Torticaput versus Torticollis: Clinical Effects with Modified Classification and Muscle Selection

Background: Several different subtypes are distinguished in cervical dystonia, depending on their different levels of movement. In simple rotation, classified as torticollis spasmodicus, we now differentiate between torticollis and torticaput dependent on whether only the head or the neck is turned. The new classification system permits for different injection schemes. Case reports: In a retrospective study of 22 patients, we examined whether modifying the injected muscles leads to improvement in the results as evaluated in the Toronto Western Spasmodic Torticollis Rating Scale (TWSTRS). The results showed that both injection schemes do in fact lead to improvements while differentiating between caput and collum has significantly better effects. Discussion: Due to our results we recommend the classification differentiating between torticollis and torticaput type

Injection into the Longus Colli Muscle via the Thyroid Gland

Background: Anterior forms of cervical dystonia are considered to be the most difficult to treat because of the deep cervical muscles that can be involved. Case Report: We report the case of a woman with cervical dystonia who presented with anterior sagittal shift, which required injections through the longus colli muscle to obtain a satisfactory outcome. The approach via the thyroid gland was chosen. Discussion: The longus colli muscle can be injected under electromyography (EMG), computed tomography (CT), ultrasonography (US), or endoscopy guidance. We recommend using both ultrasonography and electromyography guidance as excellent complementary techniques for injection at the C5-C6 leve

Difficulties with differentiating botulinum toxin treatment effects in essential blepharospasm

Blepharospasm is a focal dystonia in which the extraocular muscles contract repetitively, leading to excessive blinking and forced eyelid closure. Botulinum toxin type A (BoNTA) is the primary symptomatic treatment for blepharospasm and its effects have been evaluated using numerous rating scales. The main scales in use today were initially used to determine whether BoNTA treatment was superior to placebo, and most controlled trials have confirmed this. More recently, these scales have been used to determine whether there are efficacy differences between different BoNTs in blepharospasm. However, although the scales used in these trials are able to differentiate the effects of BoNT from placebo, they may not be sensitive enough to differentiate between BoNTs. Most of the scales include only four possible points for each item, which would necessitate a 25% greater improvement in one group than the other to detect any differences. Current scales are also relatively insensitive to patients with mild disability who may experience mainly psychosocial problems related to their blepharospasm. Clinical trials comparing BoNTs that include substantial numbers of mildly affected patients may be unlikely to find differences because the scales do not adequately measure mild symptoms. Additional challenges with evaluating blepharospasm include the lack of precision and objectivity of current measures, symptom variability, the need to evaluate aspects of the disorder that are most important to patients, and the different types of blepharospasm. Although no single scale may be able to capture all relevant aspects of blepharospasm, more sensitive and patient-centered scales are needed

The role of glutamatergic neurotransmission in the motor and non-motor symptoms in Parkinson's disease: clinical cases and a review of the literature

Glutamate is the major excitatory neurotransmitter in the central nervous system and, as such, many brain regions, including the basal ganglia, are rich in glutamatergic neurons. The importance of the basal ganglia in the control of voluntary movement has long been recognised, with the effect of dysfunction of the region exemplified by the motor symptoms seen in Parkinson's disease (PD). However, the basal ganglia and the associated glutamatergic system also play a role in the modulation of emotion, nociception and cognition, dysregulation of which result in some of the non-motor symptoms of PD (depression/anxiety, pain and cognitive deficits). Thus, while the treatment of PD has traditionally been approached from the perspective of dopaminergic replacement, using agents such as levodopa and dopamine receptor agonists, the glutamatergic system offers a novel treatment target for the disease. Safinamide has been approved in over 20 countries globally for fluctuating PD as add-on therapy to levodopa regimens for the management of 'off' episodes. The drug has both dopaminergic and non-dopaminergic pharmacological effects, the latter including inhibition of abnormal glutamate release. The effect of safinamide on the glutamatergic system might present some advantages over dopamine-based therapies for PD by providing efficacy for motor (levodopa-induced dyskinesia) as well as non-motor (anxiety, mood disorders, pain) symptoms. In this article, we discuss the potential role of glutamatergic inhibition on these symptoms, using illustrative real-world examples of patients we have treated with safinamide

Rating scales for cervical dystonia: a critical evaluation of tools for outcome assessment of botulinum toxin therapy

