Twig-like middle cerebral artery as a variety of isolated middle cerebral artery disease with new vessel formation: illustrative case

Hideki Nakajima Department of Neurosurgery, Kansai Electric Power Hospital, Osaka, Japan

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Ryota Miyake Department of Neurosurgery, Kansai Electric Power Hospital, Osaka, Japan

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Katsuma Iwaki Department of Neurosurgery, Kansai Electric Power Hospital, Osaka, Japan

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Taku Hongo Department of Neurosurgery, Kansai Electric Power Hospital, Osaka, Japan

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Morio Takasaki Department of Neurosurgery, Kansai Electric Power Hospital, Osaka, Japan

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Yasuhiro Fujimoto Department of Neurosurgery, Kansai Electric Power Hospital, Osaka, Japan

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BACKGROUND

Twig-like middle cerebral artery (T-MCA) is reported as a rare vascular anomaly characterized by reconstitution of the M1 segment of the middle cerebral artery (MCA) by a plexiform network of small vessels. Although it is generally believed that the etiology of T-MCA is an embryological persistence, some investigators have suggested that T-MCA may be a secondary consequence. Here, the authors report a second case of de novo T-MCA formation and reconsider the concept of T-MCA in connection with isolated MCA disease.

OBSERVATIONS

A 40-year-old man’s brain magnetic resonance imaging (MRI) checkup showed moderate stenosis of the M1 segment of the left MCA. Annual MRI follow-up was planned, and when performed 1 year later, it showed occlusion of the M1 segment of the left MCA. Cerebral angiography revealed occlusion of that M1 segment with abnormal arterial networks. This case was diagnosed as de novo T-MCA. The patient has remained asymptomatic for 2 years.

LESSONS

The reports of de novo T-MCA indicate that acquired factors may be involved in the pathogenesis of T-MCA. We suggest that T-MCA should be defined as a variety of isolated MCA disease with new vessel formation.

ABBREVIATIONS

AMORE = Asymptomatic Moyamoya Registry study; MCA = middle cerebral artery; MMD = moyamoya disease; MRI = magnetic resonance imaging; NVF = new vessel formation; Q = questionable; T-MCA = twig-like middle cerebral artery

BACKGROUND

Twig-like middle cerebral artery (T-MCA) is reported as a rare vascular anomaly characterized by reconstitution of the M1 segment of the middle cerebral artery (MCA) by a plexiform network of small vessels. Although it is generally believed that the etiology of T-MCA is an embryological persistence, some investigators have suggested that T-MCA may be a secondary consequence. Here, the authors report a second case of de novo T-MCA formation and reconsider the concept of T-MCA in connection with isolated MCA disease.

OBSERVATIONS

A 40-year-old man’s brain magnetic resonance imaging (MRI) checkup showed moderate stenosis of the M1 segment of the left MCA. Annual MRI follow-up was planned, and when performed 1 year later, it showed occlusion of the M1 segment of the left MCA. Cerebral angiography revealed occlusion of that M1 segment with abnormal arterial networks. This case was diagnosed as de novo T-MCA. The patient has remained asymptomatic for 2 years.

LESSONS

The reports of de novo T-MCA indicate that acquired factors may be involved in the pathogenesis of T-MCA. We suggest that T-MCA should be defined as a variety of isolated MCA disease with new vessel formation.

ABBREVIATIONS

AMORE = Asymptomatic Moyamoya Registry study; MCA = middle cerebral artery; MMD = moyamoya disease; MRI = magnetic resonance imaging; NVF = new vessel formation; Q = questionable; T-MCA = twig-like middle cerebral artery

Twig-like middle cerebral artery (T-MCA) is reported as a rare vascular anomaly characterized by reconstitution of the M1 segment of the middle cerebral artery (MCA) by a plexiform network of small vessels.1 T-MCA was detected in 0.11%–1.17% of individuals who had undergone diagnostic angiography.2–4 Approximately 70% of those patients developed hemorrhagic stroke, 20% developed ischemic stroke, and a few remained asymptomatic.5 The most important differential diagnosis is moyamoya disease (MMD), but differentiation is no longer difficult according to the current diagnostic criteria of MMD.6 On the other hand, recent studies have reported isolated MCA disease with or without new vessel formation (NVF)7,8 and have indicated that with NVF, the disease is similar to T-MCA.

