Case Presentation: Neurology/Neurosurgery Grand Rounds February 28, 2006 Gabriel Zada, MD Christopher Aho, MD Neurosurgery Blue LAC-USC Medical Center

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

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 3 rd 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 3 rd 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 Bilateral SDH Evidence of SAH Diffuse meningeal enhancement Decreased caliber of right ICA and MCA, may be suggestive of vasospasm.

Hospital Course (continued) 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 3 rd 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 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 (flat)

Post-treatment Course Post-patch day 1: Patient awake, alert x 2. Complete resolution of 3 rd 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 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 –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

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 –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 2 nd 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)

References 1. Paldino M et al. Intracranial hypotension Syndrome: a comprehensive review. Neurosurgical Focus 15 (6). 2003, Schievink WI et al. Pseudo-subarachnoid hemorrhage: A CT finding in SIH. Neurology 2005;65: Schievink WI et al. Misdiagnosis of spontaneous intracranial hypotension. Arch Neurol. 60 (12) Inenaga C. Diagnostic and surgical strategies for intractable SIH. J Neurosurg. 94(4) Schievink WI et al. SIH mimicking aneurysmal SAH. Neurosurgery. 48(3) Rai A et al. Epidural Blood Patch at C2: Diagnosis and Treatment of SIH. AJNR Berroir S et al. Early epidural blood patch in SIH. Neurology 63; , Kong, DS et al. Clinical features and long-term results of SIH. Neurosurgery. 57(1)

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