neurologic disorders
Progressive Supranuclear Palsy (PSP)
Symptoms and Signs of PSP
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. Progressive supranuclear palsy has several different clinical types based on the predominant symptoms or signs ( Diagnosis references ): Richardson syndrome: Classic progressive supranuclear palsy with progressive supranuclear ophthalmoplegia and severe balance problems (the most common form, in ≥ 70% of cases) PSP-P: Parkinsonian type of progressive supranuclear palsy, which responds tolevodopa, resulting in transient, slight improvement PSP-PAFG: Characterized by pure akinesia with freezing of gait PSP-PGF: Characterized by progressive gait freezing PSP-PNFA (PSP-SL): Characterized by progressive nonfluent aphasia or speech or language disorders PSP-AOS: Characterized by apraxia of speech PSP-FTLD (PSP-F): Characterized by frontotemporal degeneration PSP-CBS: Characterized by corticobasal syndrome, causing profound asymmetric symptoms PSP-MN (PSP-PLS): Characterized by motoneuron symptoms related to Primary lateral sclerosis None Other possible forms include PSP-C: Characterized by cerebellar signs 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). 