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Spastic hemiplegia is a neuromuscular condition of spasticity that results in the muscles on one side of the body being in a constant state of contraction. It is the "one-sided version" of spastic diplegia. It falls under the mobility impairment umbrella of cerebral palsy. About 20–30% of people with cerebral palsy have spastic hemiplegia. [1]
Hemiplegia, in its most severe form, is the complete paralysis of one entire side of the body. Either hemiparesis or hemiplegia can result from a variety of medical causes, including congenital conditions, trauma, tumors, traumatic brain injury and stroke. [1]
The disease presents with progressive stiffness and contraction in the lower limbs. [1] HSP is also known as hereditary spastic paraparesis, familial spastic paraplegia, French settlement disease, Strumpell disease, or Strumpell-Lorrain disease. The symptoms are a result of dysfunction of long axons in the spinal cord.
The types of spastic cerebral palsy are generally distinguished by the primary areas of the body that are affected. [2] Spastic hemiplegia Hemiplegia is a type of cerebral palsy affecting one vertical half of the body (such as one arm and one leg). [15] [16] The affected side of the body is opposite the affected area of the brain in hemiplegia.
Alternating hemiplegia (also known as crossed hemiplegia) is a form of hemiplegia that has an ipsilateral cranial nerve palsies and contralateral hemiplegia or hemiparesis of extremities of the body. The disorder is characterized by recurrent episodes of paralysis on one side of the body. [ 1 ]
Spasticity, a velocity-dependent change in muscle tone; Clasp-knife response where initial higher resistance to movement is followed by a lesser resistance; Babinski sign is present, where the big toe is raised (extended) rather than curled downwards (flexed) upon appropriate stimulation of the sole of the foot. The presence of the Babinski ...
L1 syndrome is a group of mild to severe X-linked recessive disorders that share a common genetic basis. The spectrum of L1 syndrome disorders includes X-linked complicated corpus callosum dysgenesis, spastic paraplegia 1, MASA syndrome, and X-linked hydrocephalus with stenosis of the aqueduct of Sylvius (HSAS).
Besides hemiplegia, symptoms of the disorder include an extremely broad range of neurological and developmental impairments which are not well understood. Normally, hemiplegia and other associated symptoms cease completely with sleep, but they may recur upon waking. [1] Most frequently AHC is caused by a spontaneous mutation in the ATP1A3 gene.