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Spinal cord compression due to extramedullary haematopoiesis in two patients with thalassaemia: complete regression with blood transfusion therapy.

Extramedullary haematopoiesis, a common finding in thalassaemia, is rarely localized in the spinal cord and even more rarely has neurological manifestations. We present two patients suffering from thalassaemia (intermedia and beta homozygous) and paraparesis due to spinal cord compression from intrathoracic extramedullary haematopoietic masses. Diagnosis was based on magnetic resonance imaging findings, and treatment consisted of blood hypertransfusions. The majority of published cases have been successfully treated by radiation and in some cases by blood transfusions. Our two patients completely recovered, and there has been no recurrence during the 4 years since their treatment. Diagnosis and treatment of this rare condition are discussed.

Adult↗

Initial bolus of conventional versus high-dose dexamethasone in metastatic spinal cord compression.

We randomly assigned dexamethasone in an initial bolus of 10 mg IV or 100 mg IV followed by 16 mg daily orally to 37 patients with metastatic spinal cord compression. The average pain score before the start of treatment was 5.2 (SD = 2.8) and decreased significantly (p less than 0.001) to 3.8 at 3 hrs, 2.8 at 24 hrs, and 1.4 after 1 week. There were no differences between the conventional and high-dose group on pain, ambulation, or bladder function.

Adult↗

Extradural spinal cord compression by rheumatoid nodule.

Rheumatoid disease is a systemic disorder affecting multiple organs. It is known to affect the nervous system in a variety of ways, but its presentation with spinal cord compression by a rheumatoid nodule is rare. We report two cases presenting with cord compression by a rheumatoid nodule who underwent surgical decompression with good recovery.

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[Diffuse eosinophilic fasciitis (Shulman's syndrome). Apropos of a case disclosed by spinal cord compression].

The case of Shulman's syndrome reported in this article is unusual, because of the topography of the lesions and the lethal course. The perivertebral fasciitis, which was pseudotumoral in appearance, induced a myelomalacia by compression of the cervical spinal cord. In addition, the autopsy revealed severe tissue eosinophilia, cutaneous sclerodermiform sclerosis and pharyngoesophageal lesions unusual in this disease. The pathogenesis of the syndrome is discussed.

Adult↗

High incidence of serious side effects of high-dose dexamethasone treatment in patients with epidural spinal cord compression.

Twenty-eight consecutive patients were given high-dose dexamethasone (96 mg i.v. loading dose, decreasing doses to zero in 14 days) and radiotherapy for epidural spinal cord compression due to malignant disease. There were eight events classified as side effects of the dexamethasone treatment. Four of these were considered as serious (one fatal ulcer with haemorrhage, one rectal bleeding and one gastrointestinal perforation from undetermined origins, and one perforation of the sigmoid colon) giving a total rate of serious side effects of 14.3 percent. Due to the high incidence of serious side effects of the high dexamethasone dose, the regimen was abandoned in favor of a standard dexamethasone dose of 16 mg daily reduced to zero in 14 days. There were three events classified as side effects, but none were considered as serious in 38 consecutive patients receiving this dose. The differences both in total number of side effects and number of serious side effects are statistically significant. There was no significant difference in the number of ambulant patients in the group that received the high dexamethasone dose. We conclude that the high dexamethasone dose in our experience gives an unacceptably high incidence of serious side effects and we have therefore abandoned the regimen in favour of a more standard dexamethasone dose.

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Cervical spinal cord compression attributable to a calcified intervertebral disc in a patient with X-linked hypophosphatemic rickets: case report and review of the literature.

OBJECTIVE AND IMPORTANCE: X-linked hypophosphatemic rickets is a common inherited phosphate-wasting disorder, but it is a rare cause of spinal cord compression. We present the first reported case of a calcified intervertebral disc causing spinal canal stenosis in X-linked hypophosphatemic rickets. CLINICAL PRESENTATION: A 44-year-old woman presented with paresthesia of her left arm and a loss of grip in both hands. Magnetic resonance imaging revealed a calcified intervertebral disc, as well as a posterior osteophytic bar causing marked cervical cord compression at C6/C7. INTERVENTION: An anterior cervical discectomy at C6/C7 and fusion with autologous bone graft were performed. The patient then exhibited significant improvement. CONCLUSION: A review of the 16 published cases demonstrates that thickening of the vertebral laminae, facet joint hypertrophy, and ossification of the intervertebral discs, posterior longitudinal ligament, and/or ligamentum flavum contribute to spinal canal stenosis in X-linked hypophosphatemic rickets. Those changes are caused by the disease itself and are unlikely to be related to long-term vitamin D treatment. Eleven of 16 patients were reported to have experienced favorable outcomes after surgery.

Adult↗

Atlantoaxial cartilaginous exostosis causing spinal cord compression in a mature Bernese mountain dog.

Cartilaginous exostosis developed in the atlantoaxial region of a three-and-a-half-year-old Bernese mountain dog. The dog exhibited ataxia in the hindlimbs and flailing movements in the forelimbs. On survey radiographs of the cervical spine there was a focal calcified mass between the dorsal arch of the atlas and the spinous process of the axis. Lumbar myelography revealed severe dorsal spinal cord compression. The mass was removed surgically and the dog made a complete recovery. Histopathology of the excised mass was consistent with a diagnosis of cartilaginous exostosis.

Animals↗

Spinal cord compression of primary extragonadal giant yolk sac tumor.

STUDY DESIGN: Case report. OBJECTIVE: To report an adult male patient with a primary extragonadal giant yolk sac tumor presenting with acute spinal cord compression. SETTING: Faculty of Medicine, University of Dicle, Diyarbakir, Turkey. METHOD: A 31-year-old man was referred to our department with a diagnosis of Pott's disease, a complaint of back pain and gait difficulty for 2 weeks. Neurological examination showed spastic paraparesis and hypoesthesia below the L2 dermatome level. He also had urinary incontinence. Abdominal computed tomography and lumbar magnetic resonance imaging study revealed a giant cystic mass lesion located in the psoas muscle. Posteriorly, the third lumbar vertebral body was destructed and the tumor was compressing the dural sac. A combined anterior and posterior approach was performed. Pathological diagnosis was a yolk sac tumor. RESULT: His neurological status improved during the postoperative course. A chemotherapy protocol was given including bleomycin, etoposide and cisplatin. Five months after the last chemotherapy, he was brought to the emergency unit in sepsis and died despite antibiotherapy. CONCLUSION: Yolk sac tumor should be considered in young adult male patients presenting with acute paraparesis even without any signs or medical history of a testis tumor. These tumors may be unresectable; however, decompression of neural structures and stabilization of the spine with instrumentation may cause substantial improvement in neurological deficit and pain relief.

Adult↗

Granulocytic sarcoma (chloroma) causing spinal cord compression.

Granulocytic sarcoma (chloroma) is a rare solid tumor of myelogenous stem cells, usually appearing in patients with acute myelogenous leukemia and less commonly in patients with chronic myelogenous leukemia or myeloproliferative disorders. We present a spinal epidural granulocytic sarcoma causing thoracic spinal cord compression in a patient with chronic anemia secondary to myelofibrosis.

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