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Christoph J. Griessenauer, Elias Rizk, Joseph H. Miller, Philipp Hendrix, R. Shane Tubbs, Mark S. Dias, Kelsie Riemenschneider and Joshua J. Chern

Object

Tectal plate gliomas are generally low-grade astrocytomas with favorable prognosis, and observation of the lesion and management of hydrocephalus remain the mainstay of treatment.

Methods

A cohort of patients with tectal plate gliomas at 2 academic institutions was retrospectively reviewed.

Results

Forty-four patients with a mean age of 10.2 years who harbored tectal plate gliomas were included in the study. The mean clinical and radiological follow-up was 7.6 ± 3.3 years (median 7.9 years, range 1.5–14.7 years) and 6.5 ± 3.1 years (median 6.5 years, range 1.1–14.7 years), respectively. The most frequent intervention was CSF diversion (81.8% of patients) followed by biopsy (11.4%), radiotherapy (4.5%), chemotherapy (4.5%), and resection (2.3%). On MR imaging tectal plate gliomas most commonly showed T1-weighted isointensity (71.4%), T2-weighted hyperintensity (88.1%), and rarely enhanced (19%). The initial mean volume was 1.6 ± 2.2 cm3 and it increased to 2.0 ± 4.4 cm3 (p = 0.628) at the last follow-up. Frontal and occipital horn ratio (FOHR) and third ventricular width statistically decreased over time (p < 0.001 and p < 0.05, respectively).

Conclusions

The authors' results support existing evidence that tectal plate gliomas frequently follow a benign clinical and radiographic course and rarely require any intervention beyond management of associated hydrocephalus.

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Christoph J. Griessenauer, Smeer Salam, Philipp Hendrix, Daxa M. Patel, R. Shane Tubbs, Jeffrey P. Blount and Peter A. Winkler

OBJECT

Evidence in support of hemispherectomy stems from a multitude of retrospective studies illustrating individual institutions' experience. A systematic review of this topic, however, is lacking in the literature.

METHODS

A systematic review of hemispherectomy for the treatment of refractory epilepsy available up to October 2013 was performed using the following inclusion criteria: reports of a total of 10 or more patients in the pediatric age group (≤ 20 years) undergoing hemispherectomy, seizure outcome reported after a minimum follow-up of 1 year after the initial procedure, and description of the type of hemispherectomy. Only the most recent paper from institutions that published multiple papers with overlapping study periods was included. Two reviewers independently applied the inclusion criteria and extracted all the data.

RESULTS

Twenty-nine studies with a total of 1161 patients met the inclusion criteria. Seizure outcome was available for 1102 patients, and the overall rate of seizure freedom at the last follow-up was 73.4%. Sixteen studies (55.2%) exclusively reported seizure outcomes of a single type of hemispherectomy. There was no statistically significant difference in seizure outcome and type of hemispherectomy (p = 0.737). Underlying etiology was reported for 85.4% of patients with documented seizure outcome, and the overall distribution of acquired, developmental, and progressive etiologies was 30.5%, 40.7%, and 28.8%, respectively. Acquired and progressive etiologies were associated with significantly higher seizure-free rates than developmental etiologies (p < 0.001). Twenty of the 29 studies (69%) reported complications. The overall rate of hydrocephalus requiring CSF diversion was 14%. Mortality within 30 days was 2.2% and was not statistically different between types of hemispherectomy (p = 0.787).

CONCLUSIONS

Hemispherectomy is highly effective for treating refractory epilepsy in the pediatric age group, particularly for acquired and progressive etiologies. While the type of hemispherectomy does not have any influence on seizure outcome, hemispherotomy procedures are associated with a more favorable complication profile.

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Philipp Hendrix, Paul M. Foreman, Mark R. Harrigan, Winfield S. Fisher III, Nilesh A. Vyas, Robert H. Lipsky, Mingkuan Lin, Beverly C. Walters, R. Shane Tubbs, Mohammadali M. Shoja, Jean-Francois Pittet, Mali Mathru and Christoph J. Griessenauer

OBJECTIVE

Cystathionine β-synthase (CBS) is involved in homocysteine and hydrogen sulfide (H2S) metabolism. Both products have been implicated in the pathophysiology of cerebrovascular diseases. The impact of CBS polymorphisms on aneurysmal subarachnoid hemorrhage (aSAH) and its clinical sequelae is poorly understood.

METHODS

Blood samples from all patients enrolled in the CARAS (Cerebral Aneurysm Renin Angiotensin System) study were used for genetic evaluation. The CARAS study prospectively enrolled aSAH patients at 2 academic institutions in the United States from 2012 to 2015. Common CBS polymorphisms were detected using 5′exonuclease genotyping assays. Analysis of associations between CBS polymorphisms and aSAH was performed.

RESULTS

Samples from 149 aSAH patients and 50 controls were available for analysis. In multivariate logistic regression analysis, the insertion allele of the 844ins68 CBS insertion polymorphism showed a dominant effect on aSAH. The GG genotype of the CBS G/A single nucleotide polymorphism (rs234706) was independently associated with unfavorable functional outcome (modified Rankin Scale Score 3–6) at discharge and last follow-up, but not clinical vasospasm or delayed cerebral ischemia (DCI).

