Movement Disorders

Movement disorders are a group of conditions characterized by alteration in normal motility, posture or tone, alone or in combination. Changes caused by motor paralysis, severe sensory loss, painful syndromes or skeletal deformities, etc. are not included in these disorders.

Though movement abnormalities may be seen with lesions of the cerebral hemispheres, cerebellum, and brainstem or in metabolic disorders, the most common site is the extrapyramidal system (basal ganglia). This is a phylogenetically older motor system and is responsible for regulation of tone, automatic movements and posture. The basal ganglia cannot produce fine voluntary movement in man, which is a function of the pyramidal system. The extrapyramidal system “sets the background for efficient functioning of the pyramidal system”.

The manifestation of an extrapyramidal lesion depends on the role of a given part within the overall organization of the system. Whereas lesions of the substantia nigra often produce typical parkinsonism (with tremor, bradykinesia and rigidity), akinetic-rigid parkinsonism may be produced by lesions of the globus pallidus. Focal lesions in the caudate can produce chorea, while lesions in the putamen may cause dystonia. The clinical findings will be contralateral to the side of the lesion.

Movement disorder manifestations are characterized as either hyperkinetic (increased movement) or hypokinetic (decreased movement). Hyperkinetic movement disorders include tremor, chorea, ballismus, athetosis, myoclonus, tics, and dyskinesias.

Parkinsonism is a hypokinetic movement disorder, with overall paucity of movement, with the exception of tremor (hyperkinesia). Dystonia is also a

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hands out in front. with the dominant picture of increased tone caused by agonist and antagonist co-contraction with rare dystonic tremor. anxiety or concomitant illness (e. (c) cerebellar tremor (slow. neck.g. They can involve any body part but typically involve the head. fatigue. patient lying supine on the examining table. asked to perform finger/nose/finger. Everyone has physiological tremor but it is not clinically evident.e. Tremor Tremor is the most common movement disorder. and patient is also ataxic). (d) other metabolic disorders (including drug-induced) (3) Kinetic (Action) – with activity i.e. (2) Postural – the body part held out against gravity i. It is defined as “an involuntary rhythmical movement about an axis. Examples include: (a) exaggerated physiological tremor as seen with anxiety. UTI. Tremor seen near the end of movement is also called “terminal” or “intention” tremor. The pattern is the same in a given patient. Tremor is classified as follows: (1) Resting – Parkinsonian tremor. caffeine. thyrotoxicosis.e. rapid coordinated movements. repetitive. movement disorders show significant or complete relief during sleep. Lesions in the cerebellar outflow tract are associated with prominent terminal tremor (as well as ataxia). The limbs are fully supported against gravity i. These movements can 2 . This type of tremor is seen in essential tremor. etc. (b) essential tremor.). As a rule.. fatigue.combination of hypokinesis and hyperkinesis. Tics These are stereotyped. etc. pneumonia.” It can involve any body part but most commonly affects the limbs or head. eyes or upper extremities. All movement disorders are worsened with stress.

There is an association between obsessivecompulsive disorder (OCD). Dystonia usually begins as a dystonic movement and further progression leads to sustained abnormal posture lasting for 30 seconds or longer – known as “dystonic posture”. Dystonia may be classified as symptomatic. Acute or chronic dystonia may be caused by neuroleptics. etc. Dystonia Dystonia is characterized by sustained muscle contractions resulting in abnormal movement or posture. In most cases. sniffing. or torticollis). with onset before age 18. Tics may be simple or complex. When the tension is “released” there are exaggerated movements and the patient feels more comfortable.be voluntarily suppressed but this causes a build-up of tension. Dystonia may involve any part of the body such as the neck (cervical dystonia.e.) lasting more than 1 year. In adults. a component of another disease such as Wilson’s disease (disorder of copper metabolism) or idiopathic. Tic disorder is not uncommon in children but typically resolves spontaneously. dystonia is typically focal (most commonly cervical) while in childhood. Idiopathic dystonia may be sporadic or inherited as an autosomal dominant or recessive disorder. The “dystonic movement” is brief. or the whole body as in dystonia musculorum deformans. grunting. i. 3 .g. intermittent. dystonia is more often generalized. hence the term “torsion dystonia”. twisting or turning in nature producing distortion of the involved part. attention-deficit hyperactivity disorder (ADHD) and Tourette’s. irregular. other vocalizations. The diagnosis of Tourette’s syndrome requires the presence of multiple motor and one or more vocal tics (e. the pathological basis of dystonic movement and dystonic posture is not known. Lesions that have been identified include the putamen or diffuse basal ganglia involvement.

