Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0013362 (dysarthria)
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The first autopsy of a case of multiple sclerosis from the District of Hokuriku was reported. The patient, a 50-year-old house-wife, born in Toyama Prefecture, had noticed a paresthesia of her face, fatigue, numbness and weakness in the right limbs, dimness of vision and gait disturbance at ave 44. Furthermore, in the course of the disease, she had suffered from visual disorder, tetraplegia, hyperreflexia, pyramidal signs and cerebellar syndroms such as dysarthria, nystagmus, intention tremor and ataxia. She also showed symptoms of euphoria and dementia. After a course of six years she died of bronchopneumonia. Remissions and exacerbations were noted four times during her clinical history. Histopathologically, there were many recent and old demyelinating lesions of varying sizes and shapes in all parts of the central nervous system, namely the cerebrum, brainstem, spinal cord and optic nerve. In contrast to the clinical symptoms, the cerebellum itself revealed less plaques than the other areas of the brain. According to the observed distributions of the lesions, our case can be classified as belonging to the optico-cerebro-spinal type in the Ikuta and Zimmerman classification. The demyelinated lesions were characterized by a perivenular distribution of the plaques, lack of tissue necrosis, paucity of inflammatory reaction and marked fibrous gliosis of varying degrees.
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PMID:First autopsy report of a multiple sclerosis case in Hokuriku District of Japan. 53 38

Sixty-six hospitalized patients suffering from chronic methylmercury poisoning were examined in Baghdad during 1972. The poisoning was attributed to consumption of home-made bread prepared from seed wheat treated with mercurial fungicide. The age incidence ranged between 4 and 70 years.Of the various clinical features encountered, neurological symptoms and signs were predominant and included muscular weakness, numbness, unsteady gait, paraesthesia, dysarthria, mental disturbances and, in severe cases, blindness, partial deafness, stupor, coma, and death. Involvement of the cardiovascular, urinary, gastrointestinal and haemopoietic systems, which was commonly encountered in ethylmercury poisoning in the 1960 outbreak in Iraq, was unusual.The severity of symptoms and signs was, broadly speaking, dose-dependent; high exposure led to severe clinical manifestations, but variations existed. Criteria, based on the clinical manifestations, were set for grading the severity of cases. The series included 2 asymptomatic cases, 20 mild, 20 moderate, 14 severe, and 10 very severe. In the latter group 5 patients died from failure of the central nervous system.The severely poisoned patients died irrespective of the medical treatment received. After 2 years of observation, most patients graded as mild or moderate cases lost their symptoms completely. Severe cases improved slowly, although ataxia, diminution of visual field and acuity and paraesthesia were still present. Thus, the previously accepted view that neurological signs were irreversible has been disproved.
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PMID:Intoxication due to alkylmercury-treated seed--1971-72 outbreak in Iraq: clinical aspects. 108 68

Three cases of later onset metachromatic leukodystrophy in one family were reported. The brother is 32 years old, younger sister is 35 years old and the elder sister is 39 years old, who were normal as child without any family history of neurological disease. The three cases began their illness at about the age of 30 years old with dysarthria, progressive dementia, motor disturbance and numbness in the extremities. CT scan showed low density in the white matter of frontal, parietal lobes and around the ventricle. The written reports of CT scan were lacunar infarction in brother, multiple lacunar infarction in younger sister and Binswanger's disease in elder sister. Nerve biopsy showed myelinated fibers were decreased in number. Metachromatical materials were seen in and around the Schwann cell cytoplasm. Metachromatical materials were lamillar inclusions in granular matrix by electromicroscopic examination. So nerve biopsy is easy, safe and effective method to diagnose the uncertain pathogenic leukoence-phalopathy.
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PMID:[Later onset metachromatic leukodystrophy diagnosed by nerve biopsy]. 133 86

Spontaneous dissections of the internal carotid arteries are uncommon but are not rare. They constitute a fairly common cause of ischemic stroke in young patients (young in terms of the age at which strokes generally occur). The common presenting manifestations are (1) unilateral headaches followed after a period of delay by focal cerebral ischemic symptoms or (2) unilateral headaches and ipsilateral incomplete Horner's syndrome. These may or may not be associated with subjective or objective bruits. In rare instances, spontaneous dissections of the internal carotid arteries may present as lower cranial nerve palsies and cause dysphonia, dysarthria, dysphagia, and numbness of the throat. Affected patients may initially present to the otolaryngologist or be referred to one. This article describes eight patients with spontaneous dissections of the internal carotid arteries and lower cranial nerve palsies, and the pertinent literature is reviewed.
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PMID:Spontaneous dissection of the cervical internal carotid artery. Presentation with lower cranial nerve palsies. 155 74

