Cerebellar Syndrome Research Articles

Left-handedness should not be overrated as a risk factor for postoperative speech impairment in children after posterior fossa tumour surgery: a prospective European multicentre study.

Cerebellar mutism syndrome (CMS) is a severe neurological complication of posterior fossa tumour surgery in children, and postoperative speech impairment (POSI) is the main component. Left-handedness was previously suggested as a strong risk factor for POSI. The aim of this study was to investigate the relationship between handedness and the risk of POSI. We prospectively included children (aged < 18years) undergoing surgery for posterior fossa tumours in 26 European centres. Handedness was assessed pre-operatively and postoperative speech status was categorised as either POSI (mutism or reduced speech) or habitual speech, based on the postoperative clinical assessment. Logistic regression was used in the risk factor analysis of POSI as a dichotomous outcome. Of the 500 children included, 37 (7%) were excluded from the present analysis due to enrolment at a reoperation; another 213 (43%) due to missing data about surgery (n = 37) and/or handedness (n = 146) and/or postoperative speech status (n = 53). Out of the remaining 250 (50%) patients, 20 (8%) were left-handed and 230 (92%) were right-handed. POSI was observed equally frequently regardless of handedness (5/20 [25%] in left-handed, 61/230 [27%] in right-handed, OR: 1.08 [95% CI: 0.40-3.44], p = 0.882), also when adjusted for tumour histology, location and age. We found no difference in the risk of POSI associated with handedness. Our data do not support the hypothesis that handedness should be of clinical relevance in the risk assessment of CMS.

Author Response: Progression of Nigrostriatal Denervation in Cerebellar Multiple System Atrophy: A Prospective Study.

We thank Teive et al. for their valuable comments on our work.1 We agree that REM sleep behavior disorders (RBD) are a typical hallmark of α-synucleinopathies, including multiple system atrophy (MSA).2 Some pieces of evidence suggest that RBD could be useful in differentiating idiopathic late-onset cerebellar ataxia (ILOCA) from cerebellar MSA (MSA-C).3 It has also been shown that RBD could predate the onset of cerebellar syndrome in MSA-C; for this reason, it has recently been suggested to create a criteria set for possible prodromal MSA-C defined by the association of RBD with cerebellar ataxia.4

INSP-08. Cerebellar mutism syndrome: incidence, symptoms, risk factors, prognosis. Results and future perspectives from the Nordic-European study of the cerebellar mutism syndrome

Cerebellar mutism syndrome (CMS) can be a devastating consequence of paediatric fossa posterior tumour surgery. This prospective observational multi-centre study was designed to investigate incidence, symptoms, risk factors and prognosis of CMS. It has been running since 2014 and is currently involving 28 centres in 10 countries. So far 600 patients have been included as of 1.2.22, and with the aim of including a total of 1000 the study is still ongoing. Analysis of the first 500 patients has described the incidence, course and associated risk factors of postoperative speech impairment (POSI). Midline location, histological diagnosis of medulloblastoma and ATRT and younger age are associated with increased risk of POSI in our cohort. Route of surgical access specifically comparing telovelar to transvermian approach does not seem to influence the risk of POSI. Second surgery is associated with a lower risk of POSI than primary surgery, whereas we found no difference in risk of cranial nerve dysfunction. Left-handedness is not associated with an increased risk of POSI. Further and ongoing analysis include language analyses of recorded patient speech samples, detailed imaging analysis and description of associations between POSI and other neurological symptoms. The results aim to facilitate pre-operative risk assessment and postoperative robust scoring methods for use in clinical trials. Presented on behalf of the CMS study group, clinicaltrials.gov: Nordic study of the cerebellar mutism syndrome.

