a | Col Cap Concept —Contents Treatment of Cervical Dystonia Patient Information Defining Treatment Goals Forms of Cervical Dystonia Clinical Examination General Neutolagical Clinical Examination xamination of Head Turning Examination of Head Tilting Examination of Head Bending Forwards Examination of Head Bending Backwards Assessment Assessing the Lateral shift to the Right or Left Assessing the Sagittal shift Forwards Assessing the Shoulder Elevation Assessing the Dystonic Head Position while Walking Assessing the Dystonic Head Position while Lying Down Cervical Dystonia Evaluation Scales Toronto Westem SpasmodicTorticolis Rating Scale Tsui Rating Scale Summary ReferencesTreatment of Cervical Dystonia @ introduction Botulinum toxin A is recommended as the first line treatment for adults with cervical dystonia (CD).' Cervical dystonia may be ‘simple’, involving movement disorder in one plane or ‘complex’, involving more than one plane. Combinations of lateral turning and tilting are most common.? Itis in the field of complex forms of dystonia that treatment difficulties tend to occur? This is because the 10 clinical forms of dystonic head position can occur in a number of different combinations. Careful analysis of the particular combination form of cervical dystonia as well as the general neurological clinical examination are vital in determining the appropriate target muscles and doses of botulinum toxin A. Care should also be taken to explain the complexity of the condition and its treatment with the patient.The following steps facilitate the successful Rerun kee eeu CUE} Patient information. Defining treatment goals General neurological clinical examination, Examination of the passive mobility of the head Clinical examination of the combination forms of cervical dystonia Determination of the target muscles and doses Injection documentation. With these examination steps, treat ment-related changes can be documented and a standardisation of the terminology can be achieved. Treatment goals should be set jointly with the patient,Patient Information @ Treatment of the different forms of cervical dystonia Patient or carer consent must be obtained before starting the medical treatment. To optimise treatment success, the clinical examination should consider the individual patients form of cervical dystonia when deciding on the precise treatment plan. Information on the clinical picture, efficacy, and side effects as well as implementation of the treatment is required and must be discussed with the patient. @ further points for the explanatory discussion General information about the clinical picture Explain that cervical dystonia is a clinical syndrome consisting of involuntary, persistent muscle contractions leading to repetitive, distortive movements and postures. Discuss that botulinum toxin A is recommended as the first line treatment for adults with cervical dystonia.’Treatment information (non-exhaustive list) Botulinum toxin A blocks the neurotransmitter acetylcholine, restricts nerve impulses for around 3 months and leads to suppression of the constant neck movements.* Hf aminoglycoside antibiotics or other agents interfering with neuromuscular transmission (e.g., curare-like agents), or muscle relaxants have to be taken, it should be noted that patients feceiving concomitant treatment with botulinum toxin A must be observed closely in case of potentiation of botulinum toxin effect.” Botulinum toxin A is administered locally into the muscles which may lead to weakening of the dystonic muscle? After the injection, swallowing problems, dry mouth, muscular weakness and injection site discomfort can occur?Defining Treatment Goals @ improving treatment success through differentiation of cervical dystonia type There is significant variation in the incidence of the different cervical dystonia patterns.” A good treatment outcome can usually be achieved if the pattern of cervical dystonia involves one of the 4 primary malpositions of the head (turning, tilting to the side, bending forwards and backwards). Complex cervical movement disorders require precise analysis with the aim of establishing an optimum individual treatment strategy. However, selecting the muscles to be treated and finding the individual botulinum toxin A dose can be difficult. Various measures can be used to aid diagnosis, including: ultrasound, I" imaging of the soft tissues of the neck (in particular the deep neck muscles), EMG** of the cervical muscles and photographs of the patient. It is especially important to consider which muscles in the neck and head are involved. Differentiation between the collis or caput variants of primary malposition