Adult-onset Idiopathic Torticollis

Definition/Description

Adult torticollis, also known as cervical dystonia or spastic torticollis, is a condition in which the muscles that control the neck are locked in constant involuntary contraction. These contractures often result in twisting of the neck, repetitive movements or abnormal postures [1]. Depending on the severity, it can be a very painful condition that can cause great distress. The presentation of torticollis is defined using causal terms, such as acute congenital chronic acquired idiopathic or secondary. [2][1]

Epidemiology/Etiology

Idiopathic cervical dystonia (ICD) is the most common form of adult-onset focal dystonia [1]. The prevalence of dystonia is difficult to determine. Early-onset cases of primary dystonia among Ashkenazi Jews in the New York area may be 11.1 per 100,000, based on best available prevalence estimates Late cases are 60 per 100,000 in northern England and 300 per 100,000 in Italy over 50. The graph in Figure 1 shows that most of the case reports occurred in the age group of 31 to 40 years This shows the prevalence of adult onset Idiopathic cervical dystonia. [3][4][5]

This common disorder is characterized by involuntary muscle contractions of the neck musculature; however, the pathogenesis in most cases is unknown. Although the pathogenesis is idiopathic, two causes have been postulated and studied extensively with clear evidence They play a role in the onset of adult-onset focal dystonia. These two reasons are:

• Genetics: Three observations support the hypothesis that abnormal genes cause some ICD. [6] These hypotheses are as follows: (i) in families with a genetic basis for childhood-onset idiopathic torsade dystonia, family members may have Focal cervical or segmental dystonia (ii) It has been recognized since 1896 that torticollis may affect siblings and adult-onset torticollis may affect multiple generations (iii) Focal A significant proportion of first-degree relatives of dystonic patients have focal dystonia or tremor, and in The prevalence of focal dystonia in families of ICD patients is higher than expected.
• Trauma: The prevalence of trauma-related cervical dystonia ranges from 5-21%. [1] Injuries sustained as a result of trauma present with immediate pain followed within days by cervical dystonia and almost total immobility. No relief in the morning, dystonia persists during sleep. The presence of cervical dystonia persisted for up to four years after follow-up and was poorly responsive to medication and botulinum toxin. It is important to note that none of the traumatized patients had a family history of dystonia. [6]

Another possible cause of ICD that has been studied is a structural abnormality of the brain. T2 bilateral abnormalities of the lenticular nucleus in conventional MRI class IV studies in the ICD. However, abnormalities were detected only on calculated T2 values; no apparent signal changes could be Identification by visual inspection of T2-weighted images. Echogenicity of these structures on transcranial ultrasonography revealed structural changes in the lenticular nucleus in the predominantly contralateral globus pallidum in a patient with adult-onset focal dystonia. [4]

Characteristics/Clinical Presentation

Adult torticollis, also known as cervical dystonia, is manifested by rotation of the head or jaw toward the shoulders. [6] Recurrent head jerks may be accompanied by intermittent clonic or tremor spasms. [1] Research by Qiyu Chen et al. Indicates the presence of head tremors (HT) and its type depend on the patient’s dominant posture (rear neck patients are more likely to develop HT than front neck patients), age (earlier onset compared with patients without HT), and duration (more compared with longer disease duration) )[7].

Cervical dystonia can cause disabling pain, and most patients exhibit a lack of postural control. [1] Unlike other dystonias, cervical dystonia is painful approximately 75% of the time. [6] Altered vestibular function and perception of body orientation, eg There may be vestibular hyperreactivity in the vestibulo-ocular reflex and difficulty recognizing posture and visual verticality. [1] These symptoms are known to vary in nature and direction. [2] In torticollis, the side to which the jaw rotates determines how the condition is described. For example, if A left-turning jaw is known as “left-turning torticollis” [2].

