Cases reported "Neurologic Manifestations"

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1/5. One-stage decompression and stabilization in the treatment of spinal tumors.

    Nowadays, extradural vertebral neoplasms which compress the spinal cord are being diagnosed earlier and therefore laminectomy for decompression relieves many patients of their neurological deficits before permanent cord damage has set in. In addition, radiotherapy and oncological treatment have prolonged life expectancy as well as the quality of life in these patients. As the indications for surgery have grown, the problem of spinal instability resulting from direct involvement of the supporting structures of the spine or due to the surgical procedure per se, must be considered. Many of these patients would rapidly return to leading their normal activities if not confined to bed rest or uncomfortable orthopedic devices. The Authors present 9 patients with extradural vertebral lesions: 4 plasmacytomas, 3 metastatic carcinomas, 1 malignant lymphoma and 1 aggressive osteoblastoma treated by simultaneous decompression and stabilization of the spine. All patients showed remarkable improvement of neurological symptoms except in one case where massive pulmonary embolism caused death a few hours after surgery. This surgical treatment offers the advantages of performing wider and better decompressive maneuvers and, at the same time, assuring fast mobilization of the patient after surgery who is often in poor general conditions, thus reducing post-operative complications due to delayed physical therapy and bed confinement.
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2/5. 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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3/5. sialic acid storage disease with sialuria: clinical and biochemical features in the severe infantile type.

    Two unrelated infants with a new disorder characterized biochemically by elevated levels of free sialic acid in urine, serum, and cell lysates have exhibited severe mental and physical impairments since the early weeks of life. Three other biochemically diagnosed cases and two possible cases from the earlier literature are reviewed to delineate this condition. Clinical features including sparse, white hair, coarse facies, hepatosplenomegaly, profound inactivity, diarrhea, and anemia permit early diagnosis of this neurovisceral storage disease. Osseous stippling may be present and clear vacuoles may be demonstrated in lymphocytes and cultured fibroblasts. The course is one of relentless deterioration with death in early childhood. Specific diagnosis depends on demonstration of elevated free sialic acid in urine and cell lysates.
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4/5. Neurological symptoms in achondroplastic dwarfs--surgical treatment.

    Fourteen patients who had neurological symptoms associated with achondroplastic dwarfism and who had spine surgery were analyzed, as were an additional twenty-nine cases reported in the literature. Four types of neurological patterns emerged, based on the onset, symptoms, and physical examination. Some etiological correlation between the anatomical lesion and the result was made in each category, and a reliable prognosis related to the pattern is now possible. Early and appropriately extensive surgery may well be recommended, based on the patients treated.
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5/5. lymphomatoid granulomatosis--a condition with affinities to Wegener's granulomatosis and lymphoma.

    A case of lymphomatoid granulomatosis of the lung is described in which the presenting features were a skin eruption and peripheral neuropathy. The onset of the pulmonary symptoms of breathlessness and productive cough was delayed nine months but, when apparent, the extent of the radiological changes contrasted with the mildness of the symptoms and the triviality of the physical signs. biopsy of the affected lung revealed a mixed lymphocytic, plasma cell, and histiocytic infiltrate following a perivascular distribution. This combination of clinical and pathological findings is in every detail that of lymphomatoid granulomatosis as recently identified by Liebow et al. (1972). Additional, previously undescribed, and unexplained findings in this case were persistent hypercalciuria and the presence in three axillary lymph nodes of subcapsular groups of cells resembling those of a benign naevus. This is the first case described in the British literature, and it is important that more cases be reported in order that the prevalence, prognosis, and aetiology of the condition should be further established.
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