Clinical Neuroscience News

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A Publication of the Cushing Neuroscience Institute, Member of North Shore-LIJ Health System Clinical NeuroscienceNews A YEAR IN REVIEW

Transcript of Clinical Neuroscience News

Page 1: Clinical Neuroscience News

A Publication of the Cushing Neuroscience Institute, Member of North Shore-LIJ Health System

ClinicalNeuroscienceNews

A YEAR IN

REVIEW

Page 2: Clinical Neuroscience News

ClinicalNeuroscienceNews

The Vulnerable Lower Back 4December 2012

Emergency Neurosurgery for the Brain 8March 2013

Advanced Treatment for Craniocervical Instability 12June 2013

Adolescent and Adult Scoliosis 16September 2013

Taking on Brain Tumors 20December 2013

A YEAR IN REVIEW

Designed, written and produced by

Onward Publishing, Inc. 631-757-8300

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NORTH SHORE-LIJ HEALTH SYSTEM 3

Dear Readers,

Welcome to New York Neurosurgery, a symposium coordinatedby the Cushing Neuroscience Institute of the North Shore-LIJHealth System. This program focuses on the state-of-the-arttreatment and management of brain tumors, cerebrovasculardisorders and head trauma.

We would like to share with you this collection of recent articlesfrom our newsletter Clinical Neuroscience News describingfacets of the neurosurgical work being done at the CushingNeuroscience Institute.

The first report focuses on the vulnerability of the lower backand the second on our multidisciplinary response to traumaticbrain injury. The third report looks at cutting edge surgicalapproaches to remodeling the complex of bony structures thatmake up the cranio-cervical junction in patients suffering fromcongenital or accident-related cranio-cervical instability (CCI).Also included here are articles on the newest neurosurgicaltreatments for scoliosis and brain tumors. The highly variable,three-dimensional spinal deformity known as scoliosis typicallydevelops in adolescence but occasionally develops de novo inadulthood, requiring individualized solutions and a suite ofadvanced intraoperative imaging tools. As set forth in our braintumor article, our team of neurosurgeons, neuro-oncologists,radiation and medical oncologists, neuroradiologists and othersprovide the latest state-of-the-art diagnostic and multi-modalitytreatment options for this potentially life-threatening disease.

We thank you for your attendance at the symposium and hopeyou find interest in this publication as well.

Raj K. Narayan, MD, FACS

Chair, Department of NeurosurgeryNorth Shore University Hospital

and Long Island Jewish Medical Center

Senior Vice President and Executive Director,Neuroscience Service Line

Chair and Professor, NeurosurgeryHofstra North Shore-LIJ School of Medicine

A Letter from Raj Narayan, MD, FACS

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The lower back carries most of the torso’s weight. Constantlystrained by lifts and twists, the lumbar region is exceptionallyvulnerable to a variety of acute and degenerative injuries.Each incident tends to be more severe and debilitating than the last, until neither simple analgesics nor steroidinjections provide relief. At that point, surgical intervention is considered.

Many lower back injuries start with an acute injury, but farmore cases of lower back pain are the result of cumulativedamage brought on by poor posture, sedentary habits, excessbody weight, osteoarthritis, sports injuries or the constantbiomechanical stresses of flexing, bending, twisting and lifting.

Over time, the discs that separate and cushion the vertebraljoints lose fluid and become less flexible, herniate or tear, orthe foramen within the spinal canal become narrower due toosteophytes, commonly known as bone spurs. What remainsare entrapped nerves and bony surfaces grinding against bonysurfaces that produce excruciating pain signals, numbness,tingling and reduced mobility. These symptoms set off avicious cycle of more physical inactivity, further loss of back muscle strength, and progressive misalignment of thevertebral column as the back adjusts to protect the weakened structure.

At the Cushing Neuroscience Institute at North Shore-LIJ,medical treatment and pain management options throughdrugs, epidural injections and physical therapy are exhaustedbefore any kind of surgery is considered. Until recently,surgery involving disorders of the spinal column requiredmajor open surgery, entailing general anesthesia, deepincisions, substantial blood loss and partial immobilization ofthe patient during lengthy rehabilitation. While the outcomesfrom open surgery can be excellent; it is rarely suitable forelderly patients, and younger patients are understandablyreluctant to devote the time it takes to recover from theeffects of deep tissue injury that accompanies open surgery.

For these and other reasons, more patients and their referringphysicians are turning to neurosurgeons and advancedmedical centers that offer minimally invasive surgery (MIS) as a better alternative for most lower back pathology. Theadvantages of MIS are many, starting with decreased tissuetrauma. Muscles can be gently separated instead of cutthrough to access the spine, and the one or possibly twoincisions needed average one inch. Less blood loss, less painand scarring, shorter time in the OR, fewer complicationsand the possibility of same-day recovery and discharge are

other persuasive considerations. Even patients in their 80sand 90s can tolerate MIS back surgery in the hands of anexperienced MIS specialist.

Minimally invasive surgery is carried out by neurosurgeonswith advanced subspecialty training. Our neurosurgeons are not only recognized pioneers in the development of MISback surgery and its supportive technology, but they areconsistently among some the most active practitioners in the region.

Our MIS surgical suites are also equipped with the newestgeneration of technology. One of the essential new materialsused in MIS surgery are osteobiologics. These includerecombinant bone morphogenic protein (BMP), smallersurgical instrumentation and a remarkable selection ofminiaturized stabilization hardware. Interbody spacers, madeespecially for MIS procedures, insert tidily between vertebraeas part of reconstruction.

We also have the newest 3D image-guided visualizationsystems, including intraoperative microscopes and the C-armfluoroscope, the latter a mobile x-ray machine that providesour neurosurgeons a 360-degree view of the spine. Lastly, ourMIS specialists have the newest and most sensitive devices tomonitor nerve function in real time as they proceed.

Among the many advanced MIS procedures offered forlumbar surgery at the Cushing Neuroscience Institute are:

• Microdiscectomy to treat a herniated lumbar disc withradicular symptoms due to nerve compression.

• Arthrodesis or fusion for refractory pain traceable to disc degeneration, fractures, spondylolisthesis and spinalinstability.

• Kyphoplasty/vertebroplasty, MIS approaches to treat andstabilize vertebral compression fractures. Primarily due to osteoporosis, VCFs occur six times more frequentlyin women than men. Both procedures involve injection of bone cement into the fracture to stabilize and seal the break.

In a kyphoplasty, a balloon is first inserted through the needle intothe fractured bone to create a cavity or space into which thebiocompatible material is injected. The needle is removed and thebiomaterial is allowed to harden. The small opening is covered with abandage. This procedure can take from 1-2 hours, about a half-hourfor each treated vertebra.

The VulnerableLower Back

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NORTH SHORE-LIJ HEALTH SYSTEM 5

Peter Hollis, MD, FACS

A 56-year-old woman was first seen in January 2011,complaining of difficulty walking due to progressive backpain, leg pain radiating down both legs and imbalance.An MRI revealed significant cervical spondyliticmyelopathy and she subsequently underwent an anteriordiscectomy and fusion at C4-C5 and C5-C6 at NorthShore-LIJ. Though the surgery was successful in relievingher cervical pain, she continued to experience intensifyinglower back and leg pains, which were initially treatedelsewhere with physical therapy, epidural steroids andchiropractic. Additional scans taken at North Shore-LIJshowed L4-L5 spondylolisthesis and stenosis and afterdiscussing her options, we determined that an L4-L5posterior interbody fusion and reduction of herspondylolisthesis via MIS would give the greatest relief.

Placed under general anesthesia, wired withneuromonitoring equipment, and under C-arm 3-D imageguidance, the patient received two 1.5-inch incisions oneither side of the spinal midline over the L4-L5complexes. Following removal of a small portion of bothsides of the lamina (laminotomy) and partialdecompression of the corresponding nerve root(facetectomy), we prepared the disc space for insertion ofan interbody prosthesis filled with a mixture of BMP andautologous bone material. Once the prosthesis was well-seated and the collapsed interior space distracted, weperformed a bilateral discectomy. The space wasexpanded using the screw extenders of the PEEKinterbody fusion system until neuromonitoring signalsindicated that nerve compression was resolved. Thesurgical area was given a final irrigation, surgical toolsremoved and the anatomical layers of soft tissue closed.

