Strahlenther Onkol DOI 10.1007/s00066-015-0857-8
R e v i e w A rt i c l e
Radiation-induced camptocormia and dropped head syndrome Review and case report of radiation-induced movement disorders Clemens Seidel · Thomas Kuhnt · Rolf-Dieter Kortmann · Kathrin Hering
Received: 20 March 2015 / Accepted: 13 May 2015 © Springer-Verlag Berlin Heidelberg 2015
Abstract Background In recent years, camptocormia and dropped head syndrome (DHS) have gained attention as particular forms of movement disorders. Camptocormia presents with involuntary forward flexion of the thoracolumbar spine that typically increases during walking or standing and may severely impede walking ability. DHS is characterized by weakness of the neck extensors and a consecutive inability to extend the neck; in severe cases the head is fixed in a “chin to chest position.” Many diseases may underlie these conditions, and there have been some reports about radiation-induced camptocormia and DHS. Methods A PubMed search with the keywords “camptocormia,” “dropped head syndrome,” “radiation-induced myopathy,” “radiation-induced neuropathy,” and “radiation-induced movement disorder” was carried out to better characterize radiation-induced movement disorders and the radiation techniques involved. In addition, the case of a patient developing camptocormia 23 years after radiation therapy of a non-Hodgkin’s lymphoma of the abdomen is described. Results In total, nine case series of radiation-induced DHS (n = 45 patients) and—including our case—three case reports (n = 3 patients) about radiogenic camptocormia were retrieved. Most cases (40/45 patients) occurred less than 15 years after radiotherapy involving extended fields for Hodgkin’s disease.
Dr. med. C. Seidel, MD () · Prof. T. Kuhnt · Prof. R.-D. Kortmann · K. Hering, MD Department of Radiotherapy and Radiation Oncology, Leipzig University, Stephanstrasse 9a, 04103 Leipzig, Germany e-mail: [email protected]
Conclusion The use of wide radiation fields including many spinal segments with paraspinal muscles may lead to radiation-induced movement disorders. If paraspinal muscles and the thoracolumbar spine are involved, the clinical presentation can be that of camptocormia. DHS may result if there is involvement of the cervical spine. To prevent these disorders, sparing of the spine and paraspinal muscles is desirable. Keywords Camptocormia · Dropped head syndrome · Radiation-induced myopathy · Radiation-induced neuropathy · Radiation-induced movement disorder Strahleninduzierte Kamptokormie und „DroppedHead“-Syndrom Review und Fallbericht von strahleninduzierten Bewegungsstörungen Zusammenfassung Hintergrund In den letzten Jahren haben Bewegungsstörungen von Wirbelsäule und paraspinaler Muskulatur in Form von „Kamptokormie“ und „Dropped Head Syndrom“ (DHS) verstärkt wissenschaftliche Beachtung gefunden. Die Kamptokormie ist durch zunehmende Flexion des Oberkörpers beim Gehen bei wenig beeinträchtigtem aufrechtem Stand charakterisiert. Das DHS imponiert durch eine Schwäche der Nackenmuskulatur, die zu erschwerter Kopfhebung führt. In schweren Fällen ist bei der Kamptokormie das Gehen stark beeinträchtigt und beim DHS das Kinn auf der Brust nahezu fixiert. Viele Erkrankungen können eine Kamptokormie verursachen, insbesondere neuromuskuläre Erkrankungen und Basalganglienerkrankungen (z. B. Mor-
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bus Parkinson). Zudem sind in der Literatur Berichte über strahleninduzierte Kamptokormie und DHS vorhanden. Methoden In einer systematischen Recherche in PubMed mit den Begriffen „camptocormia“, „dropped head syndrome“, „radiation-induced movment disorder“, „radiation-induced myopathy“, „radiation-induced neuropathy“ wurden alle Publikationen zu diesen Themen erfasst und mit dem Ziel analysiert, strahleninduzierte Bewegungsstörungen und die verwendeten Bestrahlungstechniken näher zu charakterisieren. Zudem beschreiben wir den Fall eines Patienten, der 23 Jahre nach Bestrahlung eines Non-HodgkinLymphoms des Abdomens eine erhebliche, schmerzhafte Kamptokormie entwickelte. Ergebnisse Es wurden 9 Fallberichte zu radiogenem DHS (n = 45 Patienten) und 3 Fallberichte (n = 3 Patienten) zu