Neck Extensor Myopathy- A Treatable Cause of Dropped Head Syndrome

Neck Extensor Myopathy- A Treatable Cause of Dropped Head Syndrome | Research Square window.SnipcartSettings = { analytics: { enabled: false } }; (function() { var accessVector = localStorage.getItem('access_vector') || ''; window.dataLayer = window.dataLayer || []; if (accessVector) { window.dataLayer.push({ user: { profile: { profileInfo: { snid: accessVector } } } }); } })(); (function(w,d,s,l,i){w[l]=w[l]||[];w[l].push({'gtm.start':new Date().getTime(),event:'gtm.js'});var f=d.getElementsByTagName(s)[0],j=d.createElement(s),dl=l!='dataLayer'?'&l='+l:'';j.async=true;j.src='https://www.googletagmanager.com/gtm.js?id='+i+dl;f.parentNode.insertBefore(j,f);})(window,document,'script','dataLayer','GTM-K279D39R'); Browse Preprints In Review Journals COVID-19 Preprints AJE Video Bytes Research Tools Research Promotion AJE Professional Editing AJE Rubriq About Preprint Platform In Review Editorial Policies Our Team Advisory Board Help Center Sign In Submit a Preprint Cite Share Download PDF correspondence Neck Extensor Myopathy- A Treatable Cause of Dropped Head Syndrome Nibu Varghese, Vimal Chandraghosh K D, Deepak Menon, Anoop Kattadimmal, and 3 more This is a preprint; it has not been peer reviewed by a journal. https://doi.org/ 10.21203/rs.3.rs-4327338/v1 This work is licensed under a CC BY 4.0 License Status: Published Journal Publication published 13 Aug, 2024 Read the published version in Acta Neurologica Belgica → Version 1 posted You are reading this latest preprint version Abstract Dropped head syndrome (DHS) is characterized by a passively correctable chin-on-neck deformity in erect posture and can stem from a wide variety of neurological disorders spanning the neuraxis. . Neuromuscular disorders account for a major chunk of DHS and include disease of anterior horn cell diseases, polyradiculopathies and cervical plexopathies, disease of neuromuscular junction and myopathies. Isolated DHS without additional neurological features poses a management challenge, particularly because the symptoms can significantly impact the patient's quality of life and may not always respond to treatment..(Ref)Here we present a patient with isolated DHS with evaluation revealing isolated next extensor myopathy with remarkable response to treatment. Although isolated neck extensor myopathy typically exhibit poor immunomodulatory response, timely identification and early intervention probably can lead to a favourable outcome in a subgroup of patients. Dropped head syndrome (DHS) Isolated Neck Extensor Myopathy Non-inflammatory myopathy Figures Figure 1 Figure 2 Figure 3 Introduction A 44-year-old female presented with a two-month history of cervical pain, and progressive weakness of neck. She needed to support her neck up with hand and often neck fell back on erect posture. She had no prior comorbidities, history of trauma, or known neurological deficits. Examination revealed marked weakness in head extension with noticeable atrophy of neck extensors., Neck flexors were normal [FIGURE 1,and FIGURE 2, VIDEO 1]A. Rest of the neurological examination was prominently normal with a motor examination showing 5/5 power MRC grade, strength in upper and lower extremity Council (MRC) scale, with a normal sensory system and normal deep tendon reflexes (DTRs) in all extremities. There were no evidence for any lower motor neuron involvement such as wasting or fasciculations elsewhere. Clinical laboratory investigations were normal except for mildly elevated creatine phosphokinase (CPK) levels (268 IU/L)( biological reference change upto 145 IU/L )I . Blood inflammatory markers ESR and CRP were negative. Routine nerve conduction studies from upper and lower extremitites revealed normal motor and sensory parameters. Repetitive nerve stimulation (RNS) from ADM (expand), deltoid, trapezius, facial muscles were normal. Needle Electromyography (EMG) revealed active denervation in the form of fibrillations and positive sharp waves from C4, C5, C6 paraspinal muscles and upper trapezius muscle with large amplitude long duration motor unit