Mycophenolate mofetil as induction and maintenance immunosuppressive therapy in adult primary central nervous system vasculitis: A prospective observational study.

To observe the clinical and angiographic effectiveness of mycophenolate mofetil (MMF) as induction and maintenance immunosuppressive therapy in primary central nervous system vasculitis (PCNSV). In this open-label prospective study done at a tertiary care neurology centre, adult patients with PCNSV, diagnosed by Calabrese's criteria, were recruited from 2017 to 2021 and treated with glucocorticoids, MMF and standard of care. Patients were followed-up and clinical and angiographic changes were recorded. Total 26 patients were recruited with median age 39 years (34-49) with a slight female predilection (61.5%). Angiographic diagnoses were: small vessels disease 11.5%; large vessels disease 42.3% and both in 46.2%. Median duration of follow-up was 24.5 months (14.25-38). Proportion of patients with severe disability (modified Rankin Score (mRS) 4-6) at baseline was 73.08% (19/26) which reduced to 7.69% (2/26) (p < 0.001). At the last follow-up mRS = 0 was achieved in 38.5% (10/26) and mRS of ≤ 1 was achieved in 69.2% (18/26). Median time to achieve a mRS ≤ 1 was 12 months (95% CI: 6.8-17.2). Angiography was repeated in 16 patients after a median duration of 13 months (10.5-19.7), out of which 10 (62.5%) showed improvement and 5 (31.2%) showed non-progression of lesions. MMF may be an effective immunosuppressive therapy in adult PCNSV as both induction and maintenance. Serial DSA of brain may be useful to monitor the effect of treatment. Key Points • Mycophenolate mofetil is effective as induction and maintenance immunosuppressive therapy in PCNSV. • Repeat angiogram may be useful to monitor treatment response in PCNSV.

A case of central nervous system vasculitis secondary to rheumatoid arthritis treated successfully with mycophenolate.

Here, we report a case of central nervous system vasculitis in rheumatoid arthritis (RA) treated successfully with corticosteroid and mycophenolate. A 53-year-old female with poorly controlled RA presented with unilateral lower motor neurone type seventh cranial nerve palsy. Magnetic resonance imaging (MRI) of the brain revealed bilateral signal intensity changes in posterior fossa, basal ganglia, and periventricular white matter with contrast enhancement. Rheumatoid factor, anti-cyclic citrullinated peptides, and C-reactive protein were high. Cerebrospinal fluid analysis showed pleocytosis with elevated protein. Digital subtraction angiography of the brain revealed a beading pattern in distal branches of anterior, middle, and posterior cerebral arteries bilaterally suggestive of central nervous system vasculitis. We treated the patient with intravenous methylprednisolone followed by oral prednisolone tapered off in 6 months. Mycophenolate mofetil was started at the dose of 2 g per day, along with prednisolone. Sulfasalazine and hydroxychloroquine were also started to treat her arthritis. The patient showed complete neurological improvement, along with a significant resolution of the lesions in MRI.

REtrospective Multicenter INdian Study of Derivo Embolization Device (REMIND): Periprocedural Safety.

PURPOSE: The treatment of aneurysms with characteristics such as complex morphology, fusiform, blister-like, wide neck, or large size has been revolutionized with the introduction of flow diverters. Though flow diverters have several advantages over coiling, they also have certain important disadvantages such as the lack of immediate protection against rupture, the risk of ischemic stroke, the need for antiplatelet therapy, and long latency for complete effect. The Derivo Embolization Device (DED) is a second-generation self-expanding device that is claimed to be less thrombogenic than conventional devices. We retrospectively evaluated the periprocedural safety and risks associated with the DED across 5 centers in India. MATERIALS AND METHODS: This is a multicentric, retrospective, observational study of DED, conducted at 5 high volume endovascular therapy centers in India from May 2018 to June 2020. Periprocedural demographic, clinical, and angiographic data were collected from a retrospective review of patient charts. RESULTS: A total of 96 patients, including 56 (58.3%) females, aged between 16-80 years (60±12.7 years) harboring 106 aneurysms were studied. Seven (7.3%) were noted to harbor multiple aneurysms: 6 had 3 aneurysms each, while 1 patient had 5 aneurysms. The following aneurysm characteristics were noted: average size, 9.8±8.2 mm; average neck size, 6.9±8.5 mm; wide-necked (>4 mm), 63 (59.4%); giant (>25 mm), 8 (7.5%); and anterior circulation location, 98 (92.5%). Eighteen (17%) of these were ruptured. Additional balloon angioplasty was performed in 5 (5.2%) patients. Intraprocedural problems were encountered in 3 (3.1%), of which only 1 had clinical implications, the device fish-mouthing with stent thrombosis resulting in a malignant middle cerebral artery territory infarction. The modified Rankin scale at 3 months was worse in 1 patient. CONCLUSION: DED is a newer generation flow diverter stent with a low periprocedural complication rate.

Imaging features in Hirayama disease.

