Efficacy of Revascularization Surgery for Moyamoya Syndrome Associated With Graves' Disease

Summary We describe the case of a young Hispanic female who presented with thyrotoxicosis with seizures and ischemic stroke. She was diagnosed with a rare vasculopathy – moyamoya syndrome. After starting antithyroid therapy, her neurologic symptoms did not improve. Acute neurosurgical intervention had relieved her symptoms in the immediate post-operative period after re-anastomosis surgery. However, 2 post-operative days later, she was found to be in status epilepticus and in hyperthyroid state. She quickly deteriorated clinically and had expired a few days afterward. This is the second case in literature of a fatality in a patient with moyamoya syndrome and Graves’ disease. However, unlike the other case report, our patient had undergone successful revascularization surgery. We believe her underlying non-euthyroid state had potentiated her clinical deterioration. Case studies have shown positive correlation between uncontrolled hyperthyroidism and stroke-like symptoms in moyamoya syndrome. Mostly all patients with these two disease processes become symptomatic in marked hyperthyroid states. Thus, it may be either fluctuations in baseline thyroid function or thyrotoxicosis that potentiate otherwise asymptomatic moyamoya vasculopathy. Learning points: Awareness of the association between Graves’ disease and moyamoya syndrome in younger patients presenting with stroke-like symptoms. Obtaining euthyroid states before undergoing revascularization surgery may protect the patient from perioperative mortality and morbidity. Although moyamoya disease is usually thought to be genetically associated, there are reports that thyroid antibodies may play a role in its pathogenesis and have an autoimmune link. Fluctuations in baseline thyroid function for patients with known Graves’ disease may be a potentiating factor in exacerbating moyamoya vasculopathy.

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Two Sides of a Coin: Case Report of Unilateral Synangiosis and Contralateral Stroke Highlighting Consequences of Disease Progression and Efficacy of Revascularization in Sickle Cell Disease Associated Moyamoya Syndrome

Acta Haematologica ◽

10.1159/000521361 ◽

2021 ◽

Author(s):

Anna L. Slingerland ◽

Madeline B. Karsten ◽

Edward R. Smith ◽

Amy E. Sobota ◽

Alfred P. See

Keyword(s):

Sickle Cell Disease ◽

Disease Progression ◽

Sickle Cell ◽

Heart Association ◽

Standard Of Care ◽

Collaborative Management ◽

Moyamoya Syndrome ◽

Cell Disease ◽

Revascularization Surgery ◽

Consequences Of Disease

Moyamoya syndrome increases the risk of stroke in sickle cell disease, but revascularization surgery can modify this risk. Collaborative management between hematology and neurosurgery offers effective strategies to reduce stroke risk in these patients. We describe a challenging case where a patient with sickle cell disease undergoing standard of care management as prescribed by the Stroke Prevention Trial in Sickle Cell Anemia (STOP) and revascularization with pial synangiosis subsequently developed rapidly progressive disease in other cerebral vessels and suffered ischemic hemispheric stroke. This case demonstrates the success of management in accordance with American Heart Association (AHA) and American Stroke Association (ASA) guidelines, but also demonstrates critical areas where we lack understanding of disease progression.

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Reversible chorea in association with Graves' disease and moyamoya syndrome

Movement Disorders ◽

10.1002/mds.21941 ◽

2008 ◽

Vol 23 (4) ◽

pp. 620-622 ◽

Cited By ~ 18

Author(s):

Béatrice Garcin ◽

Taina Louissaint ◽

Hassan Hosseini ◽

Raphaël Blanc ◽

Gilles Fénelon

Keyword(s):

Graves Disease ◽

Moyamoya Syndrome

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Moyamoya syndrome associated with Graves’ disease

Nosotchu ◽

10.3995/jstroke.34.82 ◽

2012 ◽

Vol 34 (2) ◽

pp. 82-88 ◽

Cited By ~ 1

Author(s):

Takeshi Mikami ◽

Toshiya Sugino ◽

Aya Kanno ◽

Kiyohiro Houkin ◽

Nobuhiro Mikuni

Keyword(s):