action potentials (MUAPs) from upper trapezius and paraspinal muscles. Rest of the muscles including SCM, deltoid, rhomboides, supra and infraspinatii were normal.. MRI cervical spine and brachial plexus were normal. Her antibodies against ANA profile, c-ANCA, p-ANCA, myositis profile, acetylcholine receptor ( AChR) and muscle specific kinase ( MUSK ) were negative. MRI of neck extensor muscles ( Figure 3 ) was done which showed severe fatty degeneration with Diffuse STIR hyperintensities noted in extensor muscles of neck ,severe fatty degeneration seen in the bilateral cervical paraspinal muscles. An isolated neck extensor myopathy was considered and patient denied the option of muscle biopsy. Patient was given a trial of immunomodulation with steroid (may have to mention dose and regime). At one month follow up patient had good improvement and was started on azathioprine as steroid sparing agent, consistent with myopathy. At 2 months follow up, patient maintained the improvement, but did not show further response. Discussion Dropped Head Syndrome (DHS) develops due to the selective or preferential weakness of the neck extensor muscles. Over two-thirds of patients belonged to just four diagnostic categories 1 : isolated neck extensor myopathy (31.8%), Parkinson's disease (20.2%), myasthenia gravis (12.4%), and amyotrophic lateral sclerosis (7.0%). Isolated neck extensor myopathy (INEM) specifically targets the cervical paraspinal muscles, predominantly affecting elderly women. INEM results from chronic injury and overloading of cervical muscles, leading to isolated myopathic changes. INEM diagnosis typically relies on neuro-physiological tests revealing myopathic changes in the neck extensors. Serum CPK is usually observed to be in normal range or slightly elevated in most of the cases 2 .In DHS cases, contrast-enhanced MRI typically reveals intramuscular enhancement of cervical extensor muscles, primarily affecting splenius capitis, rhomboid, and semispinalis cervicis muscles as noted 3 . Inflammatory INEM, on the other hand, exhibits increased muscle intensity on fat-suppressed, T2-weighted, TSE, or fast-STIR images 4 . Histopathological examinations commonly show ligament degeneration and microvessel proliferation, necrosis, and microvessel proliferation within skeletal muscle 5 in chronic cases of DHS. INEM typically exhibits a poor response to immunosuppressive therapy due to its non-inflammatory nature 2 . Our patient was unique in the relatively young age, rapid progression of symptoms over a 2 month period and marked response to steroid. This could suggest an inflammatory pathophysiology at least in a subset of patients. recent studies, including Muppidi et al., have shown improved neck strength in some INEM patients following immunosuppressive treatment. For patients who do not respond to immune therapy, a surgical fusion across the cervicothoracic junction can be considered . 1 Conclusion Neck extensor myopathies are unique in the rarity and delayed diagnosis. CPK value and even biopsy may not reliably identify the disease due to its limited nature. MR imaging proves useful and can be used as a diagnostic test especially when other tests prove inconclusive. Thic sould significantly accelerate the diagnostic process and early management can be instituted which could alter the disease course positively. Declarations Author Contribution V.C- Writing of the First Draft, Image preparationN.V and V.C - Manuscript Preparation, Writing of the First Draft, Review and Critique D M- Concept of case report, Review and Critique.J. M- Prepared images, ReviewA.P- Review and CritiqueA.K - Review and CritiqueS.B- Image preparation, Review Funding sources and conflicts of interest : No specific funding was received for this work.The authors declare that there are no conflicts of interest relevant to this work Financial disclosures : The authors declare that there are no additional disclosures to report Ethical compliance statement: A written consent was obtained from the byestanders.We confirm that we have read the journal’s position on issues involved in ethical publication and affirm that this work is consistent with those guidelines. I declare that the patient consented to participate and publish their clinical data. References Drain JP, Virk SS, Jain N, Yu E. Dropped Head Syndrome: A Systematic Review. Clin Spine Surg. 2019 Dec;32(10):423–9. Isolated neck extensor myopathy | Neurology [Internet]. [cited 2024 Mar 27]. Available from: https://www.neurology.org/doi/abs/10.1212/wnl.46.4.917 Endo K, Nishimura H, Sawaji Y, Aihara T, Suzuki H, Konishi T, et al. Contrast-enhanced Magnetic Resonance Imaging in Patients With Dropped Head Syndrome. Spine (Phila Pa 1976). 