is required, as is the identification of 2 lateral shift, a sagittal shift forwards as well as the clinical and electromyographic detection of head tremor. The more differentiated the examination of the patient, the better the anticipated treatment success.Primary goal of treatment with botulinum toxin A Improve head position due to tonus reduction of the affected muscle. Secondary goals? Reduce pain Improve appearance. Improve psychological stability Improve social contacts. Prevent of secondary complications. Key steps to achieving the treatment goals General neurological clinical examination including the assessment of active and passive head mobility. Use of diagnostic measures: ultrasound, CT imaging of the "deep neck muscles” (obliquus capitis inferior muscle, longus capitis muscle), EMG, X-ray function images, photograph of the patient to determine a neck line and head line with angle comparisons to determine the primary form (collis or caput variant). Determination of the main form of cervical dystonia: colis or caput variant. Planning the injection.Forms of Cervical Dystonia @ Muscles, dose and injection documentation There are four primary malpositions (turning, tilting to the side, bending forwards and backwards) of cervical dystonia.’ In each of these malpositions the head or neck alone can be affected. Therefore 8 dinical forms are distinguished: torticollis, laterocollis, antecollis, Fetrocollis, torticaput, laterocaput, antecaput and retrocaput. In each case of each form of malposition these variants occur at a ratio of | 1:1:3, e.g. pure torticollis : pure torticaput : both variants.? In addition, almost all the combinations of the different malpositions can occur, Most common is the combination of tilting to the side and turning.? Two combined forms yield a characteristic clinical picture: the combination of laterocollis to one side and laterocaput to the other side fesults in a lateral shift; the combination of antecollis and retrocaput results in a sagittal shift forwards. A sagittal shift backwards is also possible (combination of retrocollis and antecaput) but only occurs in very isolated cases, mostly in generalised dystonia, In order to better attain improvement, which is the objective of the treatment, the patient should be started on a low dose of botulinum toxin A. In addition, when starting treatment, only two (or in the case of complex cervical dystonia, four) muscles should be injected.Laterocollis Laterocaput Lateral shift ee aS Torticollis Torticaput Antecollis Antecaput Sagittal shift forwards Va Retrocollis Retrocaput seemForms of Cervical Dystonia @ orticollis Most frequently affected muscles involved in torticollis: Contralateral muscles CD semispinalis cervicis muscle OD Right © teft CD evator scapulae muscle OD rit OC tet D congissimus cervicis muscle OD right © tet © Rhomboideus minor muscle@® orticaput ‘Most frequently affected muscles involved in torticaput: Contralateral muscles © Meaperius pars descendens muscle © Semispinalis capitis muscle © Sternocleidomastoideus muscle © evator scapulae muscle Ipsilateral muscles © oodbliquus capitis inferior muscle © tongissimus capitis muscle © Splenius cervicis muscle © Splenius capitis muscle 0000 0000 Right Right Right Right Right Right Right Right 0000 0000 left left left Left Left Left LeftForms of Cervical Dystonia @ iaterocollis Most frequently affected muscles involved in laterocollis: Ipsilateral muscles © tevator scapulae muscle © Biatt © tet © scatenus medius muscle O eight © tet © scalenus anterior muscle OC eit © tet © Semispinalis cervicis muscle O Ristt © Left © Rhomboideus minor muscle D eit =O tet © longissimus cervicis muscle@® iaterocaput Most frequently affected muscles involved in laterocaput: Ipsilateral muscles Steinocleidomastaideus muscle Traperius pars descendens muscle Splenius cervicis muscle Semispinalis capitis muscle Obliquus capitis inferior muscle Longissimus capitis muscle Splenius capitis muscle Levator scapulae muscle 00000000 Right Right Right Right Right Right Right Right Oo Oo Oo 0 Oo Oo Oo OoForms of Cervical Dystonia @ antecollis Most frequently affected muscles involved in antecollis: Bilateral muscles © scalenus anterior muscle © scalenus medius muscle © evator scapulae muscle © > tongus colli muscle CO Bight O Right O Right O Right@® antecaput Most frequently affected muscles involved in antecaput Bilateral muscles © tongus caits muscle O riot © tet © erator scaputae muscle O cist © tetForms of Cervical Dystonia @® eetrocollis Most frequently affected muscles involved in retrocollis: Bilateral muscles © Semispinalis cervicis muscle D Right OE tet © Bhomboideus minor muscle OD right OC