Cytogenetic and survival analysis in 315 multiple myeloma patients; five with orbital and eight with extramedullary disease.

PURPOSE: To describe cytogenetic abnormalities in five patients with orbital involvement of multiple myeloma (MM) and to determine if high-risk cytogenetic abnormalities were more prevalent in patients with extramedullary disease (EMD). The final goal was to determine predictors of poor survival in the entire cohort. METHODS: Retrospective cohort study of 315 patients diagnosed with multiple myeloma over a 9-year period at a single institution. RESULTS: Males comprised 61% of the 315 patients and the majority (54.3%) were stage II at diagnosis of MM. Follow-up ranged 6 months to 18 years, mean 5.3 ± 3.7 years. Eight patients (2.5%), two of which had orbital involvement, developed EMD and an additional three patients had orbital involvement from adjacent bone disease. No genetic abnormality reached statistical significance between non-EMD and EMD groups. At the time of analysis 127 (41%) non-EMD patients and 6 (75%) EMD patients have died. The median survival time for the EMD group was 2 years, whereas for the non-EMD group it was 9 years (P-value of log rank test = .00015). Stage at time of MM diagnosis and age were associated with worse prognosis. Median survival for patients over 65 years of age was 7 years, compared to 12 years for patients 65 or younger (P-value for the log-ranked test <.001). CONCLUSIONS: Cytogenetic abnormalities are described in five patients with orbital involvement of MM. No genetic abnormality reached statistical significance between non-EMD and EMD groups. EMD status, stage of MM at diagnosis and age are associated with poorer overall survival.Abbreviation: MM: Multiple Myeloma; EMD: Extramedullary disease; FISH: fluorescence in situ hybridization; B2M: beta-2 microglobulin; LDH: lactate dehydrogenase; OS: overall survival.

Visual recovery after unilateral optic nerve sheath fenestration for pseudotumor cerebri syndrome.

PURPOSE: To evaluate the effect of optic nerve sheath fenestration (ONSF) on the rate of visual function improvement in patients with pseudotumor cerebri syndrome (PTCS). METHODS: Retrospective chart review of patients with PTCS who underwent ONSF between 1998 and 2017. Visual function was evaluated by evaluating visual field (VF), mean deviation (MD), retinal nerve fiber layer (RNFL) thickness, papilledema grade, and visual acuity (VA) prior to and after ONSF. RESULTS: Seventeen female patients aged 17 to 36 years underwent unilateral ONSF. Follow-up averaged 40.1 months. VF MD improved steadily in both eyes up to 12 months. Average RNFL thickness improved in the operated eye from 347 ± 166 mm to 92 ± 27 mm (p < .001) and the non-operated eyes from 306 ± 165 mm to 109 ± 46 mm (p < .001). The grade of papilledema improved in the operated eye from 3.3 ± 1.3 to 0.3 ± 0.7 and the non-operated eye from 3.0 ± 1.6 to 0.18 ± 0.4. There was an exponential rate of improvement in papilledema and RNFL thickness, with the greatest improvement occurring within the first 30 days. Average visual acuity remained intact in both eyes before and after surgery. CONCLUSIONS: ONSF in appropriately selected patients leads to rapid improvement in papilledema and a steady recovery in VF.

Long-term Outcome After Acute Peripheral Facial Palsy.

PURPOSE: To analyze long-term outcomes in a large cohort of patients with acute peripheral facial palsy (APFP). METHODS: Hospital-based, cross-sectional study. Data were abstracted from the electronic medical record. Time to recovery was assessed with Kaplan-Meier survival analyses. Binary logistic regression analysis was used to identify factors associated with outcome. RESULTS: Three hundred seventy-two patients with APFP seen at a tertiary hospital between February 2015 and March 2021 were analyzed. Seasonal variation of APFP peaked in the early fall (September 29) and had a peak-to-low ratio of 1.36 (R 2 = 0.329, p < 0.001). Patients who tested positive for Lyme disease (10%) had an earlier peak (July 16) compared with those who were negative (October 15). Eighty-seven percent of patients had complete recovery (averaging 64 ± 61 days). Patients, with higher House-Brackmann (H-B) grades at presentation took longer to recover, were more likely to have aberrant regeneration and had lower final rates of recovery compared with those with lower H-B grades (χ 2 = 12.03, p < 0.001). Of the patients with residual palsies, 70% had evidence of aberrant regeneration, and nearly half of those had hemifacial spasm. CONCLUSIONS: Most patients with APFP achieve complete recovery within 1 year, including those positive for Lyme. More severe palsy at presentation portends a worse outcome.

Exercise testing protocol using a roller system for manual wheelchair users with spinal cord injury.