CONCLUSIONS

The insertion allele of the 844ins68 CBS insertion polymorphism was independently associated with aSAH while the GG genotype of rs234706 was associated with an unfavorable outcome both at discharge and last follow-up. Increased CBS activity may exert its neuroprotective effects through alteration of H2S levels, and independent of clinical vasospasm and DCI.

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Christoph J. Griessenauer, Robert M. Starke, Paul M. Foreman, Philipp Hendrix, Mark R. Harrigan, Winfield S. Fisher III, Nilesh A. Vyas, Robert H. Lipsky, Mingkuan Lin, Beverly C. Walters, Jean-Francois Pittet and Mali Mathru

OBJECTIVE

Endothelin-1, a potent vasoconstrictor, and its receptors may be involved in the pathogenesis of aneurysmal subarachnoid hemorrhage (aSAH), clinical vasospasm, delayed cerebral ischemia (DCI), and functional outcome following aSAH. In the present study, common endothelin single nucleotide polymorphisms (SNPs) and their relation to aSAH were evaluated.

METHODS

Blood samples from all patients enrolled in the Cerebral Aneurysm Renin Angiotensin System (CARAS) study were used for genetic evaluation. The CARAS study prospectively enrolled patients with aSAH at 2 academic institutions in the US from 2012 to 2015. Common endothelin SNPs were detected using 5′ exonnuclease (TaqMan) genotyping assays. Analysis of associations between endothelin SNPs and aSAH and its clinical sequelae was performed.

RESULTS

Samples from 149 patients with aSAH and 50 controls were available for analysis. In multivariate logistic regression analysis, the TG (odds ratio [OR] 2.102, 95% confidence interval [CI] 1.048–4.218, p = 0.036) and TT genotypes (OR 7.884, 95% CI 1.003–61.995, p = 0.05) of the endothelin-1 T/G SNP (rs1800541) were significantly associated with aSAH. There was a dominant effect of the G allele (CG/GG genotypes; OR 4.617, 95% CI 1.311–16.262, p = 0.017) of the endothelin receptor A G/C SNP (rs5335) on clinical vasospasm. Endothelin SNPs were not associated with DCI or functional outcome.

CONCLUSIONS

Common endothelin SNPs were found to be associated with presentation with aSAH and clinical vasospasm. Further studies are required to elucidate the relevant pathophysiology and its potential implications in the treatment of patients with aSAH.

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Benedikt W. Burkhardt, Andreas Simgen, Gudrun Wagenpfeil, Philipp Hendrix, Matthias Dehnen, Wolfgang Reith and Joachim M. Oertel

OBJECTIVE

There is currently no consensus on whether adjacent-segment degeneration (ASD), loss of disc height (DH), and loss of sagittal segmental angle (SSA) are due to anterior cervical discectomy and fusion (ACDF). The purpose of the present study was to assess the grade of segmental degeneration after ACDF and to analyze if there is a difference with respect to clinical outcome, diagnosis, and number of operated levels.

METHODS

A total of 102 patients who underwent ACDF with a minimum follow-up of 18 years were retrospectively identified. At final follow-up, the clinical outcome according to Odom’s criteria, the Neck Disability Index (NDI), and reoperation for symptomatic ASD (sASD) was assessed. MRI was performed, and DH, SSA, and the segmental degeneration index (SDI, a 5-step grading system that includes disc signal intensity, anterior and posterior disc protrusion, narrowing of the disc space, and foraminal stenosis) were assessed for evaluation of the 2 adjacent and 4 adjoining segments to the ACDF. MRI findings were compared with respect to clinical outcome (NDI: 0%–20% vs > 20%; Odom’s criteria: success vs no success), reoperation for sASD, initial diagnosis (cervical disc herniation [CDH] vs cervical spondylotic myelopathy [CSM] and spondylosis), and the number of operated levels (1 vs 2–4 levels).

RESULTS

The mean follow-up was 25 years (range 18–45 years), and the diagnosis was CDH in 74.5% of patients and CSM/spondylosis in 25.5%. At follow-up, the mean NDI was 12.4% (range 0%–36%), the clinical success rate was 87.3%, and the reoperation rate for sASD was 15.7%. For SDI, no significant differences were seen with respect to NDI, Odom’s criteria, and sASD. Patients diagnosed with CDH had significantly more degeneration at the adjacent segments (cranial, p = 0.015; caudal, p = 0.017). Patients with a 2- to 4-level procedure had less degeneration at the caudal adjacent (p = 0.011) and proximal adjoining (p = 0.019) segments. Aside from a significantly lower DH at the proximal cranial adjoining segment in cases of CSM/spondylosis and without clinical success, no further differences were noted. The degree of SSA was not significantly different with respect to clinical outcome.

CONCLUSIONS

No significant differences were seen in the SDI grade and SSA with respect to clinical outcome. The SDI is higher after single-level ACDF and with the diagnosis of CDH. The DH was negligibly different with respect to clinical outcome, diagnosis, and number of operated levels.