Premium
Cortical limb myoclonus in pathologically proven progressive supranuclear palsy
Author(s) -
Kemp Sharon,
Harding Antony J.,
Halliday Glenda M.,
Mahant Neil,
Fung Victor S.C.
Publication year - 2013
Publication title -
movement disorders
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 3.352
H-Index - 198
eISSN - 1531-8257
pISSN - 0885-3185
DOI - 10.1002/mds.25693
Subject(s) - progressive supranuclear palsy , myoclonus , physical medicine and rehabilitation , corticobasal degeneration , neuroscience , psychology , medicine , computer science , information retrieval , pathology , disease
Progressive supranuclear palsy (PSP) is a sporadic tauopathy with insidious onset and a progressive course, first described in 1964 as parkinsonism, postural instability, axial rigidity, supranuclear gaze palsy, pseudobulbar palsy and cognitive changes.[1] However a much broader clinical spectrum is now recognised in association with PSP.[2] PSP may present as corticobasal syndrome (CBS) with asymmetric rigidity, apraxia, dystonia and variable cortical and basal ganglionic features.[3, 4] CBS was first reported in association with the pathological entity of corticobasal degeneration (CBD),[5] but is now recognised to develop secondary to PSP pathology as well as Alzheimers disease, frontotemporal dementia, and other less common causes.[6] This overlap contributes to difficulties with accurate ante-mortem diagnosis of both PSP and CBD. It has been suggested that myoclonus favours the likely pathological diagnosis of CBD rather than PSP,[7, 8] although very few pathologically proven cases of PSP have documented myoclonus.[9, 10] We report the clinical and neurophysiological features of cortical myoclonus in a patient with pathologically proven PSP, in order to highlight the clinical features that are helpful in distinguishing CBS arising from PSP compared with CBD pathology