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 61-year-old woman was admitted to the hospital on September 18, 1991 because of left hemiparesis, dysphagia, and dysarthria since five days before. She was formerly pointed out diabetes mellitus and hypertension, but she did not receive any treatment. The MRI showed a high signal area in the right paramedian portion of the upper pons on T2 weighted image and proton image. The angiography showed that persistent primitive proatlantal artery originated from the left internal carotid artery and joined to the horizontal portion of the left vertebral artery. The image of carotid-vertebrobasilar system and proatlantal artery showed so severely arteriosclerotic. This is the first report of brainstem infarction with persistent primitive proatlantal artery. In this case, the pontine infarction was thought to occur on the basis of the arteriosclerosis of blood vessels and change of blood flow of carotid-vertebrobasilar system due to persistent primitive proatlantal artery.
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PMID:[A case of pontine infarction with persistent primitive proatlantal artery]. 829 79

A 37-year-old female with a history of hypertension for 5 years was brought to the emergency room with swelling of the tongue and neck after the second dose of enalapril. After administration of hydrocortisone by her physician, she went to the emergency room. Her dyspnea and dysarthria were relieved. However, she experienced recurrence of the symptoms followed by respiratory arrest. She suffered severe anoxic brain damage and died three days later. Although angioedema is a rare occurrence with the use of enalapril, it is potentially life threatening.
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PMID:Fatal angioedema associated with enalapril. 835 21

Cervical vagus neurinoma with hypoglossal palsy is extremely rare and only one case has been reported so far in Japan. A 52-year-old female complained of dysarthria. She has hypoglossal palsy with left-deviated tongue and the left half of her tongue was markedly atrophic. Cervical mass was not palpable and she had no other complaint such as hoarseness or dysphagia. CT scan and MRI revealed a tumor at a caudal part of the left jugular foramen. A hypoglossal canal was intact. Cerebral angiography demonstrated arterio-venous dissociation but vascular formation or tumor stain was not revealed. The tumor was totally removed by transpetrosal approach. The vagus nerve became thicker above C2 level and changed into the yellowish tumor with clear margin. The hypoglossal nerve was compressed between the tumor and an occipital bone. The authors think this is why hypoglossal palsy appeared. Histologically, the tumor was neurinoma. Clinical symptoms, diagnosis, and treatment were discussed.
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PMID:[A case of cervical vagus neurinoma with hypoglossal palsy]. 836 53

We report a 20-year-old female with Hallervorden-Spatz syndrome (HSS). This patient is the product of consanguineous parents. She developed genu valgum, tendency to fall and mental deterioration at the age of 6, decrease of the number of spoken words at the age of 14, dysarthria, unsteady gait, postural tremor of the upper extremities, dystonic posture of hands and double incontinence at the age of 16. Her disease progressed slowly. Neurological examination on admission revealed severe mental retardation, optic atrophy, forced grasping, hyperactive tendon reflexes in the upper extremities and bilateral Babinski sign. An extensive laboratory investigation including the leukocyte lysosomal enzymes, serum amino acid analyses, copper studies and ceruloplasmin were almost within normal limits. MRI, T2 weighted images, showed markedly decreased signal intensity in the globus pallidus but substantia nigra and increased signal intensity in diffuse cerebral white matter. T1 weighted images showed marked atrophy of the brainstem and cerebellum. She met the diagnostic criteria for HSS by Swaiman; we diagnosed her as HSS group II. HSS is characterized by the presence of many spheroids in the central nervous system which is similar to neuroaxonal dystrophy (ND). However, clinical and pathological differences exist between HSS and ND, the precise classification of the two conditions has remained controversial. Although there are many reported cases in which both conditions overlap, this is the first reported case that simultaneously demonstrates increased iron deposition in the globus pallidus, marked atrophy of the brainstem and cerebellum and typical clinical course compatible with HSS.
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PMID:[A case of Hallervorden-Spatz syndrome with marked atrophy of the brainstem and cerebellum]. 836 59

Four weeks after an attack of pneumonia of unknown aetiology a 40-year-old woman was hospitalized because of a nonpurulent, predominantly basal meningoencephalitis and infratentorial abscesses. She had dysarthria, mild right-sided motor hemiparesis and central paresis affecting the 7th cranial nerve. An area of fluctuating resistance, about 3 cm in diameter, was noticed over the left thigh. Serology indicated inflammatory disease, but there was no immunodeficiency. The CSF showed lymphocytic pleocytosis with mild protein increase but no evidence of infective agent. As tubercular meningitis was suspected she was treated with rifampicin (300 mg i.v. twice daily), isoniazid (300 mg i.v. once daily), streptomycin (800 mg i.m. once daily), cefotaxime (2.0 g i.v. three times daily), fluconazole (200 mg i.v. once daily) and dexamethasone (16-8-8 mg i.v.). She suddenly died two days after admission, probably as the result of central regulatory failure. Generalized nocardiosis involving lung, subcutaneous tissue and brain was revealed at autopsy. Although nocardiosis occurs predominantly in patients under immunosuppression, this infection should be considered in the differential diagnosis of treatment-resistant pneumonia and meningoencephalitis without obvious predisposition.
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PMID:[Generalized nocardiosis with meningoencephalitis in a nonimmunosuppressed female patient]. 837 98

