Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UNIPROT:P50583 (asymmetrical)
 12,197 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We report a patient with asymmetrical patchy weakness of the limbs, and with autoantibodies against gangliosides GM1, GD1b, asialo GM1. Although electrophysiological studies did not reveal conduction block, treatment with prednisolone resulted in clinical improvement. A 52-year-old man was admitted to Kyoto University Hospital, because of gait disturbance. Neurological examination revealed a patchy distribution of weakness in the limbs. Deep tendon reflex was normal at the right knee, and was depressed at the right biceps. Other deep tendon reflexes were absent. There was a slight decrease in vibratory sensation in the distal portions of the lower extremities. Routine laboratory studies, heavy metal screen, vitamin, cryoglobulin, coproporphyrin and delta-amino levulinic acid in urine, and the protein value of the cerebrospinal fluid were normal. Head and neck MRI, and myelography were normal. Immunofixation electrophoresis showed IgM lambda M-protein in serum. Thin-layer chromatography with immunostaining showed his serum IgM reacted with GM1, GD1b, and asialo GM1. ELISA (Enzyme Linked Immunosorbent Assay) demonstrated high titers of anti GM1, GD1b and low titer of anti asialo GM1. Motor conduction studies showed no demonstrable conduction block, normal conduction velocities and the low amplitudes of CMAP. Sensory conduction studies showed no abnormalities except for slightly decreased amplitude of SNAP in sural nerve. Electromyography showed active denervation in extensor digitorum communis muscle, tibialis anterior muscle and left biceps brachii muscle. Muscle biopsy specimen revealed large and small group atrophy and there was perivascular mononuclear infiltration at one point.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Multifocal axonal motor neuropathy associated with anti-ganglioside antibodies]. 129 59

Hepatic parenchyma may hypertrophy following asymmetrical injury. The histologic characteristics of hypertrophic hepatic parenchyma are more similar to normal hepatic parenchyma than is the more severely damaged liver. We present four cases where large hypertrophic masses resembled neoplasm on other imaging modalities or at surgery, but had MRI signal characteristics similar to those of normal liver.
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PMID:Mass-like hepatic hypertrophy: MRI findings with histologic correlation. 150 24

A progressive disorder of relatively focal but asymmetric biposterior dysfunction is described in a 54 year old right handed male. Initial clinical features included letter-by-letter alexia, visual anomia, acalculia, mild agraphia, constructional apraxia, and visuospatial compromise. Serial testing demonstrated relentless deterioration with additional development of transcortical sensory aphasia, Gerstmann's tetrad, and severe visuoperceptual impairment. Amnesia was not an early clinical feature. Judgment, personality, insight, and awareness remained preserved throughout most of the clinical course. Extinction in the right visual field to bilateral stimulation was the sole neurological abnormality. Early CT was normal and late MRI showed asymmetrical bioccipitoparietal atrophy with greater involvement of the left hemisphere. Results from positron emission tomography (PET) showed bilaterally asymmetric (left greater than right) occipitotemporoparietal hypometabolism. The metabolic decrement was strikingly asymmetric with a 50% reduction in glucose consumption confined to the left occipital cortex. The picture of occipitotemporoparietal compromise verified by MRI, PET, and neurobehavioural testing would be unusual for such degenerative dementias as Alzheimer's (AD) and Pick's disease, although atypical AD with predominant occipital lobe involvement cannot be excluded. This case supports the concepts of posterior cortical dementia (PCD) as a clinically distinct entity and for the first time documents its corresponding metabolic deficit using PET.
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PMID:Posterior cortical dementia with alexia: neurobehavioural, MRI, and PET findings. 186 9

