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Query: UMLS:C0011168 (dysphagia)
 15,644 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We describe a patient with amyotrophic lateral sclerosis with dementia (ALS-D) displaying a long clinical course. A 68-year-old Japanese male with no family history of note was admitted complaining of severe dysarthria and dysphagia. At 63 years old, Pick's disease was diagnosed on the basis of abnormal behavior, such as "Denkfaulheit" and moria, and temporal lobe atrophy observed on magnetic resonance imaging (MRI). Five years after onset, dysarthria and dysphagia emerged, and gradually worsened. On admission, muscular weakness of the upper extremities, fasciculation, and exaggerated tendon stretch reflexes were noted. Needle electromyography performed on the left upper and lower extremities revealed neurogenic pattern changes. Based on these findings and clinical course, ALS-D was diagnosed. Due to severe bulbar palsy, verbal communication was impossible. However, neither specific symptoms of dementia nor abnormal behavior was demonstrated, although this latter had been observed 5 years ago, with only short-term memory impairment apparent. MRI disclosed severe knife-edge atrophy of bilateral temporal lobes, most prominently in the anterior regions. SPECT images revealed decreased uptake of tracer in bilateral inferior temporal lobes, predominantly on the left side. The patient died suddenly 4 months after admission, and post-mortem examination was not conducted. Total clinical course was about 8 years. Several cases of ALS-D have displayed similar clinical courses to the presented case. Some of these would also have initially been diagnosed as Pick's disease. We speculate that cases displaying psychiatric symptoms for several years and initially diagnosed as Pick's disease may finally be diagnosed as ALS-D upon the eventual emergence of motor symptoms(bulbar palsy).
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PMID:[A case of amyotrophic lateral sclerosis with dementia presenting long clinical course]. 1268 97

This report concerns an autopsy case of sporadic amyotrophic lateral sclerosis (ALS) clinically diagnosed as having spinal progressive muscular atrophy (SPMA). The patient was a Japanese woman without hereditary burden. She developed muscle weakness in the distal part of the right upper extremity at age 52, followed by muscle weakness in the left upper extremity and lower extremities at age 54 and 64, respectively. At age 66 she could not walk, even with assistance. Fasciculation and atrophy of the tongue appeared at age 68, followed by dysphagia and dysarthria at age 70. She died of respiratory disturbance at age 71. During the clinical course, neurological examination revealed neither Babinski sign nor hyperreflexia. No respirator administration was performed throughout the clinical course. Neuropathological examination disclosed not only neuronal loss with gliosis in the hypoglossal nucleus and anterior horns of the spinal cord, but also loss of Betz cells and degeneration of the pyramidal tract. Based on these clinicopathological findings and a literature review of sporadic autopsy cases of ALS with long clinical course (10 years or more), including four cases without pyramidal signs, we believe that sporadic ALS of long clinical course mimicking SPMA exists.
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PMID:Sporadic amyotrophic lateral sclerosis of long duration mimicking spinal progressive muscular atrophy exists: additional autopsy case with a clinical course of 19 years. 1548 1

A 50-year-old woman lost about 10 kg of body weight within two months. Thereafter, she developed dysphagia and dysphonia. She visited our hospital and presented with a weak elevation of the soft palate, fasciculation of the tongue, hoarseness of voice, muscle weakness of the neck and extremities, and a decrement in vital capacity. She was admitted with a provisional diagnosis of motor neuron disease. The results of laboratory examinations showed an elevation in serum lysozyme and liquor protein levels, and pleocytosis in the liquor. Needle electromyography showed neurogenic changes, and bilateral hilar lymphadenopathy was revealed by computerized tomography. A biopsy specimen was excised from lymph nodes near the right anterior scalene muscle, which showed noncaseating granulomas consistent with sarcoidosis. All her symptoms improved after steroid administration. Such patients can be treatable with steroids. Moreover, the differential diagnosis from motor neuron disease is important.
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PMID:[A patient with sarcoidosis needed differential diagnosis from motor neuron disease]. 1611 29

We report the case of an unusual presentation of myasthenia gravis with tongue atrophy and fasciculation. Myasthenia gravis is an autoimmune condition associated with weakness and fatigability of voluntary muscles. In >50%, the initial symptoms and signs are related to extraocular muscle weakness, such as diplopia or ptosis [Tsung K, Seggev JS. An unusual cause of dysphagia. West J Med 1995; 163: 159-60]. Rarely, it is known to affect bulbar muscles and can lead to dysphagia and respiratory compromise.
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PMID:Myasthenia gravis--a rare presentation with tongue atrophy and fasciculation. 1636 41

Amyotrophic lateral sclerosis is a progressive degenerative neuromuscular disease of insidious onset. It involves upper and lower motor neurons and causes both spastic and atrophic muscular symptoms. More than one fourth of patients have complaints relating to the head and neck (bulbar palsy); thus, the otolaryngologist may be the first physician to see them. In bulbar forms of Amyotrophic Lateral Sclerosis, voice and/or swallowing difficulties are often the initial signs of disturbance. Predominant symptoms are slurred speech, hoarseness, dysphagia, and dyspnea. Muscular weakness, atrophy, and fasciculation are noted on examination. We show a case and revise bibliography.
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PMID:[Amyotrophic lateral sclerosis that begins with voice and deglution alterations]. 1688 54