radiogener Kamptokormie gefunden. Die überwiegende Anzahl der beschriebenen Fälle (40/45 Patienten) trat > 15 Jahre nach Bestrahlung auf, überwiegend von Hodgkin-Lymphomen mit ausgedehnten Bestrahlungsfeldern. Schlussfolgerung Der Einsatz ausgedehnter Bestrahlungsfelder unter multisegmentalem Einschluss von Wirbelsäule und paraspinaler Muskulatur kann zum Auftreten von DHS nach Bestrahlung der Nackenmuskulatur und zu Kamptokormie nach Bestrahlung der Rückenstrecker führen. Zur Prävention dieser Komplikationen erscheint eine Schonung der jeweiligen Muskulatur ratsam. Schlüsselwörter Kamptokormie · DroppedHead-Syndrom · Strahleninduzierte Myopathie · Strahleninduzierte Neuropathie · Strahleninduzierte Bewegungsstörung In recent years, camptocormia and dropped head syndrome have gained attention as particular forms of movement disorders. Camptocormia derives from the Greek words “kamptos” (to bend) and “cormos” (trunk) [1]. It presents with characteristic clinical signs comprising involuntary forward flexion of the thoracolumbar spine that typically increases during walking or standing and disappears in the supine position [2]. This may lead to severe disability with loss of the ability to walk. Dropped head syndrome (DHS) is characterized by weakness of the neck extensors and a consecutive inability to extend the head; in severe cases the head is fixed in a “chin to chest position.” Many diseases may underlie these conditions, in particular neuromuscular diseases (e.g., amyotrophic lateral sclerosis), diseases of the basal ganglia (e.g., Parkinson’s disease), and rarely psychogenic disorders [3]. In fact, camptocormia was first described in soldiers as a psychogenic disorder [4]. Additionally, there are published reports about radiationinduced camptocormia [5–6] and DHS [7–15].
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Methods A PubMed search with the keywords “camptocormia,” “dropped head syndrome,” “radiation-induced myopathy,” “radiation-induced neuropathy,” and “radiation-induced movement disorder” was carried out in January 2015 to review all relevant publications and to better characterize radiation-induced movement disorders and the radiation techniques involved. In addition, the case of a patient developing camptocormia 23 years after radiation therapy for non-Hodgkin’s lymphoma of the abdomen is described. Results Case report A 63-year-old male patient presented to the radiation–oncology department for advice. In 1980, the patient was aged 29 years and received radiotherapy at our department for a low-grade non-Hodgkin’s lymphoma (NHL) of the abdominal lymph nodes. Treatment comprised the application of 45 Gy of telecobalt therapy in daily fractions of 1.2 Gy (5–6/week) with opposing ventrodorsal fields for the entire abdomen [maximum dose (Dmax) 120 % on the skin] without significant acute toxicity. Following this, adjuvant chemotherapy containing vincristine and prednisolone was administered. In 2003 (23 years after radiotherapy), he developed backache, and since then he noted insidiously increasing walking difficulties. In the last few months before presentation, even short distances were impeded by a strong tendency to flex the spine forward with pain in the back. There was no history of neuromuscular or extrapyramidal disease or neuroleptic medications. On examination, the patient developed a profoundly stooped posture after walking a few meters (Fig. 1). Lying flat was possible without difficulty. On inspection, a pronounced atrophy of the paraspinal muscles in the lower thoracal and lumbar spine was evident (Fig. 2). Otherwise, sensation and muscular power were normal; there were no abnormalities of the bladder or bowel function. Magnetic resonance imaging (MRI) of the spine showed pronounced fatty degeneration of the lumbar M. erector spinae and a complete loss of lumbar lordosis (Figs. 3 and 4). In further investigations, electromyography of the paraspinal muscles was normal, myoglobin was minimally elevated, and the creatine kinase level was normal. The patient declined a muscle biopsy. Surgical interventions were not deemed reasonable by the treating orthopedists and walking aids were recommended.
