Browse

You are looking at 41 - 50 of 38,531 items for

  • Refine by Access: all x
Clear All
Open access

Anna Borne, Marcela Perrone-Bertolotti, Isabelle Jambaqué, Clémence Castaignède, Georg Dorfmüller, Sarah Ferrand-Sorbets, Monica Baciu, and Christine Bulteau

BACKGROUND

Rasmussen encephalitis is a rare chronic neurological pathology frequently treated with functional hemispherectomy (or hemispherotomy). This surgical procedure frees patients of their severe epilepsy associated with the disease but may induce cognitive disorders and notably language alterations after disconnection of the left hemisphere.

OBSERVATIONS

The authors describe longitudinally 3 cases of female patients with Rasmussen encephalitis who underwent left hemispherotomy in childhood and benefited from a favorable cognitive outcome. In the first patient, the hemispherotomy occurred at a young age, and the recovery of language and cognitive abilities was rapid and efficient. The second patient benefited from the surgery later in childhood. In addition, she presented a reorganization of language and memory functions that seem to have been at the expense of nonverbal ones. The third patient was a teenager during surgery. She benefited from a more partial cognitive recovery with persistent disorders several years after the surgery.

LESSONS

Recovery of cognitive functions, including language, occurs after left hemispherotomy, even when performed late in childhood. Therefore, the surgery should be considered as early as possible to promote intercognitive reorganization.

Open access

Anouk E. Magara, Marc N. Gallay, David Moser, and Daniel Jeanmonod

BACKGROUND

The authors reported the case of a 66-year-old male patient with a 14-year history of right-sided severe episodic and therapy-resistant cluster headache (CH) who underwent bilateral central lateral thalamotomy (CLT) using incisionless transcranial magnetic resonance imaging–guided focused ultrasound (MRgFUS).

OBSERVATIONS

The patient experienced a single cluster headache attack 5 weeks after the procedure. There were no more pain attacks over the next 6 years of follow-up.

LESSONS

This treatment success may indicate a common pathophysiology for CH and neurogenic (neuropathic) pain, which has been treated with CLT for more than 30 years. Further experience is needed to assess the reproducibility of this case.

Open access

John P. Andrews, Thomas A. Wozny, John K. Yue, and Doris D. Wang

BACKGROUND

Epilepsy-associated psychoses are poorly understood, and management is focused on treating epilepsy. Chronic, interictal psychosis that persists despite seizure control is typically treated with antipsychotics. Whether resection of a mesial temporal lobe lesion may improve interictal psychotic symptoms that persist despite seizure control remains unknown.

OBSERVATIONS

In a 52-year-old man with well-controlled epilepsy and persistent comorbid psychosis, brain magnetic resonance imaging (MRI) revealed an infiltrative, intraaxial, T2 fluid-attenuated inversion recovery intense mass of the left amygdala. The patient received an amygdalectomy for oncological diagnosis and surgical treatment of a presumed low-grade glioma. Pathology was ganglioglioma, World Health Organization grade I. Postoperatively, the patient reported immediate resolution of auditory hallucinations. Patient has remained seizure-free on 2 antiepileptic drugs and no antipsychotic pharmacotherapy and reported lasting improvement in his psychotic symptoms.

LESSONS

This report discusses improvement of psychosis symptoms after resection of an amygdalar glioma, independent of seizure outcome. This case supports a role of the amygdala in psychopathology and suggests that low-grade gliomas of the limbic system may represent, at minimum, partially reversible etiology of psychotic symptoms.

Open access

Patrick W. Hullett, Nazineen Kandahari, Tina T. Shih, Jonathan K. Kleen, Robert C. Knowlton, Vikram R. Rao, and Edward F. Chang

BACKGROUND

In classic speech network models, the primary auditory cortex is the source of auditory input to Wernicke’s area in the posterior superior temporal gyrus (pSTG). Because resection of the primary auditory cortex in the dominant hemisphere removes inputs to the pSTG, there is a risk of speech impairment. However, recent research has shown the existence of other, nonprimary auditory cortex inputs to the pSTG, potentially reducing the risk of primary auditory cortex resection in the dominant hemisphere.

OBSERVATIONS

Here, the authors present a clinical case of a woman with severe medically refractory epilepsy with a lesional epileptic focus in the left (dominant) Heschl’s gyrus. Analysis of neural responses to speech stimuli was consistent with primary auditory cortex localization to Heschl’s gyrus. Although the primary auditory cortex was within the proposed resection margins, she underwent lesionectomy with total resection of Heschl’s gyrus. Postoperatively, she had no speech deficits and her seizures were fully controlled.

LESSONS

While resection of the dominant hemisphere Heschl’s gyrus/primary auditory cortex warrants caution, this case illustrates the ability to resect the primary auditory cortex without speech impairment and supports recent models of multiple parallel inputs to the pSTG.