Nine cases of post-herpetic encephalitis with predominant involvement of one temporal lobe at CT scan or MRI (6 on the left and 3 on the right sides) were studied 1 to 6 years after onset with repeated language and memory testing. The neuropsychological findings were well correlated with the lateralization and size of the lesions, as previously observed in unilateral temporal lobectomy. Compared with the usual bilateral form, the sequelae were mild, and all the patients, especially those with right hemisphere involvement, resumed a subnormal family life or social activity. In patients with left predominant lesions the anterograde amnesia concerned verbal information, especially logical and abstract, without visual amnesia. In some cases, the episodic verbal amnesia was associated with a semantic deficit which included the knowledge of some words. In one patient the colour, use and mental imagery of some objects corresponding to forgotten words were involved only for some specific groups (natural objects, whereas man-made objects fared better). Testing of verbal memory is proposed to determine the role of the preserved minor hemisphere in learning the context of word presentation, and words with concrete and visual connections which are better recalled than those with abstract or logical link. Disorders of behaviour or mood are usual in the 2 groups of lesions. Thus, herpes encephalitis in these asymmetrical and benign forms in an attractive model to study the role played by the temporal lobe and lateralization in memory.
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PMID:[Primarily unilateral herpes encephalitis. Long-term neuropsychological study of 9 cases]. 207 16

Long survival in subacute sclerosing panencephalitis (SSPE), including total disappearance of clinical signs, is rare. Two cases are reported. They concern a girl and a boy who, at age 13 and 15, developed SSPE and are still in remission 6 and 5 years later. After a typical onset and course over periods of 12 and 18 months, clinical improvement was observed and periodic EEG complexes disappeared. However, the electrophoretic oligoclonal pattern of CSF proteins and the elevated measles titers persisted (in one case specific CSF IgM were still increased 6 years after the onset). MRI showed asymmetrical areas of high-intensity signal in both white and gray matter, predominant in the temporal, parietal and occipital regions. The age at which SSPE begins and the interval between measles and SSPE onset are not prognostic factors. On the other hand, in reported cases with lasting remission SSPE did not progress beyond Jabbour's stage II. The second typical feature of these long-term improvements is disappearance of EEG periodic complexes and emergence of a normal basic background activity. No other prognostic factor has been reported.
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PMID:[Prolonged remission in subacute sclerosing panencephalitis: 2 cases]. 218 84

The clinical presentation and radiological features were analyzed in 30 cases of syringomyelia associated with Chiari malformation. None of the patients had spinal dysraphism. The age on admission ranged from 6 to 59 years with a mean of 27 years. Syringomyelia was diagnosed by CT myelography and or MRI from 1982 to 1988. The initial symptoms were skeletal abnormality (43%) such as scoliosis (12 cases) or pes cavus (one case), unilateral pain or numbness (40%) and unilateral motor weakness (17%). Frequently seen signs on admission were sensory deficit (100%), scoliosis (57%), muscle weakness (57%), muscle atrophy (37%) and lower cranial nerve palsy (40%). The neurological findings were asymmetrical in all patients. The characteristic neurological findings in the cases presenting under 20 years of age were unilateral sensory and motor deficits (61%) with decreased or absent deep tendon reflex on the same side. The localization of the syrinx in axial section varied according to the level even in the same case. In 15 cases with unilateral sensory disturbance or unilateral sensory and motor deficit, the syrinx was located in the region corresponding to the posterolateral portion on the same side as that of sensory disturbance in the cervical or thoracic level. On the other hand, in 15 cases with bilateral sensory and motor deficit, the syrinx was located in the central portion and extended into the posterolateral portion of the more affected side.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Clinical and neuroradiological features of syringomyelia associated with Chiari malformation]. 233 91

The authors report two children with isolated occipital lobe anomalies detected by visual evoked potentials (VEPs) and confirmed by MRI and CT scanning. Both had a markedly asymmetrical occipital distribution of flash and pattern VEPs. One child had acuity reduced to 6/36, and testing on confrontation suggested an homonymous hemianopia. The second child was visually inattentive to one side during infancy. Neither child had band atrophy of the optic disc or an afferent pupillary defect. Isolated abnormalities of the occipital lobes are difficult to detect by clinical examination during infancy and early childhood. Recording VEPs from a horizontal array of occipital electrodes can be helpful in detecting subtle occipital lobe abnormalities.
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PMID:Detection of isolated occipital lobe anomalies during early childhood. 235 56