Radiation-induced camptocormia and dropped head syndrome
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Fig. 1 Patient with stooped posture after walking a few meters
Fig. 3 Axial T2-weighted spinal magnetic resonance imaging showing extensive hyperintense degeneration of the paraspinal muscles at the level of L3
Fig. 2 Atrophy of the lumbar paraspinal muscles and skin fibrosis
Review of the literature In total, two case reports (n = 2 patients) on radiogenic camptocormia [5–6] and nine reports about radiation-induced DHS (n = 45 patients) were retrieved [7–15]. Table 1 summarizes all the cases of radiotherapy-induced DHS and camptocormia and the radiation techniques used. Most cases of radiotherapy-induced movement disorders (40/45) are reported in Hodgkin’s disease. The median interval from radiotherapy to diagnosis was 17 years. In all these cases, extended field radiotherapy techniques involving the cervical spine were used. In 36 of 45 cases of DHS, mantle fields with total doses of 35–40 Gy were used, and fractionation schedules were mostly not available. In two cases with head and neck cancer, radiotherapy with parallel opposing fields of the cervical lymph nodes was applied with higher radiation doses (64.8 Gy, 70 Gy). A shorter duration until
Fig. 4 Sagittal T2-weighted spinal magnetic resonance imaging showing hyperintense degeneration of the lumbar paraspinal muscles
the diagnosis of movement disorder (2 years) was observed in these cases [11–12]. In all three cases (including our case) with camptocormia, wide radiotherapy fields involving the thoracolumbar spine with total doses of 38–45 Gy [5–6] were applied. The median duration until the diagnosis of movement disorder was 23 years. Discussion Hodgkin’s disease is a highly curable disease [16–17]. However, after curative treatment of Hodgkin’s disease involving radiotherapy, severe movement disorders may occur. The symptoms begin more than 15 years after therapy and progress slowly. Only a few reports exist regarding DHS in tumors other than Hodgkin’s lymphoma.
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Table 1 Summary of patients with radiation-induced dropped head syndrome or camptocormia Author Cases Movement disorder Cancer Chemo- Irradiation fields therapy Johansson et al. [7] Portlock et al. [10]
18 1
Dropped head syndrome Dropped head syndrome
HD HD
Rowin et al. [9]
3
Dropped head syndrome
HD
n/a Yes
MF MF + para-aortic lymph nodes MF/ whole body RT
Total Delay after dose (Gy) radiotherapy (years) n/a n/a 35 17
Yes n/a 2–26 (case 3) Aggarwal et al. [13] 4 Dropped head syndrome HD n/a MF n/a 20–30 5–30 Furby et al. [8] 6 Dropped head syndrome HD Yes MF or MF with sparing of 36–40 axillae or MF + para-aortic + splenic areas or MF with sparing of axillae + Th11–L3 + splenic areas 64.8 2 Hashimoto et al. [11] 1 Dropped head syndrome Nasopharyngeal Yes Parallel opposing fields carcinoma (primary tumor + upper and middle cervical lymph nodes) Appels et al. [14] 2 Dropped head syndrome HD Yes MF n/a 12; 31 Schlienger et al. [15] 1 Dropped head syndrome HD Yes MF 40 17 n/a 3–45 Yes MF or neck to pelvis or Ghosh et al. [12] 9 Dropped head syndrome HD (6 cases) neck and trunk or whole- 36 pinealoblastoma Yes 7 body RT craniospinal axis 70 tongue thyroid No 2 n/a parallel opposing fields No n/a 27 (chin to chest) 42 Kelly et al. [5] 1 Camptocormia Teratoma No Para-aortic lymph nodes + 40 + 38 inguinal right area Psimaras et al. [6] 1 Camptocormia HD Yes EF (axillary + subclavicular40 7 + para-aortic lymph nodes) Our data 1 Camptocormia NHL Yes EF 45 23 Irradiation fields: EF extended field, MF mantle field, n/a not available, HD Hodgkin’s disease, NHL non-Hodgkin’s lymphoma, RT radiotherapy
The exact pathophysiology of radiation-induced movement disorders is not known; it is thought to be a combination of myopathy and anterior horn/nerve root lesion [18]. Radiotherapy can cause paresis not only by neuropathy/plexopathy but also by muscular fibrosis [19–23]. In a recent series of patients [12], Gosh et al. hypothesized that paresis of the paraspinal muscles after radiotherapy is more likely to be of myopathic origin than radiotherapy-induced paresis of the distal muscles, which more often show signs of neurogenic damage due to neuropathy/plexopathy. The principle mechanism of damage is likely to be the same in radiation-induced camptocormia and DHS; clinical pictures differ owing to the different anatomic sites involved. Whether there are additional predisposing factors for the development of the disorders (e.g., asymptomatic myopathies) remains an open question. The rarity of DHS and camptocormia is somewhat intriguing because paraspinal muscles are often included in irradiated regions not only in cases of lymphoma but also in other tumors, e.g., in head–neck cancer, with much higher radiation doses. The preferential occurrence of DHS and camptocormia in lymphoma could theoretically be related to the lymphoma itself or the sequential use of neurotoxic chemotherapies. However, it is more likely to be related to