PURPOSE: To evaluate the MR findings in clinically suspected cases of Hirayama disease. MATERIALS AND METHODS: The pre and post contrast neutral and flexion position cervical MR images of eight patients of clinically suspected Hirayama disease were evaluated for the following findings: localized lower cervical cord atrophy, asymmetric cord flattening, abnormal cervical curvature, loss of attachment between the posterior dural sac and subjacent lamina, anterior shifting of the posterior wall of the cervical dural canal and enhancing epidural component with flow voids. The distribution of the above features in our patient population was noted and correlated with their clinical presentation and electromyography findings. OBSERVATIONS: Although lower cervical cord atrophy was noted in all eight cases of suspected Hirayama disease, the rest of the findings were variably distributed with asymmetric cord flattening, abnormal cervical curvature, anterior shifting of the posterior wall of the cervical dural canal and enhancing epidural component seen in six out of eight (75%) cases. An additional finding of thoracic extension of the enhancing epidural component was also noted in five out of eight cases. CONCLUSION: Dynamic post contrast MRI evaluation of cervicothoracic spine is an accurate method for the diagnosis of Hirayama disease.

Endovascular treatment of direct carotid cavernous fistulae: a pictorial review.

INTRODUCTION: Direct carotid-cavernous fistulae (CCFs) are type A fistulae according to Barrow's classification. Endovascular treatment of these lesions is challenging. METHODS: The purpose of this review was to evaluate the endovascular treatment of direct CCFs. We also describe the technique, symptomatology and complications associated with the procedure and report on the long-term follow-up in our treated patients. RESULTS: A total of 89 patients with a direct CCF were treated. All patients had baseline brain CT or MR imaging. Treatment comprised transarterial balloon or coil embolizations. The patients were followed up at 1 month and then every 6 months thereafter. Detachable balloons were used in 79 fistulae. In 12 fistulae the balloon could not be negotiated through the fistula and these fistulae were treated with transarterial coil occlusion. Clinical outcomes of the treated patients evaluated at 1 month were: 79 patients (88.8%) cured, 9 (10.1%) significantly improved, 1 (1.1%) remaining static. CONCLUSION: Endovascular treatment of direct CCFs is safe and effective and results in long-term cure.

Evaluation, management, and long-term follow up of vein of Galen malformations.

OBJECT: Vein of Galen malformations (VGMs) are extremely rare intracranial lesions. Clinical presentation and management strategies vary significantly in different areas of the world. The authors report their experience in evaluation, management, and long-term follow up of these lesions in India. METHODS: Between October 1983 and June 2003, 25 patients with VGMs were referred to the authors' institution for evaluation and management. Ten children younger than 2 years of age presented with rapidly increasing head size as the chief complaint. Among 11 children 2 years of age or older, the most common presenting symptom was chronic headache. Four patients who presented during adulthood had chronic headache for many years before presentation. Angiographic evaluation of the lesion was performed in 21 patients. Fifteen patients were treated using endovascular techniques. Injection of the embolic material was performed after induction of systemic hypotension when the flow in the fistula was high. Complete occlusion of the arteriovenous shunt could be achieved in two patients with vein of Galen aneurysmal dilation (100% of patients with this type of malformation) and in five of the six patients with the mural type of malformation (83%). Among patients with the choroidal type of malformation, complete obliteration of the shunt could be achieved in three patients. In three patients with high-flow choroidal malformations, embolization carried out in a single sitting resulted in shunt reduction of nearly 90%. These patients received clinical follow up. CONCLUSIONS: The authors' experience in evaluation and management of VGMs reveals that in areas of the world where access to dedicated specialist care is limited, the clinical presentation of VGMs can differ appreciably from the classic descriptions in the literature. Endovascular management of these lesions results in excellent angiographic and clinical results.

Suction thrombectomy of thrombotic occlusion of the subclavian artery in a case of Takayasu's arteritis.

Takayasu's arteritis, also known as pulseless disease, is a chronic inflammatory arteritis affecting large vessels, predominantly the aorta and its main branches. Vessel inflammation leads to wall thickening, fibrosis, stenosis, and thrombus formation. Percutaneous removal of arterial thrombus with the use of several devices has been reported, with mixed results. We present a case of Takayasu's arteritis with thrombotic occlusion of the subclavian artery in which pulsed urokinase injection and suction thrombectomy were used to revascularize a threatened limb and to establish the sole arterial supply to the brain.

Bilateral cerebral venous angioma associated with varices: a case report and review of the literature.

A varix occurring with a cerebral venous angioma is a rare combination of cerebral vascular malformations. The most common vascular anomaly associated with a venous angioma is a cavernous angioma. We report a case of bilateral supratentorial venous angioma with 2 varices involving the angioma situated on the right frontotemporal region. Multiple subependymal veins were seen in the right supratentorial region, which joined to form multiple collector veins, which, in turn, unified into a tortuous channel in the region of the sylvian fissure and continued into venous varices.

Proatlantal intersegmental arteries of external carotid artery origin associated with Galen's vein malformation.

Carotid basilar anastomoses can occasionally persist beyond the embryonic period. These anomalies are most often incidentally detected in adulthood, during workups for unrelated pathologies. Persistence of the proatlantal intersegmental arteries is a rare form of primitive carotid-basilar anastomoses. Bilateral proatlantal inter- segmental arteries are an extremely rare occurrence, of which only 3 cases have been reported in the literature. An analysis of vascular anomalies associated with Galen's vein malformations revealed 3 children in whom persistence of type II proatlantal arteries was seen. These included one child in whom proatlantal arteries were persistent bilaterally. We report the clinical and angiographic findings and discuss the embryologic and therapeutic implications of this unique association.