ACNR • VOLUME 3 NUMBER 2 MAY/JUNE 2003 27

Approach to the patient with a movement disorder

The characteristic feature of all movement disorders isan abnormality of the form and velocity of move-

ments of the body. The use of the term “movement disor-der” in neurology has become synonymous with basalganglia disease and extrapyramidal features. Although itis true that many movement disorders arise from pathol-ogy within the basal ganglia, disorders such as myoclonusmay also arise from other structures. Abnormalities ofmovement may be the only manifestation of a disease (forexample, essential tremor) or may be part of a more wide-spread neurological disorder (for example, Creutzfeldt-Jakob disease). Basal ganglia disease is also commonlyassociated with neuropsychiatric symptoms and thesemay have a greater impact upon the patient and theirfamily than the movement disorder itself. As in all aspectsof neurology, it is important not to divorce the disorder ofmovement from general medical problems, since thesemay be directly or indirectly related (for example, choreain systemic lupus erythematosus; myoclonic ataxia incoeliac disease; drug-induced parkinsonism caused bymetoclopramide used to treat a hiatus hernia).

Classification & definitionsThe key to success in diagnosing and managing thesepatients is to establish the phenomenology of their move-ment disorder. Although the broad division of patientsinto those who move too much (hyperkinetic disorder) ormove too little (hypokinetic, or akinetic-rigid disorder) issimple enough, to the inexperienced physician, differenti-ating jerky dystonia from tremor, or tics from chorea, forexample, may not always be straightforward. There mayalso be a mixed movement disorder present, such asmyoclonic dystonia, for instance. Definitions of com-monly encountered movement disorders are listed inTable 1. Athetosis (a writhing, sinuous distal limb move-ment) is a term gradually falling out of use, as thesemovements are more economically classified as dystonicor choreo-dystonic.

Historical features and examination (Table 2)When approaching the patient with a movement disor-der, the value of a careful history and examination cannever be under-stated, even if the diagnosis may seemobvious from the moment the patient first walks in to theconsultation room. A videotape recording of the move-ment disorder may be helpful, particularly in the case of a“compound” or “mixed” problem. It is not uncommonfor the rest of the neurological examination to be normalin patients reporting a movement disorder; in otherwords, “what you see is what you get”. If no problem isapparent, consider whether the complaint is highly actionspecific (for example a task-specific dystonia) and maytherefore have been overlooked on the routine examina-tion (any excuse to get a golf club out in clinic, or even aviolin!). Failing this, asking the patient and their family torecord a home video-segment when the problem occursmay be very revealing.

Always consider drugs, both past and present, as apotential cause for the movement disorder. Tardive dysk-inesias (commonly stereotypic movements, often orofa-cial in distribution, although a broad spectrum of tardivedrug-induced movement disorders, from tics tomyoclonus, has been described) may develop after rela-tively short exposure to an offending dopamine receptorblocking agent (DRBA) but persist for many years. A fulllist of medications previously taken by the patient should

be obtained from the GP, if necessary. Approximately 80%of drug-induced parkinsonism will resolve within eightweeks of discontinuing a DRBA, although recovery up to18 months has been reported. If causality is suspected,always check with the hospital drug information service.For example, while DRBAs are well know to cause parkin-sonism, a link with agents like amiodarone and cinnar-izine, is less widely recognised.

Analysis of the following characteristics (adapted fromKishore and Calne 1997) may assist the diagnosis:

1. Specific distribution: for example, restless legs syn-drome (RLS, although this is now known as restlesslimb syndrome since symptoms may also be reportedin the upper limbs as well!) and painful legs and mov-ing toes (PLMT). Parkinson’s disease is typicallyasymmetric in onset.

2. Specific actions: for example task - specific tremor anddystonia (don’t forget to ask the patient to write orpick up a cup of water if history suggests this might behelpful).

