[Surgical treatment of nerve lesions]. / Operatieve behandeling van zenuwletsels.

A peripheral nerve lesion may result in loss of motor and sensory function and pain. Nerve surgery may lead to function recovery or contribute to pain reduction. Different surgical techniques are used depending on the specific type of lesion. Common techniques consist of coapting, grafting, moving or burying the nerve. The outcome depends on the interval between the time the lesion occurred and the time of repair as well as the patient's age. Here we present three case studies of patients with a different type of nerve lesion and its consequences. We describe the specific surgical intervention performed to treat the nerve lesion. Immediate repair of the nerve should be performed when the patient has an open wound. In closed lesions, the decision to repair a damaged nerve depends on the severity of the trauma, the nerve(s) involved, the location and the age of the patient. In general, repair should be performed as soon as possible.

Chordoma: a systematic review of the epidemiology and clinical prognostic factors predicting progression-free and overall survival.

BACKGROUND AND AIMS: The aim of this systematic review is to describe the epidemiology of chordoma and to provide a clear overview of clinical prognostic factors predicting progression-free and overall survival. METHODS: Four databases of medical literature were searched. Separate searches were performed for each of the two objectives. Reference and citation tracking was performed. Papers were processed by two independent reviewers according to a protocol that included risk of bias analysis. Disagreement was resolved by discussion. Pooled analyses were planned if homogeneity of data would allow. RESULTS: Incidence-incidence rates ranged between 0.18 and 0.84 per million persons per year and varied between countries and presumably between races. On average patients were diagnosed in their late fifties and gender data indicate clear male predominance. Two of the largest studies (n = 400 and n = 544) reported different anatomical distributions: one reporting the skull base and sacrococcygeal area affected in 32% and 29% of cases, whereas the other reporting that they were affected in 26% and 45% of cases, respectively. PROGNOSTIC FACTORS: Statistically significant adverse prognostic factors predicting progression-free and overall survival include female sex, older age, bigger tumour size, increasing extent of tumour invasion, non-total resection, presence of metastasis, local recurrence, and dedifferentiated histological subtype. CONCLUSIONS: Incidence rate and anatomical distribution vary between countries and presumably between races. Most chordomas arise in the skull base and sacrococcygeal spine, and the tumour shows clear male predominance. Multiple adverse prognostic factors predicting progression-free and overall survival were identified in subgroups of patients. These slides can be retrieved under Electronic Supplementary Material.

Molecular phenotype is associated with survival in breast cancer patients with spinal bone metastases.

To aid in therapy selection for patients with spinal bone metastases (SBM), predictive models have been developed. These models consider SBM from breast cancer a positive predictive factor, but do not take phenotypes based on estrogen (ER), progesterone (PR) and human epidermal growth factor 2 (HER2) receptors into account. The aim of this study was to ascertain whether receptors are associated with survival, when the disease has progressed up to SBM. All patients who were treated for SBM from breast cancer between 2005 and 2012 were included in this international multi-center retrospective study (n = 111). Reports were reviewed for ER, PR and HER2 status and subsequently subdivided into one of four categories; luminal A, luminal B, HER2 and triple negative. Survival time was calculated as the difference between start of treatment for SBM and date of death. Analysis was performed using the Kaplan-Meier method and log-rank tests. Median follow-up was 3.7 years. Survival times in the luminal B and HER2 categories were not significantly different to the luminal A category and were joined into a single receptor positive category. Eighty-five patients (77 %) had a receptor positive phenotype and 25 (23 %) had a triple negative phenotype. Median survival time was 22.5 months (95 %CI 18.0-26.9) for the receptor positive category and 6.7 months (95 %CI 2.4-10.9) for the triple negative category (p < 0.001). Patients with SBM from breast cancer with a triple negative phenotype have a shorter survival time than patients with a receptor positive phenotype. Models estimating survival should be adjusted accordingly.

Neonatal brachial plexus palsy with neurotmesis of C5 and avulsion of C6: supraclavicular reconstruction strategies and outcome.

