Bulbar Function Research Articles

The infantile neuroaxonal dystrophy rating scale (INAD-RS)

BackgroundINAD is an autosomal recessive neurogenetic disorder caused by biallelic pathogenic variants in PLA2G6. The downstream enzyme, iPLA2, plays a critical role in cell membrane homeostasis by helping to regulate levels of phospholipids. The clinical presentation occurs between 6 months and 3 years with global developmental regression, hypotonia, and progressive spastic tetraparesis. Progression is often rapid, resulting in severe spasticity, visual impairment, and cognitive decline, with many children not surviving past the first decade of life. To date, no accepted tool for assessing the severity of INAD exists; other commonly used scales (e.g. CHOP-INTEND, Modified Ashworth, Hammersmith Functional Motor Scale) do not accurately gauge the current severity of INAD, nor are they sensitive/specific enough to monitor disease progression. Finally, these other scales are not appropriate, because they do not address the combination of CNS, peripheral nerve, and visual pathology that occurs in children with INAD.MethodsWe have developed and validated a structured neurological examination for INAD (scored out of 80). The examination includes six main categories of pediatric developmental evaluation: 1) gross motor-and-truncal-stability skills, 2) fine motor skills, 3) bulbar function, 4) ocular function, 5) temporo-frontal function, and, 6) Functional evaluation of the autonomic nervous system. A cohort of patients diagnosed with INAD were followed prospectively to validate the score against disease severity and disease progression.ResultsWe show significant correlation between the total neurological assessment score and months since symptom onset with a statistically significant (p = 6.7 × 10− 07) correlation between assessment score and disease onset. As hypothesized, the coefficient of months-since-symptom-onset is strongly negative, indicating a negative correlation between total score and months since symptom onset.ConclusionWe have developed and validated a novel neurological assessment score in INAD that demonstrates strong correlation with disease severity and disease progression.

Quantitative ultrasound of the tongue: Echo intensity is a potential biomarker of bulbar dysfunction in amyotrophic lateral sclerosis

ObjectivesTo learn if quantitative ultrasound (QUS) distinguishes the tongues of healthy participants and amyotrophic lateral sclerosis (ALS) patients by echo intensity (EI) and to evaluate if EI correlates with measures of bulbar function. MethodsUltrasound was performed along the midline of the anterior tongue surface in 16 ALS patients and 16 age-matched controls using a linear hockey stick 16–7 MHz transducer. A region of interest was manually drawn and then EI was determined for the upper 1/3 of the muscle. For patients, the ALS functional rating scale – revised (ALSFRS-R) was used to calculate bulbar sub-scores and the Iowa Oral Performance Instrument (IOPI) was used to measure tongue strength. ResultsEI was significantly higher in ALS patients than in healthy participants (49.8 versus 37.8 arbitrary units, p < 0.01). In the patient group, EI was negatively correlated with ALSFRS-R bulbar sub-score (RS = −0.65, p < 0.01). An inverse correlation between EI and tongue strength did not reach significance (RS = −0.34, p = 0.28). ConclusionsThis study suggests that EI can differentiate healthy from diseased tongue muscle, and correlates with a standard functional measure in ALS patients. SignificanceTongue EI may represent a novel biomarker for bulbar dysfunction in ALS.

Latent Ornstein-Uhlenbeck models for Bayesian analysis of multivariate longitudinal categorical responses.

We propose a Bayesian latent Ornstein-Uhlenbeck (OU) model to analyze unbalanced longitudinal data of binary and ordinal variables, which are manifestations of fewer continuous latent variables. We focus on the evolution of such latent variables when they continuously change over time. Existing approaches are limited to data collected at regular time intervals. Our proposal makes use of an OU process for the latent variables to overcome this limitation. We show that assuming real eigenvalues for the drift matrix of the OU process, as is frequently done in practice, can lead to biased estimates and/or misleading inference when the true process is oscillating. In contrast, our proposal allows for both real and complex eigenvalues. We illustrate our proposed model with a motivating dataset, containing patients with amyotrophic lateral sclerosis disease. We were interested in how bulbar, cervical, and lumbar functions evolve overtime.

The Role of Radiation Therapy and Botulinum Toxin Injections in the Management of Sialorrhea in Patients With Amyotrophic Lateral Sclerosis: A Systematic Review.