We report a rare case of multiple cranial nerve palsy associated with pregnancy, the cause of which remained to be clarified despite of extensive inspections. A 28-year-old woman with type 1 diabetes on insulin therapy developed left oculomotor nerve palsy in the fourth month of pregnancy. Her symptoms improved after one month's administration of vitamins B1 and B12. Numbness appeared in the left side of the face in the 8th month of pregnancy, and then moved to the right side in two weeks. Dysarthria and dysphagia occurred one month later. Bulbar symptoms were worsened, and she became unable to eat or drink. Neurological examination revealed disturbance of sensation in the right side of the face, soft palate paresis, and bilateral atrophy and fasciculation of the tongue. Extensive laboratory examinations including immunological and endocrinological studies, cerebrospinal fluid examination and brain magnetic resonance imaging were unremarkable. These symptoms remitted spontaneously after delivery, and the methylprednisolone pulse therapy accelerated the improvement. Neurological examination done one year after delivery showed complete recovery of the symptoms except for persistent tongue atrophy.
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PMID:[Recurrent multiple cranial nerve palsy in a gravida with type 1 diabetes, that remitted after delivery and with steroid therapy]. 1893 78

We reported the two cases with bulbar-onset ALS showing isolated agraphia without overt dementia and aphasia. Patient 1 was a 69-year-old man and patient 2 was an 81-year-old woman, and both were right-handed. Each patient developed dysarthria as an initial symptom followed by dysphagia, and neurological examinations showed atrophy and fasciculation of the tongue with upper and lower motor-neuron involvement of the extremities. These characteristic features with electromyographic evidence including widespread acute and chronic denervation led to a diagnosis of bulbar-onset ALS. Around 1 year after the onset of ALS, dysarthria was mild enough to allow oral communication enabling the determination that aphasia was absent with well preserved confrontation naming, repetition, reading and comprehension. The patients were polite without abnormal behavior or character change, and their general intelligence was also well preserved with excellent scores on the Mini Mental State Examination (30 and 27 points for patients 1 and 2, respectively) and Frontal Assessment Battery (16 points for each patient). However, spontaneous writing and dictation revealed abundant writing errors characterized by omission of kana letters and paragraphia of kana and kanji letters in both patients. Some syntactic errors were also observed in writing but in spoken language. A letter-number effect on writing errors was observed in patient 1. Copying of letters or words was intact and structure and orientation of written letters was well preserved, indicating the absence of constructional, apraxic or spatial feature of agraphia. Single photon emission computed tomography demonstrated reduced uptake in the bilateral frontotemporal lobes, predominantly in the left hemisphere, with less evident alternation in magnetic resonance imaging. Our results suggest that patients with bulbar-onset ALS may develop isolated agraphia as a single-domain cognitive impairment, preceding the clinical manifestation of aphasia or dementia. We speculate that the main responsible region might be the posterior part of the middle and inferior frontal gyri including Exner's writing center and Broca's area beyond the primary motor cortex.
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PMID:[Bulbar-onset amyotrophic lateral sclerosis (ALS) with isolated agraphia]. 2019 88

Motor neurone disease (MND) is a rapidly progressive neurodegenerative condition. It affects people of all ages, but is more common with increasing age (especially over 50 years) and men are affected twice as often as women. The causes remain unknown, although around 5% of cases have a genetic basis. Survival is usually only three to five years from diagnosis. MND affects both upper and lower motor neurones, with variable contributions. The nerve involvement in MND usually has a focal onset, is asymmetrical, but tends to spread to adjacent regions of the body. If the affected region is in the legs, a common presenting feature is tripping, falls or foot drop. If it is in the arms there may be difficulty with fine tasks such as fastening buttons, or raising an arm, and if the cranial nerves are affected there may be slurring of speech, or difficulty swallowing. Key to the diagnosis is evidence of progression, and this may lead to some delay in considering and also confirming the diagnosis. When examining the patient, evidence of more widespread neuromuscular involvement should be looked for. In a patient with foot drop, and fasciculation of the tongue, MND would be a likely diagnosis. Upper motor neurone involvement may be readily determined by examining the reflexes. Brisk reflexes, in the arms, legs or jaw, in the context of features of lower motor neurone denervation are highly suggestive of MND. Suspicion of MND should lead to referral for a neurology opinion. The most useful investigation is likely to be EMG with nerve conduction studies, and probably MRI scan of relevant areas. Blood tests are arranged to screen for any other causative condition. Riluzole is a disease modifying drug licensed to extend the life of patients with MND. There is no treatment that will reverse, or halt, progression of the disease.
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PMID:GPs have key role in managing motor neurone disease. 2203 11

Motor neuron disease encompasses a group of progressive neurologic disorders that destroy cells responsible for the control of essential muscles. The disorders are characterized by progressive weakness, muscle atrophy and fasciculation, spasticity, dysarthria, dysphagia, and respiratory compromise. A 66-year-old male presented to the emergency department with progressive dyspnea and, ultimately, respiratory failure. In spite of extensive cardiopulmonary evaluation, a cause could not be identified except aging. After complete history taking, physical and neurologic examination, motor neuron disease was suspected at last. Thus, elderly patients with dyspnea in the emergency department could have hidden diseases beyond the normal aging process.
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PMID:A case of motor neuron disease presenting as dyspnea in the emergency department. 2274 95

Dysphagia is a common clinical symptom for an ENT surgeon in his clinic, evaluation of which may not be a great challenge. The objective of this article is to report a rare cause of dysphagia which may be difficult to diagnose unless specifically thought of. This is a report of a case of dysphagia secondary to myasthenia gravis seen in a young lady along with tongue fasciculation in an early onset case unlike the ones reported in the literature where the patients are elderly and more often males in the late phase of the disease. At times, dysphagia may be seen secondary to some rare causes and may mislead the doctor. A carefully taken history and methodical clinical examination will avoid unnecessary invasive procedures and delay in the diagnosis.
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PMID:A rare cause of Dysphagia: a case report. 2275 48


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