Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0011168 (dysphagia)
 15,644 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We report a 63-year-old right-handed Japanese man with progressive bulbar dysfunction and alexia of kanji (Japanese morphograms). He was well until his 62 years of age, when he noted difficulty of reading kanji, which was followed by disturbances in his speech. Reading of kana (Japanese phonograms) was preserved. He also showed naming difficulties with semantic memory loss for words, which were characterized for word meaning aphasia or semantic dementia. He showed dysarthria and mild dysphagia with atrophy and fasciculations of the tongue. The electromyographic studies disclosed diffuse neurogenic pattern. He was diagnosed as having bulbar type amyotrophic lateral sclerosis. Cranial magnetic resonance imaging and single-photon emission computed tomography revealed bilateral involvements of the temporal lobes. Our patient appeared to meet the clinical criteria for frontotemporal degeneration of motor neuron disease type, and is the first case of amyotrophic lateral sclerosis showing alexia of kanji and word meaning aphasia.
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PMID:[Amyotrophic lateral sclerosis presented with alexia of kanji and word meaning aphasia]. 1247 80

We report a Japanese autopsy case of progressive supranuclear palsy (PSP). The male patient was 74 years old at the time of death. At age 64, he developed non-fluent aphasia that progressed slowly over 8 years, eventually associated with behavioral abnormality, postural instability, and dysphagia at 2 years prior to his death. Magnetic resonance imaging of the brain at age 73 demonstrated marked atrophy of the frontal lobes, particularly on the left side. Neuropathological examination revealed the typical pathology of PSP: loss of neurons, gliosis, occurrence of neurofibrillary tangles, oligodendroglial coiled bodies, and tuft-shaped astrocytes in the frontal cortex, associated with argyrophilic threads in the underlying white matter, in the basal ganglia, including the thalamus, globus pallidus, and subthalamic nucleus, and in the brainstem nuclei, including the substantia nigra, pontine nucleus, and inferior olivary nucleus. No astrocytic plaques or ballooned neurons were observed. Protein analysis revealed accumulation of hyperphosphorylated tau of 68 and 64 kDa consisting of the four repeat tau isoforms. We conclude that the present case represented PSP with an 8-year history of primary progressive aphasia (PPA). Although focal cortical symptoms in PSP are rare or absent, we should keep in mind the possibility of atypical PSP in which cortical pathology is predominant, particularly in the frontal lobe, and could result in PPA.
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PMID:Progressive supranuclear palsy presenting with primary progressive aphasia--clinicopathological report of an autopsy case. 1266 38

A 61-year-old man had been treated for malignant fibrous histiocytoma with the pulmonary and the lymph node metastasis in the department of orthopedics in our hospital. He was admitted to our department because of an acute onset of conscious disturbance and non-fluent aphasia. Diffusion-weighted imaging (DWI) showed high signal intensity areas in the bilateral cerebella, thalami and posterior lobes. T2WI did not show any mass effects. Enhanced CT did not reveal any enhanced lesion. He was diagnosed as having cerebral embolism, and his conscious disturbance was improved after medication. Eight weeks later, he presented dysphagia, dysarthria, and ataxia in his extremities. DWI showed multiple lesions of low signal intensity located at the identical place where had showed high signal intensity in the initial DWI. T2WI showed high signal intensity area with mass effect. It was indicated that cerebral metastasis might grow after tumorgenic embolism. This is a rare case that tumor emboluses were developed to the metastatic brain tumors.
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PMID:[A case of malignant fibrous histiocytoma with metastatic brain tumors after tumorgenic embolism]. 1538 7

A 35-year-old female ingested a lethal dose of potassium cyanide in a suicide attempt. She survived following antidote therapy and intensive care. Following artificial coma she presented with an agitative state for several days followed by akinetic mutism, buccofacial and ideomotoric aphasia. Severe rigid-akinetic syndrome, dysarthria, dysphagia and generalized dystonia developed weeks later. MRI revealed lesions in the caudate and lentiform nuclei, precentral cortex, and cerebellum. SPECT by [123-I] 2 beta-carbomethoxy-3-beta-(4-iodophenyl)-Tropan on two occasions revealed progressive loss of dopamine transporter suggestive of nigral neuronal apoptosis. Striatal and frontal hypometabolism and hypoperfusion were found by FDG-PET and HMPAO SPECT.
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PMID:Cyanide-induced akinetic rigid syndrome: clinical, MRI, FDG-PET, beta-CIT and HMPAO SPECT findings. 1573 73

