Cases reported "Neurologic Manifestations"

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1/13. Scleroderma cerebritis, an unusual manifestation of progressive systemic sclerosis.

    A 42-year-old female with scleroderma experienced two exacerbations in which behavioral changes were the main clinical features. On both occasions she presented with paranoid delusions, perceptual aberrations, and disorientation. After treatment with corticosteroids, the patient's mental status returned to normal, and her electroencephalogram showed an increase in alpha wave frequency, which is consistent with a resolving delirium. Unlike systemic lupus erythematosus, scleroderma rarely involves the central nervous system. This case illustrates an unusual manifestation of progressive systemic sclerosis, primary cerebral involvement which presented as an acute organic brain syndrome. connective tissue diseases, notably systemic lupus erythematosus, often present neuropsychiatric symptoms. Despite the fact that there appears to be a clinical and pathological continuum among the connective tissue diseases, an organic psychosis rarely occurs in progressive systemic sclerosis (scleroderma. Described here is a patient with scleroderma in whom behavioral abnormalities were the main features of two exacerbations of the disease.
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2/13. Giant-cell tumors of the spine and sacrum causing neurological symptoms.

    pain and neurological disturbances were the most frequent symptoms in patients with giant-cell tumors of the spine (4 cases) and sacrum (1 case). A good prognosis is possible in patients with vertebral tumor localization. There were no recurrences at follow-up 6 to 16 years after the first admission. The patient with a sacral tumor is alive more than 15 years after the first admission with some remaining neurological symptoms. Early surgical and/or radiation therapy is important. If institution of treatment is delayed more than three months after the onset of nerve root symptoms, there is a great risk of development of irreversible neurological lesions.
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3/13. neurologic manifestations of intravascular coagulation in patients with cancer. A clinicopathologic analysis of 12 cases.

    Among 1,459 autopsied patients with cancer, 12 had multifocal infarcts of the brain that appeared to be caused by intravascular coagulation. Most of these patients were women with leukemia or lymphoma, and all had a clinical course in which neurologic signs and symptoms were prominent. All had evidence of generalized brain disease (delirium and stupor or coma), and several also had focal brain disease (focal seizures, hemiparesis). All patients had laboratory evidence of coagulation abnormalities, although these were often not severe when neurologic symptoms began. Pathologically, there were multifocal hemorrhagic or ischemic infarcts in the distribution of several cerebral vessels, without a systemic source for cerebral emboli. fibrin thrombi were identified in cerebral vessels and in vessels of several other organs. The clinical findings fit the pathologic picture, and in most instances the correct diagnosis might have been made earlier had it been considered.
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4/13. Neurologic disturbances in Paget disease of bone: response to calcitonin.

    The neurologic manifestations of Paget disease and the therapeutic effect of calcitonin were studied in 49 patients. Twenty-four patients (49%) had neurologic disorders involving cranial nerves other than the auditory system, brainstem, spinal cord, or spinal roots and nerves. Eighteen of the 24 patients (75%) showed significant subjective or objective improvement after calcitonin treatment. The effect of calcitonin treatment on spinal cord compression was dramatic in three of six patients. The observations made of these patients support previous data suggesting that the neurologic signs and symptoms of Paget disease have their pathogenesis in both mechanical impingement and vascular distortion. The importance of early detection of neurologic signs and symptoms is emphasized, since prompt treatment with calcitonin may prevent severe complications.
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5/13. neurologic manifestations of cogan syndrome.

    cogan syndrome is a multisystem inflammatory vascular disease, characterized by nonsyphilitic interstitial keratitis and vestibuloauditory symptoms. Recent reports have directed attention to involvement of other organ systems. Respiratory, cardiovascular, gastrointestinal, and musculoskeletal problems are common, as are laboratory abnormalities and general symptoms such as fever, chills, and weight loss. Prominent neurologic problems in two patients prompted a review of 79 cases of cogan syndrome. More than half had nervous system involvement, including electroencephalographic or spinal fluid abnormality, headache, psychosis, coma, convulsion, neuropathy, and stroke. cogan syndrome should be considered when neurologic deficits are accompanied by eye, ear, and systemic symptoms.
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6/13. Hypertensive crisis.

