Clinical and radiological spectrum of anti-myelin oligodendrocyte glycoprotein (MOG) antibody encephalitis: single-center observational study.

OBJECTIVE: The objective was to describe the clinical presentations, radiologic features, and outcomes of patients with autoimmune encephalitis associated with myelin oligodendrocyte glycoprotein antibody (MOG). BACKGROUND: During the past decade, the spectrum of the myelin oligodendrocyte glycoprotein antibody-associated diseases (MOGAD) has expanded. Recently, patients with MOG antibody encephalitis (MOG-E) who do not fulfill the criteria for ADEM have been reported. In this study, we aimed to describe the spectrum of MOG-E. METHODS: Sixty-four patients with MOGAD were screened for encephalitis-like presentation. We collected the clinical, radiological, laboratory, and outcome data of the patients who presented with encephalitis and compared it with the non-encephalitis group. RESULTS: We identified sixteen patients (nine males and seven females) with MOG-E. The median age of the encephalitis population was significantly lower than the non-encephalitis group (14.5 years (11.75-18) vs. 28 years (19.75-42), p = 0.0004). Twelve out of sixteen patients (75%) had fever at the time of encephalitis. Headache and seizure were present in 9/16 (56.2%) and 7/16 (43.75%) patients, respectively. FLAIR cortical hyperintensity was present in 10/16 (62.5%) patients. Supratentorial deep gray nuclei were involved in 10/16 (62.5%) patients. Three patients had tumefactive demyelination, and one patient had a leukodystrophy-like lesion. Twelve of 16 (75%) patients had a good clinical outcome. Patient with leukodystrophy pattern and other with generalized CNS atrophy showed a chronic progressive course. CONCLUSION: MOG-E can have heterogeneous radiological presentations. FLAIR cortical hyperintensity, tumefactive demyelination, and leukodystrophy-like presentations are novel radiological presentations associated with MOGAD. Though majority of MOG-E have a good clinical outcome, few patients can have chronic progressive disease even on immunosuppressive therapy.

Pediatric intracranial aneurysms--our experience and review of literature.

PURPOSE: Intracranial aneurysms in children are not as common as in adults and there are many differences in the etiology, demographic variables, aneurysm location, aneurysm morphological characteristics, clinical presentation, and outcome in pediatric and adult intracranial aneurysms. METHODS: All children (≤18 years) suffering from intracranial aneurysm managed at our center from July 2001 through June 2013 were included in the study, and the details of these patients were retrieved from the computerized database of our hospital. OBSERVATIONS: A total of 62 pediatric patients were treated for 74 aneurysms during the study period and constituted 2.3% of all intracranial aneurysms treated during the same period. The mean age at presentation was 13.5 years. Headache (82%) was the commonest presenting feature; other symptoms included seizures (21%), ictal loss of consciousness (27%), and motor/cranial nerve deficits (22.6%). Computed tomogram revealed subarachnoid hemorrhage in 58% of patients. Eighty-two percent of aneurysms were in anterior circulation. Sixty-seven percent of aneurysms were complex aneurysms. Fifty-eight percent of patients underwent surgical intervention while 30% underwent endovascular procedures. Twenty-one percent of the patients developed vasospasm. There was no postoperative mortality. Favorable outcome was seen in 72% of the patients. CONCLUSIONS: Pediatric intracranial aneurysms are uncommon as compared to in adult patients. Seizures and cranial nerve involvement are seen more often as the presenting features in children. Posterior circulation aneurysms are more common in children, as are the internal carotid artery bifurcation aneurysms. There is high incidence of giant, posttraumatic, and mycotic aneurysms in children.

Imaging Feature of Leukoencephalopathy with Calcifications and Cysts (Labrune Syndrome).

Labrune syndrome is an uncommon central nervous system disorder characterized by leukoencephalopathy, cerebral calcifications, and cysts on brain imaging. The basic pathology is microangiopathy resulting from a mutation in the SNORD118 gene. Radiological imaging is the hallmark of the disease.

