Case reportThe dropped head sign: an unusual presenting feature of myasthenia gravis
Introduction
The dropped head sign is a descriptive term applied to patients with neuromuscular disease whose extensor muscles of the neck lack sufficient strength to overcome gravity and keep the head in an upright neutral position. The affected patient has a characteristic appearance with the head tiled forward and the chin resting on the chest. The inability to prevent the forward inclination of the head occurs gradually and is painless. The eventual position of the chin on the chest wall results in severe impairment of the activities of daily life and social function.
Since, its first description in 1986, interested clinicians have found that dropped head sign can occur in a variety of myopathic and neutrogenic disorders [1], [2]. It has also been described as a result of defective neuromuscular transmission and we describe its occurrence in a patient with myasthenia gravis. While this report is not the first to make the association of the dropped head sign with this disorder, our patient was unusual in that his difficulty with head posture was his presenting complaint [3].
Section snippets
Case report
A 78-year-old male complained that for the past 2 months he could not hold his head upright when he walked. In addition, he had weakness in his legs and difficulty arising from a chair. Two weeks prior to the initial interview, he developed ptosis in his left eyelid, diplopia, and slurring of his speech.
The patient had left eyelid ptosis covering 1/2 of his orbit. His pupils were of equal size and responded normally to light and accomodation. The strength of the neck extensors was diminished to
Discussion
Neck flexion weakness, sometimes with a degree of neck extension weakness, is a common feature of a variety of neuromuscular diseases. However, prominent or isolated neck extensor weakness is distinctly less common. The resulting dropped head position from neck extensor muscle weakness is very disabling due to the impaired visual cues and discomfort. Treatment with cervical collars and other methods of supports are of limited benefit. Only a few known neuromuscular diseases have weakness of the
References (6)
- et al.
Myopathic dropped head syndrome: a syndrome of mixed etiology
J Clin Neurosci
(2000) - et al.
The floppy head syndrome
Ann Neurol
(1986) - et al.
The dropped head syndrome
Neurology
(1992)
Cited by (20)
Treatment of myasthenia gravis with dropped head: A report of 2 cases and review of the literature
2015, Neuromuscular DisordersCitation Excerpt :The titer of anti-AChR antibody decreased to 4.7 nmol/l 2 months after initiation of steroid treatment. We found 9 cases of MG with dropped head in the literature (Table 1) [4–11], and we analyzed clinical features of 11 cases including ours. The age at onset ranged from 46 to 80 years (mean ± SD, 62.9 ± 9.2 years old), and the female:male ratio was 7:4.
Double seronegative myasthenia gravis with anti-LRP 4 antibodies
2013, Neuromuscular DisordersMyasthenia gravis: An unrecognized cause of head drop in Parkinson's disease
2008, Parkinsonism and Related DisordersCitation Excerpt :In addition, head drop in multiple system atrophy and PD has been related with myopathy of the neck extensor muscles [1]. In our patient, head drop was due to MG characterized by an isolated involvement of neck extensors, as yet described in patients without PD [2,3]. The association of PD and MG has been reported by different authors [4–7]: one patient of four with PD and MG described by Levin and colleagues developed myasthenic head drop concomitantly with generalized weakness 5 years after the onset of PD [7].
Musk-antibody positive myasthenia gravis presenting with isolated neck extensor weakness
2007, Neuromuscular DisordersCitation Excerpt :The more general term “dropped head sign” is used to refer to the condition in which the extensor muscles of the neck are too weak to overcome gravity holding the head up straight [1]. Dropped head sign may be an infrequent first manifestation of myasthenia gravis (MG) [1]. We describe the occurrence of this sign in a patient as the only presenting manifestation of an anti-MuSK MG.
'Dropped head sign' in myasthenia gravis
2004, Neuromuscular DisordersDropped head syndrome in anti-MuSK antibody-positive myasthenia gravis with possible concurrent axial myopathy
2024, Clinical and Experimental Neuroimmunology