Progressive supranuclear palsy is a rare, degenerative central nervous system disorder that progressively impairs voluntary eye movements and causes bradykinesia, muscular rigidity with progressive axial dystonia, pseudobulbar palsy, and dementia. Diagnosis is clinical. Treatment focuses on relieving symptoms.
(See also Overview of Movement and Cerebellar Disorders.)
The cause of progressive supranuclear palsy is unknown.
Neurons in the basal ganglia and brain stem degenerate; neurofibrillary tangles containing an abnormally phosphorylated tau protein are also present.
Symptoms and Signs of Progressive Supranuclear Palsy
Symptoms of progressive supranuclear palsy usually begin in late middle age.
The first symptom may be
Difficulty looking up or down without moving the neck or difficulty climbing up and down stairs
Voluntary eye movements, particularly downward gaze, become difficult, but vertical reflex eye movements triggered by passive head movement (neck flexion, extension) are unaffected.
Movements are slowed, muscles become rigid, and axial dystonia develops. Patients tend to fall backward.
Dysphagia, dysarthria with emotional lability (pseudobulbar palsy), depression, and disordered sleep are common. Resting tremor may develop.
Dementia eventually occurs. Many patients become incapacitated within about 5 years and die within about 10 years (1).
Progressive supranuclear palsy has several different clinical types based on the predominant symptoms or signs (2):
PSP with Richardson syndrome (PSP-RS): Classic progressive supranuclear palsy with progressive supranuclear ophthalmoplegia and severe balance problems (the most common form, in ≥ 70% of cases)
PSP with predominant Parkinsonism (PSP-P): Parkinsonian type of progressive supranuclear palsy, which responds to levodopa, resulting in transient, slight improvement
PSP with ocular motor dysfunction (PSP-OM): Characterized by oculomotor abnormalities (eg, vertical supranuclear gaze palsy)
PSP with progressive gait freezing (PSP-PGF): Characterized by progressive gait freezing
PSP with predominant speech and language disorder (PSP-SL): Characterized by progressive nonfluent aphasia or speech or language disorders
PSP with predominant frontal presentation (PSP-F): Characterized by frontotemporal degeneration with cognitive impairment or behavioral change
PSP with predominant corticobasal syndrome (PSP-CBS): Characterized by corticobasal syndrome, causing profound asymmetric symptoms
PSP with predominant primarily lateral sclerosis (PSP-PLS): Characterized by motoneuron symptoms related to primary lateral sclerosis
PSP with predominant cerebellar ataxia (PSP-C): Characterized by cerebellar signs
PSP with predominant postural instability (PSP-PI): Characterized by postural instability
In forms other than Richardson syndrome (nonclassical forms [called atypical parkinsonism]), ophthalmoplegia may be delayed by years.
The different clinical types of progressive supranuclear palsy have different characteristics (eg, parkinsonian features, corticobasal syndrome, speech problems, frontotemporal degenerative signs, akinesia, freezing of gait).
Довідкові матеріали щодо симптомів та ознак
1. Cosseddu M, Benussi A, Gazzina S, et al: Natural history and predictors of survival in progressive supranuclear palsy. J Neurol Sci 15:382:105–107, 2017. doi: 10.1016/j.jns.2017.09.043
2. Höglinger GU, Respondek G, Stamelou M, et al: Clinical diagnosis of progressive supranuclear palsy: The Movement Disorder Society criteria. Mov Disord 32 (6):853–864, 2017. doi:10.1002/mds.26987
Diagnosis of Progressive Supranuclear Palsy
Clinical evaluation
Diagnosis of progressive supranuclear palsy is clinical (1).
MRI is usually done to exclude other disorders. In advanced cases, MRI shows a characteristic decrease in midbrain size that is best seen on midsagittal views and that causes the midbrain to be shaped like a hummingbird or emperor penguin. On axial views, the midbrain may resemble a morning glory (2).
Living Art Enterprises/SCIENCE PHOTO LIBRARY
Довідкові матеріали щодо діагностики
1.Höglinger GU, Respondek G, Stamelou M, et al: Clinical diagnosis of progressive supranuclear palsy: The Movement Disorder Society criteria. Mov Disord (32) 6,:853–864, 2017. Epub 2017 May 3. doi: 10.1002/mds.26987
2. Adachi M, Kawanami T, Ohshima H, et al: Morning glory sign: a particular MR finding in progressive supranuclear palsy. Magn Reson Med Sci 3 (3):125–132, 2004.
Treatment of Progressive Supranuclear Palsy
Supportive care
Treatment of PSP focuses on relieving symptoms; antiparkinsonian medications may be used, but they relieve symptoms much less effectively than they do in patients with Parkinson disease. Physical and occupational therapy may help improve mobility and function and reduce the risk of falls.
Because patients with PSP have a poor prognosis, they and their families are encouraged to discuss advanced cared directives early. Expected decline in PSP involves early cognitive impairment with dysphagia. Aspiration is a common cause of death.
Ключові моменти
The first symptom of classic progressive supranuclear palsy may be difficulty looking up or down without moving the neck or difficulty climbing up and down stairs.
The different clinical types of progressive supranuclear palsy have different characteristics (eg, parkinsonian features, corticobasal syndrome, speech problems, frontotemporal degenerative signs, akinesia, freezing of gait).
Diagnose based on symptoms, but do MRI to exclude other disorders.
Focus on relieving symptoms; consider levodopa and/or amantadine to relieve rigidity, and prescribe physical and occupational therapy.
