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Creutzfeldt-Jakob Disease (CJD)

Knaus CMK - MedPix (2014)

View Article: MedPix Image - MedPix Case

Affiliation: Walter Reed National Military Medical Center

ABSTRACT

Diagnosis: Creutzfeldt-Jakob Disease (CJD)

History: 59 y.o. woman three months ago acute onset of neurologic symptoms. She has developed generalized hypokinesia, masked facies, tremors, and dysmetria. Additionally, her short term memory function has become severely compromised. She also reports rapidly progressive ataxia, speech abnormalities, diplopia. She has lived in Germany for 20 years as enlisted spouse. No prior history of neurologic disease or early dementia in her family. She has three adult children who are all healthy and living in the states.

Findings: Abnormal increased signal intensity of putamen and caudate on T2 FLAIR

Ddx: Clinical Differential: Alzheimers dementia, Lewy body dementia, frontotemporal dementia, Parkinson’s dementia, metabolic disorders (Hashimoto’s encephalitis, hepatic encephalopathy), infectious encephalitis, HIV, malignancy, toxic encephalopathy, cerebral degeneration secondary to chronic toxin ingestion, psychiatric disease, cerebrovascular disease, autoimmune disorders

Dxhow: Clinical presentation with confirmatory imaging

Exam: Vitals signs within normal limits and are stable. Neuro Exam MS: Oriented to person, place and time. Attention not intact by MOYB>20 seconds, MOYF =12 secs. Pt was able to count number of people in the room correctly. Pt able to follow only 2 step commands. Pt able to register 3/3 words, diminished recall of items 20 mins after asked demonstrated 1/3 words correct. Language is fluent however phrase length diminished. Repetition and naming intact. No right left confusion. Pt does demonstrate some item substitution with ideomotor praxis testing with some evidence of praxis impairment during exam. No evidence of neglect CN: II: VA 20/30, VFFTC, pupils symmetric with sluggish reactivity III,IV,VI: Full versions, patient has diplopia with right gaze with apparent nystagmus movements bl on right gaze. Pt's pursuits are saccadic. Pt's saccades demonstrate considerable overshoot specifically when looking from right to left. Absent upgaze however able to look up with oculogyric testing. OKN is absent in all directions. No ptosis present. Noted esotropia on right gaze. VOR performed and demonstrated numerous catch-up saccades in right and left directions. V: V1-3 grossly intact, MOM intact, prominent jaw titubation with mouth mildly open VII: Decreased expression of facies at rest however symmetrical smile with myoclonic movement of jaw and lip VIII: Grossly intact to conversation IX,X: demonstrates profound hypophonia while speaking XI: Intact bilaterally XII: Demonstrates lingual titubation however grossly intact. Motor: Increased tone in upper extremities, with moderately increased tone in the lower extremities. No pronator drift. Noted postural myoclonic/titubation movements of the distal extremities R>L with appendicular movement hesitation BL. Noted for truncal titubation while sitting up. Pt strength in upper and lower extremities. Reflexes: Pt is hyper-reflexic and brisk 3/4 throughout with equivocal to down going toes to Babinski Coordination: Pt FTN and heel to shin demonstrate bl dysmetria. RAM in upper and lower extremities are slow but do not demonstrate a decremental response. Sensory: FT and temperature intact bilaterally. Proprioception intact distally and vibarotyr intact. Romberg is normal. Graphesthesia intact Gait: Pt with significant difficulty standing up form seated position with evidence of truncal titubation/myoclonus. Narrow base stance with positive pull test demonstrating retropulsion. Evidence of axial instability during gait.

No MeSH data available.


Small, hyperintense lesions are seen in the white matter
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MPX1951_synpic58764: Small, hyperintense lesions are seen in the white matter


Creutzfeldt-Jakob Disease (CJD)

Knaus CMK - MedPix (2014)

Small, hyperintense lesions are seen in the white matter
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=MPX1951&req=5

MPX1951_synpic58764: Small, hyperintense lesions are seen in the white matter

View Article: MedPix Image - MedPix Case

Affiliation: Walter Reed National Military Medical Center

ABSTRACT

Diagnosis: Creutzfeldt-Jakob Disease (CJD)

History: 59 y.o. woman three months ago acute onset of neurologic symptoms. She has developed generalized hypokinesia, masked facies, tremors, and dysmetria. Additionally, her short term memory function has become severely compromised. She also reports rapidly progressive ataxia, speech abnormalities, diplopia. She has lived in Germany for 20 years as enlisted spouse. No prior history of neurologic disease or early dementia in her family. She has three adult children who are all healthy and living in the states.

Findings: Abnormal increased signal intensity of putamen and caudate on T2 FLAIR

Ddx: Clinical Differential: Alzheimers dementia, Lewy body dementia, frontotemporal dementia, Parkinson’s dementia, metabolic disorders (Hashimoto’s encephalitis, hepatic encephalopathy), infectious encephalitis, HIV, malignancy, toxic encephalopathy, cerebral degeneration secondary to chronic toxin ingestion, psychiatric disease, cerebrovascular disease, autoimmune disorders

Dxhow: Clinical presentation with confirmatory imaging

Exam: Vitals signs within normal limits and are stable. Neuro Exam MS: Oriented to person, place and time. Attention not intact by MOYB>20 seconds, MOYF =12 secs. Pt was able to count number of people in the room correctly. Pt able to follow only 2 step commands. Pt able to register 3/3 words, diminished recall of items 20 mins after asked demonstrated 1/3 words correct. Language is fluent however phrase length diminished. Repetition and naming intact. No right left confusion. Pt does demonstrate some item substitution with ideomotor praxis testing with some evidence of praxis impairment during exam. No evidence of neglect CN: II: VA 20/30, VFFTC, pupils symmetric with sluggish reactivity III,IV,VI: Full versions, patient has diplopia with right gaze with apparent nystagmus movements bl on right gaze. Pt's pursuits are saccadic. Pt's saccades demonstrate considerable overshoot specifically when looking from right to left. Absent upgaze however able to look up with oculogyric testing. OKN is absent in all directions. No ptosis present. Noted esotropia on right gaze. VOR performed and demonstrated numerous catch-up saccades in right and left directions. V: V1-3 grossly intact, MOM intact, prominent jaw titubation with mouth mildly open VII: Decreased expression of facies at rest however symmetrical smile with myoclonic movement of jaw and lip VIII: Grossly intact to conversation IX,X: demonstrates profound hypophonia while speaking XI: Intact bilaterally XII: Demonstrates lingual titubation however grossly intact. Motor: Increased tone in upper extremities, with moderately increased tone in the lower extremities. No pronator drift. Noted postural myoclonic/titubation movements of the distal extremities R>L with appendicular movement hesitation BL. Noted for truncal titubation while sitting up. Pt strength in upper and lower extremities. Reflexes: Pt is hyper-reflexic and brisk 3/4 throughout with equivocal to down going toes to Babinski Coordination: Pt FTN and heel to shin demonstrate bl dysmetria. RAM in upper and lower extremities are slow but do not demonstrate a decremental response. Sensory: FT and temperature intact bilaterally. Proprioception intact distally and vibarotyr intact. Romberg is normal. Graphesthesia intact Gait: Pt with significant difficulty standing up form seated position with evidence of truncal titubation/myoclonus. Narrow base stance with positive pull test demonstrating retropulsion. Evidence of axial instability during gait.

No MeSH data available.