ABSTRACT
Botulinum toxin (BoNT) is a well established treatment in cerebral palsy. A uniform dose strategy is, however, missing. We reviewed 35 children with spastic cerebral palsy treated with BoNT according to a newly-developed Key-Muscle concept. All patients received at least 4 BoNT treatments. Systemic side effects or secondary non-response were not observed. After a mean follow-up of 303 months, none of these patients needed bone surgery whereas 6 underwent soft tissue procedures. The Key-Muscle concept is a safe and effective treatment in spastic cerebral palsy. It respects the need for long-term therapy during motor development. Contractures and lever arm disease can be avoided.
Subject(s)
Botulinum Toxins, Type A/administration & dosage , Neuromuscular Agents/administration & dosage , Cerebral Palsy/drug therapy , Child , Child, Preschool , Female , Humans , Injections, Intramuscular/methods , Male , Retrospective StudiesABSTRACT
The diagnosis of Marfan syndrome (MFS) is based on evaluating a large number of clinical criteria. We have observed that many persons presenting in specialized centers for "Marfan-like" features do not have MFS, but exhibit a large spectrum of other syndromes. The spectrum of these syndromes and the distribution of "Marfan-like" features remain to be characterized. Thus, we prospectively evaluated 279 consecutive patients with suspected MFS (144 men and 135 women at a mean age of 34+/-13 years) for presence of 27 clinical criteria considered characteristic of MFS. The most frequent reasons to refer individuals for suspected MFS were skeletal features (31%), a family history of MFS, or aortic complications (29%), aortic dissection or aneurysm (19%), and eye manifestations (9%). Using established criteria, we confirmed MFS in 138 individuals (group 1) and diagnosed other connective tissue diseases, both with vascular involvement in 30 (group 2) and without vascular involvement in 39 (group 3), and excluded any distinct disease in 72 individuals (group 4). Clinical manifestations of MFS were present in all four patient groups and there was no single clinical criterion that exhibited positive and negative likelihood ratios that were per se sufficient to confirm or rule out MFS. We conclude that "Marfan-like" features are not exclusively indicative of MFS but also of numerous, alternative inherited diseases with many of them carrying a hitherto poorly defined cardiovascular risk. These alternative diseases require future study to characterize their responses to therapy and long-term prognosis.
Subject(s)
Marfan Syndrome/complications , Marfan Syndrome/diagnosis , Mass Screening , Adult , Cohort Studies , Female , Fibrillins , Humans , Male , Microfilament Proteins/genetics , Mutation/genetics , SyndromeABSTRACT
Throughout the history of medicine, many great physicians have originated or popularized ideas that have resulted in their names being attached to a device, procedure, or disease process. Hyphenated eponyms are especially interesting, for there are always underlying stories that explicate how people became associates in history. This paper will describe a bit of hyphenated history regarding orthopaedic trauma, specifically, the development of the Böhler-Braun frame. The lives of two great surgeons associated with this device, Lorenz Böhler, the "father of traumatology," and Heinrich Braun, the "father of local anesthesia," will also be reviewed.
