A case of ochronosis: MRI of the lumbar spine

We present the MRI features of the lumbar spine in a patient with ochronosis. Received: 25 October 1999/Accepted: 8 April 2000     

Post operative supplementary motor area syndrome: clinical features and outcome

The syndrome of the supplementary motor area (SMA) is not well recognized and its features can easily be confused with pyramidal weakness. The authors describe the SMA syndrome in six patients who underwent surgery for tumours located in the SMA, three in the dominant and three in the non-dominant hemispheres. All of them underwent complete resection of the anatomically described SMA, with partial (n = 4) or total resection (n = 2) of the tumour. In the postoperative period, all these patients exhibited reduction of spontaneous movements and difficulty in performing voluntary motor acts to command in the contralateral limbs, although the tone in the limbs was maintained or increased. The function of these limbs in serial automatic motor activities (for example, dressing and walking) was, however, relatively unaffected. Speech deficits were seen in only one of three patients with the dominant SMA syndrome. Besides a severe impairment of volitional movements, the salient features of the deficits in this syndrome are hemineglect and dyspraxia or apraxia involving the contralateral limbs. All patients recovered their motor functions over varying periods of time ranging from one to a few weeks. Long-term follow-up (median 24 months) in five patients revealed complete return of function in the affected limbs. It is important to recognize the entity of the SMA syndrome and differentiate it from the deficits that result from operative damage to the motor cortex as the deficits associated with the former are likely to recover almost completely over a short period of time.     

Orbital apex leiomyoma with intracranial extension

BACKGROUND: Leiomyoma is a rare, benign smooth muscle tumor of the orbit. It occasionally shows some histologic resemblance to other common tumors of the orbit like neurofibroma and schwannoma. Its location at the orbital apex is uncommon and only one case with intracranial extension has been reported so far. CASE DESCRIPTION: A nine-year-old boy presented to us with left orbital pain. Four years earlier he had undergone partial excision, elsewhere, of a tumor at the orbital apex, which was reported as a schwannoma. The computed tomography (CT) scan showed regrowth of the tumor with intracranial extension. The tumor was totally resected by an intracranial route. One year postoperatively there was no recurrence of the tumor. CONCLUSIONS: Leiomyoma of the orbit, though a benign tumor, does show regrowth after partial excision. Total excision, including any intracranial component, is advised. The cases reported so far are reviewed and the histopathology and possible etiopathogenesis of this tumor are discussed.     

Taenia solium metacestode glycoproteins as diagnostic antigens for solitary cysticercus granuloma in Indian patients

Taenia solium metacestode glycoproteins specific for lentil lectin were evaluated as diagnostic antigens for solitary cysticercus granulomas in Indian patients, using both an ELISA and immunoblotting. In 250 patients suspected to have neurocysticercosis and subjected to a computerized tomography scan or magnetic resonance imaging, the proteins were diagnostic by the ELISA in 86 patients (80%) and by immunoblots in 67 (62%) of 107 patients with solitary cysticerus granuloma. Among 100 non-cysticercosis patients, the ELISA and immunoblot were negative in 94% and 97% respectively. No cross-reactions were observed with sera from patients with central nervous system tuberculosis. Proteins of 相似文献   

Characterisation and prognostic impact of immunoparesis in relapsed multiple myeloma

Characterisation and prognostic impact of immunoparesis in relapsed multiple myeloma (MM) is lacking in the current literature. We evaluated 258 patients with relapsed MM, diagnosed from 2008 to 2015, to investigate the prognostic impact of deep immunoparesis on post-relapse survival. On qualitative immunoparesis assessment, no, partial and full immunoparesis was present in 9%, 30% and 61% of patients, respectively. Quantitative immunoparesis was assessed by computing the average relative difference (ARD) between polyclonal immunoglobulin(s) and corresponding lower normal limit(s), with greater negative values indicating deeper immunoparesis. The median ARD was −39%, with an optimal cut-off of −50% for overall survival (OS) by recursive partitioning analysis. Deep immunoparesis (ARD ≤–50%) was associated with a higher tumour burden at first relapse compared to none/shallow [ARD >−50%] immunoparesis. The OS (P = 0·007) and progression-free survival (PFS; P < 0·001) differed significantly between the deep and none/shallow immunoparesis groups. Kaplan–Meier estimates for 3-year OS were 36% and 46%, and for 2-year PFS were 17% and 27%, respectively. On multivariable analysis (MVA) for PFS, both qualitative and quantitative immunoparesis retained negative prognostic impact independently. However, only quantitative immunoparesis was independently prognostic for OS on MVA. Depth of immunoparesis in relapsed MM is an important prognostic factor for post-relapse survival in the era of novel agents and continuous therapy.     

Changes in cervical spine curvature in pediatric patients following occipitocervical fusion

Objective  The study aims to assess changes in cervical spine curvature following occipitocervical fusion (OCF) in the pediatric population. Methods  In a retrospective study, the angle of sagittal curvature and whole cervical spine alignment were determined in the preoperative, immediate postoperative, and follow-up radiographs in 14 patients (<20 years of age) who underwent OCF for developmental atlantoaxial instability between 1995 and 2006. At follow-up, the mean angle of sagittal curvature showed a statistically significant increase from 22+/−10.1° immediately following surgery to 35.9+/−18° at follow-up (p = 0.001). Six patients had exaggerated lordosis (defined as >10° increase in the angle of sagittal curvature). The sagittal curvature angle did not show any worsening in seven patients following removal of the implant. Conclusions  OCF in the pediatric population can result in an increase in the lordotic curvature of the cervical spine that might stabilize following removal of the metal implant within a year of surgery.     

Progression with clinical features is associated with worse subsequent survival in multiple myeloma

Response rate and survival in multiple myeloma (MM) has improved in the era of proteasome inhibitors and immunomodulatory drugs. However, most patients eventually relapse with biochemical progression (BP) alone or with clinical features of end-organ damage (CP: clinical progression), with or without extramedullary (EM) disease. We conducted a retrospective cohort study of 252 patients with MM experiencing first relapse (time, T₀) to evaluate survival following CP with and without EM as a function of BP. Patients were divided into three groups: BP (n = 134; 53%), CP/EM- (n = 87; 35%) and CP/EM+ (n = 31; 12%). The median time from diagnosis to T₀ was significantly shorter in CP/EM+ compared to CP/EM- and BP groups (13 vs 25 vs 25 months; P < 0.001). The incidence of abnormal metaphase cytogenetics at diagnosis was significantly higher in CP/EM+ compared to CP/EM- and BP groups (46% vs 18% vs 11% respectively; P < 0.001). At a median follow-up of 26 months from T₀, median overall survival was 50, 19 and 10 months for BP, CP/EM- and CP/EM+ groups, respectively (P < 0.001). On multivariable analysis, pattern of progression was a significant prognostic factor for OS (HR for CP/EM- vs BP: 3.6; CP/EM+ vs BP: 8.7 and CP/EM+ vs CP/EM-: 2.42; P < 0.001 for all comparisons), along with age at T₀. In conclusion, progression pattern is an important prognostic factor in the current era, with subsequent survival being dismal in patients with end-organ damage or EM disease at relapse. Clinical trials in relapsed MM should consider reporting patterns of progression at baseline to ensure balance between study arms.