Geschwind, unpublished data).[13] In our cohort, our initial data suggest that two additional surrogate biomarkers for sCJD, total tau (t-tau), neuron specific enolase (NSE)have somewhat higher level of sensitivity and specificity for CJD than 14-3-3 or EEG. most of which are referred having a potential analysis Jakob-Creutzfeldt disease (CJD). The number of referrals increased dramatically with the recognition of quinacrine like a potential therapy for CJD and commencement of the 1st U.S. CJD treatment trail in 20051.[1, 2] We recognized the need for any broader diagnostic approach to RPD when we observed that 15-20% of these referrals were due to additional non-prion conditions, many of which were treatable. Physicians, and even neurologists, generally are not qualified formally in the assessment of RPDs. With this review, we hope to provide a more thorough appreciation of the myriad of etiologies for RPDS and to offer a possible diagnostic decision tree or algorithm, centered mainly on the experience of our center. Most dementias develop slowly, permitting an unhurried outpatient evaluation. Algorithms for the assessment of these individuals have been developed and processed, and most neurologists are well acquainted with these methods. A careful history will usually detect dementia secondary to medications or major depression and routine laboratory assessments help to eliminate metabolic conditions that can cause dementia including anemia, electrolyte imbalance, liver or kidney failure, thyroid disease and vitamin B12 deficiency. The majority of slowly progressive dementias are secondary to Alzheimer’s disease (AD), although there is an increasing acknowledgement of non-AD dementias, including frontotemporal dementia (FTD; observe Chapter 1), subcortical ischemia vascular disease (SIVD; observe Chapter 8), dementia with Lewy body (DLB; see Chapter 9), and additional parkinsonian dementias such as cortical Voruciclib basal degeneration (CBD) and progressive supranuclear palsy.[3] (see Chapter 11). However, with the possible exceptions of DLB and CBD, the disorders that generally lead to slowly progressive adult dementia, such as AD and FTD, rarely present as RPDs. [4-6] The patient with an RPD often requires consideration of a different set of disorders. The primary aim of this chapter is to instruct clinicians in an approach to the differential analysis of RPD that may broaden their scope of inquiry and, particularly, to identify RPDs that are treatable and potentially reversible. Rabbit Polyclonal to CDK5 We have structured this chapter around the following groups: neurodegenerative, autoimmune, harmful/metabolic, infectious, neoplastic, vascular, and we will emphasize the RPDs that are most difficult to diagnose or least likely to be identified. As many types of conditions can cause RPD and they can progress quickly, it is important to have an structured, systematic approach to analysis. The mnemonic VITAMINS is often useful for summarizing some of the major categories of etiologies for RPDs (Table 13.1). Table 13.2 lists many etiologies of RPD, many of Voruciclib which there Voruciclib is unfortunately not space to discuss with this review. When considering an RPD patient, it may be helpful to use these furniture to ensure a complete differential has been regarded as. RPDs that present with space occupying mind lesions very easily recognized by CT or MRI scan, are not discussed in this chapter. Numbers 13.1 and 13.2 provide an format for the diagnostic approach that we use in evaluating a patient with RPD. Number 13.1 shows the overall approach, while 13.2 details an expanded evaluation when standard testing is definitely inconclusive. Open in a separate window Number 13.1 RPD Evaluation Open in a separate window Number 13.2 Further RPD Evaluation Table 13.1 VITAMINS – mnemonic for categories of conditions causing RPDs VascularInfectiousToxic-MetabolicAutoimmuneMetastases/NeoplasmIatrogenicNeurodegenerativeSystemic Open in a separate window Table 13.2 Differential Analysis of Rapidly Progressive Dementias NeurodegenerativeJakob-Creutzfeldt disease (CJD; sporadic, iatrogenic, familial)Alzheimer’s disease (AD)Dementia with Lewy Body (DLB)Frontotemporal dementia (FTD)Corticobasal degeneration (CBD)Progressive Supranuclear Palsy (PSP)InfectiousViral encephalitis, including HSVHIV dementiaProgressive Multifocal Leukoencephalopathy (PML)Subacute sclerosing panencephalitis (SSPE; young adults)Fungal infections (immunosuppression e.g., CNS aspergillosis,)SyphilisParasitesLyme disease (hardly ever encephalopathy)BalamuthiaWhipple’s DiseaseToxic/MetabolicVitamin B12 (cyanocobalmin) Voruciclib deficiencyVitamin B1 (Thiamine) deficiencyNiacin deficiencyFolate deficiency (dementia rare)UremiaWilson’s diseasePortosystemic encephalopathyAcquired hepatocerebral degenerationPorphyriaBismuth toxicityLithium toxicityMercury toxicityArsenic toxicityElectrolyte AbnormalitiesAutoimmuneHashimoto’s EncephalopathyParaneoplastic (autoimmune) limbic encephalopathy (PLE)Non-paraneoplastic autoimmune (e.g., anti-VGKC mediated)Lupus cerebritisOther CNS VasculitidesSarcoidEndocrine AbnormalitiesThyroid disturbancesParathyroid abnormalitiesAdrenal diseasesNeoplasm-relatedNon-autoimmune paraneoplastic conditionsMetastases to CNSPrimary CNS lymphomaIntravascular lymphomaLymphomatoid granulomatosisGliomatosis cerebri Open in a separate window Over the past five years, our dementia center has been referred approximately 825 RPD instances, many having a presumptive analysis of CJD. After critiquing records and in many cases evaluating the.
Geschwind, unpublished data)