Presence Of Brainstem Lesions Research Articles

Clinical significance and prognostic value of serum autoantibody tests in multiple sclerosis.

It is known that multiple sclerosis (MS) often coexists with other autoimmune diseases. Hence, autoantibody (auto-Ab) tests may prove useful in the differential diagnosis of MS. The objectives of this study were to: (a) investigate the prevalence of auto-Ab positivity at the beginning of the MS diagnostic process; (b) assess whether Auto-Ab+ and Auto-Ab- patients differ in baseline clinical, laboratory, and radiological parameters; and (c) investigate the prognostic value during a two-year follow-up period. This retrospective study consisted of 450 patients aged between 18 and 55 years. All patients underwent a wide range of auto-Ab tests, anti-nuclear antibody (ANA) tests in particular. The expanded disability status scale (EDSS) scores of the patients were recorded at the time of diagnosis and at the end of a two-year follow-up period. The mean age of the 212 patients, 148 (69.8%) female and 64 (30.2%) male, included in the study sample was 37 ± 10.83 years. The rate of relapsing cases was 84% (178). Oligoclonal band (OCB) was positive in 142 (86.6%) of the 164 tested cases. At least one of the auto-Ab tests was positive in 51 (24.1%) of the cases. ANA test was positive in 21 (9.9%) cases. There was no significant difference between patients with at least one positive auto-Ab test and without any positive auto-Ab test and between ANA-positive and ANA-negative patients in terms of age, gender, clinical features of MS, presence of brain stem lesion, presence of spinal lesion, OCB positivity, level of clinical improvement after the first pulse steroid treatment, family history, presence of comorbidity, presence of autoimmune disease, or EDSS scores recorded at the end of the two-year follow-up period (p > 0.05). Our study findings revealed that Auto-Ab positivity was more common in MS patients than in the general population. However, given their limited contribution to the diagnosis and differential diagnosis of MS with no effect on the prognostic process, auto-Ab tests should be requested only in the event of accompanying autoimmune disease symptoms, and in cases where the diagnosis of MS may be suspected.

Disease Activity and Progression of Disability in Multiple Sclerosis Patients Aged Over 50 With or Without Disease-Modifying Drug Treatment: A Retrospective Cohort Study.

Background This study aimed to clarify the need for disease-modifying drug (DMD) treatment in elderly patients with multiple sclerosis (MS) aged 50 years or older. MS is an autoimmune, demyelinating disease of the central nervous system that predominantly affects young women. Various DMDs are effective in preventing relapses and slowing the progression of disability in patients with MS. Although disease activity in MS is believed to decrease with aging, a consensus on the appropriate DMD treatment for elderly patients with MS is lacking. Methodology This study included elderly patients with MS (>50 years old). We compared the occurrence of relapses, worsening of disability, and conversion to secondary progressive MS (SPMS) between patients with DMD treatment and those without. Logistic regression analysis was performed to determine the predictors of these outcomes. Confounding factors were adjusted using propensity scores. Results From January 1991 to October 2022, 76 elderly patients with MS were included. The mean age at the last visit was 57.4 ± 6.3 years, with 51 patients being female. The mean age of onset of MS was 37.1 ± 10.1 years. Fifty-four patients were included in the DMD treatment group. The overall relapse rate was 38% (33% and 48% in the DMD treatment and untreated groups, respectively). No significant differences in relapse rates (p = 0.72) or in the Expanded Disability Status Scale (EDSS) scores were identified between the two groups. Kaplan-Meier curves showed no differences in the time to first relapse within five years between the two groups. Additionally, no significant predictors of relapse were identified. Among 61 patients with relapsing-remitting MS, 25% converted to SPMS during the observation period. Logistic regression analysis showed that older age at the final visit and the presence of brainstem lesions at the age of 50 years were associated with a higher rate of transition to SPMS. Conclusions In the present study, no significant difference was found in the rate of relapse, disability progression, and conversion to SPMS between the DMD treatment and untreated groups in elderly patients with MS. Therefore, in patients without long-term relapse, no poor prognostic functional factorsor predictors of conversion to SPMS, discontinuation of DMDs may beconsidered. In addition, the presence of brainstem lesions at 50 years of age may predict the conversion to SPMS. Thus, the continuation of DMD or conversion to an appropriate DMD should be considered in patients with brainstem lesions at 50 years of age.

