The necessity of electrodiagnostic studies and ultrasound in ulnar nerve entrapment according to surgeons in the Netherlands.

BACKGROUND: Ulnar nerve entrapment at the elbow (UNE) is the second most prevalent entrapment neuropathy after carpal tunnel syndrome. The objective of this study was to evaluate the expert opinion of different surgical disciplines regarding the need for electrodiagnostic or ultrasound confirmation of UNE and, if so, which test was preferred for confirmation. METHODS: A questionnaire was sent to all neurosurgeons and plastic or hand surgeons in the Netherlands to evaluate the current practice in planning surgical treatment of UNE. RESULTS: The response rate was 36.4 % (134 out of 368). 94 % of surgeons reported that > 95 % of their patients had EDX or ultrasound studies before surgery. 80.6 % of all surgeons who responded reported that they seldom operated on UNE without electrodiagnostic confirmation. Hand surgeons (25.9 %) were more willing to operate on clinically diagnosed UNE without EDX than neurosurgeons (9.4 %) CONCLUSIONS: Dutch surgeons prefer diagnostic confirmation of UNE either by ultrasound or EDX, with a preference for EDX and the vast majority of operated patients do have either EDX or ultrasound or both before surgery. Compared to neurosurgeons, hand surgeons are more willing to operate on patients with clinically defined UNE but normal electrodiagnostic studies.

Dutch injection versus surgery trial in patients with carpal tunnel syndrome (DISTRICTS): protocol of a randomised controlled trial comparing two treatment strategies.

INTRODUCTION: Carpal tunnel syndrome (CTS) is the most common peripheral neuropathy. The optimal treatment strategy is still unknown. The objective of the Dutch Injection versus Surgery TRIal in patients with CTS (DISTRICTS) is to investigate if initial surgery of CTS results in a better clinical outcome and is more cost-effective when compared with initial treatment with corticosteroid injection. METHODS AND ANALYSIS: The DISTRICTS is an ongoing multicenter, open-label randomised controlled trial. Participants with CTS are randomised to treatment with surgery or with a corticosteroid injection. If needed, any additional treatments after this first treatment are allowed and these are not dictated by the study protocol. The primary outcome is the difference between the groups in the proportion of participants recovered at 18 months. Recovery is defined as having no or mild symptoms as measured with the 6-item carpal tunnel symptoms scale. Secondary outcome measurements are among others: time to recovery, hand function, patient satisfaction, quality of life, additional treatments, adverse events, and use of care and health-related costs. ETHICS AND DISSEMINATION: The study was approved by the Medical Ethical Committee of the Amsterdam University Medical Centers (study number 2017-171). Study results will be disseminated in peer-reviewed journals and conferences. TRIAL REGISTRATION NUMBER: ISRCTN Registry: 13164336.

Development of a patient-reported outcome measure for patients who have recovered from a subarachnoid hemorrhage: the "questionnaire for the screening of symptoms in aneurysmal subarachnoid hemorrhage" (SOS-SAH).

BACKGROUND: Patients who have been successfully treated for an aneurysmal subarachnoid hemorrhage (aSAH) often retain multiple health complaints, including mood disorders, cognitive complaints, fatigue, and problems with social participation. These problems are not always fully addressed during hospital visits or in current outcome measures, such as the modified Rankin score and the Glasgow Outcome Scale. Here, we present the development of the "Questionnaire for the Screening of Symptoms in aneurysmal Subarachnoid Hemorrhage" (SOS-SAH), which screens for the self-reported symptoms of patients with mild disabilities. METHODS: During the development of the SOS-SAH we adhered to the PROM-cycle framework for the selection and implementation of patient-reported outcome measures (PROMs). The SOS-SAH was developed in an iterative process informed by a literature study. Patients and healthcare professionals were involved in the development process through participating in a working group, interviews, and a cognitive validation study. RESULTS AND CONCLUSIONS: Relevant patient-reported outcomes (PROs) were identified for patients with aSAH. The SOS-SAH was developed primarily using domains and items from existing PROMs and, if necessary, by developing new items. The SOS-SAH consists of 40 items and covers 14 domains: cognitive abilities, hypersensitivity to stimuli, anxiety, depression, fatigue, social roles, personality change, language, vision, taste, smell, hearing, headache, and sexual function. It also includes a proxy measurement for use by family members to assess cognitive functioning and personality change.

