Miya E. Bernson-Leung

Synthetic Cannabis and Acute Ischemic Stroke

An association between marijuana use and stroke has been previously reported. However, the health risks of newer synthetic cannabinoid compounds are less well known. We describe 2 cases that introduce a previously unreported association between synthetic cannabis use and ischemic stroke in young adults. A 22-year-old woman presented with dysarthria, left hemiplegia, and left hemianesthesia within hours of first use of synthetic cannabis. She was healthy and without identified stroke risk factors other than oral contraceptive use and a patent foramen ovale without venous thromboses. A 26-year-old woman presented with nonfluent aphasia, left facial droop, and left hemianesthesia approximately 12 hours after first use of synthetic cannabis. Her other stroke risk factors included migraine with aura, oral contraceptive use, smoking, and a family history of superficial thrombophlebitis. Both women were found to have acute, large-territory infarctions of the right middle cerebral artery. Our 2 cases had risk factors for ischemic stroke but were otherwise young and healthy and the onset of their deficits occurred within hours after first-time exposure to synthetic cannabis. Synthetic cannabis use is an important consideration in the investigation of stroke in young adults.

Stroke in Neonates and Children

1. Miya E. Bernson-Leung, MD*,§ 2. Michael J. Rivkin, MD*,†,‡,§ 1. Departments of *Neurology, 2. †Radiology, and 3. ‡Psychiatry, Boston Children’s Hospital, Boston, MA 4. §Department of Neurology, Harvard Medical School, Boston, MA 1. Pediatricians should be aware of the appropriate initial evaluation of suspected stroke in children to combat documented delays in diagnosis of greater than 24 hours. (1) 2. Pediatricians should be aware of primary and secondary prevention measures for stroke in at-risk populations, such as transcranial Doppler ultrasonography screening for children with sickle cell disease. 3. Pediatricians must recognize the clinical findings associated with childhood stroke, including ischemic and hemorrhagic stroke, understand the prognosis for each type of stroke, and delineate optimal ongoing management and rehabilitation. After completing this article, readers should be able to: 1. Describe the pathophysiology of stroke in infants, children, and adolescents. 2. Recognize the clinical findings associated with childhood stroke and with arteriovenous malformations and other cerebrovascular abnormalities predisposing to childhood stroke. 3. Delineate the appropriate initial evaluation of suspected stroke in children. Stroke in children is surprisingly common, affecting 1 in 1,600 to 4,000 neonates at birth (2)(3) and 2.3 to 13 per 100,000 older children per year. (4)(5)(6) Strokes can occur in seemingly healthy infants, children, and adolescents. Despite the plasticity of the developing brain, stroke in children carries substantial morbidity. Early recognition of childhood stroke by pediatric clinicians is crucial for the rapid implementation of targeted and neuroprotective therapies. Clinicians should be familiar with risk factors for pediatric stroke and appropriate prevention strategies. Finally, pediatricians are key members of an integrated, multidisciplinary approach to stroke treatment and rehabilitation. This review begins with a discussion of stroke in children older than 28 days, followed by a discussion of perinatal stroke (before 28 days of age) because strokes in these 2 age groups have …

Increased Pediatric Functional Neurological Symptom Disorders After the Boston Marathon Bombings: A Case Series

BACKGROUND Functional neurological symptom disorders are frequently the basis for acute neurological consultation. In children, they are often precipitated by high-frequency everyday stressors. The extent to which a severe traumatic experience may also precipitate functional neurological abnormalities is unknown. METHODS For the 2-week period after the Boston Marathon bombings, we prospectively collected data on patients whose presentation suggested a functional neurological symptom disorder. We assessed clinical and demographic variables, duration of symptoms, extent of educational impact, and degree of connection to the Marathon bombing. We contacted all patients at 6 months after presentation to determine the outcome and accuracy of the diagnosis. RESULTS In a parallel study, we reported a baseline of 2.6 functional neurological presentations per week in our emergency room. In the week after the Marathon bombings, this frequency tripled. Ninety-one percent of presentations were delayed by 1 week, with onset around the first school day after a city-wide lockdown. Seventy-three percent had a history of a prior psychiatric diagnosis. At the 6 months follow-up, no functional neurological symptom disorder diagnoses were overturned and no new organic diagnosis was made. CONCLUSIONS Pediatric functional neurological symptom disorder may be precipitated by both casual and high-intensity stressors. The 3.4-fold increase in incidence after the Boston Marathon bombings and city-wide lockdown demonstrates the marked effect that a community-wide tragedy can have on the mental health of children. Care providers must be aware of functional neurological symptom disorders after stressful community events in vulnerable patient populations, particularly those with prior psychiatric diagnoses.

