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

A new diagnosis identifying a nonorganic basis of dysphagia is proposed in relation to behavioral conditioning processes and thereby labeled "conditioned dysphagia" (CD). Literature review of both animal and human studies documents that rapidly acquired and persistent avoidance responses are learned when autonomic functions are contiguous with traumatic physical events. Three case studies with primary diagnoses of TE fistula, congenital heart disease and pseudobulbar palsy demonstrated an association between CD and early childhood incidence of trauma involving the esophagus. Successful treatment was achieved by operant deconditioning therapy. Results of a pilot epidemiological study of 28 postpharyngeal flap surgery children and 47 controls showed significantly higher incidence of mild to moderate CD in the surgery group.
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PMID:Traumatically acquired conditioned dysphagia in children. 68 94

A 68-year-old right-handed woman was admitted to Tokyo Metropolitan Geriatric Hospital because of slowly progressive dysarthria and writing disability over 2-year period. On admission, severe dysarthria was observed, but no dysphagia. The dysarthria mostly resembled a type of pseudobulbar palsy, although it was associated with effortful speech production. An oro-facial apraxia was also found. She could name objects, and could understand spoken words correctly. Examination using the Western Aphasia Battery showed diminution of word fluency, impaired repetition and perseveration and writing errors. On the Wechsler Adult Intelligence Scale-R verbal IQ was 100 and performance IQ was 87. These scores did not suggest any significant degree of general intellectual deterioration. Wisconsin card sorting test disclosed mild frontal dysfunction. Magnetic resonance imaging showed cortical atrophy in the bilateral frontal and temporal lobes. Measurements of regional cerebral metabolic rate by 18F-FDG-PET demonstrated decreased uptake in the latero-dorso-inferior area of the bilateral frontal lobes, especially on the left side. The present case showed slowly progressive dysarthria and progressive aphasia without generalized dementia, and without typical aphasia. These symptoms are speculated to be related to the atrophy in the bilateral frontal and temporal lobes shown by MRI and the decreased metabolic rate in the left dominant bilateral frontal lobes on PET study. The pathologic process responsible for these lesions remains obscure.
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PMID:[Slowly progressive dysarthria and impaired language function--a case report]. 128 97

A patient of mutism with pseudobulbar palsy and frontal lobe syndrome resulting from lacunar state was reported. The patient, a 64-year-old man, was admitted to Gifu University Hospital because of a decrease in spontaneous activity, lack of volition and anorexia. The CT scan, performed on July 29, 1987, demonstrated lacune in the right internal capsule (IC), periventricular lucency especially around the anterior horn of lateral ventricles, and ventricular dilatation. He was transferred to a medical ward because of repeated aspiration pneumonia. Neurological examination revealed mutism, pseudobulbar palsy, and frontal lobe signs. The CT scan, performed on March 30 1988, demonstrated the newly developed lacune in the left IC. The MRI also showed two coinciding lacunes, one in the genu of the right IC and the other in the anterior limb of the left IC. The SPECT with 123I iodoamphetamine showed decreased blood supply predominantly to the frontal lobes. A mechanism by which the mutism occurs is discussed from two points of view, pseudobulbar palsy and frontal lobe syndrome. He developed initially frontal lobe syndrome in which paucity of spontaneous speech was noted. The CT scan at that time demonstrated lacune in the right IC. About eight months later when he became mute, the CT scan showed lacunes in bilateral ICs without any other low density areas in frontal language areas such as Broca's area, subcortical area and supplementary motor area. As the MRI showed that the right lacune was in the genu but the left lacune was in the anterior limb of IC, the left cortico-bulbar tract was thought to be not directly involved. The SPECT showed decreased blood supply predominantly to the frontal lobes. Although dysphagia improved, mutism did not improve at all. Therefore it is postulated that both pseudobulbar palsy and frontal lobe dysfunction might play a role in producing the mutism of this patient.
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PMID:[Capsular pseudobulbar mutism in a patient of lacunar state]. 236 32

