1/2. Hereditary neuropathy with liability to pressure palsies (HNPP) in a toddler presenting with toe-walking, pain and stiffness.The typical clinical presentation of hereditary neuropathy with liability to pressure palsies is an adult-onset recurrent, painless monoparesis. Electrophysiological abnormalities--decreased nerve conduction velocities and delayed distal latencies--can be detected even in asymptomatic patients. We describe a toddler, who presented with asymmetric toe walking, painful cramps and stiffness in the legs. He had calf hypertrophy, brisk tendon reflexes and bilateral Babinski signs and the electrophysiological examination was normal. The unlikely diagnosis of hereditary neuropathy with liability to pressure palsies was reached 5 years later, when the boy started to complain of episodic numbness and weakness in the upper extremities. His father, paternal aunt and grandmother had similar symptoms, but they had never been investigated. The typical 1.5 Mb deletion on chromosome 17p11.2-12 was found in our patient and his affected relatives.- - - - - - - - - - ranking = 1keywords = pressure (Clic here for more details about this article) |
2/2. Reduction of hypertonicity by early casting in a comatose head-injured individual. A case report.The patient in this report sustained a closed-head injury. Within six days of the injury, he demonstrated generalized hypertonicity accompanied by alternating strong decerebrate and decorticate posturing. By Day 10 after injury, normal passive ROM could no longer be maintained using neurophysiological therapeutic techniques. Therefore, we applied plaster casts to both ankles. Within 24 hours of cast application, the patient's general hypertonus had decreased, and by 72 hours after casting, the patient demonstrated seemingly purposeful movements of the left extremities. Although casts have been used previously to maintain ROM or decrease contractures and to improve balance of muscle function of the joint that was immobilized, we believe that the marked, generalized reduction in tone in body segments far removed from the ankles was an important clinical observation. We explored several possible mechanisms for these observations. Because the usual casting conditions for reflex inhibition were not obtained with this patient, other factors were suggested to be important: total even pressure and neutral warmth. research is currently in progress at the Medical Center Hospital of vermont to study further the effects of plaster casting and to understand the mechanisms underlying the effects.- - - - - - - - - - ranking = 0.16666666666667keywords = pressure (Clic here for more details about this article) |