Although it is generally believed that the etiology of T-MCA is an embryological persistence of a plexiform network of small vessels,9,10 some investigators have suggested that T-MCA may be a secondary consequence.11–13 Its etiology remains controversial and unclear. In this article, we report a second case of de novo T-MCA formation and reconsider the concept of T-MCA in connection with isolated MCA disease.

Illustrative Case

A 40-year-old man was previously healthy and had no vascular risk factor for atherosclerosis. He exhibited no symptoms and no neurological deficit. A brain magnetic resonance imaging (MRI) checkup showed moderate stenosis of the M1 segment of the left MCA (Fig. 1A). Because the patient was asymptomatic and no abnormalities were seen in the brain parenchyma, an annual MRI follow-up was planned. The MRI follow-up performed 1 year later showed occlusion of the M1 segment of the left MCA (Fig. 1B) without cerebral infarction. Cerebral angiography revealed occlusion of that M1 segment with abnormal arterial networks (Fig. 2B). The vessel caliber of the M2 segment was nearly normal. There was no stenosis in the other vessels, and leptomeningeal anastomoses from the left distal anterior cerebral artery and left distal posterior cerebral artery were demonstrated (Fig. 2). Transdural collaterals from the external carotid systems were not visible, and 123I-iodoamphetamine single-photon emission computed tomography at rest showed no laterality. All the laboratory data were within normal ranges, including coagulation function, thyroid function, and collagen-related disease factor. This case was diagnosed as de novo T-MCA. Because the patient had been asymptomatic until then, only conservative follow-up was performed. He has remained asymptomatic for 2 years, and the results of follow-up MRI have been stable.

FIG. 1
FIG. 1

Magnetic resonance angiography (MRA) when the patient was 40 years old (A) showed moderate stenosis of the M1 segment of the left MCA. MRA when the patient was 41 (B) showed occlusion of the M1 segment of the left MCA.

FIG. 2
FIG. 2

Left internal carotid angiography (ICAG; B) showing occlusion of the M1 segment of the left MCA with abnormal arterial networks and leptomeningeal anastomoses from the left distal anterior cerebral artery. Bilateral ICAG (A, B) and right vertebral angiography (VAG; C) showing no stenosis in the other vessels. Right VAG (C) showing leptomeningeal anastomoses from the left distal posterior cerebral artery.

Patient Informed Consent

The necessary patient informed consent was obtained in this study.

Discussion

T-MCA is reported as a rare vascular anomaly characterized by reconstitution of the M1 segment of the MCA by a plexiform network of small vessels.1 The most important differential diagnosis is MMD, especially unilateral MMD. According to the current diagnostic criteria, MMD (and the other steno-occlusive diseases, including T-MCA) could be safely eliminated as differential diagnoses.6 The RNF213 variant is the susceptibility gene of MMD and is sometimes found in patients with T-MCA.14,15 But the association between the RNF213 variant and T-MCA remains unclear.

Although it is generally believed that the etiology of T-MCA is an embryological persistence of a plexiform network of small vessels,9,10 some investigators have suggested that T-MCA may be a secondary consequence.11–13 It is difficult to prove that T-MCA is a persistent congenital anomaly, and there is no definitive evidence to support the hypothesis that T-MCA is a persistent embryonal arterial network. There is also a theory that it is an acquired abnormality for the following reasons.16 1) Although cerebral hemisphere development requires the normal formation of the internal carotid artery and the MCA, patients with T-MCA have isolated abnormal changes in the M1 segment but typically develop cerebral hemispheres and a distal MCA.13 2) Most twig-like patterns are found only in the proximal M1, but similar morphological changes have also been reported in the distal MCA.17,18 3) There would be many pediatric cases if T-MCA were a congenital anomaly, but such reports are extremely rare.11,19 Ota and Komiyama13 suggested that T-MCA may be a secondary collateral after segmental steno-occlusive change of the proximal MCA and that this twig-like configuration may occur in the distal MCA or other arteries.12,13 No cases or evidence of acquired T-MCA formation have been reported so far, but the first case of de novo T-MCA formation was reported recently.16 Our case is the second, and these cases indicate that acquired factors may be involved in the pathogenesis of T-MCA.