Open access

Jaime R. Guerrero, Khaled M. Taghlabi, Sara A. Meyer, Lokeshwar S. Bhenderu, Saeed S. Sadrameli, Clive I. Shkedy, Amir H. Faraji, and Robert C. Rostomily

BACKGROUND

Metastatic cancer may involve the central and peripheral nervous system, usually in the late stages of disease. At this point, most patients have been diagnosed and treated for widespread systemic disease. Rarely is the involvement of the peripheral nervous system the presenting manifestation of malignancy. One reason for this is a proposed “blood-nerve barrier” that renders the nerve sheath a relatively privileged site for metastases.

OBSERVATIONS

The authors presented a novel case of metastatic melanoma presenting as intractable leg pain and numbness. Further workup revealed concurrent disease in the brain and breast, prompting urgent treatment with radiation and targeted immunotherapy.

LESSONS

This case highlights the rare presentation of metastatic melanoma as a mononeuropathy. Although neurological complications of metastases tend to occur in later stages of disease after initial diagnosis and treatment, one must remember to consider malignancy in the initial differential diagnosis of mononeuropathy.

Restricted access

Matthew Muir, Ron Gadot, Sarah Prinsloo, Hayley Michener, Jeffrey Traylor, Prazwal Athukuri, Sudhakar Tummala, Vinodh A. Kumar, and Sujit S. Prabhu

OBJECTIVE

Robust preoperative imaging can improve the extent of resection in patients with brain tumors while minimizing postoperative neurological morbidity. Both structural and functional imaging techniques can provide helpful preoperative information. A recent study found that transcranial magnetic stimulation (TMS) tractography has significant predictive value for permanent deficits. The present study directly compares the predictive value of TMS tractography and task-based functional MRI (fMRI) tractography in the same cohort of glioma patients.

METHODS

Clinical outcome data were collected from charts of patients with motor eloquent glioma and preoperative fMRI and TMS studies. The primary outcome was a new or worsened motor deficit present at the 3-month postoperative follow-up, which was termed a "permanent deficit." Postoperative MR images were overlaid onto preoperative plans to determine which imaging features were resected. Multiple fractional anisotropic thresholds (FATs) were screened for both TMS and fMRI tractography. The predictive value of the various thresholds was modeled using receiver operating characteristic curve analysis.

RESULTS

Forty patients were included in this study. Six patients (15%) sustained permanent postoperative motor deficits. A significantly greater predictive value was found for TMS tractography than for fMRI tractography regardless of the FAT. Despite 35% of patients showing clinically relevant neuroplasticity captured by TMS, only 2.5% of patients showed a blood oxygen level–dependent signal displaced from the precentral gyrus. Comparing the best-performing FAT for both modalities, TMS seeded tractography showed superior predictive value across all metrics: sensitivity, specificity, positive predictive value, and negative predictive value.

CONCLUSIONS

The results from this study indicate that the prediction of permanent deficits with TMS tractography is superior to that with fMRI tractography, possibly because TMS tractography captures clinically relevant neuroplasticity. However, future large-scale prospective studies are needed to fully illuminate the proper role of each modality in comprehensive presurgical workups for patients with motor-eloquent tumors.

Restricted access

Gabriella Pendola, George W. Koutsouras, Joseph Piatt, Bruce A. Kaufman, Carolina Sandoval-Garcia, and Annie I. Drapeau

OBJECTIVE

Quality improvement (QI) is a methodology used to implement sustainable, meaningful change to improve patient outcomes. Given the complex pathologies observed in pediatric neurosurgery, QI projects could potentially improve patient care. Overall, there is a need to characterize the degree of QI opportunities, training, and initiatives within the field of pediatric neurosurgery. Herein the authors aimed to define the current QI landscape in pediatric neurosurgery.

METHODS

A cross-sectional survey was sent to all members of the American Association of Neurological Surgeons/Congress of Neurological Surgeons Joint Section on Pediatric Neurological Surgery via email. The responses were anonymized. Questions addressed several relatable QI topics including 1) training and participation in QI; 2) QI infrastructure; 3) QI program incentives; and 4) general opinions on the National Surgical Quality Improvement Program (NSQIP) database, various QI topics, and QI productivity.