Reviewing the literature since recognition of progressive supranuclear palsy (PSP) as a clinicopathological entity 20 years ago, the present state of knowledge is delineated. The etiology of PSP is still unknown. The clinical hallmarks are supranuclear palsy of vertical gaze, axial dystonia in extension and pseudobulbar palsy with marked dysarthria and dysphagia. Accessory features include subcortical dementia, mental, extrapyramidal, pyramidal and cerebellar symptoms. PSP is a disease of the presenium (average age at onset, 59.6 years) with a male preponderance (60% men). The onset is insidious with vague complaints of dysequilibrium (60%), mental changes (46%) and disturbed vision (21%), often preceding abnormal neurological findings. The important borderland and main differential diagnosis is parkinsonism. However, in PSP, responsiveness to antiparkinsonian agents is poor and progression is rapid and fatal within few years (average survival time, 5.7 years). Promising diagnostic tools at present include CT-scanning and neuro-otologic and -ophthalmologic examination. Neuropathological findings, confined to specific diencephalic, brainstem and cerebellar nuclei, include neurofibrillary tangles (ultrastructurally different from those seen in other CNS disorders), neuron loss and gliosis. The importance of research on neurocytochemistry, brain ultrastructure and immunology in the current investigation of PSP is outlined.
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PMID:Progressive supranuclear palsy--20 years later. 399 25

An 18-year-old girl died following a slowly progressive neurodegenerative disease of nine years duration. At 9 years of age, she developed intellectual deterioration associated with speech difficulty, pseudobulbar palsy and ataxia. The progression included spastic quadriplegia, anarthria, severe dysphagia, ophthalmoplegia, and pes cavus. There was no family history. The brain was uniformly small and the substantia nigra was not pigmented. Neuronal loss and gliosis involving globus pallidus, subthalamic nucleus, thalamic nuclei, brain stem, cerebellum, and spinal cord gave the picture of multisystem atrophy. Intranuclear hyaline inclusions were observed in numerous neurons of the central and peripheral nervous system. These were auto-fluorescent and were made up of intermingled straight filaments (8-9 nm in diameter). Only two previously reported cases showing these same inclusions are known. They are reviewed, compared, and discussed in relation to primary neuronal degenerations.
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PMID:Multiple system atrophy with neuronal intranuclear hyaline inclusions. Report of a new case with light and electron microscopic studies. 626 27

Cricopharyngeal myotomy has been used in the treatment of pharyngoesophageal diverticulum and various neurogenic, myogenic and myoneurogenic disorders. An appreciable number of patients with pseudobulbar palsy due to cerebrovascular accidents and patients with idiopathic hypertrophy of the cricopharyngeal muscle will greatly benefit from this procedure. This paper describes the indications for, and results of, 20 consecutive cricopharyngeal myotomies carried out with or without diverticulectomy. All 20 patients experienced cervical esophageal dysphagia and 55% had substantial weight loss. The most valuable investigation is roentgenography of the pharynx and esophagus, which will confirm megapharynx, hypopharyngeal stasis, weak or absent pharyngeal contractions and regurgitation. Hypertrophic cricopharyngeal muscle was demonstrated in 9 of the 20 patients. The diagnostic value of endoscopy and esophageal manometry is limited. The results were considered excellent in all patients with pharyngoesophageal diverticulum and idiopathic hypertrophy of the cricopharyngeal muscle. Marked symptomatic and objective improvement was achieved in patients with cerebrovascular accidents, vagal injuries and amyotrophic lateral sclerosis. However, the result was poor in patients with myoneurogenic disorders.
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PMID:Cricopharyngeal myotomy. 629 86

A 26-year-old housewife, born of consanguineous parentage, began to have gait and speech disturbance. Her brother had died from suffocation because of dysphagia. At thirty-two, she developed difficulty in swallowing, clumsiness and incontinence. When she was thirty-six she had pseudobulbar palsy, vertical gaze paresis, hyperreflexia and muscular atrophy of the upper half of the body. CT scan showed cerebral atrophy. Her mental function progressively deteriorated and amyotrophic lateral sclerosis associated with dementia was suspected. She died at the age of thirty-seven. Diagnosis was made only by autopsy. There was no particular general pathologic finding excepting aspiration pneumonia. Microscopical examination revealed numerous distended neurons with accumulation of light brown pigments by Luxol fast blue/H & E stains, especially in hypothalamus, substantia nigra and nuclei of oculomotor nerves. To a lesser extent such neurons were noted ubiquitously. The stored material was mainly composed of lipofuscin and ceroid. Ultrastructurally they presented the various structures which have previously been reported, except for finger print profiles. The pigmentary deposits were shown to be immunoreactive with polyclonal antibody directed against amyloid beta-protein.
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PMID:[Dementia and amyotrophy in Kufs disease. The adult type of neuronal ceroid lipofuscinosis]. 774 8

