Table 1 Key clinical features of our patient from 2004 to 2011
From: An autoinflammatory neurological disease due to interleukin 6 hypersecretion
April 20041 | March 2006 | September 2006 | October 2006 | September 2007 | July 2008 | November 2008 | October 2009 | September 2011 | September 2011 | |
|---|---|---|---|---|---|---|---|---|---|---|
Clinical manifestations | Transient double vision | Intermittent fever, chronic headache (> morning), recurrent episodes of double vision, papilledema, one tonic-clonic seizure | Intermittent fever, chronic headache (> morning), recurrent episodes of double vision, papilledema | No symptom | No symptom | Morning headache, one episode of vomiting | Morning headache, hearing loss, cushingoid features | Morning headache, hearing loss, postural tremor, cushingoid features | Hearing loss, Babinski sign | Hearing loss, Babinski sign |
Potentially disease-modifying therapy | No | No | No | Dexameth (8 mg/day × 7 days and, then, 4 mg/day × 7 days) | Prednisone 37.5 mg/day | Prednisone 30 mg/day, AZA 200 mg/day | Prednisone 35 mg/day | Prednisone 30 mg/day | Prednisone 12.5/day, Tocilizumab 800 mg/28 days | Methylpred 1,000 mg/day × 5 days |
ESR (≤20 mm) | 104 | 110 | 110 | 35 | 90 | 85 | 100 | 80 | 10 | N/D |
CRP (≤5 mg/L) | 61 | 70.7 | 62 | 3 | 45 | 39 | 80 | 53 | 1 | N/D |
White Cell (%PMN; %L) (3,500 to 10,000/μL; 40 to 74%; 19 to 48%) | 14,100 (67.4; 22) | 12,410 (74.7; 14.1) | 10,060 (75.1; 15.6) | 18,240 (70.6; 21.1) | 18,180 (90.5; 6.3) | 12,610 (73.6; 17.5) | 12,710 (77.1; 13.8) | 13,680 (71.7; 19.9) | 11,880 (78.5; 15.2) | N/D |
Hemoglobin (14 to 16 g/dl) | 11.7 | 10.6 | 10.1 | 11.8 | 12.8 | 11.4 | 11.5 | 11.7 | 15.8 | N/D |
IL-6 in serum (≤10 pg/ml) | N/A | N/A | N/A | N/A | 44.23 | 22.4* | 11.42 | 38.7 | 127 | 44.8 |
CSF pressure (5 to 15 cmH2O) | N/D | N/D | 40 | 40 | 35 | N/D | N/D | 24 | N/D | Normal |
CSF White Cell/μl (L; PMN) (≤4/μl) | 16 (N/D) | 40 (25; 7) | 40 (24; 12) | 2.6 | 24 (12; 10) | 45 (20; 12) | 63 (10; 47)2 | 32 (26; 5) | 65 (25; 22) | 8 (7.3; 0.6) |
CSF proteins (10 to 45 mg/dl) | 63 | 84.1 | 98.4 | 33.0 | 73.9 | 90.6 | 98.2 | 123.2 | 102.2 | 54.5 |
CSF albumin / serum albumin × 1,000 (≤6.5) | 6.37 | 12.36 | 15.51 | 8.37 | 12.08 | 18.26 | 21.18 | 20.59 | 16.86 | 11.12 |
Link IgG index (0.1 to 0.7); ReiberIgG index (≤0) | 0.66; N/A | 0.79; -5.07 | 0.96; 22.30 | 0.50; -21.95 | 1.90; 104.57 | 1.01; 25,61 | 1.09; 38.59 | 1.49; 118.81 | 0.77; -9.9 | 0.63; -14.89 |
OBs (negative) | No (type 1) | No (type 1) | No (type 1) | No (type 1) | No (type 1) | Yes (type 3) | No (type 4) | Yes (type 3) | No (type 4) | No (type 1) |
IL-6 in CSF (≤10 pg/ml) | N/A | N/A | N/A | 232.8 | 4544 | N/A | 9585 | 5389 | 7874 | 85 |
ANAs (negative) | N/D | 1:320 | 1:640 | 1:640 | 1:40 | 1:320 | 1:80 | 1:80 | 1:40 | N/D |
Proteinuria (0 to 150 g/day) | N/D | N/D | N/D | N/D | 800 | 1299 | 460 | 694 | 151 | N/D |
Other | Chronic recurrent osteomyelitis of the mandible | - | - | - | SSA 62.90 mg/l (nv ≤ 6.4) | Hepcidin 9.07 nmol/l (nv 4.3-7.06) | - | - | ||