3. Speed: for example-

slow intermediate fast

parkinsonism, chorea, tremor myoclonus,dystonia & myoclonic

dystonic tics tics

4. Rhythm: continuous – for example, tremor, PLMT; orintermittent, for example, asterixis (“negativemyoclonus”).

5. Relation to posture: for example, orthostatic tremor(presents as unsteadiness when standing still butimproved when walking).

6. Relation to sleep: few movement disorders persist dur-ing sleep; examples include palatal tremor and seg-mental myoclonus.

David J Burn is theeditor of our conferencenews section andConsultant and SeniorLecturer in Neurologyat the RegionalNeurosciences Centre,Newcastle upon Tyne.He qualified fromOxford University andNewcastle upon TyneMedical School in 1985.His MD was in thefunctional imaging ofparkinsonism. He runsMovement Disordersclinics in Newcastleupon Tyne andSunderland. Researchinterests includeprogressivesupranuclear palsy anddementia with Lewybodies. He is alsoinvolved in severaldrugs studies forParkinson's Disease.

SectionManagement Topic

Table 1 Definition of Commonly Encountered Movement Disorders

Movement Definition

parkinsonism a clinical syndrome with bradykinesia as the defining feature, almost always accompanied by rigidity, and often by tremor

dyskinesia may be applied to any involuntary movement (although often used to refer to drug-induced choreas and dystonias)

tremor a rhythmical, involuntary oscillatory movement of a body part; may be qualified by addition of a descriptive term (e.g. resting, postural)

chorea a quick, irregular, semi-purposive and predomi-nantly distal involuntary movement (patient may look “fidgety”)

dystonia an abnormal movement characterised bysustained muscle contractions, frequentlycausing twisting and repetitive movements or abnormal postures

ballism a proximal, high amplitude movement, oftenviolent and flinging in nature; usually unilateralin nature and often resolves through a choreic phase

tic an abrupt, jerky non-rhythmic movement (motor tic) or sound (vocal tic) that istemporarily suppressible by will power; tics may be simple or complex

stereotypy purposeless voluntary movements carried out ina uniform repetitive fashion at the expense of other activity (e.g. hand wringing, clapping,mouthing)

28 ACNR • VOLUME 3 NUMBER 2 MAY/JUNE 2003

7. Relation to voluntary movement: for example –action tremor and action dystonia.

8. Associated sensory symptoms: PLMT, RLS and phan-tom dyskinesias; tics may be associated with an vaguediscomfort or unusual sensation in the prodromebefore the movement.

9. Suppressibility: volitional in tics (but associated withincreasing unease and rebound worsening of ticsupon release), by sensory tricks in dystonia (includingthe “geste antagoniste”) and by activity in rest tremor.

10. Aggravating or precipitating factors: stress and anxi-ety are of no discriminating value in that they worsenall movement disorders; myoclonus may be worsenedby specific stimuli e.g. sudden, loud noise; heavy car-bohydrate-containing meals and fatigue may precipi-tate paroxysmal non-kinesogenic dystonia; rapidmovement triggering paroxysmal kinesogenic dyski-nesia.

11. Ameliorating factors: alcohol may relieve essentialtremor and myoclonic-dystonia, sometimes quitedramatically; walking backwards or running mayimprove a dystonic gait, leading the unwary to suspecta non-organic cause.

Ancillary studiesAn increasing range of blood and cerebrospinal fluidanalyses, genetic tests, electrophysiological, structural andfunctional imaging studies exist to supplement clinicalacumen. Occasionally, tissue biopsy (including skin, mus-cle, small bowel, bone marrow aspirate) may even be nec-essary. These will be dealt with more fully by other arti-cles in this management series. Suffice it to say that estab-lishing the correct phenomenology of the movement dis-order is essential as to which ‘line’ of more complex inves-tigations is initiated.

There should, however, be a low threshold to under-taking serum caeruloplasmin estimation, since Wilson’sdisease may present with tremors, dystonia or parkinson-ism and is eminently treatable. At a cut-off of 0.2g/l,serum caeruloplasmin is a cheap and simple test,although not very sensitive as 5-20% of homozygous car-riers will have normal results. Thus, while an abnormalresult should prompt further screening (ophthalmologi-cal assessment and urinary copper excretion minimum) anormal serum caeruloplasmin level does not fully excludeWilson’s disease.

