Febrile episode unmasking neuropsychiatric systemic lupus erythematosus with lytic lesions caused by secondary autoimmune myelofibrosis: Case report.

RATIONALE: Systemic lupus erythematosus (SLE) is characterized by numerous immunological abnormalities that lead to multiorgan involvement. Central and peripheral nervous system manifestations are present in 8% to 92% of the cases of SLE. Furthermore, there have been reported cases of secondary autoimmune myelofibrosis associated with SLE. PATIENT CONCERNS: We present the case of a 64-year-old female who was transferred from the Cardiology Department, where she was admitted for pericardial-pleural-peritoneal effusion after being discharged from another hospital following the resolution of a febrile episode. During hospitalization, she presented multiple oculomotor nerves palsies and weakness in the lower limbs. Serial cerebral magnetic resonance imaging (MRI) revealed extensive cerebral venous thrombosis. Nerve conduction studies showed sensory-motor axonal polyneuropathy. Thoracic MRI revealed a rare finding in patients with SLE - lytic lesions. DIAGNOSES: Extensive clinical, imaging, blood, and urine tests were performed. The patient exhibited pancytopenia, elevated inflammatory markers, hyperhomocysteinemia, mild hypoproteinemia, and severe proteinuria. The Hematology consultation ascertained that the peripheral blood smear and the bone marrow aspiration showed no alterations suggestive for a primary hematological disease and the thoracic vertebral-medullary MRI changes had a very low probability of representing osteolytic lesions in the context of plasma cells dyscrasia, but could not exclude their being result of a secondary autoimmune myelofibrosis. Immunology blood tests highlighted the presence of antinuclear antibodies and lupus anticoagulants. In this context, the Rheumatology consultation established the diagnosis of SLE with multiple complications. INTERVENTIONS: The patient received treatment with cyclophosphamide. OUTCOMES: The ocular motricity problems and the paraparesis showed improvement. However, 1 week later, the patient developed weakness, dyspnea, and right lower quadrant abdominal pain. The abdominal-pelvic computed tomography scan indicated an acute right retroperitoneal hematoma with active bleeding for which she underwent arterial embolization of the spinal lumbar arteries with optimal result, but she died a few days later. LESSONS: We chose to present this case in order to highlight the importance of interdisciplinarity in diagnosing and managing patients with SLE and multiorgan ailments, especially when faced with rare constellations of complications such as extensive cerebral venous thrombosis and osseous lytic lesions caused by secondary autoimmune myelofibrosis.

Internal Carotid Artery Dissection - A Case for Antithrombotic Therapy in the Era of (Minimally) Invasive Procedures.

Objectives: Carotid artery dissection represents a common cause of stroke among people aged 30-45. We present two clinical cases and a review of the literature concerning the management of internal carotid artery dissections (ICADs). Materials and methods: The two patients are a 54-year-old male and a 40-year-old female. The first patient presented to our Neurology Department for one-week-old intense occipital headache. His clinical examination revealed left-sided miosis and upper eyelid ptosis. He underwent cerebral-cervical computed tomography (CT) and computed tomography angiography (CTA) scans and the latter revealed hemodynamically significant narrowing of both ICAs (right C1-C5 and left C1-C2 segments). Transcranial Doppler ultrasonography and Doppler ultrasonography (DUS) of the cervical-cerebral arteries showed right ICA occlusion at its origin (dissection fold and intraluminal thrombosis). Cervical magnetic resonance imaging (MRI) and time-of-flight magnetic resonance angiography (MRA) revealed a semilunar-shaped T2-weighted hypersignal present in the walls of the C1-C5 segments of the right ICA and of the C1-C2 segments of the left ICA, with bilaterally reduced intraluminal flow (right more than left). These findings indicated the presence of bilateral ICA intramural hematomas caused by subacute bilateral ICAD. The second patient presented to our Neurology Department for recurrent episodes of headache and lateral cervical pain on both sides. She underwent transcranial DUS and DUS of the cervicalcerebral arteries. They revealed right ICAD fold in its upper cervical segments. The CTA scan of the supra-aortic trunks showed hemodynamically significant narrowing with subsequent diminished blood flow in the upper cervical segments of right ICA. The patient was diagnosed with right ICAD. Results:Both patients were treated using antiplatelet therapy for primary prevention of ischaemic events. Follow-up at seven months and at six months, respectively, by means of CTA of the supra-aortic trunks or MRA of the cervical region, revealed the restoration of arterial patency with subsequent normal blood flow in both cases. Conclusions: The long-term outcomes of ICADs should be kept in mind when assigning medical or endovascular management on a case-by-case basis. Antiplatelet or anticoagulant therapy is a safe and effective first-line strategy in such patients, especially in cases that do not warrant particular management.