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Moyamoya Syndrome: A Window of Moyamoya Disease.

Phi JH, Wang KC, Lee JY, Kim SK - J Korean Neurosurg Soc (2015)

Bottom Line: The study of moyamoya syndrome can provide clinicians and researchers with valuable knowledge and insight.Although it is infrequently encountered in clinical practice, moyamoya-like vasculopathy can severely complicate outcomes for patients with various underlying diseases when the clinician fails to expect or diagnose moyamoya syndrome development.More comprehensive survey and investigation are required to uncover the secrets of all the moyamoya-like phenomena.

View Article: PubMed Central - PubMed

Affiliation: Division of Pediatric Neurosurgery, Seoul National University Children's Hospital, Seoul National University College of Medicine, Seoul, Korea.

ABSTRACT
Moyamoya-like vasculopathy develops in association with various systemic diseases and conditions, which is termed moyamoya syndrome. Relatively common diseases and conditions are related to moyamoya syndrome, including neurofibromatosis type 1, Down syndrome, thyroid disease, and cranial irradiation. Moyamoya syndrome shares phenotypical characteristics with idiopathic moyamoya disease. However, they differ in other details, including clinical presentations, natural history, and treatment considerations. The study of moyamoya syndrome can provide clinicians and researchers with valuable knowledge and insight. Although it is infrequently encountered in clinical practice, moyamoya-like vasculopathy can severely complicate outcomes for patients with various underlying diseases when the clinician fails to expect or diagnose moyamoya syndrome development. Furthermore, moyamoya syndrome could be used as a doorway to more enigmatic moyamoya disease in research. More comprehensive survey and investigation are required to uncover the secrets of all the moyamoya-like phenomena.

No MeSH data available.


Related in: MedlinePlus

A 9-year-old girl with Down syndrome had recurrent vomiting for 2 years. Examination of gastrointestinal system revealed no causes of the vomiting. A : A brain magnetic resonance image (MRI) was taken and an old small infarction was found in the left anterior watershed zone. The left middle cerebral artery trunk was invisible (not shown). B : A delayed time-to-peak in the left middle cerebral artery territory was observed in perfusion MRI. C and D : A subsequent angiography revealed a normal-looking right internal carotid artery and a near occlusion of left distal internal carotid artery with minute collateral vessel formation. Moyamoya syndrome was diagnosed. Although it was unclear that her vomiting was related to the cerebral vasculopathy, a pre-existing infarction warranted a surgical intervention. Encephaloduroarteriosynangiosis was performed on the left side. After surgery, her vomiting frequency decreased significantly. This patient illustrates the difficulty in evaluating neurological symptoms in Down syndrome patients.
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Figure 2: A 9-year-old girl with Down syndrome had recurrent vomiting for 2 years. Examination of gastrointestinal system revealed no causes of the vomiting. A : A brain magnetic resonance image (MRI) was taken and an old small infarction was found in the left anterior watershed zone. The left middle cerebral artery trunk was invisible (not shown). B : A delayed time-to-peak in the left middle cerebral artery territory was observed in perfusion MRI. C and D : A subsequent angiography revealed a normal-looking right internal carotid artery and a near occlusion of left distal internal carotid artery with minute collateral vessel formation. Moyamoya syndrome was diagnosed. Although it was unclear that her vomiting was related to the cerebral vasculopathy, a pre-existing infarction warranted a surgical intervention. Encephaloduroarteriosynangiosis was performed on the left side. After surgery, her vomiting frequency decreased significantly. This patient illustrates the difficulty in evaluating neurological symptoms in Down syndrome patients.

Mentions: Moyamoya syndrome is observed in the Down syndrome population at a higher frequency than expected (Fig. 2). Although the association of these conditions has been recognized for a long time, it is a rare occurrence. There are scarce data regarding the incidence of concurrent moyamoya syndrome in Down syndrome patients. However, in a recently published study analyzing a nationwide admission database in the United States, the estimated Down syndrome prevalence was 3.8% in patients admitted with moyamoya disease21). The majority of patients were white and Hispanic, and 15% of the patients had ischemic strokes. This 3.8% concurrent prevalence may be an overestimate or limited to specific ethnic backgrounds, because Down syndrome is never represented in moyamoya disease populations to such a high degree in East Asian countries (personal communications).


