Grand Rounds

1. Grand Rounds Avi Schiowitz, D.O. 4/24/07

2. Case PresentationChief Complaint Pt is a 37 y/o female who presented to the ED on 2/17/07 with a complaint of a constant pressure like headache which had lasted for the last 2 weeks. The headaches awoke her from sleep.

3. History of Present Illness • Onset was 2/4/2007. • At first got better with OTC medication, now is constant. • Pt describes headaches as a tight feeling bifrontal, extending posterior, left side hurts more than right. • Exacerbated by light and in the sitting, standing position. • Relieved somewhat by lying flat.

4. HPI cont. • Complains of new onset nausea and vomiting. • Patient felt lightheaded for last few days, “nearly walking into walls”. • Unable to perform daily activities or go to work. • Pt was seen in OSH – SWGH twice during last 2 weeks for same condition.

5. Allergies: Latex • PMHx: denies • PSHx: C/S twice under spinal. • Social Hx: Non-smoker, social drinker, denies drug use • Takes no medications.

6. In the ED • Physical exam: • BP 144/81, HR 77, T 96.8, RR 14, 99% RA Gen: Pt lying on cot, cloth covering her face. Slow to move but is A & O x 3. Winced to light being turned on, but no photophobia to fundoscopic exam. Neuro: CN’s normal and intact. 5/5 strength and motor in UE/LE. Movement is slightly slow but not clumsy. • Rest of physical exam normal.

7. Hospital Course • IV started. 1 liter NS bolus given, second liter running. • CBC, BMP and urine Hcg ordered. • CT Head without contrast and CT angio of head ordered. • Pt given pain meds with slight improvement.

8. Pt admitted to 9C • Diagnosis of Headache, Pain control and dehydration. • Neurology consult requested.

9. Migraine Tension HA Cluster HA Stroke Subarachnoid Hemorrhage Intracerebral Hemorrhage Cerebral Infarction Arterial Dissection – Carotid or Vertebral Acute Hydrocephalus Meningitis Encephalitis Giant Cell Arteritis Tumor Trauma Differential diagnosis of Acute Headache.

10. Neurology consult • Pt seen by neurologist next day. • History consistent with that given in ED. • No visual disturbances • No tinnitus or diplopia. • No history of trauma.

11. Neurology Exam • ROS negative • Physical Exam WNL • Neurologic Exam: A & O x 3 , CN intact Motor, sensation, coordination – WNL Reflexes 2+ & symmetric Gait normal, no romberg.

12. Imaging • CT of head – slit like ventricles and low lying cerebellar tonsils. Findings could be either due to pseudotumor cerebri with coexistent Chiari I malformation or CSF hypotension.

13. Imaging • CT Angio of brain – Negative except for somewhat prominent venous structures supplying the transverse sinuses bilaterally. Findings could be manifestation of CSF hypotension syndrome. Although given the plain CT results pseudotumor cerbri also warrants consideration. Most significantly, no evidence of dural sinus thrombosis.

14. Pseudotumor cerebri • AKA Benign Intracranial hypertension • Defined as a syndrome of elevated ICP without evidence of hydrocephalus, focal lesions or frank brain edema. • Usually occurs in obese women between ages 15-45. • Symptoms include HA, visual disturbances, or diplopia secondary to abducens nerve palsies. • Fundoscopic exam shows papilledema.

15. Pseudotumor cerebri • Usually a benign and self limiting disorder. • Condition associated with drugs such as Vit A intoxication, Danazol, Accutane, Tetracyclines, oral contraceptives and corticosteroid withdrawal. • Systemic disorders include hypoparathyroidism, lupus.

16. CT of head can show small ventricles • Treatment includes carbonic anhydrase inhibitors – reduce CSF production. Corticosteroids, Lasix. • Weight loss. • Correct underlying systemic disorder. • In the most severe cases Ventriculoperitoneal shunt can be done.

17. Radiology Impression: Orthostatic HA suspect spontaneous low CSF pressure HA. Neurology Recommendation: Trial of caffeine 500mg IV. If no relief in 1 hour give 2nd dose. If no relief in AM will do MRI of head.

18. MRI of head done – Results show inferior displacement of the brainstem and cerebellum towards the foramen magnum with diffuse dural venous enhancement. Findings most consistant with intracranial hypotension. • Neurology recommends epidural blood patch and Anesthesia was consulted.