Differential Diagnosis

Conditions that a physical therapist should consider during initial evaluation and subsequent treatment include, but are not limited to:

• Parkinson’s disease: may present with a torticollis-like head position. May test positive for other neurological involvement. Tremors will appear in a sinusoidal oscillatory pattern. The therapist may also notice instability in gait stiffness and dyskinesia. [1][8][9]
• Posttraumatic dystonia: History of recent trauma with dystonia in an area of the body. Considered a type of secondary dystonia, onset of symptoms may occur immediately or up to 12 months after the insult. An estimated 5-21% of patients with cervical dystonia have a history of neck trauma head or neck, although it’s unclear whether insults were the “trigger” for this condition. [8]
• Wilson’s disease: Wilson’s disease is an inherited disorder that causes copper to build up in the body. Screening for Wilson disease is indicated in patients under the age of 40 with gradual onset of unexplained symptoms. [1][10]
• Adult-onset idiopathic torticollis: Symptoms develop gradually, and up to 75% of patients report concurrent neck pain. There may also be jerky movements, brief cramps, stiffness/tightness in shoulder elevation, and tremors. [1][8][3][11]

Patients with any form of adult torticollis will eventually be treated with an injury-based approach.

Examination

Evaluation of adults with signs or symptoms of torticollis follows a normal cervical examination, beginning with a thorough history followed by a physical examination:

History

• First few episodes with unusual head poses
• Neck pain
• Headache
• Birth history
• Family history
• Medication history
• Trauma
• Recent infection

Physical Examination

Vitals

• Height
• Body-weight
• Blood pressure
• Pulse rate
• Respiration rate
• Temperature (if indicated)

*Establish a baseline of the patient’s overall health status*

Observation in Standing

Posture:

• Head alignment on cervical spine
• Cervico-thoracic spine
• Scapula
• Glenohumeral joint
• Arm & forearm
• Hand

Soft tissue:

• Muscular atrophy
• Muscular spasms
• Mass in tissue
• Phasic jerking of muscle
• Shape/symmetry of structures BIL

The examination may also include the commonly used outcome measure, the Toronto Western Spastic Torticollis Rating Scale (TWSTRS). The scale includes an assessment of head neck and shoulder dystonia position, effectiveness of sensory cues, and length of time the patient can maintain this position Head at midline and range of head and neck motion. [1]

*Identifies key injuries in musculoskeletal disorders and builds a comprehensive understanding of relevant structures as indicated by posture. Patients experience an out-of-normal alignment of the head, often rotating and bowing to one side. *

Neurological Screen

• Reflexes (C5,C6,C7)
• Myotomes (C4-T1)
• Dermatomes (C4-T1)

*To determine if there is any central nervous system involvement, abnormal findings are not found due to torticollis. *

Thoracic Provocation Test

• Seated Rotation BIL

*Rule out thoracic spine*

Ligamentous Instability

• Sharp-Purser
• Alar ligament

*Exclude ligament structure trauma*

Cervical active range of motion assessment

• Flexion (45 degrees)
• Extension (75 degrees)
• Lateral flexion (40 degrees)
• Rotation (85 degrees)

* ROM with damaged documentation. Patients may be unable to rotate the head beyond the midline to the affected side. *

Shoulder Active Range of Motion

• Flexion
• Extension
• Abduction
• Adduction
• Internal rotation
• External rotation

*Watch for any serious abnormalities in movement BIL assists in ruling out shoulder*

Palpation

Sitting:

• Upper trapezius
• First rib
• Anterior/posterior rib cage

Supine:

• Sternocleidomastoid (STM)
• C7-C1 cervical paravertebral muscles
• Nuchal line and suboccipital muscles
• Mastoid process
• C1 transverse processes
• C2-C7 spinous processes
• C2/3 through C6-7 articular pillars

*Be sure to note any tenderness due to trigger points and/or abnormal masses found in surrounding tissue. Note local spasms and/or tight musculature. Affected musculature (especially the SCM) will be prominent on palpation in patients. *

