Progressive supranuclear palsy presents with parkinsonian syndrome and dementia. The disease is dangerous, because it immobilizes the patient in a relatively short time, and additional medicine is currently helpless - there is no effective treatment for the causal treatment of progressive supranuclear palsy.

Contents:

  1. Progressive supranuclear palsy: causes
  2. Progressive supranuclear palsy: symptoms
  3. Progressive supranuclear palsy: diagnosis
  4. Progressive supranuclear palsy: treatment

Progressive supranuclear palsy(PSP for short, from the English nameprogressive supranuclear palsy ) belongs to the group of neurodegenerative diseases. This disease is quite rare: statistically, it affects 6 out of 100,000 people. Symptoms of the disease usually appear in the 6th decade of life, the average age of onset is 63 years. Men suffer from PSP more often. Another name for the disease, i.e.Steel-Richardson-Olszewski syndrome , comes from the authors of the first description of the disease.

Progressive supranuclear palsy: causes

Progressive supranuclear palsy belongs to the so-called tauopathies, which are a group of diseases in which there is an excessive accumulation of tau proteins within the nervous tissue. In the case of PSP, protein deposits are usually located in certain well-defined places, such as the mesencephalic or the black substance.

Tau proteins lead to disturbances in these places both in the functioning of neurons (they can impair the processes of transport of nerve stimuli between neurons) and in their quantity itself - ultimately, excessive death of nerve tissue cells may occur. that in Steel-Richardson-Olszewski disease the tau protein accumulates in the brain, but why this is the case is not clear to this day. Various causes of this phenomenon are considered, taking into account, inter alia, participation of free radicals. The involvement of genetic disorders was suspected as one of the possible mechanisms of the disease development.

Scientists have indeed discovered a mutation that may be associated with progressive supranuclear palsy. It turned out that in some patients it is possible to detect mutationsin the MAPT gene. However, this only applies to a small number of patients with the disease - most cases of PSP are sporadic and not related to genetic disorders.

Progressive supranuclear palsy: symptoms

Patients with progressive supranuclear palsy may have different ailments. This is due to the fact that in different patients, tau protein deposits may accumulate in slightly different locations in the brain. Symptoms of Steel-Richardson-Olszewski disease can be:

  • impairment of the ability to perform movements (associated with both slowing down of movement and gait instability, and with a significantly increased tendency to falls)
  • disturbances in the mobility of the eyeballs (initially the inability to move them vertically, later the impairment concerns moving the eyeballs and looking in all directions)
  • dysphagia (dysphagia)
  • retrocolitis (dystonic tilt of the neck towards the back)
  • eyelid apraxia (patients are not able to do so when asked to lift the eyelids and open their eyes)
  • dysarthria (speech disorders)
  • dementia disorders (slow thinking, apathy, slow decision making)
  • emotional lability (patients may experience, for example, compulsion to cry, but also bouts of laughter)
  • increase muscle tone in the form of stiffness
  • verbal perseveration

Progressive supranuclear palsy: diagnosis

Symptoms that appear in the course of Steel-Richardson-Olszewski disease may also occur in other neurological diseases (e.g. Parkinson's disease or multi-system atrophy), therefore it is important to carry out an accurate differential diagnosis. There are criteria, the fulfillment of which indicates that the patient is likely to suffer from progressive supranuclear palsy. The basic criteria in this case are:

  • patient's age over 40
  • paralysis of eye movements
  • progressive course of existing disorders

Additional PSP recognition criteria include: tendency to falls (present from the onset of the onset of the disease) and slowness of movement. Deviations suggestive of progressive supranuclear palsy may be detected by neurological examination. Patients may be subject to the so-called pull test. To do this, the neurologist walks up to the patient from behind and gently pulls the patient's arms. In the course of PSP, the presence of extremely high instability of the patient's body posture causes that even extremely delicate pulling movements lead tofall - in this case the pull test is positive. In the diagnosis of progressive supranuclear palsy, imaging tests (such as MRI of the head) may also be performed, which may show atrophy of the brainstem structures (mainly in the midbrain) and the associated dilatation ventricular system. In one of the planes assessed in the imaging studies, i.e. in the sagittal section, it is possible to notice a symptom resulting from the disappearance of elements of the midbrain - it is referred to as a penguin symptom or a hummingbird symptom.

Progressive supranuclear palsy: treatment

The causal treatment of Steel-Richardson-Olszewski syndrome is currently unknown. Patients are given medications to reduce the intensity of their symptoms. To control parkinsonian symptoms (such as stiffness and slowness of movement), for example, levodopa and amantadine are used. Mood lability problems may be reduced by administering psychotropic drugs, such as serotonin reuptake inhibitors, to patients. In order to resolve retrocollitis or eyelid apraxia, patients are given injections of botulinum toxin. In addition to pharmacological treatment, patients with PSP may be prescribed rehabilitation. Regular exercise is aimed at extending the time of patients staying in relatively good physical fitness.

Progressive supranuclear palsy: prognosis

The disease, as its name implies, is progressive - the symptoms present from the beginning of the disease intensify over time. In just a few, i.e. after 5-7 years on average, progressive supranuclear palsy leads to complete immobilization of patients. Death occurs after a time similar to immobilization and is associated with its consequences (including the need to use parenteral nutrition or an increased risk of pneumonia).

Category:

Neurology