    Although rare, hypertensive crises may rapidly lead to irreversible organ damage or death. Their prompt recognition, based primarily on physical signs and symptoms, is essential. Appropriately aggressive therapy, and evaluation of precipitating and exacerbating conditions, will often result in a satisfactory outcome.
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7/13. Electromyogram and nerve conduction in patients with acute intermittent porphyria.

    Diminished activity of uroporphyrinogen I-synthetase in the liver and other tissues may be regarded to be the primary genetic deficiency of acute intermittent porphyria (AIP). Increased production and renal excretion of delta-aminolevulinic acid (ALA) und porphobilinogen (PBG) are secondary phenomena. The neuropsychiatric symptomatology of AIP consists of neuropathy, vegetative crises and exogenous psychoses. In this study electromyographic and neurographic investigations were performed on 20 persons with AIP. 16 patients had experienced attacks of AIP, 10 of them including neuropathy. 4 persons showed the biochemical findings of AIP but had not yet had symptoms. In cases with persistent pareses following porphyric neuropathy denervation signs or sequelae were still present. In patients without clinical symptoms and in latent cases there were normal or borderline findings. Motor nerve conduction velocity was mostly decreased in combination with denervation signs and in a range that indicated a primarily axonal nerve lesion and consequent myelin damage rather than primary demyelinization. The mean motor conduction velocity of n. tibialis was somewhat lower in patients with porphyric crises without neuropathy than in latent cases without any clinical crises. The differences were not significant in other nerves. The findings are discussed under consideration of the electrodiagnostic results of other investigations and of neuropathological and clinical data.
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8/13. Neurological manifestations of Paget's disease.

    The neurological features of 96 patients whose skull and spinal x-rays showed the features of Paget's disease of bone were reviewed. The clinical phenomena were compared with those in another group of patients, identified by having a normal skull X-ray. Cranial nerve lesions and spinal syndromes could be attributed to the Paget's disease in many cases but the evidence did not suggest that other clinical phenomena such as dementia and epilepsy were related to the bony changes. Amongst cranial nerve lesions deafness is certainly attributable to Paget's disease, hemifacial spasm when combined with other signs is probably related, and possibly also trigeminal neuralgia. Basilar invagination can be asymptomatic, but deafness, hemifacial spasm and pyramidal signs were more prevalent in its presence. The importance of reviewing the cause and effect relationship between Paget's disease and neurological problems is discussed in the context of the therapeutic use of calcitonin.
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keywords = nerve
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9/13. Disseminated melanoma presenting as a catastrophic event.

    The possibility of a patient with malignant melanoma having a catastrophic event as the presenting sign of tumor dissemination cannot be dismissed. Should such an event occur, it would pose not only a risk to the patient, but also a potential risk to others. Since 1971, 712 patients with malignant melanoma have been evaluated. Twenty patients presented with brain metastases and an additional 12 patients developed brain metastases simultaneously with other organ involvement. Four patients (0.6%) had a catastrophic event, such as a stroke or seizure, with no antecedent symptoms. Microstaging of a primary melanoma by the methods of Clark and Breslow, in addition to the recognition of the presence or absence of regional lymph node metastases, provides reliable information for predicting the probability of tumor dissemination. patients with deep primary melanomas or with lymph node metastases should be advised regarding their participation in potentially hazardous occupations or recreations.
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10/13. Intracranial ceruminous gland adenocarcinoma.

    patients with adenocarcinoma of the ceruminous gland arising in the middle ear have a typical syndrome consisting of unilateral hearing loss, otalgia, facial paresis, and a middle-ear mass. Adjacent cranial nerves also may be affected. Some patients may have an ipsilateral cerebellar ataxia if the lesion extends into the cerebellopontine angle and compresses the cerebellar hemisphere. Obstructive hydrocephalus may occur secondary to obstruction of the aqueduct or fourth ventricle. The initial clinical findings may be those of a jugular foramen syndrome. These lesions are usually slow-growing and may be associated with a very prolonged clinical course. Although the tumors are rare, the physician should be aware of their existence if proper care is to be given.
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