A novel diagnostic examination for dropped head syndrome (DHS) (Prone position cervical extension test; DHS test)

Background

Dropped head syndrome (DHS) is followed by severe cervical extension muscle weakness that results in chin-on chest deformity. However, maintaining a neutral cervical position can be temporarily possible, and the diagnosis of DHS might sometimes be difficult. The purpose of the present study is to examine a novel clinical test (DHS test) as the diagnostic utility for objective evaluation that focuses on cervical extension condition in the prone position.

Methods

One hundred subjects were diagnosed with isolated neck extensor myopathy (INEM)-DHS at our hospital (17 men and 83 women, mean age 75.0 ± 8.5 years), and 62 subjects were enrolled as age-matched controls. The DHS test consisted of three examinations; the first was “Ceiling gazing test” in standing position, the second was horizontal gazing in “Sphinx prone position test”, and the third was horizontal gazing in “Hands and knees prone position test”. We investigated the sensitivity and specificity of the DHS test for DHS.

Results

The patients showing positive in the INEM-DHS group were 63/100 in Ceiling gaze test, 73/100 in the Sphinx prone position test, and 91/100 in the Hands and knees prone position test. In the control group, 0/62 patients presented positive in the Ceiling gaze test, 4/62 in the Sphinx prone position test, and 0/62 in the Hands and knees prone position test. Sensitivity and specificity of the DHS test were 63.0%/100%, 73.0%/93.5%, and 91.0%/100% in the Ceiling gaze test, Sphinx position prone position test, and Hands and knees prone position test, respectively.

Conclusion

The prone position cervical extension test (DHS test) would be useful as a novel objective diagnostic tool for INEM-DHS.