Long-term results after posterior fossa decompression in syringomyelia with adult Chiari Type I malformation. Chiari I Malformation: Potential Role for Intraoperative Electrophysiologic Monitoring Surgical treatment of Chiari I malformation--analysis of intraoperative findings, complications, and outcome for 371 foramen magnum decompressions. The need in dural graft suturing in Chiari I malformation decompression: A prospective, single-blind, randomized trial comparing sutured and sutureless duraplasty materials Surgical outcomes of foramen magnum decompression for syringomyelia associated with Chiari I malformation: relation between the location of the syrinx and body pain Prospective analysis of self-perceived quality of life before and after posterior fossa decompression in 112 patients with Chiari malformation with or without syringomyelia. CSF flow study in Chiari I malformation Chiari 1 malformation with syringomyelia: correlation of phase-contrast cine MR imaging and outcome. Posterior fossa volume increase after surgery for Chiari malformation Type I: a quantitative assessment using magnetic resonance imaging and correlations with the treatment response. Classification of symptomatic Chiari I malformation to guide surgical strategy. Dura-splitting decompression of the craniocervical junction: reduced operative time, hospital stay, and cost with equivalent early outcome. Correlation of hindbrain CSF flow and outcome after surgical decompression for Chiari I malformation Pain improvement in patients with syringomyelia and Chiari I malformation treated with suboccipital decompression and tonsillar coagulation Chiari I malformation related syringomyelia: radionuclide cisternography as a predictor of outcome Pathophysiology of persistent syringomyelia after decompressive craniocervical surgery. Clinical article. Endoscopic surgery versus medical treatment for spontaneous intracerebral hematoma: a randomized study. Safety and efficacy of peripheral nerve stimulation of the occipital nerves for the management of chronic migraine: Results from a randomized, multicenter, double-blinded, controlled study Clinical outcome of autologous peripheral blood stem cell transplantation in opticospinal and conventional forms of secondary progressive multiple sclerosis in a Chinese population Accurately measuring outcomes after surgery for adult Chiari I malformation: determining the most valid and responsive instruments. Neurological Condition of Infants Born After In Vitro Fertilization With Preimplantation Genetic Screening Recurrence following neurosurgeon-determined gross-total resection of adult supratentorial low-grade glioma: results of a prospective clinical trial. The Heart in Friedreich Ataxia: Definition of Cardiomyopathy, Disease Severity, and Correlation With Neurological Symptoms Volume-outcome analysis in rectal cancer: a plea for enquiry, evidence and evolution.
OBJECT There is little information about the long-term effectiveness and complications following decompressive surgery for syringomyelia related to Chiari malformation Type I ( CM-I ) . METHODS Examining long-term clinical and radiological follow-up , the authors studied a mixed retrospective and prospect i ve single-institution cohort of 109 consecutive surgically treated adult patients with syringomyelia and CM-I. All patients underwent a st and ardized surgical protocol : decompression of the craniocervical junction , arachnoid exploration , and shrinkage of the cerebellar tonsils . Factors predicting outcome were investigated . RESULTS The retrospective arm consisted of 41 cases treated between 1990 and 1994 , and the prospect i ve arm comprised 68 patients treated between 1994 and 2001 . The mean overall age was 45.9 years , and 58.8 % of the population was female . The median follow-up period was 12.7 years . The most frequent initial symptoms were pain and sensory and gait disturbances . There was no perioperative death or neurological deterioration . The comprehensive perioperative complication rate was approximately 11 % , with 3 cases ( 2.7 % ) of CSF leakage . Regression analysis showed that the best combination of clinical and radiological outcome predictors was age and duration of symptoms . Clinical follow-up confirmed surgical result stability with clinical improvement of greater than 90 % of the spinal and cranial manifestations over a long-term period . Two patients had radiological recurrences of syringomyelia without clinical signs 85 and 124 months after surgery . CONCLUSIONS Certain clinical predictors of poor clinical and radiological prognosis were identified-namely , age at time of surgery and symptom duration . The results of the study provide additional long-term data that support the effectiveness and safety of relieving CSF block at the craniocervical junction in CM-I-related syringomyelia & NA ; Intraoperative electrophysiologic monitoring can diminish the risk of neurologic injury by enabling the detection of injury at a time when it can be reversed or minimized . Although it is clear that in patients with cervical spine disease monitoring during surgery reduces the incidence of neurologic injury , almost no data are available regarding its utility in patients undergoing suboccipital decompression for Chiari I malformation . Patients with Chiari I malformation have caudal displacement of the cerebellar tonsils below the skull base , thereby creating a tight foramen magnum and cervical canal . Although the majority of pediatric neurosurgeons perform a bony decompression with duraplasty for symptomatic patients , there is much controversy regarding the amount of bony decompression required for clinical improvement and whether a duraplasty is essential . The authors therefore conducted a prospect i ve , observational study using intraoperative brainstem auditory evoked potentials ( BAEPs ) and somatosensory evoked potentials in pediatric patients undergoing suboccipital decompressions for Chiari I malformations to determine whether there were consistent changes in intraoperative BAEPs that could help the operating surgeon decide how extensive a decompression was needed in these patients , and whether changes in BAEPs or somatosensory evoked potentials occurred during operative positioning that could be modified to reduce the risk of neurologic injury BACKGROUND Foramen magnum decompression is widely accepted as the treatment of choice for Chiari I malformation . However , important surgical details of the procedure are controversial . OBJECTIVE This study analyzes 371 decompressions  focusing on intraoperative findings , analysis of complications , and long-term outcomes . METHODS Among 644 patients between 1985 and 2010 , 359 patients underwent 371 decompressions .  