Introduction
Methods
Patients and definitions
Ophthalmological assessment
Magnetic resonance imaging
Lumbar puncture
Treatment
Follow-up
Ethics
Data availability statement
Statistics
Results
Cohort characteristics
(n = 113) | ||
---|---|---|
Females | n (%) | 101 (89.4) |
Age at diagnosis (years) | Mean (SD) | 32.3 (10.7) |
IIH with papilledema | n (%) | 107 (94.7) |
IIH without papilledema | n (%) | 6 (5.3) |
Symptoms at initial presentation | ||
Headache | n (%) | 95 (84.1) |
Features of migraine | n (%) | 41 (43.2) |
Features of tension-type headache | n (%) | 16 (16.8) |
Unspecified | n (%) | 38 (40.0) |
Visual symptoms | n (%) | 86 (76.1) |
Abducent nerve palsy | n (%) | 6 (5.3) |
Divergence insufficiency | n (%) | 4 (3.6) |
Pulsatile tinnitus | n (%) | 27 (23.9) |
BMI | Median (IQR; AR) | 31.8 (27.6–39.8; 17.3–60.9) |
Overweight (BMI > 25) | n (%) | 96 (85.0) |
Obesity (BMI > 30) | n (%) | 60 (53.1) |
Other risk factors and comorbidities | ||
Hormonal contraception | n (%) | 9 (8.0) |
Tetracyclines | n (%) | 2 (1.8) |
Retinoids | n (%) | 1 (0.9) |
Hypothyroidism | n (%) | 21 (18.6) |
Diabetes mellitus | n (%) | 8 (7.1) |
Arterial hypertension | n (%) | 14 (12.4) |
COPD | n (%) | 1 (0.9) |
OSAS | n (%) | 3 (2.7) |
Depression | n (%) | 19 (16.8) |
MRI findings at initial diagnosis
Neuro-ophthalmological findings at initial diagnosis
Visual acuity | – | n = 106 |
Abnormal | n (%) | 17 (16.0) |
Worse eye (logMAR) | Median (IQR; AR) | 0 (−0.1–0.0; −0.1–1.0) |
Perimetry | – | n = 96 |
Abnormal | n (%) | 64 (66.7) |
Mean deviation in perimetry of worse eye (dB) | Median (IQR; AR) | −3.1 (−11.6–−1.1; −32.6–0) |
Fundoscopy | – | n = 103 |
Abnormal | n (%) | 98 (89.1) |
Frisén scale | Median (IQR; AR) | 3 (2–4; 0–5) |
Optical coherence tomography | – | n = 99 |
pRNFL thickness (µm) | Median (IQR; AR) | 199 (132–299; 55–560) |
GCL volume (mm3) | Median (IQR; AR) | 1.13 (1.05–1.17; 0.60–1.25) |
Ultrasonography | – | n = 58 |
ONSD > 4.5 mm and/or bat sign | n (%) | 53 (86.9) |
ONSD of worse eye (mm) | Median (IQR; AR) | 5.4 (5.0–5.7; 3.9–7.3) |
ONSD intereye difference (mm) | Median (IQR; AR) | 0.15 (0.04–0.28; 0.00–0.82) |
Treatment
Weight loss | n | 86 |
Weight loss ≥ 6% from weight at diagnosis | n (%) | 49 (57.0) |
Change of weight absolute (kg) | Median (IQR; AR) | −7 (−15–−1; −75–43) |
Change of weight percentage (%) | Median (IQR; AR) | −6.8 (−14.9–−1.3; −58.6–40.2) |
IIH specific medication | n | 113 |
Acetazolamide | n (%) | 112 (99.1) |
Maximum acetazolamide dosage (mg) | Median (IQR; AR) | 750 (500–1000; 250–2000) |
Topiramate | n (%) | 15 (13.3) |
Maximum topiramate dosage (mg) | Median (IQR; AR) | 62.5 (50–75; 25–200) |
Furosemide | n (%) | 12 (10.6) |
Maximum furosemide dosage (mg) | Median (IQR; AR) | 35.0 (20–40; 20–160) |
Combination treatment | n (%) | 23 (20.4) |
Therapeutic lumbar puncture | n | 113 |
Entire study perioda | n (%) | 63 (55.8) |
Cumulative numbera | Median (IQR; AR) | 1 (0–4; 0–26) |
Follow-up (> 4 weeks after diagnosis) | n (%) | 27 (26.2) |
Cumulative number | Median (IQR; AR) | 0 (0–1; 0–23) |
Surgery | n | 113 |
Ventricular peritoneal/atrial shunt | n (%) | 14 (12.4) |
Shunt revision | n (%) | 3 (21.4) |
Bariatric surgery | n (%) | 1 (0.9) |
Outcome
Duration of follow-up (years) | Median (IQR; AR) | 3.7 (1.8–6.1; 0.3–17.4) |
Headache | n | 104 |
Headache-free | n (%) | 25 (24.0) |
Persistent headache | n (%) | 79 (76.0) |
Improveda | n (%) | 60 (75.9) |
Visual acuity | n | 110 |
Abnormal | n (%) | 17 (15.0) |
Worse eye (logMAR) | Median (IQR; AR) | −0.1 (−0.1–0.0; −0.1–2.0) |
Change of visual acuity in worse eye (logMAR)a | Median (IQR; AR) | 0.0 (−0.1–0.0, −0.4–1.0) |
Worseneda (> +0.2 logMAR) | n (%) | 5 (4.5) |
Improveda (> −0.2 logMAR | n (%) | 5 (4.5) |
Perimetry (n = 106) | ||
Abnormal | n (%) | 57 (49.6) |
Mean deviation of worse eye (dB) | Median (IQR; AR) | −0.8 (−5.2–−0.3; −33.9–1.3) |
Change of mean deviation of worse eye (dB)a | Median (IQR; AR) | 1.6 (0.1–3.6; −10.8–25.4) |
Worsened (> −2 dB)a | n (%) | 8 (8.1) |
Improved (> +2 dB)a | n (%) | 27 (23.9) |
Fundoscopy (n = 109) | ||
Abnormal | n (%) | 64 (59.3) |
Worseneda | n (%) | 5 (4.6) |
Improveda | n (%) | 62 (57.4) |
Optical coherence tomography (n = 95) | ||
pRNFL thickness (µm) | Median (IQR; AR) | 99 (87–110; 29–245) |
Change of pRNFL (µm)a | Median (IQR; AR) | −105 (−202–−30; −504–21) |
GCL volume (mm3) | Median (IQR; AR) | 1.05 (0.94–1.10; 0.62–1.13) |
Change of GCL volume (mm3)a | Median (IQR; AR) | −0.05 (−0.14–−0.03; −0.52–0.09) |
Ultrasonography (n = 76) | ||
ONSD > 4.5 mm and/or bat sign | n (%) | 49 (64.5) |
Normalized ONSD (n = 56) | n (%) | 7 (11.5) |
ONSD of worse eye (mm) | Median (IQR; AR) | 4.9 (4.3–5.3; 3.0–6.3) |