Idiopathic Intracranial Hypertension

Transcription

1 Idiopathic Intracranial Hypertension Dr. Mar'n Su+onBrown MD. FRCPC Neuro-Ophthalmology, Neurology Div of Neurology, Island Health Clinical Assistant Professor, Div of Neurology, UBC Stroke Rapid Assessment Unit - Nanaimo Pacific Coast Eye Centre

2 Outline

3 Modified Dandy Criteria 1 1. Symptoms and signs only reflect those of generalized intracranial hypertension or papilledema. 2. Elevated ICP must be documented, measured in the lateral decubitus posinon. 3. Normal CSF composinon. 4. No evidence of hydrocephalus, mass, structural or vascular lesion on structural neuroimaging for typical panents, and magnenc resonance imaging (MRI) and magnenc resonance venography for all others. 5. No other cause of intracranial hypertension idennfied. 1. Neurology 2002;59:1492-5

4 Modified Dandy Criteria 1 1. Symptoms and signs Headache most common. Enlarged Blind Spot or disc base field loss, TVO, Diplopia (VI palsy) Other rare findings (VII palsy) 2. Elevated ICP Normal, <25 Boarderline, 25 Elevated 2. Consider repeat if high index 3. Normal CSF composinon Cells, Protein, Glucose. Others depending on context 4. No evidence of lesion on structural neuroimaging 1. Look at the jugular. 2. Consider spinal imaging in Men or atypical cases 5. No other cause of intracranial hypertension idennfied 1. But when to stop? I don t use the Criteria suggested in Neurology 2013;81: for Pseudotumor cerebri Neurology 2002;59:1492-5

5 Demographics Clinical Profile 1 Young (mean 29) Women (97% Female) Obese (mean BMI 40) Incidence increasing 2,3 in Minnesota 1/100k ( ) 1.8/100k in 1990 to (actually 2.4 in second half of study!) 1. JAMA Neruol 2014;71: Arch Neurol 1993;50: Ophthal 2017;124:

6 LP by U/S guidance

7 Secondary DifferenNal Sinus Thrombosis Drug induced, Vit A, Steroids, Estrogen, Testosterone Spinal Lesions or Tumor 1 Jugular vein tumor or thrombosis Leptomeningeal Cancer 3 Primary or Secondary Hyperaldosteronism Syphilis 4 Iron Deficiency 5 Superficial Siderosis 1. Journal of Neuro-Ophthalmology 2013;33: Neuroophthalmology Jul 15;39: Journal of Neuro-Ophthalmology 2011;31: BMJ Case Rep. 2011; Arch Dis Child. 2013;98:418.

8 Normal BMI 1 4% (18/407) Normal BMI MedicaNon induced (28 vs 7%): Vit A, ProgesNns, Steroids Men (22 vs 7%) Age 50 5% (19/407) Lower BMI (33 vs 38) Less ininal Headache (36 vs 76%) More vision (42 vs 21%) Atypical IIH 1. Neurology 2010;74:

9 IIH in Men Case series of 66 men vs 721 Women (9%) 1 OSA 24% vs 4% (may be a sig. underesnmate) 2 Older 37 vs 28 Less Headache 55% vs 75% More Visual Disturbance 35% vs 20% More Severe Vision Loss 2.1 RR 1. Neurology 2009;72: Neurology 2009;72:300-1

10 Fulminant IIH < 4 weeks between onset of ininal symptoms and severe visual loss or rapid worsening of visual loss over a few days 16 cases 81% noted vision loss onset at Nme of headache Mean OP 54 Vision Progression 7-28 days to max loss Aoempted treatments: Diamox 1-2g/day, IV methylpred (4), Repeat LP (11), ONSF (5), LP Shunt (9), VP Shunt (2) Median Delay from NO evaluanon to Sx was 3 days. 50% remained legally blind Case study showed T2 lesion with the opnc canal 1. Neurology 2007;68: NeuroOphthalmology 2017;41:84-9.

11 Pathophysiology Excessive CSF producnon Impaired CSF absorpnon Arachnoid granulanons Increased Venous Pressure Venous Stenosis or compression Elevated Jugular pressure QuesNons Is it because of my weight? Is it genenc?

12 Obesity Obesity Clearly common in IIH but is it Causal? IIH is uncommon in the Obese. Consented and screened 606 Obese Clinic pts. 17 with possible symptoms or disc edema 4 with Grade I disc edema on exam, ONLY 3 (0.5%) had LP ; ICP of 24,25, panents, 532 photograph screened 4 prior diagnosis of IIH 3 addinonal detected 7/1084 = 0.65% Vs. PopulaNon studies of 2.4/100k or % But we are not screening the populanon for mild IIH! 1. J Neuroophthlamol 2011;31: Surg Obes Relat Dis 2013; Ophthal 2017;124:

13 GeneNcs Rare case reports of Familial IIH 1,2, 3 Mother and 4 daughters MonozygoNc twins 1, 2 Rarity suggests liole genenc basis 1. J Neuroophthal 2008;28: Eur Neurol 1989;29: J Child Neurol 1992;7: Fujiwara et al. JNNP 1997;62: World Neurosurgery. March 2017;e1-4

14 Medical Therapy Pressure reducing medicanons Acetazolamide (Carbonic Anhydrase inhibitor) Topiramate Furosemide Life style Weight ReducNon: As liole as 6% 1 Weight reducnon Acetazolamide kg in IIHTT! LiragluNde Inj (Sexenda) 2 1. Ophthalmology 1998;105: Obes Rev Jan;18:86-98.