Surgery for symptomatic patients consisted of suboccipital craniectomy , C1 laminectomy , arachnoid dissection , and duraplasty . Short-term results were determined after 3 months ; long-term outcomes were evaluated with Kaplan-Meier statistics . RESULTS The mean age was 40 ± 16 years ; mean follow-up was 49 ± 56 months ; 75.8 % demonstrated syringomyelia . The complication rate was 21.8 % with permanent surgical morbidity of 3.2 % and surgical mortality of 1.3 % . Of the patients , 73.6 % reported improvement after 3 months ; 21 % were unchanged . Overall , 14.3 % demonstrated a neurological deterioration within 5 years and 15.4 % within 10 years . The severity of neurological symptoms correlated with the grade of arachnoid pathology . Outcome data correlated with the number of previous decompressions , severity of arachnoid pathology , h and ling of the arachnoid , type of duraplasty , and surgical experience . First-time decompressions with arachnoid dissection and an alloplastic duraplasty result ed in surgical morbidity for 2.0 % , a 0.9 % mortality rate , postoperative improvement after 3 months for 82 % , and neurological recurrence rates of 7 % after 5 years and 8.7 % after 10 years . CONCLUSION Arachnoid pathology in Chiari I malformation has an impact on clinical symptoms and postoperative results . Decompressions with arachnoid dissection and an alloplastic duraplasty performed by surgeons experienced with this pathology offer a favorable long-term prognosis Background : This study compared the use of two commonly utilized dural closure techniques used in augmentation duraplasty for Chiari malformation I ( CM I ) and evaluated their efficacy and outcome in terms of quality of life assessment s. Methods : This prospect i ve r and omized study compared sutureless ( DuraGen ) and suturable ( Dura-Guard ) techniques in CM I decompression . Clinical parameters , cost analysis , and SF-36 Quality of Life Question naire ( QLQ ) were utilized to assess outcome . Results : Thirty-four patients were enrolled . Average age was 38.7 ± 12.2 years ( mean ± SD ( St and ard Deviation ) ) and 82 % of patients were female . Sixteen patients received DuraGen and 18  Dura-Guard . Age and gender were similar among groups . Postoperative complications did not differ between groups . Operative cost and time were less for DuraGen , whereas hospital stay was less with Dura-Guard , neither was statistically significant . Average QLQ scores at months 1 , 2 , and 3 improved in both groups . Dura-Guard patients showed greater improvement in quality of life at month 2 ( P < 0.05 ) but groups did not differ at final survey . All patient 's physical health ( P < 0.005 ) and function ( P < 0.005 ) were significantly improved . Outcome did not differ between groups and all patients showed significant improvement ( P < 0.05 ) . Conclusion : Both techniques are effective in reaching the goals of decompressive surgery for CM I and did not differ in quality of life at final survey . All patients showed significant improvement in physical function , physical health , and outcome following surgery . With all variables being equal the choice of duraplasty material may be based upon surgeon 's preference Background There have been few reports about the relation between the morphology of syrinxes and body pain in syringomyelia associated with Chiari I malformation . To investigate this phenomenon , the relation between the location of the syrinx and body pain before and after foramen magnum decompression ( FMD ) were evaluated . Methods The subjects were 20 patients with Chiari I malformation associated with syringomyelia who underwent FMD .  The morphology of the syrinxes was classified into three types — enlarged type , which was a distended syrinx at the central spinal cord ; deviated type , which was a deviated syrinx posterolaterally within the spinal cord ; central type , which was a small syrinx at the central canal of the spinal cord — based on axial magnetic resonance imaging ( MRI ) . Preoperative and postoperative clinical symptoms and the body pain were evaluated by the Japanese Orthopaedic Association ( JOA ) score and a visual analogue scale ( VAS ) prospect ively . Results Preoperative and postoperative JOA scores showed no statistically significant differences between the three syrinx types . Intensity of body pain evaluated by the VAS showed that patients with a deviated-type syrinx on pre- and postoperative MRI tended to be associated with more intense pain than the other two types . Conclusions It was indicated that pain before and after surgery is more intense when the syrinx is deviated toward the spinal dorsal horn as seen on MRI OBJECT The purpose of this prospect i ve study was to determine if there was a difference in the self-perceived quality of life ( QOL ) before and after surgery among patients with Chiari I malformations with or without syringomyelia . Most patients with Chiari I malformations report improvement in their QOL after decompression surgery ; however , specific outcome data have not been empirically studied in this patient