Half of patients with amyotrophic lateral sclerosis experience sialorrhea due to facial weakness. Although anticholinergic medications are first-line therapy, they often lead to unacceptable side effects. Radiation therapy and botulinum toxin may be considered when medical management fails. In this systematic review, we investigated the effectiveness of these interventions. Eligible studies were retrieved from PubMed and Scopus databases up to March 2017 along with hand-searching of references from primary articles. Fourteen studies (N = 138) examined the benefits of botulinum toxin. Studies varied in salivary glands treated, dosages used, and the use of botulinum toxin subtype A or B. A majority of studies showed benefit after treatment. Although most studies reported only mild adverse effects, 2 case studies revealed severe complications including recurrent TMJ dislocations and rapid deterioration in bulbar function. Ten studies (N = 171) examined the benefits of radiation. Most studies reported improvement with only mild adverse events reported. Both radiation and botulinum toxin are effective treatments for sialorrhea in patients with amyotrophic lateral sclerosis and should be considered when medical management fails. Radiation may offer longer duration of symptom improvement with fewer complications.

Maximum bite force in patients with spinal muscular atrophy during the first year of nusinersen therapy - A pilot study.

ObjectivesSpinal muscular atrophy is a monogenic disease characterized by progressive spinal and bulbar muscle weakness and atrophy. It is caused by the degeneration of alpha-motoneurons. The recent approval of the antisense oligonucleotide nusinersen highlights the need for reliable clinical tools to evaluate motor function in patients with neuromuscular disorders. Measurement of the bulbar neuromuscular function (e.g., bite force) could be an extension to existing motor scales, sensitive to more nuanced changes, especially in symptomatic patients with severely reduced functional abilities.Materials and methodsMaximum bite force measurement was used to quantify changes of the masticatory function in adult monozygotic female twins with SMA type II. Using piezoelectric transducers, 550 observations were recorded for each patient during the first year of nusinersen therapy.ResultsDuring the application of four loading doses of nusinersen, bite force levels steadily increased and reached a statistically significantly higher level compared to the initial state in both patients. Subsequent maintenance doses coincided with smaller or no statistically significant changes in maximum bite force.ConclusionsThis pilot study indicates that the measurement of maximum bite force may be a useful tool to detect changes of the bulbar function in SMA patients. As such, it may supplement existing scales to identify treatment-related changes in motor function.

Subxiphoid completion thymectomy for refractory non-thymomatous myasthenia gravis.

BackgroundCompletion thymectomy may be performed in patients with non-thymomatous refractory myasthenia gravis (MG) to allow a complete and definitive clearance from residual thymic tissue located in the mediastinum or in lower neck. Hereby we present our short- and long-term results of completion thymectomy using subxiphoid video-assisted thoracoscopy.MethodsBetween July 2010 and December 2017, 15 consecutive patients with refractory non-thymomatous myasthenia, 8 women and 7 men with a median age of 44 [interquartile range (IQR) 38.5–53.5] years, underwent video-thoracoscopic completion thymectomy through a subxiphoid approach.ResultsPositron emission tomography (PET) showed mildly avid areas [standardized uptake value (SUV) more than or equal to 1.8] in 11 instances. Median operative time was 106 (IQR, 77–141) minutes. No operative deaths nor major morbidity occurred. Mean 1-day postoperative Visual Analogue Scale value was 2.53±0.63. Median hospital stay was 2 (IQR, 1–3.5) days. A significant decrease of the anti-acetylcholine receptor antibodies was observed after 1 month [median percentage changes −67% (IQR, −39% to −83%)]. Median follow-up was 45 (IQR, 21–58) months. At the most recent follow-up complete stable remission was achieved in 5 patients. Another 9 patients had significant improvement in bulbar and limb function, requiring lower doses of corticosteroids and anticholinesterase drugs. Only one patient remained clinically stable albeit drug doses were reduced. One-month postoperative drop of anti-acetylcholine receptor antibodies was significantly correlated with complete stable remission (P=0.002).ConclusionsThis initial experience confirms that removal of ectopic and residual thymus through a subxiphoid approach can reduce anti-acetylcholine receptor antibody titer correlating to good outcome of refractory MG.

The Maximum Bite Force for Treatment Evaluation in Severely Affected Adult SMA Patients-Protocol for a Longitudinal Study.

