Akinetic-rigid syndrome: causes, symptoms, diagnosis

The term "hypokinesia" (akinesia) can be used in a narrow and broader sense.

In the narrow sense, hypokinesia refers to extrapyramidal disorder, in which the failure of movements manifests itself in their insufficient duration, speed, amplitude, a decrease in the number of muscles participating in them and the degree of diversity of motor acts.

In a broad sense, hypokinesia means a more or less prolonged restriction of general motor activity of any other origin. Such hypokinesia is inevitably caused by many neurological disorders: monoparesis (in the legs), hemi, para- and tetraparesis, gross gait disturbances due to ataxia, apraxia, or a sharp increase in muscle tone. Hypokinesia in this sense is typical for depression, catatonia, and some psychogenic disorders of movements. Finally, its origin can also have a purely physiological character (hypokinesia, due to external environmental requirements or own motives). The neurological interpretation of hypokinetic syndrome always requires consideration of many possible causes of hypokinesia and the conduct of a syndromic differential diagnosis, which sometimes seems to be a highly difficult diagnostic task. The term "rigidity" is also not unambiguous. It suffices to recall such commonly used terminology as "extrapyramidal rigidity" (the most commonly used meaning of the word "rigidity"), "decerebrate rigidity and decortic rigidity"; the term "stiffness" (muscular tension of spinal or peripheral origin) is also translated by many domestic and foreign neurologists as rigidity. In Russian there is no generally accepted synonym for this term. Recognition of the true nature of "rigidity" refers to no less complicated tasks than clarifying the nature of hypokinesia.

The term "akinetic-rigid syndrome" is used in the narrow sense, as a synonym for the extrapyramidal phenomenon of "parkinsonism."

The initial stages of Parkinsonism, contrary to popular belief, are extremely difficult to diagnose. In some publications, unfortunately, the criteria for the diagnosis of Parkinson's syndrome are not quite accurately described.

For the diagnosis of a true parkinsonism syndrome, it is necessary to have hypokinesia plus at least one of three other symptoms: muscle rigidity, low-frequency rest tremor, or postural disorders.

[1], [2], [3], [4], [5], [6], [7], [8], [9], [10], [11]

The main causes of akinetic-rigid syndrome:

• Parkinson's disease
• Stri-nigral degeneration
• Shaya - Dreiger syndrome
• OPCA (sporadic form)
• Progressive supranuclear palsy
• The youthful form of Huntington's chorea
• Disease Wilson - Konovalov.
• Hepato-cerebral syndromes
• Parkinsonism - ALS - dementia
• Senile encephalopathy
• Normotensive hydrocephalus
• Calcification of basal ganglia
• Disease of accumulation
• Cortico-basal degeneration
• Chronic intoxications (including medicinal)
• Atrophic processes in the brain (including Alzheimer's and Pick's diseases)
• Processes that limit space
• Post-traumatic
• Postencephalitic
• Vascular
• Segawa's disease
• Creutzfeldt disease-Yakob
• Rare forms of multiple sclerosis and leukoencephalitis
• Hypoxic encephalopathy (including "ailment of the animated brain").
• Hereditary dystonia-Parkinsonism with a rapid onset.
• Disease of diffuse Levi bodies
• Spino-cerebellar degeneration
• Mitochondrial encephalomyopathy
• Neuroacanthocytosis
• Hereditary dystonia-Parkinsonism, linked to the X-chromosome.
• HIV infection
• Neurosyphilis
• Hypothyroidism
• Hypoparathyroidism
• Hereditary insufficiency of taurine
• Syringomesencephaly
• The syndrome of hemiparkinsonism is hemiatrophy.

Since approximately 80% of all cases of parkinsonism syndrome refer to idiopathic parkinsonism, that is to Parkinson's disease, knowledge of modern criteria for the diagnosis of Parkinson's disease already provides a correct recognition of the etiology of most cases of parkinsonism. The generally accepted criteria for the diagnosis of Parkinson's disease suggest a three-stage diagnosis:

• The first stage is the recognition of Parkinson's syndrome,
• The second stage is the search for symptoms that exclude Parkinson's disease and
• The third stage is the identification of symptoms that confirm Parkinson's disease.

Diagnostic criteria for Parkinson's disease (by: Hughes et al., 1992)

Criteria for excluding Parkinson's disease:

• Anamnestic indications for repeated strokes with a step-like progression of parkinsonian symptoms, repeated craniocerebral trauma or significant encephalitis.
• Oculogic crises.
• Treatment with antipsychotics before the debut of the disease.
• Prolonged remission.
• Strictly unilateral manifestations for more than 3 years.
• Supranuclear paralysis of the eye.
• Cerebellar signs.
• Early appearance of symptoms of pronounced autonomic failure.
• Early appearance of severe dementia.
• Symptom of Babinsky.
• Presence of a cerebral tumor or open (communicating) hydrocephalus.
• Negative response to large doses of L-DOPA (if malabsorption is excluded).
• Intoxication of MIPTP (methyl-phenyl-tetrahydro-pyridine).

