✓ The authors describe a case of nontraumatic atlantoaxial dislocation secondary to acute rheumatic fever, in which there appeared to be fibrous fusion between the axis and the atlas in the subluxed position. The dislocation was reduced by means of combined traction and steroid therapy. Fibrous fusion occurred in the realigned position after immobilization.
John T. Lucas, Gordon D. Hungerford and Phanor L. Perot Jr.
John T. Lucas, Thomas B. Ducker and Phanor L. Perot Jr.
✓ The authors report the results of a questionnaire regarding the use of hypertonic saline for the control of pain. Of 2105 patients so treated, nearly 11% had an adverse temporary symptom or sign, reported as an untoward reaction; slightly over 1% suffered a significant morbidity, of which paraplegia or quadriplegia was by far the most common, and two patients died (0.1%).
Ann Liu, Elizabeth N. Kuhn, John T. Lucas Jr., Adrian W. Laxton, Stephen B. Tatter and Michael D. Chan
Neurofibromatosis Type 2 (NF2) is a rare autosomal dominant disorder predisposing patients to meningiomatosis. The role of stereotactic radiosurgery (SRS) is poorly defined in NF2, and although the procedure has excellent control rates in the non-NF2 population, its utility has been questioned because radiation has been hypothesized to predispose patients to malignant transformation of benign tumors. To the authors' knowledge, this is the first study to examine the use of SRS specifically for meningiomas in patients with NF2.
The authors searched a tumor registry for all patients with NF2 who had undergone Gamma Knife radiosurgery (GKRS) for meningioma in the period from January 1, 1999, to September 19, 2013, at a single tertiary care cancer center. Medical records were retrospectively reviewed for patient and tumor characteristics and outcomes.
Among the 12 patients who met the search criteria, 125 meningiomas were identified, 87 (70%) of which were symptomatic or progressive and thus treated with GKRS. The median age at the first GKRS was 31 years (interquartile range [IQR] 27–37 years). Five patients (42%) had multiple treatments with a median of 27 months (IQR 14–50 months) until the subsequent GKRS. The median follow-up in surviving patients was 43 months (IQR 34–110 months). The 5-year local tumor control and distant treatment failure rates were 92% and 77%, respectively. Toxicities occurred in 25% of the GKRS treatments, although the majority were Grade 1 or 2. At the last follow-up, 4 patients (33%) had died a neurological death at a median age of 39 years (IQR 37–46 years), and their cases accounted for 45% of all tumors, 55% of all treated tumors, and 58% of all GKRSs. Univariate analysis revealed several predictive variables for distant failure, including male sex (HR 0.28, 95% CI 0.086–0.92, p = 0.036), age at distant failure (HR 0.92, 95% CI 0.90–0.95, p < 0.0001), and prior number of GKRS treatments (HR 1.2, 95% CI 1.1–1.4, p = 0.0049). Local failure, maximum size of the treated tumor, delivered tumor margin dose, and WHO grade were not significant. On multivariate analysis, age at distant failure (HR 0.91, 95% CI 0.88–0.95, p < 0.0001) and prior number of GKRSs (HR 1.3, 95% CI 1.1–1.5, p = 0.004) remained significant. No malignant transformation events among treated tumors were observed.
Radiosurgery represents a feasible modality with minimal toxicity for NF2-associated meningiomas. Increasing patient age was associated with a decreased rate of distant failure, whereas an increasing number of prior GKRS treatments predicted distant failure. Further studies are necessary to determine the long-term patterns of treatment failure in these patients.
Alois A. Obwegeser, Ryan J. Uitti, John A. Lucas, Robert J. Witte, Margaret F. Turk and Robert E. Wharen Jr.
Object. The authors studied neuropsychological performance following microelectrode-guided posteroventral pallidotomy in patients with Parkinson's disease (PD) and evaluated correlations with presurgical and surgical factors.
Methods. Neuropsychological changes 3 months (43 patients) and 12 months (27 patients) after microelectrode-guided pallidotomy for PD are reported in a series of 44 consecutive patients with the disease, who improved neurologically, as measured by the Unified Parkinson's Disease Rating Scale (UPDRS) in both the “off” (p < 0.001) and best “on” (p < 0.001) states.
Findings of the vocabulary subtest of the Wechsler Adult Intelligence Scale—Revised (p < 0.01), Letter Fluency (p < 0.001), Verbal Fluency for semantic categories (p < 0.001), and the Wisconsin Card Sorting Test (p < 0.01) showed a significant decline in neuropsychological performance in patients 3 months after undergoing left-sided pallidotomy. Impairment in the language domain (semantic fluency) persisted at the 12-month follow-up examination (p < 0.01). Visual memory improved after right-sided pallidotomies (p < 0.01 after 3 months), with a nonsignificant trend toward persistent improvement 1 year postsurgery (p < 0.02 after 12 months). Preoperative semantic fluency was influenced by patient age (p < 0.001) and by the width of the third ventricle (p < 0.05), as measured by magnetic resonance imaging.
A regression model revealed that semantic fluency 3 months postoperatively was significantly affected by the baseline score (p < 0.001), side of surgery (p < 0.001), handedness (p < 0.01), and patient age (p < 0.05). However, postoperative lesion volume, lesion location, number of tracks, number of lesions, distance from anatomical landmarks, or UPDRS score did not significantly contribute to neuropsychological outcome.
