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Query: UMLS:C0013362 (dysarthria)
 3,768 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

This is the first large scale case series of motor neurone disease (MND) in Thailand. Seventy-seven patients were identified between 1978 and 1984 at Siriraj Hospital Medical School, Bangkok, Thailand. Fifty-five patients were male (71.43%) and the mean age of the patients was 51.55 (SD 14.26) years with the range of 17 to 78 years. Clinical classification of MND was categorized as progressive bulbar palsy (PBP), 26 patients (33.77%); amyotrophic lateral sclerosis (ALS), 42 patients (54.54%); and progressive spinal atrophy (PSA), 9 patients (11.69%). The mean age of PBP, ALS and PSA were in the order of 57.61 (SD 12.09), 52.81 (SD 11.18), and 28.11 (SD 9.44) years. Progressive spinal atrophy group was younger than PBP and ALS groups significantly at the P-value less than 0.05 by analysis of variance and Duncan tests. Fifty-three patients (72.60%) were resident in Bangkok and the central part of Thailand. The main presenting symptoms were wasting of the small muscles of both hands, leg weakness, and speech and/or swallowing difficulties. These symptoms were found in 62 patients (81.58%). Nearly half of the patients (48.68%) came to our care within six months of onset, 22.8 per cent presented with asymmetry of motor wasting, while limb and trunk fasciculation was seen in 73.61 per cent. Dysarthria, dysphagia and tongue fasciculation were recorded as 51.32, 48.68, 60.53 per cent respectively. Exaggerated deep tendon reflexes were noted as 65.79 and 80.26 per cent over the upper and lower limbs, while Babinski sign was elicited in only 23.3 per cent of the patients.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Motor neurone disease in Thailand: the clinical aspects of 77 patients. 194 Jul 1

A case is presented of a patient with progressive bulbar palsy, a form of motor neuron disease, which is a progressive degenerative disorder of the motor nuclei in the medulla producing atrophy and fasciculations of the musculature of the tongue, dysarthria, dysphagia, and excessive accumulation of secretions. The patient may initially seek dental consultation. Clinicians should be aware of the signs and symptoms of this inexorably fatal disease so that an early neurologic referral is made and the appropriate symptomatic therapies instituted.
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PMID:Progressive bulbar palsy: a case report of a type of motor neuron disease presenting with oral symptoms. 230 45

To evaluate dysarthria in patients with ALS, we used MRI (gradient rephasing echo method) and compared it with the computed acoustic analysis. Five ALS male patients of progressive bulbar palsy type and five normal male were asked to phonate the five Japanese vowels, /a/./i/./u/./e/./o/. MRI of the sagittal tongue and vocal tract was obtained by the gradient rephasing echo method (0.2 Tesla, TR:30 ms, TE:10 ms, FA 25 degrees C, Hitachi). We could clearly visualize the change of tongue shape and the narrow site of the vocal tract for each vowel phonation. In normal subjects, the tongue shape and the narrow site of the vocal tract were distinguishable between each vowel, but unclear in ALS. Acoustic analysis showed that the first formant frequency of /i/./u/ in ALS was higher than normal and the second formant frequency of /i/./e/ in ALS was significantly lower than normal. The discrepancy from the normal first, second and third formant frequency for each vowel of ALS was most seen in /i/./e/. It was speculated that /i/ and /e/ were the most disturbed vowels in ALS. The first and second formant frequency of vowel depends on the tongue shape and the width of the oral cavity. Therefore the results of the acoustic analysis in ALS indicated poor movement of tongue in /i/./u/./e/ and were compatible with the findings of the sagittal tongue MRI. The sagittal view of the tongue in the gradient rephasing echo MRI and the acoustic analysis are useful in evaluation dysarthria in ALS.
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PMID:[Analysis of dysarthria in amyotrophic lateral sclerosis--MRI of the tongue and formant analysis of vowels]. 820 Jan 37

