Extracranial ectasia and embolic infarcts in HIV: two case reports and a clinical decision-making algorithm.

HIV is known to increase the risk of both ischemic and hemorrhagic strokes. There are many postulated mechanisms for this elevated risk including an HIV-induced vasculopathy and/or coagulopathy, opportunistic infections, and cardioembolic etiologies, among others. Regarding vasculopathy, prior reports have described the various changes to the arterial vasculature that can occur in the setting of HIV, yet the appropriate workup and management of this condition remains poorly defined. Here we describe two cases of patients with HIV presenting with large vessel intracranial occlusions in the setting of ectatic extracranial vasculature accompanied by intraluminal thrombus formation. One patient underwent thrombectomy, while the other improved after receiving IV-tPA. Inferring on these cases and the existing literature, a standardized workup and treatment algorithm is proposed, emphasizing the key management decisions that should be considered on a case-by-case basis.

Reversible Cerebral Angiopathy after Viral Infection in a Pediatric Patient with Genetic Variant of RNF213.

Ring finger protein (RNF) 213 is known as a susceptibility gene for moyamoya disease (MMD), which is characterized by bilateral carotid folk stenosis. Cerebral angiopathy after viral infection has been known to present angiographical appearance resembling MMD, however its pathogenesis and genetic background are not well known. We report a case of reversible cerebral angiopathy after viral infection in a pediatric patient with genetic variant of RNF213 mutation. The patient had developed a severe headache after hand, foot, and mouth disease. Magnetic resonance imaging and magnetic resonance angiography (MRA) performed 2-3 weeks after disease onset revealed bilateral carotid folk stenosis and an old cerebral infarction in the left putamen. The patient's headache spontaneously resolved and the follow-up MRA showed a complete spontaneous resolution of the arterial stenosis after 9 months. We were able to determine genetic predisposition to angiopathy by identifying the RNF213 c.14576G>A (rs112735431, p.R4859K) mutation. Based on the present case, we hypothesize that an RNF213 variant might play an important role for the onset of postviral cerebral angiopathy.

History of parvovirus B19 infection is associated with silent cerebral infarcts.

BACKGROUND: The relationship between silent cerebral infarcts (SCIs) and history of parvovirus B19 (B19V) has not been systematically evaluated. As an ancillary study from the Silent Cerebral Infarct Trial (SIT) (NCT00072761), we tested the hypothesis that a history of B19V infection is associated with an increased prevalence of SCIs in children with sickle cell anemia. PROCEDURE: We used a retrospective cross-sectional cohort study design; each participant underwent a brain magnetic resonance imaging (MRI) scan and medical record review for prior B19V infection (n = 958). RESULTS: SCI was present in 30% (287 of 958) of participants and 17% (165 of 958) had a history of B19V infection. Based on prior evidence that low baseline hemoglobin (Hgb) levels are associated with increased odds of SCI, Hgb levels were divided into tertiles (<7.6 g/dl, ≥7.6-≤8.5 g/dl, ≥8.6 g/dl) and multivariable analysis was used to determine the relationship between the joint effect of prior B19V infection, Hgb levels, and SCI. Prior B19V infection and the lowest Hgb tertile were associated with increased risk of SCI (odds ratio [OR] 2.12; 95% CI, 1.17-3.84; P = 0.013); no prior B19V infection and the highest Hgb tertile were associated with a decreased risk (OR 0.56; 95% CI, 0.38-0.84; P = 0.004). CONCLUSIONS: Efforts to decrease the incidence of B19V infection, such as the development of a B19V vaccine, may decrease SCI prevalence.

Varicella-zoster virus vasculopathy. A review description of a new case with multifocal brain hemorrhage.

BACKGROUND: The varicella zoster virus (VZV) is a highly neurotropic virus that, after the primary infection, remains latent in the nerve cells and can reactivate many years later, resulting in various conditions affecting the central nervous system, such as vasculopathy and stroke. METHODS: We report on a review of the published literature that included all case reports identified via PubMed and an additional unpublished case of VZV vasculopathy. All epidemiological, clinical, laboratory, imaging, virologic, treatment and outcome data collected are described. RESULTS: Of the 62 patients, 41.6% were immunocompromised. Ischemic stroke occurred in 77.2% of the patients, comprising cases of isolated (37.1%) and multifocal stroke (17.7%). Multifocal, ischemic and hemorrhagic stroke was only described in the newly reported case. The magnetic resonance imaging results were normal in 2.9% of the cases. The vascular studies (angiography and magnetic resonance angiography [MRA]) revealed signs of angiitis in 74.4% of the cases; the small arteries were involved in 38.5% of the cases, large arteries in 17.7% and mixed in 43.5%. For 95.2% of the patients, the cerebrospinal fluid (CSF) was positive for VZV IgG antibodies, and for 46.1% of the patients, the CSF was positive for polymerase chain reaction (PCR); however, the diagnosis was confirmed in only 3 of 6 biopsies. DISCUSSION: VZV vasculopathy can occur in both immunocompetent and immunosuppressed patients. Neuroimaging can reveal stroke and angiitis, and the detection of VZV-specific IgG antibodies in the CSF is a reliable and highly sensitive diagnostic tool. The multifocal nature of VZV vasculopathy makes biopsy a test with low sensitivity and high morbidity.

Human immunodeficiency virus-associated cytomegalovirus infection with multiple small vessel cerebral infarcts in the setting of early immune reconstitution.

Cytomegalovirus (CMV) infection is an important cause of neurologic disease in the context of advanced human immunodeficiency virus (HIV) infection and is recognized as a cause of immune reconstitution inflammatory syndrome (IRIS) after initiation of highly active antiretroviral therapy (HAART). Central nervous system vasculitis secondary to CMV has only rarely been described in the context of HIV, despite the established ability of CMV to infect microvascular endothelial cells in the brain. However, we report a case that demonstrates the association between CMV and multiple small vessel cerebral infarct lesions after initiation of HAART.

Progressive multifocal leukoencephalopathy showing extensive spinal cord involvement in a patient with lymphocytopenia.

A 64-year-old Japanese man who was diagnosed as having cerebral infarcts at an early clinical stage was found to have progressive multifocal leukoencephalopathy (PML). A decrease of leukocytes and lymphocytes had been detected in the previous year. During a total clinical course of 11 months, he showed marked depletion of lymphocytes ranging from 264/microL to 459/microL. Autopsy disclosed no underlying diseases such as malignancies or tuberculosis. Extensive PML lesions were seen in the cerebral white matter. Small perivascular cuffs comprising many CD8+ T lymphocytes and a few CD4+ T cells were scattered in the PML lesions. CD20+ B cells were rarely evident. The subsets of the infiltrating lymphocytes differed from those of primary or spontaneous PML. Similar extensive PML lesions were observed not only in the cerebellum and brainstem but also in the spinal cord. All 26 segments of the spinal cord, especially the cervical, lumbar and sacral cord, showed extensive lesions involving the lateral and anterior columns. To our knowledge, only three cases of PML with such extensive spinal cord lesions have been reported previously. These three cases, and the present one, may represent a group of PML that shows extensive lesions in the spinal cord as well as the cerebrum, cerebellum and brainstem. The underlying disease in the present case was unclear. Because lymphocytopenia is not observed in primary or spontaneous PML, and the immunohistochemical findings of the infiltrating lymphocytes in the present case are different from primary or spontaneous PML, the decrease in his total blood lymphocytes may have played a significant role in his immunosuppressed condition as the underlying disease.