Isolated ipsilateral abducens nerve palsy and contralateral homonymous hemianopsia associated with unruptured posterior cerebral artery aneurysm: A rare neurological finding.

Cranial nerve palsies can be presenting signs of intracranial aneurysms. There is a classic pairing between an aneurysmal vessel and adjacent nerves leading to cranial neuropathy. Isolated abducens nerve palsy can be a localizing sign of an unruptured vertebrobasilar circulation aneurysm. Aneurysms involving Anterior Inferior Cerebellar Artery (AICA) and Posterior Inferior Cerebellar Artery (PICA) have been reported to be associated with abducens nerve palsy. The symptoms in unruptured aneurysms are due to the mass effect on adjacent neurovascular structures. Most of the abducens nerve palsy resolves following microsurgical clipping. Here, we present a rare case of an unruptured Posterior Cerebral Artery (PCA) aneurysm presenting with abducens nerve palsy and diplopia associated with contralateral hemianopsia which markedly improved following endovascular coil embolization.

Duplication of right vertebral artery in Klippel-Feil anomaly.

The duplicated origin of the vertebral artery (VA) is an uncommon anatomical variant, which is generally identified incidentally during angiography and can be misdiagnosed as dissection in the setting of posterior circulation stroke. Here, we describe a case of the right V1 VA duplication with embryological aspects in a patient with Klippel-Feil anomaly, which was diagnosed during preoperative evaluation. Surgeons must be aware to avoid vascular injury from a duplicated VA before head-neck and spinal surgery.

Radiological Features and Management of Intracranial Aneurysms Associated With Moyamoya Disease: A Case Series of Single-Center Experience.

Background Moyamoya disease (MMD) can be a major cause of hemorrhagic stroke. Though extensive angiographic studies have been undertaken, the understanding of the association between aneurysms and MMD remains unanswered. In this study, we explore the association of the aneurysm with MMD and its management. We have also reviewed such associations described in the literature and how the present cases differ from those previously described. Materials and methods The clinical and radiologic data of moyamoya disease cases were accessed from medical and radiological records between January 2010 and July 2017. Two neuroradiologists independently analyzed the data and imaging details. Results Out of 103 patients with MMD, eight patients (7.77%) had associated intracranial aneurysms with eleven aneurysms. Out of the 11 aneurysms, five were the tip of the basilar artery aneurysms and were the most common location for aneurysm (45.5%), followed by lenticulostriate artery, PCA perforator, and distal ACA (DACA) in the P1 PCA, P2 PCA, and P3 PCA artery aneurysms. Out of eight patients, five (62.5%) had a hemorrhage on a non-contrast computed tomography (NCCT) scan of the brain, whereas three (37.5%) had an ischemic presentation. Out of 11 aneurysms, seven aneurysms, including three basilar tip aneurysms (unruptured) and one PCA perforator (ruptured), and three saccular PCA (P1, P2, and P3) (ruptured) were treated by endovascular coiling. Follow-up angiography showed stable aneurysmal occlusion except in one basilar tip, where recurrence was observed. Conclusions MMD-intracranial aneurysm is commonly observed in patients with intracranial hemorrhage and carries a higher risk of rupture. Therefore, identification of the aneurysm is essential for management. Endovascular treatment, either with coil or glue embolization, can be a safe and effective treatment method for such aneurysms with long-term good results.

Emerging insights into cephalic neural crest disorders: A single center experience.

