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

A 32-year-old male patient with chronic myelocytic leukemia in accelerated phase received a bone marrow allograft from his 42-year-old HLA/MLC-identical sister. He recovered from acute graft-versus-host disease (GVHD) grade III-IV of skin, liver and gut, but chronic GVHD of progressive onset developed. On day 556 post-graft severe thrombocytopenia was resistant to prednisolone, cyclophosphamide and high dose immunoglobulin. Splenectomy was followed by a normalization of platelet counts. The subsequent clinical course was characterized by progressive muscular atrophy and weight loss. Dysphagia, dysarthria, cachexia and ultimately recurrent pneumonic episodes ensued. The cachectic patient developed a highly abnormal breathing pattern with hypoventilation and intermittent apnea requiring mechanical ventilation. Auditory evoked potentials revealed a considerable dysfunction of the brainstem. The patient died on day 1120 post-graft from pneumonia, aggravated by thoracic muscular insufficiency. Postmortem examination revealed diffuse predominantly lymphoid perivascular infiltration in meninges and CNS tissue; proliferation of activated microglial cells expressing the HLA-DR antigen was prominent in the brainstem. These histologic changes are similar to those observed in the CNS in experimental GVHD. We suggest that this case represents the first documentation of CNS involvement in chronic GVHD.
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PMID:Fatal encephalitis in a patient with chronic graft-versus-host disease. 239 Jun 33

Acute mutism with paralysis of the bulbar and facial muscles following discrete bilateral internal capsular infarction is a rare stroke syndrome. We describe a 62 year-old male who suddenly became unable to speak or swallow. The paucity of facial expression and inability to voluntarily move the facial, lingual and pharyngeal muscles were persistent and contrasted with a relatively mild limb paresis which recovered. High resolution CT scan revealed infarcts in the posterior limbs of both internal capsules. It is important to recognise this stroke syndrome because of the permanence of dysarthria and dysphagia associated with it.
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PMID:Persistent mutism and dysphagia of acute onset due to bilateral internal capsule infarction. 239 43

An 81-year-old woman with a 13-year history of hypoparathyroidism developed dysarthria and dysphagia. Cranial computed tomography demonstrated extensive calcification involving the basal ganglia, corona radiata, and deep cerebellar structures. The cerebral small-vessel calcification that occurs in chronic hypoparathyroidism may produce the syndrome of progressive dysarthria and dysphagia.
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PMID:Extensive brain calcification and progressive dysarthria and dysphagia associated with chronic hypoparathyroidism. 239 32

Neuronal intranuclear hyaline inclusion disease (NIHID) has been recognized in 14 patients. It usually occurs in the first and second decades but has been seen in the sixth. Both sexes are affected by this sporadic multisystem degenerative disorder that has involved the central and peripheral nervous systems with fibrillar and granular intranuclear inclusions. NIHID appears to be several variants of a multisystem degenerative disease as illustrated by the combination of a spontaneous, degenerative central and peripheral nervous system disorder with neuronal intranuclear inclusions and severe atherosclerotic coronary artery disease in a 23-year-old white man. Beginning at 11 years of age, this patient had experienced diffuse muscle spasms, dysarthria, dysphagia, tremors, ataxia, oculogyric crises, progressive muscle weakness, and atrophy. At autopsy, neuronal intranuclear hyaline inclusions and neuronal loss were seen in his brain, brainstem, cerebellum, spinal cord, bowel, bladder, and esophagus. These fibrillary and granular Cowdry type A and B intraneuronal inclusions were consistent with the diagnosis of NIHID associated with severe coronary atherosclerosis.
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PMID:Neuronal intranuclear hyaline inclusion disease associated with premature coronary atherosclerosis. 244 45

Patients with amyotrophic lateral sclerosis (ALS), a progressive neurodegenerative disorder, commonly develop bulbar symptoms including dysphagia, dysarthria, and defective airway protection. Otolaryngologists/head and neck surgeons are frequently asked to assist in the management of these problems. We reviewed our experience of 13 surgical procedures in 7 patients with advanced bulbar ALS. In spite of technically good surgery, 3 patients died within 1 week of hospitalization. Four patients appeared to benefit from the surgery. Patients with bulbar ALS are a high risk group because of chronic malnutrition and dehydration, covert aspiration and pneumonitis, and diminished ventilation. A team approach to these problems is stressed. Perioperative morbidity can be reduced with careful preoperative preparation and patient selection.
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PMID:Palliative surgery in patients with bulbar amyotrophic lateral sclerosis. 247 67

The authors report a case of ataxic hemiparesis (dysarthria-clumsy hand syndrome plus crural hemiparesis with homolateral ataxia), secondary to a single cerebellar lacuna (CT scan), in a left rostral para-vermian position in the vicinity of the peduncle and secondary to arterial hypertension. They discuss the nosological aspects of this recently established entity and the origin of particularly severe and persistent dysphagia in their case (cerebellar dysphagia?)
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PMID:[Ataxic hemiparesis due to a cerebellar lacuna]. 251 Apr 50

Clinical and neuropathological studies of a case of pallido-nigro-luysian atrophy with thalamic degeneration and ossification of the posterior longitudinal ligament (OPLL) is reported. The patient was a 72-year-old man, suffering from gait disturbance caused by OPLL for about 3 years. The clinical features were characterized by gradual development of disorientation in place, time and person, memory disturbance, vertical gaze palsy and rigidity of extremities. Dysarthria, dysphagia, bradykinesia, masked face and neck dystonia appeared at the advanced stage of his illness. There was no tremor or other involuntary movements. A clinical diagnosis of parkinsonism was suspected. The main neuropathological findings were neuronal loss and gliosis in globus pallidus, substantia nigra, subthalamic nucleus and thalamus. In addition, neuronal loss of the anterior horn of the cervical spinal cord due to compression by OPLL (C4-C7) was recognized. The neuropathological findings of the present case were consistent with systemic degenerative disorder of the nervous system affecting the pallido-nigro-luysian tract. This rare disorder should be considered in the differential diagnosis of parkinsonism in old people.
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PMID:[An autopsy case of pallido-nigro-luysian atrophy associated with OPLL]. 251 8

A 42-year-old woman suffered unexplained weight loss followed by action tremor and difficulty initiating gait. Three months after onset of symptoms, infiltrating ductal carcinoma of the breast, metastatic to liver and lymph nodes, was diagnosed and treated briefly with cyclophosphamide, methotrexate, and 5-flourouracil (5FU). Severe symmetric action and postural tremor with a myoclonic component developed, with minimal rest tremor, severe dysarthria and dysphagia, small-stepped and slightly ataxic gait progressing to a bedbound state, and severe widespread dystonic posturing. The latter began as a typical parkinsonian posture of trunk and upper extremities and progressed to a fixed and painful flexion of the elbows and wrists and extension of fingers and neck. Sinemet, anticholinergics, baclofen, diazepam, and plasmapheresis gave no benefit. The patient died of complications of immobility 5 months after neurologic symptom onset. Autopsy revealed many pigment-laden macrophages in substantia nigra and moderate loss of pigmented neurons. Inflammation, Lewy bodies, and tumor were absent. Cerebellar Purkinje cells were moderately depleted. Mild neuronal loss and gliosis were present in globus pallidus and cerebellar cortex. Stains for anti-human IgG, IgM, kappa, and lambda were negative. This, to our knowledge, is the first report of paraneoplastic degeneration of substantia nigra or paraneoplastic parkinsonism.
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PMID:Paraneoplastic degeneration of the substantia nigra with dystonia and parkinsonism. 254 19

A 64-year-old male patient of amyotrophic lateral sclerosis (ALS) with frozen gait, axial rigidity and supranuclear upper gaze palsy was reported. We have followed this patient more than four years. He was well until November 1982, when he noticed weakness of left arm. In March 1983, he noticed hypogeusia and in July, he developed dysarthria and frozen gait. On admission, he was alert and oriented. Neurological examination revealed dysarthria, dysphagia and muscular weakness and atrophy in bilateral upper extremities, dominantly in left side. He showed remarkable frozen gait, retropulsion and could not walk. Brain CT showed mild dilatation of the third ventricle. In August 1988, he showed tongue atrophy, and weakness and atrophy of the extremities progressed during these four years. He also showed axial rigidity and frozen gait. Brain CT showed severe third ventricular dilatation and atrophy of tegmentum of the midbrain and cerebellum that were compatible with progressive supranuclear palsy (PSP). Six months later, he developed upper gaze palsy. From these findings, we concluded that this patient had a quite unique clinical features of both ALS and PSP.
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PMID:[A case of amyotrophic lateral sclerosis associated with clinical features of progressive supranuclear palsy]. 259 46

A 57-year-old woman suffered from polyarthralgia for 7 years, and was treated by using NSAID with the diagnosis of RA. From Jan. 20th 1987, she complained of back pain and numbness of both hands, and from May 7th 1987, she also complained of dysphagia and dysarthria, and she was not able to button up. Soon afterwards she could eat only one custard pudding a day, so she admitted to our hospital on March 17th 1987. The neurological examination showed hyporeflexia and muscle weakness of the four extremities; and hypesthesia of the 7th to 11th intercostal nerve area and both lateral sides of the dorsum pedis. The laboratory examination showed ESR 17 mm/h, gamma-glob 1.66 g/dl, CRP(+), RAHA 80 x, CH50 24.0 U/dl, HLA-antigen; DR 4(+). Cerebrospinal fluid examination showed cell 5/mm3, protein 63 mg/dl, IgG 13 mg/dl, IgG% 20.6%. X-ray examination indicated destruction of both wrists, left elbow, right 2-5th MTP, and left 5th MTP joints. A light microscopic examination of the left sural nerve showed perivascular infiltration with lymphocyte, occasional macrophages and giant cells at the epineurium, and no demyelination or Wallerian degeneration at the nerve fiber. These histological findings were the same as type-I arteritis in nerves in RA proposed by D.L. Conn. Clinical improvement was obtained after administration of prednisolone 30-60 mg/day.
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PMID:[A case of rheumatoid arthritis associated with polyneuritis]. 266 32


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