eet OD eight © tet © Levator scapulae muscle@ Retrocaput Most frequently affected muscles involved in retrocaput: Bilateral muscles Stemocleidomastoideus muscle Right Splenius capitis muscle Right Obliquus capitis inferior muscle Right Semispinalis capitis muscle Right Splenius cervicis muscle Right Trapezius pars descendens muscle RightForms of Cervical Dystonia @® ‘sagittal shift forwards Most frequently affected muscles involved in sagittal shift forwards: Bilateral muscles © Stemocleidomastoideus muscle O Richt © tet © Scalenus anterior muscle O Risht © teft © Scalenus medius muscle D Bion CO tet © Mraperius pars descendens muscle — () Right) tet © evator scapulae muscle D Right © teft © oObliquus capitis inferior muscle D gist =O tet CD Semispinais capitis muscle O ein CO tett © Splenius capitis muscle O Rist © tet © Splenius cervicis muscle O tion@ Lateral shift Most frequently affected muscles involved in lateral shift: Ipsilateral muscles © Saalenus anterior muscle © Scalenus medius muscle CO Levator scapulae muscle Contralateral muscles CO sternoceidomastoideus muscle CO Meperius pars descendens muscleClinical Examination @ Neurological clinical examination When diagnosing cervical dystonia, the neurological clinical examination is the basis for ruling out other neurological disorders or incipient segmental or generalised dystonia, it should include a passive examination of the head to exclude mechanical causes for a change in the head position. in the event of diagnostic uncertainties a functional X-ray examination should be performed. Examination while sitting The patient is asked to assume a relaxed sitting position on a revolving chair. During the entire examination the upper body rests against the back of the chair and the eyes are closed. On request, the patient turns his/her head to the right and left, tilts the head to the left and fight and bends it forwards and backwards. Between the changes in head position the patient should find a self-determined midway position of the head.@ Examination while walking and lying The patient walks a short distance with the eyes open, the head and neck position is assessed in all planes. Active movement examination of the head is necessary because dystonic muscles can influence the mobility of the neck or head in the various planes. This examination helps in determining the main form of dystonia and can help identify any additional dystonic patterns which appear while walking. It is also useful for post-treatment assessment, helping to determine the strategy for further treatment. The neck and head position at rest as well as the active movements of the head (turning, lateral tilting, bending forwards and backwards) are examined while lying down. The evaluation should note whether the form of dystonic head position or malposition changes. Examination of the active movement-posi of the head by the patient Examination of the active movement of the head (turning, lateral tilting, bending forwards or backwards) is necessary to assess the dystonic changes in the various head positions, as cervical dystonia can intensify or improve in certain positions. This facilitates the clinical classification of the primary head malposition of the cervical dystonia and forms the basis of the initial treatment with botulinum toxin A.Clinical Examination @ Examination of turning torticollis and torticaput (transversal plane) Various aids can support the examination. To make a distinction between torticollis and torticaput, the region in which the turning takes, place must be differentiated: in the area C3-C7 (torticollis) or in the lower head joint between C1-C2 (torticaput). To make this distinction, a marking (using a skin marker) is applied on the larynx above the incisura thyroidea superior and on the manubrium sterni above the incisura jugularis. When the head is turned, if the mark on the larynx moves laterally with regard to the one on the manubrium this indicates torticollis; if these marks remain above one another when the head is turned, torticaput is suspected. In the case of uncertainty a CT scan of the soft tissue of the neck should be performed. An assessment of the bone window between C1 and (2s sufficient. Turning of the two vertebrae with regard to each other only occurs in the case of torticaput. Examination of lateral tilting laterocollis and laterocaput (frontal plane) In the case of lateral tilting, a distinction between tilting of the head (laterocaput) and the cervical spine (laterocollis) is made. In the case of tilting of the head, only muscles that act on the skull or on the lower head joint are involved. In lateral tilting of the neck, only muscles that attach to or originate from the cervical spine are active.