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Case Presentation:
Neurology/Neurosurgery Grand Rounds
February 28, 2006
Gabriel Zada, MD
Christopher Aho, MD
Neurosurgery Blue
LAC-USC Medical Center
Patient G.P.
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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
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
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.
Physical Examination
• Motor:
– Tone Normal
– No pronator drift
– Power 5/5 in all extremities
• Reflexes:
– 2+, symmetric throughout
– No Hoffman’s sign
– Toes downgoing bilaterally
• Sensory:
– Sensation intact in all extremities.
• Cerebellar/Gait:
– Finger-nose-finger normal. Gait exam deferred.
Head CT
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
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.
Brain MRI
MRI: Final Report
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Bilateral SDH
Evidence of SAH
Diffuse meningeal enhancement
Decreased caliber of right ICA and MCA,
may be suggestive of vasospasm.
Hospital Course (continued)
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Lumbar Puncture felt to be contraindicated
Right ventriculostomy placed on HD#5
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,.
MRI: Final Report
• Interval placement of R frontal
ventriculostomy
• Left greater than right SDH
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
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
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)
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
Spontaneous Intracranial Hypotension (SIH)
• Patient Demographics:
– Often occurs in middle-aged patients
– Mean age ~40 years
– Female preponderance
– Higher incidences in patients with Marfan’s
disease, other connective tissue diseases,
and weightlifters
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
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 < 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,xanthochromia
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%)
SIH: Radiographic Findings
• CT Myelography
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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
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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
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Superior ophthalmic vein engorgement on TCDs
Sensitive/specific in 26 of 26 patients (100%)
Compared to healthy volunteers
Improved with treatment
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.
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
SIH: Treatment Options
• Epidural Blood Patch
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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
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
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:
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13 patients treated with nonsurgical measures
Mean follow-up 51 months
One recurrence (8%)
Six patients with persistent HAs (4 mild, 2 moderate)
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 2005;65: 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.
Thank You