Within six weeks, the patient reported that herclaudication symptoms were gone, that her wounds hadhealed without incident, that her rehabilitation exerciseswere restoring muscle tone and that she was lookingforward to returning to work shortly.

Case StudyMIS Interbody Fusion and Bilateral Discectomy

Preoperative imaging showing L4-L5spondylolisthesis.

Preoperative imaging showing spinalstenosis and spinal cord compression.

Postoperative imaging showingplateless cervical fusion.

Postoperative imaging showing reduction of spondylolisthesis and stenosisand the screw rod fixation.

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A major source of lower back pain is degeneration ofcartilage within the intervertebral discs (IVDs) of the spine. While years of wear and tear, acute injuries and agingare variously cited as the triggers of degeneration and attendant pain, it has also been posited thatinflammatory mechanisms may play a destructive role,independent of injury.

To test this hypothesis, neurosurgeon Mitchell Levine, MDand investigator Nadeen Chahine, PhD, at The FeinsteinInstitute for Medical Research Biomechanics andBioengineering Lab, set out to track the expression and responses of the Toll-Like Receptor TLR4. Thoughpreviously implicated in the disease mechanisms ofcartilaginous breakdown, no detailed study had ever tested the premise in the context of IVD degeneration.

Classed as a “pattern recognition receptor,” TLR4 is aprotein associated with the innate immune system,providing host defense against microbial infections. Further,TLR4 is activated by stimulation with the TLR4 ligandlipopolysaccharide, resulting in upregulation ofproinflammatory cytokines; it also downregulates thesynthesis of extracellular matrix which slows tissue repair.

Dr. Chahine’s study, which involved stimulating IVD cellsby injecting the TLR4 ligand (LPS) in the intervertebraldiscs of animal subjects and measuring inflammatorycytokine levels, showed moderate degenerative changes.Drs. Levine and Chahine conclude that this study providesthe first solid evidence that inflammation can exert a directrole in cartilage degeneration of the IVD.

The next step, Dr. Chahine reports, will be to examinehuman responses to TLR4 activation and inflammation.They plan to enroll 100 patient volunteers undergoingspinal surgery, using damaged tissue removed during theirprocedures for laboratory investigation. The New YorkState Department of Health is providing a grant to bring on board a research fellow for one year to furthertheir work.

Inflammatory Mechanisms in Cartilage Degeneration

TLR4

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Peter Hollis, MD, FACS

A 52-year-old woman was referred to us for aneurosurgical evaluation in May 2012. She describedhaving experienced a “pop” in her lower back two weeksearlier. The pop had rapidly developed into severe sciaticaof the left leg, accompanied by numbness, tingling andoccasional buckling. She was placed on oral steroids andanalgesics, and the pain, initially 10/10, subsidedsignificantly to 4/10. The weakness and numbnesscontinued and further MRI analysis found L3-L4 lateraldisc herniation. During the examination, straight legraising was found to be positive on the left side, left kneereflex was absent, and there was decreased sensation onthe left thigh.

The patient’s surgeon recommended a microdiscectomy,which was performed one week later.

The patient was prepped in the usual manner, receivinggeneral anesthesia, lower extremity EMG monitoring, andC-arm visualization. A small incision was made and thefascia beneath opened. A quadrant retractor was placedover the L3-L4 facet and lamina revealing the L3 and L4transverse processes. A high-speed burr was used to carryout a left hemilaminectomy. Upon further penetration ofthe ligamentum flavum, a severe compression of the leftL3 nerve root was also revealed and a completefacetectomy performed to achieve decompression.

Finally, a discectomy was carried out and the disc spaceprepared for an appropriately sized interbody prosthesis.A combination of BMP and autologous bone was tampedinto place within the prosthesis and the entire unitinserted under C-arm guidance. The pedicles were thenthreaded, titanium screws inserted, and a rod inserted onthe left at L3-L4 to lock the fixation. The wound wasclosed, a sterile dressing applied, and the patient was sentto recovery.

She was instructed to do light exercising and stretching athome, and returned for post-status check-ups at one andfour weeks. She reported greatly reduced radicularsymptoms as well as improvement in strength andfunction. X-rays showed solid construct and alignment ofthe spine at the surgical site.

Case StudyMicrodiscectomy

Preoperative MRI showing L3-L4 disc herniation.

Postoperative MRI showing stabilizationafter discectomy and fusion.

Postoperative MRI showing stabilizationafter discectomy and fusion.

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Traumatic brain injury (TBI) affects up to two percent of thepopulation per year. These incidents take a higher toll amongthe young, who are more likely to be involved in high-impactsports, dangerous lines of work, car accidents and assaults.Close behind are the elderly, who are prone to serious falls,and whose injuries are often complicated by being on blood-thinning medications.

The degree of permanent damage due to a TBI may dependless on the primary injury than it does on the cascade ofsecondary complications that can set in hours or even daysafter the primary injury. The ability to identify problemsbefore they worsen and to respond aggressively is critical tothe ultimate outcome.

For these reasons, TBIs require a range of highly specializedneurosurgical services and post-surgical monitoring that isonly available at certain specialized hospitals. Our dedicatedteam is on call 24/7 to evaluate patients in the emergencyroom, identify those who may require surgical interventionand carefully monitor the others as appropriate.

The first critical task in the ER is to evaluate the degree ofinjury. This determination is usually made based on aphysical examination combined with information from theattending paramedics or witnesses to the injury. Mostpatients also are given a CT scan of the head.

Generally, 80 percent of TBIs are classified as mild with aGlasgow Coma Score (GCS) of 13 to 15. These patients areawake and are able to speak and follow commands. Theymay be confused and lethargic, but should be easilyarousable. The other 20 percent are evenly divided betweenmoderate (GCS of 9-12) and severe (GCS of 3-8). Themoderate TBI patients have an altered sensorium, but are stillable to follow commands. The severe TBI patients arecomatose and are unable to follow even simple commands.For these patients the outcome is less certain.

Closed head injuries are typically the result of blunt traumaor a fall in which the head takes a significant hit. The impactresults in stresses as the brain sloshes repeatedly from side toside within the skull before coming to rest. This motion canstretch and tear neural cells, causing diffuse disruptions in thebrain’s communication network. Closed TBI may also resultin a brain contusion or bruising. This is usually managedwithout surgery, unless the associated brain swelling becomes severe.

Mild TBI:Most patients with a mild TBI do not requiresurgical intervention, and ultimately make a good recoveryafter a period of observation, medications and time.However, a small proportion of these patients can suffer frompost-concussion syndrome, with symptoms that can includememory loss, headaches, dizziness and loss of smell.

Moderate and severe TBI: The likelihood of needingsurgical intervention rises in patients with moderate or severeTBI, and is usually determined by an initial CT scan followedby a CT scan a few hours after admission to check for agrowing hematoma. This is especially true if the initial CTscan shows any bleeding or if the patient is on blood-thinningmedications. Intracranial pressure (ICP) monitors are oftensurgically placed on comatose patients.

Hematomas: There may be a large hematoma outside thedura (epidural hematoma), or within the dura (subduralhematoma). If these hematomas are causing significantpressure on the brain, they need to be surgically removedwith a craniotomy, which involves cutting a hole in the skullto temporarily remove a bone flap in order to access thebrain. Sometimes, in a procedure known as decompressivecraniectomy, the skull bone is not replaced or may beremoved a few days after the injury, in order to allow the brain to swell without the ICP going to dangerously high levels.

Penetrating or open head injuries:Most penetratinginjuries are caused by firearms, and vary in the damagecaused by the velocity and rotational impact of the type of bullet. The management of these injuries is very specific to the type of injury, its location and the patient’s overall condition.

