Creutzfeldt-Jakob disease presenting with alien limb sign

2006 ◽  
Vol 21 (7) ◽  
pp. 1040-1042 ◽  
Author(s):  
Brent Fogel ◽  
Mark Wu ◽  
Sarah Kremen ◽  
Kolar Murthy ◽  
George Jackson ◽  
...  
Keyword(s):  
Alien Limb ◽  
Jakob Disease

The alien limb phenomenon and Creutzfeldt–Jakob disease

2012 ◽  
Vol 18 (7) ◽  
pp. 842-846 ◽  
Author(s):  
Mark Rubin ◽  
Jonathan Graff-Radford ◽  
Bradley Boeve ◽  
Keith A. Josephs ◽  
Allen J. Aksamit
Keyword(s):  
Alien Limb ◽  
Jakob Disease

Unilateral spatial neglect and alien limb phenomenon may be early features of Creutzfeldt–Jakob disease

2013 ◽  
Vol 19 (3) ◽  
pp. 379-381 ◽  
Author(s):  
Silvana Zannino ◽  
Claudio Liguori ◽  
Maria Albanese ◽  
Fabio Placidi ◽  
Francesca Izzi ◽  
...  
Keyword(s):  
Spatial Neglect ◽  
Unilateral Spatial Neglect ◽  
Alien Limb ◽  
Jakob Disease ◽  
Early Features

SPORADIC CREUTZFELDT-JAKOB DISEASE PRESENTING WITH A ‘JERKY ALIEN LIMB’

2015 ◽  
Vol 86 (11) ◽  
pp. e4.186-e4
Author(s):  
Maruthi Ravi Vinjam ◽  
Steven Butterworth ◽  
Richard Davey
Keyword(s):  
Cognitive Function ◽  
Acute Onset ◽  
Motor Tasks ◽  
Initial Examination ◽  
Alien Limb ◽  
Simple Motor ◽  
History Of ◽  
Jakob Disease ◽  
Time Of Presentation ◽  
Sporadic Cjd

A 64 yr old right-handed mechanic presented with four weeks history of clumsy right hand with ongoing problems at work. He described stiffness and problems with co-ordination. At the time of presentation he was noted to be walking with his right arm behind him and not being aware of this. His initial examination showed Mini Mental Score of 26/30, with ongoing involuntary movements of his right arm (Video).Video shows spontaneous elevation of right arm with occasional myoclonic jerks. Video also demonstrates patient's difficulty in following simple motor tasks and his comments that his arm “has a mind of its own”.His MRI head (Figure 1) showed typical cortical ribboning pattern described in sporadic CJD and CSF Protein for 14-3-3, s100b and RT-QUIC findings were consistent with the diagnosis of CJD.Over next 4 weeks his cognitive function rapidly deteriorated with progressive worsening of his myoclonus. He died 4 weeks after his hospital discharge.There are three broad varieties of alien limb phenomenon (ALP) types described in the literature, frontal, callosal and sensory. Jerky (myoclonic) ALP is well described in patients with CJD, so CJD should be in the differentials in any patient presenting with sub-acute onset of ALP.

The Alien Limb Phenomenon & Creutzfeldt-Jakob Disease (P03.257)

Neurology ◽  
2012 ◽  
Vol 78 (Meeting Abstracts 1) ◽  
pp. P03.257-P03.257
Author(s):  
M. Rubin ◽  
J. Graff-Radford ◽  
B. Boeve ◽  
K. Josephs ◽  
A. Aksamit
Keyword(s):  
Alien Limb ◽  
Jakob Disease

The alien limb phenomenon and Creutzfeldt–Jakob disease

2013 ◽  
Vol 19 (3) ◽  
pp. 378
Author(s):  
Mark N. Rubin
Keyword(s):  
Alien Limb ◽  
Jakob Disease

scholarly journals Rare histotype of sporadic Creutzfeldt-Jakob disease, clinically suspected as corticobasal degeneration

2019 ◽  
Vol 12 (3) ◽  
pp. e228305 ◽  
Author(s):  
Bension Shlomo Tilley ◽  
Colin Smith ◽  
Nicola Pavese ◽  
Johannes Attems
Keyword(s):  
Neurodegenerative Disease ◽  
Neurological Disorders ◽  
Tissue Bank ◽  
Corticobasal Degeneration ◽  
Hippocampal Atrophy ◽  
Hyperphosphorylated Tau ◽  
Alien Limb ◽  
Jakob Disease ◽  
Atypical Presentations ◽  
And Diffusion

Sporadic Creutzfeldt-Jakob disease (sCJD) is a rare neurodegenerative disease that can mimic other neurological disorders. We present a case of sCJD in a 64-year-old man that presented with corticobasal syndrome and survived for 3 years. He presented initially with dementia, hemiparkinsonism and alien limb phenomenon and was diagnosed with corticobasal degeneration, ultimately progressing to immobility and akinetic mutism. With a normal MRI 1 year before onset, his neuroimaging 1 year later revealed abnormal DaTscan, cortical and hippocampal atrophy with ventricular dilatation on MRI, and diffusion-weighted cortical ribboning and thalamic hyperintensity. Postmortem, the patient’s brain was collected by the Parkinson’s UK Tissue Bank. Prion protein immunohistochemistry revealed widespread diffuse microvacuolar staining without kuru-type plaques. Hyperphosphorylated tau was only found in the entorhinal cortex and hippocampus. This case highlights the clinical heterogeneity of sCJD presentation and the important inclusion of CJD in the differential diagnosis of atypical presentations of neurodegenerative disease.

The classic and variant forms of Creutzfeldt-Jakob disease

2003 ◽  
Vol 8 (1) ◽  
pp. 71-79 ◽  
Author(s):  
David N. Irani
Keyword(s):  
Jakob Disease

Upbeat nystagmus as the initial clinical sign of Creutzfeldt-Jakob disease

2005 ◽  
Vol 32 (S 4) ◽  
Author(s):  
M Strupp ◽  
V.C Zingler ◽  
K Jahn ◽  
M Glaser ◽  
H Kretzschmar ◽  
...  
Keyword(s):  
Clinical Sign ◽  
Jakob Disease ◽  
Upbeat Nystagmus
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