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Query: UMLS:C0026838 (spasticity)
 6,471 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Eight patients with a homogeneous syndrome of progressive symmetric spinobulbar spasticity were studied. Clinical features were limited to those associated with dysfunction of the descending motor tracts and included spastic quadriparesis, pseudobulbar affect, spastic dysarthria, hyper-reflexia and bilateral Babinski signs. Lower motor neuron findings were absent and higher cognitive function preserved. Median age of onset was 50.5 yrs and median disease duration was 19 yrs. Neuropathologic features (including morphometric analysis) in the single autopsied case confirmed the selective involvement of the motor cortex. There was complete absence of Betz cells from layer 5 of the precentral cortex and the remaining pyramidal cells were significantly smaller than those seen in normal controls. Magnetic resonance imaging (MRI) revealed atrophy of the precentral gyrus and positron emission tomography (PET) scans showed diminished glucose [18F]fluorodeoxyglucose uptake in the pericentral cortex. Magnetic motor cortex stimulation revealed markedly prolonged central motor conduction times. The literature is reviewed and diagnostic criteria for primary lateral sclerosis based on clinical, laboratory and imaging features are proposed.
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PMID:Primary lateral sclerosis. Clinical features, neuropathology and diagnostic criteria. 160 79

Sarcoidosis is a multisystem granulomatous disorder that rarely involves the spinal cord. This report describes the presentation and rehabilitative course of a 31-yr-old man with quadriparesis secondary to spinal cord sarcoidosis. The patient had insidious, progressive weakness in his arms and legs for six weeks before evaluation. Examination revealed a C4 incomplete spinal cord injury. Computed tomography demonstrated an intrinsic cord lesion from the brainstem to approximately T8. Magnetic resonance imaging (MRI) suggested the lesion was granulomatous and cervical laminectomy confirmed noncaseating granulomas. The patient was started on high dose steroids, subsequently gained strength in the distal upper extremities, and was sent for spinal cord rehabilitation. Examination revealed 3 to 4+/5 strength in the upper extremities, 2- to 3-/5 in the lower extremities. The right side was slightly stronger than the left, with proximal musculature stronger than distal. Sensory examination was intact except in the C-8 to T-2 dermatomes. The patient was dependent in self-care and mobility except for feeding. Initial progress was inhibited by severe spasticity requiring medication, but by discharge he was independent at the wheelchair level with 4/5 strength in all four extremities except for his hands, which had 3/5 strength. Sensory exam did not change. Follow-up MRI studies revealed reduction of the lesion. Review of previous cases revealed that myelopathy is the most common presenting complaint and cervical segments are most commonly involved. Survival averaged almost three years and significant gains were made in functional status. Rehabilitative course and special considerations, treatment and follow-up recommendations are discussed.
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PMID:Rehabilitation of quadriparesis secondary to spinal cord sarcoidosis. 200 60

The case is reported of a 2-year-old boy born with Marshall-Smith syndrome who had difficulty in swallowing and who exhibited spasticity and quadriparesis due to compression of the medulla and cervical spine. This is the first child with this rare condition reported to have brain-stem compression from bone abnormalities at the craniovertebral junction and who has required surgery.
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PMID:Cervicomedullary junction decompression in a case of Marshall-Smith syndrome. Case report. 207 73

Scarring around the electrically stimulating electrodes has been of concern since dorsal column stimulation was introduced. This concern resurfaced in the days of cerebellar electrodes and, with the advent of epidural stimulating techniques for the control of pain and spasticity, it again arises as a potential problem. We present a patient who underwent the placement of a C-2-C-4 electrode to treat torticollis; 3 months later, a mild spastic quadriparesis developed and the stimulation became ineffective. At reexploration, dense scar surrounded the electrode and confined the cervical spinal cord. With microdissection techniques, the scar was removed from the dura mater and the dura began to pulsate freely. The quadriparesis reversed. Examination of the scar tissue microscopically showed linearly arrayed fibroblastic nuclei, and we are uncertain whether the exuberant fibroblastic response is a response to electrical stimulation, the materials used in the electrode, or some technical aspects of the operation.
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PMID:Scarring around cervical epidural stimulating electrode. 660 94

A case report of an infant whose mother used phencyclidine (PCP, "angel dust") during pregnancy is presented. As a neonate, the infant showed abnormal behavior and an unusual appearance, and later, spastic quadriparesis. Based on previous animal studies, it is likely that this infant had prolonged exposure to PCP as a fetus. His abnormal neonatal behavior was consistent with previously reported effects of this drug. The relationship between his exposure to PCP and his dysmorphology and spasticity remains speculative. It is suggested that clinicians be alert to further cases of these associations.
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PMID:Angel dust: possible effects on the fetus. 735 23

Pelizaeus-Merzbacher disease (PMD) is a degenerative leukodystrophy of the central nervous system resulting in progressive spasticity and neurologic deterioration. Seitelberger (13) divided this rare disease into six types. Five patients with the type 1 and two patients with the type II form of PMD have been treated at the Children's Hospital of Eastern Ontario. Our study sought to identify the orthopaedic manifestations of PMD and to develop a common orthopaedic profile for these patients. All children with types I and II PMD developed spastic quadriparesis, truncal hypotonia, thoracolumbar scoliosis, soft-tissue contractures of the adductors and hamstrings, osteopenia, bilateral coxa valga, and associated hip dislocation.
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PMID:The orthopaedic manifestations of Pelizaeus-Merzbacher disease in children. 890 38

