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Predictive value of brain 18F-FDG PET/CT in macrophagic myofasciitis?: A case report

Van Der Gucht, Axel MDa,*; Abulizi, Mukedaisi MDa; Blanc-Durand, Paul MDa; Aoun-Sebaiti, Mehdi MDb,c; Emsen, Berivan MDa; Gherardi, Romain K. MD, PhDb,d,e; Verger, Antoine MD, PhDf; Authier, François-Jérôme MD, PhDb,d,e; Itti, Emmanuel MD, PhDa,g

Section Editor(s): Rosca., Elena Cecilia

doi: 10.1097/MD.0000000000008134
Research Article: Clinical Case Report

Rationale: Although several functional studies have demonstrated that positron emission tomography/computed tomography with 18F-fluorodeoxyglucose (18F-FDG PET/CT) appears to be efficient to identify a cerebral substrate in patients with known macrophagic myofasciitis (MMF), the predictive value of this imaging technique for MMF remains unclear.

Patient concerns: We presented data and images of a 46-year-old woman.

Diagnoses: The patient was referred to our center for suspected MMF due to diffuse arthromyalgias and cognitive disorder (involving an impairment of visual selective attention and a weakness in executive functions revealed by neuropsychological assessment) which occurred few years after last vaccine injections.

Interventions: After a first negative deltoid muscle biopsy, a brain 18F-FDG PET/CT was performed and revealed the known spatial pattern of a cerebral glucose hypometabolism involving occipital cortex, medial temporal areas, and cerebellum.

Outcomes: Given the clinical suspicion of MMF and brain 18F-FDG PET/CT findings, a 2nd deltoid muscle biopsy was performed and confirmed the diagnosis of MMF with typical histopathological features.

Lessons: This case highlights the predictive value of brain 18F-FDG PET/CT as a noninvasive imaging tool for MMF diagnosis, even when muscle biopsy result comes back negative.

aDepartment of Nuclear Medicine, H. Mondor Hospital, Assistance Publique-Hôpitaux de Paris/Paris-Est University

bINSERM U955-Team 10

cDepartment of Neurology

dDepartment of Pathology, H. Mondor Hospital, Assistance Publique-Hôpitaux de Paris/Paris-Est University

eReference Center for Neuromuscular Disorders, H. Mondor Hospital, Assistance Publique-Hôpitaux de Paris, Créteil

fCHU Nancy, Nuclear Medecine and Nancyclotep Experimental Imaging Platform, Nancy

gINSERM U955-GRC Amyloid Research Institute, Créteil, France.

Correspondence: Axel Van Der Gucht, Department of Nuclear Medicine, Hôpital Henri Mondor, Assistance Publique-Hôpitaux de Paris, Paris-Est University, 51 Ave. du Mal de Lattre de Tassigny, Créteil, France (e-mail: axel.vandergucht@gmail.com).

Abbreviations: 18F-FDG = 18F-fluorodeoxyglucose, MMF = macrophagic myofasciitis, PET/CT = positron emission tomography/computed tomography.

Funding/support: This work was supported by grants from Région Ile-de-France and patient's association E3 M through “Partenariats institutions-citoyens pour la recherche et l’innovation” (PICRI programs 2010 and 2014), the Association Française contre les Myopathies though Translamuscle program, and A*MIDEX project (n° ANR-11-IDEX-0001–02) funded by the “Investissements d’Avenir” French Government program, managed by the French National Research Agency (ANR).

The authors have no conflicts of interest to disclose.

This is an open access article distributed under the Creative Commons Attribution-NoDerivatives License 4.0, which allows for redistribution, commercial and non-commercial, as long as it is passed along unchanged and in whole, with credit to the author. http://creativecommons.org/licenses/by-nd/4.0

Received May 11, 2017

Received in revised form August 28, 2017

Accepted September 1, 2017

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1 Introduction

Macrophagic myofasciitis (MMF) is an unusual inflammatory myopathy characterized by specific muscle lesions. Lesions are assessing abnormal long-term persistence of aluminum hydroxide within macrophages at the site of previous vaccine injection containing aluminum hydroxide adjuvant particles. Evolution of this chronic disease is slow and symptoms (which typically include arthromyalgias, chronic fatigue, and a cognitive dysfunction) first may occur from months or years after the last vaccine injection.[1–5] Several positron emission tomography/computed tomography with 18F-fluorodeoxyglucose (18FFDG PET/CT) studies have investigated these cognitive disorders.[6,7] Although this imaging technique appears to be efficient to identify a cerebral substrate in patients with a known MMF, the predictive value of brain 18F-FDG PET/CT to diagnose MMF remains unclear.

