Consecutive new cases were screened to identify those referred by other specialties. The referred cases were compared with a randomly selected control group from the non referred cases. Referred cases constituted 16.4% of all the clinic new cases but the demographic characteristics were similar to those of the other clinic cases. Functional somatic complaints were significantly more often (p less than 0.01) reported by the referred cases. Depressive and anxiety symptoms were also significantly more often observed in the referred group (p less than 0.01). Most of the referred cases had identifiable psychiatric problems which could be managed in an out-patient setting. Studies on referral patterns and modes of clinical presentation are useful in planning and organising consultation liaison services in general and specialist centres.
Wilson's disease (WD) has neuro-anatomical, pathophysiological and neurochemical basis for sleep disturbances. The aim of the study was objective evaluation of the frequency and nature of sleep abnormalities using polysomnography (PSG) in patients with WD. The study included 25 subjects with WD (males, 18; age , 24.4 ± 9.25 years) and 25 healthy controls (all males; age, 33.1 ± 9.7 years). After phenotypic assessment and magnetic resonance imaging (MRI), sleep-related questionnaires were administered, and PSG was performed. Patients had significantly reduced total sleep-time (P=.001), sleep-efficiency (P=.001), percentage of deep sleep (P=.01), and REM-sleep (P=.04) with prolonged sleep-onset latency (P=.05) and latency to stage 2 (P=.02). Subgroup analyses of patients based on demographic and clinical parameters were done. Men had significantly more bradycardia both during awake (P=.002) and sleep (P=.03) states. Younger patients (<20 years) had frequent tachycardia (P=.01), higher Periodic Limb Movement (PLM) Index (P=.01) and lesser REM% sleep (P=.05). Patients on de-coppering therapy had prolonged REM-sleep-onset latency (P=.03) and mixed apnea events (P=.04). The isolated limb movements were more in the severe form of disease (P=.05) and in patients taking anticonvulsants (P=.03). This study, the first of its kind in literature, revealed significant sleep disturbances in patients with Wilson's disease.
Sleep disturbances in Guillain-Barre Syndrome (GBS), though common, have not received focused attention.To study frequency and nature of sleep disturbances in patients with GBS, using validated questionnaires, and analyze the contributing factors.This prospective study included 60 patients fulfilling National Institute of Neurological and Communicative Diseases and Stroke (NINCDS) criteria for GBS (mean age: 32.7 ± 12.9 years; median: 30 years; M:F = 46:14), evaluated from 2008 to 2010. Data regarding sleep were collected on 10 consecutive days following admission using Richard Campbell Sleep score, St Mary's Hospital Sleep Questionnaire, and Pittsburgh Sleep Quality Index (PSQI) and correlated with various possible contributing factors like pain, paresthesia, anxiety, depression, autonomic dysfunctions, severity of disease, and therapeutic interventions among others.Qualitative and quantitative sleep disturbances were rather frequent and involved over 50% patients: abnormal PSQI - 13.3%, abnormal score on Richard scale - 51.6%, abnormal sleep onset latency - 35%, sleep fragmentation - 40%, and reduced sleep duration - 46.6%. The symptoms were severe during the first week of hospitalization and reduced thereafter. Sleep disturbances as scored on Richard scale significantly correlated with anxiety, pain, paresthesia, and severity of immobility (P < 0.05) but not with depression and use of analgesics or antineuritic drugs.This study first of its kind suggests that sleep disturbance in GBS is frequent, multi-factorial, often disturbing, and varies during the course of illness. Routine enquiry into the sleep disturbances and timely intervention may reduce morbidity and improve their quality of life.
This study was undertaken to analyse serially the effects of decoppering therapy on the clinical features, disability and MRI brain including DTI metrics in patients with Wilson's disease.Thirty-five patients with clinically and serologically confirmed neuropsychiatric form of Wilson's disease (WD) on decoppering therapy were followed for a minimum duration of 1 year with serial assessment of their clinical features, disability status and serial MR imaging of the brain including DTI. The cohort included 18 treatment-naïve patients and 17 patients already on decoppering therapy (M/F = 2.18:1). The mean age at which they underwent baseline assessment for this study was 18.6 ± 7.6 years, and follow-up assessment was done after a mean duration of 23.5 ± 8.8 months (range, 12 to 45 months). Along with the overall clinical improvement noted at follow-up, the disability assessed using Chu staging and MSEADL showed significant reduction in the number of patients with severe disability and the mean NSS reducing from 9.74 to 6.37 (p = 0.002). The mean MRI scores showed significantly reduced disease burden from a baseline score of 5.9 (±4.2) to 4.9 (±4.7) in follow-up scans (p < 0.05). Voxel-wise comparison of serial DTI metrics on TBSS (tract-based spatial statistics) analysis showed that the entire cohort had significant (p < 0.05) improvement in all the four parameters (MD, FA, DA and RD) indicated by a decrease in MD, DA and RD values and increase in FA values. Comparison of whole-brain white matter DTI measures between pre- and posttreatment did not show any significant difference (p < 0.05).Patients with Wilson's disease on decoppering therapy showed clinical improvement accompanied with improvement in DTI metrics. Quantitative DTI metrics may be used as surrogate markers of clinical status following initiation of medical therapy in Wilson's disease.
