Middle Meningeal Artery Aneurysm in a Giant Meningioma.

Nontraumatic perilesional aneurysms are thought to occur because of high-flow demands of feeding arteries and neoangiogenesis of hypervascular lesions, most commonly in vascular malformations, but they can rarely be associated with tumors as well. Most of these perilesional aneurysms are from intracranial vasculature associated with branches off the internal carotid artery. We report the unique case of a middle meningeal artery aneurysm associated with a giant convexity meningioma seen on a preoperative angiogram.

High Long-Term Mortality Rate in Elderly Patients with Mild Traumatic Brain Injury and Subdural Hematoma due to Ground-Level Fall: Neurosurgery's Hip Fracture?

BACKGROUND: Moderate-to-severe traumatic brain injury (TBI) is a major source of morbidity and mortality in elderly patients. Little is known about long-term mortality in elderly patients following mild, nonfatal TBI and how the injury mechanism predicts survival. This study aimed to compare long-term mortality in elderly patients with mild TBI and traumatic subdural hematoma (tSDH) due to ground-level fall (GLF) versus those with TBI and tSDH due to another cause (i.e., non-ground-level fall [nGLF]). METHODS: This retrospective study comprised 288 patients ≥60 years old from a single Level I trauma center with tSDH and Glasgow Coma Scale scores 13-15. RESULTS: Median follow-up after initial TBI presentation was 2.9 years for the GLF group and 2.4 years for the nGLF group. During follow-up, 98 patients died, and median survival for all elderly patients with mild TBI and tSDH was 4.6 years. The GLF group had a higher mortality rate than the nGLF group, with 93 patients in GLF group dying during follow-up compared with 5 in nGLF group (P < 0.0001). The annual death rate for patients in the GLF group was 12.5% per year. For patients 60-69 years old, 39% in GLF group died compared with 4% in nGLF group during follow-up (P = 0.0002). Likewise, for patients 70-79 years old, 29% in GLF group died compared with 7% in nGLF group (P = 0.021). Finally, 56% of patients >80 years old in GLF group compared with 18% in nGLF group (P = 0.11). CONCLUSIONS: Elderly patients with mild TBI and tSDH due to GLF have significantly higher long-term mortality than patients with injuries due to nGLF.

Change in pelvic incidence between the supine and standing positions in patients with bilateral sacroiliac joint vacuum signs.

OBJECTIVE: Pelvic incidence (PI) is a commonly utilized spinopelvic parameter in the evaluation and treatment of patients with spinal deformity and is believed to be a fixed parameter. However, a fixed PI assumes that there is no motion across the sacroiliac (SI) joint, which has been disputed in recent literature. The objective of this study was to determine if patients with SI joint vacuum sign have a change in PI between the supine and standing positions. METHODS: A retrospective chart review identified patients with a standing radiograph, supine radiograph, and CT scan encompassing the SI joints within a 6-month period. Patients were grouped according to their SI joints having either no vacuum sign, unilateral vacuum sign, or bilateral vacuum sign. PI was measured by two independent reviewers. RESULTS: Seventy-three patients were identified with an average age of 66 years and a BMI of 30 kg/m2. Patients with bilateral SI joint vacuum sign (n = 27) had an average absolute change in PI of 7.2° (p < 0.0001) between the standing and supine positions compared to patients with unilateral SI joint vacuum sign (n = 20) who had a change of 5.2° (p = 0.0008), and patients without an SI joint vacuum sign (n = 26) who experienced a change of 4.1° (p = 0.74). ANOVA with post hoc Tukey test showed a statistically significant difference in the change in PI between patients with the bilateral SI joint vacuum sign and those without an SI joint vacuum sign (p = 0.023). The intraclass correlation coefficient between the two reviewers was 0.97 for standing PI and 0.96 for supine PI (p < 0.0001). CONCLUSIONS: Patients with bilateral SI joint vacuum signs had a change in PI between the standing and supine positions, suggesting there may be increasing motion across the SI joint with significant joint degeneration.

Regional improvements in lumbosacropelvic Hounsfield units following teriparatide treatment.