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2 Case report

We presented the case of a 46-year-old woman which was referred to our center for suspected MMF due to chronic diffuse arthromyalgias, fatigue, and cognitive impairment which occurred at age 37, in the context of multiple aluminum hydroxide-based vaccines administration (hepatitis B vaccine at age 27, diphteria/tetanus/polio at age 32, 33, and 43). A first deltoid muscle biopsy was performed at age 45 and was normal showing no inflammatory lesion. In spite of this result, the clinical probability of MMF was high, neurocognitive tests showing an impairment of visual selective attention and a weakness in executive functions. Therefore, a brain 18F-FDG PET/CT was performed as part of the work-up on a Gemini GXL instrument PET/CT scanner (Philips, Da Best, The Netherlands) after intravenous injection of 265 MBq of 18F-FDG. Informed consent was obtained. The patient was required to fast for at least 6 hours before undergoing the scan, had a normal blood sugar level, and an update neurosensory rest for 30 minutes. A low-dose helical CT was first performed for anatomical correlation and attenuation correction with the following parameters: X-ray tube tension of 120 kV, current of 80 to 100 mAs, rotation time 0.5 seconds, pitch 0.938, and slice thickness 2 mm. Images were reconstructed using line of response-row action maximum likelihood algorithm (2 iterations, 28 subsets, and postfilter 5.1 mm), with and without CT attenuation correction (matrix size of 512 × 512, voxel size 4 × 4 × 4 mm3). Visual analysis showed the known spatial pattern of a cerebral glucose hypometabolism involving occipital cortex, medial temporal areas, and cerebellum (Fig. 1).

Figure 1

Figure 1

Given the clinical suspicion of MMF and brain 18F-FDG PET/CT findings, deltoid muscle biopsy was reiterated at age 46 and confirmed the diagnosis of MMF with typical histopathological features (Fig. 2).

Figure 2

Figure 2

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3 Discussion

It is now well established that 18F-FDG PET imaging, which measures glucose consumption in neuron bodies, is a highly useful imaging modality for the diagnosis of neurodegenerative disorders, in particular for specific types of dementia such as frontotemporal dementia, Alzheimer disease, dementia with Lewy bodies and more recently in MMF each of which has characteristics metabolic patterns.[8,9] In a large series of 100 patients, we described a peculiar spatial pattern of a cerebral glucose hypometabolism involving occipital lobes, temporal lobes, limbic system, cerebellum, and frontoparietal cortices and showed that MMF is a slowly progressive or nonprogressive disease, in accordance with the fact that neurologic symptoms – even if they fluctuate – do not worsen or improve over time.[10,11]

The teaching point of this report is that brain 18F-FDG PET/CT should be performed in patients with suspected MMF and cognitive impairment. The risk of false-negative muscle biopsy is known, due to the heterogeneous and focal distribution of inflammatory sites.[12] Then, in case of suspected MMF associated with suggestive brain 18F-FDG PET pattern, muscle biopsy at site of vaccine injections may have to be repeated after an initial negative result. This case highlights the predictive value of the brain 18F-FDG PET/CT for MMF. Brain 18F-FDG PET/CT could be considered as a noninvasive imaging tool to diagnose MMF even when muscle biopsy result comes back negative. Further studies are warranted to validate our findings.

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Acknowledgments

The authors thank the grants from Région Ile-de-France and patient's association E3 M through “Partenariats institutions-citoyens pour la recherche et l’innovation” (PICRI programs 2010 and 2014), Association Française contre les Myopathies though Translamuscle program, and A*MIDEX project (no ANR-11-IDEX-0001–02) funded by the “Investissements d’Avenir” French Government program, managed by the French National Research Agency (ANR).

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References

[1]. Gherardi RK, Coquet M, Chérin P, et al. Macrophagic myofasciitis: an emerging entity. Groupe d’Etudes et Recherche sur les Maladies Musculaires Acquises et Dysimmunitaires (GERMMAD) de l’Association Française contre les Myopathies (AFM). Lancet 1998;352:347–52.
[2]. Gherardi RK, Coquet M, Cherin P, et al. Macrophagic myofasciitis lesions assess long-term persistence of vaccine-derived aluminium hydroxide in muscle. Brain J Neurol 2001;124:1821–31.
[3]. Authier F-J, Sauvat S, Champey J, et al. Chronic fatigue syndrome in patients with macrophagic myofasciitis. Arthritis Rheum 2003;48:569–70.
[4]. Rigolet M, Aouizerate J, Couette M, et al. Clinical features in patients with long-lasting macrophagic myofasciitis. Front Neurol 2014;5:230.
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[6]. Van Der Gucht A, Aoun Sebaiti M, Itti E, et al. Neuropsychological correlates of brain perfusion SPECT in patients with macrophagic myofasciitis. PloS One 2015;10:e0128353.
[7]. Van Der Gucht A, Aoun-Sebaiti M, Kauv P, et al. FDG-PET/CT brain findings in a patient with macrophagic myofasciitis. Nucl Med Mol Imaging 2016;50:80–4.
[8]. Schaller BJ. Strategies for molecular imaging dementia and neurodegenerative diseases. Neuropsychiatr Dis Treat 2008;4:585–612.
[9]. Berti V, Pupi A, Mosconi L. PET/CT in diagnosis of dementia. Ann N Y Acad Sci 2011;1228:81–92.
[10]. Van Der Gucht A, Aoun Sebaiti M, Guedj E, et al. Brain FDG-PET metabolic abnormalities in patients with long-lasting macrophagic myofasciitis. J Nucl Med 2017;58:492–8.
[11]. Blanc-Durand P, Van Der Gucht A, Aoun Sebaiti M, et al. Brain FDG-PET metabolic abnormalities in macrophagic myofasciitis: are they stable? J Nucl Med 2017;58:1532–3.
[12]. Gherardi RK, Authier FJ. Macrophagic myofasciitis: characterization and pathophysiology. Lupus 2012;21:184–9.
Keywords:

aluminum hydroxide; brain imaging; 18F-FDG; macrophagic myofasciitis; PET/CT

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