The Interaural Time Difference for High-Pass Filtered Noise and Its Relationship With Brainstem Dysfunction and Disability in Multiple Sclerosis.

Just noticeable difference for interaural time difference (JND-ITD) is a sensitive test to detect silent lesions and neural asynchrony along the auditory pathways among individuals with multiple sclerosis (MS), but it has not been studied with brainstem functional system scores (BFSS) and expanded disability status scale (EDSS). The study aims to assess the usefulness of JND-ITD thresholds in individuals with MS and relate to brainstem magnetic resonance imaging (MRI) lesions, BFSS, and disability (EDSS). Standard group comparison design was adapted to compare the JND-ITD thresholds between individuals with MS (n = 45) and age and gender-matched healthy participants (n = 45). All participants underwent case history, neurological examination including BFSS and EDSS scoring, MRI brain imaging, minimental state examination, routine audiological evaluation, and ITD testing for high-pass filtered noise stimuli. Of the 36 MS participants with abnormal JND-ITD thresholds, 22 (48.9%) participants could not identify maximum JND-ITD values (1,280 μs) in the ITD task. Abnormal JND-ITDs thresholds (139-1,280 μs) were obtained in 14 (31.11%) participants with MS. The JND-ITD thresholds were significantly different between the healthy and MS group. No significant association was found between the presence of ITD abnormality with the presence of brainstem lesions (MRI) and brainstem dysfunction (BFSS). Also, this study did not find any relationship between JND-ITD thresholds with disability (EDSS). This study supports the findings that JND-ITD for high-pass filtered noise is a sensitive test to detect lesions along the auditory system. Even though JND-ITD thresholds did not relate with BFSS and EDSS scores, JND-ITD abnormalities can be of great value in identifying lesions along the auditory system, especially in the early stages of MS, when clinical neurological examination does not show any signs of brainstem dysfunction, disability, and MRI without any lesions in the brain.

Brainstem lesions are associated with diffuse spinal cord involvement in early multiple sclerosis

BackgroundEarly infratentorial and focal spinal cord lesions on magnetic resonance imaging (MRI) are associated with a higher risk of long-term disability in patients with multiple sclerosis (MS). The role of diffuse spinal cord lesions remains less understood. The purpose of this study was to evaluate focal and especially diffuse spinal cord lesions in patients with early relapsing-remitting MS and their association with intracranial lesion topography, global and regional brain volume, and spinal cord volume.MethodsWe investigated 58 MS patients with short disease duration (< 5 years) from a large academic MS center and 58 healthy controls matched for age and sex. Brain, spinal cord, and intracranial lesion volumes were compared among patients with- and without diffuse spinal cord lesions and controls. Binary logistic regression models were used to analyse the association between the volume and topology of intracranial lesions and the presence of focal and diffuse spinal cord lesions.ResultsWe found spinal cord involvement in 75% of the patients (43/58), including diffuse changes in 41.4% (24/58). Patients with diffuse spinal cord changes exhibited higher volumes of brainstem lesion volume (p = 0.008). The presence of at least one brainstem lesion was associated with a higher probability of the presence of diffuse spinal cord lesions (odds ratio 47.1; 95% confidence interval 6.9–321.6 p < 0.001) as opposed to focal spinal cord lesions (odds ratio 0.22; p = 0.320). Patients with diffuse spinal cord lesions had a lower thalamus volume compared to patients without diffuse spinal cord lesions (p = 0.007) or healthy controls (p = 0.002).ConclusionsDiffuse spinal cord lesions are associated with the presence of brainstem lesions and with a lower volume of the thalamus. This association was not found in patients with focal spinal cord lesions. If confirmed, thalamic atrophy in patients with diffuse lesions could increase our knowledge on the worse prognosis in patients with infratentorial and SC lesions.

Sleep-Related Breathing Disorders in Multiple Sclerosis: Prevalence, Features and Associated Factors.

