Isolated cortical vasogenic edema and hyperintense vessel signs may be early features of reversible cerebral vasoconstriction syndrome: Case reports.

Background The temporal and anatomical features of vasoconstriction in patients with reversible cerebral vasoconstriction syndrome within hours after symptom onset, in the hyperacute phase, are unclear. Case result Herein we report the cases of two patients with acute severe headache who were diagnosed with reversible cerebral vasoconstriction syndrome. Magnetic resonance imaging within hours after symptom onset revealed multiple areas of isolated cortical vasogenic edema and hyperintense vessel signs of the distal cerebral arteries. Follow-up imaging performed four days later in both cases showed diffuse segmental arterial vasoconstriction in the proximal regions of the cerebral arteries. Both patients received antivasoconstrictive therapy shortly after admission, and neither had neurological sequelae at discharge. The magnetic resonance imaging findings improved gradually within three months after symptom onset. Conclusion Isolated cortical vasogenic edema and hyperintense vessel signs, when observed within hours from sudden severe headache onset, may be useful early markers of reversible cerebral vasoconstriction syndrome.

De Novo Vertebral Artery Dissecting Aneurysm after Parent Artery Occlusion of the Contralateral Vertebral Artery.

After treatment of unilateral vertebral artery dissecting aneurysm (VADA), de novo VADA rarely occurs on the contralateral side. In this article, we report a case of subarachnoid hemorrhage (SAH) due to de novo VADA in the contralateral vertebral artery (VA) 3 years after parent artery occlusion of unilateral VADA, with a review of the literature. A 47-year-old woman was admitted to our hospital complaining of headache and impaired consciousness. Head computed tomography showed SAH, and three-dimensional computed tomography angiography showed a fusiform aneurysm in the left VA. We performed an emergency parent artery occlusion. Three years and 3 months after the initial treatment, the patient presented to our hospital with complaints of headache and neck pain. Magnetic resonance imaging revealed SAH, and magnetic resonance angiography revealed de novo VADA in the right VA. We performed a stent-assisted coil embolization. The patient had a good postoperative course and was discharged with a modified Rankin scale score of 0. Long-term follow-up is necessary in patients with VADA because contralateral de novo VADA can develop even several years after the initial treatment.

Secondary parkinsonism caused by chronic subdural hematomas owing to compressed cortex and a disturbed cortico-basal ganglia-thalamocortical circuit: illustrative case.

BACKGROUND: Chronic subdural hematoma (CSDH) is a commonly encountered condition in neurological and neurosurgical practice, but the presence of concomitant parkinsonism is extremely rare. Basal ganglia disturbance is a well-known underlying mechanism; however, few cases present with cerebral cortex compression as the cause of symptoms. OBSERVATIONS: A 52-year-old man was referred to the authors' hospital with a 5-week history of gait disturbance and suspected Parkinson's disease. Neurological examination revealed a mask-like face, stooped posture, left-predominant rigidity, and postural instability. The authors initiated dopamine agonist administration, and brain magnetic resonance imaging (MRI) was scheduled. One week later, MRI showed bilateral CSDHs. The hematomas markedly compressed the bilateral cerebral cortex, whereas the midbrain and basal ganglia structures were intact. The patient underwent burr hole drainage and was discharged after 9 days without sequelae. LESSONS: CSDH can cause parkinsonism by compressing the cerebral cortex, which is a part of the cortico-basal ganglia-thalamocortical circuit. Surgery leads to positive outcomes, as illustrated by this case, in which cerebral cortex compression caused parkinsonism.

Abnormalities of brain imaging in patients after left ventricular assist device support following explantation.

BACKGROUND: The potential impact of long-term left ventricular assist device (LVAD) support on the brain remains unclear. This study aimed to investigate cerebral microvascular damage in patients after long-term LVAD implantation using magnetic resonance imaging (MRI). METHODS: We reviewed the medical records of patients after continuous-flow LVAD implantation in our hospital from 2006 to 2016, who underwent brain MRI after LVAD explantation for either transplantation or recovery. Age- and sex-matched healthy controls and patients with chronic heart failure (CHF) were collected from our pooled MRI database. The presence of cerebral microbleeds (CMBs) and cortical superficial siderosis and the severity of white matter hyperintensity (WMH) and cerebral atrophy were compared between patients with prior LVAD and 2 control groups. RESULTS: This study included 49 patients with prior LVAD, 49 healthy controls, and 45 patients with CHF. CMBs and cortical superficial siderosis were detected in 98% (p < 0.001) and 31% (p < 0.001) of patients with prior LVAD, respectively. The number of CMBs was higher in patients with prior LVAD than in the 2 control groups. The severity of the WMH was higher in patients with prior LVAD than in healthy controls but similar to that in patients with CHF. Quantitative analyses of cerebral atrophy revealed a significantly higher bicaudate ratio and cella media index in patients with prior LVAD than in the 2 control groups. CONCLUSIONS: Patients after long-term LVAD support showed a higher prevalence of CMBs and cortical superficial siderosis and more severe cerebral atrophy than did controls. These findings may indicate cerebral microvascular damages in long-term LVAD support patients.

