Extramedullary hematopoiesis and paraplegia in a patient with hemoglobin e-Beta thalassemia.

Extramedullary hematopoiesis (EMH) occurs in patients with various hematologic disorders involving a chronic increase in the production of red blood cells, and is often associated polycythemia vera and sickle cell anaemia, but is less common with thalassemia especially with hemoglobin E-beta thalassemia. Spinal cord compression due to EMH is a extremely rare complication of thalassemia and may present with paraparesis or paraplegia with or without sensory impairment. Treatment options mostly include surgery and/or radiotherapy. Whereas cases presenting with paraplegia have been treated with either surgery or radiotherapy with equal frequency and efficacy, almost all reported cases with paraplegia have been treated with surgery with or without radiation therapy. We hereby report a case of hemoglobin E-beta thalassemia with paraplegia treated successfully with radiotherapy.

Paraplegia of spinal epidural compression by metastatic breast cancer and urgent radiotherapy-timeliness for naught?

Four patients who became paraplegic because of spinal epidural compression by metastatic breast cancer were treated for palliation by external beam radiation. None of the four regained ambulation after therapy. Our findings place in question the urgent need for radiotherapy in these paralytic people with the disorder, especially when they are pain-free.

Intraoperative radiotherapy combined with posterior decompression and stabilization for non-ambulant paralytic patients due to spinal metastasis.

STUDY DESIGN: Retrospective examination of 96 nonambulant paralytic patients with spinal cord compression caused by metastatic cancer treated with intraoperative radiotherapy combined with conventional posterior surgery. OBJECTIVE: To improve local control of spinal metastasis by conducting posterior surgery combined with intraoperative radiotherapy (IORT) in patients with severe neurologic deficits. SUMMARY OF BACKGROUND DATA: Few studies of conventional posterior surgery demonstrated satisfactory neurologic recovery for nonambulant paralytic patients with advanced spinal metastases. METHODS: Ninety-six patients underwent IORT (107 procedures) for the treatment of severe spinal cord compression because of spinal metastases. All patients were nonambulatory before surgery. Eighty-three cases (86%) were in an advanced stage of multiple spinal metastases (types 6 or 7 of the surgical classification of vertebral tumors). After posterior decompression, a single large dose of electron beam irradiation was delivered to the exposed metastatic lesion while the spinal cord was protected using a lead shield. Posterior instrumentation was also performed for most patients. RESULTS: Ninety-five of 107 cases (89%) obtained at least one level of neurologic improvement according to Frankel's classification and 86 cases (80%) became ambulatory after surgery. The main factors related to a nonambulatory status after surgery were preoperative neurologic status, performance status, and the presence of internal organ metastases. Of 86 postoperative ambulatory cases, only 3 became nonambulatory because of local recurrence during the follow-up period. CONCLUSION: The IORT procedure is a useful technique for the treatment of spinal cord compression because of spinal metastasis, offering significant neurologic recovery and a low rate of local recurrence.

Management of epidural cauda equina disease of prostate cancer by radiotherapy.

OBJECTIVE: Secondary prostate cancer affection of the cauda equina (SPCCE) can be an ultimate cause of morbidity and mortality. Since the results of management of this particular disease condition remain largely unknown, a retrospective case review was undertaken to determine the effects of treatment by radiation in SPCCE patients. METHODS: The records of 12 patients with SPCCE treated at the Division of Therapeutic Radiology during the period 1984 to 1998 were reviewed. The administered total radiation dosage ranged from 6 Gy to 32 Gy (average, 26.6 +/- 2.0 Gy). Two individuals underwent decompressive laminectomy and bilateral orchiectomy at the time of SPCCE and prostate cancer diagnoses. Ten patients had prior hormonal manipulative treatment (orchiectomy, estrogen, or Flutamide therapy). RESULTS: Pain was relieved in three of four symptomatic patients (75%). Five of nine patients unable to walk before therapy could walk after treatment. One of two individuals with anal or bladder sphincter dysfunction improved following irradiation. The overall mean duration of survival was five months. With treatment, survival was approximately three times as long for ambulatory versus non-ambulatory patients. CONCLUSION: We conclude that radiation treatment is efficacious in promoting palliation of SPCCE, although it may not prolong life.

Effects of spinal cord X-irradiation on the recovery of paraplegic rats.