Placental Pathology in Neonatal Stroke: A Retrospective Case-Control Study

Objective To assess the association of placental abnormalities with neonatal stroke. Study design This retrospective case‐control study at 3 academic medical centers examined placental specimens for 46 children with neonatal arterial or venous ischemic stroke and 99 control children without stroke, using a standard protocol. Between‐group comparisons used χ2 and Fisher exact t test. Correlations used Spearman correlation coefficient. Results Case placentas were more likely than controls to meet criteria for ≥1 of 5 major categories of pathologic abnormality (89% vs 62%; OR, 5.1; 95% CI, 1.9–14.0; P = .0007) and for ≥2 categories (38% vs 8%; OR, 7.3; 95% CI, 2.9–19.0; P < .0001). Fetal vascular malperfusion occurred in 50% of cases and 17% of controls (OR, 4.8; 95% CI, 2.2–10.5; P = .0001). Amniotic fluid inflammation occurred in 46% of cases with arterial ischemic stroke vs 25% of controls (OR, 2.6; 95% CI, 1.1–6.1; P = .037). There was evidence of a “stress response” (meconium plus elevated nucleated red blood cells) in 24% of cases compared with 1% of controls (OR, 31; 95% CI, 3.8–247.0; P < .0001). Conclusions Placental abnormality was more common in children with neonatal stroke compared with controls. All placental findings represent subacute‐to‐chronic intrauterine stressors. Placental thrombotic processes were associated with both arterial and venous stroke. Our findings provide evidence for specific mechanisms that may predispose to acute perinatal stroke. Amniotic fluid inflammation associated with neonatal arterial ischemic stroke deserves further investigation.

Transient Focal Neurologic Symptoms Correspond to Regional Cerebral Hypoperfusion by MRI: A Stroke Mimic in Children

SUMMARY: Children who present with acute transient focal neurologic symptoms raise concern for stroke or transient ischemic attack. We present a series of 16 children who presented with transient focal neurologic symptoms that raised concern for acute stroke but who had no evidence of infarction and had unilateral, potentially reversible imaging features on vascular and perfusion-sensitive brain MR imaging. Patients were examined with routine brain MR imaging, MRA, perfusion-sensitive sequences, and DWI. Fourteen (88%) children had lateralized MRA evidence of arterial tree pruning without occlusion, all had negative DWI findings, and all showed evidence of hemispheric hypoperfusion by susceptibility-weighted imaging or arterial spin-labeling perfusion imaging at presentation. These findings normalized following resolution of symptoms in all children who had follow-up imaging (6/16, 38%). The use of MR imaging with perfusion-sensitive sequences, DWI, and MRA can help to rapidly distinguish children with conditions mimicking stroke from those with acute stroke.

Workup for Perinatal Stroke Does Not Predict Recurrence

Background and Purpose— Perinatal stroke, including neonatal and presumed perinatal presentation, represents the age in childhood in which stroke occurs most frequently. The roles of thrombophilia, arteriopathy, and cardiac anomalies in perinatal ischemic stroke are currently unclear. We took a uniform approach to perinatal ischemic stroke evaluation to study these risk factors and their association with recurrent stroke. Methods— We reviewed records of perinatal stroke patients evaluated from August 2008 to February 2016 at a single referral center. Demographics, echocardiography, arterial imaging, and thrombophilia testing were collected. Statistical analysis was performed using Fisher exact test. Results— Across 215 cases, the median follow-up was 3.17 years (1.49, 6.46). Females comprised 42.8% of cases. Age of presentation was neonatal (110, 51.2%) or presumed perinatal (105, 48.8%). The median age at diagnosis was 2.9 days (interquartile range, 2.0–9.9) for neonatal stroke and 12.9 months (interquartile range, 8.7–32.8) for presumed perinatal stroke. Strokes were classified as arterial (149, 69.3%), venous (60, 27.9%), both (4, 1.9%), or uncertain (2, 0.9%) by consensus imaging review. Of the 215 cases, there were 6 (2.8%) recurrent ischemic cerebrovascular events. Abnormal thrombophilia testing was not associated with recurrent stroke, except for a single patient with combined antithrombin deficiency and protein C deficiency. After excluding venous events, 155 patients were evaluated for arteriopathy and cardioembolic risk factors; neither was associated with recurrent stroke. Positive family history of thrombosis was not predictive of abnormal thrombophilia testing. Conclusions— Thrombophilia, arteriopathy, or cardioembolic risk factors were not predictive of recurrent events after perinatal stroke. Thrombophilia evaluation in perinatal stroke should only rarely be considered.

