ABSTRACT
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.
Subject(s)
Hepatolenticular Degeneration , Humans , Adolescent , Young Adult , Adult , Hepatolenticular Degeneration/diagnostic imaging , Magnetoencephalography , Magnetic Resonance ImagingABSTRACT
Identifying surgical patients with obstructive sleep apnoea may assist with anaesthetic management to minimise postoperative complications. Using trial sequential analysis, we evaluated the impact of obstructive sleep apnoea diagnosed by polysomnography or home sleep apnoea testing on postoperative outcomes in surgical patients. Multiple databases were systematically searched. Outcomes included: total postoperative complications, systemic complications (cardiovascular, respiratory, neurological, renal, infectious) and specific complications (atrial fibrillation, myocardial infarction, combined hospital and intensive care unit re-admission, mortality). The pooled odds ratios of postoperative complications were evaluated by the Mantel-Haenszel method random-effects model. Meta-analysis and meta-regression were conducted, and the GRADE approach was used to evaluate the certainty of evidence. Twenty prospective cohort studies with 3756 patients (2127 obstructive sleep apnoea and 1629 non-obstructive sleep apnoea) were included (9 in non-cardiac surgery and 11 in cardiac surgery). Postoperative complications were almost two-fold higher with obstructive sleep apnoea, OR (95%CI) 1.92 (1.52-2.42), p < 0.001; certainty of evidence, moderate. Obstructive sleep apnoea was associated with a 1.5 times increased risk of postoperative cardiovascular complications, OR (95%CI) 1.56 (1.20-2.02), p = 0.001; certainty of evidence, moderate; an almost two-fold increase in respiratory complications, OR (95%CI) 1.91 (1.39-2.62), p < 0.001; certainty of evidence, moderate; and hospital and ICU re-admission, OR (95%CI) 2.25 (1.21-4.19), p = 0.01; certainty of evidence, low. Trial sequential analysis showed adequate information size for postoperative complications. Baseline confounding factors were adjusted by meta-regression, and the sub-group analysis did not materially change our results. This increased risk occurred especially in patients in whom obstructive sleep apnoea had been newly diagnosed, emphasising the importance of pre-operative screening.
Subject(s)
Sleep Apnea, Obstructive , Humans , Polysomnography , Postoperative Complications/epidemiology , Prospective Studies , Sleep , Sleep Apnea, Obstructive/complications , Sleep Apnea, Obstructive/diagnosisABSTRACT
OBJECTIVES: To study the effect of monthly pulses of intravenous methylprednisolone (IVMP) on seizure and global outcomes in children with epileptic encephalopathy (EE). METHODS: This retrospective study was undertaken in a tertiary care epilepsy center in India. Consecutive patients with EE who had received IVMP as adjunctive therapy for a minimum of 3 months and had at least one pre-and post-steroid EEG each, were identified and a structured questionnaire was used to collect information including outcomes at 3 months post-steroid course completion and beyond, as available. RESULTS: Ninety-seven patients (M:F=71:26) fulfilling the inclusion criteria with a mean age at onset of seizures being 20.52 ± 25.69 months were included. Commonest seizure types were myoclonic (66%); Lennaux-Gastaut and West Syndromes accounted for 57 % and 24 % patients respectively. The etiology was unknown in 52 %. All children were on a combination of standard anti-seizure drugs. The duration of IVMP pulse therapy was 7.72 ± 6.25 months. One-fourth (26 %) patients experienced minor adverse events. Greater than 50 % seizure burden reduction was seen in 66 % patients at 3 months with seizure-freedom in 25 %. A total of 45 (46 %) patients became seizure-free in the cohort eventually with continuation of steroids beyond 3 months. Children with idiopathic EEs, normal neuroimaging, myoclonic jerks, and West syndrome showed the best response. The presence of burst-suppression and generalized paroxysmal fast activity (GPFA) predicted inadequate response. CONCLUSIONS: Adjunct pulse doses of IVMP are safe, well-tolerated, and effective in reducing seizures and improving global outcomes in children with idiopathic EEs, West syndrome, normal neuroimaging, and myoclonic jerks. Seizure freedom might be delayed in a subset of these patients, hence duration of therapy beyond 3 months may be warranted.
