Case Report Reversible Dropped Head Syndrome after Hemispheric Striatal Infarction Sayaka Funabe, MD, PhD,* Ryota Tanaka, MD, PhD,* Akito Hayashi, MD, PhD,† Kazuo Yamashiro, MD, PhD,* Hideki Shimura, MD, PhD,‡ and Nobutaka Hattori, MD, PhD* We report a rare case of transient ‘‘dropped head syndrome’’ (DHS) after acute ischemic stroke. A 64-year-old man noticed a sudden onset of mild weakness in his left hand and also difficulty in preventing his head from dropping onto his chest without weakness of the neck extensor muscles. Magnetic resonance images showed acute ischemic changes at the right putamen and caudate nucleus. Surface electromyography (EMG) performed 3 days after the stroke showed that both trapeziuses were hypertonic at rest, whereas the activity of the sternocleidomastoids was gradually increased on passive head lifting, indicating dystonia of the neck muscles. His dropped head fully improved by 9 days after the stroke. Re-examination by surface EMG 30 days after the stroke showed no hypertonic activity in the neck muscles. DHS is characterized by an abnormal ante-fixed posture of the neck, usually observed in patients with neurodegenerative disorders such as multiple system atrophy and Parkinson disease. This is the first case of reversible DHS after acute ischemic stroke, and the accumulation of similar cases will be important to elucidate the mechanisms underlying the development of DHS and stroke-associated movement disorders. Key Words: Kubisagari—cervical dystonia—basal ganglia—stroke. Ó 2014 by National Stroke Association Introduction ‘‘Dropped head syndrome’’ (DHS) is characterized by an abnormal ante-fixed posture of the neck while in the standing or sitting position, which is correctable by passive neck extension. The causes of DHS have been re- ported in patients with several types of neuromuscular diseases,1 whereas other cases have been associated with neurodegenerative diseases, such as multiple system atrophy and Parkinson disease.2 We report a case of transient DHS associated with acute striatal infarction. Case Presentation From the *Department of Neurology, Juntendo University School of Medicine, Tokyo; †Department of Rehabilitation, Juntendo University Urayasu Hospital, Chiba; and ‡Department of Neurology, Juntendo University Urayasu Hospital, Chiba, Japan. Received March 26, 2013; revision received April 26, 2013; accepted May 10, 2013. Address correspondence to Ryota Tanaka, MD, PhD, Department of Neurology, Juntendo University School of Medicine, 2-1-1 Hongo, Bunkyo-ku, Tokyo, 113-0033, Japan. E-mail: r_tanaka@juntendo.ac.jp. 1052-3057/$ - see front matter Ó 2014 by National Stroke Association http://dx.doi.org/10.1016/j.jstrokecerebrovasdis.2013.05.017 A 64-year-old Japanese man noticed mild weakness of his left hand and leg. He had a history of diabetes, untreated hypertension, and dyslipidemia. The symptoms fully resolved within 24 hours. He was, therefore, diagnosed with a transient ischemic attack and treated with 100 mg of aspirin, an antihypertensive agent, and ethyl icosapentate. One week later, he noticed mild weakness of his left hand again and also difficulty preventing his head from dropping onto his chest. A neurological examination disclosed mild weakness of the muscles of the left Journal of Stroke and Cerebrovascular Diseases, Vol. 23, No. 4 (April), 2014: pp 785-787 785 S. FUNABE ET AL. 786 Figure 1. The patient’s head dropped forward and mildly rotated to the right (A and B). FLAIR MR images taken 24 hours after the onset showed high intensities at the right putamen and caudate nucleus (C). MR angiography performed at the same time showed moderate atherosclerotic changes in the right middle cerebral artery (D; white arrow). Surface EMG performed 3 days after the stroke showed that the bilateral trapezius muscles were hypertonic at rest (E; black arrow), whereas the SCM activity was gradually increased on passive head lifting (E; white arrow), indicating that there was dystonia of the neck muscles. Abbreviations: EMG, electromyography; FLAIR, fluid attenuation inversion recovery; MR, magnetic resonance; SCM, sternocleidomastoid. extremities, but his neck extensor muscles were not