Botulinum neurotoxin is the therapy of choice for all forms of cervical dystonia (CD), but treatment regimens still vary considerably. The interpretation of treatment outcome is mainly based on the clinical experience and on the scientific value of the rating scales applied. The aim of this review is to describe the historical development of rating scales for the assessment of CD and to provide an appraisal of their advantages and drawbacks. The Tsui score and the Toronto Western Spasmodic Torticollis Rating Scale (TWSTRS) have been widely employed in numerous clinical studies as specific instruments for CD. The obvious advantage of the Tsui score is its simplicity so that it can be easily implemented in clinical routine. The TWSTRS allows a more sophisticated assessment of functional features of CD, but only the Tsui score includes a rating for tremor. Other benefits of the TWSTRS are the disability and pain subscales, but despite its value in clinical trials, it might be too complex for routine clinical practice. None of the rating scales used at present has been rigorously tested for responsiveness to detect significant changes in clinical status after therapeutic interventions. Moreover, clinical data support a new classification of CD leading to a differentiation between head and neck subtypes. As the current rating scales are not able to cover all these aspects of the disorder, further research is needed to develop a valid and reliable instrument which considers the most current classification of CD

Pramipexole Extended Release: A Novel Treatment Option in Parkinson's Disease

Pramipexole, the most commonly prescribed dopamine agonist worldwide, meanwhile serves as a reference substance for evaluation of new drugs. Based on numerous clinical data and vast experiences, efficacy and safety profiles of this non-ergoline dopamine agonist are well characterized. Since October 2009, an extended-release formulation of pramipexole has been available for symptomatic treatment of Parkinson's disease. Pramipexole administration can be cut down from three times to once a day due to the newly developed extended-release formulation. This is considerable progress in regard to minimizing pill burden and enhancing compliance. Moreover, the 24 h continuous drug release of the once-daily extended-release formulation results in fewer fluctuations in plasma concentrations over time compared to immediate-release pramipexole, given three times daily. The present study summarizes pharmacokinetics and all essential pharmacological and clinical characteristics of the extended-release formulation. In addition, it provides all study data, available so far, with regard to transition and de-novo administration of extended-release formulation for patients with Parkinson's disease. It further compares efficacy and safety data of immediate-release pramipexole with the extended-release formulation of pramipexole

Botulinum neurotoxin in cervical dystonia revisited — recent advances and unanswered questions

Cervical dystonia (CD) usually presents a complex pattern of head/neck movements accompanied by tremor, myoclonic jerks and a wide spectrum of non-motor disturbances such as pain, depression, anxiety, and sleep problems. This is the most challenging indication for botulinum neurotoxin (BoNT) treatment. It can offer significant improvement, but it can be difficult after the first injection. Thorough examination and identification of the proper CD pattern, the identification of the muscles responsible, and adjusting doses given precisely under ultrasound and/or electromyographic guidance seem to be the key success modifiers. Nevertheless, this is a lifelong treatment and should be planned and conducted carefully to avoid failures and drop outs. The aim of this paper was to examine the current concepts in terms of anatomy, physiology and CD patterns (Col-Cap concept) as well as the proper dosages and any possible obstacles impeding successful treatment

Cervical dystonia — improving the effectiveness of botulinum toxin therapy

Introduction. Cervical dystonia is the most frequent form of focal dystonia. It is characterised by involuntary muscular contractions resulting in abnormal head/neck and shoulder movements and postures, which can be associated with tremor and pain. Local intramuscular injections of botulinum toxin type A (BoNT-A) is the treatment of choice, being both effective and well-tolerated. However, a considerable number (c. 30%) of patients discontinue this treatment. The aim of this review was to analyse the factors possibly responsible for treatment failures of cervical dystonia (CD), with special regard to the new classification known as the ‘Col-Cap’ concept and non-motor symptoms.Clinical implications. Several factors analysed in this review are responsible for effective treatment: proper diagnosis of dystonia and exclusion of pseudodystonias, correct recognition of dystonia pattern and identification of new patterns according to the Col-Cap concept, muscle selection and precise injections under electromyography (EMG) and/or ultrasonography (US) guidance. Furthermore, concomitant diagnosis and treatment of non-motor symptoms such as depression, anxiety, fatigue, sleep problems, phobias and stigmatisation are crucial in obtaining the best overall effect of the treatment. Primary and secondary immunisation and non-responsiveness seem to be marginal problems nowadays due to a low potential of new BoNT-A formulations to produce neutralising antibodies.Future directions. There is a need for new and relevant scales combining the Col-Cap concept patterns with non-motor symptoms and quality of life. There is also a lack of specific rehabilitation protocols which could enhance BoNT-A treatment results