BACKGROUND: Nerve reconstruction strategies for restoration of elbow flexion and shoulder function in patients with neonatal brachial plexus palsy with neurotmesis of C5 and avulsion of C6 are not well defined and the outcomes are unclear. METHODS: From 1990 to 2008, nerve surgery was performed in 421 patients with neonatal brachial plexus palsy. This study focused on thirty-four infants who had a neurotmetic lesion of C5 and avulsion or intraforaminal neurotmesis of C6, irrespective of C7. The C8 and T1 functions were intact. Intraplexal transfer of C6 to C5 with direct coaptation was preferred for restoration of elbow flexion. The suprascapular nerve was reconnected either by extra-intraplexal transfer of the accessory nerve or by grafting from C5 to restore shoulder function. Additional grafts were attached from C5 to the C5 contribution of the posterior division of the superior trunk when technically possible. RESULTS: Transfer of either the C6 anterior root filaments or the entire C6 nerve to C5 was performed in seventeen patients (group A) with direct coaptation in fifteen of them. Grafting from C5 to the anterior division of the superior trunk was performed in the remaining seventeen infants (group B). An accessory-to-suprascapular nerve transfer was applied in twenty-nine infants. The suprascapular nerve was reconnected in five patients by grafting from C5. It was possible to attach one, two, or three additional grafts from C5 to the posterior division of the superior trunk in twenty-one patients. All infants had biceps muscle recovery to a Medical Research Council (MRC) grade of ≥4, twenty-two (65%) of the thirty-four patients obtained Mallet grade-IV abduction, and eleven (32%) of the thirty-four obtained Mallet grade-IV external rotation. CONCLUSIONS: In patients with neonatal brachial plexus palsy who have neurotmesis of C5 and avulsion of C6, elbow flexion can be successfully restored with supraclavicular intraplexal reconstruction with use of C5 as the proximal outlet. However, shoulder function recovery following suprascapular nerve reinnervation and additional grafting from C5 to the posterior division of the superior trunk is less successful. LEVEL OF EVIDENCE: Therapeutic Level III. See Instructions for Authors for a complete description of levels of evidence.

A de novo mutation in ZMYND11, a candidate gene for 10p15.3 deletion syndrome, is associated with syndromic intellectual disability.

We report a boy with severe syndromic intellectual disability who has a de novo mutation in the ZMYND11 gene. Arguments for pathogenicity of this mutation are found in cases from the literature, especially several with 10p15.3 deletions, harbouring ZMYND11. Additional reports of ZMYND11 mutations in cases with syndromic intellectual disability are needed before the ZMYND11 mutation identified in our case can be considered as definitely pathogenic.

Intercostal and pectoral nerve transfers to re-innervate the biceps muscle in obstetric brachial plexus lesions.

In obstetric brachial plexus lesions with avulsion injury, nerve grafting for biceps muscle re-innervation may not be possible owing to the unavailability of a proximal stump. In such cases, the intercostal nerves or medial pectoral nerve can serve as donor nerves in an end-to-end transfer to the musculocutaneous nerve. The present study reports the results of both techniques from a single institution in a consecutive series of 42 patients between 1995 and 2008. From 1995 to 2000 we always used the intercostal nerve transfer, and from 2001 to 2008 both techniques were used. Biceps muscle force ≥ Medical Research Council Grade 3 was achieved in 37 of 42 patients after a mean follow-up of 44 months. There was no statistical difference in the results in the medial pectoral nerve transfer group (n = 25) and the intercostal nerve transfer group (n = 17).

Risk factors for survival of 106 surgically treated patients with symptomatic spinal epidural metastases.

PURPOSE: Evaluation of risk factors for survival in patients surgically treated for symptomatic spinal epidural metastases (SEM). METHODS: One hundred and six patients who were surgically treated for symptomatic SEM in a 10-year period in two cooperatively working hospitals were retrospectively studied for nine risk factors: age, gender, site of the primary tumor, location of the symptomatic spinal metastasis, functional and neurologic status, the presence of visceral metastases and the presence of other spinal and extraspinal bone metastases. Analysis was performed using the Kaplan-Meier method, univariate log-rank tests and Cox-regression models. RESULTS: Overall median survival was 10.7 months (0.2-107.5 months). Overall 30-day complication rate was 33 %. Multivariate Cox-regression analysis showed that fast growing primary tumors (HR 3.1, 95 % CI 1.6-6.2, p = 0.001), the presence of visceral metastases (HR 1.7, 95 % CI 1.0-2.9, p = 0.033) and a low performance status (HR 2.7, 95 % CI 1.1-6.6, p = 0.025) negatively influenced the survival. CONCLUSION: Primary tumor type, presence of visceral metastases and performance status are significant predictors for survival after surgery for symptomatic SEM and should be evaluated before deciding on the extent of treatment. More accurate prediction models are needed to select the best treatment option for the individual patient.