MEDB-29. Application of Rotterdam Post-Operative Cerebellar Mutism Syndrome Prediction Model to Patients Operated for Medulloblastoma in a Single Institution

BACKGROUND: Post-operative cerebellar mutism syndrome (CMS) develops in up to 30% of children. The Rotterdam model (RM) predicts a 66% risk of CMS in patients with a score ≥100. However, our findings suggested that surgical experience contributes to CMS risk. The aim of this study was to retrospectively apply the RM and report incidence of CMS in high-risk patients from our institution. METHODS: Participants had to have first tumor resection at our institution and be enrolled on SJMB12 protocol (NCT01878617). All participants got structured serial neurologic evaluations. CMS, when present, was categorized into type 1 (complete mutism) and type 2 (paucity of speech with an inability to string 3-word sentence). Rotterdam score is calculated based on pre-operative imaging parameters and study neurologist (RBK) obtained it while blinded to CMS status. RESULTS: Of the 40 (14 female, 26 male) study participants, 4 (10%) had CMS (3 CMS1, 1 CMS2). Median age at tumor resection was 11.7 years (range 3.5-17.8). Tumor location was midline in 30 (75%), right lateral 6 (15%) and left lateral 4 (10%). Median Evans index was 0.3 (0.2-0.4) and 34 (85%) were ≥0.3 (indicative of hydrocephalus); 5 participants needed ventricular shunt. Median tumor volume was 50 cm3 (2-180.6). Gross total resection was achieved in 35 (87.5%), near total in 4 (10%) and subtotal in 1. Twelve tumors were SHH, 7 WNT, and 29 NWNS. Median RM score was 90 (25 – 145). Eighteen participants had a score of ≥100 and 16.7% of these (n=3) had CMS. Scores for the 4 with CMS were 85, 125, 145 and 145. CONCLUSION: At our institution, the incidence of CMS in those that had RM of ≥100 was much lower than reported risk of 66%. This data supports our hypothesis that neurosurgical experience remains a significant risk factor in the development of CMS.

IMG-02. Improved prediction of postoperative paediatric cerebellar mutism syndrome using an artificial neural network

Abstract BACKGROUND: Postoperative paediatric cerebellar mutism syndrome (pCMS) is a common but severe complication which may arise following the resection of posterior fossa tumours in children. Two previous studies have aimed to preoperatively predict pCMS, with varying results. In this work, we examine the generalisation of these models and determine if pCMS can be predicted more accurately using an artificial neural network (ANN). METHODS: An overview of reviews was performed to identify risk factors for pCMS, and a retrospective dataset collected as per these defined risk factors from children undergoing resection of primary posterior fossa tumours. The ANN was trained on this dataset and its performance evaluated in comparison to logistic regression and other predictive indices via analysis of receiver operator characteristic curves. Area under the curve (AUC) and accuracy were calculated and compared using a Wilcoxon signed rank test, with p&amp;lt;0.05 considered statistically significant. RESULTS: 204 children were included, of whom 80 developed pCMS. The performance of the ANN (AUC 0.949; accuracy 90.9%) exceeded that of logistic regression (p&amp;lt;0.05) and both external models (p&amp;lt;0.001). CONCLUSION: Using an ANN, we show improved prediction of pCMS in comparison to previous models and conventional methods.

SURG-17. Impact of introduction of sagittal dimensions to predictive model of post-operative cerebellar mutism (CMS): A report of the Nottingham CMS Workshop