Joint Mobility

Sitting:

• 1st rib (hypo, normal, hyper)

Supine:

• OA (flexion: 10 degrees extension: 25 degrees lateral bending BIL: 5 degrees)
• AA (flexion: 8 degrees, extension: 10 degrees, rotation: 45 degrees)
• Joint column BIL (Hypo Normal Hyper)

Prone:

• Passive intervertebral range of motion (bilateral and unilateral)

*Assess available motion within the joint, noting areas of restricted motion and/or pain*

Muscle Length

• Suboccipital muscles
• Trapezius
• Levator scapulae
• Scalenes
• Sternocleidomastoid

*Assess muscle imbalances for resistance to passive movement, sense of end and pain. The SCM involved will be very resistant to passive length evaluation. *

Diagnostic Testing

Tests are not required to diagnose torticollis in adults, but they can be used to assess the integrity of associated structures. Such diagnostic tests include X-rays, CT scans, and MRIs. Laboratory studies may be useful if infection is suspected as the cause of torticollis. [1]

*If at any time during the evaluation a finding indicates the need for consultation with a specialist (ophthalmologist, orthopedist, neurologist). *

Medical Management

Pharmacologic

Oral Medications

Prescription drugs offer only modest symptomatic benefit in treating dystonia. The main purpose of oral medication is to avoid exposure to the causative agent. In the early stages of cervical dystonia, drugs used at low doses (such as the benzodiazepines baclofen or anticholinergics) may it works. Anticholinergics have been reported to be more effective than those receiving benzodiazepines or baclofen. Side effects may include dry mouth, cognitive impairment, double vision, drowsiness, glaucoma, or urinary retention. [1]

Intrathecal Baclofen (ITB)

This intervention is most commonly used to treat hyperosmolarity in various clinical populations and has proven successful. ITB has been shown to be effective in the treatment of generalized dystonia, especially when the catheter is placed above the T4 level. of 77 In one trial receiving an ITB pump, participants had significantly lower dystonia scores at 3, 6, 12 and 24 months – according to the Barry-Albright Dystonia Scale. Eighty-six percent of these subjects reported improved quality of life and easier access to care. [1]

Botulinum Toxin

The introduction of chemical denervation with botulinum toxin has fundamentally changed the prognosis of patients with idiopathic cervical dystonia. [6] It is the most commonly used pharmacotherapy for the treatment of hyperactive and dystonic neck musculature. Botulinum toxin A provides graded reversible denervation Modulates neuromuscular junctions by preventing acetylcholine release from presynaptic axons at motor endplates. [1] This therapy is now the first-line treatment for cervical dystonia, but its true efficacy remains unknown. [11] However, botulinum toxin had the highest proportion of benefits patient in the shortest possible time. The most important aspect to consider when administering Botox is identifying the site of pain and the muscles causing abnormal posture. SCM trapezius chuck and levator scapula are most commonly injected website. [6] The benefits of Botox are usually visible within the first week, but are rarely delayed for up to eight weeks. The effects last an average of 12 weeks, and most doctors recommend repeating the injection every 3-4 months. Drugs are often used as an adjunct Botox though no trials have attempted to demonstrate synergy. Side effects of this treatment may include pain at the injection site, difficulty swallowing, dry mouth, excessive weakness and fatigue in the injection muscle or nearby muscles. [1]

Surgical

Selective Peripheral Denervation

This surgical approach deinnervates the muscle responsible for the abnormal movement and preserves the innervation of the non-contributing muscles. Surgical outcomes in 260 subjects reported an 88% success rate, based on a 4-point scale (poor, fair, very good, or excellent), according to one study Whether there is any detectable abnormal motion. The choice of muscle to denervate should be based on examination of abnormal movements and confirmation of EMG recordings. Patients with simple rotational torticollis with mild stretching have the best postoperative outcomes. side Effects may include transient balance problems, denervated posterior cervical wound infection, and transient dysesthesia or loss of sensation with difficulty swallowing. [1]

