Hypertonia is the abnormal increase in muscle tone as a result of upper motor neuron lesions. There are three following clinical types: spasticity, dystonia, and rigidity. Management of hypertonia is individualized and should be directed by the patient and/or family׳s goals of care as well as the underlying cause of the hypertonia. Treatment options include stretching, strengthening, positioning, oral medications, botulinum toxin injections, phenol injections, as well as surgical procedures. Without effective management, hypertonia can result in muscle imbalance, abnormal movement patterns, pain, joint contracture, joint deformity, and ultimately negatively impact a patient׳s function. This discussion serves as an overview of hypertonia, focusing on spasticity and dystonia, in the pediatric population by examining the causes and epidemiology, elucidating its symptoms, discussing available treatment and management options, and clarifying why this all matters.
Summary:Purpose: When epileptogenic regions encroach on eloquent brain, surgery may incur unacceptable deficits. Reversible cooling may control seizures while preserving function. We describe the effects of cooling kindled seizures in awake, freely moving rats.Methods: We kindled rats after placement of a bipolar electrode and a copper cooling coil in dorsal hippocampus. Fully kindled animals (three consecutive grade 5 seizures) were cooled to one of two target temperatures (24• or 27• C) for 3 min preceding a kindling stimulation and 2 minutes after. We compared seizure score (0-5) and afterdischarge duration (ADD) with and without cooling. Target temperatures were confirmed in identical animals by using a needle thermocouple advanced to the kindling target while circulating coolant.Results: Circulation of 16 • C and 8• C coolant reliably achieved transcortical cooling of the hippocampal target to 27.0 ± 1.2• C and 23.8 ± 2.0• C, respectively, by 180 s. Cooling with 16• C coolant (n = 5) significantly reduced seizure scores from 5 to 2.57 ± 1.56, and ADD from 142 ± 94.5 s to 45.7 ± 20.5 s.Cooling with 8• C coolant (n = 5) reduced seizure scores from 5 to 2.0 ± 0.42, and ADD from 132.3 ± 29.6 s to 55.5 ± 25.9 s. In 33.3% of all cooled stimulations, grade 0 seizures resulted; grade 5 seizures recurred during subsequent stimulations when cooling was withheld.Conclusions: Fully kindled, tonic-clonic seizures can be suppressed or aborted with periictal cooling of the kindling target. Anticonvulsant activity occurred at temperatures well above those known to result in tissue injury or inhibition of normal neurologic function. These findings have important implications for the potential use of implantable cooling devices in humans with refractory epilepsies in or near eloquent cortex or dominant hippocampal formations.
Dystonia is a complex movement disorder that is challenging to identify and quantify. The aim of this article is to review the clinical scales, kinematic measures, and functional implications of dystonia. Clinical measures include the Barry-Albright Dystonia Scale, the Burke-Fahn-Marsden Movement Scale, the Unified Dystonia Rating Scale, the Global Dystonia Rating Scale, and the Movement Disorder-Childhood Rating Scale. The evidence, reliability, and validity of each scale will be outlined. The Hypertonia Assessment Tool will be discussed emphasizing the importance of discriminating hypertonia. The role of kinematic measures in analyzing dystonia will be explored, as well as the potential for its future clinical applications.
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