A Japanese woman, aged 42, was admitted because of paroxysmal attacks consisting of paresthesia of the left face, tremor in the right hand, epigastric pain and urinary incontinence. A year prior to the admission, she noticed some difficulty in writing, dysarthria and unsteadiness of walking. These symptoms had been persistent since then. At the end of March, 1991, these symptoms rapidly worsened, and she fell down frequently. She also experienced pain behind both eyes, numbness in her left fingers and toe, urinary frequency and the above-mentioned attacks. Neurological examination disclosed bilateral internuclear ophthalmoplegia and upbeating nystagmus on upward gaze, titubation in the head, scanning speech, dysmetria in all limbs, exaggerated reflexes in jaw and both legs, bilateral extensor plantar reflexes and ankle clonus. SEP showed delayed cortical response with stimulation of the median nerves bilaterally and of the right posterior tibial nerve. P40 was absent with the left posterior tibial nerve stimulation. VEP was normal. T2-weighted image of MRI showed multiple high intensity areas located around the third ventricle, crus cerebri and the right upper part of the pons. The diagnosis of multiple sclerosis was made. Each paroxysmal attack started with numbness in the left face and burning sensation in the neck. Almost simultaneously tremor in the right hand began. The surface EMG showed the rhythmic contractions in the dorsal hand muscles and wrist extensors at a frequency of 6-7 Hz, and sometimes it revealed synchronized contractions of finger flexors and the dorsal hand muscles. A few seconds later she felt painful sensation in the epigastric region, and the tremor gradually increased in its intensity.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[A case of multiple sclerosis with paroxysmal attacks of facial paresthesia, unilateral hand tremor, epigastric pain and urinary incontinence]. 162 36

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

Eight patients were evaluated for severe vertebrobasilar insufficiency (VBI). There were five males and three females, with an average age of 60.2 years (range 42 to 67 years). Three were diabetic and five hypertensive, including two patients who had both diseases. Seven of the eight had ongoing episodes of VBI refractory to anticoagulant and/or antiplatelet agents. Symptoms included two or more of the following in all patients: dizziness, diplopia, hemiparesis, hemihypesthesia, perioral numbness, bilateral visual blurring, dysarthria, and ataxia. Angiography revealed severe atherosclerotic stenosis of the proximal or midsection of the basilar artery in all patients. A 10-cm segment of the anterior or posterior division of the superficial temporal artery (STA) was anastomosed to a proximal segment of the superior cerebellar artery (SCA) through a right subtemporal approach. Seven of eight (87%) postoperative angiograms demonstrated patency as evidenced by filling of the SCA and, in most cases, of the basilar artery. Six of the eight patients were improved or asymptomatic after the operation, one was unchanged, and one died. The average follow-up period was 14 months, with a range of 4 to 23 months. Transient morbidity included temporal lobe swelling in four patients and a subdural hematoma in one. Anastomosis of the STA to the SCA is a feasible therapeutic option in the patient with VBI secondary to stenosis of the proximal or midsection of the basilar artery.
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PMID:Posterior circulation revascularization. Superficial temporal artery to superior cerebellar artery anastomosis. 707 75

Thirty-four patients were submitted to the conventional cervical myelography by administration of metrizamide (Amipaque) through three routes (lumbar 23, suboccipital 6, C1-C2 lateral 5). After the injection of metrizamide (4-11 ml, 170-250 mgI/ml), all procedures of the cervical myelography were done as soon as possible within 9 minutes. The adverse reactions of Amipaque were observed in 29 cases (85%) out of 34 cases initially 1 hour after cervical myelography and disappeared completely in an average of 16 hours. The total number of the side effects was 140 incidences such as meningeal irritation (headache 18, nausea 17, vomiting 17), cerebellar signs (dizziness 11, dysarthria 8, tremor 5, bradylalia 2, dysmetria 2, tipsy feeling 2, dysdiadochokinesis 1), autonomic signs (flushing 7, pale face 4, fever 4, sweating 2, hiccup 2, fatigability 2, micturition disturbance 1), sensory signs (exacerbation of numbness 6, perioral numbness 3, back pain 1, chest pain 1), motor signs (focal muscle spasm 5, exacerbation of paresis 4, areflexia 1), psychiatric signs (dysphasia 3, disturbance of consciousness 2, euphoria 1, persecutory delusion 1) and muddiness 7. We observed that waxing and waning of side effects correlated tightly with transient cortical penetration of dye in CT and cortical dysfunction mainly slowing of the background activity and slow wave burst in EEG. According to high frequency of side effects in our study, we suggest that a greater incidence of side effects may result when high concentration of Amipaque comes in contact with the cerebral cortex by using an inadequate fluoroscopic table which has only fixed one plane image and rough positioning control. Slow absorption into blood stream may affect appearance and maintenance of side effects. In order to decrease side effects after Amipaque cervical myelography, we propose that we should introduce a mobile rotating chair coupled with high power image and chose C1-C2 lateral route using 1500-1700mgI of Amipaque.
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PMID:[Side effects of metrizamide (Amipaque) cervical myelography (author's transl)]. 711 May 15