Deep Brain Stimulation (DBS)

DBS of the pallidus interior (GPi) or subthalamic nucleus (STN) was performed in patients with refractory cervical dystonia. This procedure involves placing microelectrodes into the GPi, usually bilaterally, while recognizing the GPi and guiding Microelectrodes are placed by microstimulation. [1] DBS has been shown to sustain significant long-term improvement in symptoms and function in most dystonia patients, and similar long-term improvements appear to be achieved in patients with cervical dystonia and generalized dystonia Improve. [12] A downside of this intervention is the need for multiple visits to properly program the settings of the stimulator, but some advantages of the procedure include reversibility of the procedure, ability to adjust stimulation parameters, and continuous visits to the treatment goal. DBS has also been suggested to reduce the use of oral medications. Side effects include infection leading to fractures, battery failure and perioral tightness during DBS adjustment visits. No studies have examined the use of physical therapy as an aid to DBS surgery. [1]

Both deep brain stimulation (DBS) and selective peripheral denervation groups showed gradual improvement. There was no significant difference between the two interventions, but DBS may tend to reduce pain. [13]

Physical Therapy Management

There are few studies on the physical therapy management of torticollis in adults. Randomized controlled trials have not been conducted, studies of specific interventions (such as vibration therapy and progressive muscle relaxation) have included case studies or small studies There was no sample size for the control group. Therefore, adult patients with torticollis will follow an individualized injury-based approach. [1][6][14][15]

Zetterberg et al. conducted an ABA-style case series investigating the effect of progressive muscle relaxation isometric muscle contraction exercises on improving coordination, balance and perception, and stretching. Outcome measures were patient quality of life and pain using TWSTRS. patient The short-term benefits of the interventions provided were demonstrated and regressed when the protocol was stopped. Half of the patients returned to baseline VAS scores at the 6-month follow-up. [14]

Kanas et al. The effect of vibration stimulation on a single patient was recorded. The ability to relax muscles and normal head posture was demonstrated by direct vibration to contracted muscles for 5 seconds or 15 minutes. relax time no It has been documented, however, that patients were able to hold the relaxed position longer after the 15-minute treatment. [15]

The main injuries associated with torticollis in adults are pain from reduced range of motion and problems with postural control. Since torticollis usually involves contraction of the sternocleidomastoid and scalene muscles, these muscles perform neck flexion, lateral flexion, and rotation antagonists Strengthening can improve posture. Strengthening the contralateral sternocleidomastoid and scalene muscles and the major neck extensors can help the patient achieve correct head position. If the patient is in the contracted position for an extended period of time, with Relaxation techniques may help restore natural muscle length. Avoid this intervention if this causes increased pain or does not produce a positive outcome. Given that mobilization is a widely accepted intervention for general cervical pain, this may be an effective technique for pain Adjust in this patient population. However, because the injury-based approach focuses on the individual needs of the patient, the therapist will need to determine whether the patient is able to relax enough to benefit from mobilization. Palliative interventions may also include soft tissue Mobilization is at the discretion of the therapist.

Presentations

Adult-Onset Cervical Dystonia: Diagnosis and Management Evidence-Based Practice Items from the Texas 2014 PT7539 Orthopedic Spine Course. view presentation

Clinical Bottom Line

Research on physical therapy interventions for torticollis in adults is generally lacking given the lack of randomized controlled trials, and given the fact that physical therapists cannot address the causes of the idiopathic condition, there is no universally accepted intervention State of health. An injury-based approach that focuses on the limitations and constraints of the individual patient will yield the best results. Major components of treatment should include pain control range of motion and postural cues. If the attending therapist suspects that Wilson or Alzheimer’s disease not specified in the patient’s medical history should follow an appropriate referral protocol.