Isolated Neck Extensor Myopathy Research Articles

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

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

Contrast-enhanced Magnetic Resonance Imaging in Patients With Dropped Head Syndrome.

This is a retrospective study of a consecutive case series of dropped head syndrome (DHS). To identify the characteristic features of contrast-enhanced magnetic resonance imaging (MRI) in DHS patients. Isolated neck extensor myopathy DHS is thought to be caused by severe cervical extensor muscle weakness from age-related loss of elasticity. However, the MRI findings of the cervical extensor muscles in DHS patients have not yet been characterized. The subjects were 34 patients with isolated neck extensor myopathy DHS who underwent contrast-enhanced MRI within seven months after onset and 32 patients with age-matched cervical spondylosis or cervical soft-tissue tumor as controls. The presence of enhanced findings in the cervical extensor muscles, the involved cervical levels, and the characteristically enhanced shape of those muscles were evaluated using contrast-enhanced MRI. In the DHS group, the contrast-enhanced MRI showed pronounced enhancement at splenius capitis in 34 cases, rhomboid in 23 cases, semispinalis cervicis in seven cases, and levator scapulae in three cases. In the non-DHS group, none of those extensor muscles were enhanced. The enhanced pattern was butterfly shaped in 29 cases (85.3%) and linear in five cases (14.7%). All were located at the spinous process attachment at C6 or C7 in the DHS group. In the non-DHS group, seven cases presented an enhanced image of the spinous process, with C5-6 in one case, C6 in five cases, and C7 in one case. In DHS, contrast-enhanced MRI showed intramuscular enhancement of the cervical extensor muscles, which was not present in non-DHS cases. The enhanced muscles included the splenius capitis muscle in all cases. These findings may be useful for developing a strategy for DHS treatment.

Global Sagittal Spinal Compensation for Dropped Head Alignment.

This is a retrospective radiographic study of a consecutive series of cases in patients with dropped head syndrome (DHS) at a single tertiary referral center. The aim was to clarify the compensation among parameters of spinal sagittal alignment in patients with DHS. The treatment strategy for DHS should vary according to the types of global sagittal spinal alignment. However, theoretical evidence in consideration of spinal sagittal compensation against the dropped head condition is lacking. One hundred sixteen patients diagnosed with isolated neck extensor myopathy were enrolled. Radiographic measurements were made, including parameters of spinal sagittal alignment. The patients were divided into three groups according to sagittal spinal balance: C7SVA (sagittal vertical axis) ≥ +50mm (P-DHS; positive imbalanced DHS), -50mm ≤C7SVA <+50mm (B-DHS; balanced DHS), and C7SVA <-50mm (N-DHS; negative imbalanced DHS). Correlations among the various spinal parameters were analyzed. Among all types of DHS, there was no correlation between C2-C7 angle (C2-C7A) and T1 slope. In B-DHS, other correlations among the adjacent spinal segments were maintained. In N-DHS, there was no correlation between C2-C7A and TK, and in P-DHS, there was also no correlation between TK and lumbar lordosis. The loss of compensation at the cervicothoracic junction was observed in all DHS types. B-DHS showed decompensation only at the cervicothoracic junction. N-DHS presented additional decompensation of the thoracic spine, and P-DHS showed decompensation between the thoracic and lumbar spine. Evaluation of global sagittal spinal balance is important for determining global spinal compensation associated with DHS and when considering treatment strategy.

Dropped Head Syndrome: A Systematic Review.

