CASE REPORT
Nagoya J. Med. Sci. 85. 141–149, 2023
doi:10.18999/nagjms.85.1.141

Surgical revascularization for quasi-moyamoya
disease associated with polyneuropathy, organomegaly,
endocrinopathy, monoclonal gammopathy, andskin changes (POEMS) syndrome: a case reportand literature review
Yuki Kubota1, Yoshiki Hanaoka1, Jun-ichi Koyama2, Yusuke Takahashi3,
Nagaaki Katoh3, Mai Iwaya4, Yu Fujii1, Toshihiro Ogiwara1
and Tetsuyoshi Horiuchi1
Department of Neurosurgery, Shinshu University School of Medicine, Matsumoto, Japan2
Neuroendovascular Therapy Center, Shinshu University Hospital, Matsumoto, Japan
Department of Medicine (Neurology and Rheumatology), Shinshu University School of Medicine,Matsumoto, Japan
4
Department of Laboratory Medicine, Shinshu University Hospital, Matsumoto, Japan
13ABSTRACTPOEMS (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes)
syndrome is a rare multisystem disease characterized by plasma cell dyscrasia and overproduction ofvascular endothelial growth factor, which is related to disease activity. Recent treatment strategies have
improved survival of patients suffering from this disorder; however, ischemic stroke remains a poor
prognostic factor. POEMS patients with ischemic stroke frequently develop cerebral large artery stenosis/
occlusion, followed by progressive stroke. Post literature review, we present an ischemic stroke case ofquasi-moyamoya disease linked with this syndrome that was successfully treated with surgical revascularization. A 41-year-old woman diagnosed with POEMS syndrome developed progressive ischemic stroke due
to quasi-moyamoya disease, despite decreased vascular endothelial growth factor level with lenalidomide
and dexamethasone treatment. She underwent superficial temporal artery to middle cerebral artery bypass
with encephalo-duro-myo-synangiosis bilaterally. The postoperative course was uneventful. Two years
and five months after the stroke, neuroimaging demonstrated bypass patency, neovascularization after
encephalo-duro-myo-synangiosis, and no recurrence of stroke. Our case is the first to report successful
surgical revascularization for a POEMS patient. Surgical revascularization may be a useful treatment option
for patients with quasi-moyamoya disease associated with POEMS syndrome, especially for those whodevelop refractory ischemic stroke despite reduced vascular endothelial growth factor level.
Keywords: cerebral infarction, cerebral revascularization, Crow–Fukase syndrome, STA-MCA anastomosis,
vascular endothelial growth factor
Abbreviations:
ICA: internal carotid artery

Received: January 14, 2022; accepted: February 24, 2022
Corresponding Author: Yoshiki Hanaoka, MD, PhD
Department of Neurosurgery, Shinshu University School of Medicine,
3-1-1 Asahi, Matsumoto 390-8621, Japan
E-mail: hanaoka@shinshu-u.ac.jp
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Yuki Kubota et al123
I-IMP SPECT: N-isopropyl-p-[123I]-iodoamphetamine single photon emission computed tomography
MCA: middle cerebral artery
MRA: magnetic resonance angiography
STA: superficial temporal artery
POEMS: polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes
VEGF: vascular endothelial growth factor

This is an Open Access article distributed under the Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International
License. To view the details of this license, please visit (http://creativecommons.org/licenses/by-nc-nd/4.0/).

INTRODUCTION
Polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes(POEMS) syndrome is a rare paraneoplastic syndrome associated with plasma cell dyscrasia.1,2
Mandatory major criteria for the syndrome are polyneuropathy and monoclonal plasma cellproliferative disorder. Other major criteria are the presence of Castleman disease, scleroticbone lesions and vascular endothelial growth factor (VEGF) elevation. Minor criteria include
organomegaly, extravascular volume overload, endocrinopathy, skin changes, papilledema, andthrombocytosis/polycythemia. A diagnosis of POEMS syndrome is made when both of the mandatory major criteria, one of the other major criteria, and one of the minor criteria are present.2
Recent treatment strategies have favorably improved survival of POEMS patients.3 However,prognosis remains extremely poor for POEMS patients who developed ischemic stroke.4
To date, there has been no report of successful surgical revascularization for POEMS patients
who developed ischemic stroke. Herein, we present an ischemic stroke case of quasi-moyamoya
disease associated with POEMS syndrome, who successfully underwent surgical revascularization,and we review the literature on patients with quasi-moyamoya disease associated with POEMS
syndrome.

