Cretzfeldt-Jakob Disease Mimicking Progressive Supranuclear Palsy Presenting With Supranuclear Gaze Palsy and Early Falling

Progressive supranuclear palsy is a four-repeat tauopathy that is confirmed, like all neurodegenerative disease, at postmortem examination. An expanding group of clinical syndromes are now linked with this pathology in its early stages, although with disease progression there tends to be greater clinical similarity with the classical Richardson’s syndrome, an akinetic rigid form of parkinsonism with a progressive supranuclear gaze palsy and prominent frontal cognitive impairment. Currently, there are no disease-modifying therapies for progressive supranuclear palsy; however, there continues to be interest in immunotherapies targeted at tau pathology. Liaison with colleagues with an interest in palliative neurology is appropriate for patients in the advanced stages of the disease.

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Palliative care in non-malignant neurological disease

10.1093/med/9780199234356.003.0029 ◽

2010 ◽

Author(s):

Max Watson ◽

Caroline Lucas ◽

Andrew Hoy ◽

Jo Wells

Keyword(s):

Multiple Sclerosis ◽

Palliative Care ◽

Multiple System Atrophy ◽

Motor Neuron ◽

Progressive Supranuclear Palsy ◽

Motor Neuron Disease ◽

Symptom Management ◽

Neurological Disease ◽

Neurological Complications ◽

Jakob Disease

This chapter focuses on the symptom management of multiple sclerosis, Parkinson’s disease, multiple system atrophy (MSA), progressive supranuclear palsy (PSP), motor neuron disease, neurological complications of AIDS, Creutzfeldt-Jakob disease (CJD), and useful contacts.

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Neuropareidolia: diagnostic clues apropos of visual illusions

Arquivos de Neuro-Psiquiatria ◽

10.1590/s0004-282x2009000600033 ◽

2009 ◽

Vol 67 (4) ◽

pp. 1117-1123 ◽

Cited By ~ 5

Author(s):

Péricles Maranhão-Filho ◽

Maurice B. Vincent

Keyword(s):

Neurodegenerative Disease ◽

Progressive Supranuclear Palsy ◽

Visual Illusions ◽

Radiology Practice ◽

Diagnostic Strategies ◽

Diagnostic Skills ◽

Progressive Scoliosis ◽

Radiological Aspect ◽

Horizontal Gaze ◽

Gaze Palsy

Diagnosis in neuroimaging involves the recognition of specific patterns indicative of particular diseases. Pareidolia, the misperception of vague or obscure stimuli being perceived as something clear and distinct, is somewhat beneficial for the physician in the pursuit of diagnostic strategies. Animals may be pareidolically recognized in neuroimages according to the presence of specific diseases. By associating a given radiological aspect with an animal, doctors improve their diagnostic skills and reinforce mnemonic strategies in radiology practice. The most important pareidolical perceptions of animals in neuroimaging are the hummingbird sign in progressive supranuclear palsy, the panda sign in Wilson's disease, the panda sign in sarcoidosis, the butterfly sign in glioblastomas, the butterfly sign in progressive scoliosis and horizontal gaze palsy, the elephant sign in Alzheimer's disease and the eye-of-the-tiger sign in pantothenate kinase-associated neurodegenerative disease.

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Genetic Creutzfeldt-Jakob disease with R208H mutation presenting as progressive supranuclear palsy

Movement Disorders ◽

10.1002/mds.24002 ◽

2012 ◽

Vol 27 (4) ◽

pp. 476-479 ◽

Cited By ~ 19

Author(s):

Radoslav Matěj ◽

Gabor G. Kovacs ◽

Silvie Johanidesová ◽

Jiří Keller ◽

Milada Matějčková ◽

...

Keyword(s):

Progressive Supranuclear Palsy ◽

Jakob Disease

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Gaze Control and Foot Kinematics During Stair Climbing: Characteristics Leading to Fall Risk in Progressive Supranuclear Palsy

Physical Therapy ◽

10.2522/ptj.20070159 ◽

2008 ◽

Vol 88 (2) ◽

pp. 240-250 ◽

Cited By ~ 10

Author(s):

Richard P Di Fabio ◽

Cris Zampieri ◽

Paul Tuite

Keyword(s):

Progressive Supranuclear Palsy ◽

Stair Climbing ◽

Gaze Control ◽

Ocular Motor ◽

Gaze Shift ◽

Foot Kinematics ◽

Gaze Fixation ◽

Step Initiation ◽

Vertical Gaze ◽

Gaze Palsy

Background and PurposeDoes gaze control influence lower-extremity motor coordination in people with neurological deficits? The purpose of this study was to determine whether foot kinematics during stair climbing are influenced by gaze shifts prior to stair step initiation.Subjects and MethodsTwelve subjects with gaze palsy (mild versus severe) secondary to progressive supranuclear palsy were evaluated during a stair-climbing task in a cross-sectional study of mechanisms influencing eye-foot coordination. Infrared oculography and electromagnetic tracking sensors measured eye and foot kinematics, respectively. The primary outcome measures were vertical gaze fixation scores, foot lift asymmetries, and sagittal-plane foot trajectories.ResultsThe subjects with severe gaze palsy had significantly lower lag foot lift relative to lead foot lift than those with a mild form of gaze palsy. The lag foot trajectory for the subjects with severe gaze palsy tended to be low, with a heading toward contact with the edge of the stair. Subjects with severe gaze palsy were 28 times more likely to experience “fixation intrusion” (high vertical gaze fixation score) during an attempted shift of gaze downward than those with mild ocular motor deficits (odds ratio [OR]=28.3, 95% confidence interval [CI]=6.4–124.8). Subjects with severe gaze shift deficits also were 4 times more likely to have lower lag foot lift with respect to lead foot lift than those with mild ocular motor dysfunction (OR=4.0, 95% CI=1.7–9.7).Discussion and ConclusionThe small number of subjects and the variation in symptom profiles make the generalization of findings preliminary. Deficits in gaze control may influence stepping behaviors and increase the risk of trips or falls during stair climbing. Neural and kinematic hypotheses are discussed as possible contributing mechanisms.

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