431:
of
Diseases (ICD). These biomarkers of moyamoya are "stenosis of the distal ICA's up to and including the bifurcation, along with segments of the proximal ACA and MCA...dilated basal collateral vessels must be present" Some other common findings that have not been added to the classification index of those with moyamoya disease which are found using radiography involve very distinct changes in the vessels of the brain. These changes include newly formed vessels made to compensate for another change noted, ischemia and cerebrovascular reserve, both found on MRI. Functional changes include evidence of ischemia in vessels of the brain (ICA, ACA, MCA, specifically). It is important to also note that the radiographic biomarkers, in order to be classified as moyamoya disease, all findings must be bilateral. If this is not the case and the findings are unilateral, it is diagnosed as moyamoya syndrome. This recently changed in 2021 as the Research Committee of Moyamoya Disease (RCMD) has "removed limitations of the previous definition that required bilateral involvement of the intracranial carotid artery. Now, proximal middle cerebral artery or anterior cerebral artery involvement suffices, and unilateral disease is acceptable to make the diagnosis, given the increasing evidence of progression to bilateral involvement in unilateral MMD."
272:(RNF213) has been implicated. In September 2021, a south Indian researcher has proposed a pathbreaking theory on moyamoya pathogenesis. Coined the "Mechano-biological theory", the disease has a multifactorial pathogenesis. The authors provide a tangible explanation of the occurrence of moyamoya phenomenon in the idiopathic and syndromic variants of the disease. In short, the authors report that moyamoya disease likely occurs due to a number of factors (e.g., differences in vascular anatomy) that ultimately contribute to broad cerebral blood vessel occlusion and consequent shifts in vessel connections to try to provide blood for the compromised brain.
520:
in this improvement by increasing cerebral blood flow (CBF) after the operation. A significant correlation is found between the postoperative effect and the stages of preoperative angiograms. It is crucial for surgery that the anesthesiologist have experience in managing children being treated for moyamoya, as the type of anesthesia they require is very different from the standard anesthetic children get for almost any other type of
43:
339:
435:
Other studies have confirmed the correlation of moyamoya and adhesion molecule 1 (ICAM-1) being increased as compared to normal vascular function counterparts. Furthermore, it has been concluded that the localization of inflammatory cells suggests that the inflammation stimulus itself may be responsible for the proliferation and occlusion in the ICA, ACA, and MCA found in those with moyamoya disease.
556:. In this case a 44-year-old man presented with headache, tinnitus, and an intraventricular hemorrhage, as seen on computed tomographic scans. Cerebral angiography showed a right moyamoya pattern and an ipsilateral dural AVF fed by branches of the external carotid artery and draining into the transverse sinus. This extremely rare coincidental presentation may have deeper pathogenic implications.
286:, and often extends to the middle and anterior cerebral arteries, branches of the internal carotid artery inside the skull. When the internal carotid artery becomes completely blocked, the fine collateral circulation that it supplies is obliterated. Patients often survive on the collateral circulation from the back (posterior) of the circle of Willis, arising from the
375:
thickening, or proliferation, of the innermost layer of the vessels affected by moyamoya. These vessels are the ACA (anterior cerebral artery), MCA (middle cerebral artery), and ICA (internal carotid artery). The occlusion of the ICA results in concomitant diminution of the "puff-of-smoke" collaterals, as they are supplied by the ICA.
466:(MCA) bypass (also known as ECIC bypass) performed with a combination of in-direct procedures is considered the treatment of choice. Although its efficacy, particularly for hemorrhagic disease, remains uncertain, the procedure is thought to reduce the hemodynamic burden on the engorged collateral blood vessels. Multiple
382:(single photon emission computerized tomography) are used to demonstrate the decreased blood and oxygen supply to areas of the brain involved with moyamoya disease. Conventional angiography provides the conclusive diagnosis of moyamoya disease in most cases and should be performed before any surgical considerations.
552:(AVFs) of the lining of the brain, the dura, are associated with dural angiogenesis. These factors may represent a mechanism for ischemia contributing to the formation of dural AVFs. At least one case of simultaneous unilateral moyamoya syndrome and ipsilateral dural arteriovenous fistula has been reported at the
519:
All of these operations have in common the concept of a blood and oxygen "starved" brain reaching out to grasp and develop new and more efficient means of bringing blood to the brain and bypassing the areas of blockage. The modified direct anastomosis and encephalo-myo-arterio-synangiosis play a role
430:
There is also research that has shown that certain radiographic biomarkers that lead to the diagnosis of moyamoya disease have been identified. The specific radiographic markers are now considered an acceptable key component to moyamoya disease and have been added to the
International Classification
434:
There are also several protein biomarkers that have been linked to the moyamoya disease diagnosis. Although the sample size of the studies performed are small due to the rarity of the disease, the findings are indicative of a correlation between the disease and several specific protein biomarkers.
