Dysautonomia and related outcomes in Guillain-Barre syndrome.

BACKGROUND: Guillain-Barre syndrome (GBS) presents an annual incidence of 1.2-2.3 per 100,000. Sympathetic and parasympathetic nervous systems' peripheral control of visceral organs is affected by GBS aberrant immune response. Associated cardiovascular, gastrointestinal, sudomotor, pupillary, and other systems disturbances cause significant morbidity and mortality. This study aims to evaluate the dysautonomia spectrum in GBS patients, its relationship with patient outcomes, and compare it with those without autonomic disturbances. METHODS: We performed an ambispective review study of patients with GBS and dysautonomia admitted to the Institute of Neurology from 2017 to 2021. We recorded demographics, comorbidities, nerve conduction studies, clinical course, hospital complications, and functional outcomes. RESULTS: We included 214 patients, mean age 46.44 ± 16.49 years, 51 (31 %) presented dysautonomia, hypertension in most of the patients 39 (84.8 %), hypotension 35 (76.1 %), tachycardia 35 (76.1 %), enteric dysmotility 35 (76.1 %), and need for vasopressor 27 (58.7 %) were common characteristics. Twenty (39.2 %) with a demyelinating form and twenty (39.2 %) with an axonal motor form. The bivariate analysis report factors associated with dysautonomia, were lower cranial nerves (VII, IX, X) involvement (p = 0.002), need for mechanical ventilation (p = 0.0001) and intensive care (p = 0.0001), higher mEGOS (p = 0.05), EGRIS (p = 0.004), GBS disability score (p = 0.004), and delirium presence (p = 0.001). Kaplan-Meier survival analysis showed that dysautonomic patients needed more days for the independent walk (p = 0.004). There was no associated mortality. CONCLUSIONS: Autonomic dysfunction in GBS significantly affects the peripheral nervous system. With consequently worse functional results. Further investigation needs to clarify whether more aggressive treatment is beneficial in this category of GBS.

Prognostic Factors in Elderly Patients With Guillain-Barré Syndrome: Does Age Matter?

INTRODUCTION: Twenty to 40% of Guillain Barré syndrome (GBS) patients will not be able to walk independently despite effective treatment. Older patients carry additional risks for worse outcomes. METHODS: A single center, ambispective cohort study was performed. Only subjects ≥18 years with a 3-month follow-up were included. Elderly patients were considered as a whole if ≥ 60 years. Demographics, CSF and nerve conduction studies were compared. A binomial logistic regression and Kaplan-Meier analyses were carried out to estimate good prognosis (Hugues ≤2) at 3-month follow-up. RESULTS: From 130 patients recruited, 27.6% were elderly adults. They had a more severe disease, higher mEGOS and more cranial nerve involvement. Age ≥70 years, invasive mechanical ventilation and axonal subtype, portrayed an unfavorable 3-month outcome. Further analysis demonstrated an earlier recovery in independent walk at 3 months for patients <70 years. CONCLUSIONS: Elderly patients with GBS have a more severe disease at admission and encounter worse prognosis at 3-month follow-up, especially those above 70 years.

Socioeconomic status and access to multiple sclerosis treatment in Mexico.

INTRODUCTION: Multiple sclerosis (MS) is a chronic neurological autoimmune condition and the leading non-traumatic cause of neurological disability worldwide. Disease-modifying therapies (DMT) directly impact on the long-term prognosis of patients with MS preventing relapses and the associated disability progression. Here, we analyzed the impact of socioeconomic status (SES) on DMT access in Mexican patients. METHODS: We evaluated the association between SES and DMT access using the MS registry from the National Institute of Neurology and Neurosurgery in Mexico City. We included 974 patients with MS (McDonald 2010 criteria). We categorized SES according to the 2018 Mexican Association of Market Research Agencies (AMAI) SES classification. We analyzed DMT type, MS phenotype, educational level, symptomatic onset to diagnosis, EDSS at arrival, as well as the progression index. Chi-squared and Wilcoxon tests were used, and multivariable analysis performed for DMT access. RESULTS: When comparing the lower versus higher levels of SES, a significant association was found on the percentage of patients with higher levels of disability (EDSS >6) at arrival, the proportion of patients not receiving any DMT and a higher proportion of secondary progressive MS (p=0.006, p<0.001and p=0.004, respectively). We also found that lower educational levels had a significance and inverse association with EDSS on first visit (p=0.019), symptomatic onset to diagnosis (p<0.001) and a higher disability status at arrival (EDSS >6, p=0.010). CONCLUSIONS: Our study suggests that SES is an important factor determining not only prompt but overall access to highly effective DMT. Lower SES are associated with greater levels of disability at the first clinic visit and a higher proportion of patients not receiving DMT up to 12 months of follow-up.

