Cerebellar cognitive affective syndrome with psychotic features in a patient with hypertrophic olivary degeneration.

Objective: Hypertrophic Olivary Degeneration is a rare condition causing transneuronal degeneration of the inferior olivary nucleus. Symptoms manifest as progressively worsening palatal tremor, ataxia, and eye movement disturbances that plateau after several months. Though rarely documented in the literature of this specific condition, disconnection of the inferior olivary nucleus from the cerebellum, and cerebellar atrophy represent a pathway to developing subsequent cerebellar cognitive affective syndrome. The presented case documents the neuropsychological sequelae of a 39-year-old female with a history of hypertrophic olivary degeneration and symptoms of palatal tremor, opsoclonus myoclonus, ataxia, and delusions. Method: Review of the patient's medical records, interviews with the patient and her father, and a neuropsychological assessment battery were used to collect data. Review of currently published literature lent to case conceptualization. Results: Neuropsychological testing revealed deficits in executive functioning, attention, and language. An anomalous, fixed persecutory delusion was revealed. Conclusion: Hypertrophic olivary degeneration creates disconnection syndromes between the inferior olivary nucleus, red nucleus, and cerebellum. Late stages of the disorder cause atrophy of the inferior olivary nucleus and adjacent structures. While the motor sequela is well documented, the neuropsychological and psychiatric impact is infrequently discussed in existing literature. We present the first case to detail the neuropsychological sequelae of hypertrophic olivary degeneration and propose a mechanism for the development of cognitive impairment and psychotic features within this condition.

Presence of Irritable Bowel Syndrome Symptoms in Quiescent Inflammatory Bowel Disease Is Associated with High Rate of Anxiety and Depression.

BACKGROUND: Inflammatory bowel disease (IBD; Crohn's disease, CD and Ulcerative colitis, UC) and irritable bowel syndrome (IBS) have overlapping symptoms. Few prevalence studies of IBS in quiescent IBD have used colonoscopy with histology to confirm inactive disease. The aims were (1) to determine the percentage of IBD patients in deep remission whose persistent IBS-like symptoms (IBD/IBS+) would cause them to be classified as having active disease, based on the calculation of Harvey Bradshaw Index (HBI) or UC disease activity index (UCDAI); (2) to identify demographic and disease characteristics that are associated with IBD/IBS+. METHODS: This was a prospective study at a single tertiary care IBD center. 96/112 patients with colonoscopy and histology confirmed quiescent disease consented and completed Rome III criteria for IBS Survey, and the hospital anxiety and depression scale (HADS). Other demographic and disease specific data were collected. RESULTS: 36% (28/77) and 37% (7/19) of CD and UC patients, respectively, met diagnostic criteria for IBS. Significantly higher HBI/UCDAI scores (p = 0.005) and low short inflammatory bowel disease questionnaire (SIBDQ) scores (p ≤ 0.0001) were seen in IBD/IBS+ patients. 29% of patients in deep remission were mis-categorized by HBI/UCDAI as having active disease when they fulfilled Rome III criteria for IBS. Psychiatric diagnosis (OR 3.53 95% CI 1.2-10.2) and earlier onset of IBD (OR 1.056 95% CI 1.015-1.096) were associated with IBD/IBS+. Patients fulfilling IBS criteria had higher hospital anxiety and depression scale (HADS). CONCLUSION: IBD/IBS+ affect scoring of IBD disease activity scales and become less useful in guiding treatment plans.

Advanced Age Does Not Negatively Impact Health-Related Quality of Life in Inflammatory Bowel Disease.

BACKGROUND: Health-related quality of life (HRQoL) is significantly impacted in patients with inflammatory bowel disease (IBD). Many studies have assessed HRQoL in patients of all ages, and few focus on the elderly. AIM: To determine the influence of advanced age (> 65 years) and age at diagnosis on patients with IBD. METHODS: This is a retrospective study of prospectively collected data from a single IBD tertiary referral center. Patients had disease activity indices [Harvey-Bradshaw index (HBI), Ulcerative Colitis Disease Activity Index (UCDAI), and Short Inflammatory Bowel Disease Questionnaire (SIBDQ)] recorded during every clinic visit. Three groups of patients: > 65 years, 41-64 years, and < 40 years with > 5 SIBDQ entries were included. Influence of disease type, disease duration, extent of involvement, and comorbidities such as cardiovascular (CV) disease, pulmonary disease, diabetes mellitus (DM), and psychological disorders were noted as confounders. Statistical analysis was performed using ANOVA, Pearson correlation, and logistic regression model. RESULTS: Disease severity indices significantly affected SIBDQ score in both Crohn's disease (CD) and ulcerative colitis (UC) (p < 0.001 for HBI in CD, p < 0.001 UCDAI in UC). Disease extent (p = 0.011) and psychological disorders (p < 0.001) significantly affected SIBDQ score in CD. Chronological age, age at diagnosis, disease duration, number of clinic visits, CV disease, pulmonary disease, and DM were not significant predictors of SIBDQ score (p > 0.05). CONCLUSIONS: HRQoL was negatively influenced by disease extent and psychological disorders in CD but not in UC patients. Advanced age was not a predictor of poor HRQoL in both CD and UC.

Dyssynergic defecation: a treatable cause of persistent symptoms when inflammatory bowel disease is in remission.

BACKGROUND: Introduction of biologic agents in inflammatory bowel disease (IBD) has increased the likelihood of disease remission. Despite resolution of active inflammation, a subset of IBD patients report persistent defecatory symptoms. AIM: To evaluate a group of patients with inflammatory bowel disease with suspected functional defecatory disorders, by use of anorectal manometric testing and subsequent biofeedback therapy. METHODS: A group of IBD patients with persistent defecatory problems despite clinical improvement were included in this study. These patients had no evidence of left-sided disease. Endoscopic and radiographic study findings and timing in relation to the manometry study were recorded. Anorectal manometry was performed by the standard protocol and included rectal sensory assessment, ability to expel a balloon, and pressure dynamics with simulated defecation. RESULTS: Thirty IBD patients (Crohn's 23 patients; ulcerative colitis six patients) presented with defecatory disorders including constipation (67%) increased stooling (10%), and rectal urgency and/or incontinence and rectal pain (6%). All but one patient had anorectal manometric criteria of dyssynergia (presence of anismus motor pattern and inability to expel the balloon). Of the patients who completed biofeedback therapy, 30% had a clinically significant (≥7-point) improvement in SIBDQ score, with a reduction in health-care utilization after a six-month period (p=0.02). CONCLUSIONS: Despite remission, some inflammatory bowel disease patients have persistent defecatory symptoms. Defecatory symptoms may not be predictive of an underlying inflammatory disorder. Lack of inflammatory activity and absence of left-sided disease should prompt investigation of functional disorders. Anorectal manometric testing and biofeedback therapy for patients with a diagnosis of dyssynergia may be a useful therapy.