There is a diurnal pattern. Writer’s cramp is the most common type of task-specific dystonia. the handwriting becoming progressively more effortful and illegible the longer the person writes.Blepharospasm is a form of cranial dystonia consisting of involuntary eye closure affecting the orbicularis oculi muscles. and golfing (the “yips” with putting). purposeless. Sometimes patients 4 . Botulinum toxin injections are useful for cranial and cervical dystonia. asymmetrical. Other activities such as typing are unaffected. Chorea From the Greek word “dance”. Dopa-Responsive Dystonia (DRD) is an uncommon form of dystonia with onset in childhood or adolescence. and improvement after sleep. Botulinum toxin produces weakness by inhibiting presynaptic release of acetylcholine at the neuromuscular junction. with some patients wheelchair bound for years until correctly diagnosed and treated. Onset of benefit is typically within 1-2 weeks following the injection. non-rhythmic movements. DRD is very sensitive to small amounts of levodopa – excellent response and long-term benefit is the rule. however injections are generally well tolerated. irregular. The rule is that any child with dystonia warrants a trial of levodopa. and benefit lasts for approximately 3 months. Other task-specific dystonias include musician’s dystonia (such as playing the piano or violin). Initially there may be symptoms of increased blinking. with dystonia typically worse in the afternoon or evening. Adverse effects may include weakness. Taskspecific dystonias are thought to be due to repeated movements resulting in abnormal sensorimotor integration. It may be mistaken for cerebral palsy. as well as focal dystonias. The hand assumes a dystonic posture with writing. chorea is characterized by involuntary. in severe cases patients are rendered functionally blind from sustained eye closure. dysphagia and dysarthria.

It is also known as “St. calcium channel blockers. (There are many more entities associated with chorea. The tone is often increased. Abnormal posturing is often associated with voluntary movements i. personality change and progressive dementia are common features. systemic lupus erythematosus. (4) Vascular/Autoimmune disease e. thyrotoxicosis. oral contraceptives. There is anticipation. There is lack of agonist and antagonist coordination. The anatomical substrate is varied – a lesion affecting the caudate. This has autosomal dominant inheritance (chromosome 4p. trinucleotide CAG repeat expansion) and is the most common genetic cause of chorea. Vitus’ dance”. but not as 5 . In addition to chorea.attempt to incorporate these movements into semi-voluntary movements (parakinesia) e. hypoglycemia. (2) Sydenham’s Chorea. writhing and “snakelike”. hypocalcemia. the movements are slower. These include Wilson’s disease.) Athetosis This means “without fixed position”. or diffuse cerebral hemisphere or subcortical dysfunction. This is seen following streptococcal infection (usually in children) and is self-limited. complex and writhing. more sustained. with future generations developing earlier and more severe disease. amphetamines. polycythemia. and it makes for an excellent Royal College Fellowship examination question. Athetosis is a relatively uncommon movement disorder. etc. The movements are purposeless. In comparison to chorea.g. (3) Metabolic/systemic conditions. (5) Drugs/medications – includes cocaine.e.g. bizarre. neuroleptics. involuntary movement of the arm is incorporated in an attempt to scratch the head. outstretched hands may show extension of the thumb and fingers but flexion at the wrist and pronation of the forearm. The differential diagnosis of chorea includes: (1) Huntington’s Disease.

the patient is often unaware of the symptoms. occurring an hour or so after levodopa ingestion.pronounced as in dystonia. The most common causes of athetosis are: (1) birth trauma or neonatal asphyxia. but this may worsen other parkinsonian features. In mild cases. Dyskinesias are seen in some cases after prolonged use of neuroleptics and may become persistent (tardive dyskinesia). and may be focal. irregular or rhythmic. The facial muscles and the upper extremities are most commonly affected. segmental or generalized. the dyskinesias improve and the parkinsonian features return. in moderate to severe cases. or. Parkinson’s disease patients who are on levodopa may develop dyskinesias.) Dyskinesias These are abnormal movements. (3) post-encephalitic. The mainstay of treatment is removal of the offending agent. (2) Wilson’s disease. (Note: this is more information than you will ever need about athetosis. in practice these movements are usually called “choreoathetoid”. As the medication wears off. 4) symptomatic. Myoclonus These are sudden brief jerks. 3) epileptic. usually choreiform in nature. 2) essential. The most common site of lesion is the putamen and/or caudate. and (5) carbon monoxide poisoning. either sporadic or inherited. with onset in childhood or early adulthood. (4) kernicterus. Epileptic myoclonus includes the myoclonic seizures one may see in juvenile myoclonic epilepsy. dopamine depleting agents (tetrabenazine or reserpine) may be tried. Reducing the amount of levodopa can improve dyskinesias. Essential myoclonus is rare. Physiologic myoclonus includes hiccups and hypnic jerks (the sudden generalized body jerk one experiences when falling asleep during a lecture). Myoclonus may be classified as: 1) physiological. 6 . These dyskinesias are typically “peak dose”. but sometimes with athetoid and dystonic movements as well.

