Functional Assessment of Lower Extremities in Hereditary Spastic Paraplegia

Braschinsky M, Parts K, Maamägi H, Gross-Paju K, Haldre S. Functional assessment of lower extremities in hereditary spastic paraplegia.

Objectives

To characterize the spasticity and range of motion (ROM) in patients with hereditary spastic paraplegia (HSP) and to correlate these parameters with walking speed.

Design

An observational population-based cohort study.

Setting

Patient data were acquired from a population-based epidemiologic study performed earlier in Estonia.

Participants

Persons (N=46) (mean age, 50.1y) with clinically confirmed HSP diagnosis (mean duration, 20.9y) participated in the study.

Interventions

Active and passive ROMs were measured with a plastic 360° goniometer. Spasticity was evaluated by using the modified Ashworth scale (MAS). The time it took a patient to walk 10m was recorded.

Main Outcome Measures

Measurements included testing of active and passive ROM as a marker for mobility, the MAS for spasticity, and time to complete a 10-m walk.

Results

A higher degree of spasticity in hip muscles was associated with lower values of active ROM and slower walking. Walking speed was negatively correlated to disease duration and participant age.

Conclusions

The present study provides analysis of the contributions of spasticity and ROM to walking speed in HSP, both factors negatively influence gait in persons with HSP.     

Medulloblastoma/primitive neuroectodermal tumor in adults: prognostic factors and treatment results: a single-center experience from Turkey

We performed retrospective review of 29 adult patients with cerebellar medulloblastoma/primitive neuroectodermal tumor (PNET) who received craniospinal radiotherapy in Ankara Oncology Hospital between years 2000 and 2005. All patients were operated followed by craniospinal irradiation; 11 of 29 patients also received chemotherapy. All patients had no distant or spinal metastases at the time of diagnosis. Median follow up time was 26 months. Progression-free survival was 86% at 2 years, 55% at 5 years. Mean progression-free survival was 25 months in patients with PNET; 61.4 months in patients with medulloblastoma (P = 0.0016). Mean survival was 61.33% months in patients <25 age, 38 months in patients >25 age. (P = 0.04). Overall mean survival was 59.80 months in patients who received chemotherapy and 41.4 months in patients who did not have chemotherapy (P = 0.15). Cranial relapses were observed in 3 of 29 patients, and 3 of 29 patients had distant metastases. The mean time to cranial recurrence was 19 months; to distant metastases was 18 months. In conclusion, adult patients with PNET have worse survival rates than patients with medulloblastoma, like in childhood patients. Patients younger than 25 years of age also had statistically significant better survival.     

Clinical characteristics and outcome of patients with stage III esophageal carcinoma: a single-center experience from Turkey

Esophageal carcinoma is an extremely deadly disease, and prognosis is poor. We retrospectively evaluated stage III esophageal carcinoma patients in our center. Median age of the patients was 52. Men to women ratio were 3/1. Epidermoid carcinoma was the major histology. Incidence of esophageal carcinoma was higher in the distal and middle third of the esophagus. In 19 patients tumor size was more than 5 cm. In total of 17 of the patients were operated. About 58 patients were irradiated. About 45 of the patients were irradiated with curative intent, 31 of them were primarily irradiated and 14 of them were irradiated postoperatively. Only 13 of the patients received concurrent chemoradiotherapy. Overall 1, 2, 3, and 4 year survival rates were 38.9%, 11.1%, 5.6%, and %1.9, respectively and median survival was 12 months. Median survival for tumors located in cervical esophageal, middle esophagus, and distal esophagus were 23, 8, and 14 months, respectively. One, 2, 3, 4 year survival rates of operated patients were 58.8%, 29.4%, 17.6%, 5.9%, respectively and median survival was 23 months. For inoperable patients 1 and 2 year survival rates were 29.7% and 2.7% and median survival was 8 months. Differences between operable and inoperable patients were statistically significant (P: 0.0003). One, 2, 3, 4 years survival results of patients treated with surgery and postoperative radiotherapy was 62.5%, 25%, 12.5%, 12.5% and median survival was 21 months, 1, 2, 3, 4 years survival results of patients treated with surgery and concurrent chemoradiotherapy was 55.6%, 33.3%, 22.2%, and 0% and median survival was 27 months. There was no statistically significant difference between groups (P: 0.5390). During the therapy, disphagia was the major side effect observed in seven patients. Fatigue, pain, and mild weight loss were the other side effects. Three patients could not tolerate the treatment and left the therapy. We demonstrated that stage III esophageal carcinoma is an extremely deadly disease, and in spite of major advances in cancer treatment, prognosis is still poor.