CASE REPORT
A 41-year-old woman was admitted to the department of neurology at our institution with a
6-month history of numbness and weakness in the lower extremities. The patient had no historyof smoking, drinking alcohol, taking any medications, diabetes mellitus and dyslipidemia, andno relevant family history. Physical and neurological examinations showed symmetrical pitting
edema, lower-extremity hypoesthesia/motor weakness, and absence of deep tendon reflexes. Nerve
conduction studies demonstrated demyelinating polyneuropathy. Computed tomography revealed
hepatomegaly, splenomegaly, and sclerotic bone lesions. Serum VEGF was high (7,350.0 pg/ml; normal < 38.3 pg/ml), and serum immunoelectrophoresis disclosed IgA lambda monoclonal
gammopathy. Thus, the patient was diagnosed with POEMS syndrome.5 Lenalidomide and dexamethasone treatment was started. Serum VEGF level decreased to 1,920.0 pg/ml after this therapy.
One month later, the patient presented with left facial palsy and hemiplegia. Diffusion-weighted
imaging revealed acute cerebral infarction lesions, including the right centrum semiovale (Fig.
1A). Magnetic resonance angiography (MRA) showed stenosis at the bilateral terminal internal
carotid arteries (ICA) (Fig. 1A). Dual antiplatelet therapy with aspirin (100 mg/day) and clopidogrel (75 mg/day) was started.
Five months later, the patient suddenly became apathetic and somnolent. The Mini-Mental State
Examination score was 16/30. Diffusion-weighted imaging showed acute cerebral infarction witha focus on the left frontal lobe (Fig. 1B). MRA revealed loss of signal intensity at the bilateral
terminal ICAs, proximal anterior cerebral arteries and proximal middle cerebral arteries (MCA)
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Cerebral revascularization in POEMS case(Fig. 1B). Carotid ultrasonography revealed no specific findings, except for maximal intima-media
thickness of the left common carotid artery (1.5 mm). The patient was referred to our department
for evaluations of progressive stroke. Angiography detected terminal ICA occlusion and presenceof basal moyamoya vessels bilaterally; the patient was diagnosed as having quasi-moyamoya
disease associated with POEMS syndrome (Fig. 2A, 2B). N-isopropyl-p-[123I]-iodoamphetamine
single photon emission computed tomography (123I-IMP SPECT) showed decreased cerebral bloodflow in the anterior cerebral artery and MCA territories bilaterally, predominantly on the left (Fig.
2C). To prevent further cerebral ischemic stroke, left superficial temporal artery to MCA bypass(STA–MCA bypass) with encephalo-duro-myo-synangiosis was conducted. One month after thesurgery, right STA-MCA bypass with encephalo-duro-myo-synangiosis was performed. The STA
was partially resected for histological analysis. The postoperative course was uneventful. The
patient’s consciousness recovered; the postoperative Mini-Mental State Examination score raised
to 25/30. Left facial palsy and hemiplegia gradually improved. Histologically, the STA showed
severe intimal thickening with fibrous proliferation (Fig. 3). The vessel wall had no inflammation,cholesterol crystals, or calcification.
Two years and five months after the last stroke, magnetic resonance imaging demonstrated
no recurrence of cerebral infarction (Fig. 4A). Angiography revealed the bypass patency andneovascularization after encephalo-duro-myo-synangiosis bilaterally (Fig. 4B, 4C). 123I-IMP SPECT
showed improved cerebral blood flow in the bilateral MCA territories (Fig. 4D). Lenalidomide

Fig. 1 Preoperative magnetic resonance imaging including diffusion-weighted imaging, apparent diffusion
coefficient maps, fluid-attenuated inversion recovery imaging, and magnetic resonance angiography (MRA)
Fig. 1A: Magnetic resonance imaging at the first stroke onset. MRA demonstrates stenosis at the bilateral terminalinternal carotid arteries (ICA).
Fig. 1B: Magnetic resonance imaging at the second stroke onset. MRA shows loss of signal intensity at thebilateral terminal ICA, proximal anterior cerebral arteries, and proximal middle cerebral arteries.
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Yuki Kubota et al

Fig. 2Preoperative cerebral angiography (A, B) and N-isopropyl-p-[123I]-iodoamphetamine
single photon emission computed tomography (123I-IMP SPECT) imaging (C)Fig. 2A, 2B: Preoperative common carotid angiography shows the terminal internal carotid artery (ICA) occlusionand presence of the basal moyamoya vessels bilaterally.
Fig. 2C: Preoperative 123I-IMP SPECT imaging shows decreased cerebral blood flow in the bilateral ICA territories,predominantly on the left.

