Insights from Magnetic Evoked Field Analysis in Patients with Wilson's Disease.

Aims: To study the latency, amplitude, and source localization of magnetic evoked field (MEF) responses to visual, auditory, and somatosensory stimuli in Wilson's disease (WD) using magnetoencephalography (MEG) and compare it with "healthy" controls, and correlate the observations with disease severity and brain MRI. Methods: MEF of 28 patients with neurological WD (age: 22.82 ± 5.8 years; M:F = 12:16) and 21 matched controls (age: 25.0 ± 4.6 years; M:F = 10:11) were recorded using MEG. Source localization was performed using standard models on the components of M100, M20, and M100 for visual, somatosensory, and auditory evoked fields, respectively and its latency/amplitude was correlated with disease severity. Results: There were significant differences in source location between control and WD during visual evoked field (VEF) and auditory evoked field (AEF) studies. Latencies of M20 (right-p = 0.02; left-p = 0.04) and M32 (right-p = 0.01) components of SSEF were significantly prolonged. The amplitude of M20 was significantly reduced in patients bilaterally (P = 0.001). There was a trend for the prolonged latency of M100 of VEF in patients (P = 0.09). Five patients had reduced right M145 compared to 8 controls. The left somatosensory evoked fields (SSEF) latency correlated with disease severity (P = 0.04). There was no significant correlation between major components of other MEF with disease severity or MRI score. Conclusions: This study, first of its kind to use MEF analysis in a large cohort of patients with WD, detected subclinical but a variable degree of abnormalities, most consistently of SSEF. It provides valuable insights of functioning and localization of various pathways in a disease known to have protean clinical manifestations and widespread MRI changes.

Molecular modelling as the spark for active learning approaches for interdisciplinary biology teaching.

We present here an interdisciplinary workshop on the subject of biomolecules offered to undergraduate and high school students with the aim of boosting their interest toward all areas of science contributing to the study of life. The workshop involves mathematics, physics, chemistry, computer science and biology. Based on our own areas of research, molecular modelling is chosen as the central axis as it involves all disciplines. To provide a strong biological motivation for the study of the dynamics of biomolecules, the theme of the workshop is the origin of life. All sessions are built around active pedagogy, including games, and a final poster presentation.

Guillain Barre Syndrome in the elderly: Experience from a tertiary-care hospital in India.

Age influences incidence and prognosis of Guillain Barre Syndrome (GBS), common cause of ascending areflexic quadriparesis. Dedicated studies on elderly GBS are infrequent. This study aimed to describe clinical features and outcome at hospital-discharge in patients aged≥60years with GBS. Medical records of 70 elderly GBS over 15years were analysed. Mean symptom-duration was 5.78±4.5days and onset-to-peak 5.14±4.4days. Antecedent events preceded GBS by 8.07±9.9days and included: fever (n=19), respiratory infection (n=6), and gastroenteritis (n=5). Clinical features were weakness of facial (n=34), bulbar (n=13), extraocular (n=4) and respiratory (n=20) muscles and recurrence (n=4). Nine had Hughes disability score (HDS) of three or less. Sensory symptoms and signs included paresthesias (n=40), pain (n=24), and impaired kinaesthetic sensation (n=14). Laboratory abnormalities included albumino-cytological dissociation (n=50), hyponatremia (n=36) and elevated creatine kinase (n=18). Electrophysiological subtypes were: primary demyelinating (n=52), inexcitable (n=3), equivocal (n=2) and axonal (n=1). Fifty-seven patients treated with plasmapheresis (n=48) or intravenous immunoglobulin (n=9) had mean HDS of 3.53±0.7 at discharge. Twenty-one were ambulant (HDS≥3), one had persisting respiratory weakness and one died. Striking differences between the 'elderly' and 100 'adults' seen over 20months were shorter symptom-duration, higher frequency of facial palsy and hyponatremia, lower frequency of pain, lower mean MRC sum score and worse HDS at study-entry and discharge (p<0.05). Requirement for mechanical ventilation and cardiac autonomic dysfunction was higher among elderly (p:0.02). In conclusion, in this cohort of elderly GBS, there was a higher frequency severe GBS and demyelinating electrophysiology.

Lack of heart rate variability during sleep-related apnea in patients with temporal lobe epilepsy (TLE)-an indirect marker of SUDEP?