Minimal clinically important difference for the Boston Carpal Tunnel Questionnaire: new insights and review of literature.

No consensus exists about the minimal clinically important difference for the Boston Carpal Tunnel Questionnaire, which hampers its clinical application. This study assessed the minimal clinically important difference of this questionnaire. The Boston Carpal Tunnel Questionnaire was completed by 180 patients, with clinically defined carpal tunnel syndrome, preoperatively and at about 8 months follow-up after carpal tunnel release, together with a six-point scale for perceived improvement. Receiver operator characteristics curves showed that relative changes in Symptom Severity Scale and Functional Status Scale scores correspond better to a clinically relevant improvement than absolute changes. The minimal clinically important difference should be individually calculated from baseline Symptom Severity Scale and Functional Status Scale scores, as patients experiencing more symptoms require more improvement to notice a clinically important difference. By taking this into account, the Boston Carpal Tunnel Questionnaire is more meaningful as an outcome measure in research and clinical practice.

Substantial clinical benefit for Neck Disability Index revisited: establishing the goal for treatment?

PURPOSE: It is difficult for clinicians to inform patients about the success rate of a treatment as a cervical anterior discectomy procedure. Ideally, a proportion of good outcome as rated by patients is known. Patient-reported outcome measurements are helpful. The purpose is to relate the difference in Neck Disability Index (NDI) after a cervical anterior discectomy procedure for single level cervical degenerative disc disease with the patients' rating of their actual clinical situation after long-term follow-up to define the substantial clinical benefit (SCB). METHODS: After completion of the NDI, patients who were surgically treated for cervical single level degenerative disease were asked to complete a five-item Likert scale to rate their clinical situation. After dichotomisation of the outcome in good versus less than good, a cut-off value was defined by determining the value of the difference of NDI with the highest specificity and sensitivity. Funding was not obtained. RESULTS: SCB for NDI after surgery for cervical single level degenerative disease should be set at ten with area under the curve (AUC) of 0.71 for sensitivity as well specificity. CONCLUSIONS: The goal for each treatment is a good outcome. While comparing treatments for cervical degenerative disc disease only those with an SCB of ten will be relevant for the patient, as patients who achieved this difference in NDI rated their actual situation at long-term follow-up as good.

Symptomatic Adjacent Segment Disease After Anterior Cervical Discectomy for Single-level Degenerative Disk Disease.

STUDY DESIGN: A prospective cohort of 142 patients underwent either anterior cervical discectomy alone, anterior cervical discectomy with fusion by cage stand-alone, or anterior cervical discectomy with arthroplasty. We then followed up on their condition for a mean of 9.1±1.9 years (5.6-12.2 y) later. OBJECTIVE: We aimed to evaluate the annual rate of clinically symptomatic adjacent segment disease (ASD) and to analyze predictive factors. SUMMARY OF BACKGROUND DATA: Until recent, ASD has been predominantly evaluated radiologically. It is not known whether all patients had complaints. A frequent cited annual rate of ASD is 2.9%, but a growing number of studies report a lower annual rate. Furthermore, maintaining motion to prevent ASD is one reason for implanting a cervical disk prosthesis. However, the results of studies contradict one another. METHODS: Participants took part in a randomized controlled trial that ended prematurely because of the publication of evidence that did not justify continuation of the trial. The patients were randomly allocated to 3 groups, each of which received one of the abovementioned treatments. We defined symptomatic ASD as signs and symptoms caused by degeneration of an intervertebral disk adjacent to a level of previous anterior cervical disk surgery. At the last follow-up, we were able to ascertain whether clinically symptomatic ASD was present in any of the participants. RESULTS: The overall annual rate of symptomatic ASD was 0.7%. We found no statistically significant correlations between any of the investigated factors and symptomatic ASD except for the surgical method used. Symptomatic ASD was seen less often in anterior cervical discectomy solely or anterior cervical discectomy with arthroplasty than in anterior cervical discectomy with fusion by plate fixation. CONCLUSIONS: The annual rate of symptomatic ASD after an anterior cervical discectomy procedure was estimated to be 0.7%. This seems to be related to the procedure, although firm conclusions cannot be drawn. LEVEL OF EVIDENCE: Level 2-prospective cohort.

Treatment outcome in patients with clinically defined carpal tunnel syndrome but normal electrodiagnostic test results: a randomized controlled trial.

