Headache as unusual presentation in moyamoya disease

Our case highlights basics of clinical presentations, causes, risk factors, epidemiology, physiology, and pathophysiology in Moyamoya diseases, along with introduction to terminologies, prospect of evaluation and various modalities available for diagnosis. Moyamoya angiopathy is characterized by a progressive stenosis of the terminal portion of the internal carotid arteries and the development of a network of abnormal collateral vessels. This chronic cerebral angiopathy is observed in children and adults. It mainly leads to brain ischemic events in children, and to ischemic and hemorrhagic events in adults. This is a rare condition, with a marked prevalence gradient between Asian countries and Western countries. Two main nosological entities are identified. On the one hand, moyamoya disease corresponds to isolated moyamoya angiopathy, defined as being “idiopathic” according to the Guidelines of the Research Committee on the Pathology and Treatment of Spontaneous Occlusion of the Circle of Willis. This entity is probably multifactorial and polygenic in most patients. On the other hand, moyamoya syndrome is a moyamoya angiopathy associated with an underlying condition and forms a very heterogeneous group with various clinical presentations, various modes of inheritance, and a variable penetrance of the cerebrovascular phenotype. Diagnostic and evaluation techniques rely on magnetic resonance imaging (MRI), magnetic resonance angiography (MRA) conventional angiography, and cerebral hemodynamics measurements. Revascularization surgery can be indicated, with several techniques. Characteristics of genetic moyamoya syndromes are presented, with a focus on recently reported mutations in BRCC3/MTCP1 and GUCY1A3 genes. Identification of the genes involved in moyamoya disease and several monogenic moyamoya syndromes unravelled different pathways involved in the development of this angiopathy. Studying genes and pathways involved in monogenic moyamoya syndromes may help to give insights into pathophysiological models and discover potential candidates for medical treatment strategies.

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Magnetic resonance imaging template to standardize reporting of anal fistulas

Techniques in Coloproctology ◽

10.1007/s10151-020-02384-6 ◽

2021 ◽

Author(s):

I. Sudoł-Szopińska ◽

G. A. Santoro ◽

M. Kołodziejczak ◽

A. Wiaczek ◽

U. Grossi

Keyword(s):

Magnetic Resonance Imaging ◽

Magnetic Resonance ◽

Treatment Strategies ◽

Cross Sectional Study ◽

Resonance Imaging ◽

Anal Fistulas ◽

Cross Sectional ◽

Radiology Reports ◽

Colorectal Surgeons ◽

Magnetic Resonance Imaging Mri

AbstractAnal fistula (AF) is a common referral to colorectal surgeons. Management remains challenging and sometimes controversial. Magnetic resonance imaging (MRI) is commonly performed in initial workup for AF. However, reports often lack key information for guiding treatment strategies. It has been shown that with structured radiology reports, there is less missing information. We present a structured MRI template report including 8 key descriptors of anal fistulas, whose effectiveness and acceptability are being assessed in a cross-sectional study (NCT04541238).

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Abstract WMP71: Moyamoya-atherosclerosis Syndrome: Clinical and High-resolution Magnetic Resonance Imaging Features

Stroke ◽

10.1161/str.48.suppl_1.wmp71 ◽

2017 ◽

Vol 48 (suppl_1) ◽

Author(s):

Lian Duan ◽

Wei-Hai Xu ◽

Cong Han

Keyword(s):

