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Overview of Movement and Cerebellar Disorders

by Hector A. Gonzalez-Usigli, MD, Alberto Espay, MD

Voluntary movement requires complex interaction of the corticospinal (pyramidal) tracts, basal ganglia, and cerebellum (the center for motor coordination) to ensure smooth, purposeful movement without extraneous muscular contractions. The pyramidal tracts pass through the medullary pyramids to connect the cerebral cortex to lower motor centers of the brain stem and spinal cord. The basal ganglia (caudate nucleus, putamen, globus pallidus, subthalamic nucleus, and substantia nigra) form the extrapyramidal system. They are located deep in the forebrain and direct their output mainly rostrally through the thalamus to the cerebral cortex. Most neural lesions that cause movement disorders occur in the extrapyramidal system; thus, movement disorders are sometimes called extrapyramidal disorders.

Classification

Movement disorders are commonly classified as those with decreased or slow movement (hypokinetic disorders) and those with increased movement (hyperkinetic disorders). The classic and most common hypokinetic disorder is Parkinson disease. Hyperkinetic disorders include tremor, myoclonus, dystonia, chorea (including hemiballismus [rapid chorea] and athetosis [slow chorea]), and tics. However, this classification does not account for overlap between categories (eg, tremors that occur in Parkinson disease).

Hyperkinetic disorders

Hyperkinetic disorders can be rhythmic or nonrhythmic ( Classification of common hyperkinetic disorders. and Hyperkinetic Disorders).

Rhythmic disorders are primarily tremors—regular alternating or oscillatory movements, which can occur mainly at rest, while maintaining a position, or during attempted movement. However, in some cases, a tremor, though rhythmic, is irregular, as occurs when tremor is associated with dystonic disorders.

Nonrhythmic hyperkinetic disorders can be slow (eg, athetosis), sustained (eg, dystonias), or rapid (eg, myoclonus, chorea, tics, hemiballismus). Rapid hyperkinetic disorders may be suppressible (eg, tics) or nonsuppressible (eg, hemiballismus, chorea, myoclonus). Athetosis and chorea may occur together as choreoathetosis. Chorea is the most characteristic movement disorder in Huntington disease (see Huntington Disease). Multiple motor and phonatory tics are the defining feature of Tourette syndrome (see Tic Disorders and Tourette Syndrome in Children and Adolescents).

Classification of common hyperkinetic disorders.

Hyperkinetic Disorders

Abnormal Movement

Causes

Description

Athetosis

Huntington disease, encephalitis, hepatic encephalopathy

Drugs (eg, cocaine, amphetamines, antipsychotics)

Movements are nonrhythmic, slow, writhing, and sinuous, primarily in distal muscles; alternating postures of the proximal limbs often blend continuously to produce a flowing stream of movement.

Athetosis has features of dystonia and chorea and often occurs with chorea as choreoathetosis.

Chorea

Huntington disease, hyperthyroidism, hypoparathyroidism, paraneoplastic syndromes, SLE affecting the CNS, other autoimmune disorders, rheumatic fever, tumors or infarcts of the caudate nucleus or putamen

Pregnancy, often in women who had rheumatic fever

Senile chorea

Drugs that can cause chorea (eg, levodopa, phenytoin, cocaine, oral contraceptives)

Drugs that can cause tardive dyskinesia (eg, antipsychotics)

Movements are nonrhythmic, jerky, rapid, and nonsuppressible, primarily in distal muscles or the face.

Sometimes abnormal movements are incorporated into semipurposeful acts that mask the involuntary movements.

Chorea often occurs with athetosis as choreoathetosis.

Dystonias

Primary (idiopathic)

Degenerative or metabolic disorders (eg, Wilson disease, PKAN due to a PANK2 mutation [previously, Hallervorden-Spatz disease], various lipidoses, multiple sclerosis, cerebral palsy, stroke, brain hypoxia)

Drugs that block dopamine receptors, most often antipsychotics (eg, phenothiazines, thioxanthenes, butyrophenones) or antiemetics

Sustained muscle contractions often distort body posture or cause twisting, repetitive movements.

Hemiballismus

Lesions (most often due to stroke) in the contralateral subthalamic nucleus or in connecting afferent or efferent pathways

Movements are nonrhythmic, rapid, nonsuppressible, violent, and flinging.

Myoclonus

Very rapid and jerky, nonsuppressible, shocklike twitches occur; they may be focal, segmental, or generalized.

Tics

Primary: Tourette syndrome

Secondary: Huntington disease, neuroacanthocytosis, PKAN, infections, stroke, drugs (eg, methylphenidate, cocaine, amphetamines)

Movements are nonrhythmic, stereotypical, rapid, and repetitive; characteristically, patients have an urge to do them and feel brief relief after doing them.

Tics can be suppressed only for brief periods and with conscious effort.

Tics may be motor or phonatory; they may be simple (eg, eye blinking, growling, clearing the throat) or complex (eg, shoulder shrugging, arm swinging, shouting words or sentences, including obscenities).

Tremor

Essential tremor

Parkinson disease, Wilson disease

Certain drugs (eg, lithium, valproate, olanzapine, other typical and atypical antipsychotics—see Table: Some Causes of Secondary and Atypical Parkinsonism)

Movements are regular, mostly rhythmic, and oscillatory.

PKAN = pantothenate kinase‒associated neurodegeneration.

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Drugs Mentioned In This Article

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  • ZYPREXA
  • DILANTIN
  • CONCERTA, RITALIN
  • LITHOBID

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