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)

Extraskeletal Ewing's sarcoma (EES) is rarely found in the head and neck region. We report here a case of EES of the parapharyngeal space in a 53-year-old man who presented with blurred vision, dysphagia, hoarseness and right facial numbness. CT examination showed a large, seemingly well-defined soft tissue mass in the right parapharyngeal space with skull base destruction and intracranial extension. The patient showed poor response to chemotherapy and radiotherapy and died 6 months after initial presentation. A review of the literature revealed no previous reports of EES occurring in the parapharyngeal space.
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PMID:Extraskeletal Ewing's sarcoma of the parapharyngeal space. 1556 49

Despite major advances in skull base surgery and microsurgical techniques, surgery for vestibular schwannoma (VS) carries a risk of complications. Some are inherent to general anesthesia and surgery of any type and include myocardial infarction, pneumonia, pulmonary embolism, and infection. Some are specific to neurosurgery in this area of the brain, and include hydrocephalus, cerebrospinal fluid leak, facial nerve paralysis, facial numbness, hearing loss, ataxia, dysphagia, and major stroke. Even in the hands of very experienced acoustic surgeons, these risks cannot be eliminated. Radiosurgery provides an outpatient, noninvasive alternative for the treatment of small acoustic schwannomas. Initially radiosurgery was undertaken in "high-risk" patients, including the elderly, those with severe medical comorbidities, and those in whom tumors recurred after surgery. Additionally, a high rate of cranial nerve morbidity was reported. With improvements in dosimetry planning and dose selection, however, authors practicing at radiosurgical centers now report very low complication rates, as well as high tumor control rates. In this report the authors specifically review the results of linear accelerator-based radiosurgery for VS and compare these outcomes with the best surgical alternatives.
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PMID:Linear accelerator-based radiosurgery for vestibular schwannoma. 1566 13

A 'syringomyelia-like' syndrome has been infrequently reported in neurological disorders such as Tangiers disease and lepromatous leprosy. This study reports a novel 'syringomyelia-like' syndrome in four adult male patients, which we have termed facial onset sensory and motor neuronopathy, or FOSMN syndrome, that appears to have a neurodegenerative aetiology. Clinical, neurophysiological and pathological data of four patients were reviewed, including the autopsy in one patient. Four male patients (mean age at onset 43), initially developed paraesthesiae and numbness in a trigeminal nerve distribution, which slowly progressed to involve the scalp, neck, upper trunk and upper limbs in sequential order. Motor manifestations, including cramps, fasciculations, dysphagia, dysarthria, muscle weakness and atrophy developed later in the course of the illness. Neurophysiological findings revealed a generalized sensory motor neuronopathy of caudally decreasing severity in all four patients. Autopsy in one patient disclosed loss of motoneurons in the hypoglossal nucleus and cervical anterior horns, along with loss of sensory neurons in the main trigeminal sensory nucleus and dorsal root ganglia. FOSMN syndrome appears to be a slowly progressive neurodegenerative disorder, whose pathogenesis remains to be determined.
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PMID:Facial onset sensory and motor neuronopathy (FOSMN syndrome): a novel syndrome in neurology. 1701 96

Leprosy affects the larynx, damaging its mucosa and sensory nerves and loss of sensation may result in aspiration of food and secretions. The laryngeal lesion may be insidious. Post-mortem studies showed bronchopneumonia that could have originated from aspiration. In patients with laryngeal symptoms, dysphagia or aspiration pneumonia loss of laryngeal sensation should be looked for.
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PMID:Laryngeal lesion in leprosy and the risk of aspiration. 1751

Takayasu arteritis is a rare vasculitis of the aorta and its branches. Neurological manifestation usually results from central nervous system ischaemia. We report a case presenting with unilateral paresis of the cranial nerves (V, IX and XII nerve) caused by a vascular conflict due to Takayasu arteritis. A 38-year-old male was admitted to the hospital complaining of dysarthria, dysphagia, numbness of the right side of the tongue and a headache localized behind the right eye. The symptoms had sudden onset. Neurological examination revealed isolated trigeminal, glossopharyngeal and hypoglossal nerve dysfunction on the right side without other neurological symptoms. Magnetic resonance angiography showed internal carotid artery dissection and prominent thickening of walls of both vertebral arteries as well as the left renal artery with narrowing of lumen. Compression of glossopharyngeal and hypoglossal nerves and the trigeminal ganglion was a result of a markedly dilated intracranial segment of the right carotid artery. The clinical and radiological findings were consistent with the diagnosis of Takayasu arteritis.
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PMID:[Compression neuropathy of cranial nerves in the course of Takayasu arteritis]. 1822 78