We presented a rare care who had right frontal lobe infarction, with left side pseudoataxia, and the mechanism, causing pseudoataxia, was considered. The patient, a 51 year-old, righ-handed male, was admitted on August 9, 1980, complaining of left-side pseudoataxia. About p.m. 7:00, July 29, 1980, he suddenly noticed numbness of the left foot, and he found himself difficulty in standing in the next morning. He had a mild paresis and tactile-tactile of the left side including the face, which was rapidly improved. However, there was pseudoataxia of the left extremities, which had not been improved. On physical examination, dysarthria, aphasia, finger agnosia, difficulty in right left orientation or muscle weakness was not recognized, and there was no sensory disturbance except for slight impairment of stereognosis, two point discrimination and vibratory sense. Demonstrable impairment of tactiletactile from was observed in the left hand. Notable dysmetria, terminal tremor and dysdiadochokinesia were seen in the left limbs, which were remarkably worsened with eyes closed. However, tapping and line-drawing tests were normal. Babinski-Weil's test disclosed typical compass gait. There was marked swaying in Romberg position. Tandem gait was impossible with a tendency to decline the left. Deep reflexies were normal except for mildly hyperactive radial reflex in the left. Carotid and vertebral angiographies revealed neither evidence of vascular occlusion nor displacement of vessels CT scan demonstrated a low density area, which included the right inferior and middle frontal gyri, the head of the right caudate nucleus and a part of anterior crus of right internal capsule. There was enlargement of anterior horn of the right lateral ventricle. Caloric test, electronystagmography, eye tracking test or optokinetic nystagmus test disclosed no abnormalities. Vibration induced falling, which is the postural reaction to muscle vibration during standing (Ekuland, G., 1972), was not recognized when the left Achiles' tendon was stimulated. Pseudoataxia of this patient differed from the typical cerebellar or vestibular ataxia. From a review of the literatures concerning frontal pseudoataxia, almost all cases had no distinct cerebellar signs, and showed positive Romberg's sign. The impairment of tactile-tactile form and postural reaction to vibratory stimulation to the left leg, appeared in this case, could be hardly explained by the lesion of parietal lobe or deconnection syndrome. Sensory perception of parietal lobe and pyramidal motor system were thought to be almost normal in this case. Therefore, these findings should be due to impairment of integration center between sensory and motor systems. The pseudoataxia in frontal lesion seems to occur as the results of involvement of this center, in which caudate nucleus maybe has important role, but not as the results of disturbances in the front-ponto-cerebellar or front vestibular pathway.
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PMID:[Frontal pseudoataxia, discussion on its mechanism (author's transl)]. 732 87

This is a report of unruptured aneurysms with occlusion of the basilar artery. A 61-year-old female was admitted to our hospital because of dysarthria and numbness of her left face. Angiography revealed occlusion of the basilar artery and severe arteriosclerosis of the bilateral cerebral carotid arteries. Pcom was not visualized on bilateral carotid angiogram. These neurological signs were considered to be derived from vertebrobasilar insufficiency by occlusion of the basilar artery. Right STA-SCA anatomosis was performed to prevent brain stem infarction. Postoperative angiography showed a good filling of both PCA and SCA by collateral circulation via a right STA and an unruptured basilar top aneurysm. Seven months after the bypass surgery, angiography disclosed that the basilar top aneurysm was visualized clearly, and its size was unchanged. The fact that there was no thrombus formation in the aneurysm was considered to be due to ticlopidine, and the hemodynamic changes after the bypass surgery were suspected to have increased the intraaneurysmal pressure. Therefore we performed neck clipping of the basilar top aneurysm by using a right pterional approach. Two years after the second operation, the patient complained of severe headache and vomiting. CT scan showed subarachnoid hemorrhage, and angiography demonstrated a newly developed aneurysm which might have ruptured on left internal carotid anterior choroidal artery bifurcation. Emergency neck clipping of the second aneurysm was performed, and the patient showed a good postoperative course. The newly developed second aneurysm might have been caused by severe arteriosclerosis and hypertension in addition to hemodynamic stress.
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PMID:[A case of growing up aneurysms with occlusion of basilar artery]. 766 40


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