Fig. 3

Histopathological pictures of resected superficial temporal artery (A, B: Hematoxylin-Eosin stain,
C: Elastica van Gieson stain, D: Alcian Brue PAS stain)
Stenotic superficial temporal artery without evident infiltration of inflammatory cells is shown (A). Dottedrectangle in A indicates the area of enlarged view shown in pictures B, C, and D. Asterisks in pictures B, C,and D indicate significantly thickened intima. Internal elastic membrane (arrowheads) was preserved (C). Alcian
Brue-positive acid mucopolysaccharide deposition in the thickened intima was detected (D).

and dexamethasone treatment successfully had suppressed serum VEGF level. The patient had
no ischemic stroke events clinically after the last stroke.
This study was approved by the institutional review board, and a written informed consent
for treatment was obtained from the patient.

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Cerebral revascularization in POEMS caseFig. 4 Postoperative magnetic resonance imaging (A), cerebral angiography (B), andN-isopropyl-p-[123I]-iodoamphetamine single photon emission computed tomography (123I-IMP SPECT)
imaging two years and five months after the last stroke
Fig. 4A: Postoperative magnetic resonance imaging shows no recurrence of ischemic stroke. In magnetic resonanceangiography, bilateral superficial temporal arteries and right middle meningeal artery were observed.
Fig. 4B: Postoperative lateral views of right external carotid artery angiography and three-dimensional rotational
angiography. Bypass patency (white arrows) and neovascularization via the right middle meningeal artery(white arrowheads) were identified.
Fig. 4C: Postoperative lateral views of left external carotid artery angiography and three-dimensional rotational
angiography. Bypass patency (yellow arrows) and neovascularization via the left deep temporal artery(yellow arrowheads) were identified.
Fig. 4D: Postoperative 123I-IMP SPECT imaging shows improved cerebral blood flow in the bilateral middle
cerebral artery territories.

DISCUSSIONPOEMS syndrome is a rare multisystem, autoinflammatory paraneoplastic condition, defined by
the presence of inflammatory peripheral neuropathy and a monoclonal plasma cell disorder.1,2 The
pathogenesis of POEMS syndrome is not fully understood. Monoclonal plasma cell dyscrasia may
influence an overproduction of proinflammatory cytokines with downstream effects.2 VEGF—apotent multifunctional cytokine responsible for angiogenesis, microvascular hyperpermeability, andinflammation of vascular endothelial cells—is markedly elevated in untreated POEMS comparedwith other hematologic malignancies and inflammatory neuropathies. Therefore, upregulated VEGF
is believed to play a pivotal role; it correlates with disease activity, prognosis, and response totherapy.6,7 POEMS syndrome is a potentially fatal disease, and patients’ quality of life deteriorates
due to progressive disabling neuropathy, massive pleural effusion or ascites, or thromboembolic
events. In the 1980s, the mean survival time was 33 months in 34 patients who were mainly
treated with corticosteroids.8 However, recent therapeutic strategies provide superior outcomes,
with overall 6-year progression-free survival of more than 50%.3 The 5-year progression-freeNagoya J. Med. Sci. 85. 141–149, 2023

