Akinesia: what is it, symptoms, causes, treatment, prognosis

What is akinesia?

Term akinesia refers to the inability to perform clinically perceptible movements. The disease can manifest itself in the form of a delayed reaction, stoppage of movement, or even complete cessation of movement. Akinesia occurs when movement is not felt, either because the range of motion is small, or because the time it takes to initiate a response increases significantly. The former is often the result of severe bradykinesia, which is often mistakenly referred to as akinesia. This makes it difficult to distinguish between the two. With akinesia, there is no rapid accumulation of sufficient force to initiate movement.

Akinetic conditions are associated with numerous etiological causes, which depend on the age of the patient. In adults, akinesia can manifest on its own or as a consequence of neurodegenerative disorders. Akinesia found in the antenatal or neonatal period is associated with fetal akinesic syndrome.

Causes

The causes of akinesia can be classified according to the age group.

1. In adults, akinesia is associated with the following two reasons:

• Pure isolated akinesia (without any concomitant conditions).
• Late stages of diseases of the basal ganglia or frontal lobes:
  • Progressive supranuclear palsy (PNP or also known as Steele-Richardson-Olszewski syndrome);
  • Parkinson's disease (BP);
  • Multiple system atrophy (MSA);
  • Normal pressure hydrocephalus (NPH).

2. Fetal akinetic syndrome (arthrogryposis of the fetus or Pena-Shocker syndrome type 1) is the cause of intrauterine fetal death or, in rare cases, the birth of a live child associated with diffuse contractures throughout the body (arthrogryposis) of the fetus and, therefore, with a decrease in mobility, which further leads to intrauterine growth retardation, short umbilical cord and hypoplasia lungs.

Epidemiology

It was found that akinesia in clinically observed parkinsonism is more often associated with the motor variant. frontotemporal dementia (PD) than with frontal degeneration associated with Parkinson's disease (PD). About 30% of patients with clinically proven PVD showed signs of parkinsonism, in particular rigidity or akinesia. Acute akinesia is relatively rare and occurs in 0.3% of patients with PD secondary to infectious pathology or other stressors.

In pediatrics, akinesia is often associated with fetal akinetic syndrome. This is associated with high intrauterine mortality. However, the frequency of live births is 1 in 3000 cases, with more than 320 genes involved in the pathology. The condition is more common among Asian, European and African populations.

Pathophysiology

The pathogenesis of akinesia in adults in general appears to be associated with pallidonigral degeneration, which mainly leads to congestion. However, the specific pathology depends on the type of akinesia. In cases of pure akinesia with freezing gait without any other symptoms or a diagnosis of PD, it is most likely associated with progressive supranuclear palsy (PNP). With PNP, degeneration of subthalamic nuclei, globus pallidus and substantia nigra is observed.

In patients with Parkinson's disease, the main cause of akinesia appears to be dysfunction of the phase and tonic release of dopamine. This contrasts with tremor at rest, caused mainly by a dysfunctional tonic dopamine release phase. Another theory suggests that akinesia in patients with Parkinson's disease is associated with bilateral damage to the frontal lobe and is also accompanied by a deterioration in mental state.

Fetal Akinesia is associated with approximately 320 genetic variations that may be present in this syndrome. From a pathological point of view, pathology manifests itself at all levels, including the brain, spinal cord, motor (motor) neuron, neuromuscular node and muscles.

Diagnostics

- History and physics.

In Parkinson's disease (PD), examination of a patient with akinesia reveals stiffness in the middle of movement, festation of both gait and speech, and repetitive movements of the upper limbs. This hardening is usually associated with increased symptoms when passing through narrow spaces such as doorways. The condition usually does not respond to treatment with antiparkinsonian drugs.

In akinetic patients secondary to PNP, the rigidity is mainly axial rather than appendicular. The earliest manifestation is an intermittent fall due to severe rigidity, but may also be present and atypical manifestations such as nonspecific dizziness, general motor deceleration and change personality. Slowing down of vertical saccades is the most frequent manifestation of eye movement, along with difficulty looking down.

In the prenatal period, a patient carrying a fetus with possible fetal akinesia may experience a decrease in fetal mobility. At later stages of pregnancy, the patient develops polyhydramnios, as evidenced by an abnormally increased height of the fundus of the uterus.

When a live child is born with fetal akinesia syndrome, the following sequence is often observed due to lack of fetal movement:

• fetal joint contractures;
• pulmonary hypoplasia;
• polyhydramnios;
• micrognathia.

- Analyzes and visualization.

The definition of akinesia in a patient is mainly clinical and is characterized by a delay or inability to respond or execute a motor command. Reaction time analysis is used to assess the degree of akinesia. Usually research shows a delay in simple reaction time, but normal choice reaction time.

Neuroimaging studies used to assess akinesia include:

• magnetic resonance imaging (MRI);
• functional MRI;
• single photon emission computed tomography;
• positron emission tomography.

Treatment

Treatment of akinesia in adults is reduced to monitoring the development of the underlying disease. Treatment for akinesia is symptomatic rather than curative.

• Drug therapy: in patients with Parkinson's disease (PD), it is mainly aimed at altering / supplementing the impaired release of dopamine.
• Surgical Therapy: The most well-studied surgical procedure contributing to the treatment of akinesia is deep brain stimulation commonly used to improve dopamine secretion and improve symptoms of tremor, akinesia, and rigidity.
• Other procedures performed in severe cases include thalamotomy, pallidotomy, neural implants, and electrical stimulation.

Treatment for fetal akinesia is supportive mainly because of the bleak prognosis at live birth. Initially, resuscitation measures are used to combat lung hypoplasia. First, such drugs, like sildenafil and iloprost, to control high pressure in the lungs. Antimicrobial drugs may be required. These patients often require long-term nutrition due to bowel malrotation, short bowel syndrome, and dysphagia. Central nervous system abnormalities such as epilepsy and endocrine disorders such as hypothyroidismneed treatment with anticonvulsants and thyroid hormone replacement therapy.

Forecast

Adult akinesias do not have curative therapy, but symptomatic treatment can significantly alter the damage to the quality of life of these patients. Although mild akinesia cannot directly lead to death, severe akinetic crises can cause dysphagia, autonomic dysfunction, and aspiration, which can be fatal.

Fetal akinesia syndrome has been found to be fatal in the womb, 30% of fetuses are stillborn. The final prognosis depends on the underlying cause. Most live births usually die within the first month of life. This may serve as a basis for proposing late termination of pregnancy in countries where it is permitted.

Complications

Fetal akinesia is often fatal within the first month of life. This is due to the myriad complications associated with this condition.

• Pulmonary complications: pulmonary hypoplasia, pulmonary hypertension, aspiration pneumonia.
• Dysfunction of the oropharynx: This leads to aspiration and subsequent pneumonia.
• Neurological complications: epilepsy and hypothalamic dysfunction.
• Gastrointestinal complications: short bowel syndrome and bowel malrotation.
• Endocrine dysfunction: hypothyroidism as a result of hypothalamic dysfunction.

Akinesia in adults can often manifest as an emergency, known as an akinetic crisis. Parkinsonian hyperpyrexia, neuroleptic malignant syndrome, acute akinesia in parkinsonism are other terms used to describe these akinetic crises. The spectrum of this variant can vary from complete akinesia with dysphagia, hyperthermia, dysautonomy, increases in muscle enzymes and changes in mental status to the point of being unable or difficult to start movement.