2024 Mar 15;49(6):385–9. Gaeta M, Mazziotti S, Toscano A, Rodolico C, Mazzeo A, Blandino A. “Dropped-head” syndrome due to isolated myositis of neck extensor muscles: MRI findings. Skeletal Radiol. 2006 Feb;35(2):110–2. Endo K, Matsubayashi J, Sawaji Y, Murata K, Konishi T, Nagao T, et al. Histopathological characteristics of cervical extensor tissue in patients with dropped head syndrome. Eur J Med Res. 2021 Nov 26;26(1):135. Additional Declarations No competing interests reported. Cite Share Download PDF Status: Published Journal Publication published 13 Aug, 2024 Read the published version in Acta Neurologica Belgica → Version 1 posted You are reading this latest preprint version Research Square lets you share your work early, gain feedback from the community, and start making changes to your manuscript prior to peer review in a journal. As a division of Research Square Company, we’re committed to making research communication faster, fairer, and more useful. We do this by developing innovative software and high quality services for the global research community. Our growing team is made up of researchers and industry professionals working together to solve the most critical problems facing scientific publishing. Also discoverable on Platform About Our Team In Review Editorial Policies Advisory Board Help Center Resources Author Services Accessibility API Access RSS feed Manage Cookie Preferences © Research Square 2026 | ISSN 2693-5015 (online) Privacy Policy Terms of Service Do Not Sell My Personal Information {"props":{"pageProps":{"initialData":{"identity":"rs-4327338","acceptedTermsAndConditions":true,"allowDirectSubmit":true,"archivedVersions":[],"articleType":"correspondence","associatedPublications":[],"authors":[{"id":303945005,"identity":"8982abd7-90d1-4875-a10d-53f191cee7fb","order_by":0,"name":"Nibu 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1","display":"","copyAsset":false,"role":"figure","size":184820,"visible":true,"origin":"","legend":"\u003cp\u003e\u003cstrong\u003eThis image shows marked head drop on standing\u003c/strong\u003e\u003c/p\u003e","description":"","filename":"1.png","url":"https://assets-eu.researchsquare.com/files/rs-4327338/v1/3cb482dcb6ac1a0aaf408ca5.png"},{"id":57306771,"identity":"e379332c-423f-471e-9ea5-44e8ce98a0ce","added_by":"auto","created_at":"2024-05-29 02:05:03","extension":"png","order_by":2,"title":"Figure 2","display":"","copyAsset":false,"role":"figure","size":325749,"visible":true,"origin":"","legend":"\u003cp\u003e\u003cstrong\u003ePatient is unable to extend her neck in prone position\u003c/strong\u003e\u003c/p\u003e","description":"","filename":"2.png","url":"https://assets-eu.researchsquare.com/files/rs-4327338/v1/5fb85eb9353b6a9cedd02674.png"},{"id":57306773,"identity":"58bcc5fc-e9ea-4cf8-a0ba-78e896537428","added_by":"auto","created_at":"2024-05-29 02:05:04","extension":"png","order_by":3,"title":"Figure 3","display":"","copyAsset":false,"role":"figure","size":612863,"visible":true,"origin":"","legend":"\u003cp\u003eSTIR axial T2, STIR Sag, STIR axial:\u003c/p\u003e\n\u003cp\u003eSevere Fatty degeneration with Diffuse STIR hyperintensities noted in extensor muscles of neck.Severe fatty degeneration seen in the bilateral paraspinal muscles.The visualised bilateral Sternocleidomastoid, trapezius and the shoulder griddle muscle appears grossly normal in bulk and signal intensity.