267:
The disease moyamoya, which is a
Japanese mimetic word, gets its characteristic name due to the appearance of smoke on relevant angiographs resultant from the tangle of tiny vessels in response to stenosis. This makes the blood leak out of the arteries, causing pressure to the brain and subsequent
559:
The research into the pathogenesis of moyamoya disease has found a breakthrough with the proposal of a "Mechano-biological theory" of pathogenesis of this disease. A research group in southern India have proposed this unifying theory based on computational fluid dynamics studies and longitudinal
223:
In the United States moyamoya has an incidence rate of 0.086 per 100,000. In Japan the overall incidence is higher (0.35 per 100,000). In North
America, women in the third or fourth decade of life are most often affected, but the condition may also occur during infancy or childhood. These women
153:
When moyamoya is diagnosed by itself, with no underlying correlational conditions, it is diagnosed as moyamoya disease. This is also the case when the arterial constriction and collateral circulation are bilateral. Moyamoya syndrome is unilateral arterial constriction, or occurs when one of the
414:
Smith (2015) conducted a study that looked into specific biological markers that correlate to moyamoya disease. Some of the categories of these biomarkers include phenotypes - conditions commonly related to moyamoya, radiographical markers for the diagnosis of moyamoya, and proteins as well as
532:
The natural history of this disorder is not well known. The long term outlook for patients with treated moyamoya seems to be good when direct bypass is used. Although symptoms may seem to improve almost immediately after the in-direct EDAS, EMS, and multiple burr holes surgeries, it will take
385:
Darren B. Orbach explains how the disease progresses as well as the role angiography plays in detecting the progression of moyamoya in a short video. In 2019, author and artist Sarah
Lippett published a graphic novel about her decade-long struggle to get a diagnosis and treatment for moyamoya
374:
Proliferation of smooth muscle cells in the walls of the moyamoya-affected arteries has been found to be representative of the disease. A study of six autopsies of six patients who died from moyamoya disease lead to the finding that there is evidence that supports the theory that there is a
1492:
Sun, Hai; Wilson, Christopher; Ozpinar, Alp; Safavi-Abbasi, Sam; Zhao, Yan; Nakaji, Peter; Wanebo, John E.; Spetzler, Robert F. (August 2016). "Perioperative complications and long-term outcomes after bypasses in adults with moyamoya disease: a systematic review and meta-analysis".
446:(including aspirin) are usually given to prevent clots, but surgery is usually recommended. Because moyamoya tends to affect only the internal carotid artery and nearby sections of the adjacent anterior and middle cerebral arteries, surgeons can direct other arteries, such as the
457:
There are many operations that have been developed for the condition, but currently the most favored are the in-direct procedures EDAS, EMS, and multiple burr holes and the direct procedure STA-MCA. Combined revascularisation procedure, which includes both the direct
515:
procedure, the scalp artery (superficial temporal artery or STA) is directly sutured to an artery on the surface of the brain (middle cerebral artery or MCA). This procedure is also commonly referred to as an EC-IC (External
Carotid-Internal Carotid) bypass.
70:
Headache, Seizures, Weakness, Numbness or paralysis in your face, arm or leg, typically on one side of your body, visual disturbances, Difficulties with speaking or understanding others (aphasia), Cognitive or developmental delays, involuntary
1626:
1611:
1238:
91:
Magnetic resonance imaging (MRI), Computerized tomography (CT) scan, Cerebral angiogram, Positron emission tomography (PET) scan or single-photon emission computerized tomography (SPECT), Electroencephalogram (EEG), Transcranial
Doppler
301:
of arteries are damaged, leading to the deposition of fat and immune cells, and ultimately the accumulation of immune cells laden with fat. In moyamoya, the inner layer of the carotid artery proliferates within the arterial
485:
procedure that requires dissection of a scalp artery over a course of several centimeters and then making a small temporary opening in the skull directly beneath the artery. The artery is then sutured to a branch of the
216:(8q23) (MYMY3; 608796). See also MYMY4 (300845), an X-linked recessive syndromic disorder characterized by moyamoya disease, short stature, hypergonadotropic hypogonadism, and facial dysmorphism, and linked to q25.3, on
418:
Similar to moyamoya disease, there are conditions that are closely associated with moyamoya disease. Some of the more common medical conditions that are closely associated with moyamoya disease include trisomy 21
228:(TIA), cerebral hemorrhage, or may not experience any symptoms at all. They have a higher risk of recurrent stroke and may be experiencing a distinct underlying pathophysiology compared to patients from Japan.
2609:
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Moyamoya disease tends to affect adults in the third to fourth decade of life. In children it tends to cause strokes or seizures. In adults it tends to cause strokes or bleeding. The clinical features are
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About 10% of cases of moyamoya disease are familial, and some cases result from specific genetic mutations. Susceptibility to moyamoya disease-2 (MYMY2; 607151) is caused by variation in the
2158:
1536:
Killory BD, Gonzalez LF, Wait SD, Ponce FA, Albuquerque FC, Spetzler RF (Jun 2008). "Simultaneous unilateral moyamoya disease and ipsilateral dural arteriovenous fistula: case report".
454:
to replace its circulation. The arteries are either sewn directly into the brain circulation, or placed on the surface of the brain to reestablish new circulation after a few weeks.
536:
Once a major stroke or bleeding takes place, even with treatment, the patient may be left with permanent loss of function so it is very important to treat this condition promptly.
427:
and congenital dwarfing syndromes as two of the more loosely associated syndromes that correlate with the possibility of being diagnosed with moyamoya disease later in life.
1704:
1361:"Adult Moyamoya Disease and Syndrome: Current Perspectives and Future Directions: A Scientific Statement From the American Heart Association/ American Stroke Association"
2354:
2349:
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Cerebral angiography is the gold standard of diagnosing moyamoya disease and its progression. According to Suzuki's system, it can be classified into six stages:
533:
probably 6 to 12 months before new vessels can develop to give a sufficient blood supply. With the direct STA-MCA surgery, increased blood supply is immediate.
154:
several specified conditions is also present. This may also be considered as moyamoya being secondary to the primary condition. Mainly, occlusion of the distal
1236:
http://www.childrenshospital.org/centers-and-services/programs/f-_-n/moyamoya-disease-program/patient-resources/videos-about-moyamoya/moyamoya-treatment-videos
2151:
2977:
1453:"Apolipoprotein CIII Induces Expression of Vascular Cell Adhesion Molecule-1 in Vascular Endothelial Cells and Increases Adhesion of Monocytic Cells"
2967:
2144:
1406:
1314:
759:
Duan, Lian; Bao, Xiang-Yang; Yang, Wei-Zhong; Shi, Wan-Chao; Li, De-Sheng; Zhang, Zheng-Shan; Zong, Rui; Han, Cong; Zhao, Feng; Feng, Jie (2012).