Asymptomatic Pontine Lesion and Diabetic Amyotrophy after Rapid Improvement of Poor Glycemic Control in a Patient with Type 1 Diabetes.

We herein report a 28-year-old woman with type 1 diabetes with an asymptomatic pontine lesion and diabetic amyotrophy. She had suffered from diabetes from 10 years old. Treatment in a hospital reduced the hemoglobin A1c level from 14.2% to 7.2% for approximately 2 months. She suffered from acute-onset pain and weakness of the lower limb muscles without central nervous system manifestations. Magnetic resonance imaging showed high-intensity lesions at the brainstem and lower limb muscles on T2-weighted images. These findings and symptoms gradually resolved. Rapid treatment of poor glycemic control might increase the risk of asymptomatic pontine lesions and diabetic amyotrophy.

Complement and inflammasome overactivation mediates paroxysmal nocturnal hemoglobinuria with autoinflammation.

Patients with paroxysmal nocturnal hemoglobinuria (PNH) have a clonal population of blood cells deficient in glycosylphosphatidylinositol-anchored (GPI-anchored) proteins, resulting from a mutation in the X-linked gene PIGA. Here we report on a set of patients in whom PNH results instead from biallelic mutation of PIGT on chromosome 20. These PIGT-PNH patients have clinically typical PNH, but they have in addition prominent autoinflammatory features, including recurrent attacks of aseptic meningitis. In all these patients we find a germ-line point mutation in one PIGT allele, whereas the other PIGT allele is removed by somatic deletion of a 20q region comprising maternally imprinted genes implicated in myeloproliferative syndromes. Unlike in PIGA-PNH cells, GPI is synthesized in PIGT-PNH cells and, since its attachment to proteins is blocked, free GPI is expressed on the cell surface. From studies of patients' leukocytes and of PIGT-KO THP-1 cells we show that, through increased IL-1ß secretion, activation of the lectin pathway of complement and generation of C5b-9 complexes, free GPI is the agent of autoinflammation. Eculizumab treatment abrogates not only intravascular hemolysis, but also autoinflammation. Thus, PIGT-PNH differs from PIGA-PNH both in the mechanism of clonal expansion and in clinical manifestations.

Prevalence of Cerebral Microbleeds in Patients With Continuous-Flow Left Ventricular Assist Devices.

BACKGROUND: The prevalence of cerebral microbleeds (CMBs) in gradient echo T2*-weighted brain MRI has a positive correlation with hemorrhagic stroke incidence. However, the prevalence of CMBs in patients with left ventricular assist devices (LVADs) has not been evaluated. We evaluated the prevalence of CMBs and the relationship with hemorrhagic stroke incidence in patients with LVADs. METHOD AND RESULTS: We analyzed results from brain MRI in prospective examinations of 35 consecutive patients who had undergone LVAD explantation for heart transplantation or recovery since 2011. The number and distribution of CMBs were counted, then the relationship between baseline characteristics and adverse events during LVAD support were analyzed. The mean age was 37.7±12.4 years and the mean LVAD duration was 2.43±1.08 years. Thirty-four (97%) patients had at least one CMB. Nine (26%) developed hemorrhagic stroke during LVAD support, and patients with hemorrhagic stroke had a significantly greater number of CMBs compared with patients without hemorrhagic stroke (5 [interquartile range (IQR), 4-7] versus 9 [IQR, 5-23]; odds ratio 1.14 [95% Confidence Interval (CI), 1.02-1.32], P=0.05). There was no significant relationship between age, LVAD support duration, or systolic blood pressure during LVAD. However, patients who had at least one episode of bacteremia (9 [IQR, 4-16] versus 5 [IQR, 3-7], P=0.06) and pump pocket infection (14 [IQR, 4-27] versus 5 [IQR, 3-7], P=0.08) showed a trend toward a greater number of CMBs than patients without bacteremia. CONCLUSIONS: Thirty-four (97%) patients with continuous-flow LVAD had at least one CMB, and the number of CMBs were more prevalent in patients with hemorrhagic stroke and in patients with LVAD-related infection.