We report a 65-year-old man with rigid-bradykinetic parkinsonism, vertical gaze palsy, difficulty in eye-lid opening, and marked pseudo-bulbar palsy. He felt difficulty of it, hand movement at 59 years old. When he was 60 years old, monotonous speech and slowness of movement appeared. He visited a neurologist who noted vertical gaze palsy, neck rigidity, and bradykinesia. He was diagnosed as progressive supranuclear palsy (PSP) and given 300 mg L-Dopa/Benserazide by the neurologist. This medication improved his rigidity and bradykinesia. At 62 years of the age, his eye-lids closed involuntary and it was difficult to open. In addition, he began to complain of wearing-off, autonomic symptoms, and dysphagia. Anti-parkinsonian drugs were increased, but his bradykinesia progressed. At 64 years of the age, he was admitted to the Neurology Service of Juntendo Hospital. On admission, he was alert and not demented. No aphasia, apraxia, or agnosia was noted. In the cranial nerves, upward and downward gaze were markedly restricted. His face was hypomimic and seborrhoic. It was difficult to swallow liquid or solid for him. No weakness was noted, but he walked in small steps with freezing and falling tendency to backward. Rigidity was noted on his extremities and stronger on his left side than right. Tremor was absent. Bradykinesia of his body and extremities was marked. No cerebellar ataxia was noted. Deep tendon reflexes were within normal range. Planter response was flexor bilaterally. Myerson's sign was noted. Sensory and autonomic function were normal. He was treated with L-Dopa, Pergolide, and Bromocriptine. However, these medications improved his bradykinesia and gait disturbance only slightly, dysphagia became progressively worse. He developed aspiration pneumonia when he was 65 years old and admitted to Juntendo Hospital. A large amount of sputum was aspirated from his trachea. Two days after from admission, he was found dead on his bed. He was discussed in a neurological CPC and the chief discussant arrived at a conclusion that the patient had progressive supranuclear palsy (PSP). Other differential diagnoses included Parkinson's disease, pallido-nigroluysian atrophy (PNLA), multiple system atrophy (MSA), and corticobasal degeneration(CBD). Many participants considered that PSP or PNLA was most likely. Post-mortem exmination revealed marked nigral neuronal loss and gliosis. The globus pallidus and the luysian body changed mildly. However, the frontal cortex was relatively spared, there were many ballooned neurons in the cortical layer. Other parts were spared. With sliver (Bodian and Gallyas-Braak) and anti-phsphorylated tau stain, abundant astrocytic plaques, neurofibrillary tangles, and argyrophilic threads on the frontal cortex, striatum, and substantia nigra were seen. There was no tufted astrocyte which was hallmark of diagnosis of PSP. In addition, several Lewy bodies were seen in the brainstem. Because astrocyte plaque was considered specific for pathology of CBD, the pathologist revealed that the pathological diagnosis of this patient was CBD. Nevertheless, discussion was focused on the relatively mild degeneration of the frontal cortex for CBD.
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PMID:[A 65-year-old man with rigid-bradykinetic parkinsonism, vertical gaze palsy, difficulty of eye-lid opening, and marked pseudo-bulbar palsy]. 1578 4

Progressing stroke with neurological deterioration (PSND), i.e., neurological deterioration of patients during the first days following a stroke, although not an infrequent event, has hitherto been addressed only by few studies. This is the first investigation conducted in Israel with the aim to determine its prevalence and characteristics. Data regarding 140 patients with first ever stroke were collected prospectively between May 1999 and October 2000. All patients underwent a thorough daily neurological examination over the first 7 days, using the Canadian Neurological Scale. Most (90%), patients had hemiparesis, with dysarthria, aphasia and dysphagia being the most frequent associated neurological deficits. Thirty percent of the patients were on anti-aggregant therapy prior to the stroke. The prevalence of PSND was 23%. The 1-month in-hospital death rate of these PSND patients was 31%. Univariate analysis showed that previous anti-aggregant therapy, Parkinson's disease (PD), obesity, hyperlipidemia, and presence of aphasia were significantly more frequent in the PSND group. In addition, these patients arrived earlier to the emergency room. However, logistic regression analysis showed that only PD and obesity could possibly be considered as predictors for development of PSND. The prevalence as well as the death rate of PSND in this group of Israeli elderly is within the range reported in the literature. However, prior anti-aggregant treatment and PD, are here reported for the first time as associated conditions. Future research will possibly clarify the links between these entities and PSND.
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PMID:Progressing stroke with neurological deterioration in a group of Israeli elderly. 1591 Oct 42

Patients with primary malignant brain tumor experience deterioration of multi-focal neurological deficits such as hemiparesis, aphasia, visual field defects, dysphagia, and disturbance of recent memory at the advanced stage of disease. With these advancing neurological deficits, many patients will inevitably prepare for death and may experience psychological and spiritual distress. Active listening is an important skill to explore the fears of patients with a terminal illness but in the advanced stage of a primary brain tumor, patients usually have great difficulty with verbal expression. Even if patients do not suffer from complete expressive aphasia, they often have difficulty verbalizing their thoughts and feelings. Sadly, disturbance of vocal expression is a common accompaniment of this pathology. Unless the pathophysiology is understood, an observer may fail to comprehend the patient's non-verbal communication. Seeking to understand these issues is a prerequisite of the preservation of dignity and provision of ethical care for such patients.
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PMID:Care for dying patients with primary malignant brain tumor. 1637 57