Objectives: Neural crest cells (NCCs) are transient structures in the fetal life in vertebrates, which develop at the junctional site of the non-neural and neural ectoderm, sharing a common developmental origin for diverse diseases. After Epithelio-mesenchymal (EMT) of the NCCs within the neural tube, delamination of NCCs occurs. After delamination, the transformation of these cells into various cell lineages produces melanocytes, bones, and cartilage of the skull, cells of the enteric and peripheral nervous system. After the conversion, these cells migrate into various locations of the entire body according to the cell lineage. Abnormalities in neural crest (NC) formation and migration result in various malformations and tumors, known as neurocristopathy. Material and Methods: Herein, this case series describes a single-center experience in cephalic NC disorders over the past 3 years, including 17 cases of varying composition (i.e., vascular, dysgenetic, mixed, and neoplastic forms) involving the brain and occasionally skin, eyes, and face of the patients. Results: In our study of 17 patients with cephalic NC disease, 6 (35.3%) patients had vascular form, 5 (29.4%) had dysgenetic form, 4 (23.5%) had mixed form, and 2 (11.7%) had neoplastic form. Brain involvement in the form of vascular or parenchyma or both vascular and parenchymal was seen in all of our patients (100%), skin in 6 (35.3%) patients, eye in 2 (11.7%), and face in 1 (5.9%) patient. Treatment was planned according to the various manifestations of the disease. Conclusion: Neural crest diseases (NCDs) are a rare and under-recognized group of disorders in the literature and may have been under-reported due to a lack of awareness regarding the same. More such reporting may increase the repertoire of these rare disorders such that clinicians can have a high degree of suspicion leading to early detection and timely counseling and also improve preventive strategies and help in developing new drugs for these disorders or prevent them.

RE-OPEN: Randomised trial of biosimilar TNK versus TPA during endovascular therapy for acute ischaemic stroke due to large vessel occlusions.

Rationale: Rapid and timely treatment with intravenous thrombolysis and endovascular treatment (EVT) in patients with acute ischaemic stroke (AIS) and large vessel occlusion (LVO) significantly improves patient outcomes. Bridging therapy is the current standard of care in these patients. However, an incompletely answered question is whether one thrombolytic agent is better than another during bridging therapy. Aim: The current study aims to understand if one thrombolytic agent is superior to the other during bridging therapy in the treatment of AIS and LVO. Sample size estimates: Using 80% power and an alpha error of 5 %, presuming a 10% drop out rate, a total of 372 patients will be recruited for the study. Methods and design: This study is a prospective, randomised, multicentre, open-label trial with blinded outcome analysis design. Study outcomes: The primary outcomes include proportion of patients who will be independent at 3 months (modified Rankin score (mRS) ≤2 as good outcome) and proportion of patients who achieve recanalisation modified thrombolysis in cerebral infarction grade 2b/3 at first angiography run at the end of EVT. Secondary outcomes include proportion of patients with early neurological improvement, rate of symptomatic intracerebral haemorrhage (ICH), rate of any ICH, rate of any systemic major or minor bleeding and duration of hospital stay. Safety outcomes include any intracranial bleeding or symptomatic ICH. Discussion: This trial is envisioned to confirm the theoretical advantages and increase the strength and quality of evidence for use of tenecteplase (TNK) in practice. Also, it will help to generate data on the efficacy and safety of biosimilar TNK. Trial registration number: CTRI/2022/01/039473.

The role of susceptibility-weighted imaging & contrast-enhanced MRI in the diagnosis of primary CNS vasculitis: a large case series.

Primary CNS Vasculitis (PCNSV) is a rare, diverse, and polymorphic CNS blood vessel inflammatory condition. Due to its rarity, clinical variability, heterogeneous imaging results, and lack of definitive laboratory markers, PCNSV diagnosis is challenging. This retrospective cohort analysis identified patients with histological diagnosis of PCNSV. Demographic data, clinical presentation, neuroimaging studies, and histopathologic findings were recorded. We enrolled 56 patients with a positive biopsy of CNS vasculitis. Most patients had cerebral hemisphere or brainstem symptoms. Most brain MRI lesions were bilateral, diffuse discrete to confluent white matter lesions. Frontal lobe lesions predominated, followed by inferior cerebellar lesions. Susceptibility-weighted imaging (SWI) hemorrhages in 96.4% (54/56) of patients, either solitary microhemorrhages or a combination of micro and macrohemorrhages. Contrast-enhanced T1-WIs revealed parenchymal enhancement in 96.3% (52/54 patients). The most prevalent pattern of enhancement observed was dot-linear (87%), followed by nodular (61.1%), perivascular (25.9%), and patchy (16.7%). Venulitis was found in 19 of 20 individuals in cerebral DSA. Hemorrhages in SWI and dot-linear enhancement pattern should be incorporated as MINOR diagnostic criteria to diagnose PCNSV accurately within an appropriate clinical context. Microhemorrhages in SWI and venulitis in DSA, should be regarded as a potential marker for PCNSV.

