Poster Presentations: Tuesday, July 18, 2017 Background: Behavioral variant frontotemporal dementia (bvFTD) is a devastating early onset dementia. Symptoms of bvFTD also may be caused by spontaneous intracranial hypotension (SIH), a treatable disorder, but no comprehensive study of such patients has been reported. We therefore reviewed our extensive experience with patients with SIH and bvFTD. Methods: Using a prospectively maintained registry, we identified all patients with SIH (based on the criteria of the International Classification of Headache Disorders, third edition [ICHD-III]) who met clinical criteria for bvFTD (International Behavioral Variant FTD Criteria Consortium [FTDC]). Patients or their caregivers/family were contacted for follow-up. A modified Migraine Disability Assessment Score (MIDAS) questionnaire was used to assess the severity of the symptoms (Grade I or II: good outcome; grade III or IV: poor outcome). The patients were compared to a cohort of SIH patients without bvFTD. Results: The mean age at onset of SIH symptoms for the 21 men and eight women was 50.6 years (range, 34-65 years) and it was 52.9 years (range, 37-65 years) at the time of onset of bvFTD symptoms. All patients had hypersomnolence. MRI showed brain sagging in all patients, CSF opening pressure was low in about half of patients, but a spinal CSF leak could not be detected in any patient. Three patients only underwent epidural blood patching, with a good outcome in one patient. After unsuccessful directed percutaneous treatments, the remaining 26 patients
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underwent one or more surgical procedures with a good outcome in 20 patients (77%). Overall, a good outcome was obtained in 21 patients (72%); 20 (91%) of 22 patients who had not undergone prior Chiari surgery compared to one (14%) of seven patients who did undergo Chiari surgery (p<0.003). Compared to SIH patients without symptoms of bvFTD (n¼547), those with bvFTD symptoms were older, more often male, less often demonstrated a CSF leak on spinal imaging, and more often underwent surgery (p<0.02). Conclusions: bvFTD in SIH is rare and associated with brain sagging and hypersomnolence. Spinal CSF leaks are rarely detected. bvFTD symptoms are often refractory to the usual percutaneous procedures but most patients can be cured, although one or more surgical treatments are generally required.
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THE ASSOCIATION BETWEEN NEUROPSYCHIATRIC SYMPTOMS, APOE ε4 AND PROGRESSION OF ALZHEIMER’S DISEASE
Yi-Chien Liu1,2, Jong-Ling Fuh3,4, Kenichi Meguro2, 1Cardinal Tien Hospital, New Taipei City, Taiwan; 2Tohoku University CYRIC, Sendai, Japan; 3Taipei Veterans General Hospital, Taipei, Taiwan; 4National Yang-Ming University Schools of Medicine, Taipei, Taiwan. Contact e-mail:
[email protected] Background: The progression of Alzheimer disease is very diffi-
cult to predict. Whether neuropsychiatric symptoms, APOE ε4 may play a role is still unknown. Methods: From 2012 to 2015, we prospectively followed 492 patients with probable AD from the memory clinic of Taipei Veterans General Hospital for median of 3 years. All patients underwent standard assessments, including brain magnetic resonance imaging or computed tomography, neuropsychological tests, neuropsychiatry inventory (NPI-Q) and related blood tests. A logistic regression analysis was performed to investigate the relationship between change of clinical dementia rating scale-sum of boxes (CDR-SOB) and age, gender, education, APOE ε4 allele and various neuropsychiatric symptoms. Results: Of all the participants, 306 (62.1%) completed the initial examinations and 2 times of follow-up. Among 306 patients, 222 (73%) showed CDR-SOB decline. We observed initial neuropsychiatric symptom of agitation (OR ¼ 1.24; 95% CI ¼ 1.03-1.419, p ¼ 0.021) and APOE ε4 (OR ¼ 1.83; 95% CI ¼ 1.10-3.04, p ¼ 0.019) both related to CDR-SOB decline. Conclusions: Baseline agitation and APOE ε4 significantly associated with clinical progression of Alzheimer disease.