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Yuki Kubota et al

survival rate is 88% in patients who achieved a complete hematologic response, and 50% including patients who do not achieve a complete hematologic response. For patients with a dominantsclerotic plasmacytoma, the first-line therapy is radiation therapy.2 Patients with diffuse sclerotic
lesions or disseminated bone marrow involvement, and those who have disease progression 3–6
months after radiation therapy, should receive systemic therapy, including alkylator-based therapy
and autologous peripheral stem cell transplantation, lenalidomide, thalidomide, or bortezomib.2
The response criteria is defined as follows: complete response (the normalization of the serum
VEGF level), improvement (50% reduction from the baseline serum VEGF level), and progression(50% increase from the lowest serum VEGF level).2
Recent reports have documented ischemic stroke occurring in 8%–13% of patients diagnosed
with POEMS syndrome.9,10 A retrospective cohort study demonstrated 93% of ischemic stroke
events occurred before or immediately after the diagnosis of POEMS symptoms, implying thatischemic stroke events occurred in POEMS patients not being treated effectively.9 A recent review
article has reported death within 2 years after the stroke events in 10 (55.6%) of 18 POEMS
patients with ischemic stroke; development of ischemic stroke is a predictor of unfavorable
prognosis.4 POEMS patients with ischemic stroke are highly associated with intracranial large
artery stenosis/occlusion, which can lead to multifocal, progressive, and refractory stroke.
To date, only two cases that developed quasi-moyamoya disease associated with POEMS
syndrome have been described (Table 1).11,12 Yamaguchi et al documented a 44-year-old woman
with a 5-year history of POEMS syndrome who presented with left putaminal hemorrhage.11
The serum VEGF level was well controlled after initial treatment. MRA showed progressive lossof signal intensity at the terminal ICAs and MCAs bilaterally, compared with 5 years earlier.
Angiography detected bilateral terminal ICA occlusion, M1 occlusion, and basal moyamoya
vessels. The authors concluded that the hemorrhage was caused by rupture of a fragile basal
moyamoya vessel. Conservative therapy was applied, and no rebleeding was observed 2 years
after the hemorrhage. Sekiguchi et al described a 45-year-old man with a 1-year history ofPOEMS syndrome, who presented with progressive cerebral infarction at the left frontal lobe.12
Bortezomib with dexamethasone treatment, and thalidomide treatment failed due to their sideeffects. MRA demonstrated progressive loss of signal intensity at the terminal ICAs, anterior
cerebral arteries, and MCAs bilaterally over 2 months. Angiography revealed bilateral terminal
ICA occlusion, A1 occlusion, M1 occlusion, and basal moyamoya vessels. Left STA-MCA bypass
with encephalo-duro-arterio-myo-synangiosis was conducted. However; the patient developed
acute exacerbation of heart failure and fatal recurrent ischemic stroke; he died 4 months aftersurgery. In all three cases (including our case), intracranial large artery stenosis/occlusion andmoyamoya vessels developed progressively within relatively short time (Table 1). In our case,therapeutic improvement (50% reduction from the baseline serum VEGF level) was obtained after
lenalidomide and dexamethasone treatment. However, the normalization of the serum VEGF level
was not achieved, which may have caused the progression of intracranial large artery stenosis/occlusion and moyamoya vessels. Thus, careful angiographic monitoring will be required forpatients with POEMS who suffered ischemic stroke. A review article described that 18 (64.3%)
of 28 POEMS cases with ischemic stroke involved intracranial large artery stenosis/occlusion.4
Based on contrast enhancement magnetic resonance imaging findings, some researchers havespeculated that “vasculitis” was the main mechanism underlying large artery stenosis/occlusion
associated with POEMS syndrome.4,13 However, based on histopathological findings, Sekiguchi
et al. revealed that intracranial large artery stenosis/occlusion associated with POEMS syndrome
resulted from duplication of internal elastic lamina and marked fibrous thickening localized
to intima, which was similar to moyamoya disease.12 Additionally, there was no evidence ofvasculitis, including inflammatory cell infiltration, multinucleated giant cells, or granulomas.12
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Cerebral revascularization in POEMS caseIn our case, histopathological analysis of the resected STA revealed an obvious stenotic lesion
with significantly thickened intima (Fig. 3). This finding indicates that not only the intracranialarteries but also the extracranial arteries can be affected in patients with quasi-moyamoya diseaseassociated with POEMS syndrome, which in turn, suggests the existence of underlying systemic
vasculopathy.5 Elevated systemic VEGF level induces hypertrophy/proliferation of endothelial

Table 1

Summary of case reports of quasi-moyamoya disease associated with POEMS syndrome

Age (years)/sex
POEMS syndrome

Yamaguchi et al11(2016)
49/female

Sekiguchi et al12(2020)45/male

The current case41/female

Favourably controlled

Bortezomib anddexamethasone followedby thalidomide a
Not controlled

Favourably controlled

5 years

1 year1 monthType

Left putaminal
haemorrhage

Progressive ischaemia
at left MCA territory

Symptoms

Right hemiparesis

Right recurrent
hemiparesis

Progressive ischaemia
at bilateral ACA andMCA territories
Left hemiparesis/
apathy

