Factors Associated with Moyamoya Syndrome in a Kentucky Regional Population.

OBJECTIVES: Our study aimed to report both new and previously identified conditions associated with moyamoya syndrome in a Western population and to present our outcomes after surgical treatment with indirect bypass. METHODS: We performed a retrospective chart review of patients evaluated at our institution from June 2011 to June 2015 who were diagnosed with moyamoya. Data collected include patient demographics, presenting manifestations, vessels involved, comorbid conditions, abnormal laboratory values, treatments administered, and clinical outcomes. RESULTS: Thirty-one patients with moyamoya were enrolled (11 male and 20 female), with 84% Caucasian and 16% African-American. The most common comorbidity was hypertension in 61% of the patients. Coexisting autoimmune conditions were present in 26%, with another 13% having coexisting prothrombotic disorders. Diabetes mellitus was not found to correlate with the Suzuki grade of disease at presentation (P = .30). When noninvasive imaging was performed before the cerebral angiogram, the computed tomography angiography had a false-negative rate of 59%, and magnetic resonance angiography had a false-negative rate of 33%. Twenty-one patients underwent surgical intervention, 2 underwent intracranial stenting, and 19 underwent indirect bypass with encephaloduroarteriosynangiosis. At an average 28-month follow-up, all 15 patients who had an angiogram after intervention showed evidence of neovascularization. CONCLUSIONS: Autoimmune and prothrombotic disorders were found to be comorbid in patients with moyamoya at much higher rates than expected in the general population. Diabetes mellitus was not significantly correlated with Suzuki grade. Angiogram remains an important diagnostic modality when noninvasive imaging is negative for vasculopathy. We demonstrate excellent evidence of revascularization within 1 year with intracranial stenting and indirect bypass.

The Kentucky Appalachian Stroke Registry (KApSR).

BACKGROUND: The population of rural Kentucky and West Virginia has a disproportionately high incidence of stroke and stroke risk factors. The Kentucky Appalachian Stroke Registry (KApSR) is a novel registry of stroke patients developed to collect demographic and clinical data in real time from these patients' electronic health records. OBJECTIVE: We describe the development of this novel registry and test it for ability to provide the information necessary to identify care gaps and direct clinical management. METHODS: The KApSR was developed as described in this article. To assess utility in patient care, we developed a "Diabetes Quality Assurance Dashboard" by cross-referencing patients in the registry with a diagnosis of ischemic cerebrovascular disease with patients that were tested for hemoglobin A1c (HbA1c) levels, patients with HbA1c levels diagnostic for diabetes mellitus (DM), and patients with an elevated HbA1c that were formally diagnosed with DM. RESULTS: For the 1008 patients treated for ischemic cerebrovascular disease in the year studied, 859 (85%) had their HbA1c tested. Of those, 281 had levels of 6.5 or greater, although only 261 (93%) were discharged with a formal diagnosis of DM. CONCLUSIONS: The KApSR has practical value as a tool to assess a large population of patients quickly for care quality and for research purposes.

Perlecan Domain-V Enhances Neurogenic Brain Repair After Stroke in Mice.

The extracellular matrix fragment perlecan domain V is neuroprotective and functionally restorative following experimental stroke. As neurogenesis is an important component of chronic post-stroke repair, and previous studies have implicated perlecan in developmental neurogenesis, we hypothesized that domain V could have a broad therapeutic window by enhancing neurogenesis after stroke. We demonstrated that domain V is chronically increased in the brains of human stroke patients, suggesting that it is present during post-stroke neurogenic periods. Furthermore, perlecan deficient mice had significantly less neuroblast precursor cells after experimental stroke. Seven-day delayed domain V administration enhanced neurogenesis and restored peri-infarct excitatory synaptic drive to neocortical layer 2/3 pyramidal neurons after experimental stroke. Domain V's effects were inhibited by blockade of α2ß1 integrin, suggesting the importance of α2ß1 integrin to neurogenesis and domain V neurogenic effects. Our results demonstrate that perlecan plays a previously unrecognized role in post-stroke neurogenesis and that delayed DV administration after experimental stroke enhances neurogenesis and improves recovery in an α2ß1 integrin-mediated fashion. We conclude that domain V is a clinically relevant neuroprotective and neuroreparative novel stroke therapy with a broad therapeutic window.

Successful Extracorporeal Membrane Oxygenation (ECMO) Use without Systemic Anticoagulation for Acute Respiratory Distress Syndrome in a Patient with Aneurysmal Subarachnoid Hemorrhage.

