Histopathological characteristics of cervical extensor tissue in patients with dropped head syndrome

Background To date, the histopathologic characteristics of dropped head syndrome (DHS) have not been reported sufficiently. The present study investigates the histopathology of biopsy specimens from the cervical paravertebral region in patients with DHS. Methods Histopathological parameters were evaluated in biopsy specimens of the cervical paravertebral soft tissue from 15 patients with DHS. Results Among the 15 cases of DHS examined, skeletal muscle was identified in 7 cases, all of which showed necrosis, microvessel proliferation and atrophy. The ligament was identified in 12 cases, 8 of which showed degeneration. The lag time between the onset of symptoms and the performance of a biopsy in all 8 cases, which showed degeneration was over 3 months. Microvessel proliferation in the ligament was observed in 1 of the 4 cases, in which the lag time between the onset of symptoms and the performance of a biopsy was less than 3 months (acute or subacute phase), and in 7 of the 8 cases, in which the lag time between the symptoms and the performance of a biopsy was over 3 months (chronic phase). Chronic inflammation in the ligament was identified in 1 of the 12 cases. Conclusions The identification of necrosis, microvessel proliferation, and atrophy in the skeletal muscle of patients with DHS and the presence of ligament degeneration and microvessel proliferation in the chronic but not acute or subacute phases may suggest that persistent skeletal muscle damage of the cervical paravertebral region causes subsequent ligament damage in patients with DHS.

Surgical treatment of dropped head syndrome secondary to fascioscapulohumeral muscle dystrophy: a case report

Fascioscapulohumeral muscular dystrophy is an uncommon hereditary myopathy which affects mainly the muscle of the face and upper limb girdle. We present a rare case with dropped head syndrome as the prominent manifestation of that disease and successfully treated by surgical management. It was a 25-year-old male patient with the chief complaint of neck pain and inability to maintain his horizontal gaze for long periods and as a result he had to quit his job as a shipper. His mother also had signs and symptoms of fascioscapulohumeral muscle dystrophy. Conservative treatment consisting of physical therapy and hard collar was the first attempt in order to reduce the neck pain and had limited result. We then performed a posterior cervical surgery including C2 to T2 instrumentation and kyphotic correction for the patient. The ten-month postop clinical and radiological results were satisfactory and the patient could return to his previous job. Dropped head syndrome with failed conservative treatment can be surgically treated after considering all clinical and radiographic factors.

Dynamic alignment changes during level walking in patients with dropped head syndrome: analyses using a three-dimensional motion analysis system

In patients with dropped head syndrome (DHS), cervical malalignment is one of the risk factors for impaired horizontal gaze and restrictions to ambulation. The characteristics of gait in patients with DHS have not been clarified biomechanically from the viewpoint of dynamic alignment and lower limb kinematics. This study aimed to clarify kinematic and kinetic differences during level walking in patients with DHS compared to the healthy elderly. Twelve patients with DHS and healthy elderly individuals performed level walking at a self-selected speed. Spatiotemporal, kinematic, and kinetic data were recorded using a three-dimensional motion analysis system. Statistical analysis was performed to compare these data between the two groups, respectively. Compared with the healthy elderly, stride length and peak hip-joint extension angle in patients with DHS were significantly shorter and smaller. The thorax was also significantly tilted backwards. Peak ankle-joint plantar-flexion moment was significantly smaller despite larger dorsiflexion angle compared with the healthy elderly. The walking of DHS patients demonstrated kinematic and kinetic characteristics of the lower limb joints and alignment of the thorax and pelvis corresponding to their short stride and walking speed.