RESULTS

Responses were received from 129 participants (20% response rate). Most respondents practiced in an academic setting (59.8%) and at a free-standing pediatric hospital (65.4%). Participation in QI projects was high (81.7%), but only 23.8% of respondents had formal QI training. Only 36.5% of respondents had institutional requirements for QI work; the majority of those were only required to participate as a project team member. Nearly half of the respondents did not receive incentives or institutional support for QI. The majority agreed ("strongly" and "somewhat") that a QI course would be beneficial (75.5%), that QI projects should be considered for publication in neurosurgery journals (88.1%), and that there is a need for national quality metrics (81.4%). Over 88% have an interest in seeing QI project presentations at the annual Pediatric Joint Section meeting. Only 26.3% believed that the NSQIP was a useful QI guide. Respondents suggested further study of the following QI topics: overall rates of infection and their prevention, hydrocephalus, standardized treatment algorithms for common disorders, team communication, pediatric neurosurgery–specific database, access to care, and interprofessional education.

CONCLUSIONS

Areas of opportunity include specialty-specific QI education, tactics for obtaining support to build the QI infrastructure, increased visibility of QI work within pediatric neurosurgery, and a review of available registries to provide readily available data relevant to this specialty.

Restricted access

Matthew E. Pontell, Aaron M. Yengo-Kahn, Emily Taylor, Morgan Kane, J Michael Newton, Kelly A. Bennett, John C. Wellons III, and Stephane A. Braun

OBJECTIVE

The objective of this study was to determine the effects of in utero bipedicle flaps on maternal-fetal morbidity/mortality, the need for CSF diversion, and long-term functional outcomes.

METHODS

Eighty-six patients who underwent fetal myelomeningocele repair from 2011 to 2021 at a single institution were reviewed. Primary outcomes included intrauterine fetal demise, postnatal death, postnatal myelomeningocele repair dehiscence, and CSF diversion by final follow-up.

RESULTS

The cohorts were no different with regard to race, ethnicity, maternal age at fetal surgery, body mass index, gravidity, parity, gestational age at fetal surgery, estimated fetal weight at fetal surgery, or fetal lesion level. Of the 86 patients, 64 underwent primary linear repair and 22 underwent bipedicle flap repair. There were no significant differences in rates of intrauterine fetal demise, postnatal mortality, midline repair site dehiscence, or the need for CSF diversion by final follow-up. Operative times were longer (32.5 vs 18.7 minutes, p < 0.001) and gestational age at delivery was lower (232 vs 241 days, p = 0.01) in the bipedicle flap cohort, but long-term functional outcomes were not different.

CONCLUSIONS

Analysis of the total cohort affirms the long-term benefits of fetal myelomeningocele repair. In utero bipedicle flaps are safe and can be used for high-tension lesions without increasing perioperative risks to the mother or fetus. In utero flaps preserve the long-term benefits seen with primary linear repair and may expand inclusion criteria for fetal repair, providing life-changing care for more patients.

Restricted access

John W. Gilbert, Brydon Christensen, and Sherri Matheny

Restricted access

Douglas L. Brockmeyer, Samuel H. Cheshier, Jeff Stevens, Julio C. Facelli, Kerry Rowe, John D. Heiss, Anthony Musolf, David H. Viskochil, Kristina L. Allen-Brady, and Lisa A. Cannon-Albright

OBJECTIVE

Inherited variants predisposing patients to type 1 or 1.5 Chiari malformation (CM) have been hypothesized but have proven difficult to confirm. The authors used a unique high-risk pedigree population resource and approach to identify rare candidate variants that likely predispose individuals to CM and protein structure prediction tools to identify pathogenicity mechanisms.

METHODS

By using the Utah Population Database, the authors identified pedigrees with significantly increased numbers of members with CM diagnosis. From a separate DNA biorepository of 451 samples from CM patients and families, 32 CM patients belonging to 1 or more of 24 high-risk Chiari pedigrees were identified. Two high-risk pedigrees had 3 CM-affected relatives, and 22 pedigrees had 2 CM-affected relatives. To identify rare candidate predisposition gene variants, whole-exome sequence data from these 32 CM patients belonging to 24 CM-affected related pairs from high-risk pedigrees were analyzed. The I-TASSER package for protein structure prediction was used to predict the structures of both the wild-type and mutant proteins found here.

RESULTS

Sequence analysis of the 24 affected relative pairs identified 38 rare candidate Chiari predisposition gene variants that were shared by at least 1 CM-affected pair from a high-risk pedigree. The authors found a candidate variant in HOXC4 that was shared by 2 CM-affected patients in 2 independent pedigrees. All 4 of these CM cases, 2 in each pedigree, exhibited a specific craniocervical bony phenotype defined by a clivoaxial angle less than 125°. The protein structure prediction results suggested that the mutation considered here may reduce the binding affinity of HOXC4 to DNA.

CONCLUSIONS

Analysis of unique and powerful Utah genetic resources allowed identification of 38 strong candidate CM predisposition gene variants. These variants should be pursued in independent populations. One of the candidates, a rare HOXC4 variant, was identified in 2 high-risk CM pedigrees, with this variant possibly predisposing patients to a Chiari phenotype with craniocervical kyphosis.