Chronic lymphocytic leukemia (CLL) is a frequent hematological malignancy, with meningeal or peripheral nerve infiltrations being the most commonly encountered neurological complications. In this report, we describe a CLL patient with Miller-Fisher syndrome (MFS) who responded to immune modulation with plasmapheresis. A 47-year-old man diagnosed as B-cell CLL admitted with neutropenic fever. He complained of diplopia and numbness of both arms. Neurological examination revealed a bilateral external ophthalmoplegia, dysphagia, dysarthria, mild shoulder girdle muscle weakness and gait ataxia, accompanied by absent tendon reflexes. Nerve conduction studies were indicative of a predominantly axonal sensori-motor peripheral neuropathy. This association of CLL with MFS had not been previously reported in the literature.
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PMID:Miller-Fisher syndrome associated with chronic lymphocytic leukemia. 1868 50

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 report a 58-year-old woman with adult onset Alexander disease. At the age of 54 she noticed numbness in bilateral legs and at 57 she developed left sided spastic gait. Her walking difficulty was gradually worsened and followed by the development of weakness in left arm, dysarthria and dysphagia. Her mother and elder brother also had similar clinical presentations which suggested an autosomal dominant neurological disorder. With MRI findings showing localized atrophy of medulla oblongata and upper cervical cord with hyperintensities on T2-weighted image, diagnosis of adult onset Alexander disease was made. We performed genetic analysis and found novel variant (S398F) in the glial fibrillary acidic protein gene. In case of slowly progressive myelopathy with bulbar palsy of unknown origin, especially those with atrophy limited to medulla oblongata and upper cervical cord, adult onset Alexander disease should be taken into consideration.
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PMID:[Adult onset Alexander disease with a novel variant (S398F) in the glial fibrillary acidic protein gene]. 1961 46

Sarcoidosis is a chronic disease of unknown aetiology. Neurosarcoidosis is registered in 5% of patients with sarcoidosis. Clinical manifestations of sarcoidosis are numerous and diverse. Manifestation of Neurosarcoidosis includes partial- and grand-mal seizures, low-grade fever, headache, increased intracranial pressure, visual disturbances, diabetes insipidus, amenorrhea- galacterorrhea syndrome and pituitary failure, hypogonadotropic hypogonadism, hyperprolactinemia, unilateral and bilateral facial palsy, infiltration of meninges (aseptic meningitis) and nerve roots, leptominingitis, pachymeningitis with cranial neuropathies, pseudotumor, mild cognitive disorder, psychosis, delirium, dementia, disorientation, amnesia, progressive visual deterioration and proptosis, axonal polyneuropathies, mononeuropathies, chronic polyradiculoneuritis, peripheral neuropathy, cranial nerve abnormalities, radiculopathies, peripheral neuropathy, mononeuritis multiplex, progressive numbness and deep sensation disturbance in bilateral lower extremities, hemiplegia, hyperreflexia with pathological reflexes and hypesthesia, upward gaze palsy, spinal cord compression, dysarthria, dysphagia, weakness, episodes of blurred vision, diplopia, intracerebral hemorrhage, neuro-ophthalmic manifestations, intranuclear ophthalmoplegia, dysorientation, vasculitis presenting with strokes, intracranial hypothalamic lesion, paresthesis, hemiparesis, myelopathy in the cervico-thoracic region, lumbar pain, sensory level and inability of lateral gaze (Tab. 2, Ref. 60).
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PMID:Clinical manifestations of neurosarcoidosis. 1982 43

Between 1998 and 2007, 42 patients admitted to Choray hospital, Ho Chi Minh City, and to two hospitals in adjacent regions in southern Viet Nam brought the Malayan kraits (Bungarus candidus) that had been responsible for biting them. Half of the patients had been bitten while they were asleep. Fang marks and numbness were the only local features of the bites. Common signs of neurotoxic envenoming included bilateral ptosis, persistently dilated pupils, limb weakness, breathlessness, hypersalivation, dysphonia and dysphagia. Thirty patients (71.4%) required endotracheal intubation of whom all but one were mechanically ventilated. Fourteen patients (33.3%) developed hypertension, 13 (31.0%) shock, 31 (73.8%) hyponatraemia (plasma sodium concentration < 130 mEq/l) and 30 (71.4%) showed evidence of mild rhabdomyolysis (peak plasma creatine kinase concentration 1375 +/- 140 micro/l). None developed acute kidney injury. All the patients were treated with a new monospecific B. candidus antivenom. There were no fatalities. Hyponatraemia has been reported previously in victims of Chinese kraits (Bungarus multicinctus) in northern Viet Nam and rhabdomyolysis in patients envenomed by B. niger in Bangladesh. These features of envenoming pose new problems for the management of krait bite cases in South east Asia and should stimulate a search for the causative venom toxins.
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PMID:Hyponatraemia, rhabdomyolysis, alterations in blood pressure and persistent mydriasis in patients envenomed by Malayan kraits (Bungarus candidus) in southern Viet Nam. 2063 19


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