Post-operative care: After surgery, the patient is transferredto the ICU and continuous monitoring begins. During thistime, staff watches for the subtle signs of secondary injuries.These include excessive ICP, low blood pressure, fever, high glucose levels, low serum sodium levels and seizures. All of these factors can interfere with normal recovery.Unfortunately, they are very common and occur in almost all patients. Using an array of state-of-the-art tools, ourspecialized teams are prepared to counter with aggressive,rapid action when indicated. Delayed intracranial hematomasor brain swelling can also require urgent surgery.

Monitoring devices: Intracranial pressure monitoring androutine CT scans form the mainstay of monitoring TBIpatients. Electroencephalography (EEG) is being increasinglyused as we recognize the frequency of silent seizure activity inthe injured brain. Brain oxygen monitoring is also valuable insome cases. Advanced transcranial Doppler (TCD)ultrasonography is sometimes used to assess dynamic changein the brain’s blood flow velocity and perfusion. When thepatient is more stable, MRI diffusion tensor imaging (DTI) isperformed to give us higher resolution imaging of whitematter, not only in the acute phase but also during therecovery phase.

EmergencyNeurosurgeryfor the Brain

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Rick Madhok, MD

A 60-year-old woman was foundunresponsive at home, presumed tohave sustained a severe closed TBIdue to a fall. Rushed to the ER byambulance, her GCS was a criticallylow 6. A CT scan showed a largesubdural hematoma with resultingcerebral swelling and herniation,though much of the blood collectionwithin appeared to be old, as thoughthe primary injury had occurredseveral days earlier. The resultingcompression had caused the brain toundergo a large midline shift, and anemergency craniotomy was

performed. Re-expansion of the brainwas nearly complete 24 hours later.

Postoperatively, the patient showedsome improvement in pupil reactivityas well as in facial motor responses,but issues of functional connectivity,visible on a series of fMRIs,continued to indicate underlyingstructural damage to white matterassociated with diffuse axonal injuryand cognitive impairment.

The patient was eventually moved toa sub-acute facility where she initiallyshowed little improvement beyondwithdrawal motor responses. Bymonth five, however, when it was

time for her to be readmitted to thehospital for a cranioplasty, she wasable to move about in a wheelchairand to walk short distancesunsupported.

The patient’s skull was closed using atitanium plate.

After the procedure, her conditionsteadily improved, and she regainedthe ability to speak and could moveabout more easily. Her recoveryexceeded the expectations of her doctors and family, and she hascontinued to gain strength andcognitive function since then.

At The Feinstein Institute forMedical Research, neurosurgeon RajNarayan, MD and electrical engineerChunyan Li, PhD, have beencollaborating with scientists at theUniversity of Cincinnati since 2008on the development of what theyanticipate will be the next generationof brain monitoring devices. Their“lab-on-a-tube” is a catheter systempacked with multiple miniaturizedsensors that allow for real-timecontinuous in vivo monitoring ofcritical parameters such as braintissue oxygen, blood flow,intracranial pressure, temperature,EEG and brain chemistry.

Using microelectromechanical(MEMS) technology, their smartcatheter is compact, simple to useand highly accurate.

It includes sensors both inside thetube and outside, so in addition tomeasures taken from thecerebrospinal fluid (CSF) by itsinterior sensors, the outer sensorscan transmit data about thecondition of the injured brain tissue immediately surrounding the catheter.

Progress toward a clinically-readydevice took a great step forward in2010 when the US Department ofDefense provided $5 million towardsthe research, in recognition of thesmart catheter’s potential to improveoutcomes for soldiers suffering TBIin combat. The grant is shared byDr. Narayan and Dr. Li at NorthShore-LIJ and their colleagues at theUniversity of Cincinnati.

Lab-on-a-Tube

Case StudySubdural Hematoma with Diffuse Axonal Injury

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Rick Madhok, MD

A 59-year-old woman was struck by an automobile afterrunning into the road after her dogs. The impact resultedin a severe closed head wound. On arrival in the ER, herGCS was 9 and an initial CT scan showed diffusehemorrhaging and a small frontotemporal hematoma.

After stabilization, she was moved to the ICU and placedon an intracranial pressure monitor. Over the next fewdays, the intracranial injury evolved, generating anincrease in intracranial pressure. When the patientproved unresponsive to medical therapy, a bifrontalcraniotomy was performed and a shunt was inserted intoher right ventricle to provide drainage. The patientremained in the hospital for six weeks and was thendischarged to a subacute rehabilitation facility.

A month later, she was readmitted to the hospital foracute secondary hydrocephalus, which required insertionof another right external ventricular drain. The patientremained in hospital for another six weeks, undergoing abifrontal cranioplasty when the swelling subsided.Within two months she was making substantial progress,able to track moving objects and to speak haltingly.Within four months she had regained spontaneousmovement of all four extremities as well as her ability toread and process written words. She continues to makeprogress through rehabilitation.

Case StudyAcute Closed TBI andSecondary Sequelae

CT scan without contrast demonstrating diffuse injury.

CT scan without contrast showing evolution of intracranialinjury with increase of intracranial pressure. Patient is

unresponsive to medical therapy.

CT scan without contrast demonstrating decompressive craniectomy.

CT scan without contrast demonstrating cranial reconstructionand shunt placement.

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NORTH SHORE-LIJ HEALTH SYSTEM 11

Rick Madhok, MD

A 45-year-old woman sustained multiple skull, facialand skeletal fractures as a result of being hit by a motorvehicle while out walking on New Year’s Day.Paramedics called to the scene found her bleedingprofusely from the head, but responsive to verbalstimuli, her functional capacities intact, and her eyesswollen and closed bilaterally.

She was taken into surgery for an emergent craniotomyto relieve an epidural hematoma and for repair ofmultiple skull base fractures. An oromaxillofacialsurgeon fixed her broken jaw and a plastic surgeonattended to a 10 cm facial laceration and othercontusions caused by her contact with the pavement.

After several days in the ICU, she had her fractured leftelbow surgically repaired. Since that time, she has madea remarkable recovery through rehabilitation and homerest. She is now back at work.

Case StudyMultiple Trauma Case

CT scan without contrast demonstrating initialsmall hematoma.

CT scan without contrast showing expansion of hematoma.

CT scan without contrast showing decompression of epidural hematoma.

GlasgowComa Scale

EYE OPENINGRESPONSE

SPONTANEOUS 4 POINTS

OPENS TO VERBAL COMMAND 3 POINTS

OPENS TO PAIN 2 POINTS

NONE 1 POINT

VERBAL RESPONSE

ORIENTED 5 POINTS

CONFUSED 4 POINTS

INAPPROPRIATE 3 POINTS

INCOMPREHENSIBLE 2 POINTS

NONE 1 POINT

MOTOR RESPONSE

OBEYS COMMANDS 6 POINTS

PURPOSEFUL 5 POINTS

WITHDRAWS FROM PAIN 4 POINTS

ABNORMAL FLEXION/DECORTICATE POSTURE 3 POINTS

EXTENSOR RESPONSE/DECEREBRATE 2 POINTS

NONE 1 POINT

The Glasgow Coma Scale is a tool formeasuring level of consciousness,especially after a head injury.

Highest Score .........15Coma ......................8 or lessLowest Score ..........3

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Craniocervical instability (CCI) is a condition where thecervical spine is unable to maintain adequate alignment andsupport of the cranium during certain movements, allowingthe head to “wobble.” The underlying cause of CCI isstructural: one or more bony and/or ligamentous anomaliesinvolved in the craniocervical junction prevent normal rangeof movement of the head and neck. In many instances, theinstability reduces the cervical spaces through which thebrainstem and spinal cord pass, causing pressure on thedelicate soft tissue, with neurologic and vascularconsequences as well. Cushing Neuroscience Institute’s ChiariInstitute is one of the leading centers for the evaluation andtreatment of CCI in the country. While some patients withCCI will improve sufficiently with conservative non-surgicaltreatment, the remainder require complex surgical correctionand realignment. The sooner such interventions are begun the better the outcome; CCI tends to worsen over time,eventually causing irreversible damage to spinal nerves and muscles and loss of physical strength and mobility.