there is a loss of tone in the outstretched hands and the wrists drop briefly before coming back up again. with the wrists cocked back and the fingers spread apart (i. instead of a positive twitch.By far the most common etiological category of myoclonus is symptomatic and the most common symptomatic cause is toxic/metabolic (including medication). Botulinum toxin injections or carbamazepine (Tegretol) are the mainstays of treatment . Symptoms usually begin around the eye (closure) before involving the lower face. Myoclonus is reported in neurodegenerative conditions such as JakobCreutzfeldt disease or advanced Alzheimer’s disease. as if stopping a bus). Brain MRI and MR angiography are important to rule out a structural lesion compressing the VIIth nerve (usually an aberrant vascular loop). 7 . Asterixis is tested by asking the patient to hold their arms out. Asterixis is commonly caused by hepatic failure and is actually a form of “negative” myoclonus.e. Hemifacial spasm is a form of focal myoclonus consisting of unilateral activation of muscles innervated by cranial nerve VII.

Over a period of days to weeks. Stiff (rigidity). These are dramatic. The movements are continuous and confluent. It is typically unilateral (hemiballismus). flinging. While life expectancy is reduced in PD. though 5-10% have onset before age 40 years. and Shaky (resting tremor) There must be 2 of the 3 cardinal features to make a diagnosis.Ballismus Means “to throw”. The diagnosis of PD is clinical. slow speed of movement (bradykinesia). PD affects 1% of the general population over age 60. involuntary appendicular movements. The mean onset age is the early 60’s. and reduced 8 . as there is no lab test to confirm it. Parkinson’s disease (PD) This disorder was first described in the medical literature by James Parkinson in 1817 in An Essay on the Shaking Palsy. The use of the term bradykinesia encompasses: slowness of movement initiation or complete lack of movement (akinesia). more prominent in proximal than distal muscles. the ballistic movements lessen and evolve into choreiform movements. The 3 cardinal features of PD are: (1) bradykinesia (slowness of movement) (2) rigidity (increased tone) (3) resting tremor An easy way to remember this is the 3 S’s: Slow (bradykinesia). The most common cause is a stroke in the subthalamic nucleus or its afferent or efferent connections. with current medical therapy the difference is only a few years compared to the general population.

Some have a festinating gait. As PD progresses. Rigidity is typically “cogwheel” rigidity – a ratchety-type feel as one moves the joint (typically tested at the wrist). and nearly all patients who have PD develop resting tremor at some point. with subsequent involvement of the opposite side. The tremor has been described as a “pill-rolling” tremor (tremor affecting the thumb and index finger). In contrast to spasticity. Resting tremor is present in about two-thirds of patients at the time of diagnosis. cane) Wheelchair or bedbound 9 . it is not used as a diagnostic criterion in PD. As a rule the side that is affected first remains the most impaired.amplitude of movement (hypokinesia). attempts to change directions. The gait in PD is slow and shuffling. though this is not always the case. PD typically begins with unilateral symptoms. In 1967. two neurologists devised a staging system for PD. Stage 1 Stage 2 Stage 3 Stage 4 Stage 5 Unilateral findings Bilateral findings (regardless of severity) Postural instability Can ambulate alone with aids (walker. or inability to lift the foot off the ground. The tremor frequency is usually 4-6 Hz. may occur particularly when a patient first stands up. with a tendency to run forwards. and the posture is flexed. though occasionally is “lead-pipe” (smooth increased tone in all directions). rigidity is independent of both velocity and direction of movement. The Hoehn and Yahr scale is still widely used and provides an overall assessment of severity and disability. armswing is reduced. Resting tremor is the most specific finding of PD. or crosses a threshold (such as passing through a doorway or getting on to an elevator). As postural instability is common in the elderly population. Gait freezing. patients have difficulty changing directions (“en bloc” turning) and may lose their balance.

dysarthria. micrographia (smaller handwriting). hypomimia (reduced facial expression). ropinirole. benztropine). fatigue.Other features of PD include hypophonia (reduced speech volume). Other medications used to treat PD include anticholinergics (trihexyphenidyl. The gold standard is levodopa. While not being diagnostic criteria. This is the most effective and best tolerated treatment. urinary urgency. amantadine. dopamine agonists (bromocriptine. and the pathological hallmark of PD is the Lewy body inclusion. constipation. Pathologically there is neuronal loss and gliosis in the SNc. and sleep disturbance. Treatment of Parkinson’s disease is symptomatic. monoamine oxidase inhibitors (selegiline. 10 . and sialorrhea (drooling). though it is thought to be an interaction between environmental influence(s) and genetic susceptibility. We do not have any treatments proven to halt or slow disease progression. and pramipexole). depression. The cause of PD is unknown. which is given in combination with a dopa-decarboxylase agent as either Sinemet (levodopa/carbidopa) or Prolopa (levodopa/benserazide). The analogy is of genetics “loading” the gun and the environmental influences “pulling the trigger. rasagiline) and catecholO-methyl-transferase (COMT) inhibitors (entacapone). COMT inhibitors must be taken with levodopa to be effective. At least 50% dopaminergic cell loss is needed before the clinical (motor) features of PD appear.” There are rare cases of autosomal recessive and autosomal dominant inherited parkinsonism. hallucinations. and levodopa use improves survival when given before onset of postural instability. several non-motor features associated with PD include dementia. The biochemical basis of PD is striatal dopamine deficiency due to reduced dopamine production from the substantia nigra pars compacta (SNc).

