Abstract
Paraspinous myopathy is also referred to as isolated neck extensor myopathy (INEM) (dropped head syndrome) and isolated trunk extensor myopathy (ITEM) (bent spine syndrome and camptocormia). This increasingly recognized entity may be isolated or be a feature of various neurological and neuromuscular disorders. This chapter discusses the causes and differential diagnosis of paraspinous myopathies including its diagnosis and management.
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References
Suarez GA, Kelly Jr JJ. The dropped head syndrome. Neurology. 1992;42(8):1625–7.
Lange DJ, Fetell MR, Lovelace RE, Rowland LP. The floppy head syndrome. Ann Neurol. 1986;20(1):133.
Spuler S, Krug H, Klein C, et al. Myopathy causing camptocormia in idiopathic Parkinson’s disease: a multidisciplinary approach. Mov Disord. 2010;25(5):552–9.
Karras D, Vassilakos J, Kassimos D. Camptocormia or cormoptosis? The etymology of the word. Ann Rheum Dis. 1996;55(11):858.
Souques A. Reformes, incapacites, gratifications dans la camptocormie. Rev Neurol (Paris). 1916;30:757–8.
Laroche M, Delisle MB, Mazieres B, et al. Late myopathies located at the spinal muscles: a cause of acquired lumbar kyphosis in adults. Rev Rhum Mal Osteoartic. 1991;58(12):829–38.
Umapathi T, Chaudhry V, Cornblath D, Drachman D, Griffin J, Kuncl R. Head drop and camptocormia. J Neurol Neurosurg Psychiatry. 2002;73(1):1–7.
Skidmore F, Mikolenko I, Weiss H, Weiner W. Camptocormia in a patient with multiple system atrophy. Mov Disord. 2005;20(8):1063–4.
Gomez-Puerta JA, Peris P, Grau JM, Martinez MA, Guanabens N. Camptocormia as a clinical manifestation of mitochondrial myopathy. Clin Rheumatol. 2007;26(6):1017–9.
Laroche M, Delisle MB, Aziza R, Lagarrigue J, Mazieres B. Is camptocormia a primary muscular disease? Spine (Phila Pa 1976). 1995;20(9):1011–6.
Van Gerpen JA. Camptocormia secondary to early amyotrophic lateral sclerosis. Mov Disord. 2001;16(2):358–60.
Katz JS, Wolfe GI, Burns DK, Bryan WW, Fleckenstein JL, Barohn RJ. Isolated neck extensor myopathy: a common cause of dropped head syndrome. Neurology. 1996;46(4):917–21.
Dominick J, Sheean G, Schleimer J, Wixom C. Response of the dropped head/bent spine syndrome to treatment with intravenous immunoglobulin. Muscle Nerve. 2006;33(6):824–6.
Mahjneh I, Marconi G, Paetau A, Saarinen A, Salmi T, Somer H. Axial myopathy – an unrecognised entity. J Neurol. 2002;249(6):730–4.
Khella S, Frederic M. Dropped head syndrome. Neurology. 1993;43(4):846; author reply 846–7.
Petiot P, Vial C, de Saint Victor JF, et al. Dropped head syndrome: diagnostic discussion apropos of 3 cases. Rev Neurol (Paris). 1997;153(4):251–5.
Serratrice G, Pouget J, Pellissier JF. Bent spine syndrome. J Neurol Neurosurg Psychiatry. 1996;60(1):51–4.
Ehrenstein MR, Stoll T, Edwards JC. Not all stoops are due to osteoporosis. Ann Rheum Dis. 1996;55(1):21–3.
Hilliquin P, Menkes CJ, Laoussadi S, Job-Deslandre C, Serratrice G. Camptocormia in the elderly. A new entity by paravertebral muscle involvement? Rev Rhum Mal Osteoartic. 1992;59(3):169–75.