Measurement of Neutrophil Gelatinase-Associated Lipocalin (Ngal) Following Neuroradiological Procedure/s in Patients with Aneurysmal Subarachnoid Hemorrhage: A Preliminary Study.

Background: Radiocontrast administration during interventional neuroradiology (INR) procedures for aneurysmal subarachnoid haemorrhage (aSAH) can add to renal insult. Serum creatinine (sCr) is a conventional marker of acute kidney injury (AKI). Serum neutrophil gelatinase-associated lipocalin (sNGAL) is a novel marker which is increasingly used to predict renal injury in susceptible patients. Objectives: The primary aim of this study was to evaluate correlation between serum neutrophil gelatinase-associated lipocalin (NGAL) and sCr in aSAH patients undergoing therapeutic or diagnostic INR procedures. The secondary aim was to find the incidence of contrast-induced AKI and hemodynamic complications during the study period. Material and Methods: All consenting aSAH patients (18-60 years, Modified Hunt and Hess grade 1-4) posted for INR procedures during the study time were included. Patients with history of chronic renal disease, recent contrast exposure, or renal insufficiency were excluded. Blood samples for sCr and sNGAL were obtained preprocedure and then at 1, 6, 24, and 48 h after contrast administration. Hourly urine output was noted. AKI was defined by KDIGO guidelines. Statistical Analysis Used: Repeated measurement analysis of variance, Posthoc Bonferroni test and Pearson correlation coefficient test. Results: Fifty patients, mean age 47.34 ± 9.31 years, were enrolled for the study. Majority (48; 96%) were Hunt and Hess (H and H) grade I-III. The mean volume of contrast administered was 123.2 ± 53.08 mL. The mean sNGAL and sCr values at pre-op, 1, 6, 24, and 48 h were 124.99 ± 64.58, 148.40 ± 77.90, 147.33 ± 76.00, 125.49 ± 64.44, and 116.38 ± 61.79 ng/mL and 0.629 ± 0.23, 0.624 ± 0.22, 0.612 ± 0.21, 0.632 ± 0.19, and 0.577 ± 0.22 mg/dL, respectively. There was a correlation in sCr and sNGAL value (P < 0.001) at all study time points. However, no specific pattern was seen. No patient developed any AKI or hemodynamic complications in first 48 h. Conclusions: There is a correlation between serum NGAL and sCr at individual time points. NGAL may represent a sensitive early biomarker of renal impairment after INR Procedures. There was no incidence of AKI after contrast administration in aSAH patients without predisposing renal risk factors.

Post-Vaccination/Post-COVID Immune-Mediated Demyelination of the Brain and Spinal Cord: A Novel Neuroimaging Finding.

Introduction: Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) causes coronavirus disease 2019 (COVID-19), which not only produces respiratory symptoms but is known to involve almost every system, and its neuroinvasive properties have been well demonstrated throughout the pandemic. Also, to combat the pandemic, there was rapid development and induction of various vaccination drives, following which many adverse events following immunization (AEFIs) have been reported, which include neurological complications as well. Method: We present a series of three cases, post vaccination, with and without a history of COVID illness that showed remarkably similar findings on magnetic resonance imaging (MRI). Result: A 38-year-old male presented with complaints of weakness of the bilateral lower limbs with sensory loss and bladder disturbance a day after receiving his first dose of ChadOx1 nCoV-19 (COVISHIELD) vaccine. A 50-year-old male with hypothyroidism characterized by autoimmune thyroiditis and impaired glucose tolerance experienced difficulty in walking 11.5 weeks after being administered with COVID vaccine (COVAXIN). A 38-year-old male presented with subacute onset progressive symmetric quadriparesis 2 months after their first dose of a COVID vaccine. The patient also had sensory ataxia, and his vibration sensation was impaired below C7. All three patients had typical pattern of involvement of the brain and spine on MRI with signal changes in bilateral corticospinal tracts, trigeminal tracts in the brain, and both lateral and posterior columns in the spine. Conclusion: This pattern of brain and spine involvement on MRI is a novel finding and is likely a result of post-vaccination/post-COVID immune-mediated demyelination.