OBJECTIVE: Determine the validity and reliability of an exercise testing protocol to evaluate cardiorespiratory measures in manual wheelchair users (MWUs) with spinal cord injury (SCI) using a roller-based (RS) wheelchair system. DESIGN: Repeated measures within-subject design. SETTING: Community-based research laboratory. PARTICIPANTS: Ten adults with SCI requiring the use of a manual wheelchair. INTERVENTIONS: Not applicable. OUTCOME MEASURES: Cardiorespiratory measures (peak oxygen consumption [VO2peak], respiratory exchange ratio [RER], pulmonary ventilation [VE], energy expenditure [EE], heart rate [HR], accumulated kilocalories [AcKcal]) and perceived exertion (RPE) were measured during three separate maximal exercise tests using an arm crank ergometer (ACE) and an RS. RESULTS: At maximal exertion, there were no significant differences in variables between groups, with moderate-to-strong correlations (P < 0.05, r = 0.79-0.90) for VO2, HR, RPE, AcKcal, and rate of EE between RS and ACE trials. Significant moderate-to-strong correlations existed between RS trials for VO2, AcKcal, rate of EE, and peak power output (P < 0.01, r = 0.77-0.97). CONCLUSIONS: VO2peak was highly correlated between ACE and RS trials and between the two RS trials, indicating the RS protocol to be reliable and valid for MWUs with SCI. Differences in perceived exertion and efficiency at submaximal workloads and maximal pulmonary ventilation at peak workloads indicated potential advantages to using the RS.

A motor learning approach to training wheelchair propulsion biomechanics for new manual wheelchair users: A pilot study.

CONTEXT/OBJECTIVE: Developing an evidence-based approach to teaching wheelchair skills and proper propulsion for everyday wheelchair users with a spinal cord injury (SCI) is important to their rehabilitation. The purpose of this project was to pilot test manual wheelchair training based on motor learning and repetition-based approaches for new manual wheelchair users with an SCI. DESIGN: A repeated measures within-subject design was used with participants acting as their own controls. METHODS: Six persons with an SCI requiring the use of a manual wheelchair participated in wheelchair training. The training included nine 90-minute sessions. The primary focus was on wheelchair propulsion biomechanics with a secondary focus on wheelchair skills. OUTCOME MEASURES: During Pretest 1, Pretest 2, and Posttest, wheelchair propulsion biomechanics were measured using the Wheelchair Propulsion Test and a Video Motion Capture system. During Pretest 2 and Posttest, propulsion forces using the WheelMill System and wheelchair skills using the Wheelchair Skills Test were measured. RESULTS: Significant changes in area of the push loop, hand-to-axle relationship, and slope of push forces were found. Changes in propulsion patterns were identified post-training. No significant differences were found in peak and average push forces and wheelchair skills pre- and post-training. CONCLUSIONS: This project identified trends in change related to a repetition-based motor learning approach for propelling a manual wheelchair. The changes found were related to the propulsion patterns used by participants. Despite some challenges associated with implementing interventions for new manual wheelchair users, such as recruitment, the results of this study show that repetition-based training can improve biomechanics and propulsion patterns for new manual wheelchair users.

Incidence of lagophthalmos after aponeurotic ptosis repair.

This study was designed to determine the incidence of lagophthalmos following aponeurotic ptosis repair, and to establish any predictive factors for its development. Data from a prospective, interventional, non-comparative case series was reviewed. Of these 164 eyelids with acquired involutional ptosis, 134 eyelids of 75 patients had both preoperative and postoperative photographs of the eyelids in the primary and gently closed positions, and were therefore analyzed for this study. Lagophthalmos was present in 23/38 (60%) of eyelids on the first postoperative day; in 27/81 (33%) one week after surgery; and persisted in 12/134 (9%) eyelids and 8/75 (11%) patients six to twenty weeks after surgery (mean 11 weeks). The lagophthalmos of these twelve eyelids ranged from 0.5 to 1.5 mm with a mean of 0.6 mm. Both the pre-existing lower scleral show and the final height of the eyelid following surgery were significant predictors of postoperative lagophthalmos. Neither eyelid excursion nor the degree of change in eyelid height from pre- to post-surgery was significantly associated with eyelid closure.

Intraorbital surgery for trigeminal neuralgia.

PURPOSE: First-division trigeminal neuralgia, or tic douloureux refractory to medications, presents problems to the surgeon because of the desirability of preserving corneal sensation. A new operation is described that may provide longer duration of pain relief than conventional supraorbital neurectomy, with preservation of the corneal reflex. METHODS: Four patients underwent resection of the supratrochlear and supraorbital nerves within the orbit accessed from an upper eyelid skin crease incision. RESULTS: Three patients with typical idiopathic trigeminal neuralgia involving branches of the frontal nerve are without pain 22 to 25 months after surgery. The final patient with atypical pain had no improvement after the procedure. Frontal nerve distribution anesthesia is present in all patients. Postoperative ptosis resolved in all patients within 4 months of surgery. CONCLUSIONS: This procedure should be added to the treatment options for patients with first-division trigeminal neuralgia. By avoiding injury to the trigeminal root and ganglion, this surgery carries no risk of facial motor dysfunction, dysthesia, and/or anesthesia in the other trigeminal branches including corneal anesthesia.