Gene/Protein Disease Symptom Drug Enzyme Compound
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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

The 'excitability' and 'conductivity' of motor pathways during transcranial stimulation (TCS) have been investigated in 49 patients affected by multiple sclerosis (34), amyotrophic lateral sclerosis (7), spino-cerebellar ataxia (3), primary lateral sclerosis (4) and brain metastasis (1). Hyper-reflexia, spasticity and weakness were correlated with the central motor conduction time (CCT) and with the threshold intensity of TCS required to produce a motor evoked potential (MEP). MEPs to magnetic TCS were recorded from hand and foot muscles during relaxation, contraction and after tendon vibration. Thresholds and CCTs of the patients were compared with those of 30 healthy controls. Increased threshold was found in 37 out of 49 patients (75.5%). Prolongation of the CCT was found in 38 out of 63 clinically affected upper limbs (60.3%) and in 56 out of 77 clinically affected lower limbs (72.7%). Absent motor responses to maximal TCS were found in 20 out of 98 lower limbs (20.4%). Excluding ALS patients (in whom there was a lower threshold for MEP elicitation), a significant linear correlation was found between prolonged CCT and increased threshold. While MEPs with prolonged CCTs have elevated TCS threshold, it is important to note that an elevated threshold was found in 14 out of 49 patients (28.5%) despite unchanged CCT. Spasticity and/or hyper-reflexia were more frequently associated with increased threshold than with prolonged CCT, while weakness was correlated equally well with both these parameters. In this respect magnetic TCS proves to represent a new tool for the detection of abnormal 'excitability' of the central motor tracts.
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PMID:'Excitability changes of muscular responses to magnetic brain stimulation in patients with central motor disorders. 171 17

A patient is described with slowly progressive supranuclear motor system degeneration (primary lateral sclerosis) characterized by pure bulbar spasticity for six years until a spastic tetraparesis developed. Clinically and electrophysiologically there was and still is no evidence of lower motor neuron involvement. Recently the patient presented with a syndrome of complete inability to move face and tongue voluntarily with preservation of the ability to move them "automatically", e.g. within gestures: automatic-voluntary motor dissociation. Loss of voluntary innervation of the facio-pharyngo-glossomasticatory muscles with preservation of involuntary innervation are the features of the Foix-Chavany-Marie syndrome, which to date has most often been described in association with bilateral vascular lesions of the opercula or their cortigofugal projections.
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PMID:Opercular syndrome without opercular lesions: Foix-Chavany-Marie syndrome in progressive supranuclear motor system degeneration. 214 37

Primary lateral sclerosis (PLS), previously undescribed in children, is characterized by a spastic motor deterioration and pathologic demonstration of corticospinal tract degeneration. We report an infant who, before 12 months of age, developed a progressive motor disease characterized by generalized spasticity. He died at 47 months, and neuropathologic examination revealed only corticospinal tract degeneration. We conclude that this child had typical clinical and pathologic findings for PLS.
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PMID:Primary lateral sclerosis in a child. 281 36

Although it was first described over a century ago (by Charcot in 1865; by Erb in 1875), the concept of primary lateral sclerosis (PLS) is still not universally accepted. Despite this skepticism, several well-documented cases of isolated degeneration with varying degrees of involvement of corticospinal pyramidal pathways have been reported in the literature. The clinical manifestations in these cases can take one of two forms, ie, isolated spasmodic paraplegia or tetraplegia on the one hand or spasmodic tetraplegia associated with a pseudobulbar syndrome featuring severe spastic dysarthria (chronic progressive bilateral spinobulbar spasticity) on the other hand. Obviously, without firm pathologic data, PLS is a hazardous diagnosis for isolated paraplegia or tetraplegia. Conversely, for bilateral spinobulbar spasticity, it would appear to be the only diagnosis possible once investigate findings have eliminated the other possibilities, such as a pyramidal form of amyotrophic lateral sclerosis or a spinal form of multiple sclerosis. To underscore this point, in this report, five cases of chronic progressive bilateral spinobulbar spasticity developed over 5, 10, 12, 10, and 28 years, respectively, for which the only possible diagnosis was PLS. It was concluded that there are three forms of degenerative diseases of the principal motor pathways: one involving both central and peripheral neurons, ie, amyotrophic lateral sclerosis; one involving only peripheral neurons, ie, spinal amyotrophy; and one involving only central motor neurons, ie, PLS.
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PMID:Chronic progressive spinobulbar spasticity. A rare form of primary lateral sclerosis. 335 2

