Progressive Supranuclear Palsy

Synonyms:

a.k.a. PSP, a.k.a. -Richardson-Olszewski syndrome

Diagnosis:

Clinical:

  • Symmetric parkinsonism, vertical gaze palsy, eyelid opening apraxia, axial rigidity (axial dystonia), retrocollis, dementia (executive dysfunction, impaired abstraction & planning, relatively preserved memory), pseudobulbar palsy, pseudobulbar affect,

Clinically two syndromes:

  • Richardson syndrome: postural instability with falls, supranuclear vertical gaze palsy, cognitive deficits/dementia.
  • PSP-Parkinsonism: unilateral tremor, transient levodopa response.

Pathology:

  • Definite diagnosis possible at autopsy
  • Basal ganglia, diencephalon, brain stem, prefrontal cortex are affected
  • Globose neurofibrillary tangle, neuronal loss, gliosis
  • Tau positive (4 repeat Tau).

Findings on investigations:

MRI:

  • DWI MRI, ADC: increased regional ADC, rADC in putamen. [312]
  • Atropy in midbrain tegmentum, pons, striatum & frontal cortex

Treatment:

Rigidity & hypokinesia:

  • Dopaminergic therapy

Speech, gait & pathologic crying/laughing:

  •  Amitriptyline
  • or benzotropine orally

Dysphagia:

  • Consider Methysergide

Related articles:

References:

  1. Williams DR, de Silva R, Paviour DC, et al; Characteristics of two distinct clinical phenotypes in pathologically proven progressive supranuclear palsy: Richardson’s syndrome and PSP-parkinsonism. Brain. 2005 Jun;128(Pt 6):1247-58.
  2. Litvan I, Agid Y, Calne D, et al; Clinical research criteria for the diagnosis of progressive supranuclear palsy (Steele-Richardson-Olszewski syndrome): report of the NINDS-SPSP international workshop. Neurology. 1996 Jul;47(1):1-9.
  3. Williams DR, Lees AJ; Progressive supranuclear palsy: clinicopathological concepts and diagnostic challenges. Lancet Neurol. 2009 Mar;8(3):270-9.
  4. Rafal RD, Grimm RJ. Progressive supranuclear palsy: functional analysis of the response to methysergide and antiparkinsonian agents. Neurology. 1981 Dec;31(12):1507-18.