Subject(s)
Epilepsy , Spasms, Infantile , Child , Electroencephalography , Epilepsy/drug therapy , Humans , Methylprednisolone/therapeutic use , Retrospective Studies , Spasms, Infantile/drug therapyABSTRACT
INTRODUCTION: Excessive cortical synchrony within neural ensembles has been implicated as an important mechanism driving epileptiform activity. The current study measures and compares background electroencephalographic (EEG) phase synchronization in patients having various types of epilepsies and healthy controls during awake and sleep stages. METHODS: A total of 120 patients with epilepsy (PWE) subdivided into 3 groups (juvenile myoclonic epilepsy [JME], temporal lobe epilepsy [TLE], and extra-temporal lobe epilepsy [Ex-TLE]; n = 40 in each group) and 40 healthy controls were subjected to overnight polysomnography. EEG phase synchronization (SI) between the 8 EEG channels was assessed for delta, theta, alpha, sigma, and high beta frequency bands using ensemble measure on 10-second representative time windows and compared between patients and controls and also between awake and sleep stages. Mean ± SD of SI was compared using 2-way analysis of variance followed by pairwise comparison ( P ≤ .05). RESULTS: In both delta and theta bands, the SI was significantly higher in patients with JME, TLE, and Ex-TLE compared with controls, whereas in alpha, sigma, and high beta bands, SI was comparable between the groups. On comparison of SI between sleep stages, delta band: progressive increase in SI from wake â N1 â N2 â N3, whereas REM (rapid eye movement) was comparable to wake; theta band: decreased SI during N2 and increase during N3; alpha band: SI was highest in wake and lower in N1, N2, N3, and REM; and sigma and high beta bands: progressive increase in SI from wake â N1 â N2 â N3; however, sigma band showed lower SI during REM. CONCLUSION: This study found an increased background cortical synchronization in PWE compared with healthy controls in delta and theta bands during wake and sleep. This background hypersynchrony may be an important property of epileptogenic brain circuitry in PWE, which enables them to effortlessly generate a paroxysmal EEG depolarization shift.
Subject(s)
Electroencephalography Phase Synchronization/physiology , Electroencephalography , Myoclonic Epilepsy, Juvenile/physiopathology , Sleep/physiology , Wakefulness/physiology , Adult , Brain/physiopathology , Electroencephalography/methods , Female , Humans , Male , Sleep Stages/physiology , Sleep, REM/physiology , Young AdultABSTRACT
The care of surgical patients with obstructive sleep apnoea (OSA) invokes concerns with safety and liability because of the risk that exists for perioperative death or near-death. The purpose of this review is to analyse the available literature to identify risk factors for perioperative critical complications in patients with OSA. Literature reports were screened for life threatening complications and deaths in surgical patients with OSA. The critical complications were sub-grouped as death/near-death events (death and anoxic brain damage) vs critical respiratory events (CRE)/other events and analysed for various risk factors. Both univariate and multivariate analyses were conducted to identify the potential risk factors.In total, 15 case reports and two medico-legal reports, comprising of 60 total patients with OSA were included in our analysis. Overall, there were 43 deaths or near-death events and 12 critical respiratory events and five other life threatening events. Ten patients (17%) with OSA were undiagnosed before surgery. Only 31% (11/35) were on preoperative continuous positive airway pressure (CPAP), with 36% (4/11) of them continuing CPAP in the postoperative period. The majority of them received a morphine equivalent daily dose less than 10 mg. Eighty percent of the events occurred in the first 24 h and 67% occurred on the general hospital ward.Patients with OSA are at risk of critical complications including death during the initial 24 h after surgery. Morbid obesity, male sex, undiagnosed OSA, partially treated/untreated OSA, opioids, sedatives, and lack of monitoring are risk factors for death or near-death events.
Subject(s)
Hypoxia, Brain/epidemiology , Postoperative Complications/epidemiology , Respiratory Insufficiency/epidemiology , Sleep Apnea, Obstructive/epidemiology , Surgical Procedures, Operative/mortality , Adult , Aged , Causality , Comorbidity , Female , Humans , Male , Middle Aged , Risk FactorsABSTRACT
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.
Subject(s)
Guillain-Barre Syndrome , Aged , Female , Guillain-Barre Syndrome/epidemiology , Guillain-Barre Syndrome/physiopathology , Guillain-Barre Syndrome/therapy , Humans , India/epidemiology , Male , Middle AgedABSTRACT
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.
Subject(s)
Biomarkers , Epilepsy, Temporal Lobe/diagnosis , Epilepsy, Temporal Lobe/physiopathology , Heart Rate/physiology , Sleep Apnea Syndromes/diagnosis , Sleep Apnea Syndromes/physiopathology , Adult , Autonomic Nervous System/drug effects , Autonomic Nervous System/physiopathology , Baroreflex/drug effects , Baroreflex/physiology , Carbamazepine/therapeutic use , Case-Control Studies , Death, Sudden/etiology , Death, Sudden/prevention & control , Epilepsy, Temporal Lobe/drug therapy , Female , Heart Rate/drug effects , Humans , Male , Polysomnography , Primary Dysautonomias/drug therapy , Primary Dysautonomias/physiopathology , Sleep Apnea Syndromes/drug therapy , Young AdultABSTRACT
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.