The syndrome of primary lateral sclerosis (PLS) has been reported clinically on many occasions. Pathologic confirmation in the modern era, however, has generally been lacking. In a recently reported case of PLS, the disorder was complicated by a pontine infarct. We describe a 65-year-old woman whose illness began with spastic dysarthria, which gradually worsened to the point that 18 months later she could barely utter a sound. Meanwhile, dysphagia, brisk reflexes, and a pseudobulbar affect had developed. Three years after onset she had a spastic contractured right-sided hemiplegia and walked with short shuffling steps. The spasticity slowly progressed, and she died of aspiration pneumonia 3.5 years after the onset of dysarthria. Neuropathologic examination showed bilateral atrophy of the precentral gyri, which microscopically showed a paucity of Betz cells. There was loss of myelin throughout the corticospinal system, yet the anterior-horn cells of the spinal cord and hypoglossal nuclei were well preserved. Intracytoplasmic eosinophilic inclusion bodies, of unknown cause and significance, were observed in occasional motor neurons, one in the hypoglossal nucleus and two in spinal cord anterior horns. Clinically and pathologically, this case meets the criteria for PLS.
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PMID:Primary lateral sclerosis: a case report. 729 6

Nine patients with clinically diagnosed, radiologically supported primary lateral sclerosis underwent cognitive testing. None was demented, but eight had mild cognitive impairment. Performances were most consistently impaired on neuropsychological tests sensitive to frontal lobe functions, followed by tests sensitive to memory. Cognitive testing may be useful in helping to establish a cortical localization in patients with the syndrome of progressive spasticity. There are potential nosologic relations between primary lateral sclerosis and other degenerative frontal lobe syndromes, such as frontal lobe dementia and progressive spasticity with dementia.
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PMID:Primary lateral sclerosis: a neuropsychological study. 750 Nov 49

In two groups of patients posterior tibial nerve (PTN) and sural nerve (SN) somatosensory evoked potentials (SEPs) were compared to each other and related to classified neurological signs. Group A consisted of 7 patients with hereditary spastic paraparesis (HSP) and 8 with primary lateral sclerosis (PLS), with solely or primarily motor deficits. Group B consisted of 12 patients with different spinal cord diseases causing variable mixed sensory and motor impairments. Normal values were derived from 39 controls. A clear trend towards more frequently prolonged PTN SEP than SN SEP latencies was found in both groups and appears to make PTN SEPs more useful for clinical application than SN SEPs. No significant differences were found in SEP abnormalities when the two patient groups were compared to each other. No relationships were found between SEP abnormalities and spasticity, weakness or any single sensory modality, making the two SEPs questionable as a quantitative test for neurological deficits in our patients.
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PMID:Posterior tibial and sural nerve somatosensory evoked potentials: a study in spastic paraparesis and spinal cord lesions. 750 31

In this paper, the autopsy findings of a 78-year-old man mimicking primary lateral sclerosis (PLS) are reported. His clinical symptoms were slowly progressive spasticity, pseudobulbar palsy and character change. He died of sepsis 32 months after protracting the disease. The autopsy revealed severe atrophy of the frontal and temporal lobes. The histological findings were severe neuronal loss with gliosis in the precentral gyrus and left temporal lobe tip, loss of Betz cell, prominent demyelination throughout of the corticospinal tract, axonal swelling in the cerebral peduncule, severe degeneration of the amygdala, mild degeneration of the Ammon horn, normal substantia nigra, a few neuronal cells with central chromatolysis in the facial nerve nucleus and very mild neuronal cell loss in the spinal anterior horn. The anterior horn cell only occasionally demonstrated Bunina body by H & E and cystatin-C stainings, as well as, skein-like inclusion by ubiquitin staining. Thus, this is a case of uncommon amyotrophic lateral sclerosis (ALS) dominantly affecting the upper motor neuron including the motor cortex and temporal limbic system. In analysis of nine cases of putative primary lateral sclerosis in the literature, six cases showed loss of Betz cell in the precentral gyrus, and four cases very mild involvement of the lower motor neuron such as central chromatolysis and eosinophilic inclusion body. Degeneration of the limbic system was observed in two cases. We indicated a possible subgroup with concomitant involvement in the motor cortex and temporal lobe in motor neuron disease dominantly affecting the upper motor neuron.
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PMID:[An autopsied case of dominantly affecting upper motor neuron with atrophy of the frontal and temporal lobes--with special reference to primary lateral sclerosis]. 761 64

Primary lateral sclerosis (PLS) bears close resemblance to cases of amyotrophic lateral sclerosis (ALS) presenting with spasticity, but histopathological studies have shown significant difference between the two conditions. When the lower motor neurons in cases of ALS and PLS are compared with the equivalent cells of control subjects, morphometric studies indicate significantly decreased size and increased convexity (rounding) of the cell bodies only in ALS. In both disorders there is loss or shrinkage of the largest cortical motor neurons (Betz cells) in the primary motor cortex, though this change is not conspicuous in all cases of ALS. Morphometry reveals in both diseases a general reduction in the sizes of pyramidal cells in the precentral gyrus, indicating that smaller neurons are involved. The cortical motor neurons shrink more in PLS than in ALS. It is concluded that there is clear difference between ALS and PLS. In PLS, quantitative histopathological data show that the neuronal degeneration is confined to long descending pathways, notably the corticospinal system, with no concomitant involvement of lower motor neurons. In ALS, lower motor neuron degeneration occurs in all cases, whereas involvement of the motor cortex is variable.
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PMID:Clinicopathological features of primary lateral sclerosis are different from amyotrophic lateral sclerosis. 845 84


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