Conclusions. Neuropsychological changes in a cohort of patients with PD who underwent pallidotomy and experienced excellent clinical benefits and minimum postoperative complications, emphasize the importance of neuropsychological examinations and further investigation of predictive factors.
Christopher S. Graffeo, Avital Perry, Lucas P. Carlstrom, Fredric B. Meyer, John L. D. Atkinson, Dana Erickson, Todd B. Nippoldt, William F. Young Jr., Bruce E. Pollock and Jamie J. Van Gompel
Nelson-Salassa syndrome (NSS) is a rare consequence of bilateral adrenalectomy (ADX) for refractory hypercortisolism due to Cushing disease (CD). Although classically defined by rapid growth of a large, invasive, adrenocorticotropin hormone (ACTH)–secreting pituitary tumor after bilateral ADX that causes cutaneous hyperpigmentation, visual disturbance, and high levels of ACTH, clinical experience suggests more variability.
The authors conducted a retrospective chart review of all patients 18 years and older with a history of bilateral ADX for CD, adequate pituitary MRI, and at least 2 years of clinical follow-up. Statistical tests included Student's t-test, chi-square test, Fisher's exact test, multivariate analysis, and derived receiver operating characteristic curves.
Between 1956 and 2015, 302 patients underwent bilateral ADX for the treatment of hypercortisolism caused by CD; 88 had requisite imaging and follow-up (mean 16 years). Forty-seven patients (53%) had radiographic progression of pituitary disease and were diagnosed with NSS. Compared with patients who did not experience progression, those who developed NSS were significantly younger at the time of CD diagnosis (33 vs 44 years, p = 0.007) and at the time of bilateral ADX (35 vs 49 years, p = 0.007), had larger tumors at the time of CD diagnosis (6 mm vs 1 mm, p = 0.03), and were more likely to have undergone external-beam radiation therapy (EBRT, 43% vs 12%, p = 0.005). Among NSS patients, the mean tumor growth was 7 mm/yr (SE 6 mm/yr); the median tumor growth was 3 mm/yr. Prevalence of pathognomonic symptoms was low; the classic triad occurred in 9%, while hyperpigmentation without visual field deficit was observed in 23%, and 68% remained asymptomatic despite radiographic disease progression. NSS required treatment in 14 patients (30%).
NSS is a prevalent sequela of CD after bilateral ADX and affects more than 50% of patients. However, although radiological evidence of NSS is common, it is most often clinically indolent, with only a small minority of patients developing the more aggressive disease phenotype characterized by clinically meaningful symptoms and indications for treatment. Young age at the time of CD diagnosis or treatment with bilateral ADX, large tumor size at CD diagnosis, and EBRT are associated with progression to NSS and may be markers of aggressiveness.
Corbin A. Helis, Emory McTyre, Michael T. Munley, J. Daniel Bourland, John T. Lucas Jr., Christina K. Cramer, Stephen B. Tatter, Adrian W. Laxton and Michael D. Chan
A small subset of patients with trigeminal neuralgia (TN) will experience bilateral symptoms. Treatment in these patients is controversial because the population is heterogeneous and patients may have nonvascular etiologies of their pain. This study reports treatment outcomes in the largest cohort of patients with bilateral TN who have undergone Gamma Knife radiosurgery (GKRS) to date.
A retrospective chart review identified 51 individual nerves in 34 patients with bilateral TN who were treated with GKRS at the authors’ institution between 2001 and 2015, with 12 nerves in 11 patients undergoing repeat GKRS for recurrent or persistent symptoms. Long-term follow-up was obtained by telephone interview. Pain outcomes were measured using the Barrow Neurological Institute (BNI) pain scale, with BNI IIIb or better considered a successful treatment.
There was sufficient follow-up to determine treatment outcomes for 48 individual nerves in 33 patients. Of these nerves, 42 (88%) achieved at least BNI IIIb pain relief. The median duration of pain relief was 1.9 years, and 1-, 3-, and 5-year pain relief rates were 64%, 44%, and 44%, respectively. No patients experienced bothersome facial numbness, and 1 case of anesthesia dolorosa and 2 cases of corneal dryness were reported. Patients with a history of definite or possible multiple sclerosis were significantly more likely to experience BNI IV–V relapse. There was no statistically significant difference in treatment outcomes between patients in this series versus a large cohort of patients with unilateral TN treated at the authors’ institution. There was sufficient follow-up to determine treatment outcomes for 11 individual nerves in 10 patients treated with repeat GKRS. Ten nerves (91%) improved to at least BNI IIIb after treatment. The median duration of pain relief was 2.8 years, with 1-, 3-, and 5-year rates of pain relief of 79%, 53%, and 53%, respectively. There was no statistically significant difference in outcomes between initial and repeat GKRS. One case of bothersome facial numbness and 1 case of corneal dryness were reported, with no patients developing anesthesia dolorosa with retreatment.
GKRS is a safe, well-tolerated treatment for patients with medically refractory bilateral TN. Efficacy of treatment appears similar to that in patients with unilateral TN. GKRS can be safely repeated in this population if necessary.