been adapted from adult protocols • t-PA within 4.5-hour of onset • If symptomatic + onset of 4.5 – 24 hours → get CTA or MRA, and subsequent mechanical thrombectomy for those with neuroimaging consistent with stroke to the posterior circulation • Begin antithrombotic therapy within 24 hours of confirmed diagnosis Ultimately, the evaluation of strokes in children should be aimed at identifying the underlying cause. Additional imaging of intracranial and extracranial structures, heart structure and function, and obtaining inflammatory markers and hypercoagulable testing is recommended. ‣ Stroke diagnostic protocols ‣ Increased used of illicit drugs Introduction

A stroke in the pediatric population is not easily identified due to the nature of a child’s behavior. Our patient presented with altered mental status after an alleged fall, with no neurologic deficits on physical examination, and a normal head CT. Therefore, his behavior was misattributed to drug ingestion of his own methylphenidate. Because we did not suspect a stroke initially, adequate imaging was not performed. Brain MRI is the preferred study of choice during stroke evaluation in children. An MRI is more sensitive than a CT for acute ischemia, especially in the hyper-acute period. Additionally, it provides better visualization of the posterior fossa. If MRI is not available within one hour after arrival, CT angiography is recommended. After subsequent neurological decline of our patient, a CT angiogram was performed, which demonstrated occlusion of the right vertebral artery and right posterior inferior cerebellar artery (PICA) and occlusion of the basilar tip consistent with acute to sub-acute infarct. Upon confirmation of ischemic stroke, the patient received 300 mg of Plavix, 81mg of Aspirin (rectal) and 250 mL of 3% NaCl in order to minimize brain edema prior to transfer. No t-PA was administered because the window for treatment had passed. He was then transferred to a tertiary center, where a mechanical thrombectomy of the basilar artery and left PICA was successfully performed. Unfortunately, our patient then suffered from a hemorrhagic conversion that required a craniotomy to relieve the increased intracranial pressure. With concern for brain edema, subsequent intubation was also necessary. The etiology of stroke in our patient remains unknown as there was no direct cause identified. There were, however, decreased levels of Protein C and Protein S which potentially indicate a hematologic origin of thrombi in various brain vessels. Although his prognosis was poor, the patient recuperated and was discharged home with neurological rehabilitation therapy. The diagnosis of stroke should always be considered in pediatric patients with altered mental status because even though it is a rare finding, the repercussions are devastating in this young patient population.

An emergent CT and CT-A scan were performed. • findings were consistent with ischemic infarct of the right cerebellar hemisphere

References

1. Danchaivijitr N, Cox TC, Saunders DE, Ganesan V. Evolution of cerebral arteriopathies in childhood arterial ischemic stroke. Ann Neurol 2006; 59:620.

A. CT without contrast – at presentation to ED . Unremarkable. B. CT without contrast – 12 hours after presentation. A large, sub-acute infarct is appreciated in the right cerebellar hemisphere. There is no significant mass effect on the fourth ventricle or downward herniation at the foramen magnum. There is no acute intracranial hemorrhage, mass, or mass effect. C. CT Angiogram – 12 hours after presentation. Occluded right vertebral artery (yellow arrow) and patent left vertebral artery at the V3/V4 segment. D. CT Angiogram – 12 hours after presentation. Right cerebellar hemisphere infarction and filling defect at the basilar artery tip (yellow arrow). The patient was considered to be a candidate for mechanical thrombectomy and was transferred to a tertiary care center for the procedure. Despite successful recanalization, the patient demonstrated significant inability to cooperate with physical exam compared to previous examinations. CT scan showed a worsening mass effect secondary to brain edema . As a result, a decompressive craniotomy was performed. Patient recovered at tertiary center and is rehabilitating at home.

2. deVeber GA,Kirton A, Booth FA, et al. Epidemiology and Outcomes of Arterial Ischemic Stroke in Children: The Canadian Pediatric Ischemic Stroke Registry. Pediatr Neurol 2017; 69:58. 3. Elbers J, Wainwright MS, Amlie-Lefond C. The Pediatric Stroke Code: Early Management of the Child with Stroke. J Pediatr 2015; 167:19. 4. Shellhaas RA, Smith SE, O'Tool E, et al. Mimics of childhood stroke: characteristics of a prospective cohort. Pediatrics 2006; 118:704.

Acknowledgements

We thank Spartanburg Regional Healthcare System and PRISMA Health for their cooperation.

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