Factors that impact dysphagia and discontinuance of oral intake in patients with progressive supranuclear palsy.

Objective: To evaluate the swallowing function in the advanced stages of progressive supranuclear palsy (PSP) and clarify the factors that lead to adjustment of food consistency and discontinuation of oral intake. Methods: A total of 56 patients with PSP were recruited. Based on medical records, information about the basic attributes, clinical features (including axial rigidity and dementia), food intake, the results of a videofluoroscopic swallowing study (VFSS), and the timing of nasogastric tube transition and gastrostomy were extracted. From the VFSS images, the presence or absence of aspiration and retrocollis were assessed. Results: The average age at the onset, diagnosis, and the final follow-up examination were 67.6 ± 6.4 years, 71.6 ± 5.8 years, and 75.4 ± 5.6 years, respectively. The average duration of illness was 64.6 ± 42.8 months. Twenty-four individuals (42.9%) were continuing oral intake, while 32 were tube-fed, among whom 16 (50.0%) underwent gastrostomy tube placement. There were significant differences in the duration from the disease onset to tube feeding between the patients with and without cognitive decline at the time of the diagnosis (p < 0.01) and in the duration from the initial VFSS to tube feeding between the patients with and without aspiration on the initial VFSS (p < 0.01). There were significant differences in the duration from the diagnosis to tube feeding and from the initial VFSS to tube feeding between patients with and without axial rigidity at the time of the diagnosis (p < 0.05 and p < 0.05, respectively). Additionally, there was a significant association between axial rigidity and retrocollis (p < 0.01). Conclusion: Cognitive decline, axial rigidity and retrocollis, which are associated with the deterioration of dysphagia in PSP, are the highest risk factors for the discontinuation of oral intake. The early identification of these factors associated with the progression of dysphagia can contribute to the improvement of patient care and management.

Retroform Cervical Dystonia: Target Muscle Selection and Efficacy of Botulinum Toxin Injection.

Introduction: Retroform cervical dystonia (RCD), which includes retrocaput and retrocollis, is a rare form of cervical dystonia. Few reports have been published on RCD. The present study aimed to characterize the target muscles involved in RCD and the efficacy of botulinum toxin type A (BTX-A) injection. Methods: Patients with consecutive cervical dystonia with RCD as the most problematic feature were retrospectively analyzed over a 10-year period. Target muscles were screened and confirmed based on clinical evaluation, single-photon emission computed tomography, and electromyography. In addition, efficacy and adverse events following BTX-A injection in patients with RCD were evaluated. Results: A total of 34 patients with RCD were included, 18 of whom presented with retrocaput and 16 with retrocollis. The most frequently injected muscles in RCD were splenius capitis (SPCa, 97.1%) and semispinalis capitis (SSCa, 97.1%), followed by levator scapulae (LS, 50.0%), rectus capitis posterior major (RCPM, 47.1%), trapezius (TPZ, 41.2%), and sternocleidomastoid muscle (SCM, 41.2%). Besides cervical muscles, the erector spinae was also injected in 17.6% of patients. Most muscles were predominantly bilaterally injected. The injection schemes of retrocaput and retrocollis were similar, possibly because in patients with retrocollis, retrocaput was often combined. BTX-A injection achieved a satisfactory therapeutic effect in RCD, with an average symptom relief rate of 69.0 ± 16.7%. Mild dysphagia (17.6%) and posterior cervical muscle weakness (17.6%) were the most common adverse events. Conclusion: SPCa, SSCa, LS, RCPM, LS, and SCM were commonly and often bilaterally injected in RCD. Patients with RCD could achieve satisfactory symptom relief after BTX-A injection.

Prevention of Tracheo-Innominate Artery Fistula by Using an Adjustable Tracheostomy Tube.

Tracheo-innominate artery fistula (TIF) is a severe complication associated with a long-term tracheostomy, and TIF-associated bleeding has a high mortality rate. Here, we report two patients who were considered to be at high risk of developing TIF due to retrocollis after tracheostomy. The patients were an 82-year-old woman with Parkinson's disease (PD) and a 64-year-old man with multiple system atrophy (MSA). Both patients underwent tracheostomy at an advanced stage and later showed retrocollis. Colored and 3D-reconstructed computed tomography (CT) showed tracheal deformation into a C curve, with the tip of the tracheostomy tube attached to the anterior wall, where the innominate artery transverses. Since they were considered to be at high risk of developing TIF, we used an adjustable tracheostomy tube. Follow-up CT revealed that the tip of the new tracheostomy tube had separated from the tracheal anterior wall. Although retrocollis is rare in PD and MSA, it can develop at the end stage of these diseases. An adjustable tracheostomy tube may be an option for preventing TIF development in cases where surgical intervention would be difficult.

Levodopa-responsive retrocollis on the background of choreic dyskinesia.