Abstract BACKGROUND: The Nottingham-Liverpool risk stratification tool for predicting post-operative cerebellar mutism syndrome (CMS) requires further refinement. Previous smaller studies identified tumours arising in cerebellar hemispheres (CH+ve) as very low risk and proposed that larger sagittal dimensions may be associated with a higher CMS risk. METHODS: An international multi-centre workshop assembled clinical, imaging and surgical data from 242 posterior fossa tumour patients from 9 centres (age 2-23.8, mean 8.23 years; histology 122 medulloblastoma, 75 pilocytic astrocytoma, 25 ependymoma, 18 other tumour types, 2 not known). Post-operative CMS developed in 0/66 CH+ve and 40/176 CH-ve patients (23%, range 13-33%) and. Tumour measurements in the sagittal plane were incorporated into the predictive model. RESULTS: In CH-ve tumours, CMS was associated higher D-Sag (CMS+ve 3.91±1.08 vs CMS-ve 3.01±1.56 cm p=0.001), d-Sag (CMS+ve 0.63±0.27 vs CMS-ve 0.44±0.31 cm p=0.001) and D-Sag*d-Sag (CMS+ve 2.60±1.36 vs CMS-ve 1.65±1.45 cm2 p=0.001). The Rotterdam model identified CMS rates for CH-ve tumours for low, intermediate and high risk groups as: 4% (2/45), 20% (16/79) and 42% (22/52), respectively. CONCLUSIONS: Incorporating D-sag and d-Sag estimates of tumour size, proposed, and adopted by the Beijing and Rotterdam groups, respectively, enhanced the objectivity and supports the model’s CMS predictive capacity for the purposes of consent and future trials. Higher CMS risk, associated with larger sagittal dimensions, may account for variability in CMS rates between cohorts and, as a new finding, may have broader clinical implications around the timing of diagnosis.

QOL-24. Evaluating the diagnostic validity &amp; predictive value of the Cerebellar Cognitive Affective Syndrome (CCAS) in pediatric posterior fossa tumour patients

Abstract OBJECTIVE: The present study aimed to examine the extent of deficits associated with the Cerebellar Cognitive Affective Syndrome (CCAS) in patients treated for paediatric posterior fossa tumours (PFT). Furthermore, we wanted to assess its diagnostic validity and predictive value for late sequelae. METHOD: Neuropsychological test data from 56 PFT patients treated at our pediatric neuro-onocology unit (mean age at treatment: seven years; mean age at first neuropsychological examination: nine years) were examined for severity of deficits in the CCAS core domains of executive functions, verbal functions, visuospatial abilities and emotions/behaviour. Patients with CCAS (n=20) were compared to two control groups without the syndrome (PFT patients treated either surgically (n=18) or surgically followed by radio/chemotherapy (n=18)) in terms of long-term neuropsychological and educational outcomes (mean age: 14 years). We also examined risk factors for CCAS development. RESULTS: The results showed mild to severe impairment in at least one CCAS domain in 55 out of 56 patients. Complete CCAS was diagnosed in 35.7% of cases. Seven years after tumour diagnosis, information processing, logical thinking, verbal functions, visuospatial skills and executive functions were more impaired in CCAS patients than in controls. They also needed more special educational support. Information processing speed was equally poor in CCAS patients and in controls treated with chemotherapy/radiotherapy. A significant association was found between risk factors and deficits in information processing speed, but not with the occurrence of CCAS. CONCLUSION: Deficits in the central CCAS features are common in PFT patients, but in varying degrees, suggesting that it is a continuous syndrome rather than a dichotomous one. However, it is problematic that domains such as information processing speed are not included in the diagnosis. External criteria regarding everyday life should be considered in future studies.

INSP-11. Patient-centered monitoring and care of cognitive late effects in pediatric brain tumor survivors with and without Cerebellar Mutism Syndrome (CMS).

Abstract Risks of cognitive late effects in children with brain tumors is well-established. However, group heterogeneity may limit generalizability and result in inadequate neurocognitive monitoring and care. Children with CMS have unique long-term cognitive risks and current knowledge may not adequately describe their experience or needs. The establishment and implementation of patient-centered, empirically-derived assessment of cognitive change following tumor diagnosis and treatment is a novel, precision-based approach to patient care. METHODS: 30 patients diagnosed with posterior fossa tumor who had at least 2 neuropsychological evaluations were included. Primary outcomes were processing speed (Wechsler PSI) and working memory (WMI). Secondary outcomes were verbal and nonverbal intellect. Reliable change indices (RCIs) were computed (Chelune et al., 1993). Using an 80% CI, 10% would be expected to decline, while 90% would stay stable or improve. RESULTS: Mean age in the full sample was 9.2 (3.32) years at T1 and 13.0 (4.11) years at T2, with an average of 2.7 (2.58) years between assessments. Males made up 56.7% of the sample. Five of the 30 (17%) were CMS+. The CMS+ patients were similar in age to CMS- patients, were predominantly male (80%), and mostly received photon radiation (60%). The full sample showed greater prevalence of declining verbal (21%), spatial (58%) and working memory (29%) functions than the expected (10%). The CMS+ group had higher rates of processing speed (50% v. 7%) and working memory (50% v. 27%) declines than the CMS- group. In contrast, the CMS- group appeared to show more prevalent declines in spatial reasoning than the CMS+ group (67% v. 33%). CONCLUSIONS: There appear to be unique domains impacted in the CMS+ group, which can convey specific long-term outcomes specific to this subgroup of patients. These results highlight the utility of individualized, empirically-driven analysis of change in cognitive functioning in this patient population.