population . METHODS One hundred seventy-two consecutive patients who underwent posterior fossa decompression based on neuroimaging evidence of a Chiari I malformation with or without syringomyelia were prospect ively offered participation in the study . The Sickness Impact Profile ( SIP ) was chosen as the instrument for data collection . Completed question naires were returned by 112 patients . A statistically significant improvement ( p < 0.0001 ) in SIP scores ( self-reported QOL ) was noted in 84 % of participants after decompression surgery . Patient age , amount of tonsillar herniation , and evidence of syringomyelia before surgery did not correlate with or adversely affect outcomes . Among 16 participants who reported worsening in their QOL , anecdotal information revealed extraneous factors unrelated to the Chiari I malformation that they perceived as negatively influencing their outcome . Among the extraneous variables noted by the participants were general health status , unrelated injury , other illnesses , and significant stress . CONCLUSIONS The majority of participants who underwent posterior fossa surgery for a Chiari I malformation reported significant improvement in their QOL after surgery Objective The aim of this prospect i ve study was to define the role of cardiac gated phase-contrast ciné magnetic resonance imaging in deciding the therapeutic strategy in patients with Chiari I malformation . Material s and methods Twenty-one patients operated on between February 2000 and July 2002 were enrolled in the study . All patients underwent a detailed preoperative neurological examination . MRI of the craniovertebral junction and the whole spine was done , followed by cardiac gated phase contrast ciné magnetic resonance imaging . Results Signs and symptoms of syringomyelia were noted in 15 patients and cerebellar signs in 11 patients . Three of them had trigeminal nerve involvement , and 4 had ninth and tenth cranial nerve involvement . The sixth and accessory nerves were involved in 1 patient each . Preoperative CSF flow studies revealed obstructive flow both anteriorly and posteriorly in 6 patients and only posterior block in 15 patients . One patient investigated for failed foramen magnum decompression revealed obstruction to CSF flow ventrally .  Foramen magnum decompression with duroplasty was done in all these cases . The patient who had a persistent ventral flow block underwent odontoidectomy . Patients were followed up for a maximum of 36 months , with a mean of 18 months . MRI  CSF flow studies revealed established flow dorsally in all cases . Seventeen patients showed clinical improvement and 2 of them did not show any neurological changes . Two patients deteriorated following an initial period with a shunt . Conclusion MRI CSF flow study is an effective tool for deciding the type of surgery to be performed and also for monitoring patients postoperatively AIM This study was design ed to determine the hemodynamics of cerebrospinal fluid flow in syringomyelia patients associated with Chiari I malformation using phase-contrast velocity-encoded-cine MRI and also to find out whether treatment outcomes may be predicted by these flow measurements . MATERIAL AND METHODS Eighteen consecutive symptomatic patients with syringomyelia associated with Chiari 1 malformation were included . The PC VEC MRI was performed at the level of foramen magnum and syrinx cavity both preoperatively and 6 months postoperatively . Following surgery , the modified Asgari score was calculated , and the association between CSF flow pattern and clinical outcome was assessed . RESULTS Evaluation of clinical symptoms at postoperative 6th month revealed improvement in 11 ( 61 % ) patients and stabilization in 5 ( 28 % ) patients whereas results were poor in 2 ( 11 % ) patients . Preoperative cine MRI flow studies showed a heterogeneous pattern at the foramen magnum level in all of the patients . Postoperative cine MR flow studies demonstrated the change from heterogeneous pattern to sinusoidal pattern in 11 patients and a decrease in heterogeneity in 7 patients . CONCLUSION Our results indicate that CSF flow measurements using PC VEC MRI can give important information regarding the prognosis and follow-up of the patients with Chiari I malformation OBJECT The aim of this paper was to measure the posterior fossa ( PF ) volume increase result ing from a given-sized occipital craniectomy in Chiari malformation Type I surgery and to analyze its correlations with the PF size and the treatment response , with the perspective of tailoring the amount of bone removal to the patient-specific PF dimensions . METHODS Between January 2005 and June 2006 , 11 adult patients with symptomatic Chiari malformation Type I underwent a st and ardized PF decompression . A prospect i ve evaluation with clinical examination , functional grading , and MR imaging measurement protocol s was performed pre- and postoperatively . A method is reported for the measurement of PF volume ( PFV ) after surgery . The degree of PFV increase was compared with the preoperative size of the PF and with the clinical outcome . RESULTS All 11 patients  improved postoperatively , with complete and partial recovery in 4 and 7 patients , respectively . No postoperative complication occurred after a mean follow-up period of 45 months . The mean relative increase in PFV accounted for 10 % ( range 1.5%-19.7 % ) of the initial PFV ; the increase was greater in cases in which the PF was small ( r = -0.52 , p = 0.09 ) and the basiocciput was short ( r = -0.37 , p = 0.2 ) . A statistically significant positive correlation was found between the degree of PFV increase and the treatment response ( p = 0.014 ) ; complete recovery was observed with a PFV increase of 15 % and partial recovery with an increase of 7 % . CONCLUSIONS The treatment response is significantly influenced by the degree of PFV increase , which is