Confirming criteria for Parkinson's disease. There are three criteria or more for a reliable diagnosis of Parkinson's disease:

• Unilateral onset of disease manifestations.
• Presence of a tremor of rest.
• Constant asymmetry with more pronounced symptoms on the side of the body with which the disease began.
• A good reaction (70-100%) on L-DOPA.
• Progressing course of the disease.
• The presence of pronounced dyskinesia, induced by L-DOPA.
• Response to L-DOPA for 5 years or more.
• Prolonged course of the disease (10 years or more).

Negative criteria are important because they remind the doctor about the need to exclude Parkinson's disease if the patient, for example, does not respond to treatment with L-Dopa, is characterized by early dementia development or early postural disorders and falls, etc.

With progressing supranuclear (supranuclear) paralysis in the initial stage of the disease, only the movements of the eyeballs are downward (and only then - their movements up and to the sides). The phenomenon of "eyes and the head of a doll" is formed (violation of arbitrary vzor movements with preservation of reflexes). Dystonic rigidity of the neck and upper part of the trunk with a characteristic extensor position of the head is revealed. Very typical is general moderate hypokinesia; pseudobulbar syndrome; dysbasia with spontaneous falls; violations of cognitive functions. There are pyramidal and cerebellar symptoms. Dofasoderzhashchie drugs are not effective.

Due to the practical significance of the diagnosis of vascular parkinsonism (often its overdiagnosis is observed) we mention the principles of its diagnosis.

A necessary condition for the diagnosis of vascular parkinsonism is the presence of cerebral vascular disease (hypertension, arteriosclerosis, vasculitis), confirmed by CT or MRI data (multiple lacunar infarctions, rarely a single contralateral infarction, Binswanger's disease, expansion of perivascular spaces, amyloid angiopathy, etc.). Characteristic subacute or acute onset of the disease (but can be gradual), fluctuating course, the presence of symptoms of discirculatory encephalopathy (pyramidal, pseudobulbar, cerebellar, sensitive, mental disorders), the prevalence of Parkinsonism symptoms on the lower half of the body, rough dysbasia, lack of tremor, non-response to dopasoderzhashchie preparations (as a rule).

Binswanger's disease is often accompanied by symptoms that only resemble parkinsonism, but it is possible to develop a true Parkinson's syndrome.

The main syndromes of motor disorders, reminiscent of parkinsonism ("pseudoparkinsonism"), sometimes requiring a differential diagnosis with true parkinsonism

In clinical neurology, a syndromic diagnosis precedes the topical and etiologic diagnosis. The recognition of Parkinson's syndrome assumes first of all a differential diagnosis with pseudoparkinsonism. Pseudoparkinsonism is a conditional and collective term that in this context unites a group of neurological and psychopathological syndromes that are not related to parkinsonism, but sometimes resemble it by one or other clinical manifestations. Such clinical manifestations may be psychomotor retardation, muscle tension (stiffness), apraxia walking and some other neurological syndromes.

The diagnosis of "pseudoparkinsonism" is thus purely operational, intermediate, didactic and is set if the observed clinical picture does not meet the criteria for the syndromic diagnosis of true parkinsonism. The final syndromal diagnosis requires the indication of a specific form of pseudoparkinsonism:

Syndromes of psychomotor retardation:

1. Depressive stupor.
2. Catatonic stupor.
3. Organic stupor.
4. Hypersomnia.
5. Hypothyroidism.
6. Hypo-and hyperthyroidism.
7. Psychogenic parkinsonism.

Muscle Stress Syndromes (Stiffness):

1. Syndrome of "armadillo" Isaacs.
2. The syndrome of a rigid person.
3. Progressive encephalomyelitis with rigidity (spinal interneuronitis).
4. Syndrome Schwarz-Jampeel.
5. Syndromes of muscular tension in the defeat of peripheral nerves.
6. Dystonia.

Apraxia walking:

1. Normotensive hydrocephalus.
2. Progressive supranuclear palsy.
3. Other degenerative-atrophic processes in the brain.
4. Processes that limit space (tumors, subdural hematoma).
5. Post-traumatic encephalopathy.
6. Lacunar state.
7. Isolated apraxia walking syndrome.

Syndromes of mixed nature:

1. Syndrome of the locked person.
2. Syndrome of akinetic mutism.
3. Syndrome of a rigid spine.
4. Syndrome of painful leg and moving fingers.
5. Malignant neuroleptic syndrome.
6. Syndrome of malignant hyperthermia.
7. Idiopathic senile dysbasia.