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Table 1
Blood chemical values during a 5-year follow-up.
Parameter RL 11/04 05/05 03/07 10/07 01/08 05/08 03/09 05/09 10/09
RL: reference limits, Steroid: steroid dosage, AZT: azathioprin dosage, BSR: blood sedimentation rate, CK: creatine-kinase, Eos: eosinophils, LYC: lymphocyte count, §:
prednisolone, $: methyl-prednisolone started in 2/09 at a dosage of 80 mg, na: not available, #: AZT was discontinued after 3 months because of elevated liver function
parameters.
References
[1] Kocaaga Z, Bal S, Turan Y, et al. Camptocormia and dropped head syndrome as
a clinic picture of myotonic myopathy. Joint Bone Spine 2008;75:730–3.
[2] Leano AM, Miller K, White AC. Chronic graft-versus-host disease-related
polymyositis as a cause of respiratory failure following allogeneic bone marrow
transplant. Bone Marrow Transplant 2000;26:1117–20.
[3] Slobodin G, Rozenbaum M, Weller B, et al. Dropped head: an unusual presen-
tation of dermatomyositis. J Rheumatol 2005;32:1174–5.
[4] Kastrup A, Gdynia HJ, Nägele T, et al. Dropped-head syndrome due to steroid
responsive focal myositis: a case report and review of the literature. J Neurol
Sci 2008;267:162–5.
[5] Gaeta M, Mazziotti S, Toscano A, et al. Dropped-head” syndrome due to
isolated myositis of neck extensor muscles: MRI findings. Skeletal Radiol
2006;35:110–2.
[6] Bahnof R. The dropped head syndrome: rehabilitation of cervical focal myositis.
Disabil Rehabil 1999;21:563–5.
[7] Biran I, Cohen O, Diment J, et al. Focal, steroid responsive myositis causing
Fig. 1. Presented patient with dropped head syndrome due to polymyositis before dropped head syndrome. Muscle Nerve 1999;22:769–71.
(left panel) and 2 months after re-starting steroids (right panel). [8] Dalakas MC, Hohlfeld R. Polymyositis and dermatomyositis. Lancet
2003;362:971–82.
[9] Finsterer J. Dropped head syndrome in mitochondriopathy. Eur Spine J
myocardial thickening. Cerebral CT showed basal ganglia calcifi- 2004;13:652–6.
cation bilaterally, discrete diffuse atrophy, and lacunas. Cerebral [10] Rahim F, Gupta D, Bertorini TE, et al. Dropped head presentation of mitochon-
drial myopathy. J Clin Neuromuscul Dis 2003;5:108–14.
MRI revealed multiple glial spots exclusively. MRI of the cervical
spine was normal.
Josef Finsterer ∗
Since DHS was attributed to a relapse of PM, methyl-
Krankenanstalt Rudolfstiftung, Postfach 20,
prednisolone (80 mg/d) was restarted. Additionally, azathioprin
1180 Vienna, Austria
(100 mg/d) was given. Under this regimen, DHS improved by
60–70% within 2 months. Methyl-prednisolone was tapered down
Marlies Frank
to 4 mg within 8 months. Azathioprin had to be discontinued
Elisabeth Krexner
after 3 months because of hepatopathy. At the last follow-up,
First Medical Department, Krankenanstalt
8 months after re-starting steroids, his neurological condition was
Rudolfstiftung, Vienna, Austria
unchanged.
Arguments for a causative role of PM are that DHS has been ∗ Corresponding author. Tel.: +43 1 71165 92085;
previously described in PM [2], dermatomyositis [3], and as focal
fax: +43 1 4781711.
myositis of the extensor neck muscles [4,5,6,7,8], and that DHS
E-mail address: fifigs1@yahoo.de
responded well to steroids. Arguments for a mitochondrial disorder
(J. Finsterer).
(MID) are that there was basal ganglia calcification, hypoacu-
sia, diabetes, short stature, osteoporosis, recurrent hyponatremia,
10 February 2010
hyperlipidemia, myocardial thickening despite normal blood pres-
sure and short stature and hypoacusis in his mother, albinism, Available online18 May 2010
visual impairment, nystagmus and hypothyroidism in his daugh- doi:10.1016/j.jbspin.2010.02.028
ter, and that MID has been previously reported to cause DHS
[9,10]. Steroid-myopathy was excluded since DHS improved Unusual foreign body granuloma (gauzoma) found 46 years
under steroids and since DHS developed without taking steroids. after open reduction and fixation surgery for femoral shaft frac-
Chemotherapy was excluded for the long interval between ture
chemotherapy and occurrence of DHS. The most likely cause of DHS
in the presented patient is involvement of the neck extensor mus- Keywords:
Gauzoma
cles in PM, although the clinical picture additionally suggests an Foreign body granuloma
underlying MID. Soft tissue tumor
This case shows that DHS may be a feature of PM and responds
favorable to steroids.