Cavitating Lung Lesions with Encephalopathy in a Patient with Long Standing Sjogren's Syndrome: An Elusive Cause.

A known case of primary Sjogren's syndrome with chronic kidney disease presented with respiratory symptoms and subsequent altered sensorium. Chest imaging suggested cavitating lung lesions in both the lungs. Serum c-ANCA was positive without any upper respiratory tract involvement or active urinary sediments. Treatment with appropriate anti-microbials produced no improvement in the respiratory or neurological parameters. MRI brain revealed tell-tale signs of CNS vascultis. A diagnosis of GPA was made as per European Medical Agency algorithm and the patient was started on immune-suppressants to which there was dramatic response. Her respiratory and neurological parameters started recovering steadily. However her stay at hospital was complicated by severe hospital acquired pneumonia to which she succumbed. We review the intricacies of the case, discussing the diagnostic conundrum and treatment dilemma we faced, as well as the systematic approach employed to manage the patient.

Rare Post-Tonsillectomy Internal Carotid Artery Pseudoaneurysm: Management by Parent Artery Occlusion Using Detachable Balloons.

Iatrogenic cervical internal carotid artery pseudoaneurysm is a rare and potentially lethal complication following tonsillectomy. It can be complicated by thromboembolism, mass effect and eventually may rupture leading to death. Various endovascular treatment options are available for the management of these pseudoaneurysms, including coil embolization, detachable balloon occlusion, or stent graft placement. Parent artery occlusion using detachable balloons can be a therapeutic option in a subset of patients. However, evaluation of cross circulation with preprocedure balloon test occlusion is imperative in such cases.

Outcome of revascularization in moyamoya disease: Evaluation of a new angiographic scoring system.

BACKGROUND: Moyamoya disease (MMD) is a chronic progressive cerebrovascular occlusive disease affecting commonly the anterior circle of Willis. Matushima grade inadequately reflects the angiographic changes postrevascularization procedure. AIMS: To analyze the clinical and angiographic outcome of revascularization procedures (direct [ST-middle cerebral artery (MCA) anastomosis] and indirect [encephalo-duro-arterio-myo-synangiosis (EDAMS)]) in MMD and validate a new angiographic scoring system. MATERIALS AND METHODS: Retrospective study included symptomatic patients of MMD who underwent revascularization; both indirect and combined methods between January 2002 and April 2012. Follow-up angiography was done after at least 3 months. We devised a novel scoring system the "angiographic outcome score" (AOS) including reformation of distal MCA and anterior cerebral artery, regression of basal moyamoya vessels, leptomeningeal collaterals and overall perfusion. AOS was applied to the angiograms independently by a neuroradiologist and a neurosurgeon that were blinded toward its preoperative or postoperative status. RESULTS: Totally 33 patients underwent 36 EDAMS and 4 combined procedures (EDAMS + ST-MCA bypass). The mean follow-up was 20 months. None had recurrent transient ischemic attack or fresh infarct. Postoperative AOS was significantly higher than preoperative AOS. The Spearman rho showed positive correlation between Matushima grade and postoperative AOS. Significant regression of basal moyamoya vessels and increase in number of loci of transdural collaterals was seen. CONCLUSIONS: EDAMS is a simple yet effective method of revascularization in both pediatric as well as adult age groups. AOS is a simple, precise and easily reproducible scoring system, which reflects the favorable angiographic changes after revascularization.