Purpose/aim: Retrocollis can substantially disturb the daily living of individuals with Parkinson's disease (PD). Clinician often encounter the difficulty in managing the retrocollis. Materials and Methods: We describe a patient with PD who presented with choreic dyskinesia and levodopa-responsive retrocollis. Results: The patient had dyskinesia and the off periods, and received levodopa (700 mg, 14 times/day). The patient received levodopa-carbidopa intestinal gel (LCIG) treatment. After several months, the patient complained of difficulty in swallowing and speech due to severe retrocollis. Thirty minutes following a fast levodopa infusion of LCIG, the retrocollis improved. As a result, a frontal view was obtained, and her talking abilities showed improvement. Conclusions: Severe retrocollis can be superimposed on choreic dyskinesia, and it was likely to increase during the off periods. Duodenal levodopa infusion may reduce the severity of retrocollis.

Cervical dystonia in Parkinson’s disease: Retrospective study of later-stage clinical features

@#Objective: Cervical dystonia (CD) is a clinically under-recognized symptom occurring at the later- to end-stages of Parkinson’s disease (PD). The frequency of CD and its influence on prognosis have not been well studied. Here, we conducted an in-depth examination of CD incidence and impact on disease progression in later-stage PD. Methods: We retrospectively reviewed the clinical features of 22 deceased patients with sporadic PD treated at a hospital in Japan from 1983 to 2008. Results: The most common cause of death in PD was pneumonia. CD, in particular retrocollis, was frequent in the later stages of the disease in elderly patients (9/22, 40.9%). Pneumonia incidence increased sharply in the later period with CD. There was a positive trend between CD duration and duration of pergolide use. Conclusion: Analysis revealed that CD increases markedly in late- to end-stage PD, which may be associated with aspiration pneumonia due to dysphagia. Pathological mechanisms underlying CD might be influenced by treatments including dopamine agonists. Prevention of CD may increase quality of life and prolong survival of PD patients.

Dystonic opisthotonus: a "red flag" for neurodegeneration with brain iron accumulation syndromes?

Back arching was reported in one of the very first patients with neurodegeneration with brain iron accumulation syndrome (NBIAs) published in 1936. However, recent reports have mainly focused on the genetic and imaging aspects of these disorders, and the phenotypic characterization of the dystonia has been lost. In evaluating patients with NBIAs in our centers, we have observed that action-induced dystonic opisthotonus is a common and characteristic feature of NBIAs. Here, we present a case series of patients with NBIAs presenting this feature demonstrated by videos. We suggest that dystonic opisthotonus could be a useful "red flag" for clinicians to suspect NBIAs, and we discuss the differential diagnosis of this feature. This would be particularly useful in identifying patients with NBIAs and no iron accumulation as yet on brain imaging (for example, as in phospholipase A2, group IV (cytosolic, calcium-independent) [PLA2G6]-related disorders), and it has management implications.

Retrocollis, anterocollis or head tremor may predict the spreading of dystonic movements in primary cervical dystonia / Retrocolis, anterocolis ou tremor cefálico podem predizer a progressão dos movimentos distônicos em pacientes com distonia cervical primária

BACKGROUND AND PURPOSE: Few studies have attempted to develop clinical predictors for cervical dystonia (CD) aiming at progression of the dystonic movement. METHOD: We retrospectively evaluated 73 patients with primary CD who underwent treatment with Botulinum toxin type-A (BTX-A). The patients were assembled in two groups according to the spread of dystonia during follow-up: spreading and non-spreading CD. We performed a binary logistic regression model using spreading of cervical dystonia as dependent variable aiming to find covariates which increase the risk of spreading. RESULTS: Our logistic regression model found the following covariates and their respective risk ratios: time of disease >18.5 months=2.4, retrocollis=1.9, anterocollis=1.8, head tremor=1.6. CONCLUSION: Time of disease >18.5 months, retrocollis, anterocollis and head tremor may predict spreading of dystonic movement to other regions of the body in CD patients.

INTRODUÇÃO: Poucos estudos avaliam preditores clínicos de progressão dos movimentos distônicos, para além da região cervical, em pacientes com distonia cervical (DC) primária. MÉTODO: Avaliamos, retrospectivamente, 73 pacientes com DC primária, que tinham sido submetidos ao tratamento com a toxina botulínica tipo A (BTX-A). Estes pacientes foram divididos em dois grupos de acordo com a progressão ou não da DC para outras áreas do corpo. Aplicamos um modelo de regressão logística binária usando a progressão da distonia como variável dependente com o objetivo de identificar co-variáveis que aumentassem o risco de progressão. RESULTADOS: O modelo de regressão logístico identificou as seguintes co-variáveis como preditoras de progressão e seus respectivos índices de risco: tempo de doença >18,5 meses=2,4, retrocolis=1,9, anterocolis=1,8, tremor cefálico=1,6. CONCLUSÃO: Tempo de doença >18,5 meses, retrocolis, anterocolis, e tremor cefálico podem predizer a progressão do movimento distônico para outras regiões do corpo em pacientes com DC primária.