15 IIHTT IIHTT- MulN Centre Randomized double blinded, placebo-controlled trial for 6 months 165 panents Mild visual loss (-2 to -7 db) Low sodium weight reducnon diet plus Acetazolamide (n=86) up to 4g/day Placebo 98% F, 66% White, Age 29, BMI 40, ICP 34-5cm 1. JAMA 2014;311:

16 IIHTT - Outcome 6 month Outcome (Rx vs Placebo) Change Perimetric Mean DeviaNon: 1.43 vs Pl p=0.05 Adjusted Mean Change in PMD (Worst Eye), db Acetazolamide Placebo Change Papilledema vs, p<0.001 Change PMD (Grade 3-5 disc edema) 2.00 vs p< No. of patients Acetazolamide Placebo Month JAMA 2014;311:

17 IIHTT - Outcome 6 month Outcome (Rx vs Placebo) Change Visual Acuity 2.65 vs p=0.99 Change in CSF (n=85) -11 cm vs. -5 cm Weight ReducNon 7.5 kg vs 3.45 kg 1. JAMA 2014;311:

18 IIHTT -Tolerability and AE 1 Dose: 44% tolerated 4g/day of Acetazolamide 11% couldn t tolerate above 750 mg/day 11% stopped Acetazolamide before end of study 84% had one or more AE Common: Paresthesia 49%, Dysgeusia 15%, GI upset 30%, fangue 17% AE were more common at dose <2g/day 1. J NeuroOphthal 2016;36:13-9.

19 IIHTT -Tolerability and AE 1 9 SAE (6 Acetazolamide vs 3 placebo) 3/6 possibly or probably related Renal Impairment at 4g/day TransaminiNs at 4g/day Allergic reacnon at 2.5g/day Comments It reduces opnc disc edema in most panents at much lower doses than 4g/day (11 cm of ICP) PaNents without vision loss may not be monvated to tolerate as much medicanon Sulfonamide annbioncs allergy not a contraindicanon No significant hypokalemia on Acetazolamide alone Consider Transaminase monitoring: 2 asymptomancé 1. J NeuroOphthal 2016;36:13-9.

20 Pregnancy Acetazolamide (Category C FDA drug) Limb malformanons in rodents at high doses Sacrococcygeal teratoma in 1 case 1 Survey of NANOS society members (16%) 2 65% disconnnue, 10% dosage ê, 25% same 63/101 pregnancies on Acetazolamide 2 50 in first 13 weeks No evidence of increased risk 1. JAMA 1978;240: J Neuro-Ophthalmol 2013;33:9-12

21 Bariatric Surgery Meta Analysis of 65 cases 1 100% papilledema resolunon 90% Headache resolunon BMI reducnon 17.5kg/m2 Other Obesity benefits: DM2 2 Longevity:163 Gastric bypass, 24% yr 1, 23% yr3 3 Risks: Short bowl synd, NutriNonal deficiency related neurological disease 4 and late Osteoporosis 5 1. Manfield J H et al. Obes Surg 2017;27: Surg Endosc May;24(5): Obes Surg Sep;26(9): J NeuroOphthal 2016;36: Obes Surg Jun;24(6):

22 ONSF 2 Meta Analysis Visual Fields 64-68% Acuity 59-67% Papilledema 80-95% Repeat Surgery 9-11% Headache 41% Cheapest opnon May Only need one eye ComplicaNon 18-26%, Major complicanon rate 1.5% 1. Acta Neurochir 2016;159: AJNR 2015;36:

23 CSF ShunNng 2 Meta Analysis Visual Fields 71% Acuity 54-67% Papilledema 70-91% Repeat Surgery 43% (2.78 addinonal sx/failure) Headache 80-96% ComplicaNon 33%, Major complicanon rate 7.6% 1. Acta Neurochir 2016;159: AJNR 2015;36:

24 Venous StenNng Meta analysis of 155 cases Mean follow up 22months Primary treatment in 80% of these cases 4-10mmHg stenosis gradient Acuity 65% Fields 75% Headache 77% Papilledema 98% Tx Failure 8% ComplicaNon rate 12% Serious complicanon 4% 1. Acta Neurochir 2016;159: 33-49

25 Treatment Decisions

26 The End