Multimodal imaging of the aging brain: Baseline findings of the LoCARPoN study.

We quantified and investigated multimodal brain MRI measures in the LoCARPoN Study due to lack of normative data among Indians. A total of 401 participants (aged 50-88 years) without stroke or dementia completed MRI investigation. We assessed 31 brain measures in total using four brain MRI modalities, including macrostructural (global & lobar volumes, white matter hyperintensities [WMHs]), microstructural (global and tract-specific white matter fractional anisotropy [WM-FA] and mean diffusivity [MD]) and perfusion measures (global and lobar cerebral blood flow [CBF]). The absolute brain volumes of males were significantly larger than those of females, but such differences were relatively small (<1.2% of intracranial volume). With increasing age, lower macrostructural brain volumes, lower WM-FA, greater WMHs, higher WM-MD were found (P = 0.00018, Bonferroni threshold). Perfusion measures did not show significant differences with increasing age. Hippocampal volume showed the greatest association with age, with a reduction of approximately 0.48%/year. This preliminary study augments and provides insight into multimodal brain measures during the nascent stages of aging among the Indian population (South Asian ethnicity). Our findings establish the groundwork for future hypothetical testing studies.

Cortico-limbic disruption, material-specificity, and deficits in cognitive-affective theory of mind.

The Theory of Mind deficit due to cognitive-affective disintegration is a poorly understood cognitive consequence of cortical and subcortical disruption in right temporal lobe epilepsy. Following Marr's trilevel approach, we used the material-specific processing model to understand the Theory of Mind deficit in drug-resistant epilepsy (N = 30). We examined pre- and post-surgery changes in first-order (somatic-affective, non-verbal component) and second-order Theory of Mind (cognitive-verbal component) in three groups formed using: (i) seizure side (right versus left), (ii) right temporal epilepsy (right temporal lobe epilepsy versus non-right temporal lobe epilepsy), and (iii) right temporal lobe epilepsy with amygdalohippocampectomy (right temporal lobe epilepsy versus left temporal lobe epilepsy amygdalohippocampectomy versus non-amygdalohippocampectomy). We observed a marked deficit in the first-order Theory of Mind in the right temporal lobe amygdalohippocampectomy group; we mapped this deficit to decline in the non-verbal component of Theory of Mind (somatic-affective component). Preliminary results support using a material-specific processing model to understand the Theory of Mind deficits in right temporal lobe epilepsy amygdalohippocampectomy. Malleability of verbal processing in presence of deterioration of non-verbal processing might have clinical relevance for post-surgery recovery in right temporal lobe epilepsy amygdalohippocampectomy. Documenting the material-specific nature of deficits (verbal versus non-verbal) in non-western, linguistically, and socioeconomically diverse country enables us to understand the problem of heterogeneity in post-surgery cognitive consequences in the right amygdalohippocampectomy.

World Federation for Interventional Stroke Treatment (WIST) multispecialty training guidelines for endovascular stroke intervention.