@ Examination of sagittal bending antecollis, antecaput and retrocollis, retrocaput (sagittal plane) To differentiate between antecollis and antecaput and retrocollis and retrocaput, observe the patient from the side. In this plane, the angle between the head and cervical spine or between the cervical spine and thoracic spine can be determined. Examination of lateral shift (frontal plane) lateral shift is the combination of laterocollis and contralateral lateracaput. The angles between the head line, neck line and thoracic spine line are compared with each other. Examination of the sagittal shift forwards (sagittal plane) The sagittal shift forwards is the result of the combination of antecollis and retrocaput. Examinations of the sagittal shift forwards are performed by observing the patient from the side. This allows the angle between the head and cervical spine or between the cervical spine and thoracic spine to be determined.Clinical Examination @ xamination of shoulder elevation When examining shoulder elevation, it should be distinguished whether there is any dystonic shoulder elevation involved or if itis due to compensation (compensating for laterocollis/caput by assuming a scoliosis position of the thoracic spine). To make this differentiation, a second examiner should stand behind the patient and balance out the shoulder position. During the evaluation, the patient stands in front of the doctor, initially with the eyes open and then closed. After closing the eyes, a lateral bending of the head and/or neck is seen, and is associated with an improvement of the shoulder elevation. The second examiner (e.g. nurse, relative) balances out the shoulder elevation behind the patient's back. The patient's eyes remain closed. The examiner standing in front of the patient assesses the shoulder elevation. Compensatory shoulder elevation is present if (with the eyes closed or after passive balancing out of the shoulder elevation by the second examiner) there is an increase in the lateral bending of the head/ neck, and the scoliosis, and thereby the shoulder elevation, is offset. Dystonic shoulder elevation occuss rarely and cannot be balanced out by the second examiner. It is also important to note whether the shoulder is pulled forwards (in addition to the shoulder elevation), as this indicates involvement of the subscapularis and/or pectoralis major. If this is the case, this is not shoulder elevation but rather shoulder adduction and rotation forwards. This requires special treatment and is not taken into account within the context of botulinum toxin Ainjection.ved lue ja ind the the Jot ent ve) the the ced the 1S jor. der ent oxin Examination aids Ruler for determining the head and neck line, the thoracic vertebrae. line or the shoulder line. Photographs of the patient from the front, behind and the side to determinethe form of dystonia. second examiner to balance out the shoulder elevation. Aids: imaging methods C1 of the soft tissue of the neck (and bone window). EMG. Ultrasound. To diagnose the form of cervical dystonia, it is important to examine the active mobility of the head. The dystonic muscles influence mobility in the various movement planes. A large ruler aids certainty when determining the neck and head line for assessing the collis or caput variant, This is placed on the neck as an imaginary neck line or on the head as a head line and both lines are assessed with regard to each other. A photograph of the patient from the front, rear and side helps in checking the suspected form of dystonia. It can also include head and neck line or the assessed angle between the head and neck line.Clinical Examination GENERAL NEUROLOGICAL CLINICAL EXAMINATION This serves to clarify other neurological disorders and to rule out other focal, segmental or generalised dystonias tion of the mobility of the head @ exami This examination assesses whether there is a passive or active movement restriction of the cervical spine present. The following questions have to be clatified: Is there restricted movement of the head in the case of active/passive movement of the head on turning, tilting sideways and bending forwards or backwards? Does the patient have a “middle” head position (neutral position)? To what extent is the middle position achieved again after completing the movement? In the event of considerable movement restriction, check the extent of movement in the various directions manually. It is important that, during this examination, the patient should be relaxed. Explaining the examination of the head to the patient will help avoid defensive movements.re i