A boy with a dystonic quadriparesis presented with acquired paralysis, spasticity, and a feeding disorder. Spinal MRI revealed a cervical cord lesion and os odontoideum. Excessive movement of the neck, leading to failure of ossification of the dens and then to cervical cord trauma was the likely mechanism. The poor outcome of this subject is described, emphasising the need to pay careful attention to neurological changes in children with extrapyramidal cerebral palsy, who may be at particular risk of cord pathology. The management issues are discussed.
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PMID:Acquired spinal cord lesion associated with os odontoideum causing deterioration in dystonic cerebral palsy: case report and review of the literature. 956 58

Co-contraction of antagonist muscles is characteristic of spasticity arising from perinatal brain damage but not in spasticity occurring after brain damage in adulthood. Such co-contraction is a normal feature of early post-natal motor development. Heteronymous, monosynaptic Group Ia projections from biceps brachii to both the antagonist triceps brachii and to other synergist and non-synergist muscles of the upper limb occur in the newborn baby and become restricted during the first 4 years to motor neurons of primarily synergistic muscles. Longitudinal and cross-sectional studies have been performed to test the hypothesis that inappropriate heteronymous excitatory projections persist in children with perinatal brain damage who develop spasticity. Subjects with spasticity, from brain damage acquired in adulthood were also studied to determine if these projections simply become unmasked as part of spasticity, independent of the age of occurrence of the brain damage. Twenty-nine healthy newborn babies and 29 at high risk for cerebral palsy, 12 of whom developed spastic quadriparesis, were studied longitudinally for 4 years. Thirty-eight subjects, aged 8-30 years, with spasticity of perinatal origin (11 hemiplegic, 11 quadriplegic, 16 with Rett syndrome) and 11 subjects with stroke in adulthood and spastic hemiplegia were also studied. The results were compared with those obtained in 372 normal subjects aged from birth to 55 years. Small taps were delivered to the tendon of biceps brachii using an electromechanical tapper. Surface EMG was recorded from biceps and triceps brachii, pectoralis major and deltoid. In the longitudinal study, those developing spastic quadriparesis showed persistent low thresholds for the homonymous phasic stretch reflex, which had abnormally short onset latencies. There was persistence of short onset heteronymous excitatory responses in triceps brachii, while a normal pattern of restriction of heteronymous responses to pectoralis major and deltoid occurred. The same pattern was observed in older subject groups with spasticity of perinatal origin. In adults with hemiplegia following stroke the threshold of the homonymous phasic stretch reflex was low, but it had a normal onset latency. There was no evidence of abnormal heteronymous excitatory responses. In conclusion, exaggerated excitatory responses to primary muscle afferent input were observed in the homonymous (biceps brachii) and antagonist (triceps brachii) motor neurons in subjects with spasticity arising from perinatal brain damage. They are likely to play an important role in the predominant co-contraction of agonist/antagonist muscles during voluntary movement observed in subjects with spastic cerebral palsy.
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PMID:Abnormal development of biceps brachii phasic stretch reflex and persistence of short latency heteronymous reflexes from biceps to triceps brachii in spastic cerebral palsy. 987 88

Direct measurement of energy expended by spasticity in children with severe spastic quadriparesis is difficult. Insertion of an intrathecal baclofen pump in a 13-year-old boy with severe spasticity and profound mental retardation resulted in an estimated 30 to 40% decrease in his spasticity. As he had been on a carefully calculated ketogenic diet and fed by gastrostomy, his precise caloric intake was known. Decrease in spasticity, on the same caloric intake, led to marked weight gain. Reduction of 100 calories intake resulted in new weight stability. It was possible therefore, to estimate indirectly energy used by his spasticity. This 100 calories, representing 34% of calories above his resting energy requirement, corresponded to an independently estimated 30 to 40% of caloric expenditure of his spasticity. It was concluded that when calculation of calories is critical, energy utilization by spasticity must be taken into consideration.
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PMID:Energy requirements of spasticity. 1199 99

For children whose spasticity and movement disorders are inadequately treated by oral medications and botulinum toxins, neurosurgical procedures are now available to effectively treat spasticity, tremor, and many cases of dystonia. Spastic diplegia can be treated with selective lumbar rhizotomies, which significantly decrease spasticity, increase range of motion, and improve Gross Motor Function Measure scores. Children with spastic quadriparesis and those with secondary dystonia can be treated with intrathecal baclofen, which diminishes both spasticity and dystonia and is associated with improved function and quality of life. Children with primary dystonia and those with tremor can be treated with deep brain stimulation of the internal globus pallidus and thalamus, respectively. Some children with chorea respond to deep brain stimulation. There are no effective neurosurgical treatments for athetosis or ataxia. The effectiveness of neurosurgical treatments of pediatric movement disorders has increased significantly in the past 15 years.
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PMID:Neurosurgical treatment of spasticity and other pediatric movement disorders. 1367 72


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