Welcome to the WSC 2022 Interactive Program

Background and Aims

Moyamoya disease is progressive vascular occlusive disease that affects distal carotids and proximal segments of major intracranial vessels, it can results in cerebral ischemia or hemorrhage, the suggested underlying mechanisms likely related to genetic linking, along with other environmental factors, it represents 5.8% as cause of ischemic stroke in young age among our population, it was associated with an underlying hematologic abnormalities like protein C deficiency and protein S deficiency, other hematological diseases like sickle cell disease (SCD) and sickle cell-beta-thalassemia (S beta-thalassemia).

We aim to study symptomatology, stroke subtypes, clinical outcomes and vascular imaging findings.

Methods

At single tertiary center, we analysed case records of 8 patients with adult-onset Moyamoya disease; we were looking at suggested mechanism, symptomatology, stroke subtypes, clinical outcomes and vascular imaging findings.

Results

Eight patients were included (62.5% women, mean age of 31.4 years), all patients had ischemic events (TIA or ischemic stroke) except for one patient who had haemorrhagic stroke. The median Suzuki classification mean was 3, majority of patients had good functional status mRS ≤ 1 (62,5% had mRS of ≤ 1), the median NIHSS score was 4.

Conclusions

In this case series we present Moyamoya disease in adult population with variable suggested pathophysiology, almost all the patients had ischemic events, with the majority of them had a good functional status, further research is needed for best management in adult onset Moyamoya disease

Background and Aims

Orbital ischemia syndrome is a very rare presentation of ocular ischemic syndrome. The cause is usually vascular pathology in the carotid artery, most often on the basis of atherosclerosis or, more rarely, dissection of the carotid artery. Our aim is to draw attention in the form of a case report to an unusual clinical manifestation of carotid artery dissection.

Methods

The case report deals with a patient (male, 49 years old) who suffered from intense pain behind the right eye. After a few days of pain, he arrives at the emergency room, where he was sent by a neurologist to the CTA. Preocclusive dissection of the right carotid artery was diagnosed. Neurosonological examination shows a massive reverse flow in the right ophthalmic artery. The pain behind the eye was so intense in the following days that opiate therapy was necessary.

Results

Carotid dissection was treated with anticoagulant therapy and a statin. According to the control CTA, the dissection heals at three-month intervals. Neurosonological control also shows normalization of flow in the right ophthalmic artery. The decrease in residual pain intensity even on analgesic therapy correlated with a decrease in the amplitude of retrograde flow in the ophthalmic artery.

Conclusions

Painful presentation of ocular ischemic syndrome, moreover caused by carotid artery dissection, is very rare.. However, if intense periorbital pain is present, carotid artery CTA should always be performed as part of the diagnostic process.

Background and Aims

Moyamoya disease is a rare case that often occurs in East Asian countries, including Japan and Korea but rarely being reported from other regions, including Indonesia. Moyamoya disease is characterized by irregular network of blood vessels at the base of brain known as moyamoya vessels which in Japanese means " like a puff of smoke that floats in the air.”

Methods

First case, 37-years-old-man, East Asian ethnic, had recurrent ischemic stroke, seizures and cognitive impairment. Head CT found extensive infarction in the right insular lobe and right frontal lobe. Second case, 35-years-old-woman, had ischemic stroke and cognitive impairment. There was infarction in the right parietal lobe on head CT. Angiography on both cases showed steno-occlusion of terminal portion of bilateral internal carotid artery with 'smoke of puff' neovascularization. First case showed third stage of suzuki for the right side and fourth stage of suzuki for the left side. Second case showed second stage of suzuki for the both sides.

Results

Both cases are treated by aspirin for stroke prevention, physiotherapy and recommended to do encephalodurosynangiosis surgery.

Conclusions

Manifestations of moyamoya disease arise due to cerebral ischemia related to steno-occlusion of cerebral blood vessels. Impaired cognitive function is a manifestation that often occurs in moyamoya disease even in patients without stroke. Conservative management and revascularization primarily directed at maintaining cerebral blood flow and preventing further stroke.

Background and Aims

Moyamoya disease (MMD) is a chronic cerebrovascular disorder characterized by progressive narrowing of the intracranial portions of the distal internal carotid artery (ICA), the initial proximal components of the middle cerebral artery (MCA) and the anterior cerebral artery (ACA). MMD can also manifest as a stroke in young adult.

Methods

Case report presents a patient with MMD in Siloam Hospital Denpasar, Bali, Indonesia, diagnosed using MRI and Digital Substraction Angiography (DSA).