weakened. His head dropped forward and mildly rotated to right (Fig 1, A,B). Magnetic resonance images taken 24 hours after stroke onset showed acute ischemic changes at the right putamen and caudate nucleus (Fig 1, C). Magnetic resonance angiography showed moderate atherosclerotic changes in the ipsilateral middle cerebral artery (Fig 1, D), and neither continuous monitoring of the electrocardiogram in the ward nor 24-hour ambulatory electrocardiogram monitoring demonstrated paroxysmal arterial fibrillation. Thus, the patient was diagnosed with an atherothrombotic infarction. He was admitted to our hospital and treated with intravenous administration of an anticoagulant (argatroban), and edaravone, and also received rehabilitation therapy. Surface electromyography performed 3 days after the stroke showed that both the trapezius muscles were hypertonic at rest, whereas the activity of the sternocleidomastoid was gradually increased on passive head lifting, which meant that there was dystonia of the neck muscles (Fig 1, E), which is a common feature in patients with Parkinsonism.3 His dropped head fully improved by 6 days after admission, and re-examination of a surface electromyography taken 30 days after the stroke showed no hypertonic activity in the neck muscles (data not shown). Finally, cilostazol was introduced instead of aspirin, for secondary prevention. Discussion We presented a rare case of DHS caused by transient cervical dystonia after acute hemistriatal infarction. We searched for other cases of DHS after stroke in the PubMed database, and the search terms included ‘‘dropped head,’’ ‘‘kubisagari,’’ ’’antecollis,’’ ‘‘neck flexion,’’ with ‘‘stroke,’’ ‘‘cerebrovascular disease,’’ or ‘‘cerebral infarction.’’ However, we could not find any cases of DHS after acute ischemic stroke. Therefore, we believe that ours is the first such case. Although secondary cervical dystonia is uncommon, LeDoux and Brady4 reported 4 cases of secondary cervical dystonia and reviewed the previous literature. Structural lesions associated with cervical dystonia were most commonly localized to the brain stem (44%) and cerebellum (44%), followed by the spinal cord (24%) and basal ganglia (24%). Among these cases, only 2 cases showed antecollis, DROPPED HEAD SYNDROME AFTER STROKE and the causative lesions were in the bilateral pontine tegmentum or left putamen. Molho and Factor5 reported 2 cases of cervical dystonia caused by putaminal infarction and suggested the importance of basal ganglia infarction as a cause of cervical dystonia. Several mechanisms have been speculated to underlie the dystonia, including an anatomic or functional disconnection of the thalamus from the striatum, which leads to a disruption of the subsequent thalamic modulation of the premotor and motor cortex. Our case also showed mild head rotation to the right, in addition to the head drop. Although the direction of head rotation tends to be contralateral to the lesion side,4 its association and the underlying mechanism remain unclear. In this regard, this is the first case of reversible DHS after cerebral infarction, and the accumulation of similar cases will be important for elucidating the mechanisms underlying the development of DHS and stroke-associated movement disorders. 787 Acknowledgment: Author roles: S.F. helped in writing the first draft; R.T. in writing the manuscript, review, and critique; A.H. in critical revision of the manuscript for important intellectual design; and K.Y., H.S., and N.H. in review and critique. References 1. Umapathi T, Chaudhry V, Cornblath D, et al. Head drop and camptocormia. J Neurol Neurosurg Pshychiatry 2002;73:1-7. 2. Kashihara K, Ohno M, Tomita S. Dropped head syndrome in Parkinson’s disease. Mov Disord 2006;21:1213-1216. 3. Oyama G, Hayashi A, Mizuno Y, et al. Mechanism and treatment of dropped head syndrome associated with parkinsonism. Parkinson Relat Diord 2009;15:181-186. 4. LeDoux MS, Brady KA. Secondary cervical dystonia associated with structural lesions of the central nervous system. Mov Disord 2003;18:60-69. 5. Molho ES, Factor SA. Basal ganglia infarction as a possible cause of cervical dystonia. Mov Disord 1993;8:213-216.