Poullin P, Daumen-Legre V, Serratrice G. Camptocormia in the elderly patient: myopathy or muscular dystonia? Rev Rhum Ed Fr. 1993;60(2):159–61.
Reinsel TE, Grobler LJ, Meriam C. Progressive paraspinal muscle atrophy presenting as low-back pain: case report. J Spinal Disord. 1995;8(3):249–51.
Narayanaswami P, Bertorini TE, Halford III H. Selective paraspinal muscle amyotrophy. J Neurol Sci. 2000;176(1):70–4.
Laroche M, Cintas P. Bent spine syndrome (camptocormia): a retrospective study of 63 patients. Joint Bone Spine. 2010;77(6):593–6.
Tiple D, Fabbrini G, Colosimo C, et al. Camptocormia in Parkinson disease: an epidemiological and clinical study. J Neurol Neurosurg Psychiatry. 2009;80(2):145–8.
Lepoutre AC, Devos D, Blanchard-Dauphin A, et al. A specific clinical pattern of camptocormia in Parkinson’s disease. J Neurol Neurosurg Psychiatry. 2006;77(11):1229–34.
Margraf NG, Wrede A, Rohr A, et al. Camptocormia in idiopathic Parkinson’s disease: a focal myopathy of the paravertebral muscles. Mov Disord. 2010;25(5):542–51.
Askmark H, Eeg-Olofsson K, Johansson A, Nilsson P, Olsson Y, Aquilonius S. Parkinsonism and neck extensor myopathy: a new syndrome or coincidental findings? Arch Neurol. 2001;58(2):232–7.
Wrede A, Margraf NG, Goebel HH, Deuschl G, Schulz-Schaeffer WJ. Myofibrillar disorganization characterizes myopathy of camptocormia in Parkinson’s disease. Acta Neuropathol. 2012;123(3):419–32.
Kottlors M, Kress W, Meng G, Glocker FX. Facioscapulohumeral muscular dystrophy presenting with isolated axial myopathy and bent spine syndrome. Muscle Nerve. 2010;42(2):273–5.
Kastrup A, Gdynia HJ, Nagele T, Riecker A. Dropped-head syndrome due to steroid responsive focal myositis: a case report and review of the literature. J Neurol Sci. 2008;267(1–2):162–5.
Chuquilin M, Al-Lozi M. Primary amyloidosis presenting as “dropped head syndrome”. Muscle Nerve. 2011;43(6):905–9.
Wharton SB, Chan KK, Pickard JD, Anderson JR. Paravertebral muscles in disease of the cervical spine. J Neurol Neurosurg Psychiatry. 1996;61(5):461–5.
Hund E, Heckl R, Goebel HH, Meinck HM. Inclusion body myositis presenting with isolated erector spinae paresis. Neurology. 1995;45(5):993–4.
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;35(2):110–2.
Jaster JH, Bertorini TE, Swims MP, et al. Cervical kyphosis after resolution of myopathic head drop. A case report. Spine (Phila Pa 1976). 1996;21(17):2023–5.
Grob D, Brunner NG, Namba T. The natural course of myasthenia gravis and effect of therapeutic measures. Ann N Y Acad Sci. 1981;377:652–69.
Gourie-Devi M, Nalini A, Sandhya S. Early or late appearance of “dropped head syndrome” in amyotrophic lateral sclerosis. J Neurol Neurosurg Psychiatry. 2003;74(5):683–6.
Okamiya S, Ito H, Saito T, Kowa H, Torii J. A case of isolated neck extensor myopathy with parkinsonism. Rinsho Shinkeigaku. 1997;37(5):393–6.
Yoshiyama Y, Takama J, Hattori T. The dropped head sign in parkinsonism. J Neurol Sci. 1999;167(1):22–5.
Linazasoro G, Suarez JA. Myopathic camptocormia in a patient with levodopa unresponsive parkinsonism. Neurologia. 2002;17(3):162–4.
