Case Presentation: NeurologyNeurosurgery Grand Rounds February 28, 2006

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Case PresentationNeurology/Neurosurgery Grand
RoundsFebruary 28, 2006

• Gabriel Zada, MD
• Christopher Aho, MD
• Neurosurgery Blue
• LAC-USC Medical Center

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Patient G.P.

• History of Present Illness
• 44-year-old Latino man
• Complains of progressive headache x 2-3 months
• Headache worse throughout course of day
• Developed nausea/vomiting 1-2 weeks prior to
  admission
• Intermittent double vision, dizziness
• Hit head while working 6 months ago, but symptoms
  developed much later
• No sensory or motor complaints
• Denies fevers, chills
• Denies seizures

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History (continued)

• Past Medical History None
• Past Surgical History None
• Medications Tylenol, Ibuprofen for Has
• Allergies None known
• Social History
• Works for pool chemical company
• Smokes 5 cigarettes/day
• Denies alcohol or other drugs

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Physical Examination

• Mental Status
• Awake, alert, oriented to person, place, time,
  and situation. Speech fluent.
• Cranial Nerves
• Right partial 3rd nerve palsy (x 1 day)
• Pupil 7?5mm, sluggish.
• Partial ptosis.
• No oculomotor deficit.
• Left pupil 5?3mm, brisk.
• Face symmetric
• Cranial nerves otherwise intact.

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Physical Examination

• Motor
• Tone Normal
• No pronator drift
• Power 5/5 in all extremities
• Reflexes
• 2, symmetric throughout
• No Hoffmans sign
• Toes downgoing bilaterally
• Sensory
• Sensation intact in all extremities.
• Cerebellar/Gait
• Finger-nose-finger normal. Gait exam deferred.

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Head CT
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Initial Hospital Course

• Developing concern that patient had increased
  intracranial pressures and brainstem herniation
• Mannitol trial ? Right 3rd nerve palsy improved
• Emergent neurosurgery consult requested
• Initial concern per neurosurgery for subarachnoid
  hemorrhage and ruptured P-Comm aneurysm
• Nimodipine increased intravenous fluids started
  empirically
• Emergent cerebral angiogram ? no aneurysm, AVM
• Hospital day 3 Right 3rd palsy recurred, now
  with altered mental status and lethargy

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CT Scan Final Report

• High density material within confines of Circle
  of Willis, concerning for possible SAH.
• Left frontal subdural collection (subacute or
  chronic SDH)
• Rule out empyema, meningitis, SAH.

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Brain MRI
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MRI Final Report

• Bilateral SDH
• Evidence of SAH
• Diffuse meningeal enhancement
• Decreased caliber of right ICA and MCA, may be
  suggestive of vasospasm.

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Hospital Course (continued)

• Lumbar Puncture felt to be contraindicated
• Right ventriculostomy placed on HD5
• ICPs range -6 to 4
• CSF studies
• RBCs 485, WBCs 0, Glucose 59, Protein 8
• PMNs 84, Lymphocytes 10
• No improvement in neuro status.
• Patient became progressively more obtunded and
  developed additional left 3rd nerve palsy,.

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MRI Final Report

• Interval placement of R frontal ventriculostomy
• Left greater than right SDH

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Hospital Course (continued)

• Discussion over intracranial hypertension versus
  hypotension began.
• Patient started on trial of IV caffeine, supine
  position.
• ICP Monitor (Bolt) placed to recheck ICPs
• ICP range -7 to 5
• That night, patient developed rapid progression
  of bradycardia to the 40s apneic episodes
• Emergent CT myelogram ordered

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Diagnosis

• Spontaneous Intracranial Hypotension (SIH)
  secondary to Cervical and Thoracic CSF leak
• CSF Leak at C1-C3 Left epidural space
• Additional leak from T6-T10 ventrally
• Patient started on IV caffeine drip
• Placed in Trendelenburg position with increase in
  ICPs to 10-18 range and improvement in mental
  status

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Treatment

• Anesthesia contacted for emergent epidural blood
  patch
• Case done in IR suite under fluoroscopic guidance
• C2 region received 8 cc autologous blood patch
• T6-7 region received 21 cc blood patch
• Immediate relief of headaches and increased ICPs
  to 15-19 (flat)

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Post-treatment Course

• Post-patch day 1 Patient awake, alert x 2.
  Complete resolution of 3rd nerve palsies
• Bolt removed
• Sat up post-patch day 2
• Patient home day 7 following procedure,
  completely intact

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Spontaneous Intracranial Hypotension (SIH)

• Patient Demographics
• Often occurs in middle-aged patients
• Mean age 40 years
• Female preponderance
• Higher incidences in patients with Marfans
  disease, other connective tissue diseases, and
  weightlifters

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Spontaneous Intracranial Hypotension (SIH)

• Clinical findings
• Orthostatic headache
• similar to post-lumbar puncture spinal HA
• Exacerbated by laughing, coughing, Valsalva,
  physical exertion
• Often refractory to analgesic agents
• Nausea/vomiting, anorexia, neck pain/rigidity,
  dizziness, diplopia are common
• Cranial nerve palsies (often VI)
• Diverse presentation Hearing changes,
  galactorrhea, facial numbness, radicular
  symptoms, parkinsonism, seizures, coma, death
  have been reported