Spinal muscular atrophy (SMA) is a severe neuromuscular disorder characterized by the degeneration of motor neurons in the spinal cord, and comprises a broad clinical spectrum. With the advent of new therapies (e.g., Nusinersen) for patients of all ages and disease stages, sensitive clinical measures are needed to detect slight changes in muscle force even in immobilized, severely affected patients often unable to move limbs. As for these patients, well-established outcome scales set out to evaluate motor function do not work properly, we propose measurement of maximum bite force which is able to detect subtle changes of bulbar function. Requirements for this approach are mentioned, challenges are discussed, and first insights from a pilot study are presented. Finally, a study design is proposed to evaluate the measurement of maximum bite force during the follow up of SMA patients with and without a disease modifying therapy.

Clippers the Brain Biter: An Adolescent’s Experience

Introduction: Chronic Lymphocytic Inflammation with Pontine Perivascular Enhancement Responsive to Steroids is a rare inflammatory Central Nervous System disorder prominently affecting the brainstem. We present an adolescent’s reflection on this condition complicated with Epstein-Barr virus induced CNS Lymphoma. Case report: A 16-year-old boy presented about 5 years ago with a balance problem. He was diagnosed by MRI after ongoing debate whether this is juvenile multiple sclerosis. He initially responded to methylprednisolone but developed acute deterioration requiring 8 cycles of Infliximab and Methylprednisolone. He then confirmed cerebellar lymphoma 2 years later hence commenced on chemotherapy and radiotherapy after posterior fossa decompression. He gradually losses his motor skills, left hemiparesis and spasticity. He needs tracheostomy and gastrostomy due to poor bulbar function. Now, he is fully dependent and requires chest physiotherapy and suctioning and cystostomy for urinary incontinence. He has multiple PICU admissions due to recurrent aspiration, acute cerebellar oedema and post posterior fossa decompression. He worries about family breakdown as mum is his sole carer and having regular nightmare. He changed school to meet his care demands and unsure how to adapt to new environment. Social experience makes him anxious due to lack of understanding about his condition. He is evidently having low self-esteem and confidence. Discussion: Children palliative care team has been involved since diagnosis to support him and family. He is understandably depressed and clinical psychologist input bear minimal impact. He is fully aware of the current situation and his wish to rap Eminem’s songs like he used to do it before. Conclusion: Early aggressive treatment in CLIPPERS aim to prevent neuroaxonal loss. However, due to bellicose nature of the condition, the prognosis is relatively poor. Managing adolescent expectation after gradual deterioration is challenging especially, he is aware that achieving ‘normality’ is impossible as the treatment advancement still in limbo.

Communicative Participation in People with Amyotrophic Lateral Sclerosis

Background: Communication is affected in most people with amyotrophic lateral sclerosis (ALS); up to 80–95% will reach a point where they are no longer able to meet their communicative needs with natural speech. The deterioration of speech and communicative abilities presumably has an impact on communicative participation. However, little is known about how these factors relate to each other in this population of patients. Objective: This study aimed to investigate the association between communicative participation, functional deficits, and severity of dysarthria in individuals with ALS. Method: Thirty people with ALS were rated for (1) communicative participation, using the Communicative Participation Item Bank (CPIB, Swedish); and (2) disability related to the disease, using the Revised ALS Functional Rating Scale (Swedish). An expert listening panel assessed intelligibility and severity of dysarthria based on recorded text readings and sentences from the Swedish Test of Intelligibility. Results: CPIB scores were significantly lower for participants with moderate/severe dysarthria than for those with no/mild dysarthria and correlated with bulbar function and intelligibility. Conclusion: The study found that the CPIB provides a means to rate and discuss communicative participation with persons with ALS, which could assist in the planning of further efforts/services.

Sonographic and 3T-MRI-based evaluation of the tongue in ALS

A few systematic imaging studies employing ultrasound (HRUS) and magnetic resonance imaging (MRI) have suggested tongue measures to aid in diagnosis of amyotrophic lateral sclerosis (ALS). The relationship between structural tongue alterations and the ALS patients' bulbar and overall motor function has not yet been elucidated. We here thus aimed to understand how in-vivo tongue alterations relate to motor function and motor function evolution over time in ALS. Our study included 206 ALS patients and 104 age- and sex-matched controls that underwent HRUS and 3T MRI of the tongue at baseline. Sonographic measures comprised coronal tongue echointensity, area, height, width and height/width ratio, while MRI measures comprised sagittal T1 intensity, tongue area, position and shape. Imaging-derived markers were related to baseline and longitudinal bulbar and overall motor function. Baseline T1 intensity was lower in ALS patients with more severe bulbar involvement at baseline. Smaller baseline coronal (HRUS) and sagittal (MRI) tongue area, smaller coronal height (HRUS) and width (HRUS) as well as more rounded sagittal tongue shape predicated more rapid functional impairment - not only of bulbar, but also of overall motor function - in ALS. Our results suggest that in-vivo sonography und MRI tongue measures could aid as biomarkers to reflect bulbar and motor function impairment.