PURPOSE: Apneas occurring during sleep may precipitate autonomic instability in epilepsy patients making them susceptible to sudden death (SUDEP). Literature on heart rate variability (HRV) during apnea among patients with temporal lobe epilepsy (TLE) is sparse. The aim of this study was to characterize the HRV during the peri-apneic period in patients with TLE and compare with HRV of matched healthy individuals during the overnight polysomnographic (PSG) recording. Further, the role of carbamazepine (CBZ) in modulating peri-apneic HRV in the above cohort was also assessed. METHODS: Twenty patients diagnosed to have TLE (drug naive (n = 10) or on CBZ monotherapy (n = 10)) were compared with ten healthy controls. In both patients and controls, the time domain, frequency domain, and non-linear HRV indices were analyzed for 2 min before and after apnea/hypopnea termination and compared using paired t test (p ≤ 0.05). Additionally, the changes in HRV parameters in the peri-apnea/hypopnea period were compared between the three groups using one-way ANOVA followed by post hoc comparison (p ≤ 0.05). RESULTS: The three study groups were age (p = 0.21) and gender (p = 0.27) matched. In controls (M/F = 5:5; mean age 24.3 ± 5.0 years), there were significant changes in standard deviation of RR interval (SDNN), low frequency (LF) component and long-term HRV (SD2) parameters in the peri-apnea/hypopneic period. Conversely, in drug-naive TLE (M/F = 6:4; mean age: 22.8 ± 4.1 years), all the HRV parameters, including non-linear measures were comparable in the pre- and post-apneic period. On the other hand, patients on CBZ (M/F = 6:4; mean age 20.5 ± 4.8 years) showed significant changes in low-frequency nu (LFnu) and high-frequency nu (HFnu) components in the peri-apnea/hypopneic period. Comparison of the changes in HRV parameters in the peri-apnea/hypopnea period in patients with TLE and controls showed significantly lower changes in drug-naive TLE patients in SDNN, LF, and SD2 as compared to controls. CONCLUSIONS: This study showed that there was a lack of apnea-mediated HRV changes in patients with drug-naive TLE. This might suggest a possible alteration in reflex baroreceptor activation in patients with TLE, predisposing them to SUDEP, and this may be worsened with CBZ.

Combined MEG-EEG source localisation in patients with sub-acute sclerosing pan-encephalitis.

To study the genesis and propagation patterns of periodic complexes (PCs) associated with myoclonic jerks in sub-acute sclerosing pan-encephalitis (SSPE) using magnetoencephalography (MEG) and electroencephalography (EEG). Simultaneous recording of MEG (306 channels) and EEG (64 channels) in five patients of SSPE (M:F = 3:2; age 10.8 ± 3.2 years; symptom-duration 6.2 ± 10 months) was carried out using Elekta Neuromag(®) TRIUX™ system. Qualitative analysis of 80-160 PCs per patient was performed. Ten isomorphic classical PCs with significant field topography per patient were analysed at the 'onset' and at 'earliest significant peak' of the burst using discrete and distributed source imaging methods. MEG background was asymmetrical in 2 and slow in 3 patients. Complexes were periodic (3) or quasi-periodic (2), occurring every 4-16 s and varied in morphology among patients. Mean source localization at onset of bursts using discrete and distributed source imaging in magnetic source imaging (MSI) was in thalami and or insula (50 and 50 %, respectively) and in electric source imaging (ESI) was also in thalami and or insula (38 and 46 %, respectively). Mean source localization at the earliest rising phase of peak in MSI was in peri-central gyrus (49 and 42 %) and in ESI it was in frontal cortex (52 and 56 %). Further analysis revealed that PCs were generated in thalami and or insula and thereafter propagated to anterolateral surface of the cortices (viz. sensori-motor cortex and frontal cortex) to same side as that of the onset. This novel MEG-EEG based case series of PCs provides newer insights for understanding the plausible generators of myoclonus in SSPE and patterns of their propagation.

Neurological and functional recovery in acute transverse myelitis patients with inpatient rehabilitation and magnetic resonance imaging correlates.

