CONFESSIONS OF A PSEUDOTUMOR CEREBRIST

Transcription

1 CONFESSIONS OF A PSEUDOTUMOR CEREBRIST Jean B Kassem, M.D. Neuro-Ophthalmology, Orbital Surgery, Oculoplastics Bellingham Eye Physicians Bellingham, WA

2 Goals Understand Intracranial Hypertension and its Effects on Vision History Taking Role of Office Procedures Role of Imaging Treatment

3 Overview Terminology Differential for Optic Disc Edema/Elevated Intracanial Pressure Diagnostic Criteria for Idiopathic Intracranial Hypertension Office Procedures Imaging Lumbar Puncture Treatment - IIH Medical Surgical

4 TERMINOLOGY Papilledema Optic disc edema Papillitis Pseudopapilledema Optic neuropathy

5 TERMINOLOGY Papilledema Due to elevated cerebrospinal fluid (CSF) pressure Optic disc edema Generic term for optic disc swelling Papillitis Inflammation of optic disc Pseudopapilledema Drusen Optic neuropathy Generic term for optic nerve damage

6 TERMINOLOGY Papilledema Due to elevated cerebrospinal fluid (CSF) pressure Optic disc edema Generic term for optic disc swelling Papillitis Inflammation of optic disc Pseudopapilledema Drusen Optic neuropathy Generic term for optic nerve damage

7 TERMINOLOGY Pseudotumor Cerebri Idiopathic Intracranial Hypertension Hydrocephalus

8 TERMINOLOGY Idiopathic Intracranial Hypertension primary intracranial hypertension with no cause found (Modified Dandy Criteria) Pseudotumor Cerebri older, now generic term for intracranial hypertension I use this for the secondary forms Hydrocephalus infantile form, geriatric form (normotensive), obstructive form

9 CSF HOMEOSTASIS

10 CSF HOMEOSTASIS

11 CSF HOMEOSTASIS

12 OPTIC DISC EDEMA WHAT NOW?

13 OFFICE TESTING Is it edema? Is there vision loss (optic neuropathy)? OUTSIDE TESTING What is the cause of the elevated intracranial pressure? How high is the opening pressure?

14 OFFICE Is it edema? History Sensorimotor Exam Funduscopy IVFA/Autofluorescence OCT

15 OFFICE Is it edema? History signs and symptoms of elevated intracranial pressure Sensorimotor Exam Funduscopy IVFA/Autofluorescence OCT

16 HEADACHE most frequent symptom generally holocranial or retrobulbar relatively constant aching or throbbing variable intensity, often worse supine may be associated with nausea or lightheadedness

17 TRANSIENT VISUAL OBSCURATIONS unilateral or bilateral blurring, dimming or loss of vision lasting 2 or 3 seconds secondary to optic disc swelling often with positional changes, head turn or eye movements

18 DIPLOPIA Horizontal, Binocular 6th Nerve Palsy

19 OFFICE Is it edema? History Sensorimotor Exam Funduscopy IVFA/Autofluorescence OCT

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21 OFFICE Is it edema? History Sensorimotor Exam Funduscopy IVFA/Autofluorescence OCT

22 Diagnosis and Grading of Papilledema in Patients With Raised Intracranial Pressure Using Optical Coherence Tomography vs Clinical Expert Assessment Using a Clinical Staging Scale Arch Ophthalmol. 2010;128(6): doi: /archophthalmol Copyright 2012 American Medical Association. All rights reserved.

23 OFFICE Is it edema? History Sensorimotor Exam Funduscopy IVFA/Autofluorescence OCT

24 High Definition Images: HD 5 Line Raster OD OS Scan Angle: 0 Spacing: 0.25 mm Length: 6 mm OFFICE Is it edema? History Sensorimotor Exam Funduscopy IVFA/Autofluorescence OCT Name: Kassem, Jean ID: CZMI Exam Date: 3/11/2016 DOB: 2/8/1976 Exam Time: 3:42 PM Comments Doctor's Signature Gender: Male Serial Number: Technician: Operator, Cirrus Signal Strength: 8/10 High Definition Images: HD 5 Line Raster Scan Angle: 0 Spacing: 0.25 mm CZMI Length: 6 mm OD SW Ver: Copyright 2015 Carl Zeiss Meditec, Inc All Rights Reserved Page 1 of 1 OS