Education on the Brain: A Partnership Between a Pediatric Primary Care Center and Neurology Residency

The national shortage of pediatric neurologists is worsening, yet referral rates by pediatricians are high. Suboptimal training of pediatric residents in care of patients with neurologic disease may be a contributing factor. We formed a partnership between the Boston Children’s Primary Care at Longwood clinic and Child Neurology Residency Training Program. The educational intervention included lectures, observed neurologic examinations, in-person and virtual triage, and an electronic medical record–based consult system. Residents in other primary care clinics served as the comparison group. Intervention-group residents reported significantly improved confidence in diagnosis of chronic/recurrent headache, attention deficit hyperactivity disorder (ADHD), and developmental delay; initial management of ADHD and developmental delay; and secondary management of ADHD, developmental delay, and concussion/traumatic brain injury. Comparison-group residents reported significantly improved confidence only in diagnosis of developmental delay. Our multipronged intervention is a promising approach to improving pediatric resident training in pediatric neurology and may be generalizable to subspecialty collaborations for other residency programs.

Shared Care: Using an Electronic Consult Form to Facilitate Primary Care Provider–Specialty Care Coordination

OBJECTIVE The quality of childrens health is compromised by poor care coordination between primary care providers (PCPs) and specialists. Our objective was to determine how an electronic consultation and referral system impacts referral patterns and PCP-specialist communication. METHODS The primary care clinic at Boston Childrens Hospital piloted an electronic referral and consultation system with the neurology and gastroenterology departments from April 1, 2014, to October 31, 2016. PCPs completed an electronic consult form, and if needed, specialists replied with advice or facilitated expedited appointments. Specialist response times, referral rates, wait times, and completion rates for specialty visits were tracked. PCPs and specialists also completed a survey to evaluate feasibility and satisfaction. RESULTS A total of 82 PCPs placed 510 consults during the pilot period. Specialists responded to 88% of requests within 3 business days. Eighteen percent of specialty visits were deferred and 21% were expedited. Wait times for specialty appointments to both departments significantly decreased, from 48 to 34 days (P < .001), and completion rates improved from 58% to 70% (P < .01), but referral volumes remained stable (25 per month to 23 per month; P = .29). Most PCPs said the Shared Care system facilitated better communication with specialists (89%) and enabled them to provide superior patient care (92%). Specialists reported that the system required a minimal amount of time and enabled them to educate PCPs and triage referrals. CONCLUSIONS Implementation of an electronic referral and consultation system was feasible and provided timely access to specialty care, but did not affect referral volume. This system could serve as a model for other health care organizations and specialties.

Emerging Subspecialties in Neurology: Pediatric stroke and cerebrovascular disorders

In 1895, a neuropathologist at the University of Vienna observed that “a large number of cases of infantile cerebral palsy is caused by the same factors that bring about the majority of cases of cerebral paralysis of adults: by tearing, embolism, and thrombosis of cerebral vessels.”1 While Sigmund Freud would go on to become better known for other endeavors, his observation that children can have strokes paved the way for the development of the emerging subspecialty of pediatric stroke and cerebrovascular disorders.

Cerebrovascular Complications of Pediatric Pneumococcal Meningitis in the PCV13 Era

### Case 1 A previously healthy, full-term 5-month-old girl presented to the emergency department with a 1-day history of fever to 103°F, emesis, poor feeding, and progressive lethargy. She had recently started day care. The patient had received her routine 2- and 4-month childhood vaccines on schedule, including the 13-valent pneumococcal conjugate vaccine (PCV13). Initial examination revealed decreased responsiveness, rightward gaze preference, decreased movement of the left side, and positive Brudzinski sign. Laboratory testing is as in Table 1. Ceftriaxone and vancomycin were started at meningitic dosing. Cerebrospinal fluid grew Streptococcus pneumoniae serotype 33F, a nonvaccine serotype. View this table: TABLE 1 Laboratory Testing ### Case 2 A previously healthy 21-month-old boy presented to the emergency department with a 1-day history of fever to 103°F, emesis, cough, and lethargy. Rapid influenza testing was positive for influenza A, and oseltamivir was started. History was notable for day care attendance and multiple sick contacts. He was fully immunized, including PCV13. The next morning, he had a brief seizure with eye deviation and generalized convulsions, and became obtunded. Simultaneously, blood culture grew gram-positive cocci in pairs and chains. He returned to the emergency department, where he had right-sided weakness, posturing, rightward eye deviation, and meningismus as well as further seizures requiring lorazepam and fosphenytoin. Laboratory testing is as in Table 1. Ceftriaxone and vancomycin were started at meningitic dosing. Cerebrospinal fluid grew S pneumoniae serotype 15B, a nonvaccine serotype. ### Question: What are possible causes of altered mental status, seizures, and focal neurologic deficits in a febrile child, and what further evaluation should be performed? ### Discussion Lethargy, seizures, and focal neurologic deficits immediately raise concern for bacterial meningitis or meningoencephalitis, even …