Dropped head syndrome: diagnosis and management

ABSTRACT

Dropped head syndrome (DHS) is a relatively rare condition, with a broad differential diagnosis. This deformity has significant implications on the health and quality of life of affected individuals. While surgery seems to be an obvious therapeutic option, there is a paucity of information on surgical intervention with no clear consensus on an optimal approach or timing.

We present a case of DHS in a young woman to illustrate this condition, and review the current literature. Although at present the only definitive solution for correction and stabilization of DHS is surgical intervention involving multilevel instrumented fixation and fusion, this condition requires a persistent medical workup and treatment of reversible causes before surgical intervention is contemplated.

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COMMENTARY

Author Michael J Lee

Institution University of Washington, Seattle, WA, USA

Martin and colleagues present a case report and review of the literature surrounding cervical myopathy and resultant dropped head syndrome. They note that this relatively uncommon condition may stem from numerous neuromuscular etiologies. Nonoperative management is largely targeted to the underlying condition and thus may be wide-ranging in its treatment.

This study reveals the paucity of quality literature on this relatively uncommon condition. The largest series describes only nine patients. Consequently, there is no formal standard of care for this condition. Nevertheless, the authors demonstrated appropriate surgical treatment of this patient by adhering to the core tenets of surgical spine treatment: (1) decompression of neural elements, if necessary; (2) stabilization; and (3) correction of deformity.

As Martin and colleagues have properly reviewed, there are other surgical aspects that may provoke additional discussion: approach (anterior vs posterior vs 360), number of levels fused and instrumented, and preemptive decompression. With such a dearth of quality literature, these aspects are best determined on a case-by-case basis. They recommend intervention before onset of cervical myelopathy. In addition, it may be worthwhile to consider earlier intervention while the deformity is passively correctable. Correction of a fixed deformity can often necessitate an anterior and posterior approach, whereas the flexible deformity may be adequately treated with a single approach; thus obviating further surgical insult.

To conclude, the authors presented a well-written case report with an exhaustive literature review on this topic. Although a specific standard of care for this condition has not been defined, adherence to basic core principles of surgical spine treatment, as they have shown, can result in a restoration of function and subsequent improvement in clinical outcome.

EDITORIAL STAFF PERSPECTIVE

The article on „Dropped head syndrome” (DHS) exemplifies the value of case reports and series. Very rare conditions, such as the one described here, are not really amenable to comparative studies or prospective protocols; they are simply too infrequent and are permeated by high degree of variability. Regarding the general subject of camptocormia (of which the dropped head syndrome is a subset specific to the neck), there is far too little known. There has been no systematic review on this highly disabling and perplexing condition since it was originally described by Alexandre-Achille Souques (1860 – 1944, Hospice de la Salpêtrière, Paris, France) in 1914 [1, 2]. On a historical note, Souques was the cofounder of the Société de Neurologie de Paris together with Ivan Babinski.

One productive outcome of gathering case reports of rare conditions, eg DHS, is the potential to develop more consistent differential diagnosis tables for pathological forward curvature of the spine (with neck and thoracolumbar subsets); and emanating from that, the presentation of sensible diagnostic algorithms to aid clinicians in providing meaningful and systematic assessment approaches.

The EBSJ editorial staff encourages like-minded members of the global spine practitioner community, such as AOSpine, to collaborate on such an endeavor.

1. Azher SN, Jankovic J (2005) Camptocormia: pathogenesis, classification, and response to therapy. Neurology; 65: 355 – 359.

2. Souques A, Rodsanoff-Saloff (1914) [La camptocormie; incurvation du tronc, consécutive aux traumatismes du dos et des lombes; considération morphologiques.] Rev Neurol; 28: 937 – 939. French.