Clival Chondroma with Brainstem Invasion Findings in MRI: A Case Report

Chordoma is a rare neoplasm derived from remnants of the fetal notochord; the most commun locations are the lumbosacral spineand the clivus. Clival chordomas represent 35 to 40% of cases and are very difficult to treat because of the localization and high recurrence rate. We present the case of 60 years old male patient with no previous history consulting for diplopia and headach that worsened in the last week before the diagnosis, with a walking ability disorder. Neurological examination found a raised intracranial pressure syndrome with static cerebellar syndrome. However, he presented left hemifacial numbness due to left fifth cranial nerve palsy and diplopia at left gaze due to left sixth cranial nerve palsy. The diagnosis was made by MRI using the different sequences especially T1 SE, T1 GE, T2 FLAIR, DWI and spectroscopy.

Cognitive Syndromes Associated With Movement Disorders.

This article reviews the recognition and management of cognitive syndromes in movement disorders, including those with parkinsonism, chorea, ataxia, dystonia, and tremor. Cognitive and motor syndromes are often intertwined in neurologic disorders, including neurodegenerative diseases such as Parkinson disease, atypical parkinsonian syndromes, Huntington disease, and other movement disorders. Cognitive symptoms often affect attention, working memory, and executive and visuospatial functions preferentially, rather than language and memory, but heterogeneity can be seen in the various movement disorders. A distinct cognitive syndrome has been recognized in patients with cerebellar syndromes. Appropriate recognition and screening for cognitive changes in movement disorders may play a role in achieving accurate diagnoses and guiding patients and their families regarding progression and management decisions. In the comprehensive care of patients with movement disorders, recognition of cognitive syndromes is important. Pharmacologic treatments for the cognitive syndromes, including mild cognitive impairment and dementia, in these movement disorders lag behind the therapeutics available for motor symptoms, and more research is needed. Patient evaluation and management require a comprehensive team approach, often linking neurologists as well as neuropsychologists, psychologists, psychiatrists, social workers, and other professionals.

Manic episode secondary to single right cerebellar metastasis in context of ovarian tumor: report of clinical case

IntroductionThe cerebellum is often overlooked when evaluating neuropsychiatric disorders. Lately, evidence has increased for the existence of cerebellar connections -generally in relation to vermis and posterior lobe- with cortical areas related to pathophysiology of psychiatric disorders. The cerebellar affective cognitive syndrome, also known as Schmahmann syndrome, has even been described with an evaluation scale.ObjectivesCase report of a patient suffering a manic episode in context of single right cerebellar metastasis derived from ovarian tumor.MethodsA non-systematic literature review was conducted on PubMed database on cerebellum pathology related to psychiatrics disorders. The clinical case report was prepared through the review of the patient´s clinical record.ResultsThe authors introduce the case of a 50-year-old woman, diagnosed with high-grade serous ovarian tumor, with single right cerebellar metastasis of 42mm, who was admitted to oncology due to behavioral alteration, with no prior psychiatric history. The patient showed hyperthymic mood, with speech scanned but fluid, manifesting intense well-being and ideation of mystical-religious and megalomaniac content. Haloperidol up to 7.5mg/8 hours and clonazepam 2 mg/8 hours were prescribed, switching haloperidol to olanzapine up to 25mg/day after several days, since the symptoms did not improve. Valproic acid 500 mg/24 hours was also added. Progressive improvement was seen, without worsening of motor symptoms or instability. The CCAS scale (Cerebellar Cognitive Affective/Schmahmann syndrome) was performed, with a positive result (10/10) being&gt; 3 definitive CCAS.ConclusionsThe relationship between cerebellum and neuropsychiatric disorders is still partly unknown, requiring more research to be able to carry out specific diagnoses and treatments for patients.DisclosureNo significant relationships.

