Faculty Bibliography

IMPORTANCE: Earlier research on celiac disease (CD) and neuropathy has been hampered by the use of inpatient data, low study power, and lack of neuropathic characteristics. OBJECTIVE: To examine the relative risk and absolute risk of developing neuropathy in a nationwide population-based sample of patients with biopsy-verified CD. DESIGN, SETTING, AND PARTICIPANTS: Between October 27, 2006, and February 12, 2008, we collected data on small-intestinal biopsies performed at Sweden's 28 pathology departments between June 16, 1969, and February 4, 2008. We compared the risk of neuropathy in 28,232 patients with CD (villous atrophy, Marsh 3) with that of 139,473 age- and sex-matched controls. Cox proportional hazards regression estimated hazard ratios (HRs) and 95% CIs for neuropathy defined according to relevant International Classification of Diseases codes in the Swedish National Patient Register (consisting of both inpatient and outpatient data). MAIN OUTCOMES AND MEASURES: Neuropathy in patients with biopsy-verified CD. RESULTS: Celiac disease was associated with a 2.5-fold increased risk of later neuropathy (95% CI, 2.1-3.0; P < .001). We also found an increased risk (with results reported as HRs [95% CIs]) of chronic inflammatory demyelinating neuropathy (2.8; 1.6-5.1; P = .001), autonomic neuropathy (4.2; 1.4-12.3; P = .009), and mononeuritis multiplex (7.6; 1.8-32.4; P = .006), but no association between CD and acute inflammatory demyelinating polyneuropathy (0.8; 0.3-2.1; P = .68). CONCLUSIONS AND RELEVANCE: We found an increased risk of neuropathy in patients with CD. This statistically significant association in a population-based sample suggests that CD screening should be completed in patients with neuropathy.

OBJECTIVES: Bortezomib is a proteasome inhibitor that is frequently used for multiple myeloma and lymphoma. A sensory predominant axonal neuropathy is associated with bortezomib treatment but a demyelinating neuropathy is also described primarily based on electrodiagnostic findings. We report a series of patients treated with bortezomib who developed peripheral neuropathy and were found to have demyelinating features on electrodiagnostic testing. METHODS: Four patients who developed a bortezomib-induced peripheral neuropathy underwent electrophysiological testing, and 1 patient had a nerve biopsy. RESULTS: The four patients with bortezomib-induced peripheral neuropathy had demyelinating features on their electrophysiological testing. The nerve biopsy performed in 1 patient demonstrated a demyelinating component in a background of axonal degeneration. CONCLUSIONS: Although most toxic neuropathies are symmetrical axonal neuropathies, bortezomib is part of a small list of agents that may cause a demyelinating polyneuropathy and axonal degeneration. These findings have been confirmed by nerve biopsy.

Rationale: Reducing hospital admission length of stay (LOS) has been identified as one way to improve quality and reduce healthcare costs. The epilepsy monitoring unit (EMU) poses unique challenges to reductions in LOS, given the elective nature of the admissions and the need to safely provoke seizures. Data delineating the factors that impact EMU LOS are currently very limited. In an attempt to establish benchmarks for care, we evaluated the time to first, second, and third target events (TE) in patients admitted to the EMU at a large tertiary care epilepsy center. Methods: We reviewed the medical records of 905 consecutive patients who were admitted for diagnostic scalp video-EEG monitoring at NYU's Comprehensive Epilepsy Center from January 1, 2011 to December 31, 2011. Admission reason and time to TE were assessed. Results: 40.4% of patients (366/905) experienced a first TE; mean time to first TE for pre-surgical patients was 3.4 days (D) vs. 2.5D for non-surgical patients. 31.7% (287/905) experienced a second TE; the mean time to second TE was 4.4D for pre-surgical patients vs. 2.7D for non-surgical patients. 24.7% (224/905) experienced three or more TEs; the mean time to third TE for pre-surgical patients was 4.9D vs. 2.9D for non-surgical patients. Of the patients who experienced TEs during admission, 21.5% (79/366) had only one TE during admission; of these patients, mean LOS was 7.2D for pre-surgical patients (12/79) vs. 5.1D for non-surgical patients. 17.2% (63/366) experienced only two TEs during admission; mean LOS was 8D for pre-surgical patients (14/63) vs. 5.3D for non-surgical patients. 61.2% (224/366) experienced three or more TEs during admission; mean LOS was 6.7D for pre-surgical patients (54/224) vs. 6.3D for non-surgical patients. Conclusions: Time to first TE is longer in patients admitted to the EMU for pre-surgical evaluation vs. non-surgical patients. The latter group includes patients with psychogenic non-epileptic seizures; such patients produce events more quickly i!