Recent studies have reported on isolated MCA disease with or without NVF.7,8 The diagnostic points of the disease include unilateral MCA disease (stenosis over 50% or occlusion); no other intracranial steno-occlusive lesion; patients younger than 55 years; no atherosclerotic risk factor; and exclusion of vasculitis, dissection, and emboligenic sources. Isolated MCA disease with NVF is similar to T-MCA, and T-MCA may be defined as a variety of isolated MCA disease with NVF. The Asymptomatic Moyamoya Registry (AMORE) study, the largest prospective cohort study for asymptomatic MMD, is in progress.20 In that study, questionable (Q) cases in which the stenosis existed only in the proximal portion of the MCA were enrolled, as well as typical MMD cases. The AMORE study group mentioned that the Q cases may be almost identical to isolated MCA disease, and we speculate that a Q case identical to T-MCA may be defined as a variety of isolated MCA disease with NVF.

Observations

We report the second case of de novo T-MCA formation. The reports of de novo T-MCA indicate that acquired factors may be involved in the pathogenesis of T-MCA.

Lessons

T-MCA may be a secondary consequence, and we suggest that it should be defined as a variety of isolated MCA disease with NVF. Further case accumulation with long-term follow-up is necessary to elucidate the etiology and natural history of these diseases.

Author Contributions

Conception and design: Nakajima, Miyake, Hongo, Takasaki. Acquisition of data: Nakajima, Hongo. Analysis and interpretation of data: Nakajima, Hongo. Drafting the article: Nakajima, Hongo, Takasaki. Critically revising the article: Nakajima, Hongo, Takasaki. Reviewed submitted version of manuscript: Nakajima, Iwaki, Hongo, Takasaki. Approved the final version of the manuscript on behalf of all authors: Nakajima. Administrative/technical/material support: Nakajima, Fujimoto. Study supervision: Nakajima, Fujimoto.

References

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    Yamada D, Ishibashi R, Kinosada M, Kurosaki Y, Handa A, Chin M Aplastic or twig-like cerebral artery with short-term ischemia and bleeding. Jpn J Stroke. 2020;42(3):190195.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 2

    Gailloud P, Albayram S, Fasel JH, Beauchamp NJ, Murphy KJ Angiographic and embryologic considerations in five cases of middle cerebral artery fenestration. AJNR Am J Neuroradiol. 2002;23(4):585587.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 3

    Liu HM, Lai DM, Tu YK, Wang YH Aneurysms in twig-like middle cerebral artery. Cerebrovasc Dis. 2005;20(1):15.

  • 4

    Onoue K, Nguyen TN, Mian A, Dasenbrock H, Bedi H, Abdalkader M Twig-like middle cerebral arteries: clinical and radiological findings. Clin Imaging. 2021;73(1):3137.

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    • Search Google Scholar
    • Export Citation
  • 5

    Takarada A, Yanaka K, Onuma K, Nakamura K, Takahashi N, Ishikawa E Aplastic or twig-like middle cerebral artery harboring unruptured cerebral aneurysms treated by clipping and bypass surgery: illustrative case. J Neurosurg Case Lessons. 2021;2(9):CASE21360.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 6

    Kuroda S, Fujimura M, Takahashi J, et al. Diagnostic criteria for moyamoya disease – 2021 revised version. Neurol Med Chir (Tokyo). 2022;62(7):307312.

  • 7

    Ahn SH, Lee J, Kim YJ, et al. Isolated MCA disease in patients without significant atherosclerotic risk factors: a high-resolution magnetic resonance imaging study. Stroke. 2015;46(3):697703.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 8

    Liang M, Wang P, Ma Y, et al. New vessels formation in young strokes with isolated steno-occlusive MCA. Brain Behav. 2018;8(10):e01088.