BackgroundMultiple sclerosis (MS) represents a risk factor for sleep disorders, but there are conflicting results about the prevalence and severity of sleep-related breathing disorders (SRBD) in MS. Most available data come from self-administered questionnaires.ObjectiveTo conduct a polysomnographic study in MS focused on SRBD, compared to a group of healthy controls (HC), also considering the neuroimaging findings. To evaluate the impact of SRBD on vigilance, fatigue and depression in MS.MethodsIn this cross-sectional, observational, instrumental study, 67 MS patients (men/women: 20/47; mean age: 50.6±8.2 years) underwent PSG and maintenance of wakefulness test. Findings were compared to 67 age-, sex-, BMI-matched HC, by using parametric (Student’s t-test) and nonparametric statistics (chi-squared test). A subgroup analysis was then performed, evaluating the influence of brainstem (mesencephalic, pontine and medullary) lesions at neuroimaging on instrumental and clinical data: MS patients with at least one brainstem lesion vs MS patients without vs HC.ResultsThe frequency of SRBD was comparable in MS patients and HC. No MS patient had a central apnea index ≥2/h. The respiratory disturbance index (RDI) did not correlate to clinical parameters such as fatigue and depression. Patients with MS were drowsier than HC (47% vs 26%, p = 0.019) and showed a worse sleep pattern, in terms of duration, efficiency and architecture.ConclusionOur study does not provide evidence of an association between MS-specific symptoms such as fatigue, sleepiness, depression and central or obstructive apneas, even in the presence of brainstem lesions.

Early Predictors of 9-Year Disability in Pediatric Multiple Sclerosis.

The purpose of this study was to assess early predictors of 9-year disability in pediatric patients with multiple sclerosis. Clinical and magnetic resonance imaging (MRI) assessments of 123 pediatric patients with multiple sclerosis were obtained at disease onset and after 1 and 2 years. A 9-year clinical follow-up was also performed. Cox proportional hazard and multivariable regression models were used to assess independent predictors of time to first relapse and 9-year outcomes. Time to first relapse was predicted by optic nerve lesions (hazard ratio [HR] = 2.10, p = 0.02) and high-efficacy treatment exposure (HR = 0.31, p = 0.005). Predictors of annualized relapse rate were: at baseline, presence of cerebellar (β = -0.15, p < 0.001), cervical cord lesions (β = 0.16, p = 0.003), and high-efficacy treatment exposure (β = -0.14, p = 0.01); considering also 1-year variables, number of relapses (β = 0.14, p = 0.002), and the previous baseline predictors; considering 2-year variables, time to first relapse (2-year: β = -0.12, p = 0.01) entered, whereas high-efficacy treatment exposure exited the model. Predictors of 9-year disability worsening were: at baseline, presence of optic nerve lesions (odds ratio [OR] = 6.45, p = 0.01); considering 1-year and 2-year variables, Expanded Disability Status Scale (EDSS) changes (1-year: OR = 26.05, p < 0.001; 2-year: OR = 16.38, p = 0.02), and ≥ 2 new T2-lesions in 2 years (2-year: OR = 4.91, p = 0.02). Predictors of higher 9-year EDSS score were: at baseline, EDSS score (β = 0.58, p < 0.001), presence of brainstem lesions (β = 0.31, p = 0.04), and number of cervical cord lesions (β = 0.22, p = 0.05); considering 1-year and 2-year variables, EDSS changes (1-year: β = 0.79, p < 0.001; 2-year: β = 0.55, p < 0.001), and ≥ 2 new T2-lesions (1-year: β = 0.28, p = 0.03; 2-year: β = 0.35, p = 0.01). A complete baseline MRI assessment and an accurate clinical and MRI monitoring during the first 2 years of disease contribute to predict 9-year prognosis in pediatric patients with multiple sclerosis. ANN NEUROL 2021;89:1011-1022.

Treatment Timing, EEG, Neuroimaging, and Outcomes After Acute Necrotizing Encephalopathy in Children.