This study was a systematic review. To reveal demographic factors, etiologies, response to treatment, and to propose a novel treatment algorithm for dropped head syndrome (DHS). DHS is a rare condition defined by weakness of the cervical paraspinal muscles resulting in passively correctable flexion of the cervical spine. Patients present with neck pain, difficulty eating, and impaired horizontal gaze. Because of the rarity of DHS, a paucity of information exists with regard to demographics, etiology, and relative superiority of medical and surgical treatment. We conducted a systematic literature review by searching PubMed for "dropped head syndrome," "chin on chest," "isolated neck extensor myopathy" (INEM), and "camptocephalia." Inclusion criteria were English-language articles that applied a specific treatment regimen with outcome data. A binomial logistic regression analysis was then performed to determine which covariates (age, sex, and treatments) were predictive of a positive response to treatment. A total of 129 patients were described in 74 studies. Mean age was 63.6 and 63% were female. More than two-thirds of all patients fell into just 4 diagnostic categories (isolated neck extensor myopathy, 31.8%; Parkinson's, 20.2%; myasthenia gravis, 12.4%; amyotrophic lateral sclerosis, 7.0%). Overall positive response to treatment was 64.3%; primary medical treatment (73.5%), immune suppression (78.9%), and a combination of both (87.5%). Surgery was 93.8% successful. A treatment algorithm focused on appropriate diagnosis, initial medical management, with surgical evaluation only after failure of medical treatment was proposed. Treatment for DHS starts with accurate diagnosis of the underlying etiology and is often associated with neuromuscular disease. A treatment algorithm for appropriate management of these patients was proposed. A trial of medical management and/or immunomodulators is warranted. Failing nonoperative management, surgery is predictive of a positive outcome. Level V.

P89-F Neurophysiological findings in 11 cases of dropped head syndrome

Background Dropped head syndrome (DHS) is a rare disorder characterized by weakness of the cervical paraspinal muscles with inability to extend the neck. It can be a part of several generalized neuromuscular processes or secondary to local conditions. Also, a restricted myopathy, “isolated neck extensor myopathy” (INEM), has been described. Material and methods 11 cases of DHS were referred to our Neurophysiology department in the period 2015–2018 for evaluation, with suspected neuromuscular etiology. ENG/EMG, repetitive nerve stimulation, sfEMG or all of them were performed according to the clinical suspicion and findings. Results In 4 patients, neurophysiological studies were compatible with neuromuscular junction disorder (one of them with overlapping myopathic features): findings showed clearly positive repetitive nerve stimulation in facial and spinal nerves. sfEMG mean consecutive difference (MCD) was above 80 μs, with some blocking, in these cases. In 6 patients, EMG proved myopathic changes: mild spontaneous activity in all of them (positive waves, mainly), and short, small motor unit action potentials in 5. Of particular interest, in the myopathic group, sfEMG showed increased jitter in few pairs of fiber, but normal mean jitter and without blocking. In 2 cases, the evaluation could not be concluded. The final diagnosis in 4 cases was myasthenia gravis, and myopathy in 7 (inflammatory myopathy or isolated neck extensor myopathy). All patients improved with specific treatment. Conclusions In our series, miastenia gravis and myopathy (INEM or inflammatory) were the main etiologies. Neurophysiological studies revealed distinctive features in both groups, with high diagnostic yield.

SURGICAL CORRECTION OF SEVERE AND FIXED CHIN-ON-CHEST DEFORMITY.

ABSTRACTObjective:To describe a successful surgical treatment for the challenging severe and fixed chin-on-chest deformity due to isolated neck extensor myopathy (INEM).Background data:INEM is an idiopathic cause of dropped head syndrome (DHS) that results in severe cervicothoracic kyphosis, defined as chin-on-chest deformity. The existing literature on surgical management is limited, with outcomes ranging from poor to excellent. INEM may present to the spinal surgeon for consideration of surgical management.Methods:The authors present a technique that uses a staged posterior and anterior approach combined with osteotomies and corpectomy to correct the severe and fixed deformity. A state of the art anterior and posterior instrumentation system was used.Results:At the three-months follow-up, there was good deformity correction and the patient's satisfaction was high, with no neurological deterioration occurring.Conclusions:The technique illustrated in this study represents a successful option to treat this debilitating deformity. More evidence is needed to set up a definitive algorithm for the management of this condition. Level of evidence IV, Case Report.