\u003c/p\u003e","description":"","filename":"3.png","url":"https://assets-eu.researchsquare.com/files/rs-4327338/v1/54da01435ed581e572345bdb.png"},{"id":63071145,"identity":"0ce43c14-ec19-4e8f-82c9-77c83864c498","added_by":"auto","created_at":"2024-08-22 20:04:13","extension":"pdf","order_by":0,"title":"","display":"","copyAsset":false,"role":"manuscript-pdf","size":1274825,"visible":true,"origin":"","legend":"","description":"","filename":"manuscript.pdf","url":"https://assets-eu.researchsquare.com/files/rs-4327338/v1/c7fc544b-cfe7-4a61-a2d1-83228350b058.pdf"}],"financialInterests":"No competing interests reported.","formattedTitle":"Neck Extensor Myopathy- A Treatable Cause of Dropped Head Syndrome","fulltext":[{"header":"Introduction","content":"\u003cp\u003eA 44-year-old female presented with a two-month history of cervical pain, and progressive weakness of neck. She needed to support her neck up with hand and often neck fell back on erect posture. \u0026nbsp;She had no prior comorbidities, history of trauma, or known neurological deficits. Examination revealed marked weakness in head extension with noticeable atrophy of neck extensors., Neck flexors were normal \u0026nbsp;[FIGURE 1,and FIGURE 2, VIDEO 1]A. Rest of the neurological examination was prominently normal with a motor examination showing \u0026nbsp;5/5 power MRC grade, strength in upper and lower extremity Council (MRC) scale, with a normal sensory system and normal deep tendon reflexes (DTRs) in all extremities. There were no evidence for any lower motor neuron involvement such as wasting or fasciculations elsewhere. Clinical laboratory investigations were normal except for mildly elevated creatine phosphokinase (CPK) levels (268 IU/L)( biological reference change upto 145 IU/L )I . Blood inflammatory markers ESR and CRP were negative. Routine nerve conduction studies from upper and lower extremitites revealed normal motor and sensory parameters. Repetitive nerve stimulation (RNS) from ADM (expand), deltoid, trapezius, facial muscles were normal. Needle Electromyography (EMG) revealed active denervation in the form of fibrillations and positive sharp waves from C4, C5, C6 paraspinal muscles and upper trapezius muscle with large amplitude long duration motor unit action potentials (MUAPs) from upper trapezius and paraspinal muscles. Rest of the muscles including SCM, deltoid, rhomboides, supra and infraspinatii were normal.. MRI cervical spine and brachial plexus were normal. Her antibodies against ANA profile, c-ANCA, p-ANCA, myositis profile, acetylcholine receptor ( AChR) and \u0026nbsp; muscle specific kinase ( MUSK ) were negative. MRI of neck extensor muscles ( Figure 3 ) was done which showed severe fatty degeneration with Diffuse STIR hyperintensities noted in extensor muscles of neck ,severe fatty degeneration seen in the bilateral cervical paraspinal muscles. An isolated neck extensor myopathy was considered and patient denied the option of muscle biopsy. Patient was given a trial of immunomodulation with steroid (may have to mention dose and regime). At one month follow up patient had good \u0026nbsp;improvement and was started on azathioprine as steroid sparing agent, consistent with myopathy. At 2 months follow up, patient maintained the improvement, but did not show further response. \u0026nbsp;\u0026nbsp;\u003c/p\u003e"},{"header":"Discussion","content":"\u003cp\u003eDropped Head Syndrome (DHS) develops due to the selective or preferential weakness of the neck extensor muscles. Over two-thirds of patients belonged to just four diagnostic categories\u003csup\u003e1\u003c/sup\u003e: isolated neck extensor myopathy (31.8%), Parkinson\u0026apos;s disease (20.2%), myasthenia gravis (12.4%), and amyotrophic lateral sclerosis (7.0%). Isolated neck extensor myopathy (INEM) specifically targets the cervical paraspinal muscles, predominantly affecting elderly women. INEM results from chronic injury and overloading of cervical muscles, leading to isolated myopathic changes. INEM diagnosis typically relies on neuro-physiological tests revealing \u0026nbsp;myopathic changes in the neck extensors. Serum CPK is usually observed to be in normal range or slightly elevated in most of the cases\u003csup\u003e2\u003c/sup\u003e.In DHS cases, contrast-enhanced MRI typically reveals intramuscular enhancement of cervical extensor muscles, primarily affecting splenius capitis, rhomboid, and semispinalis cervicis muscles as noted\u003csup\u003e3\u003c/sup\u003e. Inflammatory INEM, on the other hand, exhibits increased muscle intensity on fat-suppressed, T2-weighted, TSE, or fast-STIR images\u003csup\u003e4\u003c/sup\u003e.