Little is known about treatment effect of carpal tunnel release in patients with clinically defined carpal tunnel syndrome (CTS), but normal electrodiagnostic test results (EDX). The aim of this study was to determine whether this category of patients will benefit from surgical treatment. 57 patients with clinically defined CTS and normal EDX were randomized for surgical treatment (n = 39) or non-surgical treatment (n = 18). A six-point scale for perceived improvement as well as the Boston Carpal Tunnel Questionnaire was completed at baseline and at follow-up after 6 months. A significant improvement of complaints was reported by 70.0% of the surgically treated patients and 39.4% reported full recovery 6 months after surgery. Furthermore, both Functional Status Score and Symptom Severity Score improved significantly more in the surgically treated group (p = 0.036 and p < 0.001, respectively). This study demonstrates that most patients with clinically defined CTS and normal EDX results will benefit from carpal tunnel release. Therefore, this group of CTS patients must not a priori be refrained from surgery.

What's the best surgical treatment for patients with cervical radiculopathy due to single-level degenerative disease? A randomized controlled trial.

BACKGROUND: To investigate the efficacy of adding supplemental fusion or arthroplasty after cervical anterior discectomy for symptomatic mono-level cervical degenerative disease (radiculopathy), which has not been substantiated in controlled trials until now. METHODS: A randomized controlled trial is reported with 9 years follow up comparing anterior cervical anterior discectomy without fusion, with fusion by cage standalone, or with disc prosthesis. Patients suffering from symptomatic cervical disk degeneration at one level referred to spinal sections of department of neurosurgery or orthopedic surgery of a large general hospital with educational facilities were eligible. Neck Disability Index (NDI), McGill Pain Questionnaire Dutch language version (MPQ-DLV), physical-component summary (PCS), and mental-component summary (MCS) of the 36-item Short-Form Health Survey (SF-36), and re operation rate were evaluated. FINDINGS: 142 patients between 18 and 55 years were allocated. The median follow-up was 8.9±1.9 years (5.6 to 12.2 years). The response rate at last follow-up was 98.5%. NDI at the last follow-up did not differ between the three treatment groups, nor did the secondary outcomes as MPQ-DLV and PCS or MCS from SF-36. The major improvement occurred within the first 6 weeks after surgery. Afterward, it remained stable. Eleven patients underwent surgery for recurrent symptoms and signs due to nerve root compression at the index or adjacent level. CONCLUSIONS: This randomized trial could not detect a difference between three surgical modalities for treating a single-level degenerative disk disease. Anterior cervical discectomy without implant seems to be similar to anterior cervical discectomy with fusion by cage stand-alone or with disk prosthesis. Due to the small study sample size, this statement should be considered as inconclusive so far. TRIAL REGISTRATION: ISRCTN41681847.

Premature termination of SMARCB1 translation may be followed by reinitiation in schwannomatosis-associated schwannomas, but results in absence of SMARCB1 expression in rhabdoid tumors.

In schwannomatosis, germline SMARCB1 mutations predispose to the development of multiple schwannomas, but not vestibular schwannomas. Many of these are missense or splice-site mutations or in-frame deletions, which are presumed to result in the synthesis of altered SMARCB1 proteins. However, also nonsense and frameshift mutations, which are characteristic for rhabdoid tumors and are predicted to result in the absence of SMARCB1 protein via nonsense-mediated mRNA decay, have been reported in schwannomatosis patients. We investigated the consequences of four of the latter mutations, i.e. c.30delC, c.34C>T, c.38delA, and c.46A>T, all in SMARCB1-exon 1. We could demonstrate for the c.30delC and c.34C>T mutations that the respective mRNAs were still present in the schwannomas of the patients. We hypothesized that these were prevented from degradation by translation reinitiation at the AUG codon encoding methionine at position 27 of the SMARCB1 protein. To test this, we expressed the mutations in MON cells, rhabdoid cells without endogenous SMARCB1 protein, and found that all four resulted in synthesis of the N-terminally truncated protein. Mutation of the reinitiation methionine codon into a valine codon prevented synthesis of the truncated protein, thereby confirming its identity. Immunohistochemistry with a SMARCB1 antibody revealed a mosaic staining pattern in schwannomas of the patients with the c.30delC and c.34C>T mutations. Our findings support the concept that, in contrast to the complete absence of SMARCB1 expression in rhabdoid tumors, altered SMARCB1 proteins with modified activity and reduced (mosaic) expression are formed in the schwannomas of schwannomatosis patients with a germline SMARCB1 mutation.