OBJECTIVE: Opportunistic Hounsfield unit (HU) determination from CT imaging has been increasingly used to estimate bone mineral density (BMD) in conjunction with assessments from dual energy x-ray absorptiometry (DXA). The authors sought to compare the effect of teriparatide on HUs across different regions in the pelvis, sacrum, and lumbar spine, as a surrogate measure for the effects of teriparatide on lumbosacropelvic instrumentation. METHODS: A single-institution retrospective review of patients who had been treated with at least 6 months of teriparatide was performed. All patients had at least baseline DXA as well as pre- and post-teriparatide CT imaging. HUs were measured in the pedicle, lamina, and vertebral body of the lumbar spine, in the sciatic notch, and at the S1 and S2 levels at three different points (ilium, sacral body, and sacral ala). RESULTS: Forty patients with an average age of 67 years underwent a mean of 20 months of teriparatide therapy. Mean HUs of the lumbar lamina, pedicles, and vertebral body were significantly different from each other before teriparatide treatment: 343 ± 114, 219 ± 89.2, and 111 ± 48.1, respectively (p < 0.001). Mean HUs at the S1 level for the ilium, sacral ala, and sacral body were also significantly different from each other: 124 ± 90.1, -10.7 ± 61.9, and 99.1 ± 72.1, respectively (p < 0.001). The mean HUs at the S2 level for the ilium and sacral body were not significantly different from each other, although the mean HU at the sacral ala (-11.9 ± 52.6) was significantly lower than those at the ilium and sacral body (p = 0.003 and 0.006, respectively). HU improvement occurred in most regions following teriparatide treatment. In the lumbar spine, the mean lamina HU increased from 343 to 400 (p < 0.001), the mean pedicle HU increased from 219 to 242 (p = 0.04), and the mean vertebral body HU increased from 111 to 134 (p < 0.001). There were also significant increases in the S1 sacral body (99.1 to 130, p < 0.05), S1 ilium (124 vs 165, p = 0.01), S1 sacral ala (-10.7 vs 3.68, p = 0.04), and S2 sacral body (168 vs 189, p < 0.05). CONCLUSIONS: There was significant regional variation in lumbar and sacropelvic HUs, with most regions significantly increasing following teriparatide treatment. The sacropelvic area had lower HU values than the lumbar spine, more regional variation, and a higher degree of correlation with BMD as measured on DXA. While teriparatide treatment resulted in HUs > 110 in the majority of the lumbosacral spine, the HUs in the sacral ala remained suggestive of severe osteoporosis, which may limit the effectiveness of fixation in this region.

Cranial Base Manifestations of Granulomatosis with Polyangiitis.

OBJECTIVE: Although granulomatosis with polyangiitis (GPA; Wegener's granulomatosis) is classically characterized by systemic disease involving the kidneys and airway, approximately 10% of patients who have it present with isolated central nervous system disease. When involving the skull base, GPA frequently mimics more common pathology, resulting in diagnostic challenges and delay. The primary objective of this study is to characterize the cranial base manifestations of GPA, highlighting aspects most relevant to the skull base surgeon. STUDY DESIGN: Retrospective review. SETTING: Tertiary academic referral center. SUBJECTS AND METHODS: Retrospective analysis of all patients with skull base GPA treated at a tertiary referral center from January 1, 1996, to May 1, 2018. RESULTS: Twenty-nine patients met inclusion criteria. Twenty-one (72%) initially presented with skull base symptomatology as their cardinal manifestation of GPA. Twenty-four (82%) presented with cranial neuropathy at some point in their disease course. The trigeminal nerve was most commonly involved (12 of 24, 50%), followed by the facial (11 of 24, 46%) and optic (8 of 24, 33%) nerves. Eighteen patients reported hearing loss attributed to the GPA disease process, presenting as conductive, sensorineural, or mixed. The most common locations for GPA-derived inflammatory skull base disease on imaging included the cavernous sinus (12 of 29, 41%) and the orbit (7 of 29, 24%). CONCLUSION: Establishing the diagnosis of skull base GPA remains challenging. Cranial neuropathy is diverse in presentation and often mimics more common conditions. Imaging findings are also unpredictable and frequently nonspecific. Careful review of patient history, clinical presentation, serology and biopsy results, and imaging can reveal important clues toward the diagnosis.

Manifestations of Skull Base IgG4-Related Disease: A Multi-Institutional Study.

OBJECTIVE: IgG4-related disease (IgG4-RD) is a recently recognized disease characterized by fibroinflammatory infiltrates rich in IgG4+ plasma cells that can present as isolated tumor-like lesions of the head and neck. The objective of the current study was to describe the cranial base manifestations of IgG4-RD. METHODS: Review of all cases at three tertiary-referral centers since disease description in 2003. RESULTS: Eleven patients were identified at a median age at presentation of 58 years (IQR, 38-65; 55% male). Ten (91%) patients had isolated skull base masses without systemic disease. Cranial neuropathies were commonly observed in the abducens (45%), trigeminal (18%), and facial nerves (18%). Lesions frequently involved the cavernous sinus (55%; 6/11) with extension to the petroclival junction in 50% (3/6). Infiltration of the internal auditory canal was present in 27% (3/11) with one case demonstrating erosion of the bony labyrinth. Preliminary clinical diagnoses commonly included nasopharyngeal cancer, pituitary macroadenoma, cholesteatoma, and meningioma / multiple meningioma syndrome. Local biopsy demonstrated >30 IgG4-positive plasma cells per high-powered field or an IgG4:IgG ratio greater than 40% in all cases. Rapid and durable clinical improvement was seen in 91% following corticosteroid and rituximab therapy. CONCLUSIONS: IgG4-RD nonspecifically presents as a rare cause of the skull base mass. Often presenting without concomitant systemic disease, local diagnostic biopsies are required. Obtaining adequate tissue specimen is complicated by densely fibrotic cranial base lesions that are frequently in close proximity to critical neurovascular structures. Primary medical therapy with corticosteroids and rituximab is effective in most patients. LEVEL OF EVIDENCE: 4 Laryngoscope, 130:2574-2580, 2020.