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(1) the size of the radiation fields and (2) the long-term survival of patients, which might evoke a “selection” for this mostly late occurring disorder. Thirdly, mild forms might often be missed or misdiagnosed in clinical practice. The severity of the disorders in the illustrated cases can be explained by bilateral, multisegmental loss of muscular function rather than a small muscular lesion. Consequently, large radiation fields, such as mantle fields, may result in greater functional deficit than focal radiotherapy because they cover the spine and paraspinal muscles over many segments. Following focal radiotherapy of only a few spinal segments, it is more likely that other muscles/muscular segments could compensate for the damage, and focal muscle atrophy could arise without a major deleterious effect on spine function. However, there are also very few reported cases of DHS after more focal radiotherapy of head and neck cancer. Interestingly, the higher radiotherapy doses of 65–70 Gy applied lead to a shorter interval until symptom onset [11–12]. There are no treatment options other than symptomatic ones for DHS and camptocormia. For prevention, sparing of the paraspinal muscles from radiotherapy fields is desirable. Because all case reports included total radiotherapy doses of more than 35 Gy, one could hypothesize that a total dose of conventionally fractionated 30 Gy
Radiation-induced camptocormia and dropped head syndrome
might be more tolerable for muscular function in the long run. Hopefully, since widespread use of extended-field radiotherapy in Hodgkin’s disease belongs to the past [17], fewer cases of radiation-induced movement disorders will be observed in the future. Conclusion Camptocormia and DHS are closely related disabling movement disorders that can occur late after radiotherapy involving the spine and paraspinal muscles. Sparing of the spine and paraspinal muscles from the target volume is desirable for the prevention of these disorders. Compliance with ethical guidelines Conflicts of interest C. Seidel, T. Kuhnt, R-D. Kortmann, and K. Hering state that there are no conflicts of interest. Consent was obtained from all patients identifiable from images or other information within the manuscript. In the case of underage patients, consent was obtained from a parent or legal guardian.
References 1. Bloch F, Houeto JL, Tezenas du Montcel, S, Bonneville F, Etchepare F, Welter ML, Rivaud-Pechoux S, Hahn-Barma V, Maisonobe T, Behar C, Lazennec JY, Kurys E, Arnulf I, Bonnet AM, Agid Y (2006) Parkinson’s disease with camptocormia. J Neurol Neurosurg Psychiatry 11:1223–1228 2. Finsterer J, Strobl W (2010) Presentation, etiology, diagnosis, and management of camptocormia. Eur Neurol 1:1–8 3. Glocker FX, Berninger UG (2013) Ursachen der Kamptokormie. Nervenarzt 8:1007–1015 4. Tatu L, Bogousslavsky J, Moulin T, Chopard J (2010) The\“torpillage\” neurologists of World War I: electric therapy to send hysterics back to the front. Neurology 3:279–283 5. Kelly L, Perju-Dumbrava LD, Thyagarajan D, Lee YC (2013) Delayed postirradiation camptocormia. BMJ Case Rep 9. pii: bcr2013200083. doi:10.1136/bcr-2013-200083 6. Psimaras D, Maisonobe T, Delanian S, Leclercq D, Lenglet T, Feuvret L, Ricard D, Hoang-Xuan K, Pradat P (2011) Late onset radiation-induced camptocormia. J Neurol 9:1723–1725 7. Johansson AS, Erlanson M, Lenner P, Lindh J, Osterman B (1998) Late side-effects are common after treatment of Hodgkin’s disease. Muscular atrophy following radiotherapy is a neglected risk. Lakartidningen 1–2:44–47 8. Furby A, Béhin A, Lefaucheur J, Beauvais K, Marcorelles P, Mussini J, Bassez G, Créange A, Eymard B, Pénisson-Besnier I (2010) Late-onset cervicoscapular muscle atrophy and weakness after ra-
