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Montes J.,Columbia University | McDermott M.P.,University of Rochester | Martens W.B.,University of Rochester | Dunaway S.,Columbia University | And 11 more authors.
Neurology | Year: 2010

Background: In spinal muscular atrophy (SMA), weakness, decreased endurance, and fatigue limit mobility. Scales have been developed to measure function across the wide spectrum of disease severity. However, these scales typically are observer dependent, and scores are based on sums across Likert-scaled items. The Six-Minute Walk Test (6MWT) is an objective, easily administered, and standardized evaluation of functional exercise capacity that has been proven reliable in other neurologic disorders and in children. Methods: To study the performance of the 6MWT in SMA, 18 ambulatory participants were evaluated in a cross-sectional study. Clinical measures were 6MWT, 10-m walk/run, Hammersmith Functional Motor Scale-Expanded (HFMSE), forced vital capacity, and handheld dynamometry. Associations between the 6MWT total distance and other outcomes were analyzed using Spearman correlation coefficients. A paired t test was used to compare the mean distance walked in the first and sixth minutes. Results: The 6MWT was associated with the HFMSE score (r = 0.83, p < 0.0001), 10-m walk/run (r =-0.87, p < 0.0001), and knee flexor strength (r = 0.62, p = 0.01). Gait velocity decreased during successive minutes in nearly all participants. The average first minute distance (57.5 m) was significantly more than the sixth minute distance (48 m) (p = 0.0003). Conclusion: The Six-Minute Walk Test (6MWT) can be safely performed in ambulatory patients with spinal muscular atrophy (SMA), correlates with established outcome measures, and is sensitive to fatigue-related changes. The 6MWT is a promising candidate outcome measure for clinical trials in ambulatory subjects with SMA. Copyright © 2010 by AAN Enterprises, Inc.

Brodeur G.M.,pa | Dale R.C.,The New School
Annals of Neurology | Year: 2016

Neuroblastoma is a childhood cancer derived from cells of neural crest origin. The hallmarks of its enigmatic character include its propensity for spontaneous regression under some circumstances and its association with paraneoplastic opsoclonus, myoclonus, and ataxia. The neurodevelopmental underpinnings of its origins may provide important clues for development of novel therapeutic and preventive agents for this frequently fatal malignancy and for the associated paraneoplastic syndromes. © 2016 American Neurological Association.

Abrams M.,pa
Penn State Environmental Law Review | Year: 2010

Vegetation change is brought about by natural and anthropogenic processes, as well as an interaction of the two. Natural processes that impact vegetation include climate change, ecological disturbances, insect and disease outbreaks, extreme weather events, geologic phenomenon, and plant succession. The magnitude of anthropogenic disturbances in North American forests changed dramatically following European settlement. These included extensive logging and land clearing, often associated with catastrophic fire, followed by the onset of the fire control era in the early 20th century, and the introduction of exotic insects and diseases. In contrast, fire suppression policy during the Smokey Bear era appears to be leading to the demise of many historically dominant trees in the eastern US. Another important indication of humans' role in the ecology of eastern North America is the long-term persistence of disturbance- dependent vegetation types where natural disturbances are not particularly inherent to the system.

Total ankle arthroplasty in the right circumstances cannot only relieve discomfort; but, unlike an ankle arthrodesis, can restore enhanced ambulatory capabilities. Subsequent wound healing issues have the potential to ultimately lead to implant removal, a disaster that can be avoided by as early intervention as possible that will provide sustainable wound closure. Over the past 5 years, 5 patients have presented in a delayed fashion with wound breakdown following total ankle arthroplasty that required a free flap for successful prosthesis salvage. The mean wound size was 78.0 cm2 (range 14-200 cm2). Two gracilis and 2 latissimus dorsi muscle free flaps were chosen as a malleable means not just to cover but to fill these usually large 3-dimensional wounds. A single radial forearm perforator free flap was selected in one case for a superficial wound that required a long vascular pedicle to reach outside the zone of injury. The postoperative course for all was uneventful, with a minimum follow-up of 4 months. Function preservation following total ankle arthroplasty wound breakdown even after an untimely delay in referral can still be maintained using microsurgical tissue transfers. © 2015 Wiley Periodicals, Inc.

Brown J.M.,University of California at San Diego | Barbe M.F.,University of California at San Diego | Albo M.E.,Anatomy and Cell Biology | Lai H.H.,University of Washington | And 2 more authors.
Journal of Neurosurgery: Spine | Year: 2012

Object. Nerve transfers are effective for restoring control to paralyzed somatic muscle groups and, recently, even to denervated detrusor muscle in a canine model. A pilot project was performed in cadavers to examine the feasibility of transferring somatic nerves to vesical branches of the pelvic nerve as a method for potentially restoring innervation to control the detrusor muscle in humans. Methods. Eleven cadavers were dissected bilaterally to expose intercostal, ilioinguinal, and iliohypogastric nerves, along with vesical branches of the pelvic nerve. Ease of access and ability to transfer the former 3 nerves to the pelvic vesical nerves were assessed, as were nerve cross-sectional areas. Results. The pelvic vesical nerves were accessed at the base of the bladder, inferior to the ureter and accompanied by inferior vesical vessels. The T-11 and T-12 intercostal nerves were too short for transfer to the pelvic vesical nerves without grafting. Ilioinguinal and iliohypogastric nerves (L-1 origin) were identified retroperitoneally and, with full dissection, were easily transferred to the pelvic vesical nerves intraabdominally. The mean cross-sectional area of the dominant pelvic vesical branch was 2.60 ± 0.169 mm2; ilioinguinal and iliohypogastric branches at the suggested transection site were 2.38 ± 0.32 mm2 (the means are expressed ± SEM). Conclusions. Use of the ilioinguinal or iliohypogastric nerves for heterotopic transfer to pelvic vesical nerves is surgically feasible, based on anatomical location and cross-sectional areas.

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