Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C1762617 (weakness)
 37,932 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A patient, 30-year-old man, was admitted to Shinshu University Hospital with gait disturbance and numbness of both arms and legs in June 1985. General physical findings were normal except for facial asymmetry, i.e. mild right facial hemiatrophy. Blood pressure was normal and there were no signs of arteriosclerosis. Neurological examination revealed marked bilateral pyramidal tract signs, muscular weakness of all extremities predominant of the right side, spastic gait and sensory disturbance with impairment of pin prick and light touch sensation in the second and third trigeminal nerve regions and extremities of the left side, and mild loss of vibratory sense and numbness in all extremities. X-ray examination of the cervical spine and the myelogram of the spinal canal were normal. CT scan of the brain performed 3 hours after the myelography showed that the left vertebral artery extended from the left side of the medulla oblongata to the midline and distorted it by compression. MRI confirmed the findings of the CT scan. Vertebral angiography disclosed that the elongated left vertebral artery crossed the ventral aspect of the medulla oblongata. Compression of the medulla oblongata by the vertebral artery was diagnosed and neurovascular decompression was performed. About one month after the operation his spastic gait and muscular weakness were slightly improved, but these symptoms progressed thereafter. One year after operation his spastic gait was more marked than preoperatively. In many cases with dolichoectatic intracranial arteries, hypertension and arteriosclerosis have been reported to be usually associated with this condition. In other opinions dolichoectasia has been suggested to be a congenital disease, because such vessels are occasionally present in children.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[A case report of quadriparesis due to compression of the medulla oblongata by the elongated left vertebral artery]. 274 80

We report a case with paramedian thalamic and midbrain infarcts associated with palilalia. A 62-year-old man fell into a comatose state, and was admitted to an emergency hospital. Two days later, his consciousness level began to improve. Neurological examination revealed bilateral cerebellar ataxia, oculomotor nerve palsy, lack of spontaneity, and amnesia. Deep tendon reflexes were normal on all extremities, and no muscular weakness was observed. Babinski sign was noted on the right side. Muscle tone was decreased. No sensory disturbance was found. Two months later, he began to show compulsive repetition of syllables, words, or phrases, and was transferred to our hospital. He involuntarily repeated one syllable or a word five to ten times in spontaneous speech, repetition of phrases being not so frequent as that of syllables or words. This speech abnormality was considered as palilalia, which Souques first reported in 1908. The palilalia of this patient was rarely noted when he repeated words spoken to him by the examiner. As he spoke, the rate of speech gradually increased, the loudness reduced, and finally he began to whisper (palilalie aphone). The palilalia continued for four months. MRI showed infarcts in the medial thalami, subthalamic and midbrain on both sides. Auditory brainstem response showed delayed latency of bilateral V waves, and EEG revealed bilateral theta waves. In this patient no lesion other than thalamus, subthalamus and midbrain was detected by MRI. It is suggested that bilateral lesion of the thalami and their projection areas caused palilalia in this patient.
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PMID:[Paramedian thalamic and midbrain infarcts associated with palilalia]. 275 46

A woman aged fifty-three developed paraparesis at the age of 4, which progressed slowly and required crutches by the age of 30. At the age of 51, muscle stiffness involved bilateral hands and arms gradually. At the age of 53, she suffered from painful spasms in right deltoid muscle. Her two brothers had spastic paraplegia without other neurological deficits. Her paternal grandfather and maternal grandmother were cousins. Slight dementia was noted (WAIS: IQ, 79). Her posture was stiff and muscles of upper limbs were in a persistent contraction; Subcutaneous tissue was thin, and muscles were well-defined and firm. There was moderate muscle weakness of legs and hands. Continuous fasciculations and myokymias were recognized in muscles of the arms and the limb girdles. Muscle tone was considerably increased especially in the bilateral arms. The deep tendon reflexes were exaggerated with extensor plantar responses. Profuse sweating affected palms, soles and backs. No sensory disturbance was appreciated. There was no myotonic responses to percussion of muscles. Following laboratory data were normal; thyroid functions, CSF studies, anti HTLV-I antibody and long chain fatty acid in red blood cells, myelography and brain CT except for increased basal metabolic rate (53%). Electromyographic study in the arms and hands revealed spontaneous motor unit activities including doublets at rest and increased proportion of polyphasic potentials and high amplitude potentials in voluntary contraction. Biopsy of right quadriceps femoris muscle showed hypertrophy of type I fibers and angulated atrophy of type II fibers. Continuous muscle activities in upper limbs did not change at sleep or with intravenous administration of 7 mg diazepam.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Familial spastic paraplegia with syndrome of continuous muscle fiber activity (Isaacs)]. 280 25

