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Paget's disease with spinal cord compression. Favourable course after decompression and calcitonin treatment in two cases.

Two cases with spinal cord compression due to Paget's disease (osteodystrophia deformans) are reported, which were treated with calcitonin (Salmon Calcitonin) during 12 and 24 months after decompressive laminectomy. Both presented a favourable clinical course with improvement of sensory and bladder disturbances, paraparesis and pain. Diagnostic criteria as X-ray investigation, scintigraphy of bones, biochemical parameters and the long-term treatment whith calcitonin, are discussed.

Calcitonin↗

Psoriatic arthritis. Genetics and HLA antigens.

There is convincing evidence of a genetic basis for both psoriasis and psoriatic arthritis (PsA). Part of this genetic predisposition is due to genes within the major histocompatibility complex (MHC). In psoriasis, the primary association is with HLA-Cw6. Further work on specific nucleotide frequencies, especially those in the alpha 1 domain helix of the HLA-C molecule, will be of interest in determining whether a specific nucleotide frequency is present in all patients. The situation in PsA is considerably more complex. It is now established that there is an association between HLA-B27 and PsA, both in its peripheral arthropathy and in spinal disease in which radiological sacroiliitis is present. Spinal disease without radiological sacroiliitis is probably not associated with HLA-B27. There is some suggestion that HLA-B16 or its splits, HLA-B38 and HLA-B39, may also be associated with PsA, but there is considerable heterogeneity between the series, which prevents a firm conclusion being made. It is possible, but again not conclusive, that there is an association between HLA-DR4 and the symmetrical seronegative pattern of peripheral PsA. It is also likely that genes outwith the MHC predispose to psoriasis and PsA. It is further likely that a role will be found for environmental factors in both psoriasis and PsA. There is a tantalizing possibility of a complex interplay between a variety of environmental factors and genetic factors, both within and outwith the MHC, determining not only susceptibility but also the individual clinical pattern of disease. Further clarification of these possibilities is likely to depend primarily on understanding the role of genes within the MHC in predisposing to comparatively more homogeneous diseases, such as psoriasis and ankylosing spondylitis, before the mechanisms operating in PsA can be analysed and better understood.

Arthritis, Psoriatic↗

[The effect of combined treatment with the use of magnetotherapy on the systemic hemodynamics of patients with ischemic heart disease and spinal osteochondrosis].

The authors examined 66 patients with ischemic heart disease and concomitant cervico-thoracic osteochondrosis and 22 patients without osteochondrosis. Differences were revealed in values of the systemic hemodynamics with prevalence of the hypokinetic type in patients with combined pathology. Inclusion of magnetotherapy in the treatment complex of patients with ischemic heart disease and osteochondrosis favours clinical improvement, normalization of indices of central and regional blood circulation.

Adult↗

[The results of omentomyelopexy in the late period of traumatic spinal cord disease].

The results of omental transplantation to the site of spinal cord lesion in 40 patients in late injury are given. Neurological deficit was alleviated in 17.5% of patients. Improvement of segmentary functions was observed in most cases and was recorded within 1 week to 3 months postoperatively. There was no neurological improvement after 6 months postoperatively. Patients with mild spinal cord injuries (D10-L1) had the best outcomes. Comparative analysis of the outcomes of omentomyelopexy with those of treatment in 115 patients undergone meningomyeloradicolysis did not demonstrate any significant difference. Thus, it is not justifiable to use omental transposition in late spinal cord injury.

Adolescent↗

Spinal hydatid disease.

Vertebral hydatid cysts are rare and found in less than 1% of all the cases of hydatidosis. Neural compression is common in vertebral hydatidosis. The prognosis is generally regarded as very poor. This paper examines the natural history and complications which may arise during the treatment of vertebral hydatid cyst, and discusses their treatment. Thirteen cases of hydatid disease affecting the vertebrae are presented. The patients were admitted with symptoms of spinal cord compression. Twelve were treated by laminectomy and one by costotransversectomy. Low back pain radiating to the legs and lower extremity weakness were the predominant symptoms. Different degrees of pareses were present in 12 patients. Nine patients had impaired sensation in lower extremities. In 13 patients, 27 operations were performed. The major complication of surgery was the death of one patient due to the formaline irrigation. The surgical goal should be an extensive removal of the cysts and affected bone. The surgical area needs to be irrigated with hypertonic saline. Mebendazole or albendazole therapy seems to retard the recurrences and control the disease.

Adolescent↗

[Diagnosis of diseases of the spinal cord and the vertebral column].

Magnetic resonance imaging (MRI) nowadays plays a predominant role in the diagnosis and evaluation of spinal canal pathologies and has reduced the other exploratory methods, including computerized tomography (CT) and myelography, to an ancillary role. These pathologies are divided into three groups: those where MRI is the only imaging method (syringomyelia, tumours in the spinal canal, phakomatoses, external pachymeningitis, spinal cord injuries, myelitis); those where MRI is the initial method and is completed by other examinations (vascular malformations, dysraphism, myelopathies due to cervical osteoarthritis) and those where MRI still play a lesser role than CT (degenerative lesions of the lumbar column).

Humans↗

[Neuromuscular diseases (NME). I. Spinal muscular atrophy, peripheral nerve diseases, congenital myasthenia gravis].

