Project description:Dropped head syndrome (DHS) is a relatively rare condition, with a broad differential diagnosis. This deformity has significant implications on the health and quality of life of affected individuals. While surgery seems to be an obvious therapeutic option, there is a paucity of information on surgical intervention with no clear consensus on an optimal approach or timing. We present a case of DHS in a young woman to illustrate this condition, and review the current literature. Although at present the only definitive solution for correction and stabilization of DHS is surgical intervention involving multilevel instrumented fixation and fusion, this condition requires a persistent medical workup and treatment of reversible causes before surgical intervention is contemplated.

| S-EPMC3621852 | biostudies-other

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

Project description: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.

| S-EPMC8440518 | biostudies-literature

Dropped head sign induced by transdermal application of the dopamine agonist rotigotine in parkinsonian syndrome: a case report.

Project description:Introduction'Dropped head sign' relates to a severe disproportionate antecollis in parkinsonism. We present the first report of a rotigotine-induced dropped head sign in a patient with suspected idiopathic Parkinson's disease, which was later defined as multiple system atrophy. The 'dropped head sign' is considered a rare symptom of unknown etiology in parkinsonian disorders, though a disproportionate antecollis is frequently observed in multiple system atrophy. It has also been described as a side effect of dopamine agonist medication with cabergoline and pramipexole. Rotigotine is a transdermally applied, non-ergot dopamine agonist, resulting in a continuous stimulation of dopamine receptors, which is widely used in the treatment of patients with Parkinson's disease.Case presentationWe report a case of a 64-year-old Caucasian woman with a rapidly progressive two-and-a-half-year history of a hypokinetic Parkinson's syndrome with asymmetric development of symptoms and an initially good response to levodopa medication. Due to side effects of other dopamimetic medications the patient was switched to rotigotine medication five weeks before clinical admission. Progressive antecollis without muscle weakness and prominent paraspinal muscle contraction developed within two weeks of treatment and resolved within a week after discontinuation of rotigotine and initiation of levodopa/cabergoline medication.ConclusionWhile the pathophysiology still remains unresolved, this case supports the concept of a dopaminergic imbalance as a cause of certain axial dystonias like disproportionate antecollis including the 'dropped head sign'. We believe this case is specifically useful for neurologists and general practitioners, as the easily recognizable symptom should prompt a thorough reevaluation of diagnosis and medication in patients with Parkinson's disease.

| S-EPMC3708785 | biostudies-literature

Successful treatment of refractory chylothorax with MEK inhibitor trametinib in a child with Noonan syndrome: case report

Project description:Abstract Background Refractory chylous effusions due to lymphatic dysplasia related to Noonan syndrome cause significant morbidity and mortality due to protein and immunoglobulin losses. Very few cases have been published reporting successful treatment of patients with trametinib where all conventional treatments had failed. Case summary We present a girl with Noonan syndrome and hypertrophic cardiomyopathy who presented with life-threatening refractory chylothorax where all conventional treatment options failed. She was successfully treated with mitogen-activated extracellular signal-regulated kinase inhibitor trametinib. Discussion MEK inhibition with trametinib is emerging as a possible salvage treatment option for a subset of patients with Noonan syndrome and severe pulmonary lymphangiectasia. More experience is required to establish optimal treatment regimen and long-term outcomes.

| S-EPMC10133994 | biostudies-literature

Dropped head and man-in-barrel syndrome in amyotrophic lateral sclerosis.

Project description:We report a case of progressive symmetric brachial weakness followed by cervical muscle weakness. The electromyogram confirmed the diagnosis of amyotrophic lateral sclerosis. After 3 years the patient remained able to walk unassisted and without significant bulbar manifestations or upper neuron signs. The concomitant presence of dropped head syndrome and man-in-barrel syndrome in an amyotrophic lateral sclerosis patient makes our case unique.

| S-EPMC5410875 | biostudies-literature

The Short and Intensive Rehabilitation (SHAiR) Program Improves Dropped Head Syndrome Caused by Amyotrophic Lateral Sclerosis: A Case Report.

Project description:Background and Objectives: Dropped head syndrome (DHS) is a syndrome that presents with correctable cervical kyphotic deformity as a result of weakening cervical paraspinal muscles. DHS with amyotrophic lateral sclerosis (ALS) is a relatively rare condition, and there is no established treatment. This is the first case report describing the improvement of both dropped head (DH) and cervical pain after the short and intensive rehabilitation (SHAiR) program in an ALS patient with DHS. Case Report: After being diagnosed with ALS in June 2020, a 75-year-old man visited our hospital in October 2020 to receive treatment for DHS. At the initial visit, the patient's DH was prominent during standing and walking. The pain intensity of the neck was 9 out of 10 on the numerical rating scale (NRS), which was indicative of severe pain. The patient was hospitalized for 2 weeks and admitted into the SHAiR program. DH began to decrease one week after undergoing the SHAiR program and improved two weeks later. Neck pain decreased from 9 to 6 on the NRS. Results: The SHAiR program is a rehabilitation program aimed at improving DH in patients with idiopathic DHS. The program was designed to improve neck extensor and flexor function and global spinal alignment, and the program may have contributed to the improvement of DH and neck pain. Currently, reports of conservative therapies for this disease are limited to the use of cervical orthosis. Although further research is needed on the safety and indications of treatment, the SHAiR program may be a viable treatment option.

| S-EPMC8948869 | biostudies-literature

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

Project description:ObjectivesInaugural 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.MethodsThis retrospective study included IM patients diagnosed between 2000 and 2021. The main inclusion criterion was IM revealed by axial muscle deficit (DHS/CC).ResultsTwenty-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.ConclusionThe 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.

| S-EPMC10837000 | biostudies-literature

Neuroleptic malignant-like syndrome associated multiple system atrophy: report on three cases.

Project description:BackgroundMultiple system atrophy (MSA) associated with neuroleptic malignant-like syndrome (NMLS) is rare and few cases have been described in the literature.Case presentationIn the present study, three patients with MSA associated with NMLS were analyzed from January 2012 to January 2020 to characterize their clinical presentations. Data collected from the patients for analysis included general patient history, the fluctuation and severity of disease symptoms, the indicated therapies and disease progression at follow-up. All patients had histories of sudden withdrawal or reduction of levodopa prior to the onset of symptoms. Clinical presentations were characterized by hyperthermia, autonomic dysfunction, worsening of extrapyramidal symptoms, and elevated serum creatine kinase (CK) levels. During hospitalization, one patient rapidly progressed and died, while the other two patients were successfully treated.ConclusionsEarly diagnosis and treatment are very important for patient outcomes in NMLS. Notably, the correct dose and time of administration of dopaminergic medication may be key in treating NMLS.

| S-EPMC8876065 | biostudies-literature

Dropped Head Syndrome after Minor Trauma in a Patient with Levosulpiride-Aggravated Vascular Parkinsonism.

Project description: Not available

| S-EPMC4886200 | biostudies-literature