Graves Disease ◽

Moyamoya Syndrome

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Concurrent Moyamoya syndrome and Graves’ disease: A case report

Journal of the Neurological Sciences ◽

10.1016/j.jns.2017.08.3380 ◽

2017 ◽

Vol 381 ◽

pp. 413

Author(s):

M.H. Fu

Keyword(s):

Case Report ◽

Graves Disease ◽

Moyamoya Syndrome

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Arterial spin-labeling cerebral perfusion changes after revascularization surgery in pediatric moyamoya disease and syndrome

Journal of Neurosurgery Pediatrics ◽

10.3171/2018.11.peds18498 ◽

2019 ◽

Vol 23 (4) ◽

pp. 486-492 ◽

Cited By ~ 2

Author(s):

Jennifer L. Quon ◽

Lily H. Kim ◽

Robert M. Lober ◽

Maryam Maleki ◽

Gary K. Steinberg ◽

...

Keyword(s):

Basal Ganglia ◽

Middle Cerebral Artery ◽

Moyamoya Disease ◽

Arterial Spin Labeling ◽

Cerebral Perfusion ◽

Spin Labeling ◽

Middle Cerebral Artery Territory ◽

Moyamoya Syndrome ◽

Hemodynamic Changes ◽

Revascularization Surgery

OBJECTIVEMoyamoya disease is a dynamic cerebrovascular condition that often requires vascular surveillance. Arterial spin labeling (ASL) is an MR perfusion method that is increasingly used for stroke and other various neurovascular pathologies. Unlike perfusion-weighted MRI, ASL uses endogenous water molecules for signal and therefore obviates gadolinium use; and provides direct, not relative, quantitative cerebral blood flow (CBF) measures. Presently, the potential role of ASL for evaluating postoperative pediatric moyamoya patients is relatively unexplored. This study investigated the role for ASL in evaluating cerebral hemodynamic changes in children who underwent revascularization surgery.METHODSThis retrospective study examined 15 consecutive pediatric patients with moyamoya disease (n = 7) or moyamoya syndrome (n = 8) presenting between 2010 and 2014 who underwent revascularization and in whom 3T ASL was performed pre- and postoperatively. Postoperative MRI at least 3 months after revascularization procedure was used for analysis. Quantitative CBF in various vascular territories was interrogated: anterior, middle, and posterior cerebral arteries, and basal ganglia supplied by the lenticulostriate collaterals, resulting in evaluation of 20 brain regions.RESULTSAfter revascularization, CBF in the high middle cerebral artery territory significantly increased (p = 0.0059), accompanied by a decrease in CBF to the ipsilateral lenticulostriate-supplied basal ganglia (p = 0.0053). No perfusion changes occurred in the remaining cerebral vascular territories after surgery.CONCLUSIONSASL-based quantitative CBF showed improved cerebral perfusion to the middle cerebral artery territory after revascularization in children with both moyamoya syndrome and disease. Reduced perfusion to the basal ganglia might reflect pruning of the lenticulostriate collaterals, potentially from effects of revascularization. ASL can quantitatively evaluate hemodynamic changes in children with moyamoya after revascularization, and it may be a useful adjunct to routine clinical MRI surveillance.

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Moyamoya disease with mesial temporal sclerosis

Journal of Neurosurgery Pediatrics ◽

10.3171/2013.3.peds12437 ◽

2013 ◽

Vol 11 (6) ◽

pp. 713-716 ◽

Cited By ~ 2

Author(s):

Subash Lohani ◽

Joseph R. Madsen ◽

Ann M. Bergin ◽

Edward R. Smith

Keyword(s):