Adding to the challenge for primary physicians andpediatricians, identifying CCI in a patient can be difficult.The first symptoms tend to be subtle, ambiguous andintermittent; younger patients in particular may havedifficulty describing the various sensations they experience.Since 50 percent of diagnosed CCI cases are associated withcertain congenital disorders — Down syndrome, congenitalscoliosis, cerebral palsy, ankylosing spondylitis, osteogenesisimperfecta, neurofibromatosis and connective tissue disorderssuch as Ehler’s-Danlos — special attention should be paid toany member of this cohort exhibiting one or more symptoms.The most common precursor of accidental CCI is whiplash— the dynamic extensions and flexions of head and neckassociated with automobile crashes and contact sports. Inrare instances, the cause is iatrogenic — the result of boneremoval during a prior surgery to cut away a tumor or treatChiari syndrome. Ironically, the squeezing of the parts of thebrain through the hole at the base of the skull, referred to asthe Arnold Chiari or Chiari I malformation, is sometimestaken to be the cause of signs and symptoms when actuallythe squeezing results from craniocervical instability.

Common CCI symptoms to look for in patients withotherwise undiagnosed cervical complaints include headachesin the occipital space at the back of the neck and skull,painful neck, torticollis, tingling and numbness in the upperextremities, muscle weakness, difficulty walking, difficultyswallowing, sleep apnea, snoring, drooling, progressivehearing loss and recurring fatigue. Drop attacks, provoked

by specific movements of the head and neck that causetemporary ischemia, are another. In the pediatric andadolescent populations where physical growth can play a part in the timing of CCI onset, an indicator may be assimple as an increasing reluctance to participate in play.

Primary care physicians who suspect CCI in a patient shouldinitially order an MRI of the area to visualize the anatomyand rule out other causes. At the Chiari Institute, experiencedneurologists and neurosurgeons can determine which patients are likely to improve with conservative intervention;these patients are prescribed physical therapy, strengtheningroutines, relaxation training and medications. Patients withacute or multiple CCI symptoms that will not respond totherapy alone are considered for surgical stabilization and/or fixation.

At the Chiari Institute, all CCI surgery is performed by ateam of neurosurgeons. Each patient’s anatomy is unique, soeach surgery requires extensive, pre-planned imaging andbioengineering analysis to determine the patient’s operativestabilization and fixation. Days before the surgery, the patientgoes through several hours of advanced dynamic MRI. Thispinpoints the location of millimeter-thin compressions thatare causing brain and spinal cord symptoms. We alsomeasure several critical craniocervical angles against norms;these provide a basis for intraoperative surgical stabilizationand fixation.

The surgery itself is very delicate, typically taking 5-6 hours.After the patient undergoes general anesthesia, the surgicalteam makes one opening, approximately 10-15 inches inlength from the top of the head to the back of the neck. Asthe surgical field is highly trafficked with vertebral arteriesand veins, the brainstem and critical cranial nerves, each stepin the realignment of bones, the remodeling of angles and inthe relief of compression points requires exquisite surgicalprecision. A unique feature of our approach is the use ofintraoperative ultrasound during surgery, in addition tocontinuous brainstem and spinal cord monitoring. Thisguides precise adjustments during our realigning andstabilization of the cranium on the cervical spine. Post-operative recovery in the hospital takes 4-5 days, followed by rehabilitation and a gradual return to normal activities.

Advanced Treatment forCraniocervical

Instability

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NORTH SHORE-LIJ HEALTH SYSTEM 13

Harold L. Rekate, MD, FACS, FAAP andSalvatore Insinga, DO

An eight-year-old girl with multiple birth defects related to monosomy ofchromosome 18p, was brought to us from her home in another state for aneurosurgery consult. She is of extremelyshort stature, has hypothyroidism andasthma, and is moderatelydevelopmentally delayed with littleindication of language facility; her medicalhistory includes previous surgeries toaddress various physical limitations. Weevaluated her to examine the causes of herextreme difficulty in swallowing; she wastaking all her nourishment through agastrostomy tube. She also had frequentbouts of uncontrolled coughing.

Our MRI scans showed a severe Chiari Imalformation together with bonyabnormalities of the anterior skull base,resulting in occipitocervical instability,anterior compression of the brainstemfrom a retroflexed odontoid and basilarinvagination. We advised and performed adecompressive surgery, realignment of theoccipital cervical region and fixation toreduce instability, as these are key to thepatient’s swallowing issues and possiblyother problems. Intraoperatively, we foundher spinal cord sensory signals to benormal, but her motor signals extremelyattenuated. Using ultrasound guidance,

our team first performed a surgicalreduction of her occipitocervicalinstability. Next, under microscopicvisualization, we did an extensivedissection of the scaring over the cerebellartonsils and an autologous tissue graft was sewn in place to expand that area.Subsequently the patient’s head was gentlyrepositioned in extension and distraction,followed by insertion of permanentfixation. This reduced pressure on theanterior brainstem, thus improving motorspinal cord signals dramatically.

By the fifth day, postoperatively she wasambulating under oral pain medication. By the eighth day, the patient showed amarked improvement in her ability toswallow and to eat ordinary solid foodcomfortably. She was also speaking moreclearly, walking well and was in minimalpain. She returned home where shecontinues to improve.

Look for these symptoms of CCI inpatients with otherwise undiagnosedcervical complaints:

• Headaches in the occipital space at theback of the neck and skull

• Painful neck

• Torticollis

• Tingling and numbness in the upperextremities

• Muscle weakness

• Difficulty walking

• Difficulty swallowing

• Sleep apnea

• Snoring

• Drooling

• Progressive hearing loss

• Recurring fatigue

Common Symptoms ofCraniocervical Instability

Case StudyChiari Malformation

Preoperative sagittal cervical MRI showingbasilar invagination.

Postoperative MRI of the cervical spine showingreduction of the odontoid (C2) process.

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Harold L. Rekate, MD, FACS, FAAP andSalvatore Insinga, DO

A 13-year-old female with Ehlers-Danlos syndrome, aninherited disorder of connective tissue, came to us forevaluation after undergoing two failed operations forChiari I malformation with syringomyelia at an out-of-state institution. The child presented with incapacitatingheadache, neck pain and loss of balance that confinedher to a wheelchair. Through advanced imaging andother diagnostic tests, we identified a pseudomeningoceleand an anterior compression of the brainstem due tosevere instability of the cervical spine in the regions ofC1, C2 and C3.

Our team used a combined procedure to correct theexisting defects. In the first stage, the anesthetizedpatient was put in a Mayfield head holder under mildflexion while she underwent a posterior fossadecompression revision. Several holes in the dural patchattributed to the earlier craniectomies were found to beleaking spinal fluid and were closed. At this pointintraoperative ultrasound was brought in, revealing thatthe anterior brainstem was being distorted by theodontoid process, significantly reducing ventral CSFflow. We then performed an occipitocervical fusion.Adjusting the head holder, the patient’s neck was put insignificant extension. When we determined thatsufficient space was opened between the brainstem andthe odontoid process to achieve decompression, thefusion was completed.

The patient’s recovery was uneventful and on the fifthday, postoperatively she was discharged home. Beginningphysical therapy six weeks later, she subsequentlyimproved to the point that she was able to return toschool and resume normal activities.

Case StudyChiari 1 Malformation withSyringomyelia

Preoperative sagittal cervical MRIshowing basilar invagination.

X-ray of the cervical spine showing the instruments usedto fix the position of the skull and spine to prevent

damage to the front of the brain.

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NORTH SHORE-LIJ HEALTH SYSTEM 15

Harold L. Rekate, MD, FACS, FAAP andSalvatore Insinga, DO

This 46-year-old female came to us with a long history ofhealth problems consistent with fibromyalgia and chronicfatigue syndrome. She reportedly managed well enoughwith exercise until 2007 when she hit her head on the edgeof a cabinet and suffered the first of her chronic severeprolonged headaches. Since then, the headaches began tointerfere with her ability to work. She also reportedgastroesophageal reflux, irritable bowel syndrome,lightheadedness, pervasive fatigue, burning pain in neckand shoulders, tingling and numbness in extremities andnystagmus. Her pain reportedly spiked with the Valsalvamaneuver and was relieved when lying down.