Magnetic Resonance Imaging ◽

Risk Factors ◽

Magnetic Resonance ◽

High Resolution ◽

Moyamoya Disease ◽

Treatment Strategies ◽

Diagnostic Process ◽

Imaging Features ◽

Intracranial Atherosclerosis ◽

Resonance Imaging

Introduction: The diagnosis in the patients with angiographic moyamoya findings and atherogenic risk factors is challenging. In this study, we try to incorporate high-resolution magnetic resonance imaging (HRMRI) into the diagnostic process of intracranial atherosclerosis associated moyamoya syndrome. Methods: From March 2013 to March 2014, HRMRI was consecutively performed on adult patients with angiographic moyamoya. The patients were classified as moyamoya - plaques (MMD-P) if a plaque could be identified or as moyamoya - no plaques (MMD-NP) if a plaque could not be identified. The angiography, HRMRI findings and atherogenic risk factors of these patients were analyzed. Results: Fifty-one patients (mean age 39±9, 20 males) were enrolled. On traditional angiography, probable intracranial atherosclerosis was identified in 5 patients, no definite diagnosis in 12 patients, and moyamoya disease in 34 patients. On HRMRI, 15 out of 32 patients with risk factors and 4 out of 19 patients without risk factors were found to have plaques and were diagnosed as MMD-P, while the other 32 patients were diagnosed as MMD-NP. The MMD-P patients were more likely to be older and male and were less likely to have cerebral hemorrhage and a history of disease progression. Conclusions: Our study suggests that HRMRI can help diagnose intracranial atherosclerosis more accurately in moyamoya disease patients with atherogenic risk factors. The distinct clinical features between MMD-P and MMD-NP patients suggest different underlying pathophysiology and therefore potentially different treatment strategies.

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The ivy sign

South African Journal of Radiology ◽

10.4102/sajr.v18i1.622 ◽

2014 ◽

Vol 18 (1) ◽

Author(s):

Nasreen Mahomed ◽

Evance Chisama ◽

Sanjay Prabhu

Keyword(s):

Magnetic Resonance Imaging ◽

Magnetic Resonance ◽

Moyamoya Disease ◽

Signal Intensity ◽

Perivascular Spaces ◽

Resonance Imaging ◽

Leptomeningeal Enhancement ◽

Weighted Magnetic Resonance Imaging ◽

Mri Sequences ◽

Magnetic Resonance Imaging Mri

The ivy sign refers to diffuse bilateral leptomeningeal enhancement on post- contrastT1-weighted magnetic resonance imaging (MRI) and increased signal intensity in bilateralsubarachnoid spaces and perivascular spaces on T2-weighted fluid attenuation inversionrecovery (FLAIR) MRI sequences in patients with moyamoya disease.

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Is “unilateral” moyamoya disease different from moyamoya disease?

Journal of Neurosurgery ◽

10.3171/jns.1996.85.5.0772 ◽

1996 ◽

Vol 85 (5) ◽

pp. 772-776 ◽

Cited By ~ 65

Author(s):

Kiyohiro Houkin ◽

Hiroshi Abe ◽

Tetsuyuki Yoshimoto ◽

Akihiro Takahashi

Keyword(s):

Magnetic Resonance ◽

Magnetic Resonance Angiography ◽

Moyamoya Disease ◽

Familial Occurrence ◽

Conventional Angiography ◽

Content Type ◽

Angiographic Evidence ◽

Unilateral Moyamoya Disease ◽

Revascularization Surgery

✓ Whether a diagnosis of moyamoya disease is justified in patients with typical angiographic evidence of moyamoya disease unilaterally and normal angiographic findings contralaterally remains controversial. In this study the authors analyzed longitudinal angiographic change, familial occurrence, and basic fibroblast growth factor (bFGF) concentration in the cerebrospinal fluid (CSF) of patients with “unilateral” moyamoya disease. Over a 10-year period, 10 cases of unilateral moyamoya disease were followed using conventional angiography or magnetic resonance angiography. Basic FGF in CSF, obtained from the subarachnoid space of the cerebral cortex during revascularization surgery, was measured in five cases. Among the 10 cases of unilateral moyamoya disease, only one pediatric case showed obvious signs of progression to typical bilateral disease. The other nine cases (including six adults and three children) remained stable throughout follow-up radiological examinations (magnetic resonance angiography) with a mean observation period of 3.5 years. There was no familial occurrence in these cases of unilateral moyamoya disease. Levels of bFGF, which are high in typical moyamoya disease, were low in these patients. The progression from unilateral moyamoya disease to the typical bilateral form of the disease appears to be infrequent. The low levels of bFGF in the CSF of these patients and the lack of familial occurrence strongly suggest that most cases of unilateral moyamoya disease, especially those found in adults, are distinct from typical bilateral moyamoya disease.