An exploration of neural dynamics of motor imagery for people with amyotrophic lateral sclerosis

Objective. Studies of the neuropathological effects of amyotrophic lateral sclerosis (ALS) on the underlying motor system have investigated abnormalities in the magnitude and timing of the event-related desynchronization (ERD) and synchronization (ERS) during motor execution (ME). However, the spatio-spectral-temporal dynamics of these sensorimotor oscillations during motor imagery (MI) have not been fully explored for these patients. This study explores the neural dynamics of sensorimotor oscillations for ALS patients during MI by quantifying ERD/ERS features in frequency, time, and space. Approach. Electroencephalogram (EEG) data were recorded from six patients with ALS and 11 age-matched healthy controls (HC) while performing a MI task. ERD/ERS features were extracted using wavelet-based time-frequency analysis and compared between the two groups to quantify the abnormal neural dynamics of ALS in terms of both time and frequency. Topographic correlation analysis was conducted to compare the localization of MI activity between groups and to identify subject-specific frequencies in the µ and β frequency bands. Main results. Overall, reduced and delayed ERD was observed for ALS patients, particularly during right-hand MI. ERD features were also correlated with ALS clinical scores, specifically disease duration, bulbar, and cognitive functions. Significance. The analyses in this study quantify abnormalities in the magnitude and timing of sensorimotor oscillations for ALS patients during MI tasks. Our findings reveal notable differences between MI and existing results on ME in ALS. The observed alterations are speculated to reflect disruptions in the underlying cortical networks involved in MI functions. Quantifying the neural dynamics of MI plays an important role in the study of EEG-based cortical markers for ALS.

PRO2 THE VALUE OF ONASEMNOGENE ABEPARVOVEC GENE-REPLACEMENT THERAPY IN IMPROVING SURVIVAL AND MOTOR FUNCTION AND DECREASING VENTILATORY SUPPORT AND HOSPITALIZATION CONTRASTED TO NATURAL HISTORY FOR SPINAL MUSCULAR ATROPHY TYPE 1

Spinal muscular atrophy type 1 (SMA1) is a progressive monogenic neurologic disease causing loss of motor muscle function and bulbar muscle function essential for normal breathing and swallowing. This cross-study comparison highlights the value of onasemnogene abeparvovec (formerly AVXS-101) on survival, motor milestone achievement, ventilatory support, and hospitalizations in SMA1 patients. SMA1 patients (two SMN2 copies) were treated with onasemnogene abeparvovec (CL-101; NCT02122952; Cohort 2; n=12). Event-free survival (EFS; composite endpoint of time to death/permanent ventilation), motor milestone achievement, ventilatory support, and hospitalization were assessed (CL-101, ≥20 months; two natural history studies [untreated patients, 14 months of age: PNCR, ≥50% completing 12 months follow-up, N=23, and NN101, N=16]; sham control group from ENDEAR [NCT02193074]). The proportion of patients achieving EFS at ≥14 months was 30% in PNCR and 50% in NN101, compared with 100% in onasemnogene abeparvovec-treated patients. No patient in either natural history study achieved motor milestones, while 92% of onasemnogene abeparvovec-treated patients sat unassisted, 17% stood with assistance, and 17% walked independently. The proportion of patients surviving but requiring permanent assisted ventilation was 57% in PNCR and 0% in onasemnogene abeparvovec-treated patients. The mean unadjusted annualized rate of hospitalizations (total hospitalizations/total number of subject-years followed) was 4.3 for sham control-treated patients in ENDEAR and 2.1 for onasemnogene abeparvovec-treated patients. A single dose of onasemnogene abeparvovec resulted in dramatic survival and motor milestone achievements with reduced healthcare utilization, which could decrease cost and alleviate patient, caregiver, and societal burden, suggesting an overall improved quality of life compared to natural history.