The patient, a man aged 57, was admitted to our clinic on May 1, 1987, because of severe vertigo and unsteadiness in standing. Since the age of 55 he had been suffered from hypertension and atrial fibrillation. In September, 1986, he experienced vertigo but recovered soon without therapy. On April 25, 1987, while working, he noticed severe vertigo, nausea and vomiting. He was admitted to a hospital, and then transferred to our clinic. On admission, he was alert and the mentality was normal. Slight ptosis abducent nerve paresis, hypalgesia on the forehead, nose and cheek, facial paresis of peripheral type and hypacusis were detected on the left side. No anisocoria was observed. Sweating was impaired over the left side of the face. Elevation of the soft palate was limited on the left side and the tongue deviated to the left on protrusion. Dysarthria was detected. Though there was no muscular weakness in the extremities, incoordination and dysmetria were noted in the left arm and leg. He could not stand up because of vertigo and unsteadiness. There was no sensory disturbance in the trunk and extremities. Deep tendon reflexes were well elicited and no pathological reflex was observed. These clinical manifestations, except for the ipsilateral palatal and lingual disturbances, were typical of the lateral inferior pontine syndrome caused by occlusion of anterior inferior cerebellar artery, and the lesion was clearly demonstrated by horizontal and coronal MRI. The palatal and lingual disturbances might be due to the involvements of the corticobulbar tracts of the 10th and 12th nerves after the fibers had decussated.
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PMID:[Lateral inferior pontine syndrome: a case report]. 280 19

A 42-year-old woman with progressive myelopathy and mononeuritis multiplex is reported. The neurological examination on admission revealed hyperreflexia of the four extremities with pathological reflexes and moderate muscle weakness of the lower extremities with spasticity. Sensory disturbance was distributed on the areas of the bilateral lateral cutaneous femoral, the superficial peroneal and sural nerves. The antibody to HTLV-I in the serum and cerebrospinal fluid was more than 8192X and 512X, respectively. No sensory potential was recorded in the sensory conduction study of bilateral lateral femoral cutaneous nerves. Corticosteroid therapy caused a marked improvement of the sensory and urinary disturbances and had a slight effect on the spastic gait. Our nerve conduction study found small sensory potentials with normal conduction velocities in the bilateral lateral femoral cutaneous nerves. These results suggested the presence of an axonal degeneration in the peripheral nerves in this case. There have been no reports in the literature regarding a case of HAM with mononeuritis multiplex.
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PMID:[A case of HTLV-I associated myelopathy (HAM) complicated by mononeuritis multiplex]. 285 Jun 2

Symptomatic common carotid artery occlusion (CCAO) is rare. We studied 17 patients with ischemic cerebrovascular symptoms and unilateral CCAO on angiography to help clarify clinical and radiologic features. Mean age was 62 years; 65% were women. Predominant symptoms and signs included visual-ipsilateral monocular or retrochiasmal symptoms (88%), motor weakness (88%), sensory disturbance (59%), dizziness/lightheadedness (53%), and syncope (24%). Dysarthria, headache, or involuntary limb shaking occurred less frequently. Positionally related symptoms occurred in approximately two-thirds of the patients. TIAs were often multiple and preceded a stroke or occurred without subsequent stroke in 82%. Hemispheric TIAs contralateral to the CCAO occurred in 41%. Ten patients (59%) suffered stroke, seven (70%) of which were ipsilateral to the CCAO. Vascular risk factors included cigarette use (76%), hypertension (71%), diabetes mellitus (41%), and hyperlipidemia (41%); 82% had two or more risk factors. Known cardiac disease was present in 59%. CCAO was present at the origin of the vessel in most patients. Most had atherosclerotic narrowing of multiple extracranial large vessels. During follow-up, none of the patients had a spontaneous second infarct; five had TIAs, including two with amaurosis fugax, all in the CCAO territory. More restricted external carotid collaterals may, in part, explain the higher frequency of ipsilateral stroke and contralateral TIAs than reported for internal carotid occlusion.
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PMID:Common carotid artery occlusion. 279 68