Corticospinal tract findings (hyperreflexia. severe dyskinesias can be very difficult to treat and may require surgical intervention (pallidotomy. though clinicians will diagnose cases as PD when there are no findings supporting another diagnosis. Multiple system atrophy (MSA) consists of autonomic dysfunction (orthostasis. As definitive diagnosis of PD cannot be confirmed until death. etc. While mild dyskinesias often go unnoticed by patients. multiple system atrophy.With time. or deep brain stimulation of the globus pallidus or subthalamic nucleus). 11 . impotence). with either a cerebellar syndrome or a parkinsonian syndrome poorly responsive to levodopa. Babinski sign) are not uncommon. Both PSP and MSA have a poor prognosis. supranuclear gaze palsy (particularly for vertical gaze). and responds poorly to levodopa. the correct clinical term is technically parkinsonism (and probable PD). patients may develop dyskinesias and response fluctuations. urinary incontinence. Less common causes include drug-induced parkinsonism (typically caused by dopamine-blocking agents). The most common (>80%) cause of parkinsonism is PD. Progressive supranuclear palsy (PSP) is an akinetic-rigid form of parkinsonism with early falls. PSP is the second most common neurodegenerative cause of parkinsonism after PD. usually from levodopa or sometimes from dopamine agonists. progressive supranuclear palsy.

It is also known as “benign” essential tremor. Treatments modifying the GABAergic system (alcohol. Prevalence studies of ET in the general population range from 0. to diagnose definite ET there must be bilateral postural tremor with or without kinetic tremor. A study reporting on a small number of autopsied ET cases noted increased concentrations of noradrenaline in the cerebellum and its connecting nuclei in ET patients (these results have not been replicated). The frequency of tremor is typically 8-12 Hz but slows with advancing age. that is visible and persistent. The amplitude of tremor increases with age. Overall ET is at least 5 times as common as PD. There are reports of mild cerebellar degenerative changes. According to the TRIG (Tremor Investigation Group) Criteria. in contrast to the resting tremor seen in Parkinson’s disease. The tremor of ET is present on positioning the arms in front (postural tremor) or during action (kinetic tremor). though is more common with advancing age. though on routine exam there is usually no pathological abnormality.4 to 4%. There is no consistent histological brain abnormality in ET.Essential Tremor (ET) This is the most common neurological cause of tremor seen in clinical practice. Occasionally voice tremor is seen in ET. Disability may also be due to psychological impairment. a family history of tremor may not be present in each case. and of five years duration or longer. The tremor of ET involves the upper limbs and/or head. involving the hands and forearms. and it is the amplitude and not the frequency that is the major source of disability. familial tremor or hereditary tremor. and 12 . It may affect any age from childhood to the elderly. primidone. It is considered a dominantly inherited condition with variable penetrance.

The most commonly used drug for symptomatic control is propranolol (Inderal). though tends to be less well tolerated.benzodiazapines) have been used for many years to treat tremor. and recent preliminary studies suggest that this system is involved in ET. Clonazepam and other benzodiazepines work in some cases. The anti-seizure medications topiramate and gabapentin may each improve tremor though are not first line therapies. Head tremor and voice tremor in general respond less well to oral medication and each may benefit from botulinum toxin injection. Reducing caffeine intake is recommended though seldom helpful. Injections for voice tremor are performed much less frequently than for head tremor. adverse effects include dysphagia and a hoarse voice (which resolve with time) Weighted utensils (such as a spoon or cup). In refractory cases. though other beta-blockers may be used. patients may be referred to a neurosurgeon for deep brain stimulation (DBS) or thalamotomy of the Vim nucleus. Primidone is equally effective. Alcohol is effective in improving tremor but is not specific for ET. 13 . button hooks and other assistive devices may improve the functional status in ET.

This journal is a publication of the American Academy of Neurology. Volume 16 (1). February 2010.) 14 . The Canadian Journal of Neurological Sciences. Vol. (You may also check other issues on Movement Disorders in the same journal – Volume 13 (1). Volume 10 (3).Suggested reading list: Movement Disorders: An Update. June 2004. 30 (suppl 1): March 2003. Movement Disorders. In: Continuum: Lifelong Learning in Neurology. February 2007.

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