OBJECTIVE: The objective of this study was to observe neurological and functional recovery in patients with acute transverse myelitis (ATM) with inpatient rehabilitation and correlate with magnetic resonance imaging (MRI) changes. PATIENTS AND METHODS: The study was conducted with 43 ATM patients (19 males) admitted in the tertiary university research hospital from July 2012 to June 2014. Detailed MRI findings were noted. Neurological status was assessed using the ASIA impairment scale (AIS) and functional recovery was assessed using the Barthel Index score (BI) and Spinal Cord Independence Measure (SCIM). RESULTS: Patients showed significant neurological and functional recovery with inpatient rehabilitation using AIS, BI and SCIM scales when admission and discharge scores were compared (P<0.001). Thirty-one patients (72.1%) had rostral level in the cervical region according to MR imaging, but clinically, 17 patients had tetraplegia, whereas 26 patients had lower-limb weakness only. No definitive pattern or correlation was found between level (MRI or clinical) and neurological status (AIS). CONCLUSION: The neurological outcome in patients with ATM cannot be predicted on the basis of imaging findings. There is a great variation in the imaging level and clinical presentation. Patients show significant improvement with inpatient rehabilitation even with poor functional ability in acute and sub-acute phase of illness.

Study of sleep microstructure in patients of migraine without aura.

PURPOSE: Although the relationship between sleep and migraine has been widely reported, studies on sleep microstructure are few. The aim was to study and compare microstructural polysomnographic characteristics in patients of "migraine without aura" (MOA) with controls. METHODS: Twenty-five patients of MOA and 25 age- and gender-matched healthy controls were subjected to overnight polysomnography. Microstructural sleep analysis, including arousal and cyclic alternating pattern (CAP) analysis was performed. Arousals and CAP parameters were compared between the two groups using the Mann-Whitney U test (p ≤ 0.05). RESULTS: The overall arousal index (p = 0.528) and that during non-rapid eye movement (NREM) sleep (p = 0.503) were comparable between the two groups. However, the arousal index was lower in migraineurs during rapid eye movement (REM) sleep (p = 0.001). The overall CAP rate (p = 0.020) as well as the number of CAP cycles and sequences (p = 0.032) was lower among migraineurs. The total phase A duration (p < 0.0001) was increased, and conversely, phase B duration (p = 0.001) was decreased in migraineurs. The phase A1 duration (p = 0.036) was higher in migraineurs. Finally, phase A1 (p = 0.357) index was comparable, and conversely, A2 (p < 0.0001) and A3 (p = 0.020) indices were decreased in migraineurs. CONCLUSIONS: This study showed a decreased REM arousability as well as a decreased overall CAP rate and CAP cycling in patients with migraine as compared to controls. This indicates that there is probably an alteration of the arousal mechanisms in patients with migraine that may facilitate the occurrence of headache paroxysms during sleep.

Improvement of Diffusion Tensor Imaging (DTI) Parameters with Decoppering Treatment in Wilson's Disease.

OBJECTIVE: 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. METHODS AND RESULTS: 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). CONCLUSION: 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.

Management of generalised convulsive status epilepticus (SE): A prospective randomised controlled study of combined treatment with intravenous lorazepam with either phenytoin, sodium valproate or levetiracetam--Pilot study.

OBJECTIVE: This study was conducted to compare the efficacy of phenytoin, valproate and levetiracetam in patients with GCSE. METHODS: This randomised controlled prospective study was conducted on 150 patients to compare the efficacy of phenytoin (n = 50), valproate (n = 50) and levetiracetam (n = 50) along with lorazepam in patients with GCSE. All recruited patients received i.v. lorazepam (0.1mg/kg) followed by one of the 3 AEDs viz. phenytoin (20 mg/kg), valproate (30 mg/kg), and levetiracetam (25 mg/kg). Those who remained uncontrolled with 1st AED, received the other two AEDs sequentially. Clinical, imaging, EEG, etiological factors were analysed. Predictors of poor seizure control and outcome at discharge and at one month follow-up were assessed. RESULTS: In the phenytoin subgroup, the seizures could be controlled in 34 (68%) with lorazepam+phenytoin infusion. In the valproate subgroup (n = 50), seizures could be controlled in 34 (68%) with lorazepam+valproate infusion. In the levetiracetam subgroup (n = 50), seizures could be controlled in 39 (78%) with lorazepam+levetiracetam infusion. There was no statistically significant difference between the subgroups (p = 0.44). Overall, following lorazepam and 1st AED, 107/150 (71.3%) were controlled; with addition of 2nd AED, 130/150 (86.7%) and by adding 3rd AED, 138/150 (92%) were controlled. Fifteen out of 110 (13.6%) expired within 1 month of SE: phenytoin-6; valproate-4; and levetiracetam-5. Interestingly, 3 patients in the levetiracetam had post-ictal psychosis. SIGNIFICANCE: Phenytoin, valproate, and levetiracetam are safe and equally efficacious following lorazepam in GCSE. The choice of AEDs could be individualised based on co-morbidities. SE could be controlled in 92% of patients with AEDs only and anaesthetics were not required in them.

Long-term efficacy and limitations of cyclophosphamide in myasthenia gravis.

Myasthenia gravis (MG) is a chronic autoimmune disorder with a fluctuating clinical course. The aim of immunotherapy is to bring about long-term remission. We evaluated the safety and efficacy of cyclophosphamide in generalized MG. We also highlight the limitations of cyclophosphamide therapy in inducing long-term remission. Data from 22 patients with generalized MG who received cyclophosphamide therapy were analyzed in terms of its safety and outcome. Twelve patients completed at least six pulses of intravenous cyclophosphamide therapy, and all improved symptomatically at 6 months. At 1 year, only seven patients reported sustained benefit and five had discontinued oral pyridostigmine. During a follow-up period of 56.67 months, all but one patient relapsed and required alternative immunomodulatory therapy. The average time to remission after the initiation of intravenous pulse cyclophosphamide (n=12) was 3.6 months (standard deviation [SD] 1.6 months, range 1-6 months), while the mean duration of remission was 20.3 months (SD 8.8 months, range 12-39 months). Forty-six adverse events were documented in 11 patients over 127 cyclophosphamide pulses. Most of the adverse events were managed symptomatically. In four patients, cyclophosphamide had to be discontinued due to adverse events. Intravenous pulse cyclophosphamide is effective in the management of MG; however remission may be short, necessitating long-term follow-up and alternative immunomodulation. Careful monitoring for adverse events should be mandatory.

Bilateral hypertrophic olivary nucleus degeneration on magnetic resonance imaging in children with Leigh and Leigh-like syndrome.

OBJECTIVE: Bilateral hypertrophic olivary degeneration on brain MRI has been reported in a few metabolic, genetic and neurodegenerative disorders, including mitochondrial disorders. In this report, we sought to analyse whether bilateral symmetrical inferior olivary nucleus hypertrophy is specifically associated with mitochondrial disorders in children. METHODS: This retrospective study included 125 children (mean age, 7.6 ± 5 years; male:female, 2.6:1) diagnosed with various metabolic and genetic disorders during 2005-2012. The routine MRI sequences (T1 weighted, T2 weighted and fluid-attenuated inversion-recovery sequences) were analysed for the presence of bilateral symmetrical olivary hypertrophy and central tegmental tract or dentate nuclei signal changes. The other imaging findings and the final diagnoses were noted. RESULTS: The cohort included patients with Leigh and Leigh-like syndrome (n = 25), other mitochondrial diseases (n = 25), Wilson disease (n = 40), Type 1 glutaric aciduria (n = 14), maple syrup urine disease (n = 13), giant axonal neuropathy (n = 5) and L-2 hydroxy glutaric aciduria (n = 3). Bilateral inferior olivary nucleus hypertrophy was noted in 10 patients, all of whom belonged to the Leigh and Leigh-like syndrome group. CONCLUSION: Bilateral hypertrophic olivary degeneration on MRI is relatively often, but not routinely, seen in children with Leigh and Leigh-like syndrome. Early detection of this finding by radiologists and physicians may facilitate targeted metabolic testing in these children. ADVANCES IN KNOWLEDGE: This article highlights the occurrence of bilateral hypertrophic olivary nucleus degeneration on MRI in children with Leigh and Leigh-like syndrome, compared with other metabolic disorders.

Yoga and epilepsy: What do patients perceive?

