Progressive supranuclear paresis of the gaze

The disease was first described in 1964 by John Steele, Clifford Richardson and Jersey Olszewski. The neurologist Richardson consulted a friend who complained of helplessness, vision problems and mild forgetfulness. Richardson observed the progression of the disease and described similar symptoms in several other patients. Olshevsky and Steele, also doctors, investigated brain pathology in deceased patients with the described symptoms.

Epidemiological studies have shown that progressive supranuclear paresis of the gaze is the most common among all atypical syndromes of parkinsonism. The incidence rate is estimated at 5.3 cases per year per 100 thousand people over 50 years of age, so the incidence of PSVP is comparable to that of amyotrophic lateral sclerosis ^[2] . The average age of the onset of the disease lies between 60 and 65 years, on average, death occurs after 5.6 years from the onset of the disease ^[3] . The disease proceeds, constantly progressing. In the last stages of the disease, patients are tied to a wheelchair or to a bed. PSPV patients die from complications, most often from aspiration pneumonia , neurogenic respiratory failure, and pulmonary embolism .

The disease refers to taupathy , the pathogenesis of the disease remains unclear. Despite the sporadic occurrence of the disease, a genetic predisposition associated with the H1-tau haplotype is possible. The relationship between the occurrence of the disease and exon 10 polymorphism in chromosome 17 taugen was proved.

The main clinical manifestations:

• progressive akinetic-rigid syndrome (parkinsonism syndrome);
• progressive supranuclear vertical paresis of the gaze;
• violation of postural control and walking, typical fall of the patient already in the early stages of the disease (in the first year of the disease)
• cognitive impairment
• dysphagia and dysarthria
• sleep disorders
• lack of effect in the treatment of levodopa.

Other signs of the disease are:
Eye symptoms.
As a rule, they occur on average during the first four years of the disease:

• vertical paresis of the gaze (restriction of gaze movement of more than 50% when looking up and a significant limitation when looking down)
• during the course of the disease: impaired gaze retention when looking to the side, violation of horizontal gaze and convergence
• violation of coordination of the “gaze-head movement”: when looking to the side, the head first turns and only then the eyeballs in the direction of the target;
• blinking reflex: “flashing light” -test - when checking the reaction of pupils to light, a blinking reflex is triggered, also when repeating the test several times.

Frontal disturbances: impaired speech flow, impaired abstract thinking, impaired control of automatic movements, pathological laughter and crying Optional symptoms:

• congealed facial expression with markedly delayed blinking and hypermotility of the frontal muscle, the inability to open the eyes on demand due to blepharospasm or apraxia of the opening of the eyelids, palilalia or palilogy, rapid micrography.
• Mental disorders: emotional lability, personality disorders.
  

Other neurological symptoms: pyramidal signs , rest tremor , chorea , dystonia of the extremities, respiratory dyskinesia, myoclonus.

At an early stage, the disease is not much different from classic Parkinson's disease - including the tendency to accentuate the akinetic-rigid syndrome on one side of the body and the resting tremor typical of Parkinson's disease. The difference between progressive supranuclear paresis of the gaze and Parkinson's disease is only a slight and short-term improvement in symptoms in response to treatment with levodopa drugs, the rapid progress of the disease and the early onset of apathy and falls.

Diagnosis is clinical. Additionally, magnetic resonance imaging , single-photon emission computed tomography , positron emission tomography are performed.

The differential diagnosis is carried out with corticobasal degeneration , fronto-temporal degeneration, Parkinson's disease , multisystem atrophy and other neurodegenerative diseases, accompanied by supranuclear paresis of the gaze; vascular conditioned supranuclear paresis of the gaze with ischemic lesions in the basal ganglia , inner capsule , midbrain ; intracranial hypertension, tumors of the midbrain, Whipple's disease.

Due to the extremely low number of randomized controlled trials, pragmatic therapy of progressive supranuclear paresis of the gaze is based on the so-called empirical level of evidence. Due to the very limited pharmacotherapeutic capabilities, early connection of physiotherapy , occupational therapy and speech therapy is recommended. Particular attention should be paid to the prevention of falls. In severe violation of swallowing, feeding through the gastrostomy is recommended. Nasogastric tubes should be used only briefly. Despite the small hopes for medical treatment, it is worth starting it with levodopa. The second choice drugs are amitriptyline and zolpidem - both drugs can improve motor symptoms for a period of weeks to several months. Coenzyme Q10 has been shown to be effective. Currently, there is no evidence of a positive effect of cholinergic, serotonergic or noradrenergic drugs in patients with PSPV. With apraxia, opening of the eyes and dystonia of the extremities have a good effect of local injections of botulinum toxin type A. The use of prismatic lenses in glasses did not show any effect.

• H. Steffen "Neuritis nervi optici": Journal Der Nervenarzt N12 2013
• Neurologie compact für Klinik und Praxis, herausgegeben von Andreas Hufschmidt 6. Auflage 2013
• Therapie und Verlauf neurologischer Erkrankungen 6. Auflage 2013 s. 997 "Progressive supranukleäre Blickparese".
• Guy Arnold: Früh- und Differentialdiagnose von Parkinson-Syndromen . Habilitationsschrift, 2001.