\u0026nbsp;Histopathological examinations commonly show ligament degeneration and microvessel proliferation, necrosis, and microvessel proliferation within skeletal muscle\u003csup\u003e5\u003c/sup\u003e in chronic cases of DHS.\u003c/p\u003e\n\u003cp\u003eINEM typically exhibits a poor response to immunosuppressive therapy due to its non-inflammatory nature\u0026nbsp;\u003csup\u003e2\u003c/sup\u003e. Our patient was unique in the relatively young age, rapid progression of symptoms over a 2 month period and marked response to steroid. This could suggest an inflammatory pathophysiology at least in a subset of patients. recent studies, including \u0026nbsp;Muppidi et al., have shown improved neck strength in some INEM patients following immunosuppressive treatment. For patients who do not respond to immune therapy, a surgical fusion across the cervicothoracic junction can be considered .\u0026nbsp;\u003csup\u003e1\u003c/sup\u003e\u003c/p\u003e"},{"header":"Conclusion","content":"\u003cp\u003eNeck extensor myopathies are unique in the rarity and delayed diagnosis. CPK value and even biopsy may not reliably identify the disease due to its limited nature. MR imaging proves useful and can be used as a diagnostic test especially when other tests prove inconclusive. Thic sould significantly accelerate the diagnostic process and early management can be instituted which could alter the disease course positively.\u003c/p\u003e"},{"header":"Declarations","content":"\u003ch2\u003eAuthor Contribution\u003c/h2\u003e\u003cp\u003eV.C- Writing of the First Draft, Image preparationN.V and V.C - Manuscript Preparation, Writing of the First Draft, Review and Critique D M- Concept of case report, Review and Critique.J. M- Prepared images, ReviewA.P- Review and CritiqueA.K - Review and CritiqueS.B- Image preparation, Review\u003c/p\u003e\u003cp\u003e\u003cstrong\u003e\u003cem\u003eFunding sources and conflicts of interest\u003c/em\u003e\u003c/strong\u003e : No specific funding was received for this work.The authors declare that there are no conflicts of interest relevant to this work\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003e\u003cem\u003eFinancial disclosures\u003c/em\u003e\u003c/strong\u003e : The authors declare that there are no additional disclosures to report\u0026nbsp;\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003e\u003cem\u003eEthical compliance statement:\u0026nbsp;\u003c/em\u003e\u003c/strong\u003eA written consent was obtained from the byestanders.We confirm that we have read the journal\u0026rsquo;s position on issues involved in ethical publication and affirm that this work is consistent with those guidelines.\u0026nbsp;I declare that the patient consented to participate and publish their clinical data.\u003c/p\u003e"},{"header":"References","content":"\u003col\u003e\n\u003cli\u003eDrain JP, Virk SS, Jain N, Yu E. Dropped Head Syndrome: A Systematic Review. Clin Spine Surg. 2019 Dec;32(10):423\u0026ndash;9. \u003c/li\u003e\n\u003cli\u003eIsolated neck extensor myopathy | Neurology [Internet]. [cited 2024 Mar 27]. Available from: https://www.neurology.org/doi/abs/10.1212/wnl.46.4.917\u003c/li\u003e\n\u003cli\u003eEndo K, Nishimura H, Sawaji Y, Aihara T, Suzuki H, Konishi T, et al. Contrast-enhanced Magnetic Resonance Imaging in Patients With Dropped Head Syndrome. Spine (Phila Pa 1976). 2024 Mar 15;49(6):385\u0026ndash;9. \u003c/li\u003e\n\u003cli\u003eGaeta M, Mazziotti S, Toscano A, Rodolico C, Mazzeo A, Blandino A. \u0026ldquo;Dropped-head\u0026rdquo; syndrome due to isolated myositis of neck extensor muscles: MRI findings. Skeletal Radiol. 