Results

A 29-year-old woman presented with right side hemiparesis, urinary incontinence, disinhibition syndrome, hypertension, and history of left hemiparesis. Brain MRI showed linear hyperintensity throughout the sulci (ivy sign) in bilateral frontoparietal with acute infarct in left frontal region, chronic infarct in right frontal, occlusion of bilateral carotid arteries (supraclinoid segment), bilateral MCA, and bilateral ACA with collateral (puff of smoke appearence). Cerebral DSA study showed the stenosis in right ICA and stenosis in M1-M2 junction with puff of smoke appereance. The patient had underwent current best medical management: direct bypass in right MCA and right superficial temporal artery with single oral antiplatelet.

Moyamoya disease is a rare chronic cerebrovascular disorder that can manifest into a stroke especially in young adult. This patient had a recurrent ischaemic stroke due to Moyamoya disease. The patient had also gotten direct bypass surgery from right superficial temporal artery to MCA and given single oral antiplatelet as a prevention for ischaemic stroke.

Conclusions

Moyamoya disease is a rare disease needed futher studies for its treatment in order to halt the progresion and to prevent secondary complication.

Background and Aims

Primary Central Nervous System Vasculitis (PCNSV) is a rare inflammatory condition which severely affects small and medium blood vessel walls of the brain, spinal cord, and meninges. There are no specific diagnostic biomarkers and the definitive diagnosis requires invasive brain biopsy. We aim to identify differentially expressed proteins in CSF, brain tissues and blood samples using mass spectrometry and flow cytometry analysis.

Methods

We plan to recruit 60 patients from the ongoing PCNSV registry at All India Institute of Medical Sciences, New Delhi and 84 controls. PCNSV patients are classified into three subgroup (20 samples in each) which includes biopsy proven, angiographically proven and MRI suggestive of PCNSV. Age and gender matched controls have four subgroup including patients undergoing spinal anaesthesia from Orthopaedics (40), Intracranial atherosclerosis (10), Secondary Vasculitis and Focal arteriopathy (5 each) and 24 autopsy samples from Forensic Medicine. Brain biopsy, CSF and blood samples will be assessed using mass spectrometry and flow cytometry and results will be validated by ELISA and Western Blotting.

Results

We have recruited 23 PCNSV patients and 74 controls till date. The proteomic analysis will help to identify novel expression of proteins which in turn can lead to better understanding of pathobiology of the disease.

Conclusions

There is no class I evidence in treatment of PCNSV and current management is based on expert opinion. Strong evidence for pathophysiological mechanisms should prompt newer drugs with biological plausibility to be tested in randomized controlled trials.

Funding: Indian Council of Medical Research (ICMR), Government of India

Background and Aims

A definite diagnosis of Primary Central Nervous System Vasculitis (PCNSV) requires brain biopsy which is difficult in most settings around the world. There are no specific diagnostic biomarkers. Hence many genetic vasculopathies and autoinflammatory conditions can mimic PCNSV. Advances in exome sequencing are enabling identification of various pathogenic mutations underlying many systemic vasculitides. We aim to identify whether there is a significant subgroup of genetic vasculopathy among a group of Probable PCNSV patients using whole-exome sequencing.

Methods

We plan to recruit 60 patients of probable PCNSV who do not have a brain biopsy for definitive diagnosis. Isolated DNA from blood samples of PCNSV patients will be used for whole-exome sequencing analysis. The sequencing data will be analysed to identify genetic variants. Segregation analysis using trio samples will be conducted for further analysis of variants detected during initial testing.

Results

We have recruited 28 participants satisfying inclusion criteria of Probable PCNSV. The expected major outcome is the proportion of patients with definitive molecular diagnoses of a genetic vasculopathy in a cohort of Probable Primary CNS Vasculitis. The secondary exploratory plan is to analyze whole exome sequencing data regarding novel biological pathways (including complement system) in patients with PCNSV.

Conclusions

Whole exome sequencing in a cohort of Probable Primary CNS Vasculitis may identify genetic vasculopathies and genetic autoinflammatory conditions mimicking PCNSV and can further represent an important step forward towards a better understanding of genetic background underlying the predisposition and progression of PCNSV.

Funding: All India Institute of Medical Sciences (AIIMS) Intramural Research Grant, India

Background and Aims

Central nervous system infections associated with vasculitis can cause acute ischemic stroke in rare cases. Fungal meningitis has been infrequently associated with ischemic stroke. We are here reporting one such case where, an immunocompromised patient presented with cryptococcal meningitis along with bilateral cerebellar acute ischemic stroke, which possibly occurred due to cryptococcal infection associated vasculitis.