Subject(s)
Arousal/physiology , Migraine without Aura/diagnosis , Migraine without Aura/physiopathology , Polysomnography/methods , Sleep, REM/physiology , Adult , Alpha Rhythm/physiology , Case-Control Studies , Cross-Sectional Studies , Evoked Potentials , Female , Humans , Male , Reference Values , Theta Rhythm/physiologyABSTRACT
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.
Subject(s)
Anticonvulsants/therapeutic use , Lorazepam/therapeutic use , Status Epilepticus/drug therapy , Adolescent , Adult , Aged , Anticonvulsants/adverse effects , Drug Therapy, Combination , Electroencephalography , Epilepsy, Generalized/drug therapy , Epilepsy, Generalized/physiopathology , Female , Follow-Up Studies , Humans , Injections, Intravenous , Levetiracetam , Lorazepam/adverse effects , Male , Middle Aged , Phenytoin/adverse effects , Phenytoin/therapeutic use , Pilot Projects , Piracetam/adverse effects , Piracetam/analogs & derivatives , Piracetam/therapeutic use , Prognosis , Prospective Studies , Treatment Outcome , Valproic Acid/adverse effects , Valproic Acid/therapeutic use , Young AdultABSTRACT
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.
Subject(s)
Cyclophosphamide/therapeutic use , Immunosuppressive Agents/therapeutic use , Myasthenia Gravis/drug therapy , Adult , Combined Modality Therapy , Cyclophosphamide/administration & dosage , Cyclophosphamide/adverse effects , Drug Evaluation , Drug Substitution , Drug Therapy, Combination , Female , Follow-Up Studies , Gastrointestinal Diseases/chemically induced , Hematologic Diseases/chemically induced , Humans , Immunosuppressive Agents/administration & dosage , Immunosuppressive Agents/adverse effects , Infections/etiology , Kidney Diseases/chemically induced , Male , Middle Aged , Myasthenia Gravis/therapy , Plasma Exchange , Pulse Therapy, Drug , Pyridostigmine Bromide/therapeutic use , Remission Induction , Respiration, Artificial , Retrospective Studies , Thymectomy , Treatment Outcome , Young AdultABSTRACT
We report an unusual occurrence of involuntary movement involving the tongue in a patient with confirmed Wilson's disease (WD). She manifested with slow, hypophonic speech and dysphagia of 4 months duration, associated with pseudobulbar affect, apathy, drooling and dystonia of upper extremities of 1 month duration. Our patient had an uncommon tongue movement which was arrhythmic. There was no feature to suggest tremor, chorea or dystonia. It might be described as athetoid as there was a writhing quality, but of lesser amplitude. Thus, the phenomenology was uncommon in clinical practice and the surface of the tongue was seen to "ripple" like a liquid surface agitated by an object or breeze. Isolated lingual dyskinesias are rare in WD. It is important to evaluate them for WD, a potentially treatable disorder.
ABSTRACT
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.
Subject(s)
Leigh Disease/pathology , Olivary Nucleus/pathology , Child , Cohort Studies , Diagnosis, Differential , Early Diagnosis , Female , Giant Axonal Neuropathy/pathology , Glutarates/urine , Hepatolenticular Degeneration/pathology , Humans , Hypertrophy/pathology , Magnetic Resonance Imaging , Male , Maple Syrup Urine Disease/pathology , Mitochondrial Diseases/pathology , Retrospective Studies , SyndromeABSTRACT
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.
Subject(s)
Cyclophosphamide/therapeutic use , Dermatomyositis/drug therapy , Immunosuppressive Agents/therapeutic use , Polymyositis/drug therapy , Adolescent , Adult , Anti-Inflammatory Agents/therapeutic use , Cohort Studies , Dermatomyositis/complications , Desonide/therapeutic use , Female , Humans , Male , Middle Aged , Polymyositis/complications , Young AdultABSTRACT
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.
Subject(s)
Brain/pathology , Magnetic Resonance Imaging/methods , Neurosyphilis/pathology , Spinal Cord/pathology , Academic Medical Centers , Adult , Female , Humans , Male , Phenotype , Reproducibility of Results , Sensitivity and Specificity , Statistics as Topic , Tertiary Care CentersABSTRACT
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.
Subject(s)
Demyelinating Diseases/pathology , Demyelinating Diseases/therapy , Adolescent , Adult , Aged , Brain/pathology , Child , Child, Preschool , Cohort Studies , Demyelinating Diseases/complications , Female , Humans , Magnetic Resonance Imaging , Male , Middle Aged , Recurrence , Risk Factors , Spinal Cord/pathology , Treatment Outcome , Young AdultABSTRACT
Experimental determination of L fluorescence cross-sections for elements with 45