2006 Feb;35(2):110\u0026ndash;2. \u003c/li\u003e\n\u003cli\u003eEndo K, Matsubayashi J, Sawaji Y, Murata K, Konishi T, Nagao T, et al. Histopathological characteristics of cervical extensor tissue in patients with dropped head syndrome. Eur J Med Res. 2021 Nov 26;26(1):135. \u003c/li\u003e\n\u003c/ol\u003e"}],"fulltextSource":"","fullText":"","funders":[],"hasAdminPriorityOnWorkflow":false,"hasManuscriptDocX":true,"hasOptedInToPreprint":true,"hasPassedJournalQc":"","hasAnyPriority":false,"hideJournal":true,"highlight":"","institution":"","isAcceptedByJournal":true,"isAuthorSuppliedPdf":false,"isDeskRejected":"","isHiddenFromSearch":false,"isInQc":false,"isInWorkflow":true,"isPdf":false,"isPdfUpToDate":true,"isWithdrawnOrRetracted":false,"journal":{"display":true,"email":"[email protected]","identity":"researchsquare","isNatureJournal":false,"hasQc":true,"allowDirectSubmit":true,"externalIdentity":"","sideBox":"","snPcode":"","submissionUrl":"/submission","title":"Research Square","twitterHandle":"researchsquare","acdcEnabled":true,"dfaEnabled":false,"editorialSystem":"","reportingPortfolio":"","inReviewEnabled":false,"inReviewRevisionsEnabled":true},"keywords":"Dropped head syndrome (DHS), Isolated Neck Extensor Myopathy Non-inflammatory myopathy","lastPublishedDoi":"10.21203/rs.3.rs-4327338/v1","lastPublishedDoiUrl":"https://doi.org/10.21203/rs.3.rs-4327338/v1","license":{"name":"CC BY 4.0","url":"https://creativecommons.org/licenses/by/4.0/"},"manuscriptAbstract":"\u003cp\u003eDropped head syndrome (DHS) is characterized by a passively correctable chin-on-neck deformity in erect posture and can stem from a wide variety of neurological disorders spanning the neuraxis. . Neuromuscular disorders account for a major chunk of DHS and include disease of anterior horn cell diseases, polyradiculopathies and cervical plexopathies, disease of neuromuscular junction and myopathies. Isolated DHS without additional neurological features poses a management challenge, particularly because the symptoms can significantly impact the patient's quality of life and may not always respond to treatment..(Ref)Here we present a patient with isolated DHS with evaluation revealing isolated next extensor myopathy with remarkable response to treatment. Although isolated neck extensor myopathy typically exhibit poor immunomodulatory response, timely identification and early intervention probably can lead to a favourable outcome in a subgroup of patients.\u003c/p\u003e","manuscriptTitle":"Neck Extensor Myopathy- A Treatable Cause of Dropped Head Syndrome","msid":"","msnumber":"","nonDraftVersions":[{"code":1,"date":"2024-05-29 02:04:59","doi":"10.21203/rs.3.rs-4327338/v1","editorialEvents":[{"type":"communityComments","content":0}],"status":"published","journal":{"display":true,"email":"[email protected]","identity":"researchsquare","isNatureJournal":false,"hasQc":true,"allowDirectSubmit":true,"externalIdentity":"","sideBox":"","snPcode":"","submissionUrl":"/submission","title":"Research Square","twitterHandle":"researchsquare","acdcEnabled":true,"dfaEnabled":false,"editorialSystem":"","reportingPortfolio":"","inReviewEnabled":false,"inReviewRevisionsEnabled":true}}],"origin":"","ownerIdentity":"06d1f3de-b2d1-408d-8c55-16a722f7f579","owner":[],"postedDate":"May 29th, 2024","published":true,"recentEditorialEvents":[],"rejectedJournal":[],"revision":"","amendment":"","status":"posted","subjectAreas":[],"tags":[],"updatedAt":"2024-08-22T19:33:01+00:00","versionOfRecord":{"articleIdentity":"rs-4327338","link":"https://doi.org/10.1007/s13760-024-02617-7","journal":{"identity":"acta-neurologica-belgica","isVorOnly":false,"title":"Acta Neurologica Belgica"},"publishedOn":"2024-08-13 15:57:14","publishedOnDateReadable":"August 13th, 2024"},"versionCreatedAt":"2024-05-29 02:04:59","video":"","vorDoi":"10.1007/s13760-024-02617-7","vorDoiUrl":"https://doi.org/10.1007/s13760-024-02617-7","workflowStages":[]},"version":"v1","identity":"rs-4327338","journalConfig":"researchsquare"},"__N_SSP":true},"page":"/article/[identity]/[[...version]]","query":{"redirect":"/article/rs-4327338","identity":"rs-4327338","version":["v1"]},"buildId":"8U1c8b4HqxoKbykW_rLl7","isFallback":false,"isExperimentalCompile":false,"dynamicIds":[84888],"gssp":true,"scriptLoader":[]}