Methods

A 53-year male, known case of Hepatitis-C associated chronic liver disease presented in clinic with headache and imbalance while walking for last 1 month. The headache was continuous, very severe, holocranial, and, associated with photophobia, nausea, and vomiting. After 15 days of fever, patient also developed an imbalance while walking and a change in voice. The patient had no relief with analgesics and hence got admitted. On neurological examination, the patient had mild neck rigidity, dysarthric speech, mild appendicular cerebellar signs, and broad-based gait with abnormal tandem gait.

Results

On investigations, the MRI brain showed bilateral anterolateral cerebellar lacunar infarcts without any meningeal enhancement. In view of severe headache, cerebrospinal fluid analysis was done, which showed India ink positive cryptococcal organism with detection of cryptococcal antigen. The patient was started on liposomal amphotericin-B along with fluconazole for 28-days. The patient showed good recovery, and his headache and neurological signs improved.

Conclusions

Cryptococcal vasculitis-related strokes are now recognized as an important cause of infection-related ischemic stroke besides tubercular vasculitis. A possible mechanism may be due to direct invasion of arterial walls leading to arteritis, venous outflow obstruction, and accelerated atherosclerosis through induction of cytokines leading to ischemic stroke.

Background and Aims

GCA is an immune mediated disease that affects medium and large arteries more typically presented in older people. It is classically presented as headache, blurred vision and jaw claudication. Stroke is an atypical initial presentation of GCA in old patients. Posterior circulation territory strokes, specifically thalamic strokes, are exceedingly rare in a stroke syndrome secondary to GCA. Thalamic stroke involving posterior choroidal artery infarction can present with visual disturbances, hemisensory loss, transcortical aphasia, memory disturbances, ataxia, rubral tremor, dystonia, and myoclonus.

We report the first case of GCA presenting with a history of thalamic stroke in a young patient.

Methods

An 18-year-old Hispanic male patient with a recent thalamic stroke who with a history of insomnia, headache episodes, otalgia with tinnitus, blurred vision exacerbated by physical exercise, left hemiparesis, and dysesthesias.

Physical examination showed bradilalia, bradypsychia, tangential language, thalamic aphasia with jargon aphasia, objective pain in temporal arteries, decreased visual acuity, hyperreflexia, bilateral Hoffman’s and Marinescu without Romberg's sign.

Bloodwork showed elevated CRP and vitamin B12 deficiency. MRI showed ischemic vascular microangiopathy in both lateral geniculate bodies and pulvinar nuclei of the thalamus. Doppler ultrasound of the temporal arteries showed a bilateral positive halo sign.

Results

The patient received treatment with IV methylprednisolone, oral prednisone, topiramate, amitriptyline, and carbamazepine with good clinical improvement.

Conclusions

To our knowledge this is the first case of GCA presenting with thalamic stroke, in a young patient. Thalamic stroke can be the initial presentation of GCA.

Background and Aims

The present study used computational fluid dynamics (CFD) to quantify hemodynamic parameters in patients with spontaneous vertebral artery dissection (sVAD) with vertebral artery hypoplasia (VAH) and to compare the differences between sVAD with VAH arteries and healthy vertebral arteries (VAs).

Methods

This study included 10 patients who had suffered ischemic stroke due to an sVAD of the hypoplastic VA. Three-dimensional (3D) models from CTA were created using Mimics and Geomagic Studio. ANSYS were used for perform numerical simulations. The hemodynamic parameters included pressure, velocity, time-averaged blood flow, time-averaged wall shear stress (TAWSS), oscillatory shear index (OSI), endothelial cell action potential (ECAP), and relative residence time (RRT). Analysis of data was performed using SPSS.

Results

Compared with healthy VAs, steno-occlusive sVAD with VAH arteries exhibited an increased velocity (p<0.0001) in the dissection area and a slower flow velocity in the nondissected area (p=0.033). Conversely, the contour map of velocity and pressure revealed aneurysmal dilatative sVAD with VAH manifesting decreased velocity and high pressure in the dissection area. Moreover, steno-occlusive sVAD with VAH arteries had a lower time-averaged blood flow (p<0.0001), lower TAWSS (p=0.005), higher ECAP (p=0.012) and longer RRT (p=0.017) than the contralateral VAs.

Conclusions

Our research reconstructed 3D models of sVAD with VAH arteries and revealed the hemodynamic characteristics of sVAD with VAH arteries. The results provide a good basis for further testing of sVAD hemodynamics and reveal that noninvasive hemodynamic assessment might be effective for predicting sVAD risk. More detailed hemodynamic conditions with different stages of sVAD are warranted in the future.