Shang-Yih Yan, Chia-Lin Tsai and Dueng-Yuan Hueng
Albert Lee, Shirley McCartney, Cole Burbidge, Ahmed M. Raslan and Kim J. Burchiel
Vascular compression of the trigeminal nerve is the most common factor associated with the etiology of trigeminal neuralgia (TN). Microvascular decompression (MVD) has proven to be the most successful and durable surgical approach for this disorder. However, not all patients with TN manifest unequivocal neurovascular compression (NVC). Furthermore, over time patients with an initially successful MVD manifest a relentless rate of TN recurrence.
The authors performed a retrospective review of cases of TN Type 1 (TN1) or Type 2 (TN2) involving patients 18 years or older who underwent evaluation (and surgery when indicated) at Oregon Health & Science University between July 2006 and February 2013. Surgical and imaging findings were correlated.
The review identified a total of 257 patients with TN (219 with TN1 and 38 with TN2) who underwent high-resolution MRI and MR angiography with 3D reconstruction of combined images using OsiriX. Imaging data revealed that the occurrence of TN1 and TN2 without NVC was 28.8% and 18.4%, respectively. A subgroup of 184 patients underwent surgical exploration. Imaging findings were highly correlated with surgical findings, with a sensitivity of 96% for TN1 and TN2 and a specificity of 90% for TN1 and 66% for TN2.
Magnetic resonance imaging detects NVC with a high degree of sensitivity. However, despite a diagnosis of TN1 or TN2, a significant number of patients have no NVC. Trigeminal neuralgia clearly occurs and recurs in the absence of NVC.
Diaa Bahgat, Dibyendu K. Ray, Ahmed M. Raslan, Shirley McCartney and Kim J. Burchiel
Trigeminal neuralgia (TN) is a form of facial pain that can be debilitating if left untreated. It typically affects elderly adults and is thought to be related to neurovascular compression. It is uncommon in people younger than 30 years of age, with only 1% of cases reportedly occurring in those younger than 20 years of age. The most common cause of compression in young adults is thought to be venous nerve compression either alone or in association with arterial nerve compression. The objective of this study was to review data in cases of TN in which patients were 25 years of age or younger and to identify TN disease characteristics, demographics, clinical features, operative findings, and outcome.
The authors retrospectively reviewed the clinical records, surgical treatment, and long-term outcome in patients 25 years of age or younger with TN who underwent surgery performed by the senior author (K.J.B.) at Oregon Health & Science University between 1995 and 2008.
Seven patients (2 males and 5 females) met the inclusion criteria. The average age at symptom onset was 19.6 ± 3.4 years (± SD) and the average age at surgery was 22.9 ± 1.7 years. Six patients had right-sided symptoms and 1 had left-sided symptoms. Pain distribution was the V2 in 3 cases, V2–3 in 3 cases, and V3 in 1 case, with no cases of V1 affliction. A total of 11 procedures were performed in 7 patients, and 4 patients underwent a second procedure. Surgery and imaging revealed venous compression in all cases. The average follow-up period was 35.5 ± 39.9 months (median 12 months). Three patients reported a good outcome (no pain with or without medications) and 4 reported a poor outcome (either no pain relief or mild pain relief after surgery).
Trigeminal neuralgia is uncommon in young adults. Patients tend to present with symptoms similar to those in adults: long periods of pain and venous compression, but outcome unfortunately is not as good as that reported in the older population.
Ahmed M. Raslan and Kim J. Burchiel
Ahmed M. Raslan, Reynaldo DeJesus, Caglar Berk, Andrew Zacest, Jim C. Anderson and Kim J. Burchiel
Hemifacial spasm is a clinical syndrome caused by vascular compression of the facial nerve in the cerebellopontine angle, which can be relieved by surgical intervention. Advances in medical imaging technology allow for direct visualization of the offending blood vessels in hemifacial spasm and similar conditions (such as trigeminal neuralgia). The utility of high resolution 3D MR angiography and 3D spoiled-gradient recalled (SPGR) imaging sequences for surgical decision-making in hemifacial spasm, as measured by sensitivity, specificity, and positive and negative predictive values, has not been previously determined.
A retrospective review was undertaken of 23 patients with hemifacial spasm who underwent operations between January 2001 and December 2006 at Oregon Health & Science University. All patients underwent preoperative high-resolution 3D MR angiography and 3D SPGR imaging. The sensitivity of the SPGR imaging/MR angiography interpretation of neurovascular compression (NVC) by both a neurosurgeon and 2 neuroradiologists was determined in relation to the presence of actual NVC during surgery.