Introduction: Today, endovascular treatment (EVT) is the therapy of choice for strokes due to acute large vessel occlusion, irrespective of prior thrombolysis. This necessitates fast, coordinated multi-specialty collaboration. Currently, in most countries, the number of physicians and centres with expertise in EVT is limited. Thus, only a small proportion of eligible patients receive this potentially life-saving therapy, often after significant delays. Hence, there is an unmet need to train a sufficient number of physicians and centres in acute stroke intervention in order to allow widespread and timely access to EVT. Aim: To provide multi-specialty training guidelines for competency, accreditation and certification of centres and physicians in EVT for acute large vessel occlusion strokes. Material and methods: The World Federation for Interventional Stroke Treatment (WIST) consists of experts in the field of endovascular stroke treatment. This interdisciplinary working group developed competency - rather than time-based - guidelines for operator training, taking into consideration trainees' previous skillsets and experience. Existing training concepts from mostly single specialty organizations were analysed and incorporated. Results: The WIST establishes an individualized approach to acquiring clinical knowledge and procedural skills to meet the competency requirements for certification of interventionalists of various disciplines and stroke centres in EVT. WIST guidelines encourage acquisition of skills using innovative training methods such as structured supervised high-fidelity simulation and procedural performance on human perfused cadaveric models. Conclusions: WIST multispecialty guidelines outline competency and quality standards for physicians and centres to perform safe and effective EVT. The role of quality control and quality assurance is highlighted.

World Federation for Interventional Stroke Treatment (WIST) Multispecialty Training Guidelines for Endovascular Stroke Intervention.

INTRODUCTION: Today, endovascular treatment (EVT) is the therapy of choice for strokes due to acute large vessel occlusion, irrespective of prior thrombolysis. This necessitates fast, coordinated multi-specialty collaboration. Currently, in most countries, the number of physicians and centres with expertise in EVT is limited. Thus, only a small proportion of eligible patients receive this potentially life-saving therapy, often after significant delays. Hence, there is an unmet need to train a sufficient number of physicians and centres in acute stroke intervention in order to allow widespread and timely access to EVT. AIM: To provide multi-specialty training guidelines for competency, accreditation and certification of centres and physicians in EVT for acute large vessel occlusion strokes. MATERIAL AND METHODS: The World Federation for Interventional Stroke Treatment (WIST) consists of experts in the field of endovascular stroke treatment. This interdisciplinary working group developed competency - rather than time-based - guidelines for operator training, taking into consideration trainees' previous skillsets and experience. Existing training concepts from mostly single specialty organizations were analysed and incorporated. RESULTS: The WIST establishes an individualized approach to acquiring clinical knowledge and procedural skills to meet the competency requirements for certification of interventionalists of various disciplines and stroke centres in EVT. WIST guidelines encourage acquisition of skills using innovative training methods such as structured supervised high-fidelity simulation and procedural performance on human perfused cadaveric models. CONCLUSIONS: WIST multispecialty guidelines outline competency and quality standards for physicians and centres to perform safe and effective EVT. The role of quality control and quality assurance is highlighted. SUMMARY: The World Federation for Interventional Stroke Treatment (WIST) establishes an individualized approach to acquiring clinical knowledge and procedural skills to meet the competency requirements for certification of interventionalists of various disciplines and stroke centres in endovascular treatment (EVT). WIST guidelines encourage acquisition of skills using innovative training methods such as structured supervised high-fidelity simulation and procedural performance on human perfused cadaveric models. WIST multispecialty guidelines outline competency and quality standards for physicians and centers to perform safe and effective EVT. The role of quality control and quality assurance is highlighted. SIMULTANEOUS PUBLICATION: The WIST 2023 Guidelines are published simultaneously in Europe (Adv Interv Cardiol 2023).

Efficacy of minocycline in acute ischemic stroke: a single-blinded, placebo-controlled trial.