Prevalence of sarcopenia in idiopathic dropped head syndrome patients is similar to healthy volunteers

Dropped head syndrome (DHS) exhibits cervical deformity due to weakness of the cervical extensor group, and sarcopenia is characterized by progressive and systemic reduction in skeletal muscle mass. These clinical finding are associated with reduced activity of daily living, reduced quality of life, and increased risk of mortality. We collected and reviewed prospective registry data for 16 patients with idiopathic DHS continuously collected without dropping out and 32 healthy individuals who matched their gender and age. The prevalence of sarcopenia and body composition data were compared. There were no differences in the prevalence of sarcopenia, appendicular muscle mass, and leg muscle mass between DHS patients and the healthy elderly. Trunk muscle mass in DHS patients was significantly lower than that in healthy individuals. A significant correlation was found between appendicular muscle mass and trunk muscle mass in healthy subjects but not in DHS patients. Sarcopenia was not associated with the onset of idiopathic DHS. The prevalence of sarcopenia was not high in patients with idiopathic DHS due to the preservation of their appendicular skeletal muscle mass. Patients with DHS were characterized by a significant loss of trunk muscle mass that may be related to the disease but not aging.

Dropped head syndrome as initial and predominant manifestation of inflammatory myopathy

Dropped head syndrome (DHS) is an uncommon clinical syndrome, which requires complex diagnostic evaluation. A variety of neuromuscular and neurodegenerative disease can produce weakness of head extensor muscles and consequently lead to head drop. Inflammatory myopathy has been described as a cause of DHS, however head drop has only exceptionally been reported as being the presenting symptom of this disorder. We describe an original case of DHS as an initial and predominant manifestation of inflammatory myopathy with histopathological features of polymyositis, with an excellent response to immunosuppressive treatment.

Histopathological characteristics of cervical extensor tissue in patients with dropped head syndrome

Background To date, the histopathologic characteristics of dropped head syndrome (DHS) have not been reported sufficiently. The present study investigates the histopathology of biopsy specimens from the cervical paravertebral region in patients with DHS. Methods Histopathological parameters were evaluated in biopsy specimens of the cervical paravertebral soft tissue from 15 patients with DHS. Results Among the 15 cases of DHS examined, skeletal muscle was identified in 7 cases, all of which showed necrosis, microvessel proliferation and atrophy. The ligament was identified in 12 cases, 8 of which showed degeneration. The lag time between the onset of symptoms and the performance of a biopsy in all 8 cases, which showed degeneration was over 3 months. Microvessel proliferation in the ligament was observed in 1 of the 4 cases, in which the lag time between the onset of symptoms and the performance of a biopsy was less than 3 months (acute or subacute phase), and in 7 of the 8 cases, in which the lag time between the symptoms and the performance of a biopsy was over 3 months (chronic phase). Chronic inflammation in the ligament was identified in 1 of the 12 cases. Conclusions The identification of necrosis, microvessel proliferation, and atrophy in the skeletal muscle of patients with DHS and the presence of ligament degeneration and microvessel proliferation in the chronic but not acute or subacute phases may suggest that persistent skeletal muscle damage of the cervical paravertebral region causes subsequent ligament damage in patients with DHS.

Prevalence of Sarcopenia in Idiopathic Dropped Head Syndrome Patients is Similar to Healthy Volunteers

Dropped head syndrome (DHS) exhibits cervical deformity due to weakness of the cervical extensor group, and sarcopenia is characterized by progressive and systemic reduction in skeletal muscle mass. These clinical finding are associated with reduced activity of daily living, reduced quality of life, and increased risk of mortality. We collected and reviewed prospective registry data for 16 consecutive female patients with idiopathic DHS and 32 healthy individuals who matched their gender and age. The prevalence of sarcopenia and body composition data were compared. There were no differences in the prevalence of sarcopenia, appendicular muscle mass, and leg muscle mass between DHS patients and the healthy elderly. Trunk muscle mass in DHS patients was significantly lower than that in healthy individuals. A significant correlation was found between appendicular muscle mass and trunk muscle mass in healthy subjects but not in DHS patients. Sarcopenia was not associated with the onset of idiopathic DHS. The prevalence of sarcopenia was not high in patients with idiopathic DHS due to the preservation of their appendicular skeletal muscle mass. Patients with DHS were characterized by a significant loss of trunk muscle mass that may be related to the disease but not aging.