Moyamoya Syndrome: A Window of Moyamoya Disease.

Phi JH, Wang KC, Lee JY, Kim SK - J Korean Neurosurg Soc (2015)

A 9-year-old girl with Down syndrome had recurrent vomiting for 2 years. Examination of gastrointestinal system revealed no causes of the vomiting. A : A brain magnetic resonance image (MRI) was taken and an old small infarction was found in the left anterior watershed zone. The left middle cerebral artery trunk was invisible (not shown). B : A delayed time-to-peak in the left middle cerebral artery territory was observed in perfusion MRI. C and D : A subsequent angiography revealed a normal-looking right internal carotid artery and a near occlusion of left distal internal carotid artery with minute collateral vessel formation. Moyamoya syndrome was diagnosed. Although it was unclear that her vomiting was related to the cerebral vasculopathy, a pre-existing infarction warranted a surgical intervention. Encephaloduroarteriosynangiosis was performed on the left side. After surgery, her vomiting frequency decreased significantly. This patient illustrates the difficulty in evaluating neurological symptoms in Down syndrome patients.
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4502236&req=5

Figure 2: A 9-year-old girl with Down syndrome had recurrent vomiting for 2 years. Examination of gastrointestinal system revealed no causes of the vomiting. A : A brain magnetic resonance image (MRI) was taken and an old small infarction was found in the left anterior watershed zone. The left middle cerebral artery trunk was invisible (not shown). B : A delayed time-to-peak in the left middle cerebral artery territory was observed in perfusion MRI. C and D : A subsequent angiography revealed a normal-looking right internal carotid artery and a near occlusion of left distal internal carotid artery with minute collateral vessel formation. Moyamoya syndrome was diagnosed. Although it was unclear that her vomiting was related to the cerebral vasculopathy, a pre-existing infarction warranted a surgical intervention. Encephaloduroarteriosynangiosis was performed on the left side. After surgery, her vomiting frequency decreased significantly. This patient illustrates the difficulty in evaluating neurological symptoms in Down syndrome patients.
Mentions: Moyamoya syndrome is observed in the Down syndrome population at a higher frequency than expected (Fig. 2). Although the association of these conditions has been recognized for a long time, it is a rare occurrence. There are scarce data regarding the incidence of concurrent moyamoya syndrome in Down syndrome patients. However, in a recently published study analyzing a nationwide admission database in the United States, the estimated Down syndrome prevalence was 3.8% in patients admitted with moyamoya disease21). The majority of patients were white and Hispanic, and 15% of the patients had ischemic strokes. This 3.8% concurrent prevalence may be an overestimate or limited to specific ethnic backgrounds, because Down syndrome is never represented in moyamoya disease populations to such a high degree in East Asian countries (personal communications).

Bottom Line: The study of moyamoya syndrome can provide clinicians and researchers with valuable knowledge and insight.Although it is infrequently encountered in clinical practice, moyamoya-like vasculopathy can severely complicate outcomes for patients with various underlying diseases when the clinician fails to expect or diagnose moyamoya syndrome development.More comprehensive survey and investigation are required to uncover the secrets of all the moyamoya-like phenomena.

View Article: PubMed Central - PubMed

Affiliation: Division of Pediatric Neurosurgery, Seoul National University Children's Hospital, Seoul National University College of Medicine, Seoul, Korea.

ABSTRACT
Moyamoya-like vasculopathy develops in association with various systemic diseases and conditions, which is termed moyamoya syndrome. Relatively common diseases and conditions are related to moyamoya syndrome, including neurofibromatosis type 1, Down syndrome, thyroid disease, and cranial irradiation. Moyamoya syndrome shares phenotypical characteristics with idiopathic moyamoya disease. However, they differ in other details, including clinical presentations, natural history, and treatment considerations. The study of moyamoya syndrome can provide clinicians and researchers with valuable knowledge and insight. Although it is infrequently encountered in clinical practice, moyamoya-like vasculopathy can severely complicate outcomes for patients with various underlying diseases when the clinician fails to expect or diagnose moyamoya syndrome development. Furthermore, moyamoya syndrome could be used as a doorway to more enigmatic moyamoya disease in research. More comprehensive survey and investigation are required to uncover the secrets of all the moyamoya-like phenomena.

No MeSH data available.


Related in: MedlinePlus