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:
- 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.
- 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.
- Williams DR, Lees AJ; Progressive supranuclear palsy: clinicopathological concepts and diagnostic challenges. Lancet Neurol. 2009 Mar;8(3):270-9.
- Rafal RD, Grimm RJ. Progressive supranuclear palsy: functional analysis of the response to methysergide and antiparkinsonian agents. Neurology. 1981 Dec;31(12):1507-18.