of mn ® Examination of the patient in a seated position In this intial position, all 4 primary head malpositions can be examined During the examination the patient should have the eyes closed and relax the neck as far as possible. The patient should assume a relaxed seated position on a revolving chai The feet must be resting flat on the floor. | The patient should lean against the SA back of the chair. The arms should hang down at the sides.Clinical Examination GENERAL NEUROLOGICAL CLINICAL EXAMINATION @ xamination-observations Where is the head being pulled to? What form of dystonia is present? @ Aids The following examination provides information about which primary head malposition is present. Examination of head turning/tiling to the right and left, bending forwards and backwards Show the patient the positions: turning, tilting to the right and left, bending forwards and backwards. Then ask the patient to actively perform turning and tilting to the right and left as well as bending the head forwards and backwards. During the examination the patient should always have the eyes closed. ‘Ask the patient to actively bring his/her head into a subjective middle position.Turn head to the right, then back into the middle position. Turn head to the left, then back into the middle position Tilt head to the right, then back into the middle position Tilt head to the left, then back into the middle position Bend head forwards, then back into the middle position Bend head backwards, then back into the middle position, Js the head in the middle position? O wo Which head positions are restricted in the movement directions turning/tilting/bending forwards and backwards? © Turning to the right restricted CO) shift to the right © Turning to the left restricted CO Shift to the tett CO Ming to the right restricted O Shift forwards Gi Tilting to the left restricted Bending forwards restricted CO. Bending backwards restrictedClinical Examination EXAMINATION OF HEAD TURNING @ Differenti ion between Torticollis and Torticaput Tell the patient: + Please close your eyes and keep them closed throughout the examination + Keep your neck lose, do not “push in the opposite direction’. in-observations @ exami During turning of the head to the left or right, is the physiological end position reached? How much does the position of the head differ from the physiological middle position to the right or left? Is there a movement restriction during the active turning movement? © No restriction of the tuning movement © Slight restriction of the turning movement Orion O tet Orion O tett CO Considerable restriction of the turning movement@ Aids Apply ruler between the marks on the larynx and manubrium ine deviate from the vertical? Does t No deviation O Stight deviation © Considerable deviation Marking By way of a marking on the larynx above the incisura thyroidea superior and on the manubrium stemi above the incisura jugularis the following can be observed: Torticollis The larynx marking moves from the mid-line. Torticaput There is no or only a very slight deviation of the larynx marking from the mid-line.Clinical Examination EXAMINATION OF HEAD TURNING Torticollis Torticaput®@ Examination-observations Turning of the head to the right The left ear is visible, the right one is not. During slight turning to the fight a larger portion of the left ear can be seen than of the right one. Turning of the head to the left The right ear is visible, the left one is not. During slight turning to the left a larger portion of the right ear can be seen than of the left one. Torticollis right or left Deviation of the larynx marking from the mid-line. Torticaput right or left Little or no deviation of the line between the manubrium and larynx. O Not certain @® iinically Torticollis Only muscles that turn and are attached to/originate from the neck are involved, Torticaput Only muscles that turn and are attached to/originate from the lower head joint or the skull are involved.Clinical Examination EXAMINATION OF HEAD TURNING @ further diagnostic measures carried out - details of the examination methods © of the soft tissues of the neck © Ultrasound Ome © Palpation © Photo @ further diagnostic measures cannot be carried out Reasons: @ form of dystonia Torticollis ) severe = () Moderate () mild Torticaput © severe (1) moderate) mild @ Rating scale TwstRs* — ©) carried out TSUI score C) casried out @ comments: TWSIRS: Toronto Western Spasmodte Forticalis Rating Scale.EXAMINATION OF HEAD TILTING h Tell the patient: ifferentiation between Laterocollis and Laterocaput + Please clase your eyes and keep them closed throughout the examination, @ Examination-observations Is