BACKGROUND: Minocycline is a semisynthetic derivative of the tetracycline group of antibiotics, which have neuroprotective effects. In animal stroke models, minocycline had shown promising evidence to improve clinical and functional outcomes. OBJECTIVE: To analyze the effect of oral minocycline in acute ischemic stroke patients. MATERIALS AND METHODS: This was a randomized single-blinded open-label study. The study group received oral minocycline 200 mg/day for 5 days and the control group received oral vitamin B capsules. Baseline assessment included the following: National Institute of Health Stroke Scale (NIHSS) score, modified Barthel Index (mBI), modified Rankin Scale (mRS) score, Magnetic Resonance Imaging (MRI) of brain including Diffusion Weighted Imaging (DWI), chest X-ray, and routine laboratory investigations. The clinical scales were repeated at days 1, 7, and 30. The end point was outcomes at 3 months (90 days). Statistical analysis was done with SPSS 11.5 (P<0.05). Paired t-test and multiple-measures Analysis Of Variance (ANOVA) were used. RESULTS: Fifty patients with acute ischemic stroke were included in the study. Of these, 23 patients received minocycline and 27 patients received placebo i.e., vitamin B capsules. NIHSS score in patients receiving minocycline had shown statistically significant improvement at day 30 and 90 as compared with the controls. Similarly, mRS scores and BI showed significant improvement in patients receiving minocycline at three months as compared to the control group. No mortality, myocardial infarctions, recurrent strokes, and hemorrhagic transformations were noted in both groups. CONCLUSIONS: Patients with acute ischemic stroke had significantly better outcome with minocycline treatment as compared with those administered placebo. The above findings suggest that minocycline can be helpful in reducing the clinical deficits after acute ischemic stroke.

Monitored gradual occlusion of the internal carotid artery followed by ligation for giant internal carotid artery aneurysms.

OBJECTIVE: To demonstrate a technique of gradual monitored occlusion of the internal carotid artery (ICA) followed by ligation for giant aneurysms as an option for balloon test occlusion followed by permanent ligation of ICA. MATERIALS AND METHODS: Authors retrospectively analyzed 27 patients with giant and complex ICA aneurysms who underwent carotid artery ligation between January 2001 and December 2010. Clinical presentation included headache, vision loss and diplopia. There were 19 patients with cavernous aneurysm, 5 supraclinoid, 1 ophthalmic, 1 petrous segment and 1 cervical segment aneurysm located extracranially. All demonstrated good cross-circulation. Selverstone clamp was used for gradual occlusion of the ICA over 72 h under closed observation in the intensive care unit. RESULTS: Six patients developed hemiparesis in the postoperative period. Improvement occurred in one patient over two to three weeks while the remaining five patients had residual hemiparesis. One patient developed malignant MCA infarct for which decompressive craniectomy had to be done. There was no mortality in the present series. CONCLUSIONS: Gradual monitored occlusion and ICA ligation may be a simple, safe alternative procedure to clipping in surgically inaccessible and complex aneurysms, especially for surgeons with limited experience. Cross circulation study is an absolute requisite for carotid ligation.

Megalencephaly-Capillary Malformation-Polymicrogyria Syndrome (MCAP): A Rare Dynamic Genetic Disorder.

Megalencephaly-capillary malformation-polymicrogyria syndrome (MCAP) is an uncommon malformation syndrome, characterized by primary megalencephaly, capillary malformations of the midline face and body, or distal limb anomalies such as syndactyly and polymicrogyria. Herein, we report a young male child, who presented with complaints of increasing head size, delay in speech, and one episode of focal seizure with distinctive morphological and neuroradiological manifestations which led to the diagnosis of MCAP. We have also reviewed recently published literature and the various diagnostic criteria proposed by authors to achieve the early clinical diagnosis of these patients in the outpatient department.

Moyamoya Syndrome in Schimke Immune-Osseous Dysplasia: A Rare Association.

Schimke immuno-osseous dysplasia (SIOD) is an uncommon autosomal recessive (AR) spondylo-epiphyseal dysplasia (SED) and its clinical course and phenotype are yet to be properly described. The phenotypic presentation is quite varied with involvement of the renal, skeletal, vascular, immune, and hematopoietic systems being the most common presentation. We describe a 19-year-old female who presented with adolescent-onset brain and skeletal involvement without renal manifestations. Based on imaging and clinical features, she was diagnosed with a case of SIOD. There is no definitive treatment yet for this disorder, however, clinicians should be aware of this disorder so that adequate counseling and symptomatic management, especially in controlling hypertension and dyslipidemia, can be provided to the affected patients.