CONTEXT: Benefit of yoga therapy in the management of epilepsy is emerging. However, there is no data available about the knowledge, attitude and practice (KAP) of yoga amongst people living with epilepsy (PLWE). AIMS: This study was designed to explore the KAP about yoga among PLWE. SETTINGS AND DESIGN: The study was conducted on 300 PLWE attending the neurology out-patient services of a tertiary care hospital. METHODOLOGY: Three hundred PLWE (male:female=173:127; age: 31.6±12.4 years) attending the neurology out-patient services of a neuropsychiatry hospital were administered a pre-tested KAP questionnaire. RESULTS: About 87.4% were on regular anti-epileptic drugs and half (50.3%) on monotherapy. Use of complementary and alternative medicine by the respondents included: Ayurveda (26.7%), yoga (25.6%) and homeopathy (16.3%) or folk medicine (29.1%). Nearly 33.7% of the respondents reported that yoga is beneficial in managing epilepsy. More than half the respondents (54.8%) were willing to practice yoga. Those who practiced yoga opined that regular practice of yoga might reduce dosage of medication (62.8%), their side effects (51.3%) and frequency of seizures (54.5%). Majority of the patients were willing to practice yoga, if yoga services were offered. CONCLUSION: The gaps in KAP identified in this study point to the need for more systematic effort to bring about awareness of yoga in patients with epilepsy.

Efficacy and limitations of pulse cyclophosphamide therapy in polymyositis and dermatomyositis.

OBJECTIVES: To assess the therapeutic response of intravenous (IV) pulse cyclophosphamide therapy in polymyositis and dermatomyositis. METHODS: Data of 9 patients (M:F = 2:7) who received IV pulse cyclophosphamide therapy were retrospectively analyzed. RESULTS: The mean symptom duration was 11.33 ± 10.6 months (range, 2-34 months). The cohort comprised (1) primary idiopathic polymyositis (n = 1), (2) primary idiopathic dermatomyositis (n = 1), (3) childhood type associated with vasculitis (n = 1), and (4) associated with collagen vascular disease (n = 6). All patients improved and became clinically asymptomatic after a mean period of 12.33 ± 6.5 months (range, 4-24 months); 5 remained asymptomatic at the end of a median follow-up period of 22 months. All patients received concomitant steroid therapy, and in 6, steroids could be tapered after the initiation of IV pulse cyclophosphamide therapy. CONCLUSIONS: In this cohort of polymyositis/dermatomyositis, treatment with IV pulse cyclophosphamide was associated with improvement; the therapeutic response was sustained in majority of the patients.

Possible significance of anti-heat shock protein (HSP-65) antibodies in autoimmune myasthenia gravis.

Myasthenia gravis (MG) is an autoimmune disorder caused by antibody-mediated attack against skeletal muscle nicotinic acetylcholine receptor (AchR) at neuromuscular junction. A close relationship exists between heat shock proteins (HSPs) and numerous autoimmune diseases. HSPs are over expressed to protect the host against various insults. Antibodies to HSP-65 (IgG, IgA, IgM) are evaluated in 40 MG sera by ELISA. About 80% of MG cases showed anti-HSP antibodies (62.5% IgG, 55% IgA, 40% IgM). The result suggests that, expression of HSP-65 increases in MG and most individuals produce antibodies to it. These antibodies might play a significant role in the pathogenesis of MG.

Subcortical structural abnormalities in juvenile myoclonic epilepsy (JME): MR volumetry and vertex based analysis.

PURPOSE: Imaging studies in juvenile myoclonic epilepsy (JME) have shown abnormalities of the thalamus and frontal cortex. The purpose of this study was to systematically investigate the morphological changes in the deep gray matter (GM) structures using techniques of voxel based morphometry (VBM), MR volumetry and shape analysis. METHODOLOGY: The study included 40 patients with JME (M:F=21:19; age 22.8±5.3 years) and 19 matched controls (M:F=13:6; age 24.5±4.2 years). All subjects underwent MRI using standard protocol that included T1-3D TFE (Turbo Field Echo) images with 1mm thickness. VBM analysis and MR volumetry were performed. The volumes of deep subcortical GM structures were extracted and vertex-wise shape analysis was performed using FSL-FIRST (FSL-Integrated Registration and Segmentation Toolbox) software. RESULTS: VBM analysis with a thalamic mask revealed focal thalamic alterations in the anteromedial aspect of the thalamus (p<0.05, false discovery rate (FDR) corrected) which remained significant after adjusting for age, gender and intracranial volume (ICV). Significant volume loss was noted in both the thalami. Vertex-wise shape analysis showed significant focal surface reductions in the thalami bilaterally in patients that were predominantly seen in the medial as well as lateral aspects of the thalamus (p<0.05, FDR corrected). The disease duration correlated with left hippocampus volume while age of onset correlated with right hippocampus volume. CONCLUSIONS: This study confirms the presence of thalamic alterations in patients with JME. Shape analysis technique provided complementary information and disclosed the presence of focal atrophic changes in patients' thalami. The striatum and hippocampus did not show any significant alterations.