Consulting neurosurgeons near home, she was assuredthat she did not have Chiari I. She then sought treatmentat a renowned, out-of-state headache institute, hadBotox® injections to reduce muscle tension, and tookvarious pain relief medications, none of which broughtrelief. In 2010, coincident with a lumbar puncture test, sheexperienced two weeks of low-pressure headachesfollowed by a week of improvement, leading anotherspecialist to diagnose pseudotumor cerebri or idiopathicintracranial hypertension. The patient was urged to trytherapeutic lumbar punctures every 4-6 weeks. She thensought our expertise.

We supplemented MRIs from her previous physicians withour own dynamic MRI and identified a severe degree oftonsillar descent. Her images indicated an abnormallyacute retroflexed odontoid process causing distortion ofthe upper end of the spine into the brainstem. We alsodetected the absence of a cisterna magna between thecerebellum and the medulla oblongata where CSFnormally drains.

Surgical intervention consisted of performing anoccipitocervical decompressive craniectomy to relieve thearea of neural compression. Subsequently, usingultrasound guidance, traction and realignment proceededto establish CSF flow to a newly created cisterna magnaanterior to the brainstem. Intraoperative fluoroscopic 3Dimaging showed that the cervical spine was now in normalalignment. The operation concluded with fixation andfusion of the cranium to the cervical spine in this newlyformed angle. Postoperatively the patient was in animproved state and returned home.

Case StudySevere Headache Resultingfrom Craniocervical Instability

Preoperative sagittal brain and cervical MRIshowing length of 9mm Grabb.

Postoperative MRI of the cervical spine showing that theelements of the cervical spine in front the brainstem are no

longer pushing into the brain.

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16 CUSHING NEUROSCIENCE INSTITUTE

Scoliosis is a complex three-dimensionaldeformity of the spine. The condition causeslateral curves of the spine to the right or left,decreased forward and/or lateral flexion, andasymmetric shoulder and pelvic positioning. Inmore pronounced cases of vertebral rotation, arib hump develops on the convex side of thecurve and a lesser protrusion on the chest. Inmore recent years, an increased number ofpatients have been seeing neurosurgeons to correcttheir deformities. With fully half of aneurosurgeon’s seven-year-long residency trainingdevoted to spinal disorders, and another year ormore of fellowship training specifically in treatingdeformities, our team of neurosurgical specialists atthe Cushing Neuroscience Institute are particularlywell-suited to diagnosing and treating thecomplexities of both adult and adolescent scoliosis.

Not all consultations at the Cushing NeuroscienceInstitute lead to surgical intervention; our solutionsare highly individualized, depending on eachpatient’s age, discomfort, coexisting conditions,functional limitations, progress of the disease andcosmetic appearance. We begin our evaluationwith a series of visual and radiographic tests. A key indicator is the patient’s Cobb angle, ameasurement of the angle of curvature of thespine at its apex. An adolescent with a Cobbangle less than 40 degrees is usually treatedmedically with physical therapy andtemporary orthotic bracing, on the premisethat the developing bone and musculature are still malleable enough to respond tononinvasive correction. Watchful waiting that includes yearly serial radiographicevaluations is the prescribed follow-up, with the understanding that surgery may be needed later.

By contrast, we regard any teenagerpresenting with a Cobb angle between 40-50degrees to be in a “gray zone” concerningsurgical intervention. Not only is the curve —in being more pronounced — less amenable toeasy correction with bracing, but theyoungster’s own self-image and level of activityare probably suffering too. We also look atprior history to get any clues as to how fast thecurve has progressed and whether there is afamily history of scoliosis.

Lastly, when we see an adolescent patient with aCobb angle of 50 degrees or more we startthinking about an operation, the sooner thebetter, as this degree of deformity is usually asign that it will continue to grow if unopposed.

A different set of considerations guides ourtreatment of adult scoliosis. The conditionmost commonly develops de novo at an olderage, though a small percentage of casesactually trace to mild childhood scoliosis thathas gone previously undetected. As the spinegoes through wear and tear, compensatoryligamentous and muscular stabilizers can nolonger maintain alignment, as the naturalregional curves of the spine weaken,significant sagittal plane imbalance (upperbody pitched forward) may develop. In yetanother version — degenerative scoliosis —the condition may arise in later life,particularly as the result of arthritic changes.The disc spaces and facet joints collapseasymmetrically, causing coronal plane (side-to-side) imbalances, with frequent nerveimpingement a result.

The most common clinical complaint of adultscoliosis is chronic back pain and radiculopathy(leg pain). The pain may be caused by theunequal tension placed on back muscles andligaments, or asymmetrical loading, whenindividual facet joints compress nerve roots asthey are pulled out of alignment. Radicular painand claudication, neurological symptomsincluding numbness in the extremities, are othercommon sources of discomfort.

Neurosurgeons tailor surgical treatment optionsto the patients in order to get the maximumresult with the minimum amount of surgery. Ifconservative treatments have failed andsurgery is an option, minimally invasivesurgery is considered foremost. However, ifopen surgery is elected due to the patient'sneeds, physicians will customize the surgeryto the individual patient. Thanks to thepractice of using intraoperative imaging(C-arm fluoroscopy), and when needed,stereotactic guidance to place pediclescrews, we are able to achieve positiveresults in the treatment of adolescentand adult scoliosis patients.

Adolescent and Adult Scoliosis

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NORTH SHORE-LIJ HEALTH SYSTEM 17

Ahmad Latefi, DO

A 16-year-old young woman was referredto us complaining of significant back painmostly at the thoracolumbar junction. Her postural presentation revealed threeabnormally exaggerated coronal curves, at least one of them presumed to bestructural and the other(s) compensatory.She reports having been examined forscoliosis eight years earlier at which timemild idiopathic scoliosis was detected anda conservative treatment approachrecommended. A review of those earlierfilms showed a clear progression of thecurvatures, with the apex of the deformityat the T12-L1 site estimated at over 60degrees. Physical examination showed thather sensory responses and reflexes werenormal, her muscle strength 5/5, hergeneral state of health good.

Before a treatment plan could bediscussed, the patient was sent for anumber of full-length x-rays, an MRI tovisualize the spinal cord, and a CT scan ofthe cervical, thoracic and lumbar spine toassess the integrity of bony structures andthe size of pedicles. Seeing clearly that the

thoracolumbar curve was the site of thedeformity and of the dynamic rotation, we recommended surgical correction,including insertion of instrumentationfrom T8 to L4 vertebral levels, followedby osteotomy and fusion.

During summer vacation in July, sheunderwent the proposed procedure, usingintraoperative sensory and motor EPs tomonitor integrity and CT scans to assessand plan for the correct placement of themultiple fixation screws that wouldanchor the instrumentation.

Fourteen days postoperatively, the patientreported restored balance and recedingpain. She is able to stand straight, headpositioned normally over the midline ofher pelvis. Long-cassette x-rays show herCobb angle was now corrected to anacceptable 25 degrees. At her three-monthfollow-up, she had healed nicely, felt good,was back in school and very happy withher results.

Case StudyExaggerated Coronal Curves Fixed

Ahmad Latefi, DO

A 28-year-old female native of a foreigncountry, currently studying in America,was referred to us in June 2012. Shereported that she had been diagnosed withadolescent idiopathic scoliosis, but thatshe deferred treatment until coming to thiscountry. She also stated that the curve hadprogressed over time and that it wasphysically and cosmetically painful. Hergeneral appearance revealed a pronounceddextroscoliosis that was concentrated inthe proximal thoracic. Her right shoulderwas mildly elevated compared to the left.The x-rays she brought with herconfirmed the dextroscoliosis, but in orderto get a more complete description of herdeformity, long-cassette films wereordered which included standing lateraland side-bending anteroposterior views. A CAT scan and an MRI of the cervical,thoracic and lumbar spine regions werealso ordered.