5 diotherapy for Hodgkin disease: a case series. J Neurol Neurosurg Psychiatry 1:101–104 9. Rowin J, Cheng G, Lewis SL, Meriggioli MN (2006) Late appearance of dropped head syndrome after radiotherapy for Hodgkin’s disease. Muscle Nerve 5:666–669 10. Portlock CS, Boland P, Hays AP, Antonescu CR, Rosenblum MK (2003) Nemaline myopathy: a possible late complication of Hodgkin’s disease therapy. Hum Pathol 8:816–818 11. Hashimoto Y, Maebayashi K, Izumi S, Motegi A, Mitsuhashi N (2012) Dropped head syndrome induced by chemoradiotherapy for nasopharyngeal carcinoma: a case report. Jpn J Clin Oncol 11:1091–1093 12. Ghosh PS, Milone M (2015) Clinical and laboratory findings of 21 patients with radiation-induced myopathy. J Neurol Neurosurg Psychiatry 2:152–158 13. Aggarwal S, Amoto A (2007) Radiation induced myopathy following mantle field radiation for Hodgkin’s disease: a report of 4 cases. Neurology 68(Suppl) 14. Appels C, Goekoop R (2009) Dropped-head syndrome due to high-dose irradiation. J Rheumatol 36:2316 15. Schlienger M, Ferroir JP, Huguet F, Deluen F, Pene F, Marseguerra R, Toubol E (2013) Dropped Head Syndrome after whiplash injury in a patient treated for a Hodgkin’s lymphoma by mantle field radiotherapy. Cancer Radiother 17:44–49 16. Eich HT, Kriz J, Schmidberger H, Böll B, Klimm B, Rancea M, Müller RP, Engert A ; Deutsche Gesellschaft für Hämatologie und Medizinische Onkologie (DGHO) (2013) The German evidencebased guidelines for Hodgkin’s lymphoma. Aspects for radiation oncologists. Strahlenther Onkol 189:445–447 17. Lohr F, Georg D, Cozzi L, Eich HT, Weber DC, Koeck J, Knäusl B, Dieckmann K, Abo-Madyan Y, Fiandra C, Mueller RP, Engert A, Ricardi U (2014) Novel radiotherapy techniques for involvedfield and involved-node treatment of mediastinal Hodgkin lymphoma: when should they be considered and which questions remain open? Strahlenther Onkol 190:864–866, 868–871 18. Grimm S, Chamberlain M (2011) Hodgkin’s Lymphoma: a review of neurologic complications. Adv Hematol 624578. doi:10.1155/2011/624578 19. Khan MY (1974) Radiation-induced changes in skeletal muscle. An electron microscopic study. J Neuropathol Exp Neurol 1:42–57 20. Lefaix JL, Daburon F, Martin M, Remy J (1990) Gamma irradiation and delayed effects: muscular fibrosis. Pathol Biol (Paris) 6:617–625 21. Wegrowski J, Lefaix JL, Lafuma C (1992) Accumulation of glycosaminoglycans in radiation-induced muscular fibrosis. Int J Radiat Biol 5:685–693 22. Behin A, Delattre J (2004) Complications of radiation therapy on the brain and spinal cord. Semin Neurol 4:405–417 23. Stubblefield MD (2011) Radiation fibrosis syndrome: neuromuscular and musculoskeletal complications in cancer survivors. PM R 11:1041–1054
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R e v i e w A rt i c l e
Radiation-induced camptocormia and dropped head syndrome Review and case report of radiation-induced movement disorders Clemens Seidel · Thomas Kuhnt · Rolf-Dieter Kortmann · Kathrin Hering
Received: 20 March 2015 / Accepted: 13 May 2015 © Springer-Verlag Berlin Heidelberg 2015
Abstract Background In recent years, camptocormia and dropped head syndrome (DHS) have gained attention as particular forms of movement disorders. Camptocormia presents with involuntary forward flexion of the thoracolumbar spine that typically increases during walking or standing and may severely impede walking ability. DHS is characterized by weakness of the neck extensors and a consecutive inability to extend the neck; in severe cases the head is fixed in a “chin to chest position.” Many diseases may underlie these conditions, and there have been some reports about radiation-induced camptocormia and DHS. Methods A PubMed search with the keywords “camptocormia,” “dropped head syndrome,” “radiation-induced myopathy,” “radiation-induced neuropathy,” and “radiation-induced movement disorder” was carried out to better characterize radiation-induced movement disorders and the radiation techniques involved. In addition, the case of a patient developing camptocormia 23 years after radiation therapy of a non-Hodgkin’s lymphoma of the abdomen is described. Results In total, nine case series of radiation-induced DHS (n = 45 patients) and—including our case—three case reports (n = 3 patients) about radiogenic camptocormia were retrieved. Most cases (40/45 patients) occurred less than 15 years after radiotherapy involving extended fields for Hodgkin’s disease.
