This document summarizes movement disorders. It describes hyperkinetic disorders which involve increased or uncontrolled movements such as tremor, chorea, and dystonia. Hypokinetic disorders involve reduced movement and include Parkinson's disease, Parkinsonian syndromes, and Huntington's disease. Specific conditions are described such as their causes, presentations, pathophysiology, and treatments. Movement disorders are evaluated based on features of the abnormal movements.
This document summarizes movement disorders. It describes hyperkinetic disorders which involve increased or uncontrolled movements such as tremor, chorea, and dystonia. Hypokinetic disorders involve reduced movement and include Parkinson's disease, Parkinsonian syndromes, and Huntington's disease. Specific conditions are described such as their causes, presentations, pathophysiology, and treatments. Movement disorders are evaluated based on features of the abnormal movements.
This document summarizes movement disorders. It describes hyperkinetic disorders which involve increased or uncontrolled movements such as tremor, chorea, and dystonia. Hypokinetic disorders involve reduced movement and include Parkinson's disease, Parkinsonian syndromes, and Huntington's disease. Specific conditions are described such as their causes, presentations, pathophysiology, and treatments. Movement disorders are evaluated based on features of the abnormal movements.
This document summarizes movement disorders. It describes hyperkinetic disorders which involve increased or uncontrolled movements such as tremor, chorea, and dystonia. Hypokinetic disorders involve reduced movement and include Parkinson's disease, Parkinsonian syndromes, and Huntington's disease. Specific conditions are described such as their causes, presentations, pathophysiology, and treatments. Movement disorders are evaluated based on features of the abnormal movements.
Location Extent of movement Pattern, rhythm, recurrence Course, speed, frequency Amplitude, force Relationship to posture, rest, activity/exertion, time of day Response to heat and cold Relationship to emotional tension and excitement Degree of movement suppression by attention or use of sensory tricks Presence or absence of movements during sleep
Hyperkinetic: Tremor Series of involuntary, relatively rhythmic, purposeless, oscillatory movements Distribution: unilateral or bilateral; distal parts of extremities Classification:
Simple vs. Compound
Rest vs. Action Postural vs. Kinetic Many ways to classify
Associated Conditions: Parkinsons disease: 2-6 Hz; resting tremor Essential tremor: most common movement disorder; high frequency; low amplitude Physiologic: 8-12 Hz Anxiety: postural tremor Cerebellar disease: low frequency; intention tremor Medications
Hyperkinetic: Chorea Involuntary, irregular, purposeless, random, nonrhythmic, spontaneous, abrupt, brief, rapid, jerky, unsustained Present at rest; increased with activity, tension, stress, selfconsciousness Distribution: Distal part of upper extremities Associated Conditions:
Hyperkinetic: Athetosis Slower, more sustained, larger amplitude than chorea Involuntary, irregular, coarse, somewhat rhythmic, writhing or squirming Flow from one part to another Exacerbated by voluntary activity of another body part Causes: Usually congenital perinatal injury to basal ganglia Overlap with chorea
Force affected parts of body into abnormal movements or postures Extremities, neck, trunk, eyelids, face, vocal cords Patterned and recur in same location Duration varies may be seconds to hours Associated Conditions:
Hyperkinetic: Myoclonus Single or repetitive, abrupt, brief, rapid, lightning like, jerky, arrhythmic, asynergic, involuntary contractions Portions of muscles, entire muscles, or groups Most commonly in extremities and trunk Symmetrically on both sides of body Irregular intervals Associated Conditions:
Hyperkinetic: Hemiballismus Dramatic neurologic syndrome of wild, flinging, incessant movements on one side of the body Rapid and forceful Disappear only with deep sleep Associated Conditions: Infarction or hemorrhage in contralateral subthalamic nucleus
Hyperkinetic: Dyskinesia All hyperkinetic movements are technically dyskinesias. Term for complex involuntary movements that do not fit into another category. Orofacial dyskinesia: involuntary facial movements; tardive dyskinesia
Bradykinesia: slow movement Masked facies Loss of arm swing Tremor: at rest; rhythmic pill roll Shuffling gait with en-bloc turns Poor balance and increased risk of falls
Pathophysiology: 1. 2. 3. 4.
degeneration of substantia nigra and presence of Lewy bodies in surviving neurons
Dopamine deficiency Increased output from Globus Pallidus interna and subthalamic nuclei Excessive inhibition of thalamus and suppression of cortical motor system
Treatment: . Pharmacologic Dopamine Replacement . Deep Brain Stimulation
Hypokinetic: Parkinsonian Diseases
Multisystem Atrophy: Cause: degeneration of basal ganglia, cerebellum, anterior horn cells, cerebral cortex, and brainstem in varying combinations Presentation: cerebellar ataxia, dementia, amyotrophy, parkinsonism, corticospinal tract dysfunction, dysautonomia, urinary dysfunction Various subtypes
Progressive Supranuclear Palsy:
Cause: degenerative changes in rostral brainstem and thalmus Presentation: impairment of downgaze, upgaze, then global gaze paresis; frontal lobe dementia; postural instability; pseudobulbar palsy; extensor axial rigidity; facial dystonia
Corticobasal Degeneration Cause: abnormalities in basal ganglia and cerebral cortex Pathology: asymmetric frontoparietal neuronal loss and gliosis
Diffuse Lewy Body Disease
Presentation: Dementia + Parkinson features + Psychosis
Hypokinetic: Wilsons Disease
Abnormal copper deposition in brain, especially basal ganglia, liver, eye and other tissues Defect in ATP7B, ATPase involved in copper transport Labs: low ceruloplasmisn Features:
Macroscopic rust-brown discoloration of globus pallidus and substantia nigra due to iron deposition Clinical Features:
Begins in fourth decade
Rigidity Involuntary movements Ataxia Dystonia Pyramidal signs Progressive dementia MRI: bilaterally symmetric low signal intensity in globus pallidus, due to iron deposition, surrounding a focus of high intensity due to gliosis
Hypokinetic: Dentarubropallidoluysian Atrophy CAG repeats Changes to dentate, globus pallidus externa, red nucleus, subthalamic nucleus Clinical manifestations:
