Inaugural dropped head syndrome and camptocormia in inflammatory myopathies: a retrospective study.

OBJECTIVES: Inaugural axial muscle involvement, defined as dropped head syndrome (DHS) and/or camptocormia (CC), is poorly described in inflammatory myopathies (IM). This study aimed to further characterize IM patients with inaugural DHS/CC, their outcome and care management. METHODS: This retrospective study included IM patients diagnosed between 2000 and 2021. The main inclusion criterion was IM revealed by axial muscle deficit (DHS/CC). RESULTS: Twenty-seven patients were included; median (IQR) age at first symptoms was 66.0 years (55.5-75.0); 21 were female (77.8%). There were nine IBM, 33.3%, nine overlap myositis (OM, 33.3%), five DM, 18.5%, two immune checkpoint inhibitor-related myositis (7.4%), one focal myositis (3.7%) and one myositis with anti-Hu antibodies (3.7%). Age at first symptoms was ≤70 years in 16 patients (59.3%), including all DM patients and 8/9 OM patients (88.9%). In this group, partial remission of the disease was obtained in 9/16 (56.3%) and complete remission in 1/16 patients (6.3%); regression of DHS/CC was achieved in 3/16 patients (18.8%). Conversely, in the group of 11 patients aged >70 years at first symptoms, there were eight IBM (72.7%). Partial remission was obtained in 5/11 patients (45.5%), the disease was stable in 6/11 patients (54.5%); no complete remission was obtained nor regression of DHS/CC. CONCLUSION: The analysis of IM patients with inaugural DHS/CC delineates two groups of patients according to the age at first symptoms in terms of clinical and outcome specificities, and proposes an adapted diagnostic and care management approach to prevent long-term complications.

Radiological features of dropped head syndrome.

BACKGROUND: This study aimed to elucidate the specificity of the radiological features of Dropped head syndrome (DHS) from both reginal and global aspects. METHODS: We enrolled 53 patients with DHS (8 men, 45 women; mean age 73.5 years), and captured their lateral spinopelvic radiographs in standing position. We also selected 21 age- and sex-matched controls with cervical spondylosis. Radiological parameters were measured and compared between two groups. Compensatory and decompensatory sites were also listed for each patient. RESULTS: Radiological factors such as sagittal vertical axis (SVA), clivo-axial angle (CAA), C2-7 angle, C2-7 SVA, anterior slippage of the vertebra, alignment. C1, C2, C3, C4, C5, C6 slopes, and T1 slope-C2-7 angle showed statistically significant differences between the groups. Multivariate logistic regression showed that SVA, C2-7 SVA, T1-slope-C2-7 angle, and C1 slope were the most important factors specific to DHS. Sole cervical spine and involvement of both cervical and thoracic spine accounted for 22% and 29% of the decompensatory sites in DHS respectively. Notably, 24% of the patients did not show decompensation of the cervical spine. While, 93% exhibited compensation at the craniovertebral junction. The thoracic spine contributed 70% to DHS compensation. CONCLUSIONS: This study indicated the radiological features of DHS from both regional and global aspects. Compensatory and decompensatory DHS mechanisms varied among individuals. Compensation was likely to be developed at the neighboring sites, with the craniovertebral and thoracic junctions as the proximal and distal parts for DHS, respectively.

Dropped head syndrome in severe hypotension.

INTRODUCTION: Dropped head syndrome (DHS) is characterized by a chin-on-chest deformity, correctable by passive neck extension. METHODS: Case report. RESULTS: A patient with a heavy nephrological history (dialyzed since 5 years) complained for twenty months about a falling head. The symptomatology was punctuated by dialysis sessions, with aggravation secondary to dialysis. Clinical and paraclinical exploration for neurological, neuromuscular or orthopedic disease was negative. Analysis of the post-dialysis blood pressure showed a slow and gradual decline. From the date the patient became symptomatic, blood pressure was below 80/40mmHg. The correction of blood pressure by increasing midodrine posology resulted in a cure of DHS. DISCUSSION: Considering the negativity of explorations, the cure of symptoms following the correction of arterial hypotension, the rhythmic nature of symptomatology by dialysis, and the recurrence of symptoms concomitantly with drops in blood pressure, we suggested that hypotension was the only etiology explaining this DHS.

Characteristics of sagittal spinopelvic alignment in patients with Parkinson's disease presenting with dropped head syndrome: a case series study.

