✓Although Harvey Cushing is best known for his role in developing surgical treatments for tumors of the central nervous system, he performed diverse neurosurgical procedures throughout his career, both at The Johns Hopkins Hospital (1886–1912) and at the Peter Bent Brigham Hospital (1912–1932). His unique and innovative approach to the treatment of myelomeningoceles associated with hydrocephalus, displayed early in his career, is characteristic of his attempts to circumvent the technical limitations of his time in the management of neurosurgical problems. In this report, the authors discuss the evolution of Cushing's technique in the treatment of myelomeningoceles through two illustrative patient records.
Aaron A. Cohen-Gadol, Brian V. Nahed, Jennifer R. Voorhees, Cormac O. Maher and Dennis D. Spencer
Brian V. Nahed, Aneela Darbar, Robert Doiron, Ali Saad, Caroline D. Robson and Edward R. Smith
✓Choroid plexus cysts are common and typically asymptomatic abnormal folds of the epithelial lining of the choroid plexus. Rarely, these cysts may gradually enlarge and cause outflow obstruction of cerebrospinal fluid. The authors present a case of a large choroid plexus cyst causing acute hydrocephalus in a previously healthy 2-year-old boy. The patient presented with markedly declining mental status, vomiting, and bradycardia over the course of several hours. Computed tomography scans demonstrated enlarged lateral and third ventricles with sulcal effacement, but no obvious mass lesions or hemorrhage. There was no antecedent illness or trauma. A right frontal external ventricular drain was placed in the patient, resulting in decompression of only the right lateral ventricle. Magnetic resonance (MR) imaging demonstrated a lobulated cyst arising from the choroid plexus of the left lateral ventricle and herniating through the foramen of Monro into the third ventricle, occluding both the foramen of Monro and the cerebral aqueduct. The patient underwent an endoscopic fenestration of the cyst, and histological results confirmed that it was a choroid plexus cyst. Postoperative MR imaging showed a marked reduction in the cyst size. The cyst was no longer in the third ventricle, the foramen of Monro and the aqueduct were patent, and the ventricles were decompressed. The patient was discharged home with no deficits. To the authors' knowledge, there are no previous reports of a choroid plexus cyst causing acute hydrocephalus due to herniation into the third ventricle. This case is illustrative because it describes this entity for the first time, and more importantly highlights the need to obtain a diagnosis when a patient presents with acute hydrocephalus without a clear cause.
Brian V. Nahed, Manuel Ferreira Jr., Matthew R. Naunheim, Kristopher T. Kahle, Mark R. Proctor and Edward R. Smith
Clinical and radiographic evidence of subarachnoid hemorrhage (SAH)-related vasospasm is rare in children and has not been reported in infants. In this report the authors present the case of a 22-month-old child who developed clinically symptomatic, radiographically identifiable vasospasm after traumatic SAH. To the authors' knowledge, this is the first report of vasospasm associated with SAH in a child this young. This 22-month-old boy fell and had a dense SAH. He had a history of surgically corrected craniosynostosis and nonsymptomatic ventriculomegaly. The boy was evaluated for occult vascular lesions using imaging; none were found and normal vessel caliber was noted. Ten days later, the child developed left-sided weakness and a right middle cerebral artery infarct was identified. Evaluation disclosed significant intracranial vasospasm. This diagnosis was supported by findings on CT angiography, transcranial Doppler ultrasonography, MR imaging, and conventional angiography. The child was treated using intraarterial verapamil with a good result, as well as with conventional intensive care measures to reduce vasospasm. This report documents the first known case of intracranial vasospasm with stroke after SAH in a patient under the age of 2 years. This finding is important because it demonstrates that the entity of SAH-associated vasospasm can affect the very young, widening the spectrum of ages susceptible to this condition. This case is also important because it demonstrates that even very young children can respond to conventional therapeutic interventions such as intraarterial verapamil. Thus, clinicians need to be alert to the possibility of vasospasm as a potential diagnosis when evaluating young children with SAH.
Wael F. Asaad, Brian P. Walcott, Brian V. Nahed and Christopher S. Ogilvy
Brainstem cavernous malformations (CMs) are complex lesions associated with hemorrhage and neurological deficit. In this review, the authors describe the anatomical nuances relating to the operative techniques for these challenging lesions. The resection of brainstem CMs in properly selected patients has been demonstrated to reduce the risk of rehemorrhage and can be achieved relatively safely in experienced hands.
Brian P. Walcott, Brian V. Nahed, Kristopher T. Kahle, Navid Redjal and Jean-Valery Coumans
Previous methods to determine stroke prevalence, such as nationwide surveys, are labor-intensive endeavors. Recent advances in search engine query analytics have led to a new metric for disease surveillance to evaluate symptomatic phenomenon, such as influenza. The authors hypothesized that the use of search engine query data can determine the prevalence of stroke.
The Google Insights for Search database was accessed to analyze anonymized search engine query data. The authors' search strategy utilized common search queries used when attempting either to identify the signs and symptoms of a stroke or to perform stroke education. The search logic was as follows: (stroke signs + stroke symptoms + mini stroke − heat) from January 1, 2005, to December 31, 2010.
The relative number of searches performed (the interest level) for this search logic was established for all 50 states and the District of Columbia. A Pearson product-moment correlation coefficient was calculated from the statespecific stroke prevalence data previously reported.
Web search engine interest level was available for all 50 states and the District of Columbia over the time period for January 1, 2005–December 31, 2010. The interest level was highest in Alabama and Tennessee (100 and 96, respectively) and lowest in California and Virginia (58 and 53, respectively). The Pearson correlation coefficient (r) was calculated to be 0.47 (p = 0.0005, 2-tailed).