dependent on the size of the PF and the extent of the craniectomy , suggesting that the optimal patient-specific PFV increase could be predicted on the basis of preoperative MR imaging and enhancing the perspective that the craniectomy size could be tailored to the individual PFV BACKGROUND Treatment options for Chiari I malformations include posterior fossa decompression ( PFD ) with additional techniques including laminectomy , intradural exploration , and duraplasty . Neuroimaging findings of cisterna magna volume , syringomyelia , and intraoperative ultrasonography may tailor surgical intervention . METHODS We developed an algorithm classifying symptomatic Chiari I patients into three groups to define minimum operation . Without syringomyelia , the presence of cisterna magna defined Group A and the absence defined Group B. Patients with syrinx formed Group C. Mild structural pathology ( Group A ) or adequate space following PFD ( Group B , normal intraoperative ultrasound ( IOUS ) ) should be treated by PFD alone . Conversely , presence of syringomyelia ( Group C ) or inadequate space following PFD ( Group B , abnormal IOUS ) should additionally have duraplasty . We applied this algorithm to patients treated at a single institution over 16 years . RESULTS Twenty-four symptomatic Chiari I malformation patients were divided into three groups that did not differ by age , gender , or extent of tonsillar ectopia . All patients treated by this algorithm experienced clinical and radiographic improvement . This included eight Group B patients who underwent PFD only ( n=6 ) or additional duraplasty ( n=2 ) decided by IOUS . CONCLUSION Treatment of symptomatic Chiari I malformation may have inadequate outcome with conservative strategy or complications with aggressive strategy . This algorithm utilizes preoperative neuroimaging and intraoperative ultrasound to tailor intervention , with excellent clinical outcome and radiographic syrinx resolution on application to 24 patients . Further validation requires prospect i ve multicenter evaluation with larger patient population OBJECT The choice of surgical technique for decompressive surgery in patients with Chiari I malformation is controversial . Good preliminary postoperative outcomes have been achieved in patients with Chiari I malformation ( without syringomyelia ) after using a dura-splitting technique . The authors evaluated safety , re source use , and early outcome after this surgery in patients without syringomyelia and compared the findings associated with duraplasty in patients with syringomyelia . METHODS A prospect i ve series of 24 patients with Chiari I malformation ( 12 with a syrinx ) underwent decompression of the craniocervical junction ( CCJ ) .  An allograft-augmented duraplasty was performed in patients with syringomyelia . Intraoperative ultrasonography confirmed adequate tonsillar decompression after lysis of the periosteal b and s at the foramen magnum and C-1 arch as well as partial resection of the outer leaf of the dura in patients without syringomyelia . Patients in each group were of similar mean age ( syringomyelia 10.8 years and no syringomyelia 7.6 years old ; p = 0.07 ) and functional status . The mean follow-up period was 15.3 months ( range 3 - 30 months ) . Dura-splitting decompression required significantly less mean operative time ( 99 minutes compared with 169 minutes , respectively ; p < 0.001 ) , total operating room time ( 166 minutes compared with 249 minutes , respectively ; p < 0.001 ) , duration of hospitalization ( 3 days compared with 3.75 days , respectively ; p < 0.05 ) , perioperative charges ( $ 3615 compared with $ 5538 , respectively ; p < 0.001 ) , and overall hospital charges ( $ 7705 compared with $ 9759 , respectively ; p < 0.001 ) than the duraplasty . Mean clinical outcome scores were similar ( syringomyelia 1.53 of 2 ; no syringomyelia 1.67 of 2 ; not statistically significant ) . CONCLUSIONS Dura-splitting CCJ decompression in pediatric patients with Chiari I malformation and without syringomyelia is safe , provides good early clinical results , and significantly reduces re source use . A r and omized controlled trial of dura-splitting decompression in a uniform population of patients with Chiari I malformation is indicated Objective Many patients with symptomatic Chiari I malformation experience symptom recurrence after surgical decompression . Improved radiographic predictors of outcome are needed to better select patients most likely to benefit from surgical intervention . We examined whether ventral or dorsal cerebrospinal fluid ( CSF ) flow dynamics assessed by cine phase-contrast MRI scans could predict response to posterior fossa decompression for Chiari I malformation . Methods Forty-four consecutive pediatric patients undergoing pre-operative cine phase-contrast MRI followed by posterior fossa decompression for Chiari I malformation were retrospectively review ed . The association of pre-operative ventral or dorsal CSF flow abnormalities at the foramen magnum with symptom-free survival after surgical decompression was assessed via Kaplan – Meier plots and log-rank analysis . Results Mean ± SD age at time of surgery was 8 ± 6 years . Sixteen ( 36 % ) patients demonstrated decreased CSF flow dorsal to the cervico-medullary brainstem alone . Fourteen ( 32 % ) patients demonstrated abnormal CSF flow both ventral and dorsal to the cervico-medullary brainstem . Fourteen ( 32 % ) had normal hindbrain CSF flow . Overall , 13 ( 30 % ) patients experienced some degree of symptom recurrence by last follow-up ( mean of 27 ± 16 months post-operatively ) . Symptom recurrence did not differ as a function of degree of tonsilar ectopia ( p = 0.55 ) . Abnormal CSF flow dorsal to the cervico-medullary brainstem was not associated with symptom recurrence after surgical decompression ( p = 0.10 ) . However , combined pre-operative ventral and dorsal CSF