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Background Limited dorsal myeloschisis (LDM) is postulated to be a result of incomplete dysjunction in primary neurulation. However, clinical experience of LDM located below the first-second sacral (S1-S2) vertebral level, which is formed from secondary neurulation (S2-coccyx), suggested that LDM may not be entirely explained as an error of primary neurulation. Objective To elucidate the location and characteristics of LDM to investigate the possible relation of its pathoembryogenesis to secondary neurulation. Methods Twenty-eight patients were surgically treated for LDM from 2010 to 2015. Since the level where the LDM stalk penetrates the interspinous ligament is most clearly defined on the preoperative MRI and operative field, this level was assessed to find out whether the lesions can occur in the region of secondary neurulation. Results Eleven patients (39%) with typical morphology of the stalk had interspinous defect levels lower than S1-S2. These patients were not different from 17 patients with classic LDMs at a level above or at S1-S2. This result shows that other than the low level of the interspinous level, 11 patients had lesions that could be defined as LDMs. Conclusion By elucidating the location of LDM lesions (in particular, the interspinous level), we propose that LDM may be caused by errors of secondary neurulation. The hypothesis seems more plausible due to the supportive fact that the process of separation between the cutaneous and neural ectoderm is present during secondary neurulation. Hence, incomplete disjunction of the two ectoderms during secondary neurulation may result in LDM, similar to the pathomechanism proposed during primary neurulation.

BACKGROUND The existence of tethering tracts in spinal dysraphism, other than congenital dermal sinus (CDS), has been recognized and has been summated into an integrated concept of limited dorsal myeloschisis (LDM). OBJECTIVE To elucidate the underlying embryology of LDM in relation to CDS by focusing on the pathological features of special cases of tethering tracts. METHODS Out of 389 spinal dysraphism patients who were operated on from 2010 to 2016, 5 patients who had tethering tracts composed of both CDS and LDM (or "probable LDM" if only fibrous tissue was found) were identified. Their clinical presentation, radiological images, operative findings, and pathology were thoroughly reviewed. RESULTS Three nonsaccular-type patients harbored stalks in which the squamous epithelial lined sinus (CDS) was found in the distal portion, and fibroneural (LDM) or fibrous (probable LDM) tissue in the proximal part. Two patients had saccular lesions, and a stalk was found inside the sac, connecting a small pit on the skin to the spinal cord. The tracts were pathologically identical to a CDS. CONCLUSION This study reports the coexistence of CDS and LDM (or probable LDM) components. These unique cases support the hypothesis that the CDS and LDM are among a spectrum of an anomaly that is caused by failure of complete dysjunction between cutaneous and neural ectoderms. Neurosurgeons should be aware of the possibility of coexisting "CDS" components in cases suggestive of LDM. In such cases, not only untethering but also meticulous removal of the squamous epithelium is critical.

Background There has been controversy regarding the management of syringomyelia associated with tethered spinal cord. Previous reports on the topic have included only a small number of patients, considered open/closed spinal dysraphism together, or had a short follow-up. Objective To review a uniform group of patients with syringomyelia associated with tethered cord and treated mainly by untethering alone. Methods Of the 135 patients operated on for closed spinal dysraphism between 2003 and 2008, 33 patients with preoperative syringomyelia were identified. The preoperative/postoperative clinical data and syrinx index (ratio of the syrinx area and the cord area) were retrospectively reviewed. The syrinx index of each patient was plotted as an individual graph to outline the temporal change of the syrinx before and after untethering surgery. Results Five patients showed symptom progression during the preoperative period, and 4 of the 5 had an additional magnetic resonance imaging before the operation that showed progression of the syringomyelia. Postoperatively, 31 of 32 patients (97%) who underwent postoperative follow-up imaging showed long-term stability or a decrease in the syrinx index. Four symptomatically stable patients showed a transient increase in the syrinx index during the initial postoperative 6 months, which later decreased spontaneously. In 1 patient with retethering, the syrinx index increased 6 months before the onset of new urinary symptoms. Conclusion Untethering alone may be sufficient for the management of syringomyelia associated with tethered cord. A transient increase in the syrinx index during the initial postoperative period may be observed without additional surgery if patients are symptomatically stable.

