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 15-year-old boy suddenly developed a headache, followed by progressive weakness of the right extremities. A computed tomographic scan obtained on admission showed a low-density area in the left putamen extending to the internal capsule. No neurological deficits were observed other than slight dysarthria and right hemiparesis. Left carotid angiography showed the characteristic "string of beads" deformity of fibromuscular dysplasia (FMD) extracranially and complete occlusion of the internal carotid artery at the C2 portion. The right carotid artery and both vertebral arteries were normal. Anastomosis of the left superficial temporal and middle cerebral arteries was performed 1 month after the onset. The patient recovered quickly from the hemiplegia and was discharged with no neurological deficits. Angiograms obtained 3 months later showed not only patency of the bypass, but also recanalization of the intracranial internal carotid artery. Cervico-cephalic FMD mainly affects middle-aged women, and is uncommon in children; only nine childhood cases have been reported. This disorder may play an important role in the genesis of strokes among children.
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PMID:[Fibromuscular dysplasia with cerebral infarction in children. Case report]. 248 41

TRH analogues have a longer half-life than does TRH and enhanced neuropharmacological actions. In motorneurone disease (MND), no benefit was reported with MK771 and DN1417. Focal, transient, and slight improvements in weakness and spasticity were described with CG3509. A controlled trial with a single intravenous dose of RX 77368 showed improvements in dysarthria, tongue movements, respiration, swallowing, and spasticity lasting up to 72 hours. Changes in muscle force were of no functional significance. There was an acute 25-30% increase in mean corrected fiber density and in mean macro-EMG parameters in biceps, but no change in amplitude or area of single macro-EMG motor units followed during the 2-hour infusions. An acute, direct or indirect, central effect of RX77368 on recruitment order or on activation threshold of pathological motor units is suggested. In a subacute open trial with repeated intravenous infusions of RX77368 (median 2 weeks), improvement in bulbar function in 8 of 12 responders, cramps (5 of 9), and spasticity (5 of 8) were maintained for medians of 18, 14, and 7 days, respectively. Side effects were prominent with doses above 0.2 mg/kg. Disease progression has not been halted with any analogue, but whether it may be usefully slowed down with RX77368 is worth investigating.
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PMID:Use of TRH analogues in motorneurone disease. 249 83

First reported in 1972 by Berg & colleagues, giant axonal neuropathy is a generalized disorder of cytoplasmic intermediate filaments affecting the nervous system particularly. The condition was originally thought to be a disorder of the peripheral nervous system, but clinical and pathological evidence has now accumulated which indicates that the brain and spinal cord are progressively involved. Over 20 cases have been reported to date. All cases reported have developed clumsiness and progressive weakness with hyporeflexia in the first seven years of life. Later dysarthria, cerebellar signs and pyramidal tract disturbances appear. Mental retardation, dementia and seizures are sometimes seen. Tightly curled hair is characteristic of, but not invariably present in, the condition. This disorder, as well as a similar condition affecting dogs, appears to be transmitted by autosomal recessive inheritance. No treatment is effective. Most cases are wheelchair bound or dead by the end of the second decade.
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PMID:Giant axonal neuropathy. A review. 254 97

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

Various types of associated movements of eyeballs with other cranial muscles have been described in the literature. Only a few observations, however, have been reported on the relation of ocular movements and facial muscles innervated by the facial nerve, especially the orbicularis oculi muscles. We report two cases presenting gaze-evoked involuntary contraction of the orbicularis oculi muscle and unilateral eyelid closure. Case 1 was a 38-year-old housewife who was admitted to our hospital because of gait disturbance and sensory deficits below the neck. She had a 5-year history of left facial palsy, disturbed horizontal eye movements, retrobulbar optic neuritis, spastic paraparesis and bladder and rectal disturbances, some of which had relapsed a few times. Neurological abnormalities were summarized as follows: bilateral pale discs, right Horner's sign, horizontal nystagmus, left facial nerve palsy of peripheral type, spastic paraparesis with left-sided predominance, sensory disturbances below the third cervical segment, truncal and limb ataxia, and bladder and rectal disturbances. Gaze-linked synchronized contraction of the left facial muscles was observed and on the left lateral gaze marked spasm of the left orbicularis oculi muscle with occasional lid closure was evoked. Case 2 was a 72-year-old female with cerebellar hemorrhage. Neurological abnormal findings included mild disorientation, meningeal irritation signs, horizontal nystagmus on lateral gaze, reduced response of reflex eye movement to the left, minimal weakness of the left facial muscles, ataxic dysarthria, mild left hemiparesis and hemisensory deficits with face, truncal and limb ataxia. She could move eyeballs to any direction but with effort to the left.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Gaze-evoked eyelid closure. Report of two cases]. 261 99