A rare case of HIV encephalopathy presenting with an isolated cerebellar syndrome

Cerebellar dysfunction is a well-recognised but an infrequent complication of human immunodeficiency virus (HIV) infection. We present the case of a 44-year-old man living with HIV who presented with subacute cerebellar dysfunction and in whom a thorough diagnostic work-up did not identify any opportunistic infections. Cerebrospinal fluid (CSF) analysis showed a high HIV viral load of 1160 copies/ml and magnetic resonance imaging (MRI) showed multiple high signal abnormalities, disproportionately affecting the posterior fossa especially the cerebellum. This is a rare case of HIV encephalopathy presenting with an isolated cerebellar syndrome and highlights the importance of considering HIV as the aetiology in this clinical scenario.

067 Mycoplasma auto-immune mediated encephalitis

49 Year old male police officer, presented with acute onset fever, headache, arthralgia, ataxia, dysar- thria, opthalmoplegia, and diplopia, on a background of a 3 day skin erythematous skin rash, which was previously treated as SWEET syndrome. Physical examination further showed cerebellar syndrome, with no pyramidal signs. Blood tests showed raised inflammatory markers. Patient was initially treated for meningoencephalitis, with no change in symptoms after antibiotics and antivirals were administered. MRI revealed hyper intensity at the right pons and cerebellar peduncles, raising concerns of subacute infarct or demyelination. Lumbar puncture showed high protein of 1.15 mg/dL, and no other findings. However, when PCR was performed on the cerebrospinal fluid, mycoplasma pneumoniae was successfully isolated. IV prednisolone 1g was initially trialled, with no obvious therapeutic response after 5 days. As antibiotics and steroids had proved ineffective, decision was made to trial IV-IG. Two courses of IV-IG were trialled on suspicion of mycoplasma auto-immune mediated encephalitis, and symptoms subsequently resolved after 5 days. MRI was repeated after neurorehabilitation, and the hyper intensity found on the previous MRI had resolved completely. (Full Case Report Available on Request)d.mosh666@yahoo.com

Cerebellar syndrome associated with legionellosis: A case report and literature review

IntroductionLegionnaire's disease is a community-acquired pneumonia caused by the Gram-negative bacterium Legionella pneumophila. This disease is often associated with neurological symptoms, the clinical presentation of which can be very varied. Case reportWe report a 47-year-old female patient who developed Legionnaires’ disease with cerebellar symptoms (ataxia, dysarthria and hypermetria). Laboratory tests revealed a biological inflammatory syndrome. The cerebrospinal fluid was sterile. Urinary antigen test and serology were positive for L. pneumophila. An interstitial syndrome of the right upper lobe was detected on chest computed tomography (CT) scan. Brain imaging (magnetic resonance imaging and CT angiography) showed no abnormalities. The outcome was favourable after treatment with spiramycin, levofloxacin and corticosteroids. DiscussionFew cases only (n=110) of Legionnaires’ disease with cerebellar symptoms have been reported in the literature. The pathogenic mechanism behind neurological dysfunction in patients with Legionnaires’ disease is unknown. Neurological symptoms improve with antibiotic therapy and corticosteroids. Extra-pulmonary forms of Legionnaires’ disease are frequent, with neurological symptoms being the most common symptoms. Cerebellar dysfunction may be underestimated and requires appropriate management with antibiotic therapy and corticosteroid therapy. Recommendations for the management of Legionnaire's disease with severe extra-pulmonary symptoms are needed.