Berenbaum F, Rajzbaum G, Bonnichon P, Amor B. Hyperparathyroidism disclosed by forward sagging of the head. Rev Rhum Ed Fr. 1993;60(6):467–9.
Hoffman D, Gutmann L. The dropped head syndrome with chronic inflammatory demyelinating polyneuropathy. Muscle Nerve. 1994;17(7):808–10.
Karpati G, Carpenter S, Engel AG, et al. The syndrome of systemic carnitine deficiency. Clinical, morphologic, biochemical, and pathophysiologic features. Neurology. 1975;25(1):16–24.
Lomen-Hoerth C, Simmons ML, Dearmond SJ, Layzer RB. Adult-onset nemaline myopathy: another cause of dropped head. Muscle Nerve. 1999;22(8):1146–50.
Lotz BP, Engel AG, Nishino H, Stevens JC, Litchy WJ. Inclusion body myositis. Observations in 40 patients. Brain. 1989;112(Pt 3):727–47.
Riggs JE, Bodensteiner JB, Schochet Jr SS. The dropped head sign: an unusual presentation of congenital myopathy. J Child Neurol. 1994;9(3):330–1.
VanDyke DH, Griggs RC, Markesbery W, Dimauro S. Hereditary carnitine deficiency of muscle. Neurology. 1975;25(2):154–9.
Sakiyama Y, Okamoto Y, Higuchi I, et al. A new phenotype of mitochondrial disease characterized by familial late-onset predominant axial myopathy and encephalopathy. Acta Neuropathol. 2011;121(6):775–83.
Waclawik AJ, Lotz BP, Beinlich BR, Brooks BR. Dropped head syndrome. Neurology. 1997;48(2):550–1; author reply 551–2.
Rose MR, Levin KH, Griggs RC. The dropped head plus syndrome: quantitation of response to corticosteroids. Muscle Nerve. 1999;22(1):115–8.
Oerlemans WG, de Visser M. Dropped head syndrome and bent spine syndrome: two separate clinical entities or different manifestations of axial myopathy? J Neurol Neurosurg Psychiatry. 1998;65(2):258–9.
Luque FA, Rosenkilde CE, Valsamis M, Danon MJ. Inclusion body myositis presenting as the “dropped head syndrome” (abstract). Brain Pathol. 1994;4(4):568.
Jordan B, Eger K, Koesling S, Zierz S. Camptocormia phenotype of FSHD: a clinical and MRI study on six patients. J Neurol. 2011;258(5):866–73.
Gordon AM, Huxley AF, Julian FJ. The variation in isometric tension with sarcomere length in vertebrate muscle fibres. J Physiol. 1966;184(1):170–92.
Huxley AF. Muscular contraction. J Physiol. 1974;243(1):1–43.
Alnaqeeb MA, Al Zaid NS, Goldspink G. Connective tissue changes and physical properties of developing and ageing skeletal muscle. J Anat. 1984;139(Pt 4):677–89.
Biran I, Cohen O, Diment J, Peyser A, Bahnof R, Steiner I. Focal, steroid responsive myositis causing dropped head syndrome. Muscle Nerve. 1999;22(6):769–71.
Abumi K, Shono Y, Taneichi H, et al. Correction of cervical kyphosis using pedicle screw fixation systems. Spine. 1999;24:2389–96.
Katirji B, Hachwi R, Al-Shekhlee A, et al. Isolated dropped head due to adult-onset nemaline myopathy treated by posterior fusion. Neurology. 2005;8:1504–5.
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Chitravas, N., Katirji, B. (2014). Paraspinous Myopathies: The Dropped Head and Bent Spine Syndromes. In: Katirji, B., Kaminski, H., Ruff, R. (eds) Neuromuscular Disorders in Clinical Practice. Springer, New York, NY. https://doi.org/10.1007/978-1-4614-6567-6_66
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DOI: https://doi.org/10.1007/978-1-4614-6567-6_66
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