BACKGROUND: The optimal extent of resection for surgical treatment of lesional epilepsy is a controversial issue. OBJECTIVE: For patients with mesial temporal lobe lesions visible on magnetic resonance imaging, we compared the surgical outcome of selective lesionectomy with that of standard anterior temporal lobectomy (ATL) and amygdalohippocampectomy. METHODS: We conducted a retrospective analysis of the seizure outcome of 40 patients treated for lesional mesial temporal lobe epilepsy between 1993 and 2008. Before 2006, patients were managed by ATL (n = 29) and from 2006 onward, by selective lesionectomy via the transsylvian-transcisternal approach (n = 11). RESULTS: The postoperative seizure-free rates for the 2 groups were comparable: 93% (27/29) for the ATL group and 91 % (10/11) for the selective lesionectomy group (P = .814). In both groups, patients with persistent seizures commonly showed incomplete lesion resection, with complete resection often improving seizure outcome. Postoperative visual field defects were more common in the ATL group (21 %) than in the selective lesionectomy group (0%) (P = .102). CONCLUSION: Transsylvian-transcisternal selective lesionectomy is an effective and safe therapeutic modality in children with lesional mesial temporal lobe epilepsy. Completeness of resection is an important variable for seizure control regardless of surgical modality.

BACKGROUND AND IMPORTANCE: Encephalocraniocutaneous lipomatosis (ECCL) is a rare neurocutaneous syndrome presumably derived from a mesenchymal defect. No cases of ECCL associated with a neuroepithelial brain tumor have been described. Papillary glioneuronal tumor (PGNT) is also a rare brain tumor of mixed neuronal and glial cells. We believe this is the first case of ECCL combined with a PGNT to be described. The presence of the PGNT may reflect the maldevelopment of neuroepithelium in this ECCL patient and suggests a novel explanation of the pathogenesis of ECCL. CLINICAL PRESENTATION: A 7-year-old girl with congenital lesions in the brain, spinal cord, eye, and skin was diagnosed with ECCL. Brain magnetic resonance images taken at 6 months of age showed no brain tumor other than stigmata of ECCL. Brain magnetic resonance image at the time of presentation at 7 years revealed a mass in the third ventricle. The tumor was completely removed, and pathological examination confirmed the diagnosis of PGNT. The tumor showed a divergent differentiation pattern both in the tissue histology and in primary cultures. CONCLUSION: The occurrence of PGNT, a tumor resulting from the maldevelopment of neuroepithelium, broadens the spectrum of ECCL to include some defects in the neuroepithelium.

OBJECTIVE We compared the surgical results of simple encephaloduroarteriosynangiosis (EDAS) and EDAS with bifrontal encephalogaleo(periosteal)synangiosis for the treatment of pediatric moyamoya disease. METHODS Data for 159 children (up to 15 yr of age, 76 boys and 83 girls) who underwent indirect revascularization procedures for the treatment of moyamoya disease between 1987 and 1998 were retrospectively reviewed. Group A patients underwent simple EDAS (n = 67). Group B patients underwent EDAS with bifrontal encephalogaleo(periosteal)synangiosis (n = 92). The surgical results for each group were compared, in terms of clinical outcomes, neuroimaging changes, extent of revascularization evident on angiograms, and hemodynamic changes evident on single-photon emission computed tomographic scans. The average follow-up periods were 45 months for Group A and 22 months for Group B. RESULTS The overall clinical outcomes and neuroimaging changes tended to be better for Group B. In terms of single-photon emission computed tomographic changes of the whole brain after surgery, Group B patients exhibited more favorable outcomes than did Group A patients (62 versus 36%, P = 0.003). The surgical results for the anterior cerebral artery territory were significantly better for Group B than for Group A, with respect to outcomes of anterior cerebral artery symptoms (81 versus 40%, P = 0.015), revascularization on angiograms (79 versus 16%, P < 0.001), and hemodynamic changes on single-photon emission computed tomographic scans (70 versus 52%, P = 0.002). The incidences of postoperative infarctions were not significantly different between the two groups. CONCLUSION EDAS with bifrontal encephalogaleo(periosteal)synangiosis is a more effective surgical modality for the treatment of pediatric moyamoya disease, compared with simple EDAS, because it covers both the middle cerebral artery and anterior cerebral artery territories of the brain.

