Disorders of Movement M A R T I N H A R L E Y N E U R O L O G Y

Educational Objectives Improved history taking in patients with movement disorders. Develop a systematic approach to observing and describing movement disorders. Understand the vocabulary used to describe movement disorders. Formulate differential diagnoses based on the history and examination. Begin to consider further investigations and management.

It s (almost) all in the history Time course Functional disability Past medical history, including infections and toxin exposures Drug history (current, previous, recreational) Alcohol responsiveness Family history Neuropsychiatric features Autonomic symptoms Sleep problems

It s (almost) all in the history Do specific actions provoke the movement? Do the movements occur during sleep? Are there any associated sensory symptoms? Can the movements be suppressed? Are there aggravating or alleviating factors?

Observation before formal examination What is there gait like? Any involuntary movement? Facial expression Spontaneity of movement Blink frequency BP assessment including orthostatic change Gross cognitive assessment

Movement disorders - categorisation

Movement disorders - categorisation Hypokinetic movement disorder - a partial or complete loss of voluntary muscle movement. Hyperkinetic movement disorder - an increase in muscular activity that can result in excessive abnormal movements, excessive normal movements, or a combination of both.

Movement disorders - categorisation Hypokinetic Movements Idiopathic Parkinson s Drug induced Parkinsonisim Multi-system atrophy Progressive Supernuclear Palsy Corticobasal Degeneration Dementia with Lewy bodies Hyperkinetic Movements Essential tremor Chorea Dystonia Ballism Myoclonus Tics Myokymia

Parkinsonisim Tremor at rest Bradykniesia Rigidity Flexed posture of the neck, trunk and limbs Loss of postural reflexes Freezing

Idiopathic Parkinson s Disease - Making a diagnosis To make a diagnosis of Parkinson s disease the patient must demonstrate: Bradykinesia and at least one of the following: Muscular rigidity 4-6 Hz rest tremor Postural instability not caused by primary visual, vestibular, cerebellar or proprioceptive dysfunction

Idiopathic Parkinson s Disease - Making a diagnosis The following are all features suggestive of IPD: Unilateral onset Rest tremor present Progressive disorder Persistent asymmetry affecting side of onset most Excellent response to levodopa in inital stage Severe levodopa-induced chorea Levodopa response for 5 years or more Clinical course of 10 years or more

Idiopathic Parkinson s Disease - Making a diagnosis Any of the following would suggest a diagnosis other than IPD : History of repeated head injury History of repeated strokes with stepwise progression History of definite encephalitis Oculogyric crises Neuroleptic treatment at onset of symptoms More than 1 affected relative Sustained remission Strictly unilateral features after 3 years Supranuclear gaze palsy Cerebellar signs Early severe autonomic involvement Early severe dementia with disturbances of memory, language, and praxis Babinski sign Presence of cerebral tumor or communication hydrocephalus on imaging study Negative response to large doses of levodopa in absence of malabsorption

Not Idiopathic Parkinson s?

Idiopathic Parkinson s Disease not just a motor problem

Treatment of IPD

Levodopa Induced Dyskinesia

Moderate amplitude tremor Can have significant impact on ADL Often have a long and insidious history Tends to run in families Can be eased with alcohol Propranolol is first choice for treatment Essential Tremor

Involuntary, irregular, purposeless, nonrthymic, abrupt, rapid, unsustained movements that seem to flow from one body part to another Characteristically unpredictable in timing, direction and distribution Can be partially suppressed or camouflaged into semipurposeful voluntary movements Prototypical example is Huntington s disease Chorea

Large amplitude choreic movements of the proximal parts of the limbs Flailing or flinging movements Most common cause of hemiballism is stroke Ballism

Sudden, shock-like involuntary movements caused by muscular contractions or inhibitions Can occur at rest or during activity (action myoclonus) Can be both arrhythmic or rhythmic Myoclonus

Consist of abnormal movements and abnormal sounds Vary in severity over time Usually preceded by uncomfortable feeling or sensory urge that is relieved by carrying out the movement May be simple or complex Often suppressible Tic dissorders

Unilateral facial muscle contractions Continual, rapid, brief, repetitive spasms Can evolve into sustained tonic spasms Can often be brought out when patient voluntarily and forcefully contracts the facial muscles Myokymia

Refers to twisting movements that tend to be sustained at the peak of the movement, are frequently repetitive and often progress to prolonged abnormal postures Tend to be patterned (in the same muscles) Dystonia

Dystonia Focal When a single body part is affected Segmental Involvement of 2 or more contiguous regions of the body are affected Multifocal Involves 2 or more regions, not conforming to segmental or generalized dystonia Generalized Movements of one or both of the legs, trunk and some other part of the body

Thank you Any questions? If you want to know more : Youtube - Springer Video Atlas of Movement Disorders Parkinsons.org.uk learning module for junior doctors coming January