Managing life with neurological symptoms
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- Eustace Porter
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1 Managing life with neurological symptoms Petra M Klinge Professor of Neurosurgery Director, Division of Pediatric Neurosurgery Director, CSF center of the brain and Spine Rhode Island Hospital and Hasbro Childrens Hospital Warren Alpert Medical School of Brown University
2 Overview _ the neurosurgeon s perspective q Understand diagnosis of neurological manifestations prevalent in EDS q Symptoms and SIGNS and Radiological Diagnosis q Pathology in EDS (MRI limited biomarker), SUGGESTIONS and RED FLAGS! q Discuss the OPTIONS and management! q Surgical options and techniques ( What to ask the Surgeon, Short- and Long-term Outcome and Recovery) q Non-surgical options (Monitoring and interdisciplinary Management of Symptoms ) q Personal experience: Manage your physician/surgeon and discuss research_ Case examples how patients changed my practice!
3
4 Neuro MANIFESTATIONS q Idiopathic intracranial hypertension (IIH) q Intracranial hypotension q Chiari and Low lying tonsils q Craniocervical anomalies and instability q Tethered cord and Syringomyelia
5 Diagnosis: IIH HEADACHES VISION LOSS (Papilledema) OBESITY (80%) MR Venogram +contrast > 25 mmhg
6 IIH in EDS ( collapsible venous sinuses ) Ø Nonpositional headaches Ø Episodes of vision loss or blurry vision Invasive ANGIOGRAM with transverse sinus pressure measurements : Sinus pressure supine: 5 to 10 mmhg Gradient between jugular bulb and transverse sinus: 1 to 3 mmhg (normal), IF > 8mmHg ABNORMAL
7 Diagnosis: Intracranial hypotension Headaches: Orthostatic MRMyelogram DSA Myelogram MRI brain with contrast
8 Intracranial Hypotentsion In EDS 20-34% connective tissue disorder: ECM fibrillin I defect Ø 20% of Leaks are not seen on imaging (probably more in EDS) Ø Consider also IIH with CSF pooling and not just leaking! q Mimics POTS Ø Headaches with shaking! Ø Headaches later in the day
9 Arachnoid prolapses in EDS: Example
10 Diagnosis: Chiari and LLT Myth of VALSALVA
11 Chiari and LLT in EDS Ø You have it! Ø Non-classical Signs and Symptoms Loose Myodural bridges in EDS! UPRIGHT MRI CTE was found in 5.7 % and 5.3 % in the recumbent and upright non-trauma grous vs. 9.8% and 23.3% in the recumbent and upright trauma whiplahs injury groups (p<0.01). Freeman et al. Brain Injury, 2010: 24(7-8):
12 Diagnosis: Craniocervical anomalies and instability DYSAUTONOMIA
13 Cranicervical anomalies and instability in EDS EX- FLEX Upright MRI more sensitive! Ø Distortion of the cervicomedullary junction and Dysautonomia in EDS (Henderson et al. 2013)
14 TRIAD: Diagnosis: Tethered cord Bladder/Bowel dysfunction (URO) LE symptoms and signs (NEURO) Spinal and Leg deformities (ORHTO) Ø LOW LYING CONUS ( Endplate L2 or L2/3 = borderline low) THICKENED FILUM > 2mm FAT in FILUM A stretch induced functional disorder leading to ischemia and oxidative stress Neurosurgical Focus Pathophysiology of Tethered Cord Syndrome: Correlation With Symptomatology Shokei Yamada, M.D., Ph.D.; Daniel J. Won, M.D.; Shoko M. Yamada, M.D., D.M.Sci.
15 Tethered cord in EDS Occult tethered cord Ø Ø Ø Ø Bladder symptoms! Progressive leg weakness (i.e. 6 months) SPINE and Joint deformities/ subluxation Provide your PEDIATRIC HISTORY Ø IMPORTANCE of URODYNAMIC TESTING! Why high prevalence of tethered cord in EDS? EM HISTOLOGY shows CORKSCRREW Collagen and disintegrated and disorganized collagen : FILUM_DEFECTIVE SHOCK ABSORBER!
16 Dr. Janice Santos-Cortes Urology, Rhode Island Hospital
17 Overview _ the neurosurgeon s perspective q Understand diagnosis of neurological manifestations prevalent in EDS q Symptoms and SIGNS and Radiological Diagnosis q Pathology in EDS (MRI limited biomarker), SUGGESTIONS and RED FLAGS! q Discuss the OPTIONS and management! q Surgical options and techniques ( What to ask the Surgeon, Short- and Long-term Outcome and Recovery) q Non-surgical options (Monitoring and interdisciplinary Management of Symptoms ) q Personal experience: Manage your physician/surgeon and discuss research_ Case examples how patients changed my practice!
18 Management: IIH Surgical/Interventional Non-surgical
19 Management: Intracranial Hypotension Non-surgical qepidural blood patching (95% initial response, 80% cure rate) qpercutaneous glue placement (40% cure rate)
20 Management: Chiari and LLT Surgical/Interventional Non-surgical Medication (Diamox, Migraine therapy) PHYSICAL THERAPY
21 Management: Craniocervical anomalies and instability Surgical Non-surgical External stabilization Physical therapy
22 Management: Tethered cord Surgical Non-surgical MONITORING! NORMAL T2 MRI conus position L1-2 or L2-3 Laminotomy PYSICAL THERAPY! NUTRITION!
23 REtethering Conus does not need to move Can occur with Arachnoiditis or Pseudomeningocele! Does the level of filum resection matter?
24 Overview _ the neurosurgeon s perspective q Understand diagnosis of neurological manifestations prevalent in EDS q Symptoms and SIGNS and Radiological Diagnosis q Pathology in EDS (MRI limited biomarker), SUGGESTIONS and RED FLAGS! q Discuss the OPTIONS and management! q Surgical options and techniques ( What to ask the Surgeon, Short- and Long-term Outcome and Recovery) q Non-surgical options (Monitoring and interdisciplinary Management of Symptoms ) q Personal experience: Manage your physician/surgeon and discuss research_ Case examples how patients changed my practice!
25 Can you please look at the mastcells in my filum! FILUM is NOT the BIOPSY FOR MACS! TOLUIDIN CD 117
26 I am telling you my dura is _ so please watch out for this! Valsalva maneuver might not apply to EDS! Now, I do fat grafts, if need from a donor site!
27 Urology, GI, Gynecology NeuroPathology Orthotics Nutrition Anesthesia Neurosurgery Internal Medicine Neuroimagi ng Orthopedic surgery, Plastic surgery (Cardiology, Allergology, Endocrinology) Genetics Neurology/Pediatrici ans Psychology Rheumatolo gy Social work Pain management Nursing care Physical therapy
28 YOU ARE the _Physician s Partners Promoters Protectors
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