Association des manifestations neurologiques rares à West Nile virus chez un patient immunocompétent

BackgroundNeuro-invasive disease is the most dreaded complication of West Nile virus infection. We report the case of an immunocompetent patient who developed a meningeal syndrome associated with cerebellar ataxia, facial diplegia and hemiparesis caused by West Nile virus infection with a favourable outcome. ObservationA 42-year-old man was admitted for a febrile meningeal syndrome and confusion associated with cerebellar ataxia. The patient had developed facial diplegia, left hemiparesis with worsening cerebellar syndrome. He was diagnosed with neuro-invasive West Nile virus infection given the positive West Nile virus serologies in serum and cerebrospinal fluid as well as West Nile virus polymerase chain reaction in serum and urine. The outcome was favourable. ConclusionBecause of the increasing prevalence of West Nile virus, this infection should be suspected in the event of atypical neurological manifestations even in immunocompetent patients.

Altered Brain Volume, Microstructure Metrics and Functional Connectivity Features in Multiple System Atrophy.

In order to deeply understand the specific patterns of volume, microstructure, and functional changes in Multiple System Atrophy patients with cerebellar ataxia syndrome (MSA-c), we perform the current study by simultaneously applying structural (T1-weighted imaging), Diffusion tensor imaging (DTI), functional (BOLD fMRI) and extended Network-Based Statistics (extended-NBS) analysis. Twenty-nine MSA-c type patients and twenty-seven healthy controls (HCs) were involved in this study. First, we analyzed the whole brain changes of volume, microstructure, and functional connectivity (FC) in MSA-c patients. Then, we explored the correlations between significant multimodal MRI features and the total Unified Multiple System Atrophy Rating Scale (UMSARS) scores. Finally, we searched for sensitive imaging biomarkers for the diagnosis of MSA-c using support vector machine (SVM) classifier. Results showed significant grey matter atrophy in cerebellum and white matter microstructural abnormalities in cerebellum, left fusiform gyrus, right precentral gyrus and lingual gyrus. Extended-NBS analysis found two significant different connected components, featuring altered functional connectivity related to left and right cerebellar sub-regions, respectively. Moreover, the reduced fiber bundle counts at right Cerebellum_3 (Cbe3) and decreased fractional anisotropy (FA) values at bilateral Cbe9 were negatively associated with total UMSARS scores. Finally, the significant features at left Cbe9, Cbe1, and Cbe7b were found to be useful as sensitive biomarkers to differentiate MSA-c from HCs according to the SVM analysis. These findings advanced our understanding of the neural pathophysiological mechanisms of MSA from the perspective of multimodal neuroimaging.

Ovarian Teratoma-Related Paraneoplastic Neurological Syndromes.

Paraneoplastic neurological syndromes (PNSs) are a group of neurological disorders triggered by an underlying remote tumor. Ovarian teratoma (OT) is the most common histologic type of germ cell tumor in females. The most common PNSs associated with OT is anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis. However, with the increasing number of new antibodies reported over the last decade, the clinical spectrum of OT-related PNSs is also expanding. Our knowledge of OT-related PNSs is still far from complete. Here, we provide a comprehensive review of the most recent findings in the field of OT-related PNSs, with a particular focus on their clinical and pathological characteristics. Overall, the description of neuronal antibodies in PNSs associated with OT strongly suggests that antibodies may be responsible for the clinical symptoms in some cases. OT-related PNSs are associated with various clinical manifestations, including anti-NMDAR encephalitis, limbic encephalitis, encephalomyelitis, progressive cerebellar syndrome and opsoclonus-myoclonus syndrome. The pathological characteristics of the OT suggest that the mechanism of PNSs is probably due to heteromorphic neurons in the tumor tissue, the ectopic expression of the antigens in neural tissue within the teratomas and patients’ unusual immune response. Despite the severity of the neurological syndromes, most patients with OT-related PNSs showed good neurologic response to early tumor resection combined with immunotherapy. To further advance the management of OT-related PNSs, additional studies are needed to explore this complex topic.

Postinfectious Acute