Wednesday, May 02, 2007

Criteria for Sporadic CJD

WHO 1998
1.  Progressive dementia with any 2 of (myoclonus, pyramidal/EP, visual/cerebrellar/akinetic mutism)
2, typical EEG and/or if 2 year duration + CSF 14,3,3
3. Routine evaluation shows no other diagnosis
UCSF Modified
Rapid Cognitive Decline with any 2 of following: myoclonus, pyramidal/EP, visual, cerebellar, akinetic mutism, other focal cortical signs
typical MRI and/or EEG
Comments of MRI on CJD:  91 % sensitive and 93-95 % specific.  Findings include abnormal FLAIR and DWI in cortical gyri (ribboning)  especially in CN, PUT and THAL.  Only one form of CJD-- the MM2 subtype may not always show the DWI and FLAIR changes.  vCJD shows "pulvinar" sign in thalamus. 
Reviewing Geschwind's lecture and handout, the most useful table was the "lab finding not consistent with CJD".  They include normal DWI and FLAIR, FLAIR without corresponding DWI abnormalities, abnormal contrast enhancement, leukoencephalopathy or significant white matter abnormality not attributable to another condition, mass effect or edema, CSF pleocytosis, CSF protein > 100, or elevated IgG index.
Clinical finding not typically found in CJD included early seizures, significant GI distress, ataxia without cognitive impairment, cranial neuropathy, acute stroke like onset weakness, and chorea.  By contrast, aphasia, neglect and other "cortical" findings are common. 
Body CT/PET can be considered for cases of possible paraneoplastic syndrome.  Sarcoid also can be discovered. 

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HIV dementia notes (talk A Nath AAN 2007)

1.  Post Haart, HIV dementia is seen at higher CD4 counts and the nadir CD4 count is more important than present count.
2.  Host genetic factors are important in the development, including genetic mutations in the promoter region of monocyte chemoattractant factor 1 or its receptor CCR2 and TNF alpha. 
3.  Cognitive slowing and apathy are well known signs.  Gait disturbance, bradykinesia, falls, loss of dexterity, and frontal release signs are also well known.  Less well known is mania in 5 %, or accompanying myelopathy and symmetric peripheral sensory neuropathy.
4.  Distinguishing HIV dementia from effects of drug abuse is important since the latter is unlikely to respond to HAART.  Differentiating from effects of HCV is also a challenge especially as HAART drugs are metabolized hepatically.
5.  There is increased incidence in older patients and apo E4 alleles and thus an interaction with Alzheimer's disease which can also present in older patients, albeit faster in some cases than it would have otherwise.
6.  IRIS patients may have features similar to JC virus and PML but without JC virus detectable in brain or CSF, and IRIS patients may respond partially to steroids.
7.  Potent antiretrovirals with 3 or more highly penetrant drugs are the standard of care for most cases of HIV dementia.  Highly penetrant drugs include D4T, AZT, ABV, EFV, NVP, IDV. 

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