flow abnormality was associated with a significant reduction ( 2.6-fold ) in the risk of post-operative symptom recurrence ( p < 0.05 ) . Only one patient ( 7 % ) with pre-operative ventral and dorsal CSF flow pathology experienced symptom recurrence 3.5 years after surgery versus 12 ( 40 % ) patients without ventral CSF flow pathology . There were otherwise no differences in baseline clinical , radiological , or operative variables between patients with abnormal versus normal ventral CSF flow . Conclusion The presence of decreased CSF flow both ventral and dorsal to the cervico-medullary brainstem was associated with improved response to hindbrain decompression for Chiari I malformation in children . Cine phase-contrast MRI may be a useful tool for surgical risk stratification and identifying patients that may be optimal surgical c and i date s. Combined ventral and dorsal hindbrain CSF flow pathology may better predict response to posterior fossa decompression compared to dorsal CSF flow pathology alone Pain experienced by patients who have syringomyelia associated with Chiari I malformation ( SACM ) includes headache , cervicalgia and radiculalgia . We studied the correlation of clinical and imaging factors with the evolution of pain and surgical outcome at 12 months . We performed a prospect i ve study of 13 patients who presented with SACM and who underwent suboccipital decompression , C1 laminectomy , tonsillar coagulation and duraplasty .  The Bidzinski Outcome Scale was used . Post-operatively , pain improvement was observed in 11 patients and the syrinx resolved in 6 patients and reduced in 7 . Eleven patients had a good or very good outcome , while 2 patients had a poor outcome . A post-operative medullary axial occupation of the syrinx less than 75 % of the spinal canal was associated with improved post-operative pain . Longer duration of symptomatology was associated with a poor outcome and failed pain control . The size and form of the syrinx , as well as early surgical treatment , are the factors that most influence the control of pain post-surgery in patients with SACM Summary Background . This prospect i ve study , conducted in patients with Chiari I malformation ( C I ) related syringomyelia who underwent posterior decompression and duroplasty  , utilizes radionuclide cisternography in order to study the cerebrospinal fluid ( CSF ) dynamics at the foramen magnum and to predict the clinical outcome following surgery . Methods . 17 consecutive patients of C I with syringomyelia ( but without hydrocephalus or fixed atlanto-axial dislocation ) , underwent a detailed neurological examination and were assigned a clinical disability score based on the modified Klekamp and Samii score . A radionuclide cisternography ( using Tc99m-DTPA ) was performed via the lumbar route and the ascent of the tracer was followed utilizing a gamma camera immediately after injection and then sequentially after 1 , 2 , 4 , 6 and 24 hours . After posterior decompression and duroplasty , the modified Klekamp and Samii score was repeated at follow-up visits ( range : 3 months to one year ) along with radionuclide cisternography at 3 months , and MR imaging at 6 months . Findings . Three patterns of tracer flow were observed : a ) rapid flow ( n=7 ) ; b ) supratentorial subarachnoid delay ( n=7 ) ; and , c ) foramen magnum block ( n=3 ) . The patients having foramen magnum block had the poorest clinical scores on admission . At follow up , there was an improvement in the clinical scores so that the mean scores in all three categories reached nearly the same level . Following posterior decompression , the radionuclide cisternography performed in 10 patients showed a rapid flow of the tracer without any obstruction . The syrinx resolved in 4 of the 11 patients in whom an MRI was done . Interpretation . The patients with C I with syringomyelia may often have a free flow of tracer across the FM . Posterior decompression and duroplasty provides maximum clinical relief in patients with a demonstrable foramen magnum block on radionuclide cisternography while those with a normal flow have less relief . The symptomatology related to brain-stem compression immediately responds to the surgical procedure but the syrinx-induced signs and symptoms of spinal cord dysfunction persist OBJECT Craniocervical decompression for Chiari malformation Type I ( CM-I ) and syringomyelia has been reported to fail in 10%-40 % of patients . The present prospect i ve clinical study was design ed to test the hypothesis that in cases in which syringomyelia persists after surgery , craniocervical decompression relieves neither the physiological block at the foramen magnum nor the mechanism of syringomyelia progression . METHODS The authors prospect ively evaluated and treated 16 patients with CM-I who had persistent syringomyelia despite previous craniocervical decompression .  Testing before surgery included the following : 1 ) clinical examination ; 2 ) evaluation of the anatomy using T1-weighted MR imaging ; 3 ) assessment of the syrinx and CSF velocity and flow using cine phase-contrast MR imaging ; and 4 ) appraisal of the lumbar and cervical subarachnoid pressures at rest , during a Valsalva maneuver , during jugular compression , and following the removal of CSF ( CSF compliance measurement ) . During surgery , ultrasonography was performed to observe the motion of the cerebellar tonsils and syrinx walls ; pressure measurements were obtained from the intracranial and lumbar intrathecal spaces . The surgical procedure involved enlarging the previous craniectomy and performing an expansile duraplasty with autologous pericranium . Three to 6 months after surgery , clinical examination , MR imaging , and CSF pressure recordings were repeated . Clinical examination and MR imaging studies were then repeated annually . RESULTS Before reexploration , patients had a decreased size of the CSF