268:
headaches. Over the last six decades since the disease was first described, pathogenesis of moyamoya disease remained elusive, although the gene
866:
Wakai K, Tamakoshi A, Ikezaki K, et al. (1997). "Epidemiological features of moyamoya disease in Japan: findings from a nationwide survey".
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muscle, which is in the temple region of the forehead, is dissected and through an opening in the skull placed onto the surface of the brain.
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1956:
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data. This proposal unifies the pathogenesis of moyamoya disease and moyamoya syndromes described in literature under a single mechanism.
508:
procedure, multiple small holes (burr holes) are placed in the skull to allow for growth of new vessels into the brain from the scalp.
3029:
2962:
2517:
1951:
1752:
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2535:
1742:
1690:
1255:
716:
Kleinloog, R (May 2012). "Regional differences in incidence and patient characteristics of moyamoya disease: a systematic review".
585:
400:
as a "wonderfully drawn memoir of a serious childhood illness." It was one of the paper's "graphic novels of the year" in 2019 and
371:
Magnetic resonance angiography (MRA) is also useful in diagnosing the disease with good correlation with Suzuki's grading system.
81:
permanent damage to the brain, seizures, paralysis, vision problems, speech problems, movement disorders and developmental delays.
2125:
1068:
Sudhir, Bhanu
Jayanand; Keelara, Arun Gowda; Venkat, Easwer Harihara; Kazumata, Ken; Sundararaman, Ananthalakshmy (2021-09-01).
983:"Prevalence and clinicoepidemiological features of moyamoya disease in Japan: findings from a nationwide epidemiological survey"
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1903:
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478:
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can develop moyamoya malformations. It is more common in women than in men, although about a third of those affected are male.
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develops around the blocked vessels to compensate for the blockage, but the collateral vessels are small, weak, and prone to
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Once it begins, the vascular occlusion tends to continue despite any known medical management. In some people this leads to
111:
is a disease in which certain arteries in the brain are constricted. Blood flow is blocked by constriction and blood clots (
3045:
2930:
2846:
2788:
2337:
1908:
1070:"The mechanobiological theory: a unifying hypothesis on the pathogenesis of moyamoya disease based on a systematic review"
553:
170:, ischemic/hemorrhagic stroke, or seizure. The age distribution is bimodal being either young adolescence or mid-forties.
1767:
279:
or repeated strokes with severe functional impairment or even death. In others, the blockage may not cause any symptoms.
3014:
2388:
1835:
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954:
Janda, Paul; Bellew, Jonathan; Veerappan, Venkatachalam (2009). "Moyamoya disease: case report and literature review".
2524:
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1946:
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Kawakami, Akio; Aikawa, Masanori; Alcaide, Pilar; Luscinskas, Francis W.; Libby, Peter; Sacks, Frank M. (2006).
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3019:
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1984:
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Stage 3 Intensification of the moyamoya and defects of the anterior cerebral artery and middle cerebral artery
158:
occurs. On angiography, a "puff of smoke" appearance is seen, and the treatment of choice is surgical bypass.
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1979:
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Stage 6 Disappearance of the moyamoya and circulation only via external carotid artery and vertebral artery
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1234:
Moyamoya disease program: Moyamoya treatment videos | Boston
Children's Hospital . (n.d.). Retrieved from
487:
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1998:
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Hallemeier C, Rich K, Brubb R, Chicoine M, Moran C, Cross D, Zipfel G, Dacey R, Derdeyn CP (2006).
50:
T1-weighted MR image of moyamoya disease. Flow void in the basal ganglia is indicated by the arrow.
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318:(TIAs), sensorimotor paralysis (numbness and paralysis of the extremities), convulsions and/or
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2018:
2013:
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638:
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198:(10q23.3); and moyamoya disease-6 with achalasia (MYMY6; 615750) is caused by mutation in the
147:
86:
55:
423:), sickle cell disease, and neurofibromatosis type 1. There is also evidence that identifies
2826:
2697:
2692:
2477:
2472:
1772:
1725:
1682:
1547:
1502:
1464:
1423:
1415:
1372:
1331:
1323:
1201:
1166:
1081:
1035:
994:
926:
917:
877:
838:
828:
772:
725:
680:
630:
1579:—International website for information about diagnostics and treatment of moyamoya patients
2748:
2439:
1757:
1242:
913:"Epidemiological features of moyamoya disease in Japan: findings from a nationwide survey"
807:
424:
303:
294:
1590:—Sree Chitra Tirunal Institute for Medical Sciences and Technology, Trivandrum (SCTIMST)
364:
Stage 5 Reduction of the moyamoya and development of external carotid artery collaterals
42:
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2754:
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2168:
1931:
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1428:
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820:
287:
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881:
819:
Berry, James; Cortez, Vladimir; Toor, Harjyot; Saini, Harneel; Siddiqi, Javed (2020).
621:
Scott, R. Michael; Smith, Edward R. (2009). "Moyamoya
Disease and Moyamoya Syndrome".
338:
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521:
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232:
217:
195:
185:
1221:
897:
702:
650:
470:
have been used in frontal and parietal lobes with good neovascularisation achieved.
326:. Moreover, following a stroke, secondary bleeding may occur. Such bleeding, called
2327:
2197:
2180:
402:
396:
342:
Left: MIP reconstructed MR angiography of a 11-year-old girl with moyamoya disease.
213:
209:
205:
1400:
Koh, Eun-Jeong; Kim, Han-Na; Ma, Tian-Ze; Choi, Ha-Young; Kwak, Yong-Geun (2010).
