[Overloading to neck extensor muscles is an aggravating factor to induce further neck drop in isolated neck extensor...

A 78-year-old woman was hospitalized because of progressive anterior neck drop over 4 months prior to admission. She was normal except for mild weakness of her neck, trapezius and biceps brachii muscles. EMG revealed mild myopathic changes in the neck extensors, trapezius, deltoid and sternocleidomastoid muscles. Bilateral splenius capitis muscles had high intensities on T2-weighted and STIR pulse-sequenced MRI. However, there were no inflammatory changes in the right splenius muscle biopsy. Accordingly, the abnormal MRI finding seems not to result from an inflammatory process but from an physiological increase of intracellular water content due to sustained muscle contraction. Because apparent neuromuscular diseases responsible for neck drop were excluded, her clinical features met the criteria of isolated neck extensor myopathy (INEM, Katz). After strict bed-rest for one month, her neck drop improved dramatically. When she returned to the previous life style after discharge, her symptoms of the neck drop reappeared. Although the cause of INEM remains unclear, the present case indicates that the condition is reversible at least in the early stage of the disease, and the overloading to the neck extensor muscles is an aggravating factor of the neck drop in INEM.