Skull Base Manifestations of Erdheim-Chester Disease: A Case Series and Systematic Review.

BACKGROUND: Erdheim-Chester disease (ECD) is a rare, non-Langerhans cell histiocytosis. Up to 50% of patients develop central nervous system involvement, and a subset of these patients can present with isolated tumor-like masses. OBJECTIVE: To describe the skull base manifestations of ECD with an emphasis on aspects most pertinent to surgeons who may be referred such patients for primary evaluation. METHODS: Scopus, Web of Science, and PubMed were searched from database inception to May 1, 2018 for articles reporting skull base ECD. An institutional retrospective analysis of all patients treated at the authors' institution since January 1, 1996 was also performed to supplement these data. RESULTS: Of 465 retrieved articles, 18 studies totaling 20 patients met inclusion criteria. Institutional review identified an additional 7 patients. Collectively, the median age at diagnosis was 49 yr (interquartile range, 42-58) with a 4:1 male-to-female ratio. Patients frequently presented with diplopia (48%), headache (30%), dysarthria (22%), and vertigo or imbalance (22%), though trigeminal hypesthesia (11%), facial nerve paresis (7%), hearing loss (7%), and trigeminal neuralgia (7%) were also observed. ECD commonly mimicked meningioma (33%), trigeminal schwannoma (8%), neurosarcoidosis (8%), and skull base lymphoma (8%). CONCLUSION: Discrete skull base lesions frequently mimic more common pathology such as meningioma or cranial nerve schwannomas. Medical therapy comprises the initial treatment for symptomatic skull base disease. Surgical resection is not curative and the utility of surgical intervention is largely limited to biopsy to establish diagnosis and/or surgical debulking to relieve mass effect.

Synchronous Development of Multicentric Malignant Peripheral Nerve Sheath Tumors: Institutional Review.

BACKGROUND: Malignant peripheral nerve sheath tumors (MPNSTs) are rare soft tissue sarcomas, with approximately 50% occurring in patients diagnosed with neurofibromatosis type 1 (NF-1). NF-1 occurs in approximately 1/3000 individuals, and given that the lifetime prevalence of MPNST is estimated at 8%-13%, synchronous development of separate MPNSTs is plausible. We sought to report the incidence of synchronous MPNST in a cohort of pathology-proven cases since 1994. METHODS: Records since 1994 were queried and identified 192 patients with pathology-proven MPNST. Medical records of these patients were reviewed to search for patients with synchronous MPNSTs. RESULTS: Retrospective review of 192 patients treated for MPNST at our institution (including 71 patients with NF-1) revealed only 1 patient with synchronous MPNSTs. A 48-year-old woman with NF-1 presented with progressive right upper and lower extremity pain and radicular symptoms. Biopsies of right sciatic and median nerve lesions revealed high-grade MPNST, and she underwent radiotherapy and complete resection of both masses. Due to initial nonspecific biopsy results and patient preference, treatment of the median nerve lesion was delayed by 8 months. She did not have recurrence of her disease at the 18-month follow-up. CONCLUSIONS: Synchronous development of MPNST is unusual, with an incidence of 1.4% in our cohort of NF-1 patients with MPNSTs. Given the reported incidence of synchronous MPNST, the rate of malignant transformation in NF-1 may be overestimated. However, heightened suspicion for malignant transformation should continue in patients harboring a diagnosis of MPNST.

Network oscillations modulate interictal epileptiform spike rate during human memory.

Eleven patients being evaluated with intracranial electroencephalography for medically resistant temporal lobe epilepsy participated in a visual recognition memory task. Interictal epileptiform spikes were manually marked and their rate of occurrence compared between baseline and three 2 s periods spanning a 6 s viewing period. During successful, but not unsuccessful, encoding of the images there was a significant reduction in interictal epileptiform spike rate in the amygdala, hippocampus, and temporal cortex. During the earliest encoding period (0-2000 ms after image presentation) in these trials there was a widespread decrease in the power of theta, alpha and beta band local field potential oscillations that coincided with emergent focal gamma frequency activity. Interictal epileptiform spike rate correlated with spectral band power changes and broadband (4-150 Hz) desynchronization, which predicted significant reduction in interictal epileptiform spike rate. Spike-triggered averaging of the field potential power spectrum detected a burst of low frequency synchronization 200 ms before the interictal epileptiform spikes that arose during this period of encoding. We conclude that interictal epileptiform spikes are modulated by the patterns of network oscillatory activity that accompany human memory offering a new mechanistic insight into the interplay of cognitive processing, local field potential dynamics and interictal epileptiform spike generation.