MRA and angiography

MRA and angiography
Aspirin and clopidogrel
Bilateral STA-MCA
bypass and EDMS
None
None at least 2 years
and 5 months
More than 2 years
and 5 monthsTreatment
Serum VEGF level
Stroke Time after POEMS
diagnosis

Radiation therapy

Quasi-moyamoya disease
Diagnosis
MRA and angiography
Treatment  Medication
None  Surgery

None

Histopathological study

NoneCilostazol
Left STA-MCA bypass
and EDAMS
Yes b

Recurrent stroke

None at least 2 years

Yes

Survival time after thelast stroke

More than 2 years

6 months

Lenalidomide anddexamethasone

Bortezomib and dexamethasone therapy and thalidomide therapy failed due to their side effects.
Histopathological findings revealed duplication of internal elastic lamina and marked fibrous thickening localised to intima of bilateral proximal MCAs which is similar to moyamoya disease. There
was no evidence of vasculitis, including inflammatory cell infiltration, multinucleated giant cells orgranulomas and no lipid-rich plaque suggesting atherosclerosis.
ACA: anterior cerebral artery
EDAMS: encephalo-duro- arterio-myo-synangiosis
EDMS: encephalo-duro-myo-synangiosis
MCA: middle cerebral artery
MRA: magnetic resonance angiography
POEMS: polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin changesSTA: superficial temporal artery
VEGF: vascular endothelial growth factor
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Yuki Kubota et alcells, which may lead to vessel stenosis/occlusion systemically.2,11 VEGF-induced angiogenesis
may result in fragile moyamoya vessels.11 Thus, the intracranial large artery stenosis/occlusion
associated with POEMS syndrome can be “irreversible” and “progressive” in nature. To prevent
stroke events (progression of quasi-moyamoya disease), decreased serum VEGF level should be
mandatory. In all three cases, therapeutic efficacy for POEMS syndrome may have affected thepatient’s prognosis (Table 1). According to a review article,4 intracranial large artery stenosis/
occlusion in POEMS patients who developed ischemic stroke was primarily diagnosed with MRA,
which may have overlooked occurrence of moyamoya vessels (diagnosis of quasi-moyamoya
disease). In all three cases, quasi-moyamoya disease was diagnosed based on cerebral angiography(Table 1). There can be a certain number of quasi-moyamoya disease associated with POEMSsyndrome.
Surgical revascularization for quasi-moyamoya disease has been reported useful in patientswith Graves’ disease and Down syndrome.14,15 To the best of our knowledge, our case is thefirst to report successful surgical revascularization for quasi-moyamoya disease associated withPOEMS syndrome. We applied surgical revascularization for our case because progressive
cerebral infarction and intracranial large artery stenosis/occlusion occurred despite satisfactory
control of the serum VEGF level. Mid-term follow-up results showed favorable outcome. Basedon postoperative neuroimaging findings, bilateral STA-MCA bypass with encephalo-duro-myosynangiosis will contribute to the prevention of recurrent ischemic stroke. Therefore, surgicalrevascularization may be a useful treatment option for patients with quasi-moyamoya diseaseassociated with POEMS syndrome, especially for those who develop progressive stroke despitewell-controlled serum VEGF level.

CONCLUSION
Patients with POEMS syndrome who have undergone ischemic stroke are frequently associated with intracranial large artery stenosis/occlusion, which can cause progressive stroke. Thus,
ischemic stroke remains a poor prognostic factor. To the best of our knowledge, the present
case is the first to demonstrate successful surgical revascularization of quasi-moyamoya diseaseassociated with POEMS syndrome. Hence, surgical revascularization is a potentially usefultreatment option for patients with quasi-moyamoya disease associated with POEMS syndrome,
especially for those who suffered progressive stroke despite well-controlled serum VEGF levels.

AUTHOR CONTRIBUTIONYK and YH contributed equally as first authors to this work.

CONFLICTS OF INTEREST
The authors have no conflicts of interest to disclose.

ETHICS APPROVALThis study was performed in accordance with the ethical standards of the 1964 Declarationof Helsinki and its later amendments.
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Cerebral revascularization in POEMS case

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