Dr. med. C. Seidel, MD () · Prof. T. Kuhnt · Prof. R.-D. Kortmann · K. Hering, MD Department of Radiotherapy and Radiation Oncology, Leipzig University, Stephanstrasse 9a, 04103 Leipzig, Germany e-mail: [email protected]
Conclusion The use of wide radiation fields including many spinal segments with paraspinal muscles may lead to radiation-induced movement disorders. If paraspinal muscles and the thoracolumbar spine are involved, the clinical presentation can be that of camptocormia. DHS may result if there is involvement of the cervical spine. To prevent these disorders, sparing of the spine and paraspinal muscles is desirable. Keywords Camptocormia · Dropped head syndrome · Radiation-induced myopathy · Radiation-induced neuropathy · Radiation-induced movement disorder Strahleninduzierte Kamptokormie und „DroppedHead“-Syndrom Review und Fallbericht von strahleninduzierten Bewegungsstörungen Zusammenfassung Hintergrund In den letzten Jahren haben Bewegungsstörungen von Wirbelsäule und paraspinaler Muskulatur in Form von „Kamptokormie“ und „Dropped Head Syndrom“ (DHS) verstärkt wissenschaftliche Beachtung gefunden. Die Kamptokormie ist durch zunehmende Flexion des Oberkörpers beim Gehen bei wenig beeinträchtigtem aufrechtem Stand charakterisiert. Das DHS imponiert durch eine Schwäche der Nackenmuskulatur, die zu erschwerter Kopfhebung führt. In schweren Fällen ist bei der Kamptokormie das Gehen stark beeinträchtigt und beim DHS das Kinn auf der Brust nahezu fixiert. Viele Erkrankungen können eine Kamptokormie verursachen, insbesondere neuromuskuläre Erkrankungen und Basalganglienerkrankungen (z. B. Mor-
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bus Parkinson). Zudem sind in der Literatur Berichte über strahleninduzierte Kamptokormie und DHS vorhanden. Methoden In einer systematischen Recherche in PubMed mit den Begriffen „camptocormia“, „dropped head syndrome“, „radiation-induced movment disorder“, „radiation-induced myopathy“, „radiation-induced neuropathy“ wurden alle Publikationen zu diesen Themen erfasst und mit dem Ziel analysiert, strahleninduzierte Bewegungsstörungen und die verwendeten Bestrahlungstechniken näher zu charakterisieren. Zudem beschreiben wir den Fall eines Patienten, der 23 Jahre nach Bestrahlung eines Non-HodgkinLymphoms des Abdomens eine erhebliche, schmerzhafte Kamptokormie entwickelte. Ergebnisse Es wurden 9 Fallberichte zu radiogenem DHS (n = 45 Patienten) und 3 Fallberichte (n = 3 Patienten) zu radiogener Kamptokormie gefunden. Die überwiegende Anzahl der beschriebenen Fälle (40/45 Patienten) trat > 15 Jahre nach Bestrahlung auf, überwiegend von Hodgkin-Lymphomen mit ausgedehnten Bestrahlungsfeldern. Schlussfolgerung Der Einsatz ausgedehnter Bestrahlungsfelder unter multisegmentalem Einschluss von Wirbelsäule und paraspinaler Muskulatur kann zum Auftreten von DHS nach Bestrahlung der Nackenmuskulatur und zu Kamptokormie nach Bestrahlung der Rückenstrecker führen. Zur Prävention dieser Komplikationen erscheint eine Schonung der jeweiligen Muskulatur ratsam. Schlüsselwörter Kamptokormie · DroppedHead-Syndrom · Strahleninduzierte Myopathie · Strahleninduzierte Neuropathie · Strahleninduzierte Bewegungsstörung In recent years, camptocormia and dropped head syndrome have gained attention as particular forms of movement disorders. Camptocormia derives from the Greek words “kamptos” (to bend) and “cormos” (trunk) [1]. It presents with characteristic clinical signs comprising involuntary forward flexion of the thoracolumbar spine that typically increases during walking or standing and disappears in the supine position [2]. This may lead to severe disability with loss of the ability to walk. Dropped head syndrome (DHS) is characterized by weakness of the neck extensors and a consecutive inability to extend the head; in severe cases the head is fixed in a “chin to chest position.” Many diseases may underlie these conditions, in particular neuromuscular diseases (e.g., amyotrophic lateral sclerosis), diseases of the basal ganglia (e.g., Parkinson’s disease), and rarely psychogenic disorders [3]. In fact, camptocormia was first described in soldiers as a psychogenic disorder [4]. Additionally, there are published reports about radiationinduced camptocormia [5–6] and DHS [7–15].