Extracorporeal membrane oxygenation (ECMO) is an important life-saving technology for patients with severe acute respiratory distress syndrome (ARDS). Unfortunately, ECMO has been traditionally contraindicated in patients with hemorrhagic neurologic diseases. The recent improvement in ECMO devices, increased utilization and experience with venovenous ECMO technologies among healthcare teams, and the use of ECMO without anticoagulation has expanded the potential populations that may benefit from ECMO. We present a case of successful utilization of venovenous ECMO for severe respiratory failure secondary to ARDS in a patient with aneurysmal subarachnoid hemorrhage and severe, episodic cerebral vasospasm. We also discuss important limitations and considerations for future successful use of ECMO in hemorrhagic stroke. This case report highlights the potential for this life-saving technology in patients with hemorrhagic stroke.

Novel Temporary Treatment for a Severe Case of Syndrome of Trephined.

BACKGROUND: Syndrome of the trephined is a unique neurosurgical condition that is seen in patients that have undergone craniectomy. While the symptoms of the condition range from mild to severe, the only definitive treatment for the condition is replacement of the bone flap. This article presents a novel, temporary treatment for syndrome of the trephined in a patient with severe symptoms who was unable to undergo immediate cranioplasty due to infection. CASE DESCRIPTION: A 25-year-old gentleman with a history of trauma resulting in hydrocephalus, craniectomy, and eventually ventriculoperitoneal shunt placement presented with a cranial wound infection requiring removal of his bone flap. While being treated with antibiotics, with his bone flap removed, he developed severe syndrome of the trephined. An emergency bedside procedure was developed and executed to treat his condition. CONCLUSIONS: Treating syndrome of the trephined with an external suction device proved useful and lifesaving fort the patient presented. Such a device can be made with common supplies found within any hospital. The technique used to treat the patient is novel and may be useful for others to consider if ever faced with a similar situation.

Urotensin II modulates rapid eye movement sleep through activation of brainstem cholinergic neurons.

Urotensin II (UII) is a cyclic neuropeptide with strong vasoconstrictive activity in the peripheral vasculature. UII receptor mRNA is also expressed in the CNS, in particular in cholinergic neurons located in the mesopontine tegmental area, including the pedunculopontine tegmental (PPT) and lateral dorsal tegmental nuclei. This distribution suggests that the UII system is involved in functions regulated by acetylcholine, such as the sleep-wake cycle. Here, we tested the hypothesis that UII influences cholinergic PPT neuron activity and alters rapid eye movement (REM) sleep patterns in rats. Local administration of UII into the PPT nucleus increases REM sleep without inducing changes in the cortical blood flow. Intracerebroventricular injection of UII enhances both REM sleep and wakefulness and reduces slow-wave sleep 2. Intracerebroventricular, but not local, administration of UII increases cortical blood flow. Moreover, whole-cell recordings from rat-brain slices show that UII selectively excites cholinergic PPT neurons via an inward current and membrane depolarization that were accompanied by membrane conductance decreases. This effect does not depend on action potential generation or fast synaptic transmission because it persisted in the presence of TTX and antagonists of ionotropic glutamate, GABA, and glycine receptors. Collectively, these results suggest that UII plays a role in the regulation of REM sleep independently of its cerebrovascular actions by directly activating cholinergic brainstem neurons.

Urotensin II acts as a modulator of mesopontine cholinergic neurons.

Urotensin II (UII) is a vasomodulatory peptide that was not predicted to elicit CNS activity. However, because we have recently shown that the urotensin II receptor (UII-R) is selectively expressed in rat mesopontine cholinergic (MPCh) neurons, we hypothesize that UII may have a central function. The present study demonstrates that the UII system is able to modulate MPCh neuron activity. Brain slice experiments demonstrate that UII excites MPCh neurons of the mouse laterodorsal tegmentum (LDTg) by activating a slow inward current. Furthermore, microinfusion of UII into the ventral tegmental area produces a sustained increase in dopamine efflux in the nucleus accumbens, as measured by in vivo chronoamperometry. In agreement with UII activation of MPCh neurons, intracerebroventricular injections of UII significantly modulate ambulatory movements in both rats and mice but do not significantly affect startle habituation or prepulse inhibition. The present study establishes that UII is a neuromodulator that may be exploited to target disorders involving MPCh dysfunction.