Presenile Dementia. Mary Ellen Quiceno, M.D. Case #1. 33 y.o. reported memory loss in 2000. In 2002, episodes of left-sided numbness & weakness. Febrile day prior to first admission in 2002 for h/a, n/v, and left-sided weakness. Abnormal MRI and LP.
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Mary Ellen Quiceno, M.D.
First attributed to depression, referred to psychiatrist.
Personality change, blunted affect, loss of motivation.
Frontal atrophy on MRI (may be missed).
Progressive fluent aphasia.
Mistaken for AD.
nvCJD = BSE
Genetic susceptibility in 40% of UK residents
Rapid dementia in 60’s w/death <6 mo.
Insomnia, amotivation, myoclonus, ataxia, cortical blindness.
similar to sporadic
Fatal Familial Insomnia
insomnia & dysautonomia
ataxia, dementiaSporadic & Inherited Prion D/Os
Supranuclear gaze palsy,
Altered consciousness are the commonest neurological findings.
Oculomasticatory (OMM) and oculofacial skeletal myorhythmia (OFSM),
Cranial nerve abnormalities,
Sensory deficits.Symptoms of CNS Whipple’s Ds