Rationale: Increasingly, hospitals are striving to shorten length of stay (LOS) in an effort to improve care. Beyond the impact on expense, longer stays in the hospital increase the risk of iatrogenic or other complications. The epilepsy monitoring unit (EMU) is a unique hospital setting where the admission goal focuses on capturing one or more events/seizures. Seizure provocation is performed in a controlled manner to minimize the risk of secondarily generalized convulsions and status epilepticus, and to maintain patient safety. To better establish benchmarks for care, we evaluated both the impact of admission objectives and select patient characteristics on EMU LOS. Methods: We reviewed the medical records of 905 consecutive patients who were admitted for diagnostic scalp video-EEG monitoring at NYU's Comprehensive Epilepsy Center from January 1, 2011 to December 31, 2011. Reasons for admission, number of anti-epileptic drugs on admission, and time to target events were documented. Results: Of the 905 patients included, the mean length of stay was 4.8+3.2 days. The median length of stay was 4 days. 95 subjects were admitted for pre-surgical evaluation with a mean LOS of 7.2 days, vs. 4.5 days (p<0.001) for those admitted for diagnostic evaluations. 12.8% (116/905) of patients admitted to the EMU had a previous history of epilepsy surgery and the majority of these patients were admitted for medication adjustment; patients who had undergone prior epilepsy surgery experienced longer length of stays (5.9 days) compared to those without a prior epilepsy surgical history (4.7 days) (p<0.0002). Patients admitted on three or more antiepileptic drugs (AEDs) on admission experienced longer lengths of stay (6.3 days) vs. patients on less than three concomitant AEDs (4.3 days). Patients were further stratified by reason for admission. 494/905 patients were primarily admitted for seizure characterization and these patients experienced a mean LOS of 3.9 days whereas patients who were admitted for medicatio!

BACKGROUND: Mild cognitive deficits, mainly in frontal-executive function and memory, have been reported in patients with essential tremor (ET). Furthermore, an association between ET and dementia has been reported in a single population-based study in Spain. This has not been confirmed elsewhere. OBJECTIVE: To determine whether baseline ET is associated with prevalent and incident dementia in an ethnically diverse, community-based sample of elders. METHODS: Community-dwelling elders in northern Manhattan were enrolled in a prospective cohort study. Baseline ET diagnoses were assigned from handwriting samples. Dementia was diagnosed at baseline and follow-up using DSM-III-R criteria. RESULTS: In cross-sectional analyses, 31/124 (25.0%) ET cases had prevalent dementia vs 198/2,161 (9.2%) controls (odds ratio [OR](unadjusted) = 3.31, 95% confidence interval [CI] = 2.15-5.09, p < 0.001; OR(adjusted) = 1.84, 95% CI = 1.13-2.98, p = 0.01). In prospective analyses, 17/93 (18.3%) ET cases vs 171/1,963 (8.7%) controls developed incident dementia (hazard ratio [HR](unadjusted) = 2.78, 95% CI = 1.69-4.57, p < 0.001; HR(adjusted) = 1.64, 95% CI = 0.99-2.72, p = 0.055). CONCLUSIONS: In a second population-based study of elders, essential tremor (ET) was associated with both increased odds of prevalent dementia and increased risk of incident dementia. Presence of dementia, therefore, appeared to be greater than that expected for age (i.e., a disease-associated feature). Rather than attributing cognitive complaints in patients with ET to old age, assessment and possible treatment of dementia should be routinely incorporated into the treatment plan.

BACKGROUND: Our aims were to: (1) estimate the prevalence of essential tremor (ET) in a community-based study in northern Manhattan, New York, N.Y., USA; (2) compare prevalence across ethnic groups, and (3) provide prevalence estimates for the oldest old. METHODS: This study did not rely on a screening questionnaire. Rather, as part of an in-person neurological evaluation, each participant produced several handwriting samples, from which ET diagnoses were assigned. RESULTS: There were 1,965 participants (76.7 +/- 6.9 years, range = 66-102 years); 108 had ET [5.5%, 95% confidence interval (CI) = 4.5-6.5%]. Odds of ET were robustly associated with Hispanic ethnicity versus white ethnicity [odds ratio (OR) = 2.19, 95% CI = 1.03-4.64, p = 0.04] and age (OR = 1.14, 95% CI = 1.03-1.26, p = 0.01), i.e. with every 1 year advance in age, the odds of ET increased by 14%. Prevalence reached 21.7% among the oldest old (age > or = 95 years). CONCLUSIONS: This study reports a significant ethnic difference in the prevalence of ET. The prevalence of ET was high overall (5.5%) and rose markedly with age so that in the oldest old, more than 1 in 5 individuals had this disease.