A Case of Isolated Neck Extensor Myopathy Responding Favorably to Immunotherapy

We report on a case of a 79-year-old man with dropped head syndrome, where diagnostic tests, ruling out other differential diagnoses, confirmed the relatively rarely occurring condition known as isolated neck extensor myopathy. Biopsy of the neck extensor musculature was with classical pronounced fibrosis, without signs of myositis. The patient was treated with 3 cycles of corticosteroids with pronounced clinical improvement of his symptoms. This emphasizes the importance of considering isolated neck extensor myopathy as a differential diagnosis when encountered with a patient with dropped head syndrome and the importance of trying therapy with corticosteroids even when myositis is absent.

An Unusual Cause of Dysphagia

We describe an 80 year old lady presenting with a six week history of neck pain, progressing to painless severe neck weakness and dysphagia. The absence of other positive symptoms, or tests, and an EMG consistent with a myopathic process, confirmed the diagnosis of dropped head syndrome secondary to an isolated neck extensor myopathy. A trial of steroids for one month was of subjective benefit only.

Isolated Neck Extensor Myopathy Causing a Dropped Head: A Case Report

This report is of a 71-year-old woman who presented with a dropped head and difficulty in extending her neck. She was diagnosed with isolated neck extensor myopathy. Conservative treatment (use of a cervical collar and muscular strength training) temporarily improved her symptoms. However, destruction of cervical vertebrae and myelopathy progressed secondary to repeated microfractures from mechanical stress. The patient underwent 2-stage combined anterior and posterior decompression and fusion using autografts. At one-year follow-up, numbness of the bilateral upper limbs had resolved, and bone union was achieved. The patient was able to look straight ahead and was very satisfied with the outcome.

Dropped head syndrome due to myogenic atrophy --- a case report of surgical treatment

We report a case of a 69-year-old man with dropped head syndrome associated with isolated neck extensor myopathy (INEM). Over a period of 2 years, he exhibited progressive inability to lift his chin off his chest, resulting in the dropped head position that impaired his activities of daily living. He had a disturbed gait with severe imbalance of spinal alignment. Computed tomography revealed osseous contracture of cervical vertebral bodies in flexed position. Anterior combined posterior reconstruction surgery yielded a successful outcome in his activities of daily living, including his walking balance of spinal alignment. Pathologic study confirmed myogenic atrophy in the cervical extensor muscles. We suggest that consideration for surgical management should be given to dropped head syndrome especially due to INEM.

Dramatic Response of Dropped Head Sign to Treatment with Steroid in Parkinson's Disease: Report of Three Cases

Neck dystonia is the most common cause of dropped head sign in parkinsonism. Isolated neck extensor myopathy, which is a rare condition, can also cause dropped head sign in parkinsonism, but no improvement has been achieved with immunosuppressive therapy. We report three cases of treatable neck extensor myopathy causing dropped head sign in patients with Parkinson's disease. Needle electromyography and magnetic resonance imaging suggested a restrictive active myopathy affecting neck extensor muscles. All cases responded dramatically to steroid therapy. Routine needle electromyography should be performed to explore treatable myopathy in Parkinson's disease.

Isolated Neck Extensor Myopathy: Is it Responsive to Immunotherapy?

To determine if isolated neck extensor myopathy (INEM) is responsive to immunosuppressive treatment. We retrospectively reviewed charts of patients with INEM from 2002 to 2008 to identify patients and determine the response to immunomodulatory therapy. Clinical, electrodiagnostic, histologic, and radiographic data were reviewed. Four patients were identified during the study period. Three were women. The age of onset of neck extensor weakness ranged from 58 to 78 years. Serum creatine kinase levels were within normal limits in all patients. None had clinical, laboratory, or electrophysiological findings to suggest a generalized neuromuscular disorder. On electrodiagnostic studies, all patients had myopathic changes with or without irritative features in cervical paraspinal muscles. No inflammation was present on muscle biopsy from three of the patients. All patients received one or more immunosuppressive agents. Neck strength improved by 1 point or greater on the Medical Research Council scale in all subjects with a peak response observed between 3 and 6 months after treatment initiation. A trial of immunosuppressive agents should be offered to patients with INEM because a subset will improve. Rigorously defined, INEM is a noninflammatory myopathy. However, a focal myositis could be missed on muscle biopsy and may explain the favorable response to treatment.

