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 describe a 58-year-old patient with a rare predominantly venous vascular malformation of the medulla oblongata, which caused a progressive bulbar dysfunction consisting of hiccoughs, dysphagia, hoarseness, dysarthria, gait ataxia and dysuria over a period of 11 months. On autopsy, a large dilated vein with focal marked intimal fibroelastic thickening was present on the ventral surface of the medulla. Microscopically, moderate proliferation of capillaries and veins was observed which was confined primarily to the medulla. The veins displayed abnormal dilatation and tortuosity; prominent thickening of vessel walls was also present in the veins and capillaries. The venous abnormalities were prominent in the parenchyma of the medulla, but much less apparent in its subarachnoid space. Multifocal neuronal loss and gliosis were observed, most prominently in the inferior olives, hypoglossal, dorsal vagal and ambiguus nuclei. The histopathologic findings suggested that abnormal venous drainage within the parenchyma of the medulla was the most critical factor for the pathogenesis of this patient's neurologic symptomatology.
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PMID:Progressive bulbar dysfunction caused by a predominantly venous vascular malformation of the medulla oblongata. 161 26

Two patients with chronic granulomatous disease had obstructive lesions of the gastrointestinal tract, esophagus, and genitourinary tract, which were successfully treated with corticosteroids. These obstructive lesions, caused by local granuloma formation, have been reported in 18 other patients with chronic granulomatous disease, none of whom received steroids. Our first patient, a 3-year-old boy, had emesis and weight loss associated with antral narrowing and delayed gastric emptying at age 2 years. Antibiotic therapy was ineffective, but intravenous and oral corticosteroid therapy for 10 weeks resulted in clinical cure. One year later, dysuria associated with bladder neck obstruction was also treated successfully with corticosteroids. The second child, a 10-year-old boy, had dysphagia caused by distal esophageal stenosis. Corticosteroid therapy (with concomitant antibiotics) on two occasions reversed this obstruction. Granulomatous cystitis with ureteropelvic obstruction then developed, which also responded to treatment with corticosteroids and antibiotics. Despite the risk of increased susceptibility to infection, corticosteroid therapy is justified in preventing life-threatening obstruction of vital organs.
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PMID:Corticosteroids in treatment of obstructive lesions of chronic granulomatous disease. 362 1

We report a 62-year-old man with a pelvic mass, who developed multiple cranial nerve palsies on the right side. He was well until the summer of 1977 when he developed a numb sensation in the sacral region. In the next year, a huge tumor was found in the sacral area in another hospital. Most of the tumor was resected at that time. Post-operative course was uneventful. In July 1988, there was an onset of weakness in his legs, gait disturbance, and dysuria. Myelography at the above hospital revealed a complete block at the seventh thoracic level. He was treated by laminectomy and post-operative radiation. In June 1990, he developed a neuralgic pan in his right leg. Two months later, he noted diplopia, deafness in his right ear, and swallowing difficulty. He was admitted to our hospital for further work up on January 14th of 1991. On admission, he was afebrile. General physical examination revealed a 4 cm had mass in his right anterior chest attaching the rib. Gynecomastia was noted bilaterally. Liver was felt by 5 cms under the right hypochondrium. The edge of the liver was firm. On neurologic examination he was an alert and mentally sound man. His higher cerebral functions were intact. In the cranial nerves, complete palsy of the abducens nerve, mild nerve deafness, paresis of the soft palate, atrophy and weakness of the sternocleidomastoid and upper trapezium muscles, all on the right side, deviation of the tongue to the right, slurred speech, and dysphagia were observed. The neck was supple. He was able to walk with a support. Mild weakness was present in his right lower extremity. Both legs were spastic. No ataxia or involuntary movements were noted. Deep reflexes were symmetric and normally active. No sensory loss was observed. No meningeal signs were present. Pertinent laboratory findings included moderate anemia (Hb 8.8 g/dl), LDH 2,631 U/l, CRP 7.4 mg/dl. The CSF was under an increased pressure (OP 260 mmH2O) containing 2 lymphocytes/ml, 43 mg/dl of protein, and 49 mg/dl of glucose. Radiologic examinations revealed a destructive change in the sacrum, lytic lesions in the seventh thoracic spine and in the clivus.(ABSTRACT TRUNCATED AT 400 WORDS)
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PMID:[A 62-year-old man with multiple cranial nerve palsies on the right side and a pelvic mass]. 821 5