131:, these collateral vessels have the appearance of a "puff of smoke", described as
1646:
1402:"Comparative analysis of serum proteomes of moyamoya disease and normal controls"
1377:
1360:
1024:"RNF213 polymorphism and moyamoya disease: A systematic review and meta-analysis"
999:
982:
777:
760:
361:
Stage 4 Minimization of the moyamoya and defects of the posterior cerebral artery
2286:
2247:
2189:
545:
298:
256:
128:
1670:
1506:
1327:
355:
Stage 2 Initiation of the moyamoya and dilatation of intracranial main arteries
293:
The arterial constrictions in moyamoya disease are unlike the constrictions in
2397:
2267:
1869:
1787:
1665:
1620:
1205:
1086:
498:
112:
97:
1514:
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729:
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634:
2687:
2679:
2645:
2499:
2402:
2089:
1842:
1170:
1154:
833:
319:
188:(17q25). Moyamoya disease-5 (MYMY5; 614042) is caused by mutation in the
124:
120:
1603:
1022:
You, Chao; Ma, Junpeng; Liu, Yi; Ma, Lu; Huang, Siqing; Li, Hao (2013).
17:
1974:
685:
668:
200:
2726:
2467:
2434:
1941:
1717:
1615:
269:
231:
Moyamoya disease can be either congenital or acquired. Patients with
180:
2982:
Spinal muscular atrophy with lower extremity predominance (SMALED)
330:, may also stem from rupture of the weak neovascular vessel walls.
306:. The artery also fills with blood clots, which may cause strokes.
208:(4q32). Loci for the disorder have been mapped to the short arm of
102:
Blood thinners, Calcium channel blockers, Anti-seizure medications.
1734:
379:
190:
1587:
1817:
800:
2140:
1686:
1153:
Hishikawa, Tomohito; Sugiu, Kenji; Date, Isao (August 2016).
1576:
1256:"A Puff of Smoke by Sarah Lippett review – growing pains"
406:
newspaper's graphic novel of the month in November 2019.
27:
Disease characterized by constriction of brain arteries
981:
Kuriyama S, Kusaka Y, Fujimura M, et al. (2008).
490:
on the surface of the brain and the bone is replaced.
1593:
2903:
2880:
2871:
2864:
2819:
2769:
2741:
2712:
2678:
2640:
2629:
2595:
2573:
2498:
2308:
2299:
2285:
2245:
2188:
2179:
2113:
2088:
2068:
2050:
2032:
2006:
1997:
1967:
1924:
1886:
1868:
1786:
1733:
1724:
1656:
1597:
956:
The Journal of the American Osteopathic Association
96:
85:
75:
64:
54:
35:
1192:Smith, Edward R. (2012). "Moyamoya Arteriopathy".
415:cellular changes that occur in cases of moyamoya.
282:The disease causes constrictions primarily in the
1155:"Moyamoya Disease: A Review of Clinical Research"
1148:
1146:
579:
577:
575:
573:
132:
1359:Gonzalez, Nestor; et al. (October 2023).
139:
2152:
1698:
1303:
1301:
1299:
1297:
1295:
8:
673:Developmental Medicine & Child Neurology
1583:Orphanet's disease page on moyamoya disease
669:"Moyamoya: Defining current knowledge gaps"
548:established that both moyamoya disease and
2877:
2868:
2637:
2305:
2296:
2185:
2159:
2145:
2137:
2003:
1730:
1705:
1691:
1683:
1594:
667:Ganesan, Vijeya; Smith, Edward R. (2015).
41:
32:
1468:
1427:
1376:
1335:
1085:
1039:
998:
930:
842:
832:
776:
684:
662:
660:
497:(encephalomyosynangiosis) procedure, the
394:). The book was praised in the newspaper
1274:"Best comics and graphic novels of 2019"
337:
1407:Journal of Korean Neurosurgical Society
1315:Journal of Korean Neurosurgical Society
616:
614:
612:
610:
608:
606:
569:
378:Often nuclear medicine studies such as
344:Right: healthy patient, for comparison.
2948:Distal hereditary motor neuronopathies
2621:Template:Demyelinating diseases of CNS
1194:Current Treatment Options in Neurology
1063:
1061:
1059:
799:Online Mendelian Inheritance in Man,
7:
2799:Idiopathic intracranial hypertension
1957:Partial anterior circulation infarct
3030:Infantile progressive bulbar palsy
1952:Total anterior circulation infarct
1753:Posterior cerebral artery syndrome
1552:10.1227/01.neu.0000333311.87554.9c
932:10.1161/01.STR.0000221787.70503.ca
25:
2733:Template:Cerebrovascular diseases
2536:Frontotemporal lobar degeneration
1743:Anterior cerebral artery syndrome
1470:10.1161/CIRCULATIONAHA.106.622514
1254:Cooke, Rachel (15 October 2019).
352:Stage 1 Narrowing of carotid fork
204:gene (139396) on the long arm of
194:gene (102620) on the long arm of
184:gene (613768) on the long arm of
2753:For more detailed coverage, see
2731:For more detailed coverage, see
2702:For more detailed coverage, see
2668:For more detailed coverage, see
2619:For more detailed coverage, see
2126:Cerebral venous sinus thrombosis
1546:(6): E1375–6, discussion E1376.
821:"Moyamoya: An Update and Review"
127:and thrombosis. On conventional
1904:Anterior spinal artery syndrome
1748:Middle cerebral artery syndrome
623:New England Journal of Medicine
479:encephaloduroarteriosynangiosis
166:Patients usually present with
1:
3046:Amyotrophic lateral sclerosis
2931:Hereditary spastic paraplegia
2789:Normal pressure hydrocephalus
1909:Vertebrobasilar insufficiency
882:10.1016/S0303-8467(97)00031-0
801:https://omim.org/entry/252350
718:J Neurol Neurosurg Psychiatry
554:Barrow Neurological Institute
3015:Progressive muscular atrophy
1378:10.1161/STR.0000000000000443
1000:10.1161/STROKEAHA.107.490714
778:10.1161/STROKEAHA.111.621300
212:(MYMY1) and the long arm of
2525:Primary progressive aphasia
827:. 12(10) (e10994): e10994.
