Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0013362 (dysarthria)
 3,768 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We administered local botulinum toxin injections on the leg adductors of 12 patients with spastic paraparesis (9 patients with HAM, 2 patients with spinal spastic paraparesis, 1 patient with an identified degenerative disease). Two of them were wheelchair-bound and the other patients could walk with or without help. The patients were assessed by the time to walk 10 m and the spasticity score which was derived from the degree of muscle tone and spasm frequency of leg adductors. After the initial injection, 7 of the 12 patients improved spasticity scores and 8 of the 10 patients could walk 10 m within a shorter time. The time to walk 10 m was markedly shortened in moderate cases. However, one patient complained of leg weakness and the time to walk 10 m was prolonged. Five of the 12 patients received injections 3 to 7 times, and were followed up for a mean of 16.2 months. In 4 of the 5 patients, repeated injections could maintain the improvement of spasticity score and time to walk 10 m. However, injection was discontinued in one patient because of leg weakness. The other side effects were pain and swelling at the injected site and dysarthria. However, these side effects were slight and transient and did not require treatment. No other systemic side effects were observed. In conclusion, the beneficial effects of botulinum injections to spastic paraparesis were (1) improvement of objective symptoms in mild cases, (2) improvement of ADL in moderate cases, and (3) improvement of objective symptoms and ease of nursing care in severe cases. Furthermore, we confirmed the long-term efficacy and safety of botulinum toxin.
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PMID:[Treatment of spastic paraparesis with botulinum toxin with reference to beneficial effects, disease severity and long-term treatment]. 761 46

Amyotrophic lateral sclerosis (ALS) is a typical intractable disease affecting the primary and secondary motoneurones resulting in generalized muscular atrophy and weakness with or without spasticity. Dysphagia, dysarthria, and respiratory difficulty are symptoms which cause restriction of ADL and death. Recent achievement in understanding neuronal death in ALS has invited trials on various drugs aiming at neuroprotection and prolongation of the course of ALS. They include inhibition of excitotoxicity of amino acids, suppression of free radicals by lecithinized SOD and various neurotrophic factors. Significant prolongation of life span was obtained by riluzole in a US-Europe trial, but the effects were insignificant in the Japanese nation-wide trial.
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PMID:[Neuroprotective therapy for amyotrophic lateral sclerosis (ALS)]. 912 96

The aim of the present study was to assess the efficacy and safety of chronic subthalamic nucleus deep-brain stimulation (STN-DBS) in patients with Parkinson's disease (PD). 18 consecutive severely affected PD patients were included (mean age, SD: 56.9+/-6 years; mean disease duration: 13.5+/-4.4 years). All the patients were evaluated clinically before and 6 months after the surgical procedure using the Unified Parkinson's Disease Rating Scale (UPDRS). Additionally, a 12 months follow-up was available in 14 patients. The target coordinates were determined by ventriculography under stereotactic conditions, followed by electrophysiology and intraoperative stimulation. After surgery, continuous monopolar stimulation was applied bilaterally in 17 patients at 2.9+/-0.4 V through 1 (n = 31) or 2 contacts (n = 3). One patient had bilateral bipolar stimulation. The mean frequency of stimulation was 140+/-16 Hz and pulse width 68+/-13 micros. Off medication, the UPDRS part III score (max = 108) was reduced by 55 % during on stimulation (score before surgery: 44.9+/-13.4 vs at 6 months: 20.2+/-10; p < 0.001). In the on medication state, no difference was noted between the preoperative and the postoperative off stimulation conditions (scores were respectively: 17.9+/-9.2 and 23+/-12.6). The severity of motor fluctuations and dyskinesias assessed by UPDRS IV was reduced by 76 % at 6 months (scores were respectively: 10.3+/-3 and 2.5+/-3; p < 0.001). Off medication, the UPDRS II or ADL score was reduced by 52.8 % during on stimulation (26.9+/-6.5 preop versus 12.7+/-7 at 6 months). The daily dose of antiparkinsonian treatment was diminished by 65.5 % (levodopa equivalent dose -- mg/D -- was 1045 +/- 435 before surgery and 360 +/- 377 at 6 months; p < 0.01). These results remained stable at 12 months for the 14 patients studied. Side effects comprised lower limb phlebitis (n = 2), pulmonary embolism (n = 1), depression (n = 6), dysarthria and freezing (n = 1), sialorrhea and drooling (n = 1), postural imbalance (n = 1), transient paresthesias and dyskinesias. This study confirms the great value of subthalamic nucleus stimulation in the treatment of intractable PD. Some adverse events such as depression may be taken into account in the inclusion criteria and also in the post-operative outcome.
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PMID:Subthalamic nucleus stimulation in Parkinson's disease: clinical evaluation of 18 patients. 1202 40

A 74-year-old man developed instability of gait from age 64, difficulty in writing from age 66 and dysarthria-hypohidrosis from around age 67. These symptoms progressed slowly accompanied with orthostatic hypotension and dysarthria, which decline his ADL. At age 71, he was admitted to our hospital and underwent nasogastric intubation. After admission, he also showed the decrease in his voluntary activities, accidental ingestion and loud snoring during sleep. He died of accidental aspiration pneumonia at age 74. Postmortem examination revealed severe pathological changes as multiple system atrophy (MSA) in the central nervous system. There was a small ulcer in the hypopharyngeal region, and acute inflammation of the ulcer came down to the posterior cricoarytenoid muscle. Combined with severe neurogenic atropy due to MSA, local inflammation of the ulcer associated with nasogastric intubation appeared to have resulted in severe damage of the posterior cricoarytenoid muscle.
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PMID:[Pathological study on abducent paralysis of the vocal cord in a patient having multiple system atrophy with nasogastric intubation]. 1763 6