Thermographic findings in 10 cases of syringomyelia were reported. 7 cases were associated with Arnold-Chiari malformation and 3 cases with spinal adhesive arachnoiditis. The cases included 3 men and 7 women, aged 7 to 56 years (mean age 27.7 years). Comparing the right side of the body with the left side, in 9 cases, thermography demonstrated asymmetric skin temperature of the trunk or extremities. MRI revealed laterality of the syrinx at the level of cervical or lumbo-sacral cord in 9 cases. The side with the lower temperature corresponded with the side of lateralized syrinx in 8 cases. Neurologically, 9 cases had laterality of sensory disturbance and 6 cases had asymmetric motor weakness. The side with the lower temperature also corresponded with the laterality of sensory disturbance in 8 cases, and the side of the motor weakness in 4 cases. Considering these findings, many of the patients with syringomyelia are thought to have asymmetric skin temperature. Intramedullary involvement of sympathetic pathway by the syrinx may cause such a change. As thermography is non-invasive and easy to carry out, it should be a useful method for estimating or even detecting cases of syringomyelia, especially when applied to out-patients.
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PMID:[Thermographic findings of syringomyelia]. 320 58

Autopsy cases of two brothers with bulbo-spinal muscular atrophy associated with gynecomastia, testicular atrophy and sensory neuropathy are reported. The disease started with finger tremor, proximal muscle weakness and facial muscle twitching at the second and fourth decades, accompanied by bulbar signs and glove-stocking type sensory disturbance. Systemic neurogenic patterns and diminished sensory nerve action potential amplitudes were recorded by electrophysiological studies. A marked loss of myelinated fibers was noticed upon sural nerve biopsy. Gonadal hormone values were normal, except for elevated urinary estrogen. Postmortem examinations revealed a remarkable degeneration of the facial and hypoglossal nuclei, and the spinal cord motoneurons. The skeletal muscles and the tongue showed neurogenic muscular atrophy with fatty replacement. Testicular atrophy was prominent showing hyalinized seminiferous tubuli with nodular and diffuse Leydig cell hyperplasia, containing estrogen immunoreactive substance. These clinical and histological features seemed to be highly compatible with those of Kennedy-Alter-Sung type bulbo-spinal muscular atrophy. The involvement of sensory peripheral nerves, however, was a distinct feature of this family.
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PMID:Familial bulbo-spinal muscular atrophy associated with testicular atrophy and sensory neuropathy (Kennedy-Alter-Sung syndrome). Autopsy case report of two brothers. 321 30

Five cases of histologically verified cavernous angiomas of the spinal cord are reported. Acute lower-extremity sensory disturbance was the initial symptom in four patients, and one presented with weakness of the hand. Progressive neurological deficit occurred in all patients, but the clinical course and outcome were extremely variable. Myelography revealed an intramedullary lesion in two cases but was completely normal in three; magnetic resonance imaging was diagnostic in these patients. Subtotal removal was accomplished in two cases, and myelotomy and biopsy were carried out in three. Four of the cavernous angiomas were located in the cervicothoracic region, whereas one was found in the thoracolumbar cord. All of the patients exhibited characteristic gross and microscopic features as well as hemosiderin-laden macrophages indicating remote hemorrhage. The diagnostic, therapeutic, and prognostic implications of this rare condition are discussed.
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PMID:Cavernous angiomas of the spinal cord. 333 9

The clinical features of 31 cases of syringomyelic syndrome, confirmed by metrizamide CT myelography (CTM) or magnetic resonance imaging (MRI), were analysed. Bilateral suspended sensory disturbance, previously regarded as a characteristic feature, was encountered in only 2 cases (6.5%). Thirteen patients (41.9%) showed no motor deficits of the upper limbs and 7 patients (22.6%) did not have weakness or atrophy in any of the four extremities. Cervical segmental signs were absent in 7 cases (22.6%) and in 2 (6.5%) signs were only present in the lower limbs. Scoliosis and foot deformity, such as pes cavus or equinovarus, were seen in 17 (54.8%) and 5 cases (16.1%) respectively. These skeletal abnormalities were initial symptoms in some childhood cases. In syringomyelic syndrome, clinical features are variable with many atypical clinical manifestations, apart from the previously recognized characteristic clinical features.
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PMID:Syringomyelic syndrome: clinical features in 31 cases confirmed by CT myelography or magnetic resonance imaging. 343 Jan 80


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