Case 1
A 39-year-old male patient was admitted to our hospital with complaints of bronchial asthma, rhinitis complicated by nasal polyposis, and eosinophilia in the previous seven months. He was treated with an inhalation corticosteroid, a long-acting β-stimulant, and an oral pranlukast for the asthma. He had no history of smoking or hypertension. Two months later, he suffered from diffuse arthralgia, persistent slight fever, and weight loss. Erythematosus vesiculopurpura skin rash on the dorsal aspect of the both lower leg was observed on the general physical examination.
Laboratory tests on admission to our hospital showed the following results: hematuria (trace) with proteinuria (76.2 mg/dl) (normal range 1.0-14.0 mg/dl), leukocytosis (18,800 per µl) (normal range 3,900-9,700 per µl), increased eosinophil count (3,572 per µl, 19%) (normal range 50-500 per µl, 0-10%), slightly increased Erythrocyte Sedimentation Rate (ESR) (43 mm/h) (normal range 0-10 mm/h), increased C-reactive protein (CRP) (5.41 mg/dl) (normal range 0.02-0.8 mg/dl), increased Blood Urea Nitrogen (BUN) (44.7 mg/dl) (normal range 8-25 mg/dl), and increased serum creatinine (2.5 mg/dl) (normal range 0.5-1.4 mg/dl). Other examinations revealed elevation of the eosinophil cationic protein (ECP) (38.6 µg/L) (normal range 0-13.5 µg/L). Myeloperoxidase anti-neutrophil cytoplasmic antibody (MPO-ANCA) levels were elevated at 4.0 AI (positive ≥ 1.0 AI), and negative results were observed for anti-nuclear, anti-extractable nuclear, anti-DNA, and anti-phospholipid.
Skin biopsy of the erythematosus showed leukocytoclastic vasculitis.
Chest computed tomography (CT) showed mild bronchial wall thickening in the lower lung zone and a small amount of pericardial effusion without parenchymal or interstitial infiltrates. The electrocardiogram showed normal findings, whereas the echocardiogram revealed small pericardial effusion.
Findings on abdominal CT revealed mild hepatomegaly and periportal edema with multifocal low density in the liver, more likely related to hepatic congestion or ischemia, and multiple wedge shaped low densities in both kidneys, which was diffuse involvement of nephritis. Pathological examination by kidney needle biopsy showed focal segmental glomerulosclerosis with focal and segmental mesangial Immunoglobulin A (IgA) deposits.
Based on clinical and laboratory findings, a diagnosis of CSS was made, according to the American College of Rheumatology Criteria.
9) Thus, the patient received treatment with three-day intravenous (IV) methylprednisolone (1 mg/kg/day) and IV cyclophosphamide, followed by daily oral prednisone 1 mg/kg.
Over the preceding six months, the patient had exhibited noticeable numbness of the left hand. Findings of a sensory nerve conduction study revealed low amplitude in the left median nerve. Therefore, it was determined that the numbness was caused by peripheral neuropathy of the left median nerve.
On day 12 after admission, the patient exhibited sudden onset of consciousness disturbance with generalized tonic clonic seizures. Findings on brain CT showed diffuse SAH with intraventricular hemorrhage on the fourth ventricle and acute obstructive hydrocephalus (
Fig. 1A). And findings on brain CT angiography (CTA) were suggestive of VAD (
Fig. 1B). The patient's neurological status had worsened to Hunt-Hess Grade 4. Just after the brain CTA, we performed an emergent external ventricular drainage (EVD) catheter insertion into the right lateral ventricle through the supra-orbital.
An emergent digital subtraction angiography (DSA) immediately showed the left intracranial VAD. Extravasation of the contrast media from the dissected segment was visible on the left vertebral angiogram (
Fig. 2A).
To obstruct the burst in the dissected segment, we decided to immediately occlude the VAD with coil embolization. Because of the hypoplasia of the contra-lateral VA, the trapping in the dissected segment cannot be considered for preservation of blood flow of the posterior circulation. Therefore, we planned performance of stent-assisted coiling, followed by use of a stent-within-a stent technique. Because the patient was in the acute stage of SAH, no pretreatment antiplatelet therapy was administered.