Management considerations

Some key general points I try to remember in clinic:

● Treat disability or poor quality of life, not recordedimpairments

● Remove potentially exacerbating/causative drugswhenever possible

● Always consider underlying depression when thereseems to be a marked mis-match between impairmentand reported disability

● Patients don’t always volunteer neuropsychiatric fea-tures like visual hallucinations; ask!

● Members of the multidisciplinary team generally pre-fer an early referral

● Never forget the need for genetic counselling andimplications for other family members (Gasser 2003)

● If a psychogenic movement disorder is suspected, thepatient will be best managed by a formal admissionand a staged, multidisciplinary approach

Management Topic

Correspondance to:E.Mail.David.Burn@nuth.northy.nhs.uk

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Table 2 Historical and Examination Features to Remember

History Time course / functional disability / effect upon quality of lifePast medical history, including infections & toxin exposureMusculoskeletal symptoms (e.g. frozen shoulder with early PD)Drug history – past & previous & recreational (need to contact GP?)Alcohol consumption & responsivenessFamily history (with pedigree drawn out if necessary)Neuropsychiatric features (plus carer to inform/corroborate)Autonomic symptomsSleep problems (REM sleep behavioural disorder suggests PD, DLB or MSA)

Examination Observation during history of (involuntary) movements including excessive sighing(?atypical parkinsonism)Cognitive assessment (subcorticofrontal vs cortical problems? – MMSE ofteninsensitive to former, consider verbal fluency test, Luria, go/no-go task)Cardiovascular – lying & standing blood pressure, cool periphery (MSA?)Gait, postural reflexes (pull test) & axia toneEye movements (especially saccadic speed & latency) & blink frequencyLimb examination (include specimen of writing & observe posture)

● tremors/dystonic posturing (including postural & action)● tone – use reinforcement if in doubt● power & co-ordination● fine finger and rapid alternating movementsReflexes / plantars / primitive reflexes

Table 3 Ten Useful Diagnostic Pointers

Symptom or Sign Underlying Conditions to Consider

Tremor of onset over 50 ‘Tremor-dominant’ Parkinson’s disease (or possibly dystonic tremor) > essential tremor

Able to carry two cups of tea/pints of beer ‘Yes’: Parkinson’s disease > essential tremorbut unable to do up buttons? ‘No’: Essential tremor > Parkinson’s diseaseExcessive sighing Atypical parkinsonism (MSA or PSP)Cold, dusky blue hands MSASudden, brief dystonic or choreic movements, Paroxysmal kinesogenic dyskinesiaoften unilateral, when patient moves quicklyMale>>female with early onset motor and / Gilles de la Tourette syndromeor sensory tics (voluntarily suppressible);‘magic number’, obsessive-compulsivetendencies

Clicking sound in the ear (look in the mouth!) Essential palatal tremor (tremor of tensor velipalatine connecting with Eustachian tube)

Jerks precipitated by tapping the “snout” area Reticular reflex myoclonusof the facePersonality disorder, dysarthria, asymmetric Wilson’s diseasetremor (tongue tremor?) in late teens-early20’sInconsistent or incongruous movements, Psychogenic movement disorderwith non-anatomical sensory loss & ‘giving way’

> = more likely than; MSA = multiple system atrophy; PSP = progressive supranuclear palsy

Further ReadingGasser T, Bressman S, Dürr A et al. State of the artreview: Molecular diagnosis of inherited movementdisorders. Mov Disord 2003;18: 3-18.

Kishore A, Calne DB. Approach to the patient with amovement disorder and overview of movement disor-ders. In: Movement Disorders: Neurologic Principlesand Practice. Eds, Watts RL, Koller WC. McGrawHill (New York). 1997: Chapter 1; 3-14.

Lees AJ. Odd and unusual movement disorders. JNeurol Neurosurg Psychiatry 2002; 72 (Suppl I):i17-i21.