Primary angiitis of the central nervous system in a patient with transient episodes of headache and aphasia: A case report.

Primary angiitis of the central nervous system (PACNS) refers to a rare form of vasculitis of unknown cause, with a challenging diagnostic work-up. We report the case of a 57-year-old patient who presented with transient episodes of headache and global aphasia. Cerebrospinal fluid (CSF) examination revealed lymphocytic pleocytosis with moderate elevated protein and normal glucose. CSF and serum tests for infections and autoimmune/paraneoplastic antibodies were negative, except CSF polymerase chain reaction testing that detected Epstein-Barr virus (EBV). Magnetic resonance imaging of the brain with intravenous gadolinium showed meningeal enhancement and pachymeningitis. Due to continuous relapsing episodes of aphasia, a leptomeningeal and brain tissue biopsy was performed and revealed lesions of granulomatous necrotising vasculitis of medium-sized leptomeningeal and intracranial vessels, as well as negative in situ hybridism for EBV. A diagnosis of primary granulomatous necrotising angiitis of the central nervous system was made, and the patient was treated with intravenous methylprednisolone and oral cyclophosphamide, showing excellent response to treatment. Diversity in clinical and laboratory features makes it difficult for PACNS to be distinguished by other systemic vasculitides. Laboratory tests and neuroimaging can provide guidance in evaluation of the patients and exclude other possible causes, but tissue biopsy remains the gold standard for a definite diagnosis.

Headache and status epilepticus in the postpartum period; posterior reversible encephalopathy syndrome or cerebral venous thrombosis?

We report a case of a young woman, with a history of a miscarriage and a molar pregnancy, who developed headache and status epilepticus in postpartum day three. Posterior reversible encephalopathy syndrome (PRES) and cerebral venous and sinus thrombosis (CVST) can present with identical clinical picture; however, the imaging findings can help the clinician to make the correct diagnosis and initiate the appropriate treatment. Both PRES and CVST are medical emergencies and fully reversible entities especially when treatment initiation is immediate.

Thymoma and myasthenia gravis: clinical aspects and prognosis.

Myasthenia gravis is present in a significant proportion of patients with thymoma. We investigated particular features of the clinical behavior of thymoma and its relationship to myasthenia in a retrospective study of 79 patients who underwent thymectomy for thymoma during the last 20 years. The presence of myasthenia gravis, Masaoka stage, World Health Organization histotype, myasthenia response, and survival were analyzed. The mean age of the patients was 56.1 ± 12.4 years, and 39 had myasthenia gravis. A significantly higher proportion of patients with myasthenia was found in B2 and B3 histotypes compared to A, AB, and B1. Among myasthenic patients, 33.3% had no response, 50% had a partial response, and 16.7% achieved complete remission. During the follow-up period, 16 (21.1%) patients died. Mean survival was 4.8 ± 1.4 years for patients with no myasthenia response, whereas those with a partial or complete myasthenia response had significantly better survival.

Reply to "The role of soluble interleukin-6 receptor in inflammatory diseases" [Immunol Lett 2005;98(1):171].

We agree with the comments of Mr. Maggio and colleagues. The exact role of serum IL-6R and the relation between this and the IL-6/sIL-6R complex in inflammatory disorders has not been completely clarified. We suggest a discrepancy between sIL-6R concentrations and measurable IL-6/sIL-6R complex, not excluding the positive contribution of sIL-6R as a marker of neuro-immunoregulatory and inflammatory status in the central nervous system.