761:"Moyamoya Disease in China"
460:superficial temporal artery
452:superficial temporal artery
226:transient ischaemic attacks
133:
3103:
2847:Hashimoto's encephalopathy
2553:Posterior cortical atrophy
2374:Striatonigral degeneration
2225:Cavernous sinus thrombosis
1878:Cerebellar stroke syndrome
1811:Lateral medullary syndrome
1507:10.1016/j.wneu.2016.04.083
1328:10.3340/jkns.2015.57.6.415
316:transient ischemic attacks
297:. In atherosclerosis, the
277:transient ischemic attacks
2953:Spinal muscular atrophies
2921:Primary lateral sclerosis
2779:Intracranial hypertension
2559:Creutzfeldt–Jakob disease
2103:Charcot–Bouchard aneurysm
1947:Transient ischemic attack
1914:Subclavian steal syndrome
1806:Medial medullary syndrome
1796:Brainstem stroke syndrome
1308:Smith, Edward R. (2015).
1206:10.1007/s11940-012-0195-4
1087:10.3171/2021.6.FOCUS21281
140:
49:
40:
3077:Cerebrovascular diseases
3020:Progressive bulbar palsy
2811:Intracranial hypotension
2794:Choroid plexus papilloma
2208:Herpesviral encephalitis
1985:Transient global amnesia
1832:Lateral pontine syndrome
1768:Dejerine–Roussy syndrome
1714:Cerebrovascular diseases
1420:10.3340/jkns.2010.48.1.8
1041:10.4103/0028-3886.107927
730:10.1136/jnnp-2011-301387
245:congenital heart disease
241:neurofibromatosis type 1
2532:Frontotemporal dementia
2218:Encephalitis lethargica
2076:Intracranial hemorrhage
1894:Carotid artery stenosis
1823:Medial pontine syndrome
1588:Moyamoya Disease Portal
1130:Genetics Home Reference
448:external carotid artery
284:internal carotid artery
270:ring finger protein 213
249:fibromuscular dysplasia
156:internal carotid artery
2837:Hepatic encephalopathy
550:arteriovenous fistulas
488:middle cerebral artery
464:middle cerebral artery
345:
224:frequently experience
117:collateral circulation
2895:Ataxia–telangiectasia
2852:Static encephalopathy
2575:Mitochondrial disease
2413:Spasmodic torticollis
2323:Basal ganglia disease
2098:Intracranial aneurysm
1887:Extracranial arteries
1310:"Moyamoya Biomarkers"
869:Clin Neurol Neurosurg
635:10.1056/NEJMra0804622
410:Associated biomarkers
341:
2842:Toxic encephalopathy
2548:Lewy bodies dementia
2034:Cerebral/Intra-axial
1980:Binswanger's disease
834:10.7759/cureus.10994
2890:Friedreich's ataxia
2273:Meningoencephalitis
2213:Limbic encephalitis
2121:Cerebral vasculitis
1999:Haemorrhagic stroke
1937:Cerebral infarction
1159:Acta Medica Okayama
1074:Neurosurgical Focus
584:Mayo Clinic Staff.
506:multiple burr holes
328:hemorrhagic strokes
2926:Pseudobulbar palsy
2664:Status epilepticus
2615:Multiple sclerosis
2452:Myoclonic epilepsy
2315:movement disorders
2261:Acute disseminated
2203:Viral encephalitis
2060:Duret haemorrhages
1657:External resources
1495:World Neurosurgery
1280:. 30 November 2019
1241:2022-01-03 at the
1171:10.18926/amo/54497
1126:"Moyamoya disease"
806:2021-04-16 at the
686:10.1111/dmcn.12708
586:"Moyamoya disease"
444:Antiplatelet drugs
346:
237:sickle cell anemia
3064:
3063:
3060:
3059:
3056:
3055:
2860:
2859:
2765:
2764:
2704:Template:Headache
2670:Template:Epilepsy
2591:
2590:
2565:Vascular dementia
2408:Status dystonicus
2281:
2280:
2256:Encephalomyelitis
2134:
2133:
2084:
2083:
1993:
1992:
1858:Claude's syndrome
1853:Benedikt syndrome
1680:
1679:
1371:(10): e465–e479.
876:(Suppl 2): S1–5.
629:(12): 1226–1237.
481:) procedure is a
106:
105:
87:Diagnostic method
30:Medical condition
16:(Redirected from
3094:
2878:
2869:
2827:Brain herniation
2638:
2478:Intention tremor
2473:Essential tremor
2338:Postencephalitic
2306:
2297:
2186:
2167:Diseases of the
2161:
2154:
2147:
2138:
2042:Intraventricular
2004:
1848:Weber's syndrome
1773:Watershed stroke
1763:Moyamoya disease
1731:
1726:Ischaemic stroke
1707:
1700:
1693:
1684:
1671:Moyamoya disease
1595:
1564:
1563:
1533:
1527:
1526:
1489:
1483:
1482:
1472:
1448:
1442:
1441:
1431:
1397:
1391:
1390:
1380:
1356:
1350:
1349:
1339:
1305:
1290:
1289:
1287:
1285:
1270:
1264:
1263:
1251:
1245:
1232:
1226:
1225:
1189:
1183:
1182:
1150:
1141:
1140:
1138:
1136:
1122:
1116:
1115:
1089:
1065:
1054:
1053:
1043:
1019:
1013:
1012:
1002:
978:
972:
971:
951:
945:
944:
934:
925:(6): 1490–1496.