Cantharidin, known popularly as Spanish fly, has been used for millennia as a sexual stimulant. The chemical is derived from blister beetles and is notable for its vesicant properties. While most commonly available preparations of Spanish fly contain cantharidin in negligible amounts, if at all, the chemical is available illicitly in concentrations capable of causing severe toxicity. Symptoms of cantharidin poisoning include burning of the mouth, dysphagia, nausea, hematemesis, gross hematuria, and dysuria. Mucosal erosion and hemorrhage is seen in the upper gastrointestinal (GI) tract. Renal dysfunction is common and related to acute tubular necrosis and glomerular destruction. Priapism, seizures, and cardiac abnormalities are less commonly seen. We report four cases of cantharidin poisoning presenting to our emergency department with complaints of dysuria and dark urine. Three patients had abdominal pain, one had flank pain, and the one woman had vaginal bleeding. Three had hematuria and two had occult rectal bleeding. Low-grade disseminated intravascular coagulation, not previously associated with cantharidin poisoning, was noted in two patients. Management of cantharidin poisoning is supportive. Given the widespread availability of Spanish fly, its reputation as an aphrodisiac, and the fact that ingestion is frequently unwitting, cantharidin poisoning may be a more common cause of morbidity than is generally recognized. Cantharidin poisoning should be suspected in any patient presenting with unexplained hematuria or with GI hemorrhage associated with diffuse injury of the upper GI tract.
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PMID:Poisoning from "Spanish fly" (cantharidin). 876 16

A 5-year-old boy developed hemorrhagic mucocutaneous blisters on various parts of the body leading to fetor, dysphagia, dysuria, anal pruritus, pain on defecation, and weight loss. The histopathology showed the classic features of pemphigus vulgaris, and direct immunofluorescence showed intercellular deposition of IgG and C3 in the epidermis. Circulating pemphigus antibodies were also detected. He was treated with a combination of systemic prednisone and dapsone which induced a rapid remission and controlled the disease well. He has been in remission for 1 year and 7 months with no immunosuppressive therapy except for the use of topical agents for the oral lesions. An adjuvant to corticosteroids has been used only once before in children with pemphigus vulgaris under the age of 12 years. This is the third and the youngest child in the literature treated in this fashion.
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PMID:Childhood pemphigus vulgaris treated with dapsone: a case report. 979 90

We report a 63-year-old man who died of respiratory failure. He was well until 1992 (57 years of his age), when he had an onset of progressive weakness of the bilateral upper limbs. He showed no improvement with TRH administration in other hospital. On January 12, 1994, he admitted to our department because of the progressive muscle weakness. Neurologic examination revealed a muscular atrophy associated with severe weakness and hyporeflexia in both upper limbs, and fasciculation were seen in his tongue. Electrophysiological studies revealed mild conduction block in the left medial nerve, and F-waves were not evoked in the left ulnar nerve and bilateral median nerves. After an administration of 25 g/day of human gamma-immunoglobulin for 5 days, conduction block as well as F-wave abnormalities in the left median and left ulnar nerve were improved, yet no improvement of muscle weakness was seen. The anti-GM1 IgG titer was transiently elevated in the patient's serum after gamma-immunoglobulin therapy. On September 8, 1994, subtotal gastrectomy was performed because of the early stage gastric cancer. Histological examination showed poorly differentiated adenocarcinoma (signet-ring cell carcinoma). His muscle weakness had been gradually extended to the lower limbs and he couldn't walk himself on January, 1998. On March, 1998, he developed tetraplegia, mild dysphagia, dysuria and the respiratory disturbance. On April 12, 1998, he admitted to our department for the second time. Neurologic examination revealed a muscular atrophy and fasciculation associated with severe weakness in all of his limbs, tongue and musclus masseter. Neither deep tendon reflex nor pathologic reflex was evoked in his upper and lower extremities. His ocular movements and sensations were well preserved. He died of respiratory failure on May 1, 1998. The patient was presented in a neurological CPC. Neurological and laboratory findings suggested a spinal progressive muscular atrophy (SPMA). However, there were several unusual points as a typical SPMA in this case, that is, an improvement of the electrophysiological abnormalities by gamma-globulin treatment, as well as transient elevation of anti-GM1 antibody. The clinical neurologists have arrived at the conclusion that the patient had lower motor neuron syndrome associated with anti-ganglioside antibody and cause of death was ascribed to the respiratory failure. We discussed whether this case was SPMA or multifocal motor neuropathy. Postmortem examination revealed numerous diverticulums in the ascending colon and lymphothyroiditis. No recurrent carcinoma was detected. Neuropathologically, both severe atrophy of the anterior spinal roots, and severe gliosis and neuronal loss in the anterior horn of the spinal cord were observed. Onuf nuclei were not affected. Neurogenic muscular atrophy was detected in the tongue, diaphragm, and limb muscles. Motor neurons of the brainstem were relatively preserved, but skein-like inclusions as detected by anti-ubiquitin antibody, were present in the facial and hypoglossal nuclei. Neither motor cortex nor cortico-spinal tracts were affected. Demyelination, remyelination or cellular infiltrations were not apparent in the right median nerve and sciatic nerves. The neuropathologic features were compatible with SPMA.
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PMID:[An autopsy case with lower motor neuron disease showing a transient-appearance of anti-GM1 antibody and an improvement of conduction block after gamma-globulin administration]. 1039 55