109. Isolated neck extensor myopathy in patients with multiple sclerosis

109. Isolated neck extensor myopathy in patients with multiple sclerosis

Cervical myelopathy caused by dropped head syndrome

The authors present a rare case of cervical myelopathy caused by dropped head syndrome. This 68-year-old woman presented with her head hanging forward. After 1 month, she was admitted to the medical service because of head drop progression. Examination of biopsy specimens from her cervical paraspinal muscles showed nonspecific myopathic features without inflammation, and isolated neck extensor myopathy was diagnosed. The patient's condition did not respond to the administration of corticosteroids. During follow up as an outpatient, the patient's head drop continued to gradually progress. At 1 year after onset, she developed bilateral weakness of the upper and lower extremities, clumsiness of the hands, and gait disturbance. A radiograph of the cervical spine obtained in a standing position showed a pronounced kyphotic deformity and instability at the level of C4-5. Magnetic resonance imaging demonstrated spinal cord compression at C-3 and C-4. The patient underwent a C3-4 laminectomy and occipitocervicothoracic fixation. Gait and hand coordination gradually improved, and she was able to walk with no support 1 month postoperatively. Surgical fixation was beneficial in this patient with dropped head syndrome, myelopathy, and cervical instability.

Response of the dropped head/bent spine syndrome to treatment with intravenous immunoglobulin

Weakness of neck extension causing a dropped head may result from many neuromuscular disorders. One etiology is isolated neck extensor myopathy. A similar focal myopathy of the lower axial muscles may cause the bent spine syndrome, which manifests as flexion of the trunk and inability to stand upright. Combination of both dropped head and bent spine myopathies is uncommon. Inflammation is usually not pronounced in these conditions and response to immunosuppressive treatment is rare. We present an 81-year-old man who developed progressive weakness of neck and trunk extension over several months, with a prominent inflammatory process in the thoracic paraspinal muscles, which responded dramatically to treatment with intravenous immunoglobulin (IVIg). This case, together with other rare reports, suggests that the presence of inflammation in the biopsy of an affected muscle may predict treatment response.

0255 A case report of isolated neck extensor myopathy in Chinese

0255 A case report of isolated neck extensor myopathy in Chinese

Myopathic dropped head syndrome: a syndrome of mixed aetiology

We report two patients with myopathic dropped head syndrome, a rare and interesting neuromuscular syndrome characterised by a predominant weakness of the neck extensor muscles. The first patient, a middle aged Chinese man, presented with progressive weakness of neck extension but his clinical course later stabilised despite a lack of response to corticosteroids. Muscle biopsy revealed a necrotising myopathy with no evidence of inflammation. This patient supports the existence of an idiopathic restricted non-inflammatory myopathy, a so called isolated neck extensor myopathy syndrome which is recognised to pursue a less progressive, more benign course. Our second patient had histopathological evidence for polymyositis; there was a favourable response to steroids. Our cases underscore the fact that there may be a spectrum of pathological processes associated with the myopathic dropped head syndrome ranging from non-inflammatory muscle necrosis to a full blown inflammatory myositis.

3-33-05 Isolated neck extensor myopathy: The first report of outside USA and association with parkinsonism

3-33-05 Isolated neck extensor myopathy: The first report of outside USA and association with parkinsonism

Contact eczema in agricultural workers.

We report four patients with ``dropped head syndrome,99 a recently described nonprogressive myopathy characterized by severe neck extensor weakness.This relatively benign condition may be confused with more ominous neuromuscular disorders that also present with prominent neck weakness. We compared clinical and laboratory data from the patients with dropped head syndrome with findings from patients with head drop caused by other neuromuscular conditions. Patients with ``isolated neck extensor myopathy,99 a term we prefer to ``dropped head syndrome,99 could be readily identified with electrophysiologic, radiographic, and histologic studies. <b>NEUROLOGY 1996;46: </b> 917-921