pathways and a partial blockage in CSF transmission at the foramen magnum . Cervical subarachnoid pressure and pulse pressure were abnormally elevated . During surgery , ultrasonographic imaging demonstrated active pulsation of the cerebellar tonsils , with the tonsils descending during cardiac systole and concomitant narrowing of the upper pole of the syrinx . Three months after reoperation , patency of the CSF pathways was restored and pressure transmission was improved . The flow of syrinx fluid and the diameter of the syrinx decreased after surgery in 15 of 16 patients . CONCLUSIONS Persistent blockage of the CSF pathways at the foramen magnum result ed in increased pulsation of the cerebellar tonsils , which acted on a partially enclosed cervical subarachnoid space to create elevated cervical CSF pressure waves , which in turn affected the external surface of the spinal cord to force CSF into the spinal cord through the Virchow-Robin spaces and to propel the syrinx fluid caudally , leading to syrinx progression . A surgical procedure that reestablished the CSF pathways at the foramen magnum reversed this pathophysiological mechanism and resolved syringomyelia . Elucidating the pathophysiology of persistent syringomyelia has implication s for its primary and secondary treatment A controlled r and omized study of endoscopic evacuation versus medical treatment was performed in 100 patients with spontaneous supratentorial intracerebral ( subcortical , putaminal , and thalamic ) hematomas . Patients with aneurysms , arteriovenous malformations , brain tumors , or head injuries were excluded . Criteria for inclusion were as follows : patients ' age between 30 and 80 years ; a hematoma volume of more than 10 cu cm ; the presence of neurological or consciousness impairment ; the appropriateness of surgery from a medical and anesthesiological point of view ; and the initiation of treatment within 48 hours after hemorrhage . The criteria of r and omization were the location , size , and side of the hematoma as well as the patient 's age , state of consciousness , and history of hypertension . Evaluation of outcome was performed 6 months after hemorrhage . Surgical patients with subcortical hematomas showed a significantly lower mortality rate ( 30 % ) than their medically treated counterparts ( 70 % , p less than 0.05 ) . Moreover , 40 % of these patients had a good outcome with no or only a minimal deficit versus 25 % in the medically treated group ; the difference was statistically significant for operated patients with no postoperative deficit ( p less than 0.01 ) . Surgical patients with hematomas smaller than 50 cu cm made a significantly better functional recovery than did patients of the medically treated group , but had a comparable mortality rate . By contrast , patients with larger hematomas showed significantly lower mortality rates after operation but had no better functional recovery than the medically treated group . This effect from surgery was limited to patients in a preoperatively alert or somnolent state ; stuporous or comatose patients had no better outcome after surgery . The outcome of surgical patients with putaminal or thalamic hemorrhage was no better than for those with medical treatment ; however , there was a trend toward better quality of survival and chance of survival in the operated group Background : Chronic migraine ( CM ) is a debilitating neurological disorder with few treatment options . Peripheral nerve stimulation ( PNS ) of the occipital nerves is a potentially promising therapy for CM patients . Methods : In this r and omized , controlled multicenter study , patients diagnosed with CM were implanted with a neurostimulation device near the occipital nerves and r and omized 2:1 to active ( n = 105 ) or sham ( n = 52 ) stimulation . The primary endpoint was a difference in the percentage of responders ( defined as patients that achieved a ≥50 % reduction in mean daily visual analog scale scores ) in each group at 12 weeks . Results : There was not a significant difference in the percentage of responders in the Active compared with the Control group ( 95 % lower confidence bound ( LCB ) of −0.06 ; p = 0.55 ) . However , there was a significant difference in the percentage of patients that achieved a 30 % reduction ( p = 0.01 ) . Importantly , compared with sham-treated patients , there were also significant differences in reduction of number of headache days ( Active Group = 6.1 , baseline = 22.4 ; Control Group = 3.0 , baseline = 20.1 ; p = 0.008 ) , migraine-related disability ( p = 0.001 ) and direct reports of pain relief ( p = 0.001 ) . The most common adverse event was persistent implant site pain . Conclusion : Although this study failed to meet its primary endpoint , this is the first large-scale study of PNS of the occipital nerves in CM patients that showed significant reductions in pain , headache days , and migraine-related disability . Additional controlled studies using endpoints that have recently been identified and accepted as clinical ly meaningful are warranted in this highly disabled patient population with a large unmet medical need . Trial registration : Clinical trials.gov ( NCT00615342 ) To evaluate clinical outcomes of autologous peripheral blood stem cell transplantation ( APBCST ) between opticospinal multiple sclerosis ( OSMS ) and conventional multiple sclerosis ( CMS ) during disease progressive stage in a Chinese population . Thirty-six secondary progressive MS patients , among whom 21 were with OSMS and 15 with CMS , underwent APBSCT and were followed up for an average of 48.92 months ( range , 10–91 months ) . Peripheral blood stem cells were obtained by leukapheresis after mobilization with granulocyte colony-stimulating factor . Modified BEAM conditioning regimen ( Tiniposide , melphalan , carmustin , and cytosine arabinoside ) were administered . Outcomes were evaluated using the