  • 9

    Seo BS, Lee YS, Lee HG, Lee JH, Ryu KY, Kang DG Clinical and radiological features of patients with aplastic or twiglike middle cerebral arteries. Neurosurgery. 2012;70(6):14721480.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 10

    Uchiyama N Anomalies of the middle cerebral artery. Neurol Med Chir (Tokyo). 2017;57(6):261266.

  • 11

    Brinjikji W, Lanzino G Letter to the editor. Rete middle cerebral artery anomalies. J Neurosurg. 2019;131(2):653654.

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    Ota T Letter to the editor. Twig-like middle cerebral artery and moyamoya disease. J Neurosurg. 2022;137(6):18751876.

  • 13

    Ota T, Komiyama M Twig-like middle cerebral artery: Embryological persistence or secondary consequences? Interv Neuroradiol. 2021;27(4):584587.

  • 14

    Inoue A, Kohno K, Fukumoto S, et al. A case of ECA-MCA double anastomoses for hemorrhagic type of twig-like MCA. Article in Japanese. No Shinkei Geka. 2016;44(6):463471.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 15

    Inoue H, Oomura M, Nishikawa Y, Mase M, Matsukawa N Aplastic or twig-like middle cerebral artery and cardiogenic cerebral embolism mimicking moyamoya disease with RNF213 polymorphism: a case report. Interv Neuroradiol. 2022;28(6):634638.

    • PubMed
    • Search Google Scholar
    • Export Citation
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    Kajita M, Yanaka K, Hanai S, et al. De novo formation of twig-like middle cerebral artery: an illustrative case. Surg Neurol Int. 2023;14:192.

  • 17

    Fukuyama R, Yamamura K, Murata H, Miyatake S, Matsumoto N, Abe H Ruptured aneurysm of and an aplastic or twig-like middle cerebral artery with ring finger protein 213 mutation: a case report. Article in Japanese. No Shinkei Geka. 2020;48(6):533540.

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    Goto Y, Oka H, Hiraizumi S, et al. Aplastic or twig-like middle cerebral artery presenting with intracerebral hemorrhage during pregnancy: report of two cases. World Neurosurg X. 2019;2(1):100018.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 19

    Matsunaga Y, Tsutsumi K, Morofuji Y, et al. A pediatric case of aplastic or twig-like middle cerebral artery with intracerebral hemorrhage. Surg Cereb Stroke. 2014;41(1):5157.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 20

    Kuroda S, Yamamoto S, Funaki T, et al. Five-year stroke risk and its prediction in asymptomatic moyamoya disease: Asymptomatic Moyamoya Registry (AMORE). Stroke. 2023;54(6):14941504.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • Collapse
  • Expand
  • FIG. 1

    Magnetic resonance angiography (MRA) when the patient was 40 years old (A) showed moderate stenosis of the M1 segment of the left MCA. MRA when the patient was 41 (B) showed occlusion of the M1 segment of the left MCA.

  • FIG. 2

    Left internal carotid angiography (ICAG; B) showing occlusion of the M1 segment of the left MCA with abnormal arterial networks and leptomeningeal anastomoses from the left distal anterior cerebral artery. Bilateral ICAG (A, B) and right vertebral angiography (VAG; C) showing no stenosis in the other vessels. Right VAG (C) showing leptomeningeal anastomoses from the left distal posterior cerebral artery.

  • 1

    Yamada D, Ishibashi R, Kinosada M, Kurosaki Y, Handa A, Chin M Aplastic or twig-like cerebral artery with short-term ischemia and bleeding. Jpn J Stroke. 2020;42(3):190195.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 2

    Gailloud P, Albayram S, Fasel JH, Beauchamp NJ, Murphy KJ Angiographic and embryologic considerations in five cases of middle cerebral artery fenestration. AJNR Am J Neuroradiol. 2002;23(4):585587.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 3

    Liu HM, Lai DM, Tu YK, Wang YH Aneurysms in twig-like middle cerebral artery. Cerebrovasc Dis. 2005;20(1):15.