Rituximab efficacy at different initial and maintenance doses in neuromyelitis optica spectrum disorder: Experience from a national health institute in México.

BACKGROUND: NMOSD is an inflammatory disorder of the central nervous system that primarily affects the optic nerves and spinal cord. Rituximab (RTX) is a monoclonal antibody directed against CD20, an epitope expressed on pre-B and mature B cells. It has of wide use in several antibody-mediated autoimmune diseases. OBJECTIVES: To demonstrate RTX clinical efficacy at different initial and maintenance doses administered in patients with NMOSD. METHODS: In this retrospective/observational study we recruited subjects with NMOSD with at least one RTX infusion. Annual relapse rates (ARR) were compared in several induction and maintenance regimens with RTX in 66 patients with NMOSD. RESULTS: Fifty-four (81.8%) were female and two thirds (66.7%) had positive anti-AQP4 antibodies. The most prevalent induction and maintenance regimens were 1000 mg on days 1 and 15 (51.5%) and 1000 mg every 6 months (40.9%), respectively. Overall, the annual relapse rate (ARR) decreased from 1.15 to 0.46 with RTX (p < 0.001). In patients with persistent relapses, the ARR decreased from 1.66 to 1.22, representing a relative risk reduction of 24%. Treatment with RTX decreased the ARR from 1.36 to 0.4 in the 500 mg induction and maintenance dose subgroup, and from 0.7 to 0.4 in the 1000 mg induction and maintenance dose subgroup. CONCLUSION: RTX treatment in patients with NMOSD demonstrated a marked and sustained reduction in the ARR, regardless of induction and maintenance regimens. EDSS stability was observed, even in patients with active and severe NMOSD.

Bilateral Internuclear Ophthalmoplegia in a Young Woman with Vertebral Artery Dissection.

BACKGROUND: Internuclear ophthalmoplegia (INO) is an eye movement disorder caused by a lesion in the medial longitudinal fasciculus (MLF) located in the midbrain. Adduction paralysis of both eyes and bilateral abduction nystagmus are the main features of INO[1]. CASE PRESENTATION: A 29-year-old Hispanic woman was admitted to the emergency department complaining of an intense holocranial headache lasting 9 days, associated with nausea and vomiting. She was discharged home with resolution of the headache but persistence of symptoms. However, she subsequently developed horizontal diplopia and gait abnormalities. She was readmitted to hospital because of anomalous eye movements and conjugate gaze palsy, manifested as bilateral INO. Magnetic resonance angiography (MRA) findings were consistent with dissection of the left V4 vertebral artery with multiple brain infarcts in the superior cerebellar artery territory, comprising both MLF tracts. CONCLUSIONS: In young adults, bilateral INO is normally caused by demyelinating disease. In other patients, common causes include trauma, infections and autoimmune diseases with neurological symptoms. Vascular disease is implicated in over a third of cases. LEARNING POINTS: A vascular aetiology should be suspected when internuclear ophthalmoplegia (INO) presents with an intense headache.Almost a third of patients with bilateral INO have an identified vascular cause.Magnetic resonance imaging is the gold standard for investigating INO.

Premotor symptoms and the risk of Parkinson's disease: A case-control study in Mexican population.

