ALS is a multisystem disorder affecting cognitive and motor functions. Bulbar-onset ALS (bALS) may be preferentially associated with language/cognitive impairments, compared with spinal-onset ALS (sALS), stemming from a potentially unique neuropathology. The objective of this systematic review was to compare neuropathology reported for bALS and sALS subtypes in studies of cadaveric brains. Using Cochrane guidelines, we reviewed articles in MEDLINE, Embase, and PsycINFO databases using standardized search terms for ALS and neuropathology, from inception until July 16th 2016. 17 studies were accepted. In summary, both subtypes presented with involvement in motor and frontotemporal cortices, deep cortical structures, and cerebellum, characterized by neuronal loss, spongiosis, myelin pallor, and ubiquitin+ and TDP43+ inclusion bodies. Changes in Broca and Wernicke areas, regions associated with speech and language processing, were noted exclusively in bALS. Further, some bALS cases presented with atypical pathology, neurofibrillary tangles and basophilic inclusions, which were not found in any sALS cases. Given the few studies, all with methodological biases, further work is required to better understand neuropathology of ALS subtypes.

Background Prognostic factors in adolescent and young adult (AYA) glioma are not well understood. Though clinical and molecular differences between pediatric and adult glioma have been characterized, their application to AYA populations is less clear. There is a major need to develop more robust evidence-based practices for managing AYA glioma patients. Methods Systematic review using PRISMA methodology was conducted using multiple databases with the objective of identifying demographic, clinical, molecular and treatment factors influencing AYA glioma outcomes. Results 40 studies met inclusion criteria. Overall survival was highly variable across studies depending on glioma grade, anatomic compartment and cohort characteristics. Thirty-five studies suffered from high risk of bias in at least one domain. Several studies included older adults within their cohorts; few captured purely AYA groups. Despite study heterogeneity, identified favorable prognosticators included younger age, higher functional status at diagnosis, low grade pathology, oligodendroglioma histology and increased extent of surgical resection. Though isocitrate dehydrogenase (IDH) mutant status was associated with favorable prognosis, validity of this finding within AYA was compromised though may studies including older adults. The prognostic influence of chemotherapy and radiotherapy on overall survival varied across studies with conflicting evidence. Conclusion Existing literature is heterogenous, at high risk of bias, and rarely focused solely on AYA patients. Many included studies did not reflect updated pathological and molecular AYA glioma classification. The optimal role of chemotherapy, radiotherapy, and targeted agents cannot be determined from existing literature and should be the focus of future studies.

BACKGROUND Cervical spine injuries in neonates are rare and no guidelines are available to inform management. The most common etiology of neonatal cervical injury is birth-related trauma. Management strategies that are routine in older children and adults are not feasible due to the unique anatomy of neonates. OBSERVATIONS Here, the authors present 3 cases of neonatal cervical spinal injury due to confirmed or suspected birth trauma, 2 of whom presented immediately after birth, while the other was diagnosed at 7 weeks of age. One child presented with neurological deficits due to spinal cord injury, while another had an underlying predisposition to bony injury, infantile malignant osteopetrosis. The children were treated with a custom-designed and manufactured full-body external orthoses with good clinical and radiographic outcomes. A narrative literature review further supplements this case series and highlights risk factors and the spectrum of birth-related spinal injuries reported to date. LESSONS The current report highlights the importance of recognizing the rare occurrence of cervical spinal injury in newborns and provides pragmatic recommendations for management of these injuries. Custom orthoses provide an alternate option for neonates who cannot be fitted in halo vests and who would outgrow traditional casts.

Marginal zone B-cell lymphoma (MZBCL) of mucosa-associated lymphoid tissue (MALT) type, which is primary to the central nervous system (CNS), is a rare lesion, with those originating within the parenchyma even more so. We present the case of a 64-year-old male with weakness in the left hand and focal motor seizures of his arm, who was found to have a right frontal intraparenchymal lesion. Following resection, histopathological and immunohistochemical evaluations were completed, leading to a diagnosis of a primary CNS MZBCL of MALT type in the context of a negative workup of systemic disease. Neuroimaging, histopathological, and immunohistochemical findings, as well as a comprehensive literature review of similar cases, are discussed.