Acute necrotizing encephalopathy (ANE) is a rare condition associated with rapid progression to coma and high incidence of morbidity and mortality. Clinical, electroencephalographic (EEG), and brain magnetic resonance imaging (MRI) characteristics and immunomodulatory therapy timing were retrospectively analyzed in children with ANE. ANE severity scores (ANE-SS) and MRI scores were also assessed. The associations of patient characteristics with 6-month modified Rankin scale (mRS) and length of hospitalization were determined using either univariate linear regression or one-way analysis of variance. 7 children were retrospectively evaluated. Normal EEG sleep spindles (P = .024) and early treatment (R2 = .57, P = .030) were associated with improved outcomes (ie, decreased mRS). Higher ANE-SS (R2 = .79, P = .011), higher age (R2 = .62, P = .038), and presence of brainstem lesions (P = .015) were associated with longer length of hospitalization. Other patient characteristics were not significantly associated with mRS or length of hospitalization. Early immunomodulatory therapy and normal sleep spindles are associated with better functional outcome in children with ANE.

Risk of obstructive sleep apnea in multiple sclerosis: Frequency, clinical and radiological correlates

BackgroundPrimary sleep disorder, especially, obstructive sleep apnea (OSA), are noted to occur in MS patients at higher frequency than the general population. ObjectivesOur objectives are: (1) To assess the frequency of OSA risk among MS patients. (2) To evaluate the relationship between OSA risk and self-reported fatigue and sleepiness. (3) To evaluate the relationship between OSA risk and clinical disability, radiological findings and treatment status. MethodsWe enrolled 124 patients with multiple sclerosis, there were 53 men and 71 women with mean age (31.12 ± 7.48) All participants completed questionnaires: STOP-Bang, Berlin, Fatigue Severity Scale (FSS) and Epworth sleepiness scale (ESS) and their disability was assessed using Expanded Disability Status Scale (EDSS). ResultsAmong the completed surveys, 46.8% screened as high risk for OSA based on STOP-BANG questionnaire, and 45.2% based on Berlin questionnaire. About 64.5% of subjects screened positive for fatigue using FSS and 38.7% reported excessive day time sleepiness. Comparing MS patients diagnosed with high risk of OSA and those without, there was significant difference between the two groups regarding age (P < 0.001), gender (P < 0.001), disease duration, (P = 0.04), presence of brainstem lesions (0.04) and disease modifying therapy (DMT) use (P = 0.002). ESS and FSS positive scores were each significantly correlated with positive STOP BANG and Berlin outcomes (p < 0.001). EDSS showed significant correlation with positive STOP-Bang and Berlin scores. ConclusionsOSA risk appears to be high in MS patients with increased risk of fatigue and increasing disability.

Supratentorial multiple sclerosis lesions affect the blink reflex test.

IntroductionThe Blink Reflex Test (BRT) is a neurophysiological examination used for evaluation of brainstem reflex circuits. MRI is the most precise modality for evaluation of MS lesion anatomy. Our study objective was to investigate how the functional results of the neurophysiological BRT relate to the anatomy of MS lesions in routine MRI studies.Methods65 MS patients underwent the BRT within 2 months of a brain MRI showing demyelinating lesions.Results The overall sensitivity of the BRT was 90.8%, while in patients with at least one brainstem lesion and no brainstem lesions it was 91.4% and 90%, respectively.Discussion The presence of brainstem lesions does not significantly affect BRT sensitivity. This points to the influence of supratentorial MS lesions on the BRT. Gender, age, disease duration, type of MS, acuteness of an MS event and whether MS diagnosis was recent or not were not variables affecting the results.