908:
902:
901:
863:
857:
856:
846:
836:
816:
810:
797:
791:
790:
780:
756:
750:
749:
713:
707:
706:
688:
664:
655:
654:
618:
601:
600:
598:
596:
581:
544:In June 2008, a
390:(published with
386:disease, called
145:
143:
142:
136:
109:Moyamoya disease
45:
36:Moyamoya disease
33:
21:
3102:
3101:
3097:
3096:
3095:
3093:
3092:
3091:
3082:Types of stroke
3067:
3066:
3065:
3052:
2978:Congenital DSMA
2899:
2856:
2815:
2761:
2749:Sleep disorders
2737:
2714:Cerebrovascular
2708:
2674:
2631:
2625:
2587:
2569:
2494:
2440:Choreoathetosis
2313:
2290:
2277:
2241:
2175:
2165:
2135:
2130:
2109:
2080:
2064:
2046:
2028:
1989:
1963:
1920:
1882:
1864:
1782:
1758:Amaurosis fugax
1720:
1711:
1681:
1676:
1675:
1652:
1651:
1606:
1573:
1568:
1567:
1535:
1534:
1530:
1491:
1490:
1486:
1450:
1449:
1445:
1399:
1398:
1394:
1358:
1357:
1353:
1307:
1306:
1293:
1283:
1281:
1272:
1271:
1267:
1253:
1252:
1248:
1243:Wayback Machine
1233:
1229:
1191:
1190:
1186:
1152:
1151:
1144:
1134:
1132:
1124:
1123:
1119:
1067:
1066:
1057:
1028:Neurology India
1021:
1020:
1016:
980:
979:
975:
962:(10): 547–553.
953:
952:
948:
910:
909:
905:
865:
864:
860:
818:
817:
813:
808:Wayback Machine
798:
794:
758:
757:
753:
715:
714:
710:
666:
665:
658:
620:
619:
604:
594:
592:
583:
582:
571:
566:
542:
530:
441:
425:hyperthyroidism
412:
388:A Puff of Smoke
343:
336:
295:atherosclerosis
265:
263:Pathophysiology
255:resistance, or
176:
164:
137:
31:
28:
23:
22:
15:
12:
11:
5:
3100:
3098:
3090:
3089:
3084:
3079:
3069:
3068:
3062:
3061:
3058:
3057:
3054:
3053:
3051:
3050:
3049:
3048:
3037:
3036:
3035:
3034:
3033:
3032:
3027:
3017:
3012:
3011:
3010:
3005:
3000:
2999:
2998:
2993:
2988:
2980:
2975:
2970:
2965:
2960:
2950:
2936:
2935:
2934:
2933:
2928:
2923:
2909:
2907:
2901:
2900:
2898:
2897:
2892:
2886:
2884:
2875:
2866:
2862:
2861:
2858:
2857:
2855:
2854:
2849:
2844:
2839:
2834:
2829:
2823:
2821:
2817:
2816:
2814:
2813:
2808:
2806:Cerebral edema
2803:
2802:
2801:
2796:
2791:
2786:
2775:
2773:
2767:
2766:
2763:
2762:
2760:
2759:
2758:
2757:
2755:Template:Sleep
2745:
2743:
2739:
2738:
2736:
2735:
2729:
2724:
2718:
2716:
2710:
2709:
2707:
2706:
2700:
2695:
2690:
2684:
2682:
2676:
2675:
2673:
2672:
2666:
2661:
2656:
2650:
2648:
2635:
2627:
2626:
2624:
2623:
2617:
2612:
2607:
2601:
2599:
2593:
2592:
2589:
2588:
2586:
2585:
2583:Leigh syndrome
2579:
2577:
2571:
2570:
2568:
2567:
2556:
2555:
2550:
2545:
2544:
2543:
2529:
2528:
2527:
2522:
2521:
2520:
2504:
2502:
2496:
2495:
2493:
2492:
2487:
2482:
2481:
2480:
2475:
2464:
2463:
2462:
2461:
2456:
2455:
2454:
2444:
2443:
2442:
2432:
2427:
2426:
2425:
2420:
2415:
2410:
2394:
2393:
2392:
2391:
2386:
2381:
2376:
2371:
2370:
2369:
2359:
2358:
2357:
2347:
2346:
2345:
2340:
2335:
2319:
2317:
2310:Extrapyramidal
2303:
2294:
2292:encephalopathy
2283:
2282:
2279:
2278:
2276:
2275:
2270:
2265:
2264:
2263:
2252:
2250:
2243:
2242:
2240:
2239:
2238:
2237:
2227:
2222:
2221:
2220:
2215:
2210:
2205:
2194:
2192:
2183:
2177:
2176:
2169:nervous system
2166:
2164:
2163:
2156:
2149:
2141:
2132:
2131:
2129:
2128:
2123:
2117:
2115:
2111:
2110:
2108:
2107:
2106:
2105:
2094:
2092:
2086:
2085:
2082:
2081:
2079:
2078:
2072:
2070:
2066:
2065:
2063:
2062:
2056:
2054:
2048:
2047:
2045:
2044:
2038:
2036:
2030:
2029:
2027:
2026:
2021:
2016:
2010:
2008:
2001:
1995:
1994:
1991:
1990:
1988:
1987:
1982:
1977:
1971:
1969:
1965:
1964:
1962:
1961:
1960:
1959:
1954:
1949:
1942:Classification
1939:
1934:
1932:Brain ischemia
1928:
1926:
1925:Classification
1922:
1921:
1919:
1918:
1917:
1916:
1906:
1901:
1896:
1890:
1888:
1884:
1883:
1881:
1880:
1874:
1872:
1866:
1865:
1863:
1862:
1861:
1860:
1855:
1850:
1840:
1839:
1838:
1836:Millard-Gubler
1829:
1815:
1814:
1813:
1808:
1798:
1792:
1790:
1784:
1783:
1781:
1780:
1778:Lacunar stroke
1775:
1770:
1765:
1760:
1755:
1750:
1745:
1739:
1737:
1728:
1722:
1721:
1712:
1710:
1709:
1702:
1695:
1687:
1678:
1677:
1674:
1673:
1661:
1660:
1658:
1654:
1653:
1650:
1649:
1638:
1623:
1607:
1602:
1601:
1599:
1598:Classification
1592:
1591:
1585:
1580:
1572:
1571:External links
1569:
1566:
1565:
1528:
1484:
1463:(7): 681–687.
1443:
1392:
1351:
1291:
1265:
1246:
1227:
1200:(6): 549–556.