the head tilted to the right or left? CO No deviation © Hardly any deviation when tilted to the CO fight © Lett © Considerable deviation when tilted to the O Right © teft @ Aids Laterocollis Laterocaput Apply ruler to the neck - neck line, apply ruler to the head - head line. How are the head and neck line aligned with regards to each other?Clinical Examination EXAMINATION OF HEAD TILTING @ xamination-observations Is the neck line straight with regards to the head line? O Nodeviation © Slight deviation © Considerable deviation O Which side is affected? O Right tett Is the neck line oblique with regards to the head line? O No deviation © Slight deviation CO Considerable deviation Which side is affected? CO Bight O ettTell the patient: Bring your head into a (subjective) middle position. Ii your head tothe ight Tit your head to the lef. @ Aids Restricted tilting can clinically indicate the affected dystonic side Which form of cervical dystonia is present? (© laterocollis Neck and head line are on one plane © Laterocaput Neck line vertical, head line tilted to the right or left © Incases of uncertainty Look at the angle between the neck and the head lineH Clinical Examination EXAMINATION OF HEAD TILTING Laterocollis Laterocaput WH angle of the neck fine to the horizontal. WK: angle of the vertical head ine to the thoracic spine.® Further diagnostic measures carried out - details of the examination methods (CO of the soft tissues of the neck © Ultrasound O enc © Palpation O Photo @ Further diagnostic measures cannot be carried out Reasons: ® Form of dystonia Laterocollis right/left_ ©) severe =) Moderate (mild Laterocaput right/left () severe) Moderate =) mild ® ating scale TWSTRS © aarried out TSUI score) casted out @ comments:Clinical Examination EXAMINATION OF HEAD BENDING FORWARDS @ Differentiation between Antecollis and Antecaput Tell the patient: + Please close your eyes and keep them closed throughout the examination @ Examination-observations Head angled forwards in the resting position? © Noceviation O Slight deviation O Considerable deviation @ Aids Height davidee Antecollis Antecaput Look at the patient from the side. Place the ruler on the middle of the clavicule and hold it in the direction of the neck. WH: angle of the neck lin WA: angle ofthe vetca ie spine@ xamination-observations Is the external auditory canal vertically almost above the clavicle or considerably further forwards? © No deviation O Slight deviation © Considerable deviation @ Aids To determine the antecaput, a goniometer is applied to the external auditory canal so that the angle of the vertical line of the head in relation to the horizontal line can be measured. To determine the antecollis, the goniometer is applied to the middle of the clavicle so that the angle of the neck in relation to the horizontal can be determined. eight ext canal | \ eet cove Antecollis Antecaput the neck ine to the horont of the ve nd ne tothe thorack spine,Clinical Examination EXAMINATION OF HEAD BENDING FORWARDS Sor Tell the patient: MR [\,_ -8ring your head into the midale position. [Bend your head forwards, “Lit your head. + Put your arms up, @ Examination-observations Which form of cervical dystonia is present? Antecollis Angle of the neck line to the horizontal (WH) is smaller. Antecaput Angle of the vertical head line to the thoracic spine (WK) is smaller. @ Cinically Antecollis No double chin. Antecaput Double chin. If the head lifts when the arms are lifted simultaneously, this indicates antecollis.@ further diagnostic measures carried out - details of the examination methods O C Cof the soft tissues of the neck Ultrasound EMG Palpation o000 Photo @ further diagnostic measures cannot be carried out Reasons: ® form of dystonia Antecollis O severe C) Moderate Antecaput =) severe.) Moderate ® Rating scale TWSTRS O Gariied out TsUl score — () Carried out ® comments: O mild O mint Inthe case of antecaput, opening the mouth only leads to an improvement in the head position if supra- and infrahyoid muscles are involved.Clinical Examination @ Differenti ion between retrocollis and retrocaput Tell the patient: + Please close your eyes and keep them closed during the examination, @ Examination-observations Is the head position considerably beyond physiological middle position? @ Aids Retrocollis Retrocaput Look at the patient from the side. Place a ruler on the middle of the clavicle and on the external auditory canal. EXAMINATION OF HEAD BENDING BACKWARDSClinical Examination EXAMINATION OF HEAD BENDING BACKWARDS. Tell the patient: our head into + Bend your head backwards @ Examination-observations Is the external auditory canal vertically above the middle