25 OPTIC DISC DRUSEN (PSEUDOPAPILLEDEMA) 1% of the population More frequent in caucasians Bilateral in 75% May be inherited as an AD trait with incomplete penetrance or may be spontaneous Usually not visible at birth Rarely visible < age 10 buried

26 OPTIC DISC DRUSEN Calcify with age, become more prominent Often asymptomatic, found incidentally Often mistaken for papilledema Optic discs often congenitally anomalous Crowded Loss of physiologic cup Tri-branching vessels Situs inversus

27 VISUAL FIELD LOSS Internal compressive optic neuropathy 70% develop some visual field loss Gamut of visual field deficits, mimics glaucoma Increased risk NA-ION, BRVO, CRVO

28 OPTIC DISC DRUSEN: TREATMENT No proven treatment Monitor for choroidal neovascularization Monitor HVF Consider topical therapy to lower IOP Neuroprotective agent-> Brimonidine Radial optic neurotomy Manual removal of drusen with vitrectomy

29 FLUORESCEIN ANGIOGRAM differentiate optic disc drusen from true papilledema autofluorescence of disc drusen on initial redfree photographs may show late staining with optic disc drusen true leakage in papilledema

30 B SCAN ULTRASOUND

31 PSEUDOPAPILLEDEMA

32 OFFICE Is There Vision Loss? HVF Vision Pupils

33 OFFICE HVF 30-2 Enlarged BS

34 OFFICE Visual Field in Papilledema Enlarged blind spot and generalized constriction are most common nasal step and arcuate scotomas possible cecocentral scotoma less likely

35 OFFICE Is There Vision Loss? HVF Vision Pupils - ALWAYS CHECK FOR APD

36 OPTIC DISC EDEMA WHAT NOW? > Diagnostics Imaging Intracranial Pressure

37 DIFFERENTIAL DIAGNOSIS Intracranial mass lesion with obstructive hydrocephalus Ischemic (AION) Hypertensive Urgency Papillitis (infection, inflammation) atypical optic neuritis meningitis neuroretinitis Infiltrative (leukemia, sarcoid) Pseudopapilledema IIH or Secondary Pseudotumor

38 SECONDARY PSEUDOTUMOR CEREBRI medications nalidixic acid fluoroquinolones tetracycline doxycycline minocycline Acutane growth hormone hypervitaminosis A lithium carbonate prolonged steroid use venous occlusive disease dural sinus thrombosis infiltrative disease meningeal carcinomatosis sarcoidosis systemic disease Systemic lupus erythematosis Behcet s disease Acromegaly infectious disease post streptococcal post viral

39 MODIFIED DANDY CRITERIA IIH SIGNS AND SYMPTOMS OF ELEVATED INTRACRANIAL PRESSURE ABSENCE OF LOCALIZING FINDINGS ON NEUROLOGIC EXAM NORMAL NEURO IMAGING, EXCEPT EMPTY SELLA AND CSF SPACE AROUND NERVES (MRI AND MRV) NORMAL CSF COMPOSITION AWAKE AND ALERT WITH NO OTHE RCAUSE FOR ELEV CSF CSF PRESSURE >25CM H 2 0 OR >20 IF: PULSATILE TINNITUS FRISEN GRADE 2 PAPILLEDEMA B SCAN NEGATIVE FOR DRUSEN PARTIALLY EMPTY SELLA WITH CSF SPACE NEXT TO GLOBE ON MRI

40 IMAGING MRI Brain MRV Brain - Dural Sinus Thrombosis

41 RADIOLOGIC IMAGING STUDIES normal to small sized ventricles no evidence of a mass lesion empty sella in up to 70% clear differentiation between the optic nerve and sheath enlarged, elongated subarachnoid space flattening of posterior aspect of the globe MRI better to rule out infiltrative dz, VST

42 opening pressure >20 cm H 2 O falsely high reading: position, valsalva falsely low reading: multiple puncture R/O infection, inflammation radiologic guidance if poor landmarks

43 LUMPAR PUNCTURE BLIND (BEDSIDE) FLUOROSCOPIC GUIDED (X Ray) CT GUIDED

44 LUMPAR PUNCTURE Methods SonoSite Model M-Turbo 10 IIH patients underwent 11 ultrasound guided lumbar punctures with a low frequency curvilinear probe (Model M-Turbo, Manufactured by SonoSite, Bothell, WA) between July and October 2013.