19. Acute pain service saw the patient and recommended conservative treatment at this time. • Abdominal binder, caffeine, fluids and percocet for pain. • Patient was referred to Chronic pain for followup.

20. Patient was discharged on 2/20/07. • Instructed to follow with chronic pain and neurology as outpatient. • Told to come to ED if symptoms do not improve.

21. What is Intracranial Hypotension? • Escape of fluid that normally surrounds the brain and spinal cord. • Usually characterized by an orthostatic headache – one that worsens with sitting or standing. • Headache can be chronic or patient can present without headache. • Location of headache varies between patients.

22. Orthostatic Cervical or interscapular pain that preceds orthostatic HA Lingering non orthostatic HA Exertional HA wihout orthostatic features Acute thunderclap onset of orthostatic HA Second half of day HA Paradoxic orthostatic HA No HA Headaches in intracranial hypotension

23. Cause of Headache • CSF supports the brain. • Brain weight of 1500gr in air weighs only 48gr in the cranium. • Remaining weight supported by pain sensitive structures. Meninges, cerebral and cerebellar veins, CN V, VII, IX and X, upper 3 cervical nerves. • CSF decreases – decrease in buoyancy causing traction on these structures. • In the upright position the downward displacement is exagerated.

24. Evidence of this theory is supported first by study data documenting downward displacement of cranial structures. • Evidence in support of this theory comes from data collected by Kunkle, et al who induced postural HA in healthy volunteers by draining CSF. One of his subject had undergone a section of the roots of CN V and IX and upper 4 cervical roots on L side with analgesia in the regions to which these nerves project. This pt experienced HA only on right side.

25. Another theory proposes the dilitation of the intracranial vascular structures causes the headaches. This is based on the Monroe-Kellie hypothesis which states that the sum of the volumes of intracranial blood, CSF and brain tissue must remain constant in an intact cranium. According to this hypothesis a loss of CSF will cause an increase in intracranial blood volume.

26. Most compensation occurs via venous dilitation due to greater compliance and capacitance. • Venous sinus engorgement, pachymeningeal enhancement, subdural effusions and enlargement of pituitary gland may represent compensatory changes to maintain intracranial volume.

27. Pain is exacerbated by: Laughing Coughing Sneezing Jugular Venous Compression Valsalva maneuver Analgesics have minimal effect and pain is usually relieved with lying flat.

28. Nausea Vomiting Anorexia Neck pain Dizzyness Diplopia Photophobia Changes in hearing Unsteadiness or staggering gait Facial numbness or weakness Transient visual obscuration Upper limb radicular symptoms Other Symptoms:

29. Rare symptoms • Galactorrhea • Stupor • Ataxia • Parkinsonism • Coma • Result of compression of pituitary stalk, diencephalon, posterior fossa, and midline structures.

30. Etiology of CSF volume depletion • True Hypovolemic state – reduced total body water • CSF shunt overdrainage • CSF leaks: Traumatic: After definite trauma Spinal tap or epidural Spinal or cranial surgery Spontaneous: Unknown cause – most common weakness of the dural sac – ex. Meningeal diverticula or connective tissue abnormalities

31. This patient was diagnosed with spontaneous CSF hypotension. • Syndrome recognized for more than 55 years • First proposed by Schaltenbrand in 1938 and described as a headache syndrome virtually identical to one following an LP.

32. Proposed the following mechanisms: 1. Decreased CSF production 2. Increased CSF absorption 3. CSF Leakage through small tears Today the accepted etiology is of CSF leakage. Most occur at the cervicothoracic junction and thoracic levels.

33. Can be attributed to minor trauma such as fall, sneezing, sudden twist or stretch. • Can cause rupture of preexisting spinal epidural cysts or tarlov cysts or tear in dural nerve sheath.

34. Diagnosis • CSF analysis – opening pressure can be low – around 60mmhg. • Dry taps are encountered • Rare instances the was negative pressure – sucking noise as stylet is removed. • CSF is typically clear. Protein concentration normal or high. WBC count can be normal or high. • Cytology and microbiology are always normal • Glucose concentration is never low.

35. CT of head is of limited value. It might show subdural fluid collections. Used more to rule out other causes. • MRI will show diffuse pachymeningeal enhancement following the administration of gondolinium. This is the most characteristic feature of this syndrome.

36. MRI findings in SIH • Diffuse pachymeningeal enhancement. • Descent of brain – Cerebellar tonsils, obliteration of some subarachnoid cisterns, crowding of post. Fossa • Enlargement of pituitary • Flattening or tenting of optic chiasm • Subdural fluid collections • Engorged cerebral venous sinuses • Decreased size of ventricles

37. In the spine – extra arachnoid fluid collections • Meningeal diverticula • ID of level of leak • ID of actual leak site – rare • Engorgement of epidural veins • Spinal pachymeningeal enhancement

38. CT Myelogram – used to demonstrate CSF leaks and is the study of choice to find its location. • Radioisotope cisternography – Uses indium-111. introduced intrathecally and its movement is followed by sequential scanning at certain time intervals. • Normally by 24 hours radioactivity can be detected over the cerebral convexities. • If there is a CSF leak there will be minimal activity. • Early accumulation will be detected in the kidneys and bladder

39. Treatment of CSF leaks • HA usually resolve with conservative treatment within 2-12 days. • Bed rest – supine position reduces CSF pressure at leak site and promote meningeal healing. • Fluids– increases CSF volume by fluid restoration. • Caffeine– Thought to produce arterial vasoconstriction through blockade of adenosine receptors. Intracranial blood flow venous engorgement are decreased. • Steroids – effort to restore CSF volume. No evidence indicates that steroids have any effect on CSF Production or absorption. • Abdominal binder

40. Treatment • Other treatment options include: • Epidural Blood Patch • Continuous epidural saline infusion • Epidural infusion of dextran • Epidural injection of fibrin glue • Intrathecal fluid injection • Surgical correction

41. Epidural blood patch • According to Mokri, epidural blood patch is the treatment of choice in individuals who have failed conservative therapy. • Technique introduced by Gormley who observed that the incidence of ICH following a lumbar puncture is less in individuals when the LP is traumatic and bloody.

42. Study by Mokri, Sencakova, and McClelland identified 54 patients with SIH. • 29 patients received EBP • 4 Patients were eliminated. • Followup was obtained via review of records, correspondence and phone calls. • Ages ranged for 18 to 62. 20 women 5 men.

43. All patients received EBP using 10 -20ml of blood • First EBP 9 out of 25 improved. • 15 who failed first EBP underwent 2nd. • 5 reponded well. • Of the 10 remaining, 2 were treated surgically. Remainder underwent a 3rd EBP 4 responded well. 4 other underwent surgical correction.

44. This technique involves injecting blood into the epidural space. • Pt experiences immediate relief due to volume replacement. • In spontaneous CSF leaks patients usually require more than one blood patch. • Procedure is most effective is done at level of leak. • If level unknown – blood can be injected into lumbar space and pt head can be lowered to allow the blood to ascend. Data indicates that this might be effective over 9 spinal segments

45. Complications • Most common complication is back discomfort at injection site – 30% • Paresthesia • Radiculopathy • Chemical meningitis

46. Pt arrives back in ED on 2/28/07 with worsening of HA. • Pt is actively vomiting. HA is continuous and getting worse. • Pt was compliant with abdominal binder, caffeine, fluids. Pain meds give mild relief. • Pt admitted to 9C. Neurology and Anesthesia consulted.

47. Anesthesia Acute Pain Service went to evaluate pt. on 9C. • Risks and Benefits of epidural blood patch discussed at length. All question were asked and answered. • Pt wanted to try a trial of steroids prior to epidural blood patch.

48. On 3/2/07 pt was brought to OR for epidural blood patch under fluoroscopy. • Epidural space was identified and 3cc of blood was injected. • Pt states that she has immediate resolution of symptoms. • Post op – pt sitting in bed. Denies HA, denies N/V. • Pt discharged home. Follow with neurology and chronic pain as needed.

49. 3/9/07 pt calls MHMC complaining of HA. • Pain no longer occipital. Only bifrontal L>R. • Has nausea and vomiting. • Sees neurology and a 2nd epidural blood patch is recommended. Pt started on steroids – 8mg Decadron for 5 days.

50. Repeat CT of head shows cerebellar tonsils inferiorly displaced within the foramen magnum. 4th ventricle appears small. Findings consistent with intracranial hypotension.