Progressive supranuclear palsy

Progressive supranuclear palsy
Progressive supranuclear palsy is also known as "Steele-Richardson-Olszewski syndrome. It is a rare disease that occurs around the age of 55 years, can begin even at the age of 75 years. Men are twice more affected than women.

 
Pathologically, the disease is characterized by loss of neurons in the deck, midbrain, and cerebellar nuclei of the basal ganglia first and by the presence of neurofibrilar balls in these regions.

From the biochemical point of view there is a decrease in the concentration of acid and dopamine homovanilic putamenului and the caudate nucleus. It is often confused with Parkinson's disease.

 
The clinically characterized by supranuclear ophthalmoplegic plus pseudobulbara stiffness and paralysis, bradykinesia, postural instability phenomena and prefrontal.

 
Clinical onset paresis is by vertical movements of the eyeballs joint and slowly progressing to rigidity and dementia. It meets in the early stages of the disease often unable to conjugation eyeballs watching down oculocefalice reflexes are still preserved. The neck is fixed in hyperextension, progress is slow, with frequent drops. Speech is not affected. And seizures may occur during sleep apnea. Unfortunately, death occurs in 5-6 years after symptom onset. From the neuropathological point of view, Alzheimer-type changes may occur in the cortex and even neuronal inclusions of Lewy type.

 
Most effective treatment is currently taking L-dopa, the answer is still quite limited.
Dopaminergic preparations reduce rigidity and bradykinesia, To improve speech, gait disorders and anticholinergics can be used as pseudobulbar trihexifenidil 6-15 mg / day or amitriptyline 50 to 70mg administered at night before bed.