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Assessment of Moyamoya Disease With 3.0-T Magnetic Resonance Angiography and Magnetic Resonance Imaging Versus Conventional Angiography

Neurologia medico-chirurgica ◽

10.2176/nmc.51.195 ◽

2011 ◽

Vol 51 (3) ◽

pp. 195-200 ◽

Cited By ~ 22

Author(s):

Qianna JIN ◽

Tomoyuki NOGUCHI ◽

Hiroyuki IRIE ◽

Masatou KAWASHIMA ◽

Masashi NISHIHARA ◽

...

Keyword(s):

Magnetic Resonance Imaging ◽

Magnetic Resonance ◽

Magnetic Resonance Angiography ◽

Moyamoya Disease ◽

Resonance Imaging ◽

Conventional Angiography ◽

3.0 T

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Intra‐arterial magnetic resonance angiography of the iliac arteries: clinical experience using two different protocols

Acta Radiologica ◽

10.1080/02841850510021030 ◽

2005 ◽

Vol 46 (3) ◽

pp. 250-255 ◽

Cited By ~ 1

Author(s):

C. Paetzel ◽

N. Zorger ◽

M. Völk ◽

T. Herold ◽

J. Seitz ◽

...

Keyword(s):

Magnetic Resonance ◽

Magnetic Resonance Angiography ◽

Diagnostic Value ◽

Conventional Angiography ◽

Iliac Arteries ◽

Contrast Enhanced ◽

Group 2 ◽

Magnetic Resonance Imaging Mri ◽

Space Acquisition ◽

Group 1

Purpose: To assess the feasibility of intra‐arterial magnetic resonance angiography (iaMRA) with two different protocols. Material and Methods: Twenty patients were prospectively examined after digital subtraction angiography. Contrast‐enhanced iaMRA was performed using a 1.5T magnetic resonance imaging (MRI) system. Contrast agent (gadodiamide) was injected through a conventional angiography catheter placed in the abdominal aorta. The patients were randomized into two groups each comprising 10 patients. Group 1 was examined with a FLASH‐3D (fast low‐angle shot) sequence, allowing the center of the k‐space to be acquired 0.5 s after initiation of the measurement. Group 2 was examined with the identical sequence, but the center of the k‐space was acquired after 8.7 s. The increase in the intravascular signal intensity was determined and the diagnostic value of the angiograms was independently scored by 4 investigators using a 5‐point scale. Results: Nineteen of 20 MRAs were scored as diagnostic; only 1 was scored as non‐diagnostic by 2 observers. The diagnostic value of the angiograms of group 2 was judged superior to that of group 1 owing to a more homogeneous intravascular contrast distribution. Conclusion: Intra‐arterial MRA is feasible. The diagnostic value of angiograms using a flash sequence with center of the k‐space acquisition after 8.7 s ranged from good to excellent. This sequence is appropriate for iaMRA of iliac arteries to support MR guided intervention.

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Moyamoya disease

JBNC - JORNAL BRASILEIRO DE NEUROCIRURGIA ◽

10.22290/jbnc.v32i1.1949 ◽

2021 ◽

Vol 32 (1) ◽

pp. 101-109

Author(s):

Gustavo Soares Gomes Barros Fonseca ◽

Caio Nuto Leite França ◽

Emilio Afonso Franca Fontoura ◽

Cláudia Nery do Nascimento Coelho ◽

Abilio Costa e Silva

Keyword(s):