A 28-year-old, previously healthy, normotensive woman suddenly developed an acute pseudobulbar palsy with dysarthria, dysphagia, hypernasal voice and mild right arm paresis. Extensive laboratory investigations excluded all other possible causes of acute pseudobulbar palsy (neoplastic, inflammatory, demyelinative, myasthenic) and an MRI study demonstrated bilateral isolated thalamic infarcts. Oral contraceptives and smoking were the only possible stroke risk factors found and cerebral diaschisis the most tenuous explanation proposed. To our knowledge, this is the first report indicating that bilateral thalamic infarction on specific nuclei could be manifested as acute pseudobulbar palsy.
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PMID:Bilateral thalamic infarcts presenting as acute pseudobulbar palsy. 775 Aug 10

The potential causes of neurogenic oropharyngeal dysphagia in cases in which the underlying neurologic disorder is not readily apparent are discussed. The most common basis for unexplained neurogenic dysphagia may be cerebrovascular disease in the form of either confluent periventricular infarcts or small, discrete brainstem stroke, which may be invisible by magnetic resonance imaging. The diagnosis of occult stroke causing pharyngeal dysphagia should not be overlooked, because this diagnosis carries important treatment implications. Motor neuron disease producing bulbar palsy, pseudobulbar palsy, or a combination of the two can present as gradually progressive dysphagia and dysarthria with little if any limb involvement. Myopathies, especially polymyositis, and myasthenia gravis are potentially treatable disorders that must be considered. A variety of medications may cause or exacerbate neurogenic dysphagia. Psychiatric disorders can masquerade as swallowing apraxia. The basis for unexplained neurogenic dysphagia can best be elucidated by methodical evaluation including careful history, neurologic examination, videofluoroscopy of swallowing, blood studies (CBC, chemistry panel, creatine kinase, B12, thyroid screening, and anti-acetylcholine receptor antibodies), electromyography, and magnetic resonance imaging (MRI) of the brain, plus additional procedures such as lumbar puncture and muscle biopsy as indicated. Little is known about aging and neurogenic dysphagia, specifically the relative contributions of natural age-related changes in the oropharynx and of diseases of the elderly, including periventricular MRI abnormalities, in producing dysphagia symptoms and videofluoroscopic abnormalities in this population.
Dysphagia 1994
PMID:Neurogenic dysphagia: what is the cause when the cause is not obvious? 780 24

Foix-Chavany-Marie Syndrome or anterior opercular syndrome constitutes the cortical form of pseudobulbar palsy. Its most common etiology is stroke in the region of either operculum. Clinically it can be characterized by severe dysarthria and facio-pharyngo-glosso-masticatory dysplasia with automatic-voluntary dissociation. An inversion of this classical dissociation can be seen in patients with emotional paralysis in the face, in whom asymmetry of emotional expression but conservation of voluntary movement can be seen. The lesional topography of this inverse dissociation corresponds to the supplementary motor area. both processes support the existence of an anatomical base which is different for emotional movements and voluntary types. We describe the clinical case of a 65 year old woman with a history of breast cancer who presented a subacute and progressive clinical picture of dysphagia and severe dysarthria. Upon neurological examination slight velo-palato-pharyngea was noticed; most relevant was paralysis of the facial muscles for either emotional or automatic movements while voluntary innervation was maintained. Neuroradiological studies show the presence of brain metastasis located in both Roland opercula. Although the neuroanatomic bases for automatic-voluntary dissociation remain unclear, our case lends weight to the evidence that impulses for voluntary and emotional movements originate in different cortical areas or else take a different route through the brain. We have been unable to find any cases of biopercular lesion with inverse dissociation described in the literature we reviewed.
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PMID:[Biopercular lesion with inverse dissociation]. 855 92


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