exp and ed disability status scale ( EDSS ) . No maintenance treatment was administered if there was no disease progression . No treatment-related mortality occurred . Among the 36 patients , one OSMS patient dropped during the follow-up . Among the 22 relapse-free patients , 20 were with continuous neurological improvement without any relapse events , and two remained in neurologically stable states . Among the 13 relapse patients , seven had experienced of neurological relapse , but with no progression during the follow-up period ; and six experienced neurological deterioration after transplantation and needed further immunosuppressant treatment . The confirmed relapse-free survival rate was 62.9 % and progression-free survival rate was 83.3 % after 91 months according to Kaplan and Meier survival curves . Eleven of the 20 OSMS patients ( 55 % ) and two of the 15 CMS patients ( 13.3 % ) stayed in disease active group ( P = 0.014 ) . For the 20 OSMS patients , the overall EDSS score decreased significantly after transplantation ( P = 0.016 ) , while visual functions had no significant improvement ( P = 0.716 ) . Progressive OSMS has a higher relapse rate than CMS following APBSCT BACKGROUND There has been a transition to using patient-reported outcome instruments ( PROi ) to assess surgical effectiveness . However , none of these instruments have been vali date d for outcomes of adult Chiari I malformation ( CMI ) . OBJECTIVE The aim of this study was to determine the relative validity and responsiveness of various PROi in measuring outcomes after surgery for CMI . METHODS Fifty patients undergoing suboccipital craniotomy for adult CMI were prospect ively followed for 1 year . Baseline and 1-year patient-reported outcomes ( visual analog scale for head pain and visual analog scale for neck pain , Neck Disability Index [ NDI ] , Headache Disability Index , SF-12 , Zung Self-Rating Depression Scale , and EuroQol-5D [ EQ-5D ] ) were assessed . A level of improvement in general health after surgery was defined as meaningful improvement . Receiver-operating characteristic curves were generated to assess the validity of PROi to discriminate between meaningful improvement and not . The difference between st and ardized response means ( SRMs ) in patients reporting meaningful improvement vs not as calculated to determine the relative responsiveness of each outcome instrument . RESULTS For pain and disability , the NDI was the most accurate discriminator of meaningful effectiveness ( area under the curve : 0.90 ) and also most responsive to postoperative improvement ( st and ardized response means difference : 1.87 ) . For general health and quality of life , the SF-12 PCS , EQ-5D , and Zung Self-Rating Depression Scale were all accurate discriminators ; however , SF-12 Physical Component Scale ( SF-12 PCS ) and EQ-5D were most accurate . SF-12 PCS was also most responsive . CONCLUSION For pain and disability , NDI is the most valid and responsive measure of improvement after surgery for CMI . For health-related quality of life , SF-12 PCS and EQ-5D are the most valid and responsive measures . NDI with SF-12 or EQ-5D is the most valid in patients with CMI and should be considered in cost-effectiveness studies Aim of this study was to evaluate the effect of preimplantation genetic screening ( PGS ) on  neurodevelopmental outcome in children . We conducted a prospect i ve follow-up study of children born to women r and omly assigned to in vitro fertilization with or without PGS .  Primary outcome was adverse neurologic outcome at 18 mo ; secondary outcomes were types of minor neurologic dysfunction ( MND ) , neurologic outcome before 18 mo , neonatal intensive care admission , and congenital malformations .  Twenty women in the PGS group participated with 25 children and 26 women in the control group participated with 31 children . Five PGS pregnancies ( 25 % ) and four control pregnancies ( 15 % ) result ed in birth of at least one child with an adverse neurologic outcome ( adjusted odds ratio : 2.3 [ 0.4–12.0 ] ) . Dysfunction in fine motor abilities and posture and muscle tone dysregulation tended to be present more frequently after PGS .  Neurologic outcome before 18 mo , neonatal intensive care admission , and prevalence of congenital malformations were similar in study and control pregnancies . Nevertheless , at child level , rates of adverse outcome were higher after PGS . In conclusion , outcome in pregnancies after in vitro fertilization ( IVF ) with and without PGS was similar . The small sample size precludes the conclusion that PGS is not associated with less favorable neurologic outcome . Safety of new assisted reproductive techniques should be evaluated before large-scale implementation OBJECT In 1998 , the Radiation Therapy Oncology Group initiated a Phase II study of observation for adults < 40 years old with cerebral low- grade glioma who underwent a neurosurgeon-determined gross-total resection ( GTR ) .  METHODS Patient eligibility criteria included the presence of a World Health Organization Grade II astrocytoma , oligodendroglioma , or mixed oligoastrocytoma confirmed histologically ; age 18 - 39 years ; Karnofsky Performance Scale score > or = 60 ; Neurologic Function Scale score < or = 3 ; supratentorial tumor location ; neurosurgeon-determined GTR ; and pre- and postoperative MR imaging with contrast enhancement available for central review by the principal investigator . Patients were observed following GTR and underwent MR imaging every 6 months . Prognostic factors analyzed for their contribution to patient overall survival , progression-free survival ( PFS ) , and tumor recurrence included age , sex , Karnofsky Performance Scale score , Neurologic Function Scale score , histological type , contrast enhancement on preoperative MR imaging , preoperative tumor diameter , residual disease based on postoperative MR imaging , and