P.05Feasibility and validation of modified oculobulbar facial respiratory score (mOBFRS) in sporadic inclusion body myositis

Sporadic inclusion body myositis (sIBM) is a neurodegenerative disorder that results in progressive limb muscle weakness with bulbar and respiratory involvement. The leading cause of morbidity and mortality are secondary to bulbar dysfunction. The IBMFRS is a reliable patient reported outcome measure used in sIBM, however, we lack objective scales that can quantitate deterioration in bulbar function in a clinic setting. We are conducting a prospective study to test the feasibility of using,and validate the use of the modified oculobulbar facial respiratory score (mOBFRS) in sIBM. The mOBFRS is validated in immune myasthenia gravis. We will evaluate if changes in mOBFRS in sIBM: 1) can be reliably measured on a serial basis; 2) correlate with scores of the existing subjective IBM functional rating scale (IBMFRS) and objective Bulbar Rating Scale (BRS); and 3) can be used as a potential outcome measure in clinical trials. Subjects are tested at baseline, month 6 and month 12 visit. Interim analysis was done using a linear mixed effect model to assess the BRS compared with mOBFRS as fixed effect and a random intercept to account for the with-in subject correlation. Pearson's correlation was calculated between swallow time and subjective swallow ability at each visit. Since study initiation, 50 subjects have completed the baseline visit, 28 completed month 6, and 13 completed month 12. The mOBFRS score is significantly associated with the BRS along all three visits (p< 0.0001). One score change of mOBFRS leads to an average of 0.36 point change in the BRS. The IBMFRS swallow ability question was compared to the mOBFRS timed swallow test which showed no significant correlation at any of the three visits. Conducting the mOBFRS in a clinic setting has proved to be feasible. mOBFRS is trending to be a useful measure of bulbar function but the study is ongoing

Cognitive impairment across ALS clinical stages in a population-based cohort.

To assess the association of the degree of severity of motor impairment to that of cognitive impairment in a large cohort of patients with amyotrophic lateral sclerosis (ALS). This is a population-based cross-sectional study on patients with ALS incident in Piemonte, Italy, between 2007 and 2015. Cognitive status was classified according to the revised ALS-FTD Consensus Criteria. The King system and the Milano Torino Staging system (MiToS) were used for defining the severity of motor impairment. Of the 797 patients included in the study, 163 (20.5%) had ALS-frontotemporal dementia (FTD), 38 (4.8%) cognitive and behavioral impairment (ALScbi), 132 (16.6%) cognitive impairment (ALSci), 63 (7.9%) behavioral impairment (ALSbi), 16 (2.0%) nonexecutive impairment, and 385 (48.2%) were cognitively normal. According to King staging, the frequency of cases with ALS-FTD progressively increased from 16.5% in stage 1-44.4% in stage 4; conversely, the frequency of ALSci, ALSbi, and ALScbi increased from King stage 1 to King stage 3 and decreased thereafter. A similar pattern was observed with the MiToS staging. ALS-FTD was more frequent in patients with bulbar involvement at time of cognitive testing. Patients with C9ORF72 expansion (n = 61) showed more severe cognitive impairment with increasing King and MiToS stages. Our findings suggest that ALS motor and cognitive components may worsen in parallel, and that cognitive impairment becomes more pronounced when bulbar function is involved. Our data support the hypothesis that ALS pathology disseminates in a regional ordered sequence, through a cortico-efferent spreading model.

Respiratory Care of Patients With Neuromuscular Disease.

Neuromuscular diseases are a heterogeneous group of neurologic diseases that affect a number of neural structures including the motor nerves, neuromuscular junctions, or the muscles themselves. Although many of the diseases are rare, the total number of individuals who present to a pulmonologist or respiratory care provider is significant. Approaches to care include regular and careful clinical follow-up of symptoms of sleep-disordered breathing, daytime hypoventilation, as well as cough and swallowing effectiveness. Noninvasive support with nocturnal mask ventilation and a pressure support device can be extraordinarily helpful and delay daytime ventilatory failure. When daytime ventilatory failure develops, other noninvasive methods are available for portable assistance. Support of cough function with manual assistance, a resuscitator bag, and/or mechanical insufflation-exsufflation can help prevent and treat infection. Referral for swallowing evaluation and treatment is very important for those with impaired bulbar function. This comprehensive respiratory care approach to individuals with neuromuscular disease and respiratory system involvement is essential to maintaining the health and longevity of these individuals.