1184:
1165:(4): 229–236.
1142:
1117:
1055:
1014:
973:
946:
903:
858:
811:
792:
751:
708:
679:(9): 786–787.
656:
602:
568:
567:
565:
562:
541:
538:
529:
526:
440:
437:
411:
408:
369:
368:
365:
362:
359:
356:
353:
335:
332:
288:basilar artery
264:
261:
175:
172:
163:
160:
104:
103:
100:
94:
93:
89:
83:
82:
79:
73:
72:
68:
62:
61:
58:
52:
51:
47:
46:
38:
37:
29:
26:
24:
14:
13:
10:
9:
6:
4:
3:
2:
3099:
3088:
3085:
3083:
3080:
3078:
3075:
3074:
3072:
3047:
3044:
3043:
3042:
3039:
3038:
3031:
3028:
3026:
3023:
3022:
3021:
3018:
3016:
3013:
3009:
3006:
3004:
3001:
2997:
2994:
2992:
2989:
2987:
2984:
2983:
2981:
2979:
2976:
2974:
2971:
2969:
2966:
2964:
2961:
2959:
2956:
2955:
2954:
2951:
2949:
2946:
2945:
2944:
2942:
2938:
2937:
2932:
2929:
2927:
2924:
2922:
2919:
2918:
2917:
2915:
2911:
2910:
2908:
2906:
2902:
2896:
2893:
2891:
2888:
2887:
2885:
2883:
2879:
2876:
2874:
2870:
2867:
2863:
2853:
2850:
2848:
2845:
2843:
2840:
2838:
2835:
2833:
2832:Reye syndrome
2830:
2828:
2825:
2824:
2822:
2818:
2812:
2809:
2807:
2804:
2800:
2797:
2795:
2792:
2790:
2787:
2785:
2784:Hydrocephalus
2782:
2781:
2780:
2777:
2776:
2774:
2772:
2768:
2756:
2752:
2751:
2750:
2747:
2746:
2744:
2740:
2734:
2730:
2728:
2725:
2723:
2720:
2719:
2717:
2715:
2711:
2705:
2701:
2699:
2696:
2694:
2691:
2689:
2686:
2685:
2683:
2681:
2677:
2671:
2667:
2665:
2662:
2660:
2657:
2655:
2652:
2651:
2649:
2647:
2643:
2639:
2636:
2634:
2628:
2622:
2618:
2616:
2613:
2611:
2608:
2606:
2603:
2602:
2600:
2598:
2597:Demyelinating
2594:
2584:
2581:
2580:
2578:
2576:
2572:
2566:
2563:
2562:
2561:
2560:
2554:
2551:
2549:
2546:
2542:
2539:
2538:
2537:
2533:
2530:
2526:
2523:
2519:
2516:
2515:
2514:
2511:
2510:
2509:
2506:
2505:
2503:
2501:
2497:
2491:
2488:
2486:
2485:Restless legs
2483:
2479:
2476:
2474:
2471:
2470:
2469:
2466:
2465:
2460:
2457:
2453:
2450:
2449:
2448:
2445:
2441:
2438:
2437:
2436:
2433:
2431:
2428:
2424:
2423:Blepharospasm
2421:
2419:
2416:
2414:
2411:
2409:
2406:
2405:
2404:
2401:
2400:
2399:
2396:
2395:
2390:
2387:
2385:
2382:
2380:
2379:Hemiballismus
2377:
2375:
2372:
2368:
2365:
2364:
2363:
2360:
2356:
2353:
2352:
2351:
2348:
2344:
2341:
2339:
2336:
2334:
2331:
2330:
2329:
2326:
2325:
2324:
2321:
2320:
2318:
2316:
2311:
2307:
2304:
2302:
2298:
2295:
2293:
2288:
2284:
2274:
2271:
2269:
2266:
2262:
2259:
2258:
2257:
2254:
2253:
2251:
2249:
2244:
2236:
2233:
2232:
2231:
2230:Brain abscess
2228:
2226:
2223:
2219:
2216:
2214:
2211:
2209:
2206:
2204:
2201:
2200:
2199:
2196:
2195:
2193:
2191:
2187:
2184:
2182:
2178:
2174:
2170:
2162:
2157:
2155:
2150:
2148:
2143:
2142:
2139:
2127:
2124:
2122:
2119:
2118:
2116:
2112:
2104:
2101:
2100:
2099:
2096:
2095:
2093:
2091:
2087:
2077:
2074:
2073:
2071:
2067:
2061:
2058:
2057:
2055:
2053:
2049:
2043:
2040:
2039:
2037:
2035:
2031:
2025:
2022:
2020:
2017:
2015:
2012:
2011:
2009:
2005:
2002:
2000:
1996:
1986:
1983:
1981:
1978:
1976:
1973:
1972:
1970:
1966:
1958:
1955:
1953:
1950:
1948:
1945:
1944:
1943:
1940:
1938:
1935:
1933:
1930:
1929:
1927:
1923:
1915:
1912:
1911:
1910:
1907:
1905:
1902:
1900:
1897:
1895:
1892:
1891:
1889:
1885:
1879:
1876:
1875:
1873:
1871:
1867:
1859:
1856:
1854:
1851:
1849:
1846:
1845:
1844:
1841:
1837:
1833:
1830:
1828:
1824:
1821:
1820:
1819:
1816:
1812:
1809:
1807:
1804:
1803:
1802:
1799:
1797:
1794:
1793:
1791:
1789:
1785:
1779:
1776:
1774:
1771:
1769:
1766:
1764:
1761:
1759:
1756:
1754:
1751:
1749:
1746:
1744:
1741:
1740:
1738:
1736:
1732:
1729:
1727:
1723:
1719:
1715:
1708:
1703:
1701:
1696:
1694:
1689:
1688:
1685:
1672:
1668:
1667:
1663:
1662:
1659:
1655:
1648:
1644:
1643:
1639:
1637:
1633:
1632:
1628:
1624:
1622:
1618:
1617:
1613:
1609:
1608:
1605:
1600:
1596:
1589:
1586:
1584:
1581:
1578:
1575:
1574:
1570:
1561:
1557:
1553:
1549:
1545:
1541:
1540:
1532:
1529:
1524:
1520:
1516:
1512:
1508:
1504:
1500:
1496:
1488:
1485:
1480:
1476:
1471:
1466:
1462:
1458:
1454:
1447:
1444:
1439:
1435:
1430:
1425:
1421:
1417:
1413:
1409:
1408:
1403:
1396:
1393:
1388:
1384:
1379:
1374:
1370:
1366:
1362:
1355:
1352:
1347:
1343:
1338:
1333:
1329:
1325:
1322:(6): 415–21.