baseline Mini-Mental State Examination score . RESULTS Between 1998 and 2002 , 111 eligible patients were entered into the study . In these 111 patients , the overall survival rates at 2 and 5 years were 99 and 93 % , respectively . The PFS rates in these 111 patients at 2 and 5 years were 82 and 48 % , respectively . Three prognostic factors predicted significantly poorer PFS in univariate and multivariate analyses : 1 ) preoperative tumor diameter > or = 4 cm ; 2 ) astrocytoma/oligoastrocytoma histological type ; and 3 ) residual tumor > or = 1 cm according to MR imaging . Review of the postoperative MR imaging results revealed that 59 % of patients had < 1 cm residual disease ( with a subsequent 26 % recurrence rate ) , 32 % had 1 - 2 cm residual disease ( with a subsequent 68 % recurrence rate ) , and 9 % had > 2 cm residual disease ( with a subsequent 89 % recurrence rate ) . CONCLUSIONS These data suggest that young adult patients with low- grade glioma who undergo a neurosurgeon-determined GTR have a > 50 % risk of tumor progression 5-years postoperatively , warranting close follow-up and consideration for adjuvant treatment Background — This cross-sectional study provides a practical approach for the clinical assessment of Friedreich ataxia ( FA ) cardiomyopathy ( FA-CM ) . Methods and Results — A comprehensive cardiac assessment , including  st and ard echocardiography , color Doppler myocardial imaging , cardiac magnetic resonance imaging , ECG , and exercise stress testing  , was performed in 205 FA patients . To assess myocardial hypertrophy in FA-CM , the end-diastolic interventricular septal wall thickness ( IVSTd ) was found to be the best echocardiographic parameter compared with cardiac magnetic resonance imaging – determined left ventricular mass . With the use of this parameter , 4 groups of patients with FA-CM could be defined . Patients with normal values for IVSTd ( 31.7 % ) were classified as having no FA-CM . Patients with an IVSTd exceeding the predicted normal IVSTd were classified as having mild FA-CM ( 40 % ) if IVSTd exceeded the normal value by < 18 % or as having intermediate FA-CM ( 16.1 % ) if IVSTd exceeded the normal value by ≥18 % . Patients with ejection fraction < 50 % were classified as having severe FA-CM ( 12.2 % ) . In addition to increased myocardial mass , severe FA-CM was further characterized by dilatation of the left ventricle , reduced systolic strain rate of the posterior wall , and ECG abnormalities . Regional myocardial function correlated negatively with FA-CM groups . Younger patients had a tendency for more advanced FA-CM . Importantly , no clear correlation was found between FA-CM groups and neurological function . Conclusions — We provide and describe a readily applicable clinical grouping of the cardiomyopathy associated with FA based on echocardiographic IVSTd and ejection fraction data . Because no distinct interrelations between FA-CM and neurological status could be determined , regular follow-up of potential cardiac involvement in FA patients is essential in clinical practice . Clinical Trial Registration — https://www . clinical trials.gov . Unique identifier : NCT00905268 In the last three decades , since Luft suggested a link between hospital volume and patient outcome , little has changed . Despite great efforts to formally quantify the relationship between volume and cancer survival , much uncertainty remains . It is intuitive that increased experience ( of the surgeon , the hospital or the ancillary staff ) translates to better patient care but closer analysis of the literature uncovers widespread inconsistency in study design question ing validity of conclusions . Although central isation of cancer services would seem beneficial , definitive evidence is only available regarding pancreatic and oesophageal disease and that pertaining to rectal cancer is heterogeneous and often flawed . A majority of evidence suggests better outcome for patients with rectal cancer managed in high volume centres . There are , however , many variables in data collection and interpretation rendering comparison between studies challenging . Definitions of the rectum range from distance from the anal verge ( inherent disagreement e range 10e16 cm ) , to relationship to sacral promontory ( subject to interpreter variation ) , and location of peritoneal reflection ( can not be interpreted endoscopically ) . Many authors fail to differentiate between sigmoid colon and rectum 3 and , those that do demonstrate wide variation . One of the largest studies , ( more than 7000 patients ) from the Californian Cancer Registry offered no description of tumour location . It concluded that patients undergoing surgery at high volume hospitals were less likely to have permanent colostomies and had better survival rates . The diagnosis of rectal cancer was , however , question able as it was based only on a discharge letter , often written by the most junior team member with no reference to true anatomical location . Most US studies are published from a few high volume institutions with a select patient cohort and are based on retrospective analysis of large data bases which , themselves are notoriously inaccurate . The European experience was recently explored by Ptok in a prospect i ve trial of almost 7000 patients undergoing resection for rectal cancer . This is arguably the most complete data set available to date and great effort was made to overcome common biases . A clear definition of the rectum was provided , hospital volume was
Development , validation , and incorporation of a small number of disease-specific patient-based instruments will improve the quality of research and care of CM-I patients
OBJECT Chiari malformation Type I ( CM-I ) is a common and often debilitating neurological disease .
Efforts to improve treatment of CM-I are impeded by inconsistent and limited methods of evaluating clinical outcomes .