1/22. Constant involvement of the Betz cells and pyramidal tract in amyotrophic lateral sclerosis with dementia: a clinicopathological study of eight autopsy cases.We investigated clinicopathologically pyramidal signs, including hyperreflexia, Babinski sign, and spasticity, and the involvement of the primary motor cortex and pyramidal tract, in eight Japanese autopsy cases of amyotrophic lateral sclerosis (ALS) with dementia. Pyramidal signs were observed in seven (88%) of the eight autopsy cases. Hyperreflexia and Babinski sign were evident in seven (88%) and three (38%) patients, respectively, but spasticity was not observed in any of the eight patients. Loss of Betz cells in the primary motor cortex was evident in the seven cases in which this structure was examined. Astrocytosis in the fifth layer of the primary motor cortex was noticed in three cases. In all eight cases, involvement of the pyramidal tract was obvious in the medulla oblongata, but no involvement of the pyramidal tract was found in the midbrain. Involvement of the pyramidal tract in the spinal cord, particularly of large myelinated fibers, was observed in all six cases in which the spinal cord was examined. In ALS with dementia, pyramidal signs were shown to be present more frequently than previously believed, and the clinicopathological correlation between pyramidal signs and involvement of the pyramidal tract was obvious. Constant involvement of Betz cells and the pyramidal tract in ALS with dementia has not been reported. Our clinicopathological findings may make a contribution to the understanding of the clinicopathological hallmarks of this disorder. Furthermore, we believe that this study will also contribute to the elucidation of the nosological status of ALS with dementia.- - - - - - - - - - ranking = 1keywords = sclerosis (Clic here for more details about this article) |
2/22. multiple sclerosis and oral care.multiple sclerosis is a complex neurological condition affecting sensory and motor nerve transmission. Its progression and symptoms are unpredictable and vary from person to person as well as over time. Common early symptoms include visual disturbances, facial pain or trigeminal neuralgia and paraesthesia or numbness of feet, legs, hands and arms. These, plus symptoms of spasticity, spasms, tremor, fatigue, depression and progressive disability, impact on the individual's ability to maintain oral health, cope with dental treatment and access dental services. Also, many of the medications used in the symptomatic management of the condition have the potential to cause dry mouth and associated oral disease. There is no cure for multiple sclerosis, and treatment focuses on prevention of disability and maintenance of quality of life. Increasingly a multi-disciplinary team approach is used where the individual, if appropriate his/her carer, and the specialist nurse are key figures. The dental team plays an essential role in ensuring that oral health impacts positively on general health.- - - - - - - - - - ranking = 1.2keywords = sclerosis (Clic here for more details about this article) |
3/22. Total knee replacement in patients with multiple sclerosis.Total knee replacement was performed on two patients with multiple sclerosis. Severe hamstring spasticity was encountered in both patients in the immediate post-operative period requiring further surgery. Both patients remain ambulatory at follow-up. The disease, and its implications in patients warranting total joint replacement are discussed.- - - - - - - - - - ranking = 1keywords = sclerosis (Clic here for more details about this article) |
4/22. Monozygotic twins discordant for primary lateral sclerosis.The genetics of the motor neuron diseases are becoming increasingly important. The relationship of primary lateral sclerosis and amyotrophic lateral sclerosis remains debated. Here a pair of monozygotic twins discordant for primary lateral sclerosis is presented. This monozygotic twin pair suggests that environmental factors were most relevant to this subject's development of primary lateral sclerosis. Their life history suggests very similar social and professional exposures without any apparent distinctions.- - - - - - - - - - ranking = 1.6keywords = sclerosis (Clic here for more details about this article) |
