SUMMARYObjective: Although the link between vigabatrin (VGB) and retinotoxicity is well known, little attention has been focused on the risk of VGB-associated brain abnormalities on magnetic resonance imaging (MRI) (VABAM), namely reversible-and largely asymptomatic-signal changes in the thalami, basal ganglia, brainstem tegmentum, and cerebellar nuclei. Using a large infantile spasms cohort, we set out to identify predictors of these phenomena. Methods: Children with infantile spasms were retrospectively identified. Brain MRI reports were serially reviewed without knowledge of VGB exposure. Upon VABAM discovery, records were systematically reviewed to ascertain presence of symptoms attributable to VGB. Separately, progress notes were sequentially reviewed to identify and quantify VGB exposure. Results: We identified 507 brain MRI studies among 257 patients with infantile spasms. VGB treatment was documented in 143 children, with detailed exposure data available for 104, of whom 45 had at least one MRI study during VGB treatment. Among the limited subset of asymptomatic children who underwent MRI (n = 40), 6 exhibited VABAM. Risk of asymptomatic VABAM was dose-dependent, as peak (but not cumulative) VGB dosage was strongly associated with asymptomatic VABAM (p = 0.0028). In an exploratory analysis, we encountered 4 children with symptomatic VABAM among 104 patients with detailed VGB exposure data. Risk of symptomatic VABAM was seemingly dose-independent, and potentially associated with concomitant hormonal therapy (i.e., prednisolone and adrenocorticotropic hormone [ACTH]) (p = 0.039). Significance: We have demonstrated dose-dependent risk of asymptomatic VABAM and uncovered a possible association between symptomatic VABAM and concomitant hormonal therapy. Caution should be exercised in the use of high VGB dosage (i.e., >175 mg/kg/day), and further study is warranted to confirm the potential impact of hormonal therapy. KEY WORDS: West syndrome, Epileptic spasms, Toxicity, Neuroimaging.Infantile spasms (also known as epileptic spasms in the most recently proposed International League Against Epilepsy (ILAE) classification scheme 1 ) is an often devastating form of epilepsy with onset in the first year of life; is frequently attributed to one of many structural, genetic, or metabolic disorders; and is usually accompanied by neurodevelopmental arrest or regression.2 Infantile spasms is characterized by clusters of brief seizures termed spasms and a spectrum of severe electroencephalographic abnormalities including hypsarrhythmia.3 A lack of prompt and successful treatment is associated with adverse long-term 8 Despite recognized efficacy, 9,10 the use of VGB has been limited foremost by reports of retinopathy resulting in permanent peripheral visual field defects termed VGB-associated visual field loss (VAVFL).11,12 Estimates of VAVFL risk vary substantially, though the risk appears to be lower among children, 13 and especially low among infants with treatment duration <12 months.14 Similarly, in a recent large-sca...

We found that the risk of clinically apparent vision loss is quite low among young children treated for infantile spasms. Our estimate of risk contrasts with prior studies and likely reflects our ascertainment of vision loss without the aid of perimetry or serial ERG, the short treatment duration, and the relatively young age of our patients. In the treatment of infantile spasms, risk-benefit assessment should consider both the low prevalence of ERG-identified VAVFL among patients with brief (<6-9months) exposure and the very low prevalence of clinically apparent VAVFL in this population.

Paragangliomas are rare neuroendocrine tumors that arise from chromaffin-containing tissue. Surgical resection and/or radiation are used for locoregional disease, and reduction of tumor burden with systemic therapy is reserved for metastatic disease. Iobenguane I-131, somatostatin analog (octreotide), and Sunitinib are noncytotoxic options for treatment, while cyclophosphamide, vincristine, and dacarbazine (CVD) and temozolomide are often used as initial chemotherapy options as studies have shown that they offer some tumor response. However, there are no randomized clinical trials demonstrating prolonged survival with the use of chemotherapeutics in metastatic cases. Investigation of alternative therapies that provide survival benefit is thus necessary. We present a case of a 69-year-old female with metastatic malignant paraganglioma presenting as a left parapharyngeal neck mass, which metastasized after surgery, requiring radiation therapy for bony metastasis who was treated with a radioisotope somatostatin analog for disease progression.

Objectives: To evaluate changes in imaging practices for pediatric head trauma after publication of the Pediatric Emergency Care Applied Research Network (PECARN) guidelines, explore areas for quality improvement regarding neuroradiology referrals. We also sought to determine the prevalence of incidental findings discovered on computed tomographies (CTs) attained for minor head trauma and ascertain disposition in these cases.Methods: This retrospective study was conducted at a rural academic center and included 156 children who received CTs for head trauma between 2005 and 2015. Subjects were divided into 2 groups: pre-PECARN publication and post-PECARN publication. Electronic medical records were reviewed to determine whether or not head CTs were obtained according to PECARN guidelines. The proportion of scanned cases and incidental findings in each group was then compared.Results: Significantly more subjects met PECARN criteria for head CT during the pre-PECARN period (67.1% vs 50.6%, P = 0.04). Among those who met PECARN criteria, severe mechanism of injury was the most common criterion met in both groups (43.8% pre-PECARN and 26.5% post-PECARN). Nine (5.7%) subjects had incidental findings (similar for both study periods), of which 3 prompted additional diagnostic testing or invasive intervention. Among those who did not meet PECARN criteria, the most common mechanism of injury was fall (<3 ft).Conclusions: Implementation of PECARN guidelines at our center remained limited in the 5 years after publication of this practice guide. Clinically insignificant incidental findings were often detected and may heighten patient anxiety.

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