All patients were found to have NVC at surgery. After review by a neurosurgeon and 2 neuroradiologists, imaging data from 19 of the 23 patients were evaluated. The neurosurgeon's interpretation had a sensitivity of 79% and a positive predictive value (PPV) of 100%. The first neuroradiologist's interpretation had a sensitivity of 21% with a PPV of 100%. Further interpretation by a blinded second neuroradiologist with expertise in MR imaging of hemifacial spasm and trigeminal neuralgia was conducted, and sensitivity was 59% and PPV was 100%. Specificity was not determined because there were no true negative cases. The negative predictive value was 0% for both the neurosurgeon's and neuroradiologists' evaluations.
Although high-resolution 3D MR angiography and 3D SPGR imaging was helpful in providing information about the anatomical relationship of cranial nerve VII and surrounding blood vessels, the authors determined that in the case of hemifacial spasm these types of imaging did not influence preoperative surgical decisionmaking.
Katherine G. Holste, Frances A. Hardaway, Ahmed M. Raslan and Kim J. Burchiel
Nervus intermedius neuralgia (NIN) or geniculate neuralgia is a rare facial pain condition consisting of sharp, lancinating pain deep in the ear and can occur alongside trigeminal neuralgia (TN). Studies on the clinical presentation, intraoperative findings, and ultimately postoperative outcomes are extremely limited. The aim of this study was to examine the clinical presentation and surgical findings, and determine pain-free survival after sectioning of the nervus intermedius (NI).
The authors conducted a retrospective chart review and survey of patients who were diagnosed with NIN at one institution and who underwent neurosurgical interventions. Pain-free survival was determined through chart review and phone interviews using a modified facial pain and quality of life questionnaire and represented as Kaplan-Meier curves.
The authors found 15 patients with NIN who underwent microsurgical intervention performed by two surgeons from 2002 to 2016 at a single institution. Fourteen of these patients underwent sectioning of the NI, and 8 of 14 had concomitant TN. Five patients had visible neurovascular compression (NVC) of the NI by the anterior inferior cerebellar artery in most cases where NVC was found. The most common postoperative complaints were dizziness and vertigo, diplopia, ear fullness, tinnitus, and temporary facial nerve palsy. Thirteen of the 14 patients reportedly experienced pain relief immediately after surgery. The mean length of follow-up was 6.41 years (range 8 months to 14.5 years). Overall recurrence of any pain was 42% (6 of 14), and 4 patients (isolated NIN that received NI sectioning alone) reported their pain was the same or worse than before surgery at longest follow-up. The median pain-free survival was 4.82 years ± 14.85 months. The median pain-controlled survival was 6.22 years ± 15.78 months.
In this retrospective review, sectioning of the NI produced no major complications, such as permanent facial weakness or deafness, and was effective for patients when performed in addition to other procedures. After sectioning of the NI, patients experienced 4.8 years pain free and experienced 6.2 years of less pain than before surgery. Alone, sectioning of the NI was not effective. The pathophysiology of NIN is not entirely understood. It appears that neurovascular compression plays only a minor role in the syndrome and there is a high degree of overlap with TN.
Case report and review of the literature
Konstantin V. Slavin, Randall R. Nixon, Gary M. Nesbit and Kim J. Burchiel
The authors present the case of a patient in whom progressive thoracic myelopathy was caused by the extensive ossification of the arachnoid membrane and associated intramedullary syrinx. Based on their findings and the results of a literature search, they describe a pathological basis of this rare condition, discuss its incidence and symptomatology, and suggest a simple classification of various types of the arachnoid ossification. They also discuss magnetic resonance imaging features of arachnoid ossification and associated spinal cord changes. Emphasis is placed on the particular value of plain computerized tomography, which is highly sensitive for detecting intraspinal calcifications and ossifications, in the diagnostic evaluation of patients whose clinical picture indicates progressive myelopathy.
Case report and review of the literature
Konstantin V. Slavin, Randall R. Nixon, Gary M. Nesbit and Kim J. Burchiel
✓ The authors present the case of progressive thoracic myelopathy caused by the extensive ossification of the arachnoid membrane and associated intramedullary syrinx. Based on their findings and results of the literature search, they describe a pathological basis for this rare condition, discuss its incidence and symptomatology, and suggest a simple classification for various types of the arachnoid ossification. They also discuss the magnetic resonance imaging features of arachnoid ossification and associated spinal cord changes. The particular value of plain computerized tomography, which is highly sensitive in revealing intraspinal calcifications and ossifications, in the diagnostic evaluation of patients with a clinical picture of progressive myelopathy is emphasized.