We report a 49-year-old man with progressive bulbar palsy and respiratory failure. He was well until his 48 years of the age (December 1994) when he noted a difficulty in speaking in loud voice. In February, 1995, he noted regurgitation of foods to his nose and difficulty in his speech. He was admitted to our service in May 29, 1995. On admission, he was alert and oriented to all spheres and he was not demented. His higher cerebral functions were normal. In cranial nerves, he showed dysarthria and dysphagia; muscle atrophies were seen in the tongue, the bilateral sternocleidomastoid, supraspinatus, and infraspinatus muscles. Fasciculations were seen in these muscles. He showed no muscle weakness in his limbs except for the upper limb girdle muscles, no ataxia, no reflex abnormalities, nor sensory changes. EMG showed neurogenic changes in the affected muscles. MRI of the brain and the spinal cord was entirely normal. He was discharged for out patient follow-up, however, in October of 1995, he noted difficulty in swallowing solid foods. Gastrostomy was placed and he was discharged to his home. In February 11th of 1996, he was found unresponsive and brought into the ER of our hospital. On admission, he was comatose without spontaneous respiration. BP could not be obtained. He was immediately intubated and artificial ventilation was started. On the following morning, he became alert and he was not demented. He continued to show marked dysarthria and dysphagia; again no weakness was noted in the distal parts of the upper and lower extremities. Laboratory examination showed increase in serum CK to 2,173 IU/L and amylase to 2,032 IU/L. He was extubated on February 15th, however, his spontaneous respiration was not suffice to maintain his blood gas. According to his will, he was not placed on respirator and he died on February 24th, 1996. The patient was discussed in a neurological CPC and the chief discussant arrived at the conclusion that the patient had ALS. Although no upper neuron signs were observed clinically, it is not uncommon to see degeneration in the corticospinal tract in post-mortem examination. The question was what might have been the cause of increase in CK and amylase. Many participants thought that they were secondary to multiple organ failure due to prolonged hypoxic state at his last admission; other possibilities raised included acute myocardial infarction and acute bowel necrosis. Post-mortem examination revealed muscle atrophy in the facial, lingual, cervical, intercostal, and the upper limb girdle areas. The lungs were unremarkable except for old organized pneumonic foci in the right middle and lower lobes. Marked to moderate congestion was seen in many internal organs, however, no other gross abnormality was found. It was thought that respiratory palsy itself was the direct cause of his agonal event. In the spinal cord, the anterior horns showed various degree of neuronal loss and gliosis. No clear evidence of pyramidal tract degeneration was seen at the light microscope level. Lower brain stem motor neurons were markedly reduced. But no Bunina body was found. The substantia nigra showed moderate degree of neuronal loss and extraneuronal neuromelanins. The locus coeruleus showed similar but milder changes. The degree of nigral degeneration appeared to be well beyond those which could be seen in usual ALS patients. The question was whether or not this patient might have been in an early stage of the extended form of ALS.
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PMID:[A 49-year-old man with progressive bulbar palsy and respiratory failure]. 949 5

Bulbar palsy is unusual as an initial manifestation of amyotrophic lateral sclerosis (ALS), although common in the advanced stages. In terms of bulbar palsy as a presenting symptom, dysarthria and dysphagia are of common features. Hoarseness, however, is an initial symptom of ALS in only a small number of patients. We report a 43-year-old female with hoarseness due to bilateral vocal cord paralysis as the first manifestation of ALS. Gene analysis revealed a heterozygous missense mutation in the SOD1 gene, which resulted in an amino acid substitution of isoleucine 149 by threonine. Hoarseness can be the initial symptom of ALS. Therefore, in cases of bilateral vocal cord paralysis of unknown etiology, ALS should be taken into consideration.
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PMID:Hoarseness due to bilateral vocal cord paralysis as an initial manifestation of familial amyotrophic lateral sclerosis. 1603 38

A 52-year old woman first noted dysphagia four months before admission followed by dysarthria two months later. She then developed weakness of all limbs and became unable to walk. All these symptoms, associated with tongue atrophy, slowly progressed, leading to the initial clinical impression of a motor neuron disease, although her nerve conduction study and electromyography showed no abnormalities. Her brain MRI with T2 weighted/diffusion weighted image (DWI)/fluid attenuated inversion recovery (FLAIR) revealed a high signal lesion located at dorsal medulla oblongata. She proved positive for anti-aquaporin 4 antibody, which confirmed the diagnosis of neuromyelitis optica spectrum disorders (NMOSD). We conclude that NMOSD may initially present with progressive bulbar palsy and pyramidal tract disorder over a few months, mimicking a motor neuron disease. Awareness of this atypical presentation helps establish an early diagnosis of this treatable entity.
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PMID:[A case of neuromyelitis optica spectrum disorders, with slowly progressive bulbar palsy, mimicking a motor neuron disease]. 3081 45