BACKGROUND: Dropped head syndrome (DHS) is a rare specific abnormal posture known to develop in Parkinson's disease (PD). This case series study aimed to characterize DHS by analyzing the characteristics of sagittal spinopelvic alignment in patients with PD/DHS. METHODS: The study included eight patients with PD/DHS (men = 3, women = 5; mean age, 68.1 ± 6.4 years). Sagittal spinopelvic alignment was evaluated using 10 parameters on whole-spine lateral radiographs. RESULTS: The time from the onset of PD to that of DHS varied among the patients from 0 to 15.3 years. In three patients, DHS appeared before the diagnosis of PD. The severity of motor symptoms at DHS onset varied from modified Hoehn and Yahr stage 1 to 4 among the patients. Although the spinopelvic parameters differed among PD/DHS individuals, all patients exhibited cervical kyphosis (cervical lordosis < 0˚). In patients with a larger T1 slope and greater thoracic kyphosis, anterocollis tended to be more severe. According to the assessment of the sagittal vertical axis (SVA), half of the patients showed a positive SVA (SVA ≥ 0 mm), whereas the other half showed a negative SVA (SVA < 0 mm). CONCLUSION: DHS appeared regardless of the duration or severity of PD. Although all patients with PD/DHS exhibited cervical kyphosis, the C7 plumb line was shifted anteriorly in half of the patients and posteriorly in the other half.

Conservative treatment for dropped head syndrome.

PURPOSE: Previous reports on the outcome of conservative treatment for dropped head syndrome (DHS) are scarce. The purpose of this study was to elucidate the efficacy of conservative treatment for DHS and to identify possible predictive factors relating to the outcome. METHODS: Among 76 DHS patients, conservative treatment (2-3 months collar application, active neck range of motion exercise, and occasional prescription of analgesics) succeeded in 17 patients (22.4%, group S, 4 male, 13 female, mean age 75.9 years). The treatment failed in the remaining 59 patients (group F). Clinical and radiological parameters were compared between the groups. Radiological findings of group S were compared between before treatment and at follow-up. RESULTS: Duration of disease was 6.6 ± 9.3 months in group S and 20.0 ± 27.6 months in group F. C2-7 angle (degree), the incidence of anterior slippage of the vertebra (%), reducibility (%), and upper thoracic kyphosis angle (degree) in group S/F were - 19.2 ± 17.5/- 34.6 ± 26.6, 23.5/62.7, 100/52, and 6.7 ± 8.6/17.9 ± 13.7, respectively. C2-7 angles were - 19.2 ± 17.5 degrees at pre-treatment and 10.2 ± 20.7 degrees at follow-up. These differences were statistically significant. CONCLUSIONS: The present study indicated that conservative treatment was successful in 22% of DHS patients, with improvement in their cervical kyphotic alignment. Shorter duration of disease, relatively smaller cervical kyphosis without anterior slippage of the vertebra, reducibility, and abundant compensation at the upper thoracic region were good indications for the success of conservative treatment.

Dropped head syndrome: a treatment strategy and surgical intervention.

PURPOSE: The pathology of dropped head syndrome (DHS) is diverse, and reports of surgery for DHS are scarce. We aimed to describe surgery for DHS and to investigate the surgical outcomes thereof. METHODS: We enrolled 40 consecutive patients (six males and 34 females; average age at surgery, 72.0 years) with DHS who underwent correction surgeries at a single institute. Short fusion (SF), with the extent of fixation mainly at the cervical region, was performed for 27 patients; long fusion (LF), involving the cervical and thoracic spine, for 13. Clinical and radiological outcomes were investigated, and factors analyzed using the Japanese Orthopedic Association Cervical Myelopathy Evaluation Questionnaire (JOACMEQ). RESULTS: All patients were able to gaze horizontally at the follow-up. Instances of five transient C5 palsy results, and five distal junctional kyphosis results were found, but no revisions were reported due to recurrence. Patients whose T1 slope-20° was smaller than the C2-7 angle postoperatively exhibited better clinical outcomes in the three domains of the JOACMEQ, regardless of the extent of fixation. CONCLUSION: For cases where the T1 slope is relatively small, and approximately 10° of cervical lordosis is predicted to be obtained postoperatively, SF is appropriate. Alternatively, for cases with higher T1 slope, obtaining a cervical lordosis over 20° has a risk of postoperative complications. For such cases, it is an option to perform an LF involving the cervical and thoracic spine.

Association between the Phase Angle and the Severity of Horizontal Gaze Disorder in Patients with Idiopathic Dropped Head Syndrome: A Cross-Sectional Study.

Background and Objectives: The phase angle, calculated by bioelectrical impedance analysis, can help elucidate the pathology of patients with idiopathic dropped head syndrome (IDHS) and explain the motor dysfunction associated with the horizontal gaze disorder. The aim of this study was to clarify the characteristics of phase angle in IDHS patients and the relationship between the phase angle and the severity of horizontal gaze disorder. Materials and Methods: This cross-sectional study included 43 female patients with IDHS and 69 healthy female volunteers. A multi-frequency segmental body composition analyzer was used to calculate body composition parameters, including whole-body and lower extremity phase angles. Propensity score (PS) matching analysis was performed to compare the body composition parameters between the IDHS and healthy groups. Variables that determine the PS were identified by correlation analysis, using the whole-body phase angle as the dependent variable. In addition, correlation analysis was performed between the severity of horizontal gaze disorder as assessed by McGregor's slope (McGS), phase angle, and other body composition parameters. Results: Unadjusted group comparisons showed no significant difference in whole-body and lower extremity phase angles between the IDHS and healthy groups. PS matching created a total of 38 matched pairs for age, height, and fat-free mass index. Although the comparison between groups of matched samples showed no significant difference in the whole-body phase angle, the lower extremity phase angle in the IDHS group was significantly lower than that in the healthy group (p = 0.033). Correlation analysis showed significant negative correlations only between McGS and whole-body (r = -0.31, p = 0.043) and lower extremity phase angle (r = -0.39, p = 0.009) in the IDHS group. Conclusions: Abnormal body composition of the lower extremities were observed in IDHS patients. Furthermore, it was suggested that horizontal gaze disorder in IDHS patients is associated with whole-body and lower extremity phase angles.