45 Methods 4" 24 gauge Pencan pencil-point needle or a ", 24 or 22 gauge Sprotte pencil-point needle

46 Results Left Parasagittal Ultrasound View, Lumbar Region L4 L5 S1

47 Results In 10/11 procedures, only one attempt at puncture OP was obtained, as was sufficient CSF for multiple studies No subject had a post-puncture headache 2 post-procedure complications serous drainage at puncture site (1) - resolved spont. paresthesia (1) - resolved after a dose of dexamethasone They loved it

48 EPIDEMIOLOGY: IIH F:M ratio of 8:1 in the adult population peak incidence 3rd decade (infancy to old age) incidence in general population 1:100,000 women 20-44, >10% over IBW 13:100,000 women 20-44, >20% over IBW 19.3:100,000 men 20-44, >20% over IBW 1.5:100,000

49 IIH in Men Men with IIH have more symptoms associated with testosterone deficiency OSA OSA -- cause vs. chance association Bruce et al. J Neuro Sci 290;2010, 86 89

50 PERMANENT VISUAL LOSS compressive optic nerve damage optic disc infarction choroidal folds subretinal hemorrhage

51 VISUAL FIELD LOSS Enlarged blind spot and generalized constriction are most common nasal step and arcuate scotomas possible cecocentral scotoma less likely

52 OBSERVED SIGNS papilledema may be unilateral sine papilledema relative afferent pupillary defect cranial nerve VI palsy

53 MANAGEMENT suspect exogenous agents should be discontinued LP done initially for dx may be therapeutic weight loss is the most effective treatment may get off Rx and avoid surgery consider dietician

54 CARBONIC ANHYDRASE INHIBITORS Neptazane 50 mg bid to qid Diamox 250 mg bid to 500 mg qid common adverse effects tingling and numbness in fingers/toes metallic taste K+ wasting relative contraindication 1st 4 months pregnancy sulfa based - can be used if allergy is abs aplastic anemia is a rare idiosyncratic rxn

55 ALTERNATIVE DRUGS Lasix 20 mg qd to 40 mg qid corticosteroids generally not indicated may be useful if inflammatory mechanism (I.e., SLE) further weight gain and fluid retention rebound on withdrawal iv solumedrol may be useful acutely Octreotide Topamax Beta blockers?

56 SURGICAL MEASURES - CSF SHUNTS Ventriculoperitoneal Lumboperitoneal

57 LUMBOPERITONEAL SHUNT more likely to reduce ICP, relieve H/A than ONSF Contraindicated (relative) in Chiari Malformation average replacement every 2-3 shunt years migration, closure, infection over or under-filtration

58 VENTRICULOPERITONEAL SHUNT more likely to reduce ICP, relieve H/A average replacement every 4 shunt years migration, closure, infection over or under-filtration NEUROSURGEON DECIDES WHICH TYPE TO USE

59 OPTIC NERVE SHEATH FENESTRATION window or multiple longitudinal slits made in the anterior dural covering of the optic nerve immediate decompression of optic nerve tip less likely to maintain lower ICP, relieve H/A neuroprotective

60 OPTIC NERVE SHEATH FENESTRATION: MEDIAL APPROACH

61 OPTIC NERVE SHEATH FENESTRATION: MEDIAL APPROACH

62 Visual Outcomes following Optic Nerve Sheath Fenestration via the Medial Transconjunctival Approach Steven E Katz, MD 1 et al 207 eyes of 104 patients from Outcomes: MD on HVF and Papilledema Grade Followed for 6 months Edema resolved completely in 76% in 1 week, 71% at 6 months MD at 1 week, at 6 months Conclusion: Safe and effective treatment for disc edema

63 CASE PRESENTATION 22 y.o. obese wf presents with severe holocranial progressive H/A and loss of peripheral vision over 6 months hx IIH s/p R ONSF 18 months prior left relative afferent pupillary defect generalized visual field constriction L>R on diamox 500 mg sequels p.o. qid LP with opening pressure 32 cm H 2 O