Acute autonomic dysfunction contralateral to acute strokes: a prospective study of 100 consecutive cases

Complex painful reflex syndrome is sometimes described in the chronic phase of stroke. Acute autonomic dysfunction (AAD), which is occasionally present in cases of acute stroke, has not been studied prospectively. The aim of the study was to investigate AAD on the hemibody contralateral to the lesion in the acute phase of stroke. One hundred consecutive patients (median age +/- interquartile range, 74 +/- 21; range 19-93; 51 women: 80 +/- 17 and 49 men: 70 +/- 17 years) in the acute phase of stroke were studied prospectively. Changes in skin temperature or coloration, diaphoresis, pain, or edema were noted in the first 3 days post-stroke. Associations between AAD and topography (cortical pre- and/or post-central, insular, corona radiata, basal ganglia, internal capsule, thalamus, and brainstem), age, gender, ischemic or hemorrhagic etiology, or the presence of sensorimotor deficits or ataxia were examined using the chi-squared or Fisher's exact test and logistic regression analysis. AAD was found in 71% of the patients and showed a significant positive association with the presence of a lesion in the post-central cortex (P = 0.037), internal capsule (P = 0.005), basal ganglia (P = 0.002), or insula (P = 0.011) and a negative association with the presence of a lesion in the brainstem (P = 0.004). Multivariate logistic regression analysis including all studied topographic variables showed that only brainstem lesions were significantly associated with a decreased risk of developing AAD (odds ratio = 0.08, 95% confidence interval: 0.01-0.69, P = 0.022). AAD was not associated with age, gender, the ischemic or hemorrhagic nature of the lesion, the side of lesion, hypertonic or hypotonic paresis, or hyperreflexia or hyporeflexia. AAD was found in association with sensory deficits (P = 0.001) and contralateral hyperkinesia (P = 0.004). Acute AAD is significantly more likely to occur in the presence of hemispheric lesions involving sensory pathways from the cortex to the internal capsule and insula and is significantly less prevalent in the presence of brainstem lesions.

Disfunción autonómica cardiovascular en la esclerosis múltiple no provocada únicamente por lesiones troncoencefálicas

Multiple sclerosis (MS) can cause alterations in autonomic cardiovascular functioning. Some authors attribute these anomalies detected in MS patients to brain stem lesions, due to the fact that important autonomic nuclei are located in this region of the brain. Other studies, however, have been unable to prove such a relation. AIMS. The purpose of this study was to analyse whether these alterations had an exclusive relation with the brain stem lesions that usually appear in the course of this disease.We compared the spectral analysis of the heart rate variability (SAHRV) in the frequency domain between a group of 34 patients with MS and another group of 14 patients with isolated brain stem lesions (IBSL) of a non inflammatory origin, which were measured using a 24 hour Holter recording.Heart rate, very low rates, low rates and the low rate/high rate quotient are significantly higher in the MS group than in the IBSL group, even when the former present brain stem lesions. Results in the high rate component, however, depend on the presence of brain stem lesions in the MS group.These findings suggest that autonomic cardiovascular dysfunction in MS is only related with brain stem lesions, although we obtained results that confirm the importance of this area in cardiovascular innervation.

Cerebral Autoregulation in Unconscious Patients with Brain Injury

In 18 unconscious patients with traumatic brain injury, the cerebral autoregulation was tested during the first 2-3 weeks after the acute trauma. Regional cerebral blood flow (rCBF) was measured by the intra-arterial 133xenon washout method before and after an increase of about 20% in the mean arterial blood pressure (MABP) by angiotensin. The difference between MABP and intraventricular pressure (IVP) was used as cerebral perfusion pressure (PP). Simultaneously, ventricular fluid pH, lactate and pyruvate were measured. Regional loss of autoregulation indicated by a 20% flow increase was observed in 29 out of 35 studies (83%), while hemispheric loss of autoregulation was observed in only one study. The results of the autoregulation tests were unrelated to the clinical outcome, the presence of brain-stem lesion, and the ventricular fluid pH, lactate and lactate/pyruvate ratio. In repeated studies, a gradual normalization of the autoregulation was observed about 5 days after the acute trauma.

Some electronystagmographic manifestations of central nervous system disease.

A battery of tests with electronystagmography were performed on two groups of patients, one in which posterior fossa abnormalities had been confirmed independently by neurologic findings, contrast studies, or surgery; the other group consisted of what seemed clinically clear cut peripheral VIII nerve or labyrinthine disturbances. The two groups were compared statistically for the incidence of factors thought to indicate the presence of brain stem lesions. No single factor is absolutely reliable by itself as an indicator of brain stem disease, but the use of a test battery permits a much more accurate indication of probability of the presence of brain stem lesions.