A screening test for detecting abnormal illness behaviour in patients with somatic symptoms is described here along with its psychometric properties. This screening version of the Illness Behaviour Questionnaire (SIBQ) is derived from two subscales of the Illness Behaviour Questionnaire and has 11 items. The study was carried out on 78 consecutive patients with prominent somatic complaints and 22 normal volunteers. The SIBQ was administered to the subjects and the sensitivity, specificity and hits positive rates were computed for different cut-off scores. A score of 7 and above gave a sensitivity of 86%, a specificity of 83% and a hits-positive rate of 0.7537, and this seems the optimal cut-off score. The SIBQ may prove to be a sensitive screening instrument to detect probable cases of abnormal illness behaviour and especially useful for busy clinics or centres.
Background: Myasthenia gravis (MG) is an autoimmune disorder with a chronic fluctuating course. Quality of life (QoL) is affected by physical restrictions due to disease-related symptoms and effects of long-term treatment.Purpose: The purpose of this study was to assess QoL in a cohort of MG with stable disease course on optimal therapy.Materials and Methods: MG-QoL-15 was applied prospectively to 50 subjects of MG from India.Results: Mean MG-QoL-15 was 10.34 (standard deviation: 9.4; range: 0–32). Mean MG-QoL-15 scores for subjects with Myasthenia Gravis Foundation of America (MGFA) grades I, II, and III/IV were 3.54, 9.4, and 15.94, respectively. QoL scores correlated significantly with the MGFA grade. Age, gender, thymectomized status, thymoma, and steroid therapy did not affect QoL scores. All patients with MGFA grade I scored "0" or "1" in almost all items of MG-QoL-15. Seven and 11 patients with MGFA grades III/IV reported a significant affection (scores "3" or "4") due to "trouble using my eyes" and "plan around MG," respectively; and five subjects were "frustrated by MG." None of the subjects, irrespective of their MGFA grade, reported significant difficulty in getting around public spaces due to MG, or had "trouble in performing personal grooming." One subject each reported significant "trouble driving due to MG" or felt that "MG limits ability to enjoy hobbies and fun activities."Conclusion: This is the first study from India to assess QoL in MG using MG-QoL-15. Increased disease severity was reflected in worse QoL. MG-QoL-15 is a simple, quick, and user-friendly tool. Longitudinal changes in the QoL scores may be required to determine its utility in the Indian context.
Background: Respiratory muscle paralysis is a serious complication of Guillain–Barre syndrome (GBS). Factors that govern duration and recovery from respiratory paralysis are unclear.Aim: To correlate electrophysiological parameters in critically ill GBS with duration of mechanical ventilation and outcome at discharge.Materials and Methods: Data of a large cohort (n=93; M:F 59:34; mean age: 33.51+21.4 years) of critically-ill patients with GBS seen over one decade was retrospectively analyzed.Results: The duration of mechanical ventilation was <15 days (n = 38), 16–30 days (n = 24), and >30 days (n = 31). Majority of the patients had a demyelinating electrophysiology. Reduced amplitude or absent motor potentials correlated with requirement for longer duration of ventilation. Inexcitable sensory nerves were more common in patients who could be weaned off from the ventilator within 15 days. There was no relation between the conduction blocks in motor nerves and the duration of ventilation. Low amplitude of median nerve correlated with a poor outcome at hospital discharge as assessed by Hughes disability scale.Conclusion: Distinct patterns of electrophysiological abnormalities are noted in patients and they correlate with the duration of mechanical ventilation. Future studies to unravel the underlying pathophysiological processes that govern the patterns of progression and recovery in the critically ill patients with GBS will pave way for the development of better and more potent therapies that will hasten recovery, when combined with the prevalent treatment modalities including plasmapheresis and intravenous immunoglobulin.
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