(Kiosk 3) Keep Your Head Up: Dropped Head Syndrome (A Case Report)

Case Diagnosis: Dropped Head Syndrome (DHS)

Case Description or Program Description: A 60-year-old woman with Parkinson’s Disease (PD), cervical dystonia, and suspected isolated neck extensor myopathy (diagnosed by noninflammatory myopathic changes on biopsy), presents for management of DHS, which has caused worsening sialorrhea, and difficulty vocalizing, ambulating safely and performing activities of daily living. She receives toxin injection for sialorrhea. On physical exam, she has bilateral sternocleidomastoid (SCM) spasticity; 60-degrees of cervical flexion; and inability to passively extend neck >10-15 degrees. On EMG there is increased insertional and spontaneous activity of bilateral levator scapulae, trapezii, and SCM (left>right).

Setting: outpatient

Assessment/Results: EMG-guided toxin injections were initiated at the lowest recommended doses, with gradual uptitration - targeting bilateral SCM, splenius and scalenes - with some symptomatic relief and no adverse effects. Patient also performs comprehensive neck flexor stretching exercises daily. Over the last few months, however, she has developed increased tone in bilateral upper extremities (right>left). Management plan includes: (1) Uptitrating toxin dose to optimize ability of neck extensors to return head to a more normal position; (2) If successful, consider having an orthotist progressively increase the anterior dimension of a hard collar; (3) Given the severity of patient’s positioning and development of symptoms concerning for cervical myelopathy, consider imaging to assess for compression, and evaluation by a spine surgeon.

Discussion (relevance): DHS is a rare, disabling condition caused by severe weakness of the neck extensors causing progressive kyphosis of the cervical spine and inability to hold the head up. Weakness can occur in isolation or in association with a generalized neuromuscular disorder, like PD. Persistent chin-to-chest deformity may cause/exacerbate pre-existing degenerative changes of the cervical spine and result in myelopathy. It can be incredibly challenging to manage.

Conclusions: Currently, there is no standardized treatment for DHS. Approaches have included bracing, physical therapy, toxin injections and/or decompressive cervical laminectomy and fusion.