of the clavicle or considerably further back? O Nodeviation ©} Slight deviation CO Considerable deviation Which form of cervical dystonia is present? © No bending forwards /backwards © Siight bending forwards /backwards O Considerable bending forwards /backwards 46@ further diagnostic measures carried out - details of the examination methods © Col the soft tissues of the neck CO Ultrasound O mmc © Palpation © Photo Reasons: Form of dystonia Retrocollis = C) severe Retrocaput —_() severe Rating scale TWSTRS O Carried out TSUI score (_) Carried out Comments: O Moderate O Moderate Further diagnostic measures cannot be carried out O mile O mitsAssessment ASSESSING THE LATERAL SHIFT TO THE RIGHT OR LEFT ®@ Differentiation of the shift position a Tell the patient: @ xamination-observations Look at the patient from the front and from behind. Where is the vertical neck line? CO No deviation CD Slight deviation O Considerable deviation Where is the vertical head line? © No deviation O Slight deviation O Considerable deviation O Not certain: look at the patient from the front and from behind {as well from the side @® Clinically Lateral shift indicates laterocollis and contralateral laterocaput.@ aids Apply the ruler to the neck and the head and determine the neck and head line, i angle of the neckline to the honeonta af the vertical head tine to th racic spineAssessment ASSESSING THE LATERAL SHIFT TO THE RIGHT OR LEFT @ Examination-observations Look at the patient from the front and from behind. Where is the neck line? © No deviation © Siight deviation O Considerable deviation Where is the head line? O No deviation CO Slight deviation © Considerable deviation CO Not Certain@ Further diagnostic measures carried out - details of the examination methods CT of the soft tissues of the neck oO Ultrasound EMG Palpation Photo ooo ® further diagnostic measures cannot be carried out Reasons: ® Form of dystonia Shift to the right/left () severe (© Moderate O mia ® Rating scale TWSTRS O aarried out TSUI score) canied out Comments:Assessment ASSESSING THE SAGITTAL SHIFT FORWARDS @ Differentiation of the shift position Tell the patient: @ Examination-observations with the ruler Sagittal shift forwardsApply the ruler to the neck and to the head and determine a neck and head line. Measurement of the neck angle (WH) and head angle (Wk). @ Examination-observations Is the head shifted forwards (WH and WK become smaller)? © No deviation CO Siight deviation CO Considerable deviationAssessment ASSESSING THE SAGITTAL SHIFT FORWARDS @ further diagnostic measures carried out - details of the examination methods Tf the soft tissues of the neck Ultrasound EMG Palpation 00000 Photo @ further diagnostic measures cannot be carried out Reasons: @® form of dystonia Shift forwards O OC Moderate © mild @ Rating scale TwsTRS © aarried out TSUI score () Carried out @ comments:ASSESSING THE SHOULDER ELEVATION @ ‘shoulder elevation The examination is carried out by two people. The examiner stands in front of the standing patient, the second person behind the patient. Tell the patien + Please close » them closed during the exanrination.Assessment ASSESSING THE SHOULDER ELEVATION ASS @ Examination-observations Can the shoulder elevation be balanced out by the second person? Is scoliosis present? Is the shoulder being pulled forwards? O Not certain: look from the side @ © Certain: no shoulder elevation © Involvement of the pectoralis major and subscapularis muscles @® Form of dystonia Shoulder elevation O Severe © Moderate © mild CO Not present @ Rating scale e TWSTRS §—() Canied out TsUI score () Carried outASSESSING THE DYSTONIC HEAD POSITION WHILE WALKING ®@ Differentiation of the shift position Tell the patient: ‘alka distance in the examination room. @® xamination-observations Is there a change in the form of dystonia or does the form of dystonia basically remain the same? Is the patient only affected by a form of dystonia while walking? @ Form of dystonia © New form of dystonia O Unchanged examination picture @ ating scale TWSTRS O aarried out TSUI score) carried outAssessment ASSESSING THE DYSTONIC HEAD POSITION WHILE LYING DOWN @ Differentiation of the shift po Tell the patient: Please close your eyes ond keep them closed throughout the examination. + Please keep your neck loose. Turn your neck to the right and the back to the middle + Turn your neck to the left and then back to the + Tt your neck tothe right and then back to the middle middle Tit your neck to the left and then back to @ Examination-observations Is there a change in the form of dystonia when lying down? Which new form of dystonia occurs? @ form of dystonia CO New form of dystonia © Unchanged