Upon receipt of these new images, thepatient was found to have thoracicscoliosis with a Cobb angle of 90 degrees.We recommended surgical correctionconsisting of T1 to T12 instrumentation,and T6 vertebral column resection; thetwo-phase operation was scheduled fortwo days in early September. Surgicalintervention was carried out while sensory and motor responses weremonitored; they remained at the patient’s preoperative baseline.

The patient returned for her post-opexamination in late September, reportingsome residual pain but otherwise verypleased. The visible hump in her thoracicspine was entirely gone. Her shoulders arenow symmetrical and leveled and her headis in midline position, indicating that she iscoronally balanced. Her Cobb angle hasbeen reduced by half and is now at 40degrees, the maximum possible withoutadditional surgery. A temporarystabilization brace and external bonestimulator device were provided to hastenhealing, but all the goals of the initialdeformity surgery have been met. She isnow standing and walking comfortably.She is understandably happy with the improvements.

Case StudyTwo-Phase ScoliosisIntervention

(Left) Preoperative and (right) postoperative x-rays of adolescent idiopathic scoliosis.

Anteroposterior (AP)and lateralpreoperative x-rayshowingthoracolumbarscoliosis in anadolescent patient and AP and lateralpostoperative x-rays.

Preoperative Postoperative

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18 CLINICAL NEUROSCIENCE NEWS

Peter Hollis, MD, FACS

The patient is a generally healthy, alert79-year-old female with a four-yearhistory of debilitating lower back painfor which physical therapy hasprovided no relief. In 2010 sheunderwent a failed micro lumbardiscectomy at another hospital, afterwhich she developed progressivelyworsening radiating pain in both legs.An epidural steroid injection sixmonths after surgery also brought noimprovement. In October 2011 shecame to North Shore-LIJ’s CushingNeuroscience Institute for a surgicalconsult, bringing a recent MRI of herlumbosacral spine. We identified severespinal stenosis and spondylolisthesiswith degenerative scoliosis localized atL3-4 and L4-5. After discussions withthe patient and family members werecommended a multilevel bilateraldecompressive laminectomy, medialfacetectomy and foraminotomy inconjunction with insertion of fixationhardware and intertransverseautologous bone graft to supplementspinal fusion.

The surgery, performed in November2011, began with the patient placed onher side and under general anesthesia.After being wired for lower extremity

EMG and somatosensory-evokedpotential monitoring, the first phase ofsurgery began with discectomies in theL3-4 and L4-5 disc spaces. Once thedamaged disks were removed theywere replaced with PEEK interbodyprostheses. Each prosthesis wasprecisely sized according to theinterbody space available and filledwith Osteofil bone matrix to enhancegrafting. The prostheses were thentamped into place under interoperativeAP and lateral C-arm (fluoroscopy)guidance, with excellent reductions inthe patient’s degenerative scoliosis andsubluxation obtained. Throughoutcontinuous intraoperative EMGstimulation using the Neurovisionretractor/dilator system provided safepercutaneous passage through thesurrounding tissues of the spine and tothe spinal nerve roots.

During the next phase-the patient was placed on her stomach and a small posterior midline incision made over L3 to L5 with the goal ofdecompressing associated nerves andpreparing the way for a fixation deviceto stabilize the spine at this location.The spinous processes of L3 and L4were removed after which a bilateraldecompressive laminectomy was

carried out. A high-speed drill wasthen used to drill down through facet-transverse processes bilaterally at L3,L4 and L5. (On the right side, wherethe patient’s primary pain symptomswere found, the dura was found to beeroded into a cellophane-thin layer inseveral places due to chronic wear.These were sutured closed.)

Lastly, the six holes previously drilledwere probed for stability and holdingcapacity, titanium screws inserted, andcustom-bent titanium fixation rodsinserted to create a permanent internalbrace or cage. Bony material harvestedfrom the laminectomy were preparedin a bone mill and used to complete anautologous bone graft between thetransverse processes.

Case StudyDebilitating Lower Back Pain

(Left) Preoperative CT of severe left laterallisthesis and (right) postoperative xray ofa minimally invasive XLIF with posteriorscrew placement for stabilization in an

80-year-old male.

Ahmad Latefi, DO

A 59-year-old female was referred to usin August 2012 because of worseningintractable lower back pain withradiation to her left lower extremity.She stated that she had undergone backsurgery 30 years earlier for unidentifiedissues and had subsequently receivedall forms of conservative care includingchiropractic, physical therapy andepidural steroid injections in search of relief with minimal to no effect. She also stated that she was diagnosedwith curvature of the spine when shewas younger.

On physical examination in the office,she was found to have a forwardposture with a complete absence ofnormal lumbar lordosis. Her motor,sensory and cerebellar responses werewithin the normal range and herreflexes and gait were found to benormal and symmetric. The patient’sprevious MRI studies were alsoexamined and revealed a sagittaldeformity as well as the previouslynoted flat back. In order to determinethe cause of the back pain and developtreatment, a scoliosis series of x-rayswere subsequently ordered. Theresulting preoperative diagnosis wasfixed sharp kyphotic deformity, sagittal

imbalance anddextroscoliosis. Spinalinstrumentation andfusion were deemedthe appropriate meansto achieve spinalalignment anderadicate pain due tonerve impingementand the correctivesurgeries were scheduled.

The patient, a teacher, was able toreturn to the classroom after recovery.She is able to stand straight and to lookstraight ahead. Her pain is significantlyimproved with the result that she nolonger needs narcotic pain medications.

Case StudyIntractable Lower Back Pain

(Left) Preoperative and (right)postoperative correction of a

kyphotic deformity.

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NORTH SHORE-LIJ HEALTH SYSTEM 19

Peter Hollis, MD, FACS

The patient, an 82-year-old male, came to us for aneurosurgical evaluation after having noted a relativelyrapid deterioration in his ability to walk. Once a vigoroushiker, he had been diagnosed by his primary physicianwith peripheral neuropathy, balance issues, and neckstiffness. He also has some claudication type symptomswith significant back pain radiating into the lowerextremities when he walked or stood for even briefperiods. Earlier EMG and nerve conduction studiesshowed findings compatible with peripheral neuropathyand possible multilevel lumbar radiculopathy. Imagingstudies of the lumbar spine showed spondylolisthesis withprofound stenosis at L3-4 moderating somewhat aboveand below. Degenerative spondylosis was identified as theprecipitating factor.

Upon examination at Cushing Neuroscience Institute wenoted a markedly reduced range of motion in the neckspecific to extension and right rotation. Patient’s reflexeswere depressed throughout and his plantar reflexes weremute as well. We recommended an MRI of the cervicalspine to investigate possible cervical spondyliticmyelopathy as an additional pathology. When the MRIreport came back, it revealed severe cervical stenosis atC4-5 and C5-6 with disc osteophytes causing spinal cordcompression. It was then determined through discussionswith surgeons and patient that surgery to correct thiscondition should take precedence over the lumbar issues.A week later the patient underwent surgery for spinalcord compression in the cervical spine.

The patient was given general anesthesia and his motorand sensory EPs set up for monitoring. He was positionedwith head, neck, and body all in a neutral position on adoughnut pillow so that the anterior aspect of his neckwas accessible. Using C-arm intraoperative imagingguidance, the surgeon made an incision just above the C5vertebral body to permit exposure of the cervical spinefrom the midline to the medial border of thesternocleidomastoid muscle on the left side. Theassociated muscles were retracted and the C4-5 disc wascompletely removed giving access to a broad-basedosteophyte projecting from the inferior aspect of C4. Thisbony projection was also drilled down to a thin residualledge and removed, along with a ligament across the backof the disc space. With total neural decompressionachieved at this site, an appropriately sized ROI-C®interbody prosthesis filled with demineralized bonematrix was tamped into place and locking plates appliedto complete segmental fixation.

A similar discectomy, osteophyte/ligament removal anddecompression were then carried out at C5-6, with thesame corrective results. Throughout the procedure thepatient remained stable with no change in EP potentialsand minimal firing from the EMG.