Chronic salicylate intoxication represents an unappreciated form of self-poisoning in the elderly and therefore poses a diagnostic challenge. This report describes an elderly female with chronic salicylism who presented with unexplained delirium, dysarthria, diminished short-term memory and hearing, and urinary and fecal incontinence. She was treated with intravenous hydration, urinary alkalinization, and subsequent hemodialysis for persistent aciduria, acidemia and impending circulatory collapse. Major morbidity included myocardial infarction, life-threatening dysrhythmias, and mixed bacterial urosepsis. This report highlights the need to maintain a high index of suspicion for salicylate poisoning in the elderly, who commonly present with nonfocal neurologic features.
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PMID:Accidental chronic salicylate intoxication in an elderly patient: major morbidity despite early recognition. 843 48

Among 17 patients with amyloid polyneuropathy type IV in a Japanese family, we found a 72-year-old woman, who showed extraocular symptoms approximately 10 years after the onset of the disease. she developed weakness of the right facial muscles and dysarthria at age 57. She had atrophy and disturbance of movement of the tongue, along with difficulty in swallowing at age 62. At the age of 66, she felt diplopia when she looked toward the left, followed by difficulty of ocular movement. These manifestations progressed and at age 72, she was found to have mild ptopsis, mild to moderate disturbance of almost all extraocular muscles, moderate to severe disturbance of facial, masseter, pharyngeal, tongue and neck muscles. She also had slight weakness and atrophy of the limb and truncal muscles together with slight loss of pain and vibratory sensations in the distal parts of the limbs. FAP IV has sometimes been called cranial amyloidosis, but motor disturbance is limited to the middle and lower cranial nerve territories in the majority of the reported cases, and manifestations of the extraocular muscles are quite rare. According to the present investigation of the world literature, this is the second case of FAP IV with extraocular muscle involvement.
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PMID:[Familial amyloidosis of the Finnish type (FAP) with extraocular muscle involvement]. 856 42

A 52-year-old woman was admitted to our hospital because of paralysis of her right lower limb and dysarthria. After admission, she had exacerbations of paralysis of the right lower limb twice. Brain CT scan revealed a low density area in the left anterior cerebral artery territory. MRI disclosed the lesion of the body of the corpus callosum. On the fifth day, trans-esophageal echocardiography detected a mass lesion in the left atrium. There was no recurrence after treatment with heparin. On the 23rd day, the left atrial mass was removed surgically, which was elastic and soft and was confirmed to be myxoma pathologically. It was suggested that the embolic material originated from an adherent thrombus on the surface of the myxoma, since heparin was effective. She showed unique movements of her right hand like diagonistic dyspraxia. The right hand with mild paresis took away an object held in the left hand. The patient was able to move the right hand voluntarily and to imitate with it. She did not show grasping reflex, instinctive grasp reaction and apraxia of the left hand. The lesion of the body of corpus callosum may be responsible for this peculiar neurological symptom.
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PMID:[A case of left atrial myxoma with repeated ischemia of left anterior cerebral artery territory]. 868 96

N-acetylneuraminic acid (sialic acid) storage disease is a rare autosomal recessive lysosomal disorder. Clinically two major forms exist, an infantile type with severe progression leading to early death, and a milder form (Salla disease) with a protracted course. Intermediate forms may also exist. Diagnosis rests on the determination of an excessive excretion of sialic acid in urine and concomitant storage in fibroblasts, the severe forms exhibiting the highest excretion and storage. We present clinical, morphological, and biochemical data on three non-Finnish patients with sialic acid storage disease. Patient 1 was a preterm infant with neonatal ascites, coarse face, hepatosplenomegaly, pale skin, and wispy hair. Vacuolated lymphocytes were abundant in a peripheral blood smear and he excreted large amounts of free sialic acid. High levels of free sialic acid were also found in cultured skin fibroblasts. He died at age 6 months from progressive respiratory insufficiency. Patient 2 was an 11-month-old Egyptian girl with coarse face, frequent upper respiratory tract infections, hepatosplenomegaly, and severe psycho-motor retardation. Sialic acid excretion was elevated, likewise the storage in fibroblasts. Histological investigations documented vacuolar storage in a skin biopsy and in iliac crest tissue. Patient 3 was a 16-year-old girl with slightly coarse face, severe generalized muscular hypotonia, ataxia, and kyphoscoliosis originally diagnosed as having post-partum asphyxia. She suffered progressive motor function loss and had dysarthria. Urinary sialic acid was elevated and a skin biopsy demonstrated vacuolization. The clinical variability of sialic acid storage disease is exemplified by these three cases. Simple urinary screening for free sialic acid facilitates the diagnosis. The degree of urinary excretion may indeed correlate with clinical presentation and progression.
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PMID:The spectrum of free neuraminic acid storage disease in childhood: clinical, morphological and biochemical observations in three non-Finnish patients. 872 11

We experienced a 15-year-old female, whose healthy parents were second cousins, who was suspected of having dysmyelinating disease involving only the central nervous system (CNS). She was noticed to have congenital pendula nystagmus, and spastic gait disturbance developed at the age of 10 years. Mild athetosis of the upper limbs and ataxia were recognized at age 13 years, and dysarthria presented at age 15. MRI and electrophysiological findings showed the characteristics of Pelizaeus-Merzbacher disease (PMD), although the extensive nerve conduction slowing of the CNS was less severe than that in male patients with PMD. No promoter or exonic mutations of proteolipid protein (PLP) gene were detected. Although this patient might be heterozygous for a mutation of the extraexonic PLP gene sequences or of other unknown X-linked PLP associated genes, we speculate that this case had a dysmyelinating disease with an autosomal recessive trait characterized by the same phenotype as that of PMD.
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PMID:Pelizaeus-Merzbacher-like disease: female case report. 873 1


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