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

Transient recurrent confusional and stuporous states of nonepileptic origin are clearly less frequent than epileptic ones. They are relatively common in diseases of disturbed vigilance, like narcolepsy, idiopathic hypersomnolence, and sleep apnea. These patients often suffer from attacks of hypovigilance, characterized by altered awareness, automatic behaviour and partial or complete amnesia for the attack. Because of the memory 'black outs' and the frequently associated hypnagogic hallucinations, the patients behave inappropriately and often appear confused. Confusional states also typically arise during basilar artery migraine attacks. This special form of complicated migraine predominantly affects young females and is characterized by symptoms and signs of brain stem dysfunction such as vertigo, ataxia, paresthesia, limb weakness, dysarthria; in 75% of the cases, disorders of consciousness dominate. Transient ischemic attacks are sometimes recurrent and, when involving the cranial basilar territory, may result in confusional states without significant motor dysfunction. Attacks of transient global amnesia are possibly also ischemic in nature and are assumed to arise from transient bilateral limbic failure. Affecting only memory functions, they are strictly spoken not confusional, but must nevertheless be taken into consideration when proper observation during the attack was not possible.
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PMID:[Non-epileptic impaired consciousness in neurologic diseases]. 267 60

A stroke with a somewhat unusual neurologic formula was correlated with a small linear high-intensity T2 focus in the lateral tegmentum of the lower third of the pons. The clinical features included dysarthria, staggering gait, incoordination of handwriting, right facial weakness, nystagmus, ocular overshoot, right appendicular ataxia, and left-sided dissociated sensory loss for pain and temperature. Lacunar lesions of the tegmentum in the region of the pontomedullary junction, while not uncommon, have not been studied pathologically and have not been reported in the magnetic resonance imaging literature, to my knowledge.
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PMID:Lacunar infarct of the tegmentum of the lower lateral pons. 271 52

A case of multiple sclerosis (MS) in a 39 year old Japanese female with high antibody titer to human T-lymphotropic virus type I (HTLV-I) in serum and cerebrospinal fluid (CSF) was reported. At the age of 15, gait and urinary disturbances accompanied by sensory impairment in the lower abdomen and lower extremities developed suddenly. The motor disturbance slowly progressed to tetraparesis over a period of 15 years with several remissions. At the age of 28 the patient suffered from an acute loss of visual acuity, unilateral initially, and became blind by the age of 34. On admission to our department in September, 1987, the patient was alert and had spastic paraparesis, the impairment of all sensory modalities below the level of Th 10 and urinary disturbance. Weakness of facial muscles, nystagmus, deviation of the tongue and dysarthria were also noted. By the PA method, the antibody titer to HTLV-I was measured x512 in the serum and x64 in the CSF. Western blotting analysis of the CSF disclosed the bands to p19, p24 and p28 of HTLV-I gag proteins. The CSF contained a few ATL-like atypical cells and showed mild lymphocytosis, but the total protein was not increased. The computed tomography revealed diffuse low density areas in the cerebral white matter. The magnetic resonance imaging disclosed high intensity signal areas in the cerebral white matter and in the dorsolateral portion of the medulla oblongata on the T2 weighted image. The link of HTLV-I infection to the pathogenesis of human demyelinating diseases was discussed.
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PMID:[A case of multiple sclerosis with high CSF antibody titer to HTLV-I]. 275 51

A 32 year old male is described with an onset of upper limb postural tremor in adolescence followed by muscle cramps. Progressive proximal amyotrophy and weakness in the limbs developed late in the third decade. Examination disclosed, in addition, bilateral facial weakness and mild dysarthria. Enzyme studies revealed hexosaminidase A and B deficiency, indicating a diagnosis of Sandhoff disease. Intra-axonal membranocytoplasmic bodies were present in a rectal biopsy. The presentation, which resembled that of X-linked bulbospinal neuronopathy, widens the clinical spectrum for disorders related to G(M2) gangliosidosis.
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PMID:Sandhoff disease mimicking adult-onset bulbospinal neuronopathy. 279 83


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