At the patient’s request, the lumbar correction waspostponed for a year, at which time the patient returnedto have his L2-5 spinal stenosis with degenerativescoliosis and nerve root impingement surgically relieved.Given general anesthesia and with routine EP monitoringdevices in place, the patient was placed on his right sideand further positioned so that when viewedintraoperatively on the C-arm the critical areas wereprecisely visible and in symmetrical position. Small skinincisions were made above the left iliac crest to access L3-5 and L2-3, after which dilators were placed undercontinuous EMG stimulation to open up and navigatesafe passage through the layers of muscle to the discspaces. Discectomies and insertion of appropriate PEEKinterbody prostheses filled with demineralized cell matrixwere sequentially performed at L4-5 and at L3-4. Withthese interventions the patient’s lateral scoliosis andspondylolisthesis were observed to be markedlyimproved. The remodeling also permitted safe access tothe L2-3 level, allowing the surgical team to complete athird diskectomy and interbody arthrodesis at L2-3.

The patient tolerated the procedure well and eight weekslater the patient was pain-free and back at work.

Case StudyReduced Range of Motion

Postoperative x-ray after a C4-5 and C5-6anterior cervical discectomy and fusion

without anterior plating.

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Brain tumors rank relatively low in overall cancer incidence in the US, but theyare among the most difficult malignancies to treat. Because tumor activity isassociated with a heterogeneous assortment of neurological, motor andbehavioral changes, getting the right diagnosis as early as possible can becritical to the outcome. Referral to a high-volume specialized center staffed byexperts who can bring to each case years of experience and cutting-edgeinterventions can save valuable time and, in many instances, brain tissue.

Since its establishment in 2007, our Brain Tumor Center has been staffed withspecialists in brain tumor science, many of them recognized internationally aspathfinders in this burgeoning field.

Methodology

Surgical removal via craniotomy has long been the principal treatment formasses located in parts of the brain that can be resected without damagingcritical neurological functions. Targeted radiation and chemotherapy are oftenused as secondary treatments to further suppress the ability of any tumor cellsthat may remain after resection to survive, but radiation and drugs also poserisks to surrounding healthy brain tissue.

Research into even more effective treatments for brain tumors is beginning topay off in a new generation of more effective clinical treatments. Abnormalmasses that were once regarded as surgically inoperable are now yielding tosophisticated new strategies that sometimes achieve 100 percent recision, withminimal or no neurological impairment.

Multidisciplinary Approach

The center is organized around a multidisciplinary program with a variety ofspecialists all playing roles in care. As an individualized treatment strategy isdeveloped, the patient’s progress is reviewed in detail at a weekly 20-memberCNS Tumor Board.

We also work closely with scientists at The Feinstein Institute for MedicalResearch, where work is currently focused on expanding knowledge of such keyissues as the signaling mechanisms that stimulate brain tumor invasion and thespecific proteins that protect certain malignant cells from the therapeutic (i.e.killing) effects of radiation. From this collaboration we gain first-hand exposureto the best new thinking on more effective strategies to pursue.

Naming and Grading Tumors

A key part of brain tumor diagnosis is developing a full picture of the tumor, aprocess of naming and grading. Benign tumors grow relatively slowly, havedistinct margins and can often be cured entirely through surgical recision.Malignant tumors are typically aggressive in growth and alwayspotentially life-threatening.

Brain tumors are also classified according to whether theybegin in the brain (primary tumor) or arise as secondarytumors — colonies from primary tumors that havemetastasized elsewhere in the body. Tumors are furtheridentified on the basis of their type. Gliomas developfrom the supportive, or glial tissue of the brain. Theyinclude astrocytomas, ependymomas, and mixedgliomas and represent 30 percent of all braintumors. Non-glial tumors includemedulloblastomas, meningiomas andschwannomas. Lastly, the grade of a brain tumoris also a significant determinant of treatment.Grade I is the least advanced and mostcontained; Grade IV is very advanced.

Three advanced modalities for which the Brain Tumor Center is particularlyknown are intraoperative MRI (iMRI) and awake brain mapping; laser interstitialthermal therapy (LITT); and stereotactic radiosurgery (SRS).

Intraoperative MRI (iMRI) provides real-time image guidance to the surgeon incarrying out precise resections of brain tumors in critical locations. Before theclinical introduction of intraoperative imaging it was often difficult to distinguishthe margins of a tumor from the healthy normal brain around it, despite havingexcellent preoperative maps. Dr. Schulder was the first neurosurgeon in NorthAmerica to employ the PoleStar® intraoperative MRI for brain surgery andremains the leading user of this technology in the United States.

Laser Interstitial Thermal Therapy (LITT): Our center was one of the primaryinvestigators of this new interventional treatment for tumors that havemetastasized and cannot be removed by surgical means. (See Case Study #1). Athin fiber is inserted into the tumor via a minimally invasive surgical approach.The laser beam destroys the tumor by heating it from the inside out, under real-time MRI guidance.

Stereotactic Radiosurgery (SRS) is a form of minimally invasive neurosurgerythat targets brain tumors with extremely precise high-energy radiation beams,biologically inactivating cancer cells and reducing the tumor, often in a singledose. We have been working with SRS at the Cushing Institute since 2005. Thistechnology combines CT and MRI imaging, multi-directional photon beamdelivery, and analytical software to analyze the 3-D shape and volume of thetumor and create a customized radiation program.

Gamma Knife® therapy uses doses of radiation to kill cancer cells but does soby focusing many beams each of relatively low intensity to target a brain tumor,typically up to 4 cms in size. We are pleased to announce the imminent openingof the North Shore-LIJ Gamma Knife® Center, which will add significantly to ourradiosurgery capabilities. The Gamma Knife® was the first practical device forSRS and remains a leading technological standard for this treatment method.

Sharing Our Experience: Upcoming Programs inSpring 2014

Each spring Dr. Michael Schulder and Dr. Jonathan Knisely, co-directors of theNorth Shore-LIJ Center for Stereotactic Radiosurgery, run a one-day continuingmedical education (CME) program, a combination of talks, case presentationsand panel discussions aimed at physicians, physician assistants, nursepractitioners and allied health care professionals interested in learning moreabout stereotactic radiosurgery and stereotactic body radiation and theirapplications. Our fourth such CME meeting is scheduled for Monday, March 3,2014 and will be held at The Feinstein Institute for Medical Research. For more

information or to enroll, call 516-562-3065.

We are also honored that Drs. Schulder and Knisely have beenselected as co-chairs for the 17th International LeksellGamma Knife® Society Meeting, which will be held in NewYork City May 11-15, 2014. An important objective of theSociety is the development of best practices inGamma Knife® surgery, fostered by bringingtogether internationally recognized speakers inthe fields of neurosurgery, radiation oncologyand medical physics, in addition to keypresenters in other areas of medicine andsociety. The Society is named for thevisionary Swedish neurosurgeon Lars Leksell(1907-1986), who developed the GammaKnife® and was also a principal in thedevelopment of SRS.

TAKING ONBrainTumors

20 CLINICAL NEUROSCIENCE NEWS

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NORTH SHORE-LIJ HEALTH SYSTEM 21

Michael Schulder, MD, FAANS

40-year-old woman had an eight-year history ofrecurrent carcinomas of the left breast, which had inrecent months metastasized to a half-dozen or morelesions in the brain. The patient was treated with wholebrain radiation therapy.

Two years later, the patient returned for a surveillanceMRI, at which time we found a new left posteriorfrontal tumor measuring 6 mm. These tumors weretreated with stereotactic radiosurgery (SRS). Revisitedafter seven months, another MRI showed that all thepreviously treated tumors were under control but aright high frontal tumor measuring 3.5 mm had arisen.This tumor was also treated with SRS.

A year after this last treatment it was determined thatthis same lesion continued to resist radiation treatment— its diameter had grown to 18mm — and that thebest remaining option was to use laser interstitialthermal therapy (LITT). The procedure was done underlocal anesthesia with intravenous sedation. A flexibleoptical fiber probe was inserted so as to penetrate thecenter of the tumor, which was ablated successfullyusing real-time MRI guidance. The patient wasdischarged home the next day.