Axonal regrowth is limited in the adult CNS, especially in the spinal cord, one of the major sites of traumatic lesions. Pathophysiological changes occurring after spinal cord injury include complex acute, subacute, and late processes. In this study, we assessed whether X-irradiation interferes with the acute/subacute phases, thereby improving the functional recovery of paraplegic animals. Two days after acute compression of adult rat spinal cords, various doses (0, 2, 5, 10, 20 Gy) of X-rays were administered as one single dose to the compression site. The animals were functionally evaluated over the course of 1 month after injury, using the Tarlov scale and the Rivlin and Tator scale. We also designed a "physiological" scale, including an assessment of urinary function and infection, appropriate for the evaluation of spinal-cord-lesioned animals. Behavioral analysis suggested that the high doses, 20 Gy and, to a lesser extent, 5 and 10 Gy, were toxic, as shown by morbidity rate and "physiological" score. The 2-Gy group showed better motor performances than the lesioned nonirradiated (LNI) animals and the 5- and 20-Gy groups. Motor performance in the 5-, 10-, and 20-Gy groups was poorer than that seen in the LNI group. Gliosis was reduced in the 2-Gy group compared to LNI animals, and there was high levels of gliosis in the highly (>/=5 Gy) irradiated animals. There was a 23% less lesion-induced syringomyelia in the 2-Gy group than in the other groups (LNI and 5-20 Gy). Thus, low doses of X-rays may interfere with the formation of syringomyelia and glial scar, thereby facilitating the recovery of paraplegic animals. These findings suggest that low-dose irradiation of the lesion site, in association with other therapies, is a potentially promising treatment for improving recovery after spinal cord injury.

Effectiveness of radiation therapy without surgery in metastatic spinal cord compression: final results from a prospective trial.

PURPOSE: In assessing effectiveness of radiation therapy (RT) in metastatic spinal cord compression (MSCC), we performed a prospective trial in which patients with this complication were generally treated with RT plus steroids, and surgery was reserved for selected cases. METHODS AND MATERIALS: Two hundred seventy-five consecutive patients with MSCC entered this protocol. Twenty (7%) underwent surgery plus RT, another 255 received RT alone. Of all eligible patients, 25 (10%) early deaths and 21 (8%) entering a feasibility study of RT without steroids, were not evaluable. Of the 209 evaluable cases, 110 were females and 99 males, and median age was 62 years. Median follow-up was 49 months (range, 13 to 88) and treatment consisted of 30 Gy RT (using two different schedules) together with steroids (standard or high doses, depending on motor deficit severity). Response was assessed according to back pain and motor and bladder function before and after therapy. RESULTS: Back pain total response rate was 82% (complete or partial response or stable pain, 54, 17, or 11%, respectively). About three-fourths of the patients (76%) achieved full recovery or preservation of walking ability and 44% with sphincter dysfunction improved. Early diagnosis was the most important response predictor so that a large majority of patients able to walk and with good bladder function maintained these capacities. When diagnosis was late, tumors with favorable histologies (i.e., myeloma, breast, and prostate carcinomas) above all responded to RT. Duration of response was also influenced by histology. Favorable histologies are associated to higher median response (myeloma, breast, and prostate carcinomas, 16, 12, and 10 months, respectively). Median survival time was 6 months, with a 28% probability of survival for 1 year. Survival time was longer for patients able to walk before and/or after RT, those with favourable histologies, and females. There was agreement between patient survival and duration of response, systemic relapse of disease being generally the cause of death. CONCLUSION: Early diagnosis of MSCC was a powerful predictor of outcome. Primary tumor histology had weight only when patients were nonwalking, paraplegic, or had bladder dysfunction. The effectiveness of RT plus steroids in MSCC emerged in our trial. The most important factors positively conditioning our results were: the high rate of early diagnoses (52%) and the number of tumors with favorable histologies (124 out of 209, 63%) recruited, and the choice of best treatment based on appropriate patient selection for surgery and RT or RT alone.

[Radiotherapy of paraplegia caused by extramedullary hematopoietic tissue]. / Zur Radiotherapie der durch extramedulläres hämatopoetisches Gewebe bedingten Paraplegien.

The presentation by computed tomography of an extramedullar epidural intraspinal hemopoisis has only been described sporadically. It has been possible to visualize by CT-scans the regression under radiotherapy of a sensory and motor paraplegia in a 17 years old patient with beta-thalassemia major. The central nervous system (thoracic and lumbar zone as well as os sacrum) was exposed to a total reference dose of 30 Gy which was administered within twenty days by a 60Co unit in single doses of 2 Gy each. From 4 Gy on, the paraplegic symptoms regressed continuously. The control CT taken after 26 Gy- the paraplegia had completely disappeared at this time-showed a significant regression of the intraspinal soft tissue masses. Radiotherapy is very important in the treatment of this disease. A direct correlation is found between clinical symptoms and CT-presentation.

Percutaneous radiofrequency foramenal rhizotomies.

Thirty quadriplegic and paraplegic patients with intractable spasticity underwent percutaneous radiofrequency foramenal rhizotomies. This produced improvement in 94% of the patients, with excellent results in 73%. The complication rate was 3%. The major disadvantage of the procedure was recurrent spasticity, which was dealt with by simple repetition of the procedure. Rehabilitative goals were facilitated; needless painful disability was avoided; a need for intense nursing was reduced; the prevention and treatment of decubitus ulcer formation was enhanced. The study suggests that this procedure is effective, inexpensive, and at low risk to the patient.