examination picture @ Rating scale TWSTRS © cared out TSUI score (1) Cartied outCervical Dystonia Evaluation Scales TORONTO WESTERN SPASMODIC TORTICOLLIS RATING SCALE® POE rs Head rotation to right/left 0 0) Not present - Slight = 22 Mild 23-45 Moderate 46: TWSTRS encompasses 3 subscales (Severity Only the Severity subscale is displayed here. Total score (=A-F [8 doubled] max. 35) = Antecollis Not present ate: chin half to sternum ‘Mode Severe: chin on sternum Retrocollis Moderate: approx 50% of possible range Severe: head maximal dorsal Lateral shift present Present Sagittal shift Disability; Pain),\LE*é B_ Time factor (time potion forhead deviation) ME Range of motion ©) Deviation not present ©) Full movement in direction opposite to the dystonia J) Occasional (< 25%) submaximal 1 Past midline against dystonia 2 Occasional (< 25%) maximal or (25-50%) submaximal |} Midline Intermittent (25-50%) maximal or frequent (50-75%) submaximal Frequent (50-75%) maximal or br Constant head deviation (> 75%) maxi Effect of sensory trick (0) Complete elimination of cD 4) Partial 2) Slight or no change D shoulder elevation ance ©) Absent 7) Mild (< 1/3 possible range, intermittent or constant) 2) Moderate (1/3-2/3 possible range and constant, > 75% ofthe time) or severe (> 2/3 possible range and intermittent)TSUI RATING SCALE’ CW ora) Torticollis © Absent 7 mild 2 Moderate 3) severe Severity x occurrence = Score D Total score = (Ax 8) + C+D = Cervical Dystonia Evaluation Scales B Occurrence (= Score 8) 7) Intermittent 2) continuous € Shoulder elevation (= Score ©) Mild and constant or and intermittent 2 B) severe a Severity 4) mild 2) severe Occurrence 7) intermittent 2) ContinuousSummary @ important things to remember in the clinical examination and classification of cervical dystonia The examination should be carried out on a relaxed patient sitting with their eyes closed on a revolving chair. In the case of uncertainty when assessing the various planes, the chair is turned in order to clarify the main form of cervical dystonia. there are a number of indicators for individual forms of cervical dystonia Examination of turning In dystonic turning of the head, the ear is only fully visible on the side towards which the head is not being turned. Clinical differentiation between torticollis and torticaput By way of a marking on the larynx above the incisura thyroidea superior and on the manubrium sterni above the incisura jugularis the following can be observed: Torticollis The larynx marking moves from the midline, Torticaput There is little or no deviation of the larynx marking from the midline.®@® nical differentiation between laterocollis and laterocaput In the case of laterocollis the neck and head line do not deviate from each other. In the case of laterocaput the neck line is vertical and the head line is inclined to the right. Clinical differentiation between antecollis and antecaput In the case of antecollis no double chin is visible, whereas in antecaput a double chin is seen. An important feature of antecaput is that the external auditory canal and the clavicle are vertically in line with each other. in antecollis the external auditory canal is considerably in front of the clavicle. If the head rises when the arms are raised this tends to indicate antecollis. if the head position does not change when opening the mouth supra and/or infrahyoid muscles are not involvedSummary @ Clinical differentiation between retrocollis and retrocaput In the case of retrocollis no neck folds are seen, whereas in retrocaput neck folds can develop, Retrocaput can be differentiated with certainty when the external auditory canal and the clavicle are vertically in line with each other. In retrocollis the external auditory canal is considerably behind the clavicle. @® Clinical characteristics of lateral shift A lateral shift occurs if laterocollis is combined with a contralateral laterocaput The neck line is horizontally shifted with regards to the head line. @® Clinical characteristics of sagittal shift forwards A sagittal shift occurs if antecollis is combined with retrocaput. @ Clinical examination of shoulder elevation If the shoulder elevation can be adjusted by a second examiner there is no dystonic shoulder elevation. This often reveals the presence of laterocollis.References Hallett M, Albanese A, Dressler D, Segal KR, Simpson OM, Truong 0, Jankovic J. Evidence-based review and assessment of botulinum neurotoxin for the treatment of movement disorders. Toxicon. 2013; 67: 94-114 Reichel G. Cervical dystonia: A new phenomenological classification for botulinum toxin therapy. 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