Case StudyLaser Interstitial Thermal Therapy(LITT) for Metastatic Brain Tumor

Preoperative MRI showing metastatic brain tumor.

Live image showing temperature during LITT.

Tumor ablation with expected ring enhancement post LITT.

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22 CUSHING NEUROSCIENCE INSTITUTE

Michael Schulder, MD, FAANS

A 76-year-old woman presented with a four-week history of subacute ataxia or gait. AnMRI revealed a pineal mass causing obstructivehydrocephalus. Using intraoperative MRI, aminimally invasive stereotactic biopsy wasdone, revealing the tumor to be a benignpineocytoma. At the same time, the patient’shydrocephalus was treated by placing aventricuoperitoneal shunt that drained theblocked fluid from her brain. She returned toher previous intact neurological state, and thepineocytoma was successfully treated with SRS.

However, a year later the patient returned withsymptoms of significant visual field loss; MRIshowed a new suprasellar cyst. A catheter wasplaced under intraoperative MRI guidance, andthe cyst was drained successfully. The patient’svision returned to normal.

Case StudyIntraoperative MRI andStereotactic Radiosurgeryfor Pineocytoma

MRI showing pineal mass and obstructive hydrocephalus.

Stereotactic radiosurgery treatment plan for pineocytoma.

Cyst before and after catheter drainage with intraoperative MRI.

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NORTH SHORE-LIJ HEALTH SYSTEM 23

Michael Schulder, MD, FAANS andJonathan P.S. Knisely, MD

A 64-year-old year old woman complainedof left-sided hearing loss. She hadlongstanding hearing loss in the right ear aswell. MRI revealed a 1.1 cm vestibularschwannoma on the left. Treatment options— from observation alone to surgicalremoval or stereotactic radiosurgery (SRS)— were described and we recommendedSRS as offering the highest likelihood oftumor control and the best chance ofhearing preservation.

A radiosurgical plan was created to treat thepatient over five days. The patient received atotal dosage of 25 Gy/5 (25 Gy ionizingradiation delivered over five days orfractions). The patient has since returned forannual follow-up MRIs and examinations.Two years later, the vestibular schwannomaremains controlled and she has experiencedno further hearing loss in her left ear.

Case StudyStereotactic Radiosurgeryfor VestibularSchwannoma

MRI prior to SRS showing left vestibular schwannoma.

MRI three years after SRS.

Radiosurgery plan for treatment in 5 fractions. The red line surrounding the tumoris the conformal prescription dose. The lower isodose lines show a steep dosegradient so that very little brain tissue will be irradiated.

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NEUROSURGEONS

Paolo Bolognese, MDPhone: (516) 570-4400E-mail: [email protected]

David J. Chalif, MD, FACSPhone: (516) 562-3070E-mail: [email protected]

Amir R. Dehdashti, MD, FACSPhone: (516) 562-3026E-mail: [email protected]

Mark B. Eisenberg, MDPhone: (516) 773-7737E-mail: [email protected]

Peter Hollis, MDPhone: (516) 773-7737E-mail: [email protected]

Salvatore Insinga, DOPhone: (631) 591-7470E-mail: [email protected]

Robert G. Kerr, MD, PhD,FRCPSCPhone: (631) 351-4840E-mail: [email protected]

David J. Langer, MDPhone: (212) 434-3900E-mail: [email protected]

Ahmad Latefi, DOPhone: (516) 773-7737Email: [email protected]

Mitchell E. Levine, MDPhone: (516) 773-7737E-mail: [email protected]

Ashesh Mehta, MD, PhDPhone: (516) 325-7061E-mail: [email protected]

Mark A. Mittler, MD, FACS, FAAPPhone: (516) 354-3401E-Mail: [email protected]

Raj K. Narayan, MD, FACSPhone: (516) 562-3816E-mail: [email protected]

M. Chris Overby, MDPhone: (516) 773-7737E-mail: [email protected]

Harold L. Rekate, MD,FACS, FAAPPhone: (516) 570-4400E-mail: [email protected]

Steven J. Schneider, MD, FACS, FAAPPhone: (516) 354-3401E-mail: [email protected]

Michael Schulder, MD, FAANSPhone: (516) 562-3065E-mail: [email protected]

Justin M. Thomas, MDPhone: (631) 591-7470E-mail: [email protected]

NEUROLOGISTS

Rohan Arora, MDPhone: (516) 562-3066E-mail: [email protected]

Karen Blitz, DOPhone: (516) 674-7500E-mail: [email protected]

Li-Fen Chen, MDPhone: (516) 674-1647E-mail: [email protected]

Alexis Demopoulos, MDPhone: (516) 562-3065Email: [email protected]

Robert A. Duarte, MDPhone: (516) 325-7070E-mail: [email protected]

David Eidelberg, MDPhone: (516) 325-7050E-mail: [email protected]

Zianka Fallil, MDPhone: (516) 352-7060E-mail: [email protected]

Andrew Feigin, MDPhone: (516) 325-7050E-mail: [email protected]

Marc L. Gordon, MD, FAANPhone: (516) 325-7000E-mail: [email protected]

Cynthia L. Harden, MDPhone: (516) 325-7060E-mail: [email protected]

Sean T. Hwang, MDPhone: (516) 325-7060E-mail: [email protected]

Ronald Kanner, MD, FAAN, FACPPhone: (516) 325-7000E-mail: [email protected]

Jeffrey M. Katz, MDPhone: (516) 562-3064E-mail: [email protected]

Roger W. Kula, MD, FAANPhone: (516) 570-4400E-mail: [email protected]

Danijela Levacic, MDPhone: (516) 562-3065Email: [email protected]

Richard Libman, MD, FRCP(C)Phone: (516) 562-2013E-mail: [email protected]

Aviva Lubin, MDPhone: (212) 434-4070E-mail: [email protected]

Martin Niethammer, MD, PhDPhone: (516) 325-7050E-mail: [email protected]

Rafael Alexander Ortiz, MDPhone: (212) 434-4070Email: [email protected]

Shalini Patcha, MDPhone: (631) 351-5757E-mail: [email protected]

Chilvana Patel, MDPhone: (516) 325-7000E-mail: [email protected]

Noah Rosen, MDPhone: (516) 325-7070E-mail: [email protected]

Daniel B. Rubin, MD, PhDPhone: (516) 325-7000E-mail: [email protected]

Lyubov Rubin, MDPhone: (516) 674-7500E-mail: [email protected]

Guy Schwartz, MDPhone: (516) 325-7050E-mail: [email protected]

Musarat Shareeff, MDPhone: (631) 271-1206E-mail: [email protected]

Beth Silverstein, DOPhone: (516) 674-1647E-mail: [email protected]

Scott J. Stevens, MDPhone: (516) 325-7060E-mail: [email protected]

Santo Primo Terranova, DOPhone: (516) 325-7000E-mail: [email protected]

Murthy Vishnubhakat, MDPhone: (516) 325-7000E-mail: [email protected]

INTERVENTIONALNEURORADIOLOGIST

Avi Setton, MDPhone: (516) 562-3021E-mail: [email protected]

NEUROINTENSIVISTS

Gregory Kapinos, MD, MSPhone: (516) 562-3590Email: [email protected]

David LeDoux, MDPhone: (516) 562-3590E-mail: [email protected]

Celine Rahman, MDPhone: (516) 562-3590E-mail: [email protected]

PHYSIATRIST

Shaheda Quraishi, MDPhone: (516) 325-7000E-mail: [email protected]

NEUROPSYCHOLOGISTS

Paul J. Mattis, PhD, ABPP-CNPhone: (516) 325-7000E-mail: [email protected]

Sarah Schaffer, PhD, ABPP-CNPhone: (516) 325-7000E-mail: [email protected]

PSYCHOLOGIST

Dennis Thornton, PhDPhone: (516) 325-7070E-mail: [email protected]

Cushing Neuroscience InstituteNorth Shore University Hospital300 Community Drive, 9 TowerManhasset, NY 11030

[email protected]

NEUROSCIENCE STAFF

To learn more about the CushingNeuroscience Institute visitNeuroCNI.com or scan the QR code.