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

STUDY DESIGN: This is a retrospective study of a consecutive case series of dropped head syndrome (DHS). OBJECTIVE: To identify the characteristic features of contrast-enhanced magnetic resonance imaging (MRI) in DHS patients. SUMMARY OF BACKGROUND DATA: 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. MATERIALS AND METHODS: 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. RESULTS: 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. CONCLUSIONS: 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.

Nuchal Ligament Reconstruction Surgery for Dropped Head Syndrome: A Case Report.

CASE: An 86-year-old woman suffered from dropped head syndrome (DHS). As she was not willing to undergo fusion surgery, we proposed a novel nuchal ligament reconstruction surgery, which is not a direct correction of the malalignment but rather a recovery of the function of extensor muscles under local anesthesia. Twelve months after surgery, the patient remains satisfied and is able to maintain a horizontal gaze. CONCLUSION: This is the first report regarding a less invasive nuchal ligament reconstruction surgery successfully performed for DHS.

In inflammatory myopathies, dropped head/bent spine syndrome is associated with scleromyositis: an international case-control study.

BACKGROUND: Some myopathies can lead to dropped head or bent spine syndrome (DH/BS). The significance of this symptom has not been studied in inflammatory myopathies (IM). OBJECTIVES: To assess the significance of DH/BS in patients with IM. METHODS: Practitioners from five IM networks were invited to report patients with IM suffering from DH/BS (without other known cause than IM). IM patients without DH/BS, randomly selected in each participating centre, were included as controls at a ratio of 2 to 1. RESULTS: 49 DH/BS-IM patients (DH: 57.1%, BS: 42.9%) were compared with 98 control-IM patients. DH/BS-IM patients were older (65 years vs 53 years, p<0.0001) and the diagnosis of IM was delayed (6 months vs 3 months, p=0.009). Weakness prevailing in the upper limbs (42.9% vs 15.3%), dysphagia (57.1% vs 25.5%), muscle atrophy (65.3% vs 34.7%), weight loss (61.2% vs 23.5%) and loss of the ability to walk (24.5% vs 5.1%) were hallmarks of DH/BS-IM (p≤0.0005), for which the patients more frequently received intravenous immunoglobulins (65.3% vs 34.7%, p=0.0004). Moreover, DH/BS-IM patients frequently featured signs and/or complications of systemic sclerosis (SSc), fulfilling the American College of Rheumatology/European Alliance of Associations for Rheumatology criteria for this disease in 40.8% of the cases (vs 5.1%, p<0.0001). Distribution of the myopathy, its severity and its association with SSc were independently associated with DH/BS (p<0.05). Mortality was higher in the DH/BS-IM patients and loss of walking ability was independently associated with survival (p<0.05). CONCLUSION: In IM patients, DH/BS is a marker of severity and is associated with SSc (scleromyositis).

Proposal of a diagnostic algorithm for radiation-induced dropped head syndrome in long-term childhood cancer survivors based on a prospective study in a specialized clinical setting and a review of the literature.

PURPOSE: To prospectively assess the incidence of Dropped Head Syndrome (DHS) in childhood cancer survivors (CCS) and to develop and evaluate a diagnostic algorithm for DHS. METHODS: A systematic literature search for DHS in combination with neck radiotherapy (RT) exposure was performed. Analyses and a combination of the most common examination methods were integrated into a diagnostic algorithm. Almost all CCSs visiting the local late effects clinic between May 2020 and April 2022 were included in the study. CCS exposed to neck RT with doses ≥ 19 Gy received standardized clinical and neurological assessment and, in case of abnormal results, an MRI scan to confirm muscle atrophy. RESULTS: Two hundred and five CCS were included of whom 41 received RT to the neck with ≥ 19 Gy. In the entire cohort and in the subgroup receiving RT, 2.4% and 12% of CCS were affected by DHS, respectively. Results of clinical and neurological assessment correlated well with MRI results. Neck circumference and neck/thigh ratio were lower after neck RT. Over 50% of CCS experienced neck disability and pain. CONCLUSIONS: A relevant proportion of CCS exposed to neck RT is affected by DHS. High concordance of MRI results with the neurological examination supports the clinical value of the diagnostic algorithm. Measurement of neck circumference might be an easy tool for assessment of neck muscle atrophy in survivors at risk. IMPLICATIONS FOR CANCER SURVIVORS: Integration of a diagnostic algorithm for DHS in standard long-term follow-up care facilitates diagnosis as well as initiation of early treatment and obviates the need for invasive examinations.