The objective of this paper is to evaluate available evidence for each step in autoimmune encephalitis management and provide expert opinion when evidence is lacking. The paper approaches autoimmune encephalitis as a broad category rather than focusing on individual antibody syndromes. Core authors from the Autoimmune Encephalitis Alliance Clinicians Network reviewed literature and developed the first draft. Where evidence was lacking or controversial, an electronic survey was distributed to all members to solicit individual responses. Sixty-eight members from 17 countries answered the survey. Corticosteroids alone or combined with other agents (intravenous IG or plasmapheresis) were selected as a first-line therapy by 84% of responders for patients with a general presentation, 74% for patients presenting with faciobrachial dystonic seizures, 63% for NMDAR-IgG encephalitis and 48.5% for classical paraneoplastic encephalitis. Half the responders indicated they would add a second-line agent only if there was no response to more than one first-line agent, 32% indicated adding a second-line agent if there was no response to one first-line agent, while only 15% indicated using a second-line agent in all patients. As for the preferred second-line agent, 80% of responders chose rituximab while only 10% chose cyclophosphamide in a clinical scenario with unknown antibodies. Detailed survey results are presented in the manuscript and a summary of the diagnostic and therapeutic recommendations is presented at the conclusion.
The objective of this paper is to evaluate available evidence for each step in autoimmune encephalitis management and provide expert opinion when evidence is lacking. The paper approaches autoimmune encephalitis as a broad category rather than focusing on individual antibody syndromes. Core authors from the Autoimmune Encephalitis Alliance Clinicians Network reviewed literature and developed the first draft. Where evidence was lacking or controversial, an electronic survey was distributed to all members to solicit individual responses. Sixty-eight members from 17 countries answered the survey. The most popular bridging therapy was oral prednisone taper chosen by 38% of responders while rituximab was the most popular maintenance therapy chosen by 46%. Most responders considered maintenance immunosuppression after a second relapse in patients with neuronal surface antibodies (70%) or seronegative autoimmune encephalitis (61%) as opposed to those with onconeuronal antibodies (29%). Most responders opted to cancer screening for 4 years in patients with neuronal surface antibodies (49%) or limbic encephalitis (46%) as opposed to non-limbic seronegative autoimmune encephalitis (36%). Detailed survey results are presented in the manuscript and a summary of the diagnostic and therapeutic recommendations is presented at the conclusion.
These results suggest that persons with MCI can improve their performance on cognitive and functional measures when provided with early cognitive training and it could persist in a long-term follow-up.
The different dementia types have different costs. Overall, costs increased with the presence of behavioral symptoms, depression and functional impairment of activities of daily living.
Background: "Forgetfulness" is frequent in normal aging and characteristic of the early
stages of dementia syndromes. The episodic memory test is central for detecting
amnestic mild cognitive impairment (MCI). The Memory Binding Test (MBT) is a
simple, easy and brief memory test to detect the early stage of episodic memory
impairment. Objective: To validate the Argentine version of the MBT in a Latin American population and
to estimate the diagnostic accuracy as a tool for early detection of MCI. Methods: 88 subjects (46 healthy controls and 42 patients with amnestic MCI) matched for
age and educational level were evaluated by an extensive neuropsychological
battery and the memory binding test. Results: A significantly better performance was detected in the control group; all MBT
scales were predictive of MCI diagnosis (p<.01). The MBT showed high
sensitivity (69%) and high specificity (88%), with a PPV of 93% and a NPV of 55%
for associative paired recall. A statistically significant difference
(c2=14,164, p<.001) was obtained when comparing the area under
the curve (AUC) of the MBT (0.88) and the MMSE (0.70). Conclusion: The Argentine version of the MBT correlated significantly with the MMSE and the
memory battery and is a useful tool in the detection of MCI. The operating
characteristics of the MBT are well suited, surpassing other tests commonly used
for detecting MCI.
Mutations in presenilin-1 (PSEN1) account for the majority of cases of familial autosomal dominant early-onset Alzheimer's disease (AD) as well as in sporadic forms. Atypical presentations are reported including extrapyramidal signs. In the last years, a pleiotropic effect of some PSEN1 variants has been reported in Parkinson's disease (PD).
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