Lee T. Robertson, Rebecca J. St George, Patricia Carlson-Kuhta, Penelope Hogarth, Kim J. Burchiel and Fay B. Horak
While deep brain stimulation (DBS) has proven to be an effective treatment for many symptoms of Parkinson disease (PD), a deterioration of axial symptoms frequently occurs, particularly for speech and swallowing. These unfavorable effects of DBS may depend on the site of stimulation. The authors made quantitative measures of jaw velocity to compare the relative effectiveness of DBS in the globus pallidus internus (GPi) or the subthalamic nucleus (STN). This was a randomized, double-blind, and longitudinal study, with matched healthy controls.
The peak velocities of self-scaled and externally scaled jaw movements were studied in 27 patients with PD before and after 6 months of bilateral DBS in the GPi or the STN. A mixed-effects model was used to identify differences in jaw velocity before DBS surgery (baseline) while off and on levodopa therapy, and after 6 months of DBS (postoperative) during 4 treatment conditions (off- and on-levodopa states with and without DBS).
Self-scaled jaw velocity was impaired by the DBS procedure in the STN; velocity was significantly decreased across all postoperative conditions compared with either the off- or on-levodopa baseline conditions. In contrast, the postoperative velocity in the GPi group was generally faster than the baseline off-levodopa state. Turning the DBS off and on had no effect on jaw velocity in either group. Unlike baseline, levodopa therapy postoperatively no longer increased jaw velocity in either group, and this lack of effect was not related to postoperative changes in dose. The externally scaled jaw velocity was little affected by PD, but DBS still slightly affected performance, with the STN group significantly slower than the GPi group for most conditions.
The authors' results suggest that either the electrode implant in STN or the subsequent period of continuous STN stimulation negatively affected voluntary jaw velocity, including the loss of the preoperative levodopa-induced improvement. While the GPi group showed some improvement in voluntary jaw velocity postoperatively, their performance during the combination of DBS and levodopa was not different from their best medical management presurgery. The results have implications for DBS target selection, particularly for those patients with oromotor dysfunctions.
Rebecca J. St George, Patricia Carlson-Kuhta, Kim J. Burchiel, Penelope Hogarth, Nicholas Frank and Fay B. Horak
The effect of deep brain stimulation (DBS) for Parkinson disease (PD) on balance is unclear. The goal of this study was to investigate how automatic postural responses (APRs) were affected in patients randomized to either subthalamic nucleus (STN) or globus pallidus internus (GPi) surgery.
The authors tested 24 patients with PD who underwent bilateral DBS, 9 control patients with PD who did not undergo DBS, and 17 age-matched control volunteers. The electrode placement site was randomized and blinded to the patients and to the experimenters. Kinematic, kinetic, and electromyographic recordings of postural responses to backward disequilibrium via forward translations of the standing surface were recorded in the week prior to surgery while the patients were off (OFF) and on (ON) antiparkinsonian medication (levodopa), and then 6 months after surgery in 4 conditions: 1) off medication with DBS switched off (OFF/OFF); 2) off medication with DBS on (DBS); 3) on medication with DBS off (DOPA); and 4) with both medication and DBS on (DBS+DOPA). Stability of the automatic postural response (APR) was measured as the difference between the displacement of the center of pressure and the projected location of the center of body mass.
Patients with PD had worse APR stability than controls. Turning the DBS on at either site improved APR stability compared with the postoperative OFF condition by lengthening the tibialis response, whereas medication did not show an appreciable effect. The STN group had worse APR stability in their best functional state (DBS+DOPA) 6 months after the DBS procedure compared with their best functional state (ON levodopa) before the DBS procedure. In contrast, the GPi group and the PD control group showed no change over 6 months. The APR stability impairment in the STN group was associated with smaller tibialis response amplitudes, but there was no change in response latency or coactivation with gastrocnemius.
Turning the DBS current on improved APR stability for both STN and GPi sites. However, there was a detrimental DBS procedural effect for the STN group, and this effect was greater than the benefit of the stimulating current, making overall APR stability functionally worse after surgery for the STN group.