Corticobasal degeneration (CBD) is a rare sporadic 4-repeat tauopathy. We report here the first Polish case of pathologically proven CBD. Our patient developed clumsiness of the right hand at age 63 years. During the course of his illness he suffered from progressive asymmetric parkinsonism unresponsive to dopaminergic therapy. Focal dystonia affecting right upper extremity, non-fluent aphasia, dysphagia, supranuclear vertical gaze palsy, imbalance and myoclonus ensued. The patient died of pneumonia at age 71 years. Head magnetic resonance imaging revealed the presence of asymmetric cortical atrophy contralateral to the clinically more affected right side. Median somatosensory evoked potentials performed bilaterally demonstrated significant reduction of cortical evoked potential amplitudes recorded from the left scalp electrodes. Neuropathological examination showed cortical atrophy of the frontal and parietal lobes with superficial spongiosis and diffuse cortical gliosis. Numerous ballooned neurons were found in frontal and parietal cortices. The most remarkable pathology was extensive tau-immunoreactivity of glial and neuronal cell processes, significantly pronounced in the frontotemporal cortex, basal ganglia, thalamus and brainstem. Recent research studies have resulted in better clinical, pathological and genetic characterization of sporadic tauopathies. It is hoped that similar progress will ensue in the development of symptomatic and eventually curative treatments for these rare conditions.
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PMID:Corticobasal degeneration -- clinico-pathological considerations. 1718 52

The Canadian Stroke Network Consensus Conference panel met in 2003 in Toronto, Canada, to address areas of stroke rehabilitation that require additional research as well as increased efforts to knowledge translation. The results of an extensive literature review, of a study of factors related to poststroke quality of life, and a survey of clinicians were presented to the panel. From this review, the panel compiled a consensus list of 5 priority areas in stroke rehabilitation research that warrant further investigation. The priorities are: (1) multimodal programs for reintegration into the community; (2) rehabilitation of patients with severe strokes; (3) the ideal timing and intensity of aphasia therapy; (4) cognitive rehabilitation; and (5) and the timing and intensity of rehabilitation after mild-to-moderate stroke. The panel recommended that agencies that fund research create special competitions to support large, multicenter randomized controlled studies to investigate these areas of research. In addition, the panel identified 3 priority areas for knowledge translation where research was convincing: (1) lower-extremity (leg) interventions; (2) upper-extremity (arm) interventions; and (3) detection of clients who are at risk of complications, specifically depression, dysphagia, or cognitive impairment, as well as those at risk for falls and pressure ulcers.
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PMID:Priorities for stroke rehabilitation and research: results of a 2003 Canadian Stroke Network consensus conference. 1739 56

Pathological investigation of progressive anterior operculum syndrome has rarely been reported. We describe clinico-pathological findings in a patient with progressive anterior operculum syndrome. A 74-year-old right-handed man had noticed speech and swallowing difficulties 1 year previously. Neurological examinations showed no abnormality other than a slight limitation of upward gaze and slow tongue movement without fibrillation. We investigated the patient using neuroimaging and neuropsychological examinations and observed him for 2 years until his death, at which point we obtained pathological findings. The patient's facial and masseteric muscles seemed hypotonic with drooling, but he could laugh and yawn normally, showing automatic voluntary dissociation. Palatal and pharyngeal reflexes were normal. Magnetic resonance imaging showed cortical atrophy in the temporal lobes bilaterally. (123)IMP single photon emission computed tomography and positron emission tomography showed decreased blood flow and activity in the frontotemporal lobes, predominantly on the left side. Neuropsychological examinations showed no aphasia, dementia or other neuropsychological abnormality. Intubation fiberscopy, laryngoscopy and video fluorography showed no abnormality. After 6 months his anarthria and dysphagia became aggravated. He died of aspiration pneumonia 2 years after onset. Postmortem examination revealed neuronal degeneration with TDP-43-positive inclusions in the frontal, temporal and insular cortices, consistent with frontotemporal lobar degeneration with TDP inclusions (FTLD-TDP). However, neuronal loss with gliosis was more prominent in the inferior part of the motor cortices, bilaterally. Progressive anterior operculum syndrome could be classified as a variant of FTLD-TDP.
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PMID:Progressive anterior operculum syndrome due to FTLD-TDP: a clinico-pathological investigation. 2017 96


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