A 6F guiding catheter (Envoy; Cordis, Miami Lakes, FL) was placed at the cervical portion of the left VA (V2 segment). We placed a microcatheter (Prowler Select Plus, Cordis, Miami Lakes, FL) into the distal portion of the dissected segment for stenting, and another microcatheter (Excelsior SL-10, Boston Scientific, Natick, MA) was placed into the dissected segment of the VA for occlusion of the segment. Following insertion of four ultrasoft helical Guglielmi detachable coils (total length 41 cm) into the dissected segment, a 4.5 × 28 mm Enterprise stent was then placed through the dissected segment. A second 4.5 × 28 mm Enterprise stent was inserted using a stent-within-a-stent technique in order to support the fragile dissected vessel wall and to a induce flow diversion effect. Findings on postembolization angiography revealed complete occlusion of the segment of extravasation and preservation of the blood flow on the lesion side (
Fig. 2B). Postoperatively, loading doses of aspirin (200 mg) and clopidogrel (300 mg) were given to the patient via a nasogastric tube, and the dual anti-platelets (aspirin 100 mg and clopidogrel 75 mg per day) were maintained from 24 hours after treatment. Due to his poor condition, the patient died on five days after treatment.
Case 2
A 46-year-old female patient with a 10-year history of allergic rhinitis was admitted to our hospital with complaints of severe left leg pain and weakness. She had been treated intermittently with an inhalation corticosteroid for the allergic rhinitis. Lumbar magnetic resonance imaging (MRI) revealed a herniated lumbar disc between L4 and L5.
Laboratory tests on admission showed the following results: leukocytosis (18,600 per µl) (normal range 3,900-9,700 per µl), increased eosinophil count (9,411 per µl, 50.6%) (normal range 50-500 per µl, 0-10%), increased ESR (72 mm/h) (normal range 0-10 mm/h), and slightly increased CRP (1.81 mg/dl) (normal range 0.02-0.8 mg/dl). Other examinations revealed elevated ECP (> 200 µg/L) (normal range 0-13.5 µg/L) and a negative result for the parasite test. Following intravenous injection of 125 mg methylprednisolone for treatment of hypereosinophilia, operation for lumbar ruptured disc was performed. Postoperatively, the patient showed improvement of leg pain on the visual analogue scale (VAS) score for leg pain, from 7 to 2.
On day two after lumbar disc surgery, the patient exhibited sudden onset of headache, left side weakness and drowsy mental status. Findings on brain CT revealed ICH of approximately 60 cc in the right basal ganglia (
Fig. 2A).
At that time, laboratory examinations showed leukocytosis (12,000 per µl) (normal range 3,900-9,700 per µl). MPO-ANCA levels were elevated at 8.0 AI (positive ≥ 1.0 AI) and negative results were obtained for anti-nuclear, anti-extractable nuclear, and anti-ds DNA. Sputum differential cell count showed hypereosinophilia (22%). The electrocardiogram was normal.
Based on her clinical and laboratory findings of peripheral blood hypereosinophilia, paranasal sinus abnormality, eosinophilia in the sputum (22%), and MPO-ANCA (+), a diagnosis of CSS was made. A DSA immediately showed no abnormal vascular structure. Because of the patient's neurological deterioration and a large amount of ICH, emergency stereotactic burr-hole aspiration using a navigation system (OASSTS, Medtronic, CO, USA) was performed. On day three after surgery, follow-up CT scan revealed reduction of the ICH (
Fig. 2B), and on day four after surgery, the catheter was removed from the patient. At 16 days after treatment, the patient was transferred to the Department of Rehabilitation with a Glasgow Outcome Scale (GOS) score of 3.
During more than one year of follow up, the patient was treated first with oral prednisolone, and then changed to deflazacort. Follow up blood tests showed a normal peripheral eosinophil count (100 per µl, 1.4%) (normal range 50-500 per µl). The patient's neurological status was much improved, so as to be able to walk with support and the patient's GOS score improved to 4.