The prognosis for moyamoya disease (MMD) among young patients is known to be worse than that among older patients. The aim of this study was to investigate the clinical features and treatment outcomes of young patients with MMD.A total of 204 patients with MMD who underwent encephaloduroarteriosynangiosis, with or without bifrontal encephalogaleosynangiosis, were categorized into three groups according to their ages at the time of surgery, i.e., Group A (n = 23,3 yr of age), Group B (n = 50, 3-6 yr of age), and Group C (n = 131,6 yr of age). For each group, patterns of presentation and the occurrence of subsequent preoperative or surgery-related infarctions were assessed. Clinical outcomes and postoperative hemodynamic status were analyzed.At initial presentation, infarctions were significantly more frequent in Group A (87%) and Group B (58%) than in Group C (46%). Subsequent preoperative infarctions occurred significantly more frequently in Group A (39%) than in Group B (6%) or Group C (0.8%). The median interval between the onset of symptoms and a subsequent preoperative infarction was 3 months (range, 1-14 mo). No significant difference in the rates of surgery-related infarctions among the three groups was observed. The rate of favorable clinical outcomes was significantly lower in Group A (58%) than in Group B (84%) or Group C (86%), although the rates of postoperative hemodynamic improvements were similar among the groups. The poor clinical outcomes for Group A were caused mainly by preoperative infarctions.Young-age MMD demonstrates rapid disease progression and results in poor clinical outcomes. These findings indicate the necessity of early surgery for young patients with MMD; however, the actual benefits should be verified with additional controlled studies, with long-term follow-up monitoring.

OBJECTIVE We compared the surgical results of simple encephaloduroarteriosynangiosis (EDAS) and EDAS with bifrontal encephalogaleo(periosteal)synangiosis for the treatment of pediatric moyamoya disease. METHODS Data for 159 children (up to 15 yr of age, 76 boys and 83 girls) who underwent indirect revascularization procedures for the treatment of moyamoya disease between 1987 and 1998 were retrospectively reviewed. Group A patients underwent simple EDAS (n = 67). Group B patients underwent EDAS with bifrontal encephalogaleo(periosteal)synangiosis (n = 92). The surgical results for each group were compared, in terms of clinical outcomes, neuroimaging changes, extent of revascularization evident on angiograms, and hemodynamic changes evident on single-photon emission computed tomographic scans. The average follow-up periods were 45 months for Group A and 22 months for Group B. RESULTS The overall clinical outcomes and neuroimaging changes tended to be better for Group B. In terms of single-photon emission computed tomographic changes of the whole brain after surgery, Group B patients exhibited more favorable outcomes than did Group A patients (62 versus 36%, P = 0.003). The surgical results for the anterior cerebral artery territory were significantly better for Group B than for Group A, with respect to outcomes of anterior cerebral artery symptoms (81 versus 40%, P = 0.015), revascularization on angiograms (79 versus 16%, P < 0.001), and hemodynamic changes on single-photon emission computed tomographic scans (70 versus 52%, P = 0.002). The incidences of postoperative infarctions were not significantly different between the two groups. CONCLUSION EDAS with bifrontal encephalogaleo(periosteal)synangiosis is a more effective surgical modality for the treatment of pediatric moyamoya disease, compared with simple EDAS, because it covers both the middle cerebral artery and anterior cerebral artery territories of the brain.

INTRODUCTION The coronavirus disease 2019 (COVID-19) pandemic that is caused by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection has had a dramatic impact on healthcare systems and a variable disease course Emerging evidence demonstrates that SARS-CoV-2 is associated with central nervous system (CNS) disease In this series, we describe CNS manifestations in critical COVID-19 patients at our tertiary academic center METHODS A single center retrospective cross-sectional analysis of all patients admitted to our tertiary care academic center in New Orleans, Louisiana on April 22, 2020, who were in critical condition due to COVID-19 and developed new onset of neurological disease Patients were grouped into one of three categories according to imaging and clinical features: encephalopathy, acute necrotizing encephalopathy, and vasculopathy RESULTS A total of 27 of 76 (35 5%) critical COVID-19 patients met inclusion criteria Mean age was 59 8 years (range 35–91 years) and most had an underlying medical condition, including hypertension (63%), diabetes mellitus type 2 (52%), obesity (26%), and/or chronic kidney disease (22%) Sixty three percent had evidence of neurological injury on CT, 30% on MRI, 15% on non-invasive vascular imaging, and 44% on EEG CT findings most often included subacute ischemic strokes, diffuse hypoattenuation, subcortical parenchymal hemorrhages, and focal hypodensities within deep structures MRI findings included diffuse involvement of deep white matter, the corpus callosum, and the basal ganglia For patients with acute ischemic stroke, vascular findings consisted of irregular proximal focal stenosis of the supraclinoid internal carotid artery Twenty patients (74%) were designated with COVID-19 associated encephalopathy, two (7%) with COVID-19 associated acute necrotizing encephalopathy, and five (19%) with COVID-19 associated vasculopathy CONCLUSION A one-day snapshot of COVID-19 admissions at a tertiary academic center in New Orleans, LA revealed a high percentage of patients with new neurological disease Although clinical presentations varied, they were broadly categorized A better understanding of the neurological sequalae and radiographic findings will help clinicians mitigate the impact of this disease