A rare case of ANCA-associated cerebral vasculitis
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CE - MEDICAL ILLUSTRATION
A rare case of ANCA‑associated cerebral vasculitis Maria Grazia Massaro1 · Rossella Cianci1 · Emilio Lozupone2 · Pietro Trombatore2 · Giovanni Gambassi1 Received: 22 May 2020 / Accepted: 4 September 2020 © Società Italiana di Medicina Interna (SIMI) 2020
A 39-year old professional rugby player was admitted to hospital for the acute onset of neurological symptoms. Two years earlier, due to the emergence of generalized tonic–clonic seizure linked to multiple focal cerebral lesions deemed of uncertain significance at a brain MRI, the patient had been prescribed anti-seizure medications and corticosteroids. Few weeks prior to the current admission, corticosteroids were spontaneously discontinued. The patient did not report any other relevant medical conditions and denied any other symptom. The family history was silent and he did not drink alcohol or make use of illicit drugs. The patient’s clinical history began 2 years earlier when he underwent a brain MRI because of the onset of a generalized seizure. MRI documented the presence of multiple focal alterations in the right hemisphere with a larger one in the frontal lobe. The lesions were characterized by poor enhancement and peri-wound edema (Fig. 1(1a–f)). At that time, the patient completed a screening for neurotropic viruses and other infectious diseases with CSF culture and blood culture for fungi, bacteria and parasites which resulted negative. A total body CT scan, a transthoracic echocardiogram, an ocular fundus exam and a dermatological evaluation were similarly normal. Because of those inconclusive diagnostic exams, a biopsy of the right frontal lesion was obtained by a craniotomy approach. The pathological examination documented the presence of some macrophages and lymphocytes, edema and reactive gliosis, but with no evidence of necrosis, nor granulomatous or vasculitis patterns.
* Maria Grazia Massaro [email protected] 1
Department of Internal Medicine, Fondazione Policlinico Universitario A.Gemelli IRCCS, Catholic University of the Sacred Heart School of Medicine, Largo Agostino Gemelli 8, 00168 Rome, Italy
Department of Diagnostic Imaging, Oncological Radiotherapy and Hematology, Fondazione Policlinico Universitario A.Gemelli IRCCS, Catholic University of the Sacred Heart School of Medicine, Largo Agostino Gemelli 8, 00168 Rome, Italy
2
An anti-edema treatment with dexamethasone (4 mg bid) was initiated and a prophylactic anti-seizure medication was recommended (levetiracetam, 1250 mg bid). During the subsequent 2 years, the patient did remain completely asymptomatic until the recurrence of the generalized tonic–clonic seizure of the current admission. During the hospital stay, the patient underwent both a brain CT and MRI (Fig. 1 (2a-f)). The neuroimaging scans were compared with those completed two years earlier. The previously documented brain lesions had been totally replaced by multiple deposits of material with strong paramagnetic effect but without signs of blood–brain barrier leakage, and were conside
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