A Chinese pedigree with Brown-Vialetto-Van Laere syndrome due to two novel mutations of SLC52A2 gene: clinical course and response to riboflavin

BackgroundBrown-Vialetto-Van Laere Syndrome (BVVLS), a rare neurological disorder characterized by motor, sensory, and cranial neuronopathies, is mainly associated with defective riboflavin transporters encoded by SLC52A2 and SLC52A3 genes. Clinical outcomes have been shown to be improved significantly by high-dose riboflavin supplementation. The aim of this study was to identify genetic causes and further evaluate the clinical course and response to riboflavin in a Chinese pedigree with BVVLS.Case presentationWe report the novel compound heterozygous variants c.1328G>A p.(Cys443Tyr) and c.1022_1023insC p. (Leu341Profs*103) of SLC52A2 gene in a female proband who presented in our out-patient clinic at the age of one-year-old with progressive mental and motor regression, breath holding, and brain stem dysfunction including facial weakness, hearing loss, dysphagia. Following high-dose riboflavin supplementation, the respiratory insufficiency and mental, motor, and bulbar function improved. However, sensorineural hearing loss was not improved. The missense variant site was highly conserved. Both variants were not found in the population database gnomAD. The two variants were inherited from her mother and father, respectively. Both variants were predicted to be deleterious by Polyphen2, Mutation taster, and SIFT and were classified as likely pathogenic according to the ACMG guideline.ConclusionsTwo novel pathogenic variations of SLC52A2 gene were firstly found from a Chinese pedigree with BVVLS. Clinical outcomes could be improved by early diagnosis and riboflavin supplementation.

Improved Bulbar Function in Amyotrophic Lateral Sclerosis after Nuedexta (Dextromethorphan and Quinidine) Treatment

Improved Bulbar Function in Amyotrophic Lateral Sclerosis after Nuedexta (Dextromethorphan and Quinidine) Treatment

Preliminary design and validation of the "6-K-scale" for bulbar symptoms evaluation in SBMA.

Spinal and bulbar muscular atrophy (SBMA) is a late onset, X-linked neuromuscular disease. Bulbar symptoms are a main characteristic of the disease but a tool for their clinical evaluation still does not exist. The aim of this study was to design and test a new scale (6-K-scale) for evaluation of bulbar function in SBMA. We considered 60 genetically confirmed SBMA patients and built a scale to evaluate the V, VII, IX, X, and XII cranial nerves (CN) and the ansa cervicalis. Functional status was evaluated through the Spinal and Bulbar Muscular Atrophy Functional Rating Scale (SBMAFRS), 6-min-walk-test (6MWT), Adult Myopathy Assessment Tool (AMAT) scale, and FVC%. Twenty patients underwent a re-test after 3weeks, while 31 were tested longitudinally after 6months. Validation of the scale included reliability assessment and factorial analysis. To evaluate convergent validity, correlations between the 6-K-scale and functional parameters were performed. Internal consistency as measured by Cronbach's alpha was high (0.85) as was test-retest reliability. Principal component analysis yielded a six-factor solution accounting for 71.7% of the variance. The scale score was strongly correlated with the functional parameters. In conclusion, we designed and validated a new scale for bulbar evaluation in SBMA patients. This scale will be a useful tool in the clinical practice as well as a possible outcome measure in clinical trials.

Impact of Nuedexta on Bulbar Physiology and Function in ALS

Impact of Nuedexta on Bulbar Physiology and Function in ALS

Comprehensive rehabilitation treatment for pregnant women with Guillain-Barré syndrome - a case report

Guillain-Barré syndrome (GBS) is an acute demyelinating poliradioculopathy and autoimmune condition, with a fast rapid natural course, and with high disability and usually episodic immune treatment. Clinical features include areflexia, limb weakness and uncommonly, sensory loss proceeding to neuromuscular paralysis involving bulbar, facial and respiratory function with maximum severity of symptoms atin 2-4 weeks. The eEtiology of GBS is not incompletely understood, however, prognosis is usually good with early detection and prompt treatment. In thise paper, we present a unique case of comprehensive rehabilitation treatment for a pregnant woman with GBS atin the 8th week of pregnancy. On the basis of this case report, we discuss possible and safe rehabilitation treatment for women in the first trimester of pregnancy, with significantly weakened muscle strength, after stabilizsation of vital signs and immunotherapy. The paper describes comprehensive care provided to the pregnant woman with teraplegia, hyperstesia and dysphagia after stabiliszation of vital signs, which was conducted until delivery atin the 39th week of pregnancy. This article is licensed under a Creative Commons Attribution-ShareAlike 4.0 International License. null