Among the group of clinically and genetically heterogeneous spinal muscular atrophies(SMA), the autosomal recessive proximal types I-III are the most frequent. They are caused by mutations of the telomeric copy of the survival motor neuron gene (SMN1) on chromosome sq while loss of the centromeric copy (SMN2) does not lead to SMA. The conservation of exon 7 in the SMN2 copy seems to be crucial for possible causal therapy options. The genetic defect is also known for spinal muscular atrophy with respiratory distress. Hereditary polyneuropathies are caused by mutations in several genes on different chromosomes. Genetic and phenotypic heterogeneity must be considered. Data on clinical symptoms, inheritance, and neurophysiology are obligatory for the most effective molecular analysis. Hereditary congenital myasthenic syndromes are clinically, genetically, and pathogenetically heterogeneous. The diagnostic significance of molecular genetic analyses is still increasing. Therapeutic options include oral medication and assisted ventilation as needed.

Humans↗

Primary bone tumors of the femur presenting with spinal symptoms: a report of two cases and review of the literature.

Two case reports of femoral bone lesions simulating lumbar spinal disease are presented. Physical examination and case history were strongly suggestive of lumbar spinal pathology. In case 1, surgical resection of a venous hemangioma in the lumbar epidural space was performed but did not relieve pain. In case 2, conservative treatments for a protruded disk were performed for 3 months before an accurate diagnosis was made. After correct diagnoses were made, excision of the femoral tumors brought rapid relief of all abnormal findings in both cases. Compared with other causes of sciatica, femoral bone tumors are rare. However, careful attention should be paid to rule out these lesions if the diagnosis of a lumbar spinal disease is uncertain. Bone scintigraphy seems to be a sensitive diagnostic method to detect extraspinal osseous lesions.

Adult↗

Spinal cord disease in children with HIV-1 infection: a combined molecular biological and neuropathological study.

An autopsy study was performed on spinal cords from 18 children who died with HIV-1 infection, using standard histopathologic techniques as well as in situ hybridization and immunocytochemistry for HIV-1. Of 16 spinal cords examined by histology, nine had inflammatory cell infiltrates and six had multinucleated cells; both types of lesion are associated with the presence of HIV-1 in central nervous system tissue. HIV-1 type lesions were often present in the spinal cord and brain from the same patient. Pallor of myelin in corticospinal tracts in the cord was present in half of the cases; this change correlated with diffuse myelin pallor in the corresponding brains, but not with the HIV-1 associated changes in the cords. In situ hybridization for HIV-1 nucleic acid sequences gave positive results in seven of 18 spinal cords, with hybridizing signal usually localized to inflammatory cell infiltrates and multinucleated cells. Positive in situ hybridization, on frozen sections, correlated with the presence of HIV-1 associated changes on paraffin sections from the same cases. Immunocytochemistry for p25 core protein of HIV-1, using a monoclonal antibody on frozen sections, was positive in multinucleated cells, macrophages, and microglia. In this series there were two cases of vacuolar myelopathy, one a 30-month-old boy who had concomitant measles virus in the spinal cord grey matter, and the other nine-year-old girl who had severe HIV-1 infection of the cord. Other than the single case of measles virus, there were no opportunistic infections in the cords in this series. HIV-1 frequently involves the spinal cord in children with AIDS, while opportunistic infections are rare. Vacuolar myelopathy occurs in children with HIV-1 infection, although its occurrence is much less frequent than in adults with AIDS.

Acquired Immunodeficiency Syndrome↗

Combined medical and surgical treatment of spinal hydatid disease: a case report.

A case of chronic hydatid disease of the dorsal spine is described. The patient had undergone four previous operations over a 26 year period and had suffered recent further deterioration leading to complete paraplegia. On this occasion she was treated by a combination of drug therapy and surgery. She showed some neurological recovery after a two and a half month interval, without evidence of recurrence. The surgery would not have been feasible without concurrent drug therapy.

Adult↗

[Fluorotic cervical spinal cord disease].

Skeletal fluorosis was reported as a disease endemic to an area in the Madras Presidency of Indian in 1937 and prior to this, it was known as an occasional disease. There are two endemic areas in India, one in Punjab and the other in Andhra Pradesh State. This disease is also endemic in some parts of China and Africa. The manual labourers are more frequently affected as they consume huge quantities of water with high fluoride levels. The cervical spine is often affected, manifesting as cord compression. Forty cases of fluorotic cervical cord compression, who underwent surgery are studied regarding their clinical features, radiological aspects and outcome following surgery. In selected cases of cervical fluorotic myelopathy, surgical decompression appears to be necessary and also rewarding.

Adult↗

Clinical usefulness of an algorithm for the early diagnosis of spinal metastatic disease.

We have previously reported an algorithm that invokes several imaging modalities in the early detection of metastatic and benign disease of the spine in patients with cancer (J Clin Oncol 4:576, 1986). The development of new lesions (shown by Tc99m bone scans) in cancer patients with normal neurological examinations is further evaluated with plain radiographs, spinal computed tomography (CT), and CT myelography (CT-M). Of 60 patients in the original study, 28% were diagnosed as having only benign disease and the remainder had spinal metastases. Thecal sac impingement was seen in 47% of patients with metastatic disease and disruption of the posterior vertebral cortex was noted in all patients with epidural compression. We now report the 2-year follow-up of 55 of these patients. Without treatment, the 17 patients diagnosed with benign disease have shown no evidence of local failure in the spine and median survival is greater than 27 months. Thirty-eight patients diagnosed with spinal metastases had a median survival time of 16.9 months. Radiation therapy directed by CT-M findings provided pain relief in 78% of patients with back pain and metastatic disease. No patient, including 19 with thecal sac impingement, developed clinical myelopathy. These results demonstrate the usefulness of an imaging algorithm for the early identification and distinction of spinal metastatic disease and benign disease in patients with cancer.

Algorithms↗