Ultrasound imaging of the ulnar nerve: correlation of preoperative and intraoperative dimensions.

OBJECTIVES: Recently, ultrasonography of the ulnar nerve has been introduced to confirm the diagnosis of ulnar neuropathy at the elbow. The reported diameters appear to be small when compared to those known from surgery (=actual diameter). The actual diameter is compared with the diameter on ultrasonography. PATIENTS AND METHODS: Nine patients that were scheduled for decompression of the ulnar nerve at the elbow underwent an ultrasonographic examination preoperatively and intraoperatively. Longitudinal and transversal sonographical scans were made. The maximal diameters of the ulnar nerve at ultrasound were compared with the maximal diameter of the ulnar nerve intraoperatively. RESULTS: Pre- and intraoperatively on the longitudinal scans, the maximal diameter is smaller than the actual maximal diameter of the ulnar nerve. The difference was 3.7+/-1.0 mm (p<0.001) 3.0+/-1.1 mm (p<0.001), respectively. The same was true on the transverse scans: 1.5+/-1.3 mm (p=0.008) versus 1.1+/-0.6 mm (p=0.001). CONCLUSION: The diameter of the ulnar nerve as determined sonographically suggesting ulnar neuropathy does not correspond with the anatomically determined actual diameter. The maximal diameter can be best estimated with transverse scans.

Acute isodense intracerebral haematoma due to coagulopathy associated with prostate cancer.

Prostate cancer can be complicated by spontaneous intracranial haematoma. In the acute setting, an intracranial haematoma can appear isodense on CT scanning, especially if a coagulopathy is present. Diffuse intravascular coagulation (DIC) can be the underlying pathophysiological mechanism that leads to spontaneous bleeding. It is important to recognize the possibility of DIC in cancer patients, since it has important therapeutical implications that may be life saving.

Prospective randomized controlled study comparing simple decompression versus anterior subcutaneous transposition for idiopathic neuropathy of the ulnar nerve at the elbow: Part 1.

OBJECTIVE: The main objective of this study was to compare the clinical outcome of participants treated by simple decompression (SD) of the ulnar nerve versus anterior subcutaneous transposition (AST). METHODS: A prospective randomized controlled study was performed. Three hundred forty participants were referred to our institution between March 1999 and July 2002. One hundred fifty-two patients met the inclusion criteria and were randomized into two surgical groups: 75 were assigned to SD, and 77 were assigned to AST. Participants were followed for 1 year after surgery. The main outcome measure was clinical outcome 1 year after surgery. RESULTS: An excellent or good result was obtained in 49 of 75 participants who underwent SD and in 54 of 77 participants undergoing AST. The difference was not statistically significant. However, the complication rate was statistically lower in the SD group (9.6%) compared with the AST group (31.1%) (risk ratio, 0.32; 95% confidence interval, 0.14-0.69). Duration of symptoms, (sub)luxation of the ulnar nerve, and severity of the complaints did not influence outcome. CONCLUSION: Surgery for ulnar neuropathy at the elbow is effective. The outcomes of SD and AST are equivalent, except for the complication rate. Because the intervention is simpler and associated with fewer complications, SD is advised, even in the presence of (sub)luxation.

Brain metastasis from prostate small cell carcinoma: not to be neglected.

BACKGROUND: Symptomatic brain metastases from prostatic carcinoma are rare (0.05% to 0.5%). CASE REPORT: A 70-year-old man presented with a homonymous hemianopsia due to brain metastatic prostatic carcinoma shortly before becoming symptomatic of prostatic disease. CT and MRI of the brain showed a tumour deep in the right hemisphere near the thalamus and involving the optic radiation. RESULTS: Routine haematological and biochemical tests were normal. The prostate specific antigen level was low on two separate occasions. The prostatic and brain tumours showed identical appearances, namely of a poorly differentiated adenocarcinoma with neuroendocrine differentiation (small cell carcinoma). CONCLUSION: A literature review suggests that small cell carcinoma of the prostate is more likely to spread to the brain compared to adenocarcinoma and that brain metastases indicate a poor prognosis. The prostate gland should be remembered as a possible cause of brain metastases and that a normal serum prostate specific antigen does not exclude this diagnosis.