Correlating birthweight with neurological severity of obstetric brachial plexus lesions.

OBJECTIVE: To investigate the nature and extent of neurosurgically treated obstetric plexus lesions with obstetric and neonatal precedents. DESIGN: Retrospective analysis of prospectively collected data. SETTING: Leiden, the Netherlands. POPULATION: A 9-year cohort of infants (n = 206) neurosurgically treated for obstetric brachial plexus lesion at a tertiary referral centre for nerve lesions. METHOD: Obstetric and neonatal data (parity, diabetic status, pregnancy gestation, mode of cephalic delivery and birthweight) were collected using a standardised protocol and correlated to neurological severity of the brachial plexus lesion. MAIN OUTCOME MEASURE: Neurological severity of the brachial plexus lesion. RESULTS: Nulliparous women delivered significantly lower birthweight newborns (P = 0.016), injuries in those infants were associated with the least severe injury classification. The most prominent association in ordinal logistic regression was between neurological injury severity and larger birthweight (P < 0.001). CONCLUSIONS: Birthweight is correlated with neurological severity of the injury in a group of infants experiencing brachial plexus injury resulting from cephalic vaginal delivery.

[A 67-year-old woman who mistook her daughter for a double: differential diagnosis of misidentification delusion]. / Een 67-jarige vrouw die haar dochter voor een dubbelganger hield: de differentiële diagnostiek van misidentificatiewanen.

A 67-year-old woman developed a misidentification delusion after a right-sided frontally located recurrent convexity meningioma was removed by surgery. After antipsychotic therapy had been established, the patient recovered and the delusions disappeared within a few weeks. A misidentification delusion is a fixed, false beliefabout the identity ofa person, an object, a place, or the time. In the differential diagnosis, psychiatric diseases and neurological diseases are prominent. Patients with a psychiatric disease are usually younger than 40 years, often have a psychiatric history, and usually have other psychotic symptoms like paranoid delusions and hallucinations. Brain tumours and temporal lobectomy have previously been described as a neurological cause of a misidentification delusion; the surgical removal ofa meningioma as such has not been previously described. In patients with a misidentification delusion, the connection between the perception of an identity and its accompanying emotions and memories is disturbed. This connection primarily takes place in the right side of the brain, which is in accordance with the location ofthe removed meningioma in the described patient.

Obstetric lesions of the brachial plexus.

The few studies on prognosis of obstetric lesions of the brachial plexus that are not hampered by selection bias or a short follow-up suggest that functional impairment persists in 20-25% of cases, more than commonly thought. Electromyography (EMG), potentially useful for prognosis, is often considered of little value. Denervation in the first week of life has been interpreted as evidence of an antenatal lesion, but is the logical result of the short axonal length affected. EMG performed at close to the time of possible intervention (3 months) usually shows a discrepancy: motor unit potentials are seen in clinically paralyzed muscles. This can be explained in five ways: an overly pessimistic clinical examination; overestimation of EMG recruitment due to small muscle fibers; persistent fetal innervation; developmental apraxia; or misdirection, in which axons reach inappropriate muscles. Further research into the pathophysiology of obstetric lesions of the brachial plexus is needed to improve prognostication.

Disseminated intravascular coagulation related to outcome in head injury.

In 46 head-injured patients coagulation studies were performed immediately after admission. In 76% of all cases signs of disseminated intravascular coagulation (DIC) were found. DIC was related to the severity of the injury and outcome. It is suggested that DIC may be used as an important parameter in assessing craniocerebral trauma. In a previous report it was stated that disseminated intravascular coagulation (DIC) is met frequently in head-injured patients (2.5--15.3%), and is associated with a more severe grade of injury and an increased mortality. This paper explores in more detail the relationship between outcome after head-injury and signs of DIC, as detected by laboratory studies performed in the first few hours after injury.