OBJECTIVES: To assess the prevalence of pre-motor symptoms and estimate the risk for developing Parkinson's disease in Mexican population. PATIENTS AND METHODS: A case-control study was carried out with consecutive subjects with Parkinson's disease from two different referral centers in Mexico. Gender- and age-matched controls were randomly selected from the participating hospitals. All subjects were assessed using a structured questionnaire for the assessment of pre-motor symptoms (hyposmia, depression, anxiety, constipation, and sleep disorders). Odds ratios (OR) were calculated using logistic regression analysis. RESULTS: A total of 430 subjects with PD and 430 healthy subjects were included. Premotor symptoms prevalence was 77.7% (n=334) for the PD group, compared to 41.3% (n=178) in the control group (p<0.001). After logistic multivariate analysis, previous history of hyposmia (OR 2.02 [95% CI 1.33-3.06]), depression (OR 2.52 [95% CI 1.67-3.84]), anxiety (OR 4.37 [95% CI 2.73-6.98]) and sleep disorders (OR 2.03 [95% CI 1.41-2.93]) were independently associated with Parkinson's disease. Overall prediction success of the model was 81.2% for controls and 61.2% for subjects with PD. CONCLUSION: All five premotor symptoms assessed were more commonly reported in PD subjects than healthy controls. The presence of non-motor symptoms yield a prediction success of 71.2% to discriminate between PD subjects and healthy controls.

A systematic review and mixed treatment comparison of monotherapy in early Parkinson's disease: implications for Latin America.

Parkinson's disease (PD) is the second most common neurodegenerative disease. There are no clinical trials comparing all available pharmacological therapies for the treatment of early PD. The objective of this review is to indirectly analyze the efficacy of antiparkinson drugs currently available in Latin America. A systematic review was performed exploring only placebo-controlled randomized trials comparing antiparkinson monotherapy (levodopa, pramipexole, rasagiline, or selegiline) in patients with PD on Hoehn and Yahr stages I through III published from January 1994 to May 2014. The primary outcome was the mean change in the Unified PD Rating Scale (UPDRS) I, II and III. A mixed treatment comparison analysis (indirect comparisons) through a random-effects model was performed. Levodopa demonstrated the highest effects in terms of UPDRS score improvement both from baseline and when compared to other treatments. Levodopa showed a 60.1% probability of granting the greatest reduction in UPDRS I, II and III.

Self-Perceived Pre-Motor Symptoms Load in Patients with Parkinson's Disease: A Retrospective Study.

BACKGROUND: Parkinson's disease is characterized by motor and non-motor clinical features. The latter may present as pre-motor symptoms several years before the motor onset. OBJECTIVE: To analyze the association between pre-motor symptoms load and its lead-time in relation to the motor onset and time to diagnosis. METHODS: A cross-sectional study was carried including subjects with Parkinson's disease from five different movement disorders clinics in Mexico. A structured questionnaire was applied to assess the presence of six self-perceived pre-motor symptoms (hyposmia, depression, anxiety, constipation, pain and sleep disorders). RESULTS: Overall frequency of pre-motor symptoms was 76.2% . Among the most prevalent symptoms were depression (38%), sleep disorders (37%) and anxiety (36.6%). The lead time to motor onset was greater for constipation (9.2 ± 17.89 years) and pain (8.66 ± 13.36 years). Patients with more than two pre-motor symptoms had a later age at motor onset when compared to patients without pre-motor symptoms (52.04 ± 13.11 vs 56.55 ± 12.97 years, p = 0.037). Late onset patients had a higher frequency of pre-motor symptoms (79% vs 65% in early onset, p = 0.002) and a higher load (1.75 ± 1.37 vs 1.44 ± 1.38, p = 0.033) in comparison to those with early onset. Female subjects reported a higher number of pre-motor symptoms (1.91 ± 1.43 versus 1.48 ± 1.29, p ≤ 0.001). PIGD patients reported a greater frequency of pain (8%) compared to tremor (1%, p = 0.0064) and bradykinetic-rigid (0.61%, p = 0.0061). Anxiety lead-time was greater in tremor-dominant (10.83 ± 15.77 years) compared to bradykinetic-rigid patients (3.48 ± 12.56, p = 0.014). CONCLUSIONS: Pre-motor symptoms load is associated to a later motor onset of PD. Pre-motor symptoms are more frequent in subjects with late onset Parkinson's disease. Female subjects report a higher number of pre-motor symptoms, depression and anxiety being the most common.