Carotid Artery ◽

Internal Carotid Artery ◽

Moyamoya Disease ◽

Internal Carotid ◽

A Priori ◽

Recommended Treatment ◽

Magnetic Resonance Imaging Mri ◽

Collateral Vessels ◽

Conclusive Diagnosis

Background: Moyamoya disease (MMD) is a rare pathology caused by a progressive unilateral or bilateral stenosis of the terminal portion of the internal carotid artery, leading to the development of collateral vessels. Case Presentation: We report a rare case of a 46-year-old male, born in the city of São Paulo, Brazil, with sudden muscular strength deficit and right hemiparesis, associated with headache and emesis. A priori, the initial diagnosis was arteriovenous malformation (AVM) after performing a series of complementary tests during the patient’s follow-up, also considering the clinical picture similar to that of Moyamoya disease (MMD). The conclusive diagnosis of MMD was finally established when the magnetic resonance imaging (MRI) showed a network of tortuous and dilated collateral vessels, with a hazy “smoke cloud” aspect with stenosis of the M1 segment of the middle cerebral artery (MCA), branch of the artery internal carotid artery (ICA). Conclusion: The recommended treatment was surgical revascularization with extracranial-intracranial bypass, with a favorable prognosis to the patient.

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Assessment of irreducible aspects in developmental hip dysplasia by magnetic resonance imaging

10.21203/rs.3.rs-39006/v1 ◽

2020 ◽

Author(s):

Huihui Jia ◽

Liang Wang ◽

Yan Chang ◽

Yongrui Song ◽

Yuqi Liu ◽

...

Keyword(s):

Magnetic Resonance Imaging ◽

Magnetic Resonance ◽

Treatment Strategies ◽

Developmental Dysplasia ◽

Joint Effusion ◽

Iliopsoas Muscle ◽

Resonance Imaging ◽

Developmental Hip Dysplasia ◽

Hip Reduction ◽

Magnetic Resonance Imaging Mri

Abstract We aimed to assess the diagnostic capability of magnetic resonance imaging (MRI) for irreducible aspects preventing hip reduction in developmental dysplasia of the hip(DDH). With pathological results or intraoperative findings as the gold standard, the sensitivity and specificity of MRI were 90.3% and 83.3% for the affected labrum, 92% and 83.3% for thickening of the round ligament, 90.0% and 91.3% for atrophy of the iliopsoas muscle, and 100% and 100% for fibrofatty pulvinar tissue and joint effusion, respectively. The MRI showed an extraordinary capability of detecting these irreducible factors and helped surgeon choose the appropriate treatment strategies.

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Cortical Anaplastic Ependymoma with Significant Desmoplasia: A Case Report and Literature Review

Case Reports in Oncological Medicine ◽

10.1155/2013/354873 ◽

2013 ◽

Vol 2013 ◽

pp. 1-6 ◽

Cited By ~ 2

Author(s):

Alaa Eldin Elsharkawy ◽

Raid Abuamona ◽

Markus Bergmann ◽

Shadi Salem ◽

Evariste Gafumbegete ◽

...

Keyword(s):

Magnetic Resonance ◽

Literature Review ◽

Resonance Spectroscopy ◽

Solid Mass ◽

Resonance Imaging ◽

Anaplastic Ependymoma ◽

Who Grade ◽

Clinical Presentations ◽

Magnetic Resonance Imaging Mri ◽

Histopathological Grading

Ectopic brain anaplastic ependymomas with no connection to the ventricles are rare. We present a rare case of a 25-year-old male who presented with generalized convulsions. Computed tomography (CT), Magnetic Resonance Imaging (MRI), and magnetic resonance spectroscopy (MRS) showed characters of an intra- and extra-axial lesion. Intraoperatively, the lesion was a cortical solid mass that had no connections to the dura or to the ventricle. The histological diagnosis showed an anaplastic ependymoma with WHO grade III with distinctive desmoplasia. A literature review of ectopic anaplastic ependymomas regarding their clinical presentations, management, and prognostic factors was performed. There is a need to establish a clinically based histopathological grading system for anaplastic ependymomas. Ectopic anaplastic ependymomas should be included in the preoperative differential diagnosis.

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