Thoracic spondylotic myelopathies are exceptional, only 29 observations could be found in the literature; we intend to describe three new cases here. The patients, two women and one man, 64, 69 and 72 years old, complained of weakness of the lower limbs, more marked on one side, which had been progressing slowly from several months to eight years. Examination revealed asymmetrical paraparesis with distal sensitivity deficits without thoracic sensory level. In the first case, the myelography remained virtually unchanged in front of T11, T12; in the second and third cases, there was slight extradural compression at T9 and T10 respectively. Magnetic Resonance Imaging (M.R.I.) performed in two patients was evocative of a thoracic disk herniation. A chest CT scan enabled us to establish correct diagnosis: in the three cases irregular hypertrophy of the posterior elements was evident at T11 and T12, T9 and T10, T10 and T11 respectively, with osteophytes originating in the articular process and deeply embedded in the spinal canal. Decompressive laminectomy associated with medial facetectomy resulted in the gradual improvement of walking in all three patients. Myelography and MRI are both useful in demonstrating the level compression, usually situated in the low thoracic spine, however only the CT allows differential diagnosis with other etiologies, especially anterior compression such as disk herniation.
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PMID:[Myelopathies caused by dorsal spinal canal spondylotic stenosis. 3 cases and a review of the literature]. 269 79

Bi-lateral force measurements on the supporting limbs in postural sway while standing still were carried out to evaluate postural stability of craniocerebral injured (CCI) patients. Brain pathology of these patients was characterised by CT scans and MRI, as well as by their cognitive and behavioural disturbances. Normal subjects of the same age group were tested as controls. From the force tracings obtained, three oscillation frequencies were identified, with orders of magnitudes of 6, 1.5 and 0.1 Hz, respectively, of which the middle frequency, i.e. that corresponding to 1.5 Hz, was selected for subsequent processing and analysis, which included the determination of relative sequence of the force vectors on both feet and evaluation of timings and amplitudes of the waveforms. Weight-bearing imbalance was defined in the vertical direction to express the difference between the average forces supported by each of the legs. In the horizontal plane, two parameters were defined: Sway Total Activity (SA), to represent the vector summation of the absolute values of the horizontal force components acting on both legs; and Asymmetry (ASYM), to express the difference in activities between the two legs. The results presented disclose the reactive force patterns acting on each of the legs of CCI individuals, in comparison with normal individuals. Although the forces were shown to act synchronously on both legs, in most of the patients, they appeared to be asymmetrical in nature, with a typical vectorial pattern for every individual, which generally differed from that of normal subjects. Sway activity was found to be significantly higher in the CCI group as compared with that of the normal controls. These results justify the necessity to study the activity of each of the legs, rather than that corresponding to the resultant forces acting on the whole body, when treating the external forces involved in the stabilisation and regulation of the standing posture of these patients. An attempt to grade the patients according to ascending order of neurological deficits disclosed that their sway disturbances, as revealed by the magnitude of sway total activity and asymmetry, had a tendency to be related to the severity of neurobehavioural disturbances.
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PMID:Standing posture of craniocerebral injured patients: bi-lateral reactive force patterns. 271 58

The authors retrospectively studied the electroencephalograms (EEGs) of 12 patients with hemimegalencephaly-a unilateral brain malformation which is often overlooked, despite having typical CT-scan and MRI aspects. They were characterized by three types of abnormal tracing, which correlated with prognosis: triphasic complexes of large amplitude were observed in patients with the earliest onset of seizures, and were associated with the most severe prognosis; unilateral, rhythmic 'alpha-like' activity was recorded in patients with seizures occurring after three months of age, and was associated with a relatively favourable outcome; asymmetrical suppression-bursts characterised by 'alpha-like' activity on the abnormal side were seen in patients with infantile spasms. The EEG pattern seems to make an important contribution to both diagnosis and prognosis.
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PMID:Electroencephalographic aspects of hemimegalencephaly. 259 76


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