Eosinophilic granulomatosis with polyangiitis (EGPA), formerly known as a Churg–Strauss syndrome [1], is a rare systemic small vessel vasculitis mainly affecting people aged 40–60 years without gender predominance [2, 3]. Asthma, eosinophilia, chronic paranasal sinusitis, nasal polyposis, non-fixed pulmonary infiltrates, and myocarditis are typical findings of EGPA [4]. Neurologic involvement is frequently observed and peripheral nervous system (PNS) is affected in 59–86 % of patients, mainly in the form of peripheral neuropathy [5]. On the contrary, central nervous system (CNS) involvement is described in less than 10 % of cases [5]. The most common CNS manifestations are cerebral infarction and diffuse encephalopathy, while cerebral hemorrhages have been seldom associated with EGPA [6] and only one case of spinal subarachnoid hemorrhage has been previously reported in the early 1980s [7]. In June of 2012, a 31-year-old pregnant Caucasian woman with a long-term history of asthma and rhinitis on the day of the delivery presented with high spiking fever (up to 39.5 C) and arthralgias, disappearing in a few hours. Further similar episodes were then observed in the subsequent months without other additional findings. In August 2012, painful paresthesias in left hand, right arm, and dorsal surface of right foot appeared, together with bilateral distal upper limbs weakness. Electromyography/ electroneurography (EMG/ENG) confirmed a severe sensory-motor axonal neuropathy, predominant in upper limbs, characterized by patchy pattern as in vasculitic mononeuritis multiplex. Laboratory investigations showed an increase of inflammatory markers (ESR 58 mm/h, CPR 6.9 mg/dl) and leukocytosis (21,000/ll) with eosinophilia (63 %). Antineutrophil cytoplasmic antibodies (ANCA) were positive both in IIF (p-ANCA pattern) and in ELISA (anti-MPO specificity). Because of headache and neck stiffness appearance, brain MRI was performed, displaying maxillary and sphenoid sinusitis without CNS pathological findings. After the exclusion of underlying infections, the patient was diagnosed with EGPA and methylprednisolone 1 mg/kg/ day i.v. was started. Despite corticosteroid treatment, headache and neck stiffness persisted, global left upper limb weakness worsened in particular arm and wrist extension, and brisk left supinator tendon reflex appeared. Lumbar puncture revealed hemorrhagic cerebrospinal fluid, with a protein value of 755 mg/dl, glucose value of 23 mg/ dl, and 205 cells (neutrophils and red blood cells). HumanCMV and HSV infections were excluded by real-time PCR on cerebrospinal fluid, as well as other infectious processes. Spinal MRI was performed showing cervical and dorsal subarachnoid bleeding with spinal cord compression and C6–C7 intramedullary hyperintensity on the left side (Figs. 1a, 2a). Intravenous steroid (methylprednisolone 1 mg/kg/day i.v. for 6 days) was administered, followed by oral prednisone (75 mg/day) with subsequent tapering until suspension at 12 weeks. L. Diamanti (&) G. Berzero P. Bini S. Ravaglia E. Marchioni Neurology Department, IRCCS National Neurological Institute C. Mondino, University of Pavia, via Mondino 2, 27100 Pavia, Italy e-mail: luca.diamanti@mondino.it