5/22. diabetes insipidus in a patient with suspected multiple sclerosis.A 53-year-old man developed spastic ataxia associated with diabetes insipidus. The patient experienced frequent attacks of stiffness and numbness of the four limbs accompanied by difficulty of speech. During an eight years' follow-up a progressive deterioration of the motor function was observed but no extracerebral manifestations were noticed. The association of spastic ataxia is generally considered as diagnostic of histiocytosis X. In the present case, however, multiple sclerosis seems a more likely diagnosis in view of the late onset and the highly characteristic brain stem seizures.- - - - - - - - - - ranking = 1keywords = sclerosis (Clic here for more details about this article) |
6/22. Effect of cannabinoids on spasticity and ataxia in multiple sclerosis.The chronic motor handicaps of a 30-year-old multiple sclerosis patient acutely improved while he smoked a marihuana cigarette. This effect was quantitatively assessed by means of clinical rating, electromyographic investigation of the leg flexor reflexes and electromagnetic recording of the hand action tremor. It is concluded that cannabinoids may have powerful beneficial effects on both spasticity and ataxia that warrant further evaluation.- - - - - - - - - - ranking = 1keywords = sclerosis (Clic here for more details about this article) |
7/22. Oxcarbazepine and spasticity: further observations.The previously described anti-spastic effect of oxcarbazepine and 10,11-dihydro-10-hydroxycarbamazepine was found accidentally in 2 patients undergoing a double-blind comparative study for evaluation of antiepileptic effect. In this study oxcarbazepine was given orally in doses of 300-2700 mg daily to one patient with transverse myelitis and to two patients with multiple sclerosis, all of whom had clinically disabling spasticity in the form of difficulty in walking, lower limb rigidity, spastic contractions of the lower limbs and ankle clonus. Anti-spastic effect was observed at doses between 600-1200 mg daily and consisted in a substantial decrease in the above symptoms of spasticity. The anti-spastic effect appears at a dose immediately below that which produces nausea, dizziness and somnolence.- - - - - - - - - - ranking = 0.2keywords = sclerosis (Clic here for more details about this article) |
8/22. Familial spastic paraplegia, bilateral sensorineural deafness, and intellectual retardation associated with a progressive nephropathy.We present a family in which at least four persons have evidence of an inherited disorder comprising a variable spastic paraplegia, bilateral sensorineural deafness, intellectual retardation, and a progressive nephropathy. Focal segmental proliferative lesions with sclerosis suggestive of mesangial IgA nephropathy (Berger's disease) were found on renal renal biopsy in two affected persons. The glomerular basement membrane showed none of the changes characteristic of Alport's syndrome. Males and females are affected and the segregation of the disease is consistent with dominant transmission.- - - - - - - - - - ranking = 0.2keywords = sclerosis (Clic here for more details about this article) |
9/22. Chronic progressive spinobulbar spasticity. A rare form of primary lateral sclerosis.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.- - - - - - - - - - ranking = 1.6keywords = sclerosis (Clic here for more details about this article) |
10/22. Complete hemidiaphragmatic paralysis in a patient with multiple sclerosis.We present a case history of a patient with definite multiple sclerosis who developed an abrupt onset of unilateral diaphragmatic paralysis, minor increase in lower extremity spasticity and complaint of marked neck stiffness. Her vital capacity during this episode was 600 mL and she was in impending respiratory failure. The diaphragmatic paralysis was demonstrated by radiographic plain films and fluoroscopy. phrenic nerve stimulation was performed during fluoroscopy and the evoked motor response from the diaphragm recorded. There was a normal amplitude diaphragmatic twitch observed with an evoked motor response latency of 1 ms and amplitude of 300 microV. After high dose intravenous steroids, her neck stiffness and spasticity improved, her vital capacity improved to 1500 mL and her diaphragm regained its normal position and movement confirmed by followup radiographic plain films and fluoroscopy. We postulate the presence of a demyelinating plaque in the brainstem fibers descending to the phrenic nucleus as the etiology of the diaphragmatic paralysis. We are unaware of any other case reports of unilateral "upper motor neuron" phrenic nerve paralysis secondary to multiple sclerosis.- - - - - - - - - - ranking = 1.2keywords = sclerosis (Clic here for more details about this article) |
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