General
- Most severe form within the spectrum of chorea
- Chorea: an involuntary movement disorder characterized by brief, sudden, spontaneous, non-stereotyped, and dance-like movements in one side of the body.
Definition
- A hyperkinetic involuntary movement disorder characterized by intermittent, sudden, violent, involuntary, flinging, or ballistic high amplitude movements involving the ipsilateral arm and leg caused by dysfunction in the central nervous system of the contralateral side.
Number
- Prevalence: 1 to 2 per 1,000,000.
Pathology
- Lesion to the
- Subthalamic nucleus (STN)
- Nucleus hypothalamicus/corpus Luys
- Damage in the basal ganglia structures involved in the inhibitory pathways. With the decreased excitatory transmission of the globus pallidus internus (GPi) and the disinhibition of the thalamus, it creates an overactivation of the corticospinal and corticobulbar tracts with random firing.
Aetiology
- Intracranial hemorrhage
- Stroke (ischemic):
- The most common cause in patients more than 65-year-old from the small perforating branches of the basilar artery
- Neoplasm
- Head trauma
- Metabolic (e.g., nonketotic hyperglycemic hyperosmolar syndrome in uncontrolled type 2 diabetics)
- Neuroinfectious: although rare, are more common in younger patients
- Tuberculomas
- Toxoplasmosis
- From complications of the human immunodeficiency virus (HIV) given its predilection to cause lesions in the basal ganglia
- Neuroinflammatory diseases
- Such as demyelinating disorders (multiple sclerosis)
- Paraneoplastic syndromes
- Vasculitis
- SLE
- Toxic: alcohol, heavy metals exposure
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