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Methods A PubMed search with the keywords “camptocormia,” “dropped head syndrome,” “radiation-induced myopathy,” “radiation-induced neuropathy,” and “radiation-induced movement disorder” was carried out in January 2015 to review all relevant publications and to better characterize radiation-induced movement disorders and the radiation techniques involved. In addition, the case of a patient developing camptocormia 23 years after radiation therapy for non-Hodgkin’s lymphoma of the abdomen is described. Results Case report A 63-year-old male patient presented to the radiation–oncology department for advice. In 1980, the patient was aged 29 years and received radiotherapy at our department for a low-grade non-Hodgkin’s lymphoma (NHL) of the abdominal lymph nodes. Treatment comprised the application of 45 Gy of telecobalt therapy in daily fractions of 1.2 Gy (5–6/week) with opposing ventrodorsal fields for the entire abdomen [maximum dose (Dmax) 120 % on the skin] without significant acute toxicity. Following this, adjuvant chemotherapy containing vincristine and prednisolone was administered. In 2003 (23 years after radiotherapy), he developed backache, and since then he noted insidiously increasing walking difficulties. In the last few months before presentation, even short distances were impeded by a strong tendency to flex the spine forward with pain in the back. There was no history of neuromuscular or extrapyramidal disease or neuroleptic medications. On examination, the patient developed a profoundly stooped posture after walking a few meters (Fig. 1). Lying flat was possible without difficulty. On inspection, a pronounced atrophy of the paraspinal muscles in the lower thoracal and lumbar spine was evident (Fig. 2). Otherwise, sensation and muscular power were normal; there were no abnormalities of the bladder or bowel function. Magnetic resonance imaging (MRI) of the spine showed pronounced fatty degeneration of the lumbar M. erector spinae and a complete loss of lumbar lordosis (Figs. 3 and 4). In further investigations, electromyography of the paraspinal muscles was normal, myoglobin was minimally elevated, and the creatine kinase level was normal. The patient declined a muscle biopsy. Surgical interventions were not deemed reasonable by the treating orthopedists and walking aids were recommended.
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Fig. 1 Patient with stooped posture after walking a few meters
Fig. 3 Axial T2-weighted spinal magnetic resonance imaging showing extensive hyperintense degeneration of the paraspinal muscles at the level of L3
Fig. 2 Atrophy of the lumbar paraspinal muscles and skin fibrosis
Review of the literature In total, two case reports (n = 2 patients) on radiogenic camptocormia [5–6] and nine reports about radiation-induced DHS (n = 45 patients) were retrieved [7–15]. Table 1 summarizes all the cases of radiotherapy-induced DHS and camptocormia and the radiation techniques used. Most cases of radiotherapy-induced movement disorders (40/45) are reported in Hodgkin’s disease. The median interval from radiotherapy to diagnosis was 17 years. In all these cases, extended field radiotherapy techniques involving the cervical spine were used. In 36 of 45 cases of DHS, mantle fields with total doses of 35–40 Gy were used, and fractionation schedules were mostly not available. In two cases with head and neck cancer, radiotherapy with parallel opposing fields of the cervical lymph nodes was applied with higher radiation doses (64.8 Gy, 70 Gy). A shorter duration until
Fig. 4 Sagittal T2-weighted spinal magnetic resonance imaging showing hyperintense degeneration of the lumbar paraspinal muscles
the diagnosis of movement disorder (2 years) was observed in these cases [11–12]. In all three cases (including our case) with camptocormia, wide radiotherapy fields involving the thoracolumbar spine with total doses of 38–45 Gy [5–6] were applied. The median duration until the diagnosis of movement disorder was 23 years. Discussion Hodgkin’s disease is a highly curable disease [16–17]. However, after curative treatment of Hodgkin’s disease involving radiotherapy, severe movement disorders may occur. The symptoms begin more than 15 years after therapy and progress slowly. Only a few reports exist regarding DHS in tumors other than Hodgkin’s lymphoma.