Search engine query data analysis allows for the determination of relative stroke prevalence. Further investigation will reveal the reliability of this metric to determine temporal pattern analysis and prevalence in this and other symptomatic diseases.
Manuel Ferreira Jr., Brian P. Walcott, Brian V. Nahed and Laligam N. Sekhar
Hemifacial spasm (HFS) is caused by arterial or venous compression of cranial nerve VII at its root exit zone. Traditionally, microvascular decompression of the facial nerve has been an effective treatment for posterior inferior and anterior inferior cerebellar artery as well as venous compression. The traditional technique involves Teflon felt or another construct to cushion the offending vessel from the facial nerve, or cautery and division of the offending vein. However, using this technique for severe vertebral artery (VA) compression can be ineffective and fraught with complications. The authors report the use of a new technique of VA pexy to the petrous or clival dura mater in patients with HFS attributed to a severely ectatic and tortuous VA, and detail the results in a series of patients.
Six patients with HFS due to VA compression underwent a retrosigmoid craniotomy, combined with a far-lateral approach in some patients. On identification of the site of VA compression, the vessel was mobilized adequately for the decompression. Great care was taken to avoid kinking the perforating vessels arising from the VA. Two 8-0 nylon sutures were passed through to the wall of the VA and then through the clival or petrous dura, and then tied to alleviate compression on cranial nerve VII.
Patients were followed for at least 1 year postoperatively (mean 2.7 years, range 1–4 years). All 6 patients had complete resolution of their HFS. Facial function was tested postoperatively, and was stable when compared with the preoperative baseline. Two of the 3 patients with preoperative tinnitus had resolution of this symptom after the procedure. Postoperative imaging demonstrated VA decompression of the facial nerve and no evidence of stroke in all patients. One patient suffered from hearing loss, another developed a postoperative transient unilateral vocal cord paralysis, and a third patient developed a pseudomeningocele that resolved with the placement of a lumbar drain.
Hemifacial spasm and other neurovascular syndromes are effectively treated by repositioning the compressing artery. Careful study of the preoperative MR images may identify a select group of patients with HFS due to an ectatic VA. Rather than traditional decompression with only pledget placement, these patients may benefit from a VA pexy to provide an effective, safe, and durable resolution of their symptoms while minimizing surgical complications.
Benjamin D. Schanker, Brian P. Walcott, Brian V. Nahed, Kristopher T. Kahle, Yan Michael Li and Jean-Valery C. E. Coumans
Chiari malformations (Types I–IV) are abnormalities of the posterior fossa that affect the cerebellum, brainstem, and the spinal cord with prevalence rates of 0.1%–0.5%. Case reports of familial aggregation of Chiari malformation, twin studies, cosegregation of Chiari malformation with known genetic conditions, and recent gene and genome-wide association studies provide strong evidence of the genetic underpinnings of familial Chiari malformation. The authors report on a series of 3 family pairs with Chiari malformation Type I: 2 mother-daughter pairs and 1 father-daughter pair. The specific genetic causes of familial Chiari malformation have yet to be fully elucidated. The authors review the literature and discuss several candidate genes. Recent advances in the understanding of the genetic influences and pathogenesis of familial Chiari malformation are expected to improve management of affected patients and monitoring of at-risk family members.
Jean-Valery Coumans, Brian P. Walcott, William E. Butler, Brian V. Nahed and Kristopher T. Kahle
Resolution of syringomyelia is common following hindbrain decompression for Chiari malformation, yet little is known about the kinetics governing this process. The authors sought to establish the volumetric rate of syringomyelia resolution.
A retrospective cohort of patients undergoing hindbrain decompression for a Chiari malformation Type I with preoperative cervical or thoracic syringomyelia was identified. Patients were included in the study if they had at least 3 neuroimaging studies that detailed the entirety of their preoperative syringomyelia over a minimum of 6 months postoperatively. The authors reconstructed the MR images in 3 dimensions and calculated the volume of the syringomyelia. They plotted the syringomyelia volume over time and constructed regression models using the method of least squares. The Akaike information criterion and Bayesian information criterion were used to calculate the relative goodness of fit. The coefficients of determination R 2 (unadjusted and adjusted) were calculated to describe the proportion of variability in each individual data set accounted for by the statistical model.
Two patients were identified as meeting inclusion criteria. Plots of the least-squares best fit were identified as 4.01459e −0.0180804 x and 13.2556e −0.00615859 x. Decay of the syringomyelia followed an exponential model in both patients (R2 = 0.989582 and 0.948864).
Three-dimensional analysis of syringomyelia resolution over time enables the kinetics to be estimated. This technique is yet to be validated in a large cohort. Because syringomyelia is the final common pathway for a number of different pathological processes, it is possible that this exponential only applies to syringomyelia related to treatment of Chiari malformation Type I.
Brian P. Walcott, Brian V. Nahed, Kristopher T. Kahle, Ann-Christine Duhaime, Nutan Sharma and Emad N. Eskandar
Generalized dystonic syndromes may escalate into persistent episodes of generalized dystonia known as status dystonicus that can be life-threatening due to dystonia-induced rhabdomyolysis and/or respiratory compromise. Treatment of these conditions usually entails parenteral infusion of antispasmodic agents and sedatives and occasionally necessitates a medically induced coma for symptom control. The authors report a series of 3 children who presented with medically intractable, life-threatening status dystonicus and were successfully treated with bilateral pallidal deep brain stimulation. Bilateral globus pallidus internus stimulation appears to be effective in the urgent treatment of medically refractory and life-threatening movement disorders.