Senile chorea is a variant of choreic hyperkinesis, characterized by a debut in people over 60 years of age, slow progression, lack of association of the disease with infectious, cerebrovascular, tumor, etc. factors, intellectual—mnestic and mental safety of patients. Hyperkinesis may be focal in nature and affect only the perioral region. Diagnosis is carried out according to the neurological status, blood tests for the presence of acanthocytes, analysis of the level of C-reactive protein and rheumatoid factor, the results of EEG, MRI of the brain, genealogical analysis and DNA diagnostics. Therapy is symptomatic, carried out mainly with benzodiazepines and dopamine receptor blockers.
General information
Senile chorea is choreic hyperkinesis, manifested by involuntary movements similar to normal gestures and facial expressions, but being violent, discoordinated and excessively pretentious. Unlike minor chorea, Huntington’s chorea, neuroacanthocytosis, which occur at an earlier age, senile chorea debuts in the elderly, over the age of 60. For a long period, senile chorea existed in neurology as a separate nosological unit. Many neurologists have associated it with secondary atherosclerotic or cerebrovascular chorea, developing in patients with chronic cerebral ischemia or after ischemic and hemorrhagic strokes, leading to degenerative changes in the striatum. Currently, more and more authors tend to believe that senile chorea is a late manifestation of various genetic variants of chorea.
Causes
Senile chorea develops as a result of primary degenerative changes in the striatum. The late onset of the disease gives reason to assume the age-related nature of the ongoing degenerative processes. In this regard, this variant of chorea is called senile or senile. The striatum is the leading center of the so-called extrapyramidal system involved in the provision of voluntary human movements. The term “striated body” arose due to the fact that this subcortical structure has the form of alternating bands of white and gray cerebral matter. Senile chorea can also occur with the degeneration of other structural components of the extrapyramidal system directly related to the striatum.
Despite the assumption that senile chorea is a late debut of primary genetically determined forms of chorea, the family nature of the pathology is not traced in most patients. In some cases, patients note the association of the onset of chorea with previous tooth extraction or the loss of several teeth in a short period of time (the so-called “toothless chorea”).
Symptoms
The clinic of senile chorea manifests in patients who have overcome the 60-year age limit. Typically, a gradual, slowly increasing progression of pathological symptoms. Intellectual degradation with the transition to dementia, characteristic of Huntington’s chorea, is absent. The leading symptom complex is the choreic syndrome. There are involuntary movements that do not depend on the patient, due to the uncoordinated and disorderly contraction of individual muscle groups. Hyperkinesis is characterized by speed and lack of coordination. They make it much more difficult to perform arbitrary movements. Accompanied by a decrease in muscle tone.
Often senile chorea is manifested by focal hyperkinesis, capturing only the facial muscles, most often the mouth area (orofacial hyperkinesis). At the same time, involuntary smacking occurs, dilution of the corners of the mouth, protruding of the tongue. Violent movements interfere with the work of articulatory muscles, as a result, dysarthria develops — a speech disorder caused by the pathology of articulation.
In some cases, senile chorea is accompanied by generalized hyperkinesis, involving all facial muscles, musculature of the limbs and body. At the same time, there is grimacing, chaotic fussy movements of the limbs, especially pronounced in the distal parts and resembling gestures, violent squats and crossing of legs, dancing gait.
Diagnostics
In the neurological status, jerky movements of the eyeballs are determined, nystagmus is possible. Muscle strength is preserved, muscle tone is lowered, tendon reflexes from the extremities are enhanced, sensitivity is normal. The severity of hyperkinesis varies from minimal to moderate. There is moderate dysarthria, dancing gait. In the Romberg pose — shakiness, slight discoordination when performing tests.
In order to differentiate senile chorea from other diseases accompanied by choreic syndrome: encephalitis, intracerebral tumor, cerebrovascular pathology, a number of additional examinations are carried out. The absence of specific acanthocytes (stellate erythrocytes) in the clinical blood test excludes the diagnosis of neuroacanthocytosis. The biochemical blood test is within normal limits. Normal indicators of rheumatoid factor and C-reactive protein exclude the presence of minor chorea. Electroencephalography reveals the diffuse nature of changes in bioelectric activity. MRI of the brain diagnoses the presence of areas of degeneration of the cerebral substance in the striatum and subthalamic nucleus. Tomography allows to exclude tumor or post-stroke etiology of chorea.
Senile chorea is primarily differentiated from Huntington’s chorea. The latter manifests until the age of 50. It has a faster progression, accompanied by mental disorders (depression, suicidal tendencies, hallucinations), intellectual decline up to dementia. Huntington’s chorea is a genetic autosomal dominant pathology. Finally, the consultation of a geneticist helps to differentiate it and senile chorea. The absence of a characteristic inheritance based on the results of genealogical research and negative DNA analysis make it possible to exclude Huntington’s chorea.
Treatment
In relation to senile chorea, mainly symptomatic therapy is carried out. To reduce the severity of hyperkinesis, dopamine receptor blockers (trifluoperazine, reserpine, fluorophenazine, haloperidol, chlorpromazine), benzodiazepine pharmaceuticals (clonazepam, diazepam), gabaergic agents (N-nicotinoyl-gamma-aminobutyric acid, baclofen), valproic acid preparations are prescribed. The use of atypical neuroleptics (clozapine, risperidone, olanzapine) is increasingly being proposed. Some anticonvulsants (topiramate), glutamatergic receptor blockers (memantine, amantadine) may be used. In some cases, the treatment regimen includes a combination of a neuroleptic with an anticonvulsant or antiglutamatergic drug.
Forecast
Senile chorea has an unfavorable prognosis for recovery. Hyperkinesis significantly complicates work activity. However, the slow progression and absence of intellectual and mental disorders allows patients to maintain the ability to self-care.
