14.7. Miscellaneous Movement Disorders
Moving Toe Syndrome
- General considerations:
- Possibly a basal ganglia disorder
- Some patients suffer a clear peripheral neuropathy or radiculopathy
- May occur in the fingers
- Clinical features:
- Continual flexion-extension of the toes
- Usually unilateral, but may be bilateral
- Sinusoidal pattern that persists in and may alter sleep
- Deep pain of a boring nature (deep muscle pain afferents) in the affected extremity
- Not dermatomal or restricted to a nerve distribution.
- Similar clinical constellation occurs in the fingers and arm
- Normal neurological examination in both those with chronic moving toe and fingers
Restless Leg Syndrome (Ekbom's Syndrome)
- General considerations:
- Family history in some patients
- Dopaminergic mechanism is possible
- Clinical features:
- Criteria for diagnosis:
- Irresistible urge to move the legs associated with paraesthesias and dysesthesias
- Motor restlessness
- Increased by rest and relieved by motor activity
- Increased symptoms in the late evening and night
- Approximately 50% of patients have arm restlessness
- Associated pain is non-dermatomal or in a peripheral nerve distribution
- Associated myoclonic jerks or sustained dystonic postures may be associated
- A large percentage of patients suffer periodic leg movements of sleep:
- Flexor contractions of one or both legs
- Dorsiflexion of the foot and flexion of the knee
- Occur at 20 second intervals in stage I or II of non-REM sleep
- Associated medical conditions with RLS:
- Degenerative spine disease
- Dopaminergic deficiency
- Deficits of iron metabolism
- Chronic obstructive lung disease
- Vitamin deficiency
- Diabetes mellitus
- Pregnancy
- Uremia
- Malabsorption syndromes
- Cancer
- Amyloid
- Rare Associations:
- Multiple sclerosis
- Atlantoaxial dislocation
- Associated medical conditions with periodic limb movement of sleep:
- Isaac's syndrome
- Motor neuron disease
- Demyelinating disease
- Spinal cord injury
- Spinal anesthesia
- Syringomyelia
Multiple Patterns of Movement Disorder in One Patient
- Parkinson's disease:
- Parkinsonism, choreoathetosis, dystonia, myoclonus
- Huntington's Disease:
- Choreoathetosis, parkinsonism, myoclonus, dystonia
- Hallervorden–Spatz (PANK-2):
- Parkinsonism, myoclonus choreoathetosis, dystonia
- Wilson's Disease:
- Ataxia choreoathetosis, parkinsonism, myoclonus, dystonia
- Neuroacanthocytosis:
- Tics, parkinsonism, dystonia, ataxia, myoclonus choreoathetosis
Hyperekplexia
- General characteristics:
- AD in most instances
- Mutations in the GLRA1 gene
- Chromosome 5q33–35:
- Encodes the alpha 1 subunit of the glycine receptor
- Clinical features:
- Onset in the neonatal period
- Abnormal startle reaction elicited by:
- Auditory and somatosensory stimuli
- Transitory stiffness during the neonatal period
- Affected neonates have died with generalized muscular stiffness
- Adult patients:
- Falling, attacks accompanied by momentary generalized muscle stiffness
- Reduced startle response occurs in PSP and Parkinson's disease
- Coffin-Lowry syndrome:
- Sound startle induced drop attacks
- May have several types of movement disorders that include cataplexy, drop attacks, prolonged tonic reaction
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