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SIH Diagnosis

• Often misdiagnosed (94 in one series)
• 14 misdiagnosed as SAH and underwent cerebral
  angiography
• Diagnostic delay 4 days to 13 years (mean 20
  days)
• CT Scan often misleading
• Lumbar Puncture
• Opening pressures usually lt 60 mm H20 in SIH
• (normal 150-400 mm H20)
• Sucking noise reported with LP on occasion,
  indicating subatmospheric pressure
• CSF studies
• increased protein, lymphocytic pleocytosis,xanthoc
  hromia

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SIH Radiographic Findings

• CT Scan
• Effacement of basal cisterns
• Subdural hygromas/hematomas
• Pseudo-SAH (10)
• Hyperdensity in basal cisterns (? obliteration of
  cisterns with arterial venous engorgement)
• MR Imaging
• Diffuse meningeal enhancement (pachymeninges, not
  leptomeninges)
• Venous sinus engorgement
• Pituitary gland enlargement/hyperemia
• Downward displacement of brain/ tonsillar ectopia
• Subdural fluid collections and hematomas, often
  without mass effect (50)

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SIH Radiographic Findings

• CT Myelography
• Study of choice for localizing leaks
• Lower cervical and thoracic region most common
• Often reveals CSF leaks and meningeal diverticula
• Better localization than spinal MR imaging
• Sensitivity 67 in one study
• Radionuclide Cisternography
• Radioactive tracer injected into lumbar
  subarachnoid space
• Normally, CSF travels upwards and is absorbed
  into sinuses
• Can detect CSF leaks
• Sensitivity 60 for actual CSF leak, 90 for
  abnormal study
• Doppler Flow Imaging
• Superior ophthalmic vein engorgement on TCDs
• Sensitive/specific in 26 of 26 patients (100)
• Compared to healthy volunteers
• Improved with treatment

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SIH Pathophysiology

• Brain weighs approximately 1500g
• Intracranial weight is 48g because of
  suspension in CSF
• Brain otherwise supported by meninges, veins,
  cranial nerves (esp. CNs V, IX, X)
• Depletion of CSF in SIH causes downward pressure
  on these structures with traction on cranial
  nerves
• Monro-Kellie Hypothesis Decreased CSF leads to
  venous engorgement and cerebral edema/hyperemia.

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SIH Treatment Options

• Symptomatic relief (Conservative Management)
• Often successful as first-line therapy
• Supine position
• Caffeine or theophylline (IV or PO) effective in
  75 of cases (vasoconstriction resulting in
  decreased CBF)
• Fluid restoration Increased IV/oral hydration,
  salt intake, CO2 inhalation
• No proven efficacy for these therapies

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SIH Treatment Options

• Epidural Blood Patch
• Technique developed by Gromley
• 85-90 efficacy for first trial
• Up to 98 efficacy with repeat patches
• Most effective if placed within 1 level of the
  leak
• If leak site undetectable, may place patch in
  lumbar spine and place in trendelenburg position
  (up to 9 level efficacy in models)
• Immediate relief often observed (90)
• Initial relief gelatinous seal over hole
• Long-term Collagen deposition, fibroblast
  activity, scar formation

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SIH Treatment Options

• Surgical repair of CSF leak
• For refractory cases
• Especially for meningeal divertcula
• Treatment with ligation of diverticula
• Meningeal tears show less success with surgical
  repair
• Fibrin Glue reported with success

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SIH Long term Outcomes

• Berroir S, Neurology, 2004
• 30 patients receiving early epidural blood patch
• Follow-up time 1-4 years
• 77 of patients cured with epidural blood patch
• 57 after 1 patch
• 20 after 2nd patch
• Kong DS et al, Neurosurgery, 2005
• 13 patients treated with nonsurgical measures
• Mean follow-up 51 months
• One recurrence (8)
• Six patients with persistent HAs (4 mild, 2
  moderate)

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References

• 1. Paldino M et al. Intracranial hypotension
  Syndrome a comprehensive review. Neurosurgical
  Focus 15 (6). 2003, 1-8. 1.
• 2. Schievink WI et al. Pseudo-subarachnoid
  hemorrhage A CT finding in SIH. Neurology
  200565 135-137
• 3. Schievink WI et al. Misdiagnosis of
  spontaneous intracranial hypotension. Arch
  Neurol. 60 (12). 2003. 1713-18.
• 4. Inenaga C. Diagnostic and surgical strategies
  for intractable SIH. J Neurosurg. 94(4). 2001.
  914-916.
• 5. Schievink WI et al. SIH mimicking aneurysmal
  SAH. Neurosurgery. 48(3). 2001. 516-517.
• 6. Rai A et al. Epidural Blood Patch at C2
  Diagnosis and Treatment of SIH. AJNR. 26. 2005.
  2663-2666.
• 7. Berroir S et al. Early epidural blood patch in
  SIH. Neurology 63 1950-1951, 2004.
• 8. Kong, DS et al. Clinical features and
  long-term results of SIH. Neurosurgery. 57(1).
  2005. 91-96.

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Thank You