Vast hyperintense lesion in T2, FLAIR and diffusion-weight Camptothecin images, involving the right temporo-occipital lobes. Muscle biopsy was normal and genetic test to detect MELAS mtDNA mutations was unremarkable. Molecular screening (direct sequence analysis and multiplex ligation-dependent probe amplification) of DNA polymerase subunit gamma-1 (POLG1) was normal. Treatment with topiramate 200 mg/day was started and she remained asymptomatic over 2 years. Thereafter, topiramate had to be discontinued due to side effects and carbamazepine up to 800 mg/day was given. Several brain MRI performed 6, 12 and 24 months later were normal. Afterwards, she was admitted for blurred vision and frequent short-lasting sensations that "eyes move like windscreen wiper". EEG (Figure 2) disclosed recurrent occipital seizures every 3?0 minutes clinically characterized by horizontal nystagmus towards the left. Neurological examination revealed left lower homonymous quadrantanopia. SE stopped after i.v. bolus of diazepam 10 mg. Neither skin lesions nor involvement of joints were evident. Brain MRI showed a well-defined right temporo-occipital lesion (Figure 1G) without contrast enhancement. The same haematological screening as patients 1 and 2, revealed ANA 1:320 (speckled pattern as detected by indirect fluorescenceFigure 2 EEG showing recruiting, fast "tonic" activity over right posterior head regions, predominant over right occipital lead (O2), followed by sharp theta activity over ipsilateral temporo-occipital leads. Seizure lasts about 75 seconds (bipolar montage, scalp electrodes placed according to the International 10?0 system). The trace is compressed to show seizure in its all duration.Ferlazzo et al. BMC Neurology 2014, 14:148 http://www.biomedcentral.com/1471-2377/14/Page 4 ofantibody test) and positivity to p-ANCA (as detected by both indirect immunofluorescence and ELISA for both ANCA-MPO and ANCA-PR3). Moreover, C reactive protein and the erythrocyte sedimentation rate were elevated. Tests for coeliac disease, syphilis, Lyme disease, anti-glutamate receptor (2 and 3) antibodies and paraneoplastic antibodies were negative. CSF analysis, including cultures, was normal. Urinalyses, including microalbuminuria, were normal. Electroneuromyography, electrocardiography, chest X-ray, abdominal and pelvic ultrasound were unremarkable. She was given carbamazepine 800 mg/day. In the following 14 months, two similar episodes of occipital SE associated with transitory MRI abnormalities recurred and resolved with appropriate antiepileptic therapy. After the last SE, brain MRI revealed right temporo-parieto-occipital atrophy (Figures 1H, I). Due to the persistence of positive p-ANCA and ANA, she was given carbamazepine 800 mg/day, oral prednisone 50 mg/ day for one month, followed by azathioprine 75 mg/day, remaining seizure-free during 30-month follow-up. The right temporo-parietal-occipital atrophy remained unchanged in control MRI.Conclusions In our series, SE represented the main or first neurological manifestation. Although the presence of a CNS vasculitis was not confirmed by brain biopsy or angiography, positivity to p-ANCA prompted us to suspect an autoimmune aetiology and to begin PubMed ID:https://www.ncbi.nlm.nih.gov/pubmed/17139194 immunotherapy. Moreover, the proposed diagnostic criteria of AAV are based on systemic symptoms and signs, serological, radiological or histological findings, exclusion of other conditions as malignancy, infections, drugs, sarcoidosis, secondary vasculitis [8]. Patient #1 fulfille.