  • 4

    Onoue K, Nguyen TN, Mian A, Dasenbrock H, Bedi H, Abdalkader M Twig-like middle cerebral arteries: clinical and radiological findings. Clin Imaging. 2021;73(1):3137.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 5

    Takarada A, Yanaka K, Onuma K, Nakamura K, Takahashi N, Ishikawa E Aplastic or twig-like middle cerebral artery harboring unruptured cerebral aneurysms treated by clipping and bypass surgery: illustrative case. J Neurosurg Case Lessons. 2021;2(9):CASE21360.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 6

    Kuroda S, Fujimura M, Takahashi J, et al. Diagnostic criteria for moyamoya disease – 2021 revised version. Neurol Med Chir (Tokyo). 2022;62(7):307312.

  • 7

    Ahn SH, Lee J, Kim YJ, et al. Isolated MCA disease in patients without significant atherosclerotic risk factors: a high-resolution magnetic resonance imaging study. Stroke. 2015;46(3):697703.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 8

    Liang M, Wang P, Ma Y, et al. New vessels formation in young strokes with isolated steno-occlusive MCA. Brain Behav. 2018;8(10):e01088.

  • 9

    Seo BS, Lee YS, Lee HG, Lee JH, Ryu KY, Kang DG Clinical and radiological features of patients with aplastic or twiglike middle cerebral arteries. Neurosurgery. 2012;70(6):14721480.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 10

    Uchiyama N Anomalies of the middle cerebral artery. Neurol Med Chir (Tokyo). 2017;57(6):261266.

  • 11

    Brinjikji W, Lanzino G Letter to the editor. Rete middle cerebral artery anomalies. J Neurosurg. 2019;131(2):653654.

  • 12

    Ota T Letter to the editor. Twig-like middle cerebral artery and moyamoya disease. J Neurosurg. 2022;137(6):18751876.

  • 13

    Ota T, Komiyama M Twig-like middle cerebral artery: Embryological persistence or secondary consequences? Interv Neuroradiol. 2021;27(4):584587.

  • 14

    Inoue A, Kohno K, Fukumoto S, et al. A case of ECA-MCA double anastomoses for hemorrhagic type of twig-like MCA. Article in Japanese. No Shinkei Geka. 2016;44(6):463471.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 15

    Inoue H, Oomura M, Nishikawa Y, Mase M, Matsukawa N Aplastic or twig-like middle cerebral artery and cardiogenic cerebral embolism mimicking moyamoya disease with RNF213 polymorphism: a case report. Interv Neuroradiol. 2022;28(6):634638.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 16

    Kajita M, Yanaka K, Hanai S, et al. De novo formation of twig-like middle cerebral artery: an illustrative case. Surg Neurol Int. 2023;14:192.

  • 17

    Fukuyama R, Yamamura K, Murata H, Miyatake S, Matsumoto N, Abe H Ruptured aneurysm of and an aplastic or twig-like middle cerebral artery with ring finger protein 213 mutation: a case report. Article in Japanese. No Shinkei Geka. 2020;48(6):533540.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 18

    Goto Y, Oka H, Hiraizumi S, et al. Aplastic or twig-like middle cerebral artery presenting with intracerebral hemorrhage during pregnancy: report of two cases. World Neurosurg X. 2019;2(1):100018.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 19

    Matsunaga Y, Tsutsumi K, Morofuji Y, et al. A pediatric case of aplastic or twig-like middle cerebral artery with intracerebral hemorrhage. Surg Cereb Stroke. 2014;41(1):5157.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 20

    Kuroda S, Yamamoto S, Funaki T, et al. Five-year stroke risk and its prediction in asymptomatic moyamoya disease: Asymptomatic Moyamoya Registry (AMORE). Stroke. 2023;54(6):14941504.

    • PubMed
    • Search Google Scholar
    • Export Citation

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