Neurosyphilis: MRI features and their phenotypic correlation in a cohort of 35 patients from a tertiary care university hospital.

INTRODUCTION: The clinical and MR imaging features of neurosyphilis are highly varied. In this study, we describe the spectrum of the imaging findings in patients with neurosyphilis. METHODS: The MR imaging observations of 35 patients diagnosed to have neurosyphilis on the basis of cerebrospinal fluid reactive for the Venereal Disease Research Laboratory test were reviewed. RESULTS: All the 35 patients, including four with human immunodeficiency virus coinfection, met the CDC diagnostic criteria for neurosyphilis. Patients were classified into three groups: (1) neuropsychiatric, (2) meningovascular, and (3) myelopathic, based on the dominant clinical manifestations. Fourteen patients with neuropsychiatric manifestations showed diffuse cerebral atrophy (14), parenchymal signal changes in the mesial temporal region (2) and temporal and basifrontal regions (1), infarcts (3), and nonspecific white matter changes (3). Eleven patients with meningovascular form showed infarcts (6), diffuse cerebral atrophy (3), signal changes in the mesial temporal region (3), sulcal exudates (1), progressive multifocal leukoencephalopathy (1), and a mass surrounding the carotid sheath (1). Spine imaging in ten patients with myelopathy showed long-segment signal changes (5), contrast enhancement (2), and dorsal column involvement (2). Three of these patients had normal spinal study. Six patients in the myelopathic group also underwent brain MRI that showed signal changes in the temporal region (2) and frontal region (1), multiple infarcts (1), and enhancing hypothalami (1). Three patients had normal study. CONCLUSION: MRI abnormalities in neurosyphilis are protean and mimic of many other neurological disorders and thus require a high index of suspicion to reduce diagnostic omissions.

Tumefactive demyelination: clinical, imaging and follow-up observations in thirty-nine patients.

PURPOSE: We describe the clinical, neuroimaging and pathological features and therapeutic outcome in a large cohort of 39 patients with tumefactive demyelination. MATERIALS AND METHODS: A retrospective audit of 39 patients with 'tumefactive demyelination' was performed. The demographic, clinical, MR imaging and pathological details were reviewed. RESULTS: The clinical course was monophasic (n = 22) or relapsing-remitting (n = 17). Common neurological manifestations at presentation included hemiparesis - 27; ataxia - 11; vomiting - 10; headache -9; ophthalmoplegia - 7; seizure - 5; impaired vision - 4; aphasia - 4; visual field defects - 3; papilloedema - 5; extrapyramidal - 5; intellectual decline - 5; behavioural disturbances - 3; altered sensorium - 5. MRI revealed fronto-parietal lesions, which were isolated in 14 (36%) patients. Moderate perilesional oedema and/or mass effect was noted in 12 (30.8%) patients. Post-contrast MR sequences revealed partial ring enhancement in 15, complete ring in seven, patchy enhancement in six, uniform enhancement in two and lack of enhancement in nine cases. Clinical and MR characteristics did not help distinguish between monophasic and relapsing-remitting subgroups. In the monophasic group, 53.8% had complete recovery, while 38.5% had partial improvement (follow-up duration, 8.31 ± 9.3 months). In the relapsing-remitting subgroup, the median time to relapse was 4 months (n = 12, follow-up, 37.8 ± 39.4 months). Patients with monophasic course or single relapse received steroids. Patients with more than one relapse received cyclophosphamide (2), mycophenolate (1), azathioprine (1) or methotrexate (1). CONCLUSIONS: A high proportion of cases of tumefactive demyelination follow a relapsing course, thus necessitating a long-term follow-up. MRI, although helpful in diagnosis, does not predict monophasic or relapsing-remitting course. Guidelines for the management of acute episodes and prevention of relapses are required.

Prevalence and profile of sleep disturbances in Guillain-Barre Syndrome: a prospective questionnaire-based study during 10 days of hospitalization.

INTRODUCTION: Sleep disturbances in Guillain-Barre Syndrome (GBS), though common, have not received focused attention. OBJECTIVES: To study frequency and nature of sleep disturbances in patients with GBS, using validated questionnaires, and analyze the contributing factors. MATERIALS AND METHODS: 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. OBSERVATIONS: 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. CONCLUSIONS: 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.