1321:
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3040:
2939:
2912:
2873:Degenerative
2610:Inflammatory
2557:
2490:Stiff-person
2328:Parkinsonism
2301:Degenerative
2198:Encephalitis
2181:Inflammation
2171:, primarily
2024:Subarachnoid
1762:
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1539:Neurosurgery
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1282:. Retrieved
1278:The Guardian
1277:
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1260:The Guardian
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314:, recurrent
308:
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251:, activated
230:
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214:chromosome 8
210:chromosome 3
206:chromosome 4
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162:Presentation
152:
108:
107:
60:Neurosurgery
3025:Fazio–Londe
2865:Both/either
2659:Generalised
2518:Early-onset
2513:Alzheimer's
2248:spinal cord
2007:Extra-axial
1899:precerebral
1577:moyamoya.eu
1501:: 179–188.
1457:Circulation
1414:(1): 8–13.
1034:(1): 35–9.
993:(1): 42–7.
590:Mayo Clinic
546:case report
524:procedure.
483:synangiosis
257:head trauma
129:angiography
92:ultrasound.
3071:Categories
2633:paroxysmal
2605:Autoimmune
2398:Dyskinesia
2268:Meningitis
2246:Brain and
1870:Cerebellum
1788:Brain stem
1716:including
1666:Patient UK
564:References
499:temporalis
468:burr holes
113:thrombosis
98:Medication
71:movements.
3087:Syndromes
2630:Episodic/
2508:Tauopathy
2459:Akathisia
2447:Myoclonus
2430:Athetosis
2362:Tauopathy
2052:Brainstem
1827:Foville's
1515:1878-8769
1112:237388814
1096:1092-0684
1080:(3): E6.
746:207004883
528:Prognosis
462:(STA) to
439:Treatment
334:Diagnosis
324:headaches
253:protein C
56:Specialty
2996:SMALED2B
2991:SMALED2A
2688:Migraine
2680:Headache
2646:epilepsy
2642:Seizures
2500:Dementia
2403:Dystonia
2090:Aneurysm
2019:Subdural
2014:Epidural
1843:Midbrain
1560:18824958
1523:27150649
1479:16894036
1438:20717506
1387:37609846
1346:26180608
1239:Archived
1222:37402408
1214:22865293
1179:27549666
1104:34469862
1050:23466837
1009:18048855
968:19861596
941:16645133
898:24203502
853:33209550
804:Archived
787:22020027
738:22378916
703:34816246
695:25683905
651:26151018
643:19297575
595:15 March
540:Research
320:migraine
148:Japanese
134:moyamoya
125:aneurysm
121:bleeding
66:Symptoms
18:Moyamoya
3008:SMA-PME
3003:SMA-PCH
2986:SMALED1
2698:Tension
2693:Cluster
2418:Meige's
2235:Amoebic
2069:General
1975:CADASIL
1801:Medulla
1647:D009072
1429:2916155
1337:4502237
1284:22 June
890:9409395
844:7667711
513:STA-MCA
511:In the
504:In the
493:In the
450:or the
312:strokes
201:GUCY1A3
2727:Stroke
2541:Pick's
2468:Tremor
2435:Chorea
1718:stroke
1558:
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1365:Stroke
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1135:May 6,
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987:Stroke
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918:Stroke
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825:Cureus
785:
765:Stroke
744:
736:
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693:
649:
641:
322:-like
181:RNF213
3041:both:
2973:DSMA1
2968:SMAX2
2963:SMAX1
2943:only:
2916:only:
2820:Other
2742:Other
2654:Focal
2287:Brain
2190:Brain
2114:Other
1968:Other
1735:Brain
1636:437.5
1621:I67.5
1218:S2CID
1108:S2CID
894:S2CID
742:S2CID
699:S2CID
647:S2CID
380:SPECT
304:lumen
299:walls
191:ACTA2
174:Cause
115:). A
2644:and
2355:PKAN
2350:NBIA
1818:Pons
1642:MeSH
1631:9-CM
1556:PMID
1519:PMID
1511:ISSN
1475:PMID
1434:PMID
1383:PMID
1342:PMID
1286:2020
1210:PMID
1175:PMID
1137:2019
1100:PMID
1092:ISSN
1046:PMID
1005:PMID
964:PMID
937:PMID
886:PMID
849:PMID
783:PMID
734:PMID
691:PMID
639:PMID
597:2023
475:EDAS
473:The
141:ă‚‚ă‚„ă‚‚ă‚„
2958:SMA
2941:LMN
2914:UMN
2905:MND
2771:CSF
2722:TIA
2367:PSP
2343:NMS
2312:and
2173:CNS
1627:ICD
1612:ICD
1548:doi
1503:doi
1465:doi
1461:114
1424:PMC
1416:doi
1373:doi
1332:PMC
1324:doi
1202:doi
1167:doi
1082:doi
1036:doi
995:doi
960:109
927:doi
878:doi
839:PMC
829:doi
773:doi
726:doi
681:doi
631:doi
627:360
495:EMS
168:TIA
146:in
3073::
2882:SA
2389:OA
2384:HD
2333:PD
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