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Table 1 Summary of patients with radiation-induced dropped head syndrome or camptocormia Author Cases Movement disorder Cancer Chemo- Irradiation fields therapy Johansson et al. [7] Portlock et al. [10]
18 1
Dropped head syndrome Dropped head syndrome
HD HD
Rowin et al. [9]
3
Dropped head syndrome
HD
n/a Yes
MF MF + para-aortic lymph nodes MF/ whole body RT
Total Delay after dose (Gy) radiotherapy (years) n/a n/a 35 17
Yes n/a 2–26 (case 3) Aggarwal et al. [13] 4 Dropped head syndrome HD n/a MF n/a 20–30 5–30 Furby et al. [8] 6 Dropped head syndrome HD Yes MF or MF with sparing of 36–40 axillae or MF + para-aortic + splenic areas or MF with sparing of axillae + Th11–L3 + splenic areas 64.8 2 Hashimoto et al. [11] 1 Dropped head syndrome Nasopharyngeal Yes Parallel opposing fields carcinoma (primary tumor + upper and middle cervical lymph nodes) Appels et al. [14] 2 Dropped head syndrome HD Yes MF n/a 12; 31 Schlienger et al. [15] 1 Dropped head syndrome HD Yes MF 40 17 n/a 3–45 Yes MF or neck to pelvis or Ghosh et al. [12] 9 Dropped head syndrome HD (6 cases) neck and trunk or whole- 36 pinealoblastoma Yes 7 body RT craniospinal axis 70 tongue thyroid No 2 n/a parallel opposing fields No n/a 27 (chin to chest) 42 Kelly et al. [5] 1 Camptocormia Teratoma No Para-aortic lymph nodes + 40 + 38 inguinal right area Psimaras et al. [6] 1 Camptocormia HD Yes EF (axillary + subclavicular40 7 + para-aortic lymph nodes) Our data 1 Camptocormia NHL Yes EF 45 23 Irradiation fields: EF extended field, MF mantle field, n/a not available, HD Hodgkin’s disease, NHL non-Hodgkin’s lymphoma, RT radiotherapy
The exact pathophysiology of radiation-induced movement disorders is not known; it is thought to be a combination of myopathy and anterior horn/nerve root lesion [18]. Radiotherapy can cause paresis not only by neuropathy/plexopathy but also by muscular fibrosis [19–23]. In a recent series of patients [12], Gosh et al. hypothesized that paresis of the paraspinal muscles after radiotherapy is more likely to be of myopathic origin than radiotherapy-induced paresis of the distal muscles, which more often show signs of neurogenic damage due to neuropathy/plexopathy. The principle mechanism of damage is likely to be the same in radiation-induced camptocormia and DHS; clinical pictures differ owing to the different anatomic sites involved. Whether there are additional predisposing factors for the development of the disorders (e.g., asymptomatic myopathies) remains an open question. The rarity of DHS and camptocormia is somewhat intriguing because paraspinal muscles are often included in irradiated regions not only in cases of lymphoma but also in other tumors, e.g., in head–neck cancer, with much higher radiation doses. The preferential occurrence of DHS and camptocormia in lymphoma could theoretically be related to the lymphoma itself or the sequential use of neurotoxic chemotherapies. However, it is more likely to be related to
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(1) the size of the radiation fields and (2) the long-term survival of patients, which might evoke a “selection” for this mostly late occurring disorder. Thirdly, mild forms might often be missed or misdiagnosed in clinical practice. The severity of the disorders in the illustrated cases can be explained by bilateral, multisegmental loss of muscular function rather than a small muscular lesion. Consequently, large radiation fields, such as mantle fields, may result in greater functional deficit than focal radiotherapy because they cover the spine and paraspinal muscles over many segments. Following focal radiotherapy of only a few spinal segments, it is more likely that other muscles/muscular segments could compensate for the damage, and focal muscle atrophy could arise without a major deleterious effect on spine function. However, there are also very few reported cases of DHS after more focal radiotherapy of head and neck cancer. Interestingly, the higher radiotherapy doses of 65–70 Gy applied lead to a shorter interval until symptom onset [11–12]. There are no treatment options other than symptomatic ones for DHS and camptocormia. For prevention, sparing of the paraspinal muscles from radiotherapy fields is desirable. Because all case reports included total radiotherapy doses of more than 35 Gy, one could hypothesize that a total dose of conventionally fractionated 30 Gy
Radiation-induced camptocormia and dropped head syndrome
might be more tolerable for muscular function in the long run. Hopefully, since widespread use of extended-field radiotherapy in Hodgkin’s disease belongs to the past [17], fewer cases of radiation-induced movement disorders will be observed in the future. Conclusion Camptocormia and DHS are closely related disabling movement disorders that can occur late after radiotherapy involving the spine and paraspinal muscles. Sparing of the spine and paraspinal muscles from the target volume is desirable for the prevention of these disorders. Compliance with ethical guidelines Conflicts of interest C. Seidel, T. Kuhnt, R-D. Kortmann, and K. Hering state that there are no conflicts of interest. Consent was obtained from all patients identifiable from images or other information within the manuscript. In the case of underage patients, consent was obtained from a parent or legal guardian.
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