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65 PRESENTING VISUAL FIELDS

66 1 WEEK AFTER LEFT ONSF

67 1WEEK AFTER RIGHT ONSF

68 PATIENT MONITORING papilledema may not resolve completely with tx may not recur with ICP optic nerve appearance alone is not adequate to assess for recurrent elevation of ICP! subjective symptoms and visual field progression may be more reliable visual fields patient education and participation are essential

69 IIH ACCORDING TO JEAN Initial OP < 35 cm H 2 O Initial OP cm H 2 O Initial OP > 46 cm H 2 O Low threshold for ONSF Profound papilledema and ONSF

70 IDIOPATHIC INTRACRANIAL HYPERTENSION TREATMENT TRIAL (IIHTT)

71 IIHTT The IIHTT is a prospective clinical treatment trial on idiopathic intracranial hypertension that includes a genetic association study in search of single polymorphisms (SNPs) to identify metabolic and hormonal factors that differentiate between obese women who have IIH and obese women who do not - the first NORDIC multicenter study.

72 IIHTT: SPECIFIC AIM ONE IIH patients with Mild Visual Loss (-2 to -5 db baseline PMD) will be recrutied to participate in this randomized, double-masked, placebocontrolled trial to determine the additional benefit of acetazolamide (up to 4 gm a day) added to a low sodium, weight reduction diet. Hypothesis: Acetazolamide + diet is superior to diet alone in restoring vision or preventing visual loss in IIH patients with mild visual loss.

73 IIHTT: SPECIFIC AIM TWO (a) To identify proteomic and genetic risk factors for IIH by screening a large cohort of IIH patients and controls, (b) To determine the serum and CSF levels of potential mediators of IIH suggested by the genetic analysis, and (c) To conduct an association study in search of single nucleotide polymorphisms (SNPs) that confer risk for developing IIH. A cohort of 154 IIH patients and 154 controls matched on body-mass index, ethnicity and gender will be genotyped at SNPs contained within genes encoding molecules likely to be involved in the etiology of IIH using the SNPlex genotyping system. Specifically, genes associated with obesity will be profiled. (d) To test the hypotheses IIH is associated with abnormal metabolism of leptin or vitamin A or both, leptin levels, vitamin A and related factors will be measured at baseline and six months.

74 IIHTT: RESULTS

75 IIHTT: RESULTS

76 IIH: TAKE HOME POINTS History - Signs of Elevated ICP Check for APD! HVF 30-2 OCT 5 line raster may be helpful If scheduling LP - OPENING PRESSURE Vision loss is preventable/treatable

77 IT S NOT ALWAYS PSEUDOTUMOR. MANY OTHER CAUSES FOR BILATERAL DISC EDEMA

78 PSEUDO PSEUDOTUMOR (TUMOR)

79 HYPERTENSIVE CRISIS Hypertensive crisis Generally bilateral Blurred vision Headache Dizziness encephalopathy

80 IINFECTION Meningitis Stiff neck Headache Fever Skin rash Obtundation Considerations HIV+ Cryptococcus Tertiary syphylis May be associated with raised ICP

81 INFECTION/INFLAMMATION Neuroretinitis Infectious/immune-mediated Ddx Cat scratch disease Post-viral: HSV, hepatitis B, mumps Spirochetes: syphylis, Lyme, leptospirosis Possible: toxoplasmosis, toxocariasis, histoplasmosis Tuberculosis Leber s idiopathic stellate neuroretinitis Treatment Antibiotics: doxycycline, erythromycin, azithromycin, ciprofloxacin, rifampin Corticosteroids

82 INFILTRATIVE: SARCOID Uveitis Papillitis Infiltrative, compressive optic neuropathy Systemic manifestations: lungs, skin 5/100,000 caucasians 40/100,000 African Americans ACE, lysozyme serum CSF ACE MRI brain with gad Tissue diagnosis

83 INFILTRATIVE: LEUKEMIA Papillitis Uveitis Hyphema

84 INFILTRATIVE: LARGE CELL LYMPHOMA Refractory uveitis Papillitis Choroidal infiltrates Usually known CNS involvement Vitrectomy with cell cytology can be diagnostic

85 THANK YOU!