Clinical Problem ◽

Temporal Lobectomy ◽

Neurological Deficits ◽

Mesial Temporal Sclerosis ◽

Moyamoya Syndrome ◽

Simultaneous Operations ◽

History Of ◽

Mesial Temporal Lobe ◽

The Right ◽

Revascularization Surgery

The combination of moyamoya syndrome and symptomatic mesial temporal sclerosis (MTS) has not previously been reported. The authors present the case of a 5-year-old boy with symptomatic MTS who developed progressive moyamoya syndrome. This combination of progressive moyamoya and a structural seizure focus presented a unique clinical problem, with the natural history of MTS predicting a high likelihood of needing resection in the future, which could be challenging following any type of moyamoya-related revascularization surgery. In anticipation of this problem, the patient underwent resection of the right inferior and mesial temporal lobe followed by right pial synangiosis as a 1-day combined operation. Postoperatively he recovered well without any neurological deficits and had an uneventful hospital stay. This case of moyamoya is unique in its association with MTS, and for the simultaneous operations for pial synangiosis and temporal lobectomy, highlighting the importance of surgical planning in patients with dual pathological processes.

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Moyamoya Syndrome Associated with Graves' Disease: A Case Report and Review of the Literature

Journal of Stroke and Cerebrovascular Diseases ◽

10.1016/j.jstrokecerebrovasdis.2010.03.006 ◽

2011 ◽

Vol 20 (6) ◽

pp. 528-536 ◽

Cited By ~ 21

Author(s):

Shaneela Malik ◽

Andrew N. Russman ◽

Angelos M. Katramados ◽

Brian Silver ◽

Panayiotis D. Mitsias

Keyword(s):

Case Report ◽

Graves Disease ◽

Moyamoya Syndrome ◽

Review Of The Literature

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MOYAMOYA SYNDROME: RARE CASE IN 34 YEAR OLD MALE PATIENT IN INDIAN POPULATION – A CASE REPORT

10.36106/ijsr/4300315 ◽

2021 ◽

pp. 8-9

Author(s):

Dinesh Agarwal ◽

Debashish Baidya

Keyword(s):

Indian Population ◽

Past History ◽

Research Centre ◽

Moyamoya Syndrome ◽

Sudden Loss ◽

Left Hand ◽

Mri Brain ◽

History Of ◽

Collateral Vessels ◽

Revascularization Surgery

Moyamoya is a rare idiopathic progressive vaso-occlusive disease involving cerebral vessels like distal internalcarotid arteries on both sides, To compensate for the occlusion there is development of collateral vessels. Unilateral presentation of the condition is known as Moyamoya syndrome. Authors are presenting a case of a 34-year-old male who presented to the emergency department of Marwari Hospital and Research Centre with sudden loss of consciousness. Patient had past history of difculty in holding objects with left hand & difculty in walking for 3 years. The patient had undergone MRI BRAIN, diagnosed as Moyamoya disease and treated with revascularization surgery.

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Anesthetic Management of Moyamoya Syndrome Secondary to Sickle Cell Anemia

Journal of Neuroanaesthesiology and Critical Care ◽

10.1055/s-0041-1739349 ◽

2021 ◽

Author(s):

Parmod K. Bithal ◽

Ravees Jan ◽

Ved P. Pandey ◽

Parvaiz Ahmad

Keyword(s):

Sickle Cell ◽

Sickle Cell Anemia ◽

Anesthetic Management ◽

Physiological Parameters ◽

Moyamoya Syndrome ◽

Physiological Variable ◽

Physiological Variables ◽

Disease Occurrence ◽

Carbon Dioxide Reactivity ◽

Revascularization Surgery

AbstractMoyamoya disease (MMD) is caused by stenosis or occlusion of internal carotid artery in brain, thereby reducing its blood supply. To augment blood flow, brain develops abnormal anastomotic vessels with deranged carbon dioxide reactivity and tendency to bleed. Moyamoya syndrome (MMS) is the name given to MMD when the latter results from secondary to some associated disease. Occurrence of MMS secondary to sickle cell anemia (SCA) presents unique challenges to neuroanesthesiologists. Management of various physiological parameters for cerebral revascularization surgery for MMD under general anesthesia necessitates vigilant and balanced control of various physiological variables, as the manipulation of a particular physiological variable for one pathology may adversely impact the same physiological variable for the associated disease, which will result in poor outcome of the patient. Therefore, optimum outcome of MMS is determined by a watchful balancing of various physiological parameters under anesthesia.

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