A 48-year-old man residing in a mental health department inpatient program with a history of schizoaffective disorder presented to the emergency department with a chief complaint of fever and intense abdominal pain for one day. The patient stated he initially fell in the shower and afterwards experienced back pain. He was transferred to an acute care unit within the facility for further evaluation. The facility physician noted that the patient had a mild temperature elevation and abdominal rigidity on exam. At that time, he was given two doses of benztropine intramuscularly, and transferred to our hospital for further evaluation. The patient exhibited fever, diffuse abdominal pain and a nonproductive cough, but denied chills, dysuria, urinary frequency, hematuria, weakness, diarrhea, melena or hematochezia. He did have a one-week history of constipation for which he was given sodium phosphate enemas, magnesium citrate and docusate sodium, eventually resulting in a bowel movement. He also complained of new onset dysphagia. There were no recent changes to his medications, which included clonazepam, divalproex sodium extended release, olanzapine and risperidone. He denied use of tobacco, alcohol or illicit drugs.
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PMID:Clinical Case of the Month: A 48-Year-Old Man With Fever and Abdominal Pain of One Day Duration. 2715 3

Multiple system atrophy (MSA) is a spinocerebellar degenerative disease characterized by cerebellar ataxia, parkinsonism, and autonomic failure. A 75-year-old woman who had suffered from dysphagia and dysuria under a diagnosis of probable MSA with predominant cerebellar ataxia underwent autopsy. Eosinophilia was seen extensively in the muscle layer of the esophagus and urinary bladder. Eosinophilic infiltration to the esophagus was localized in the smooth muscle layer and could be considered as "eosinophilic esophageal myositis" identified in patients with nutcracker esophagus and jackhammer esophagus. Dense eosinophilia was present within the smooth muscle layer of the urinary bladder along with muscle fiber degeneration. We suspected a neuropathic etiology associated with MSA as the cause of the histological changes in the esophagus and urinary bladder; however, the possibility that some other disease might also have been responsible for the eosinophilic infiltration of the muscle layer cannot be denied. To our knowledge, this is the first report showing localized eosinophilia in the muscle layers of the esophagus and urinary bladder in the same patient. Although localized eosinophilia in visceral muscle has not been understood well, our case suggests the possibility that it is a feature of functional motility disorders and may have a neuropathic etiology.
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PMID:Eosinophilia in the muscle layer of the esophagus and the urinary bladder in a Multiple System Atrophy patient with dysphagia and dysuria. 2963 61

Autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy has been considered a novel central nervous system autoimmune disease characterized by relapse and responsiveness to corticosteroid with a specific GFAP-Immunoglobulin G (IgG) being noted in cerebrospinal fluid. We report the case of a 21-year-old girl presenting with dysuria and weariness, who subsequently developed blurry vision, slight dysphagia, slurred speech, and sensory abnormality. GFAP-IgG was detected in her cerebrospinal fluid. Magnetic resonance imaging using both T2-weighted and contrast-enhanced T1-weighted images revealed a rare finding of lesions distributed mainly in the entire spinal cord rather than typical brain lesions. After treating with corticosteroids, her clinical symptoms were alleviated, and the spinal cord lesion enhancement was reduced. Our observations extend the clinical spectrum of autoimmune GFAP astrocytopathy. We suggest that rare distributed lesions in the entire spinal cord in patients with autoimmune GFAP astrocytopathy cannot be ignored by neurologists. The identification of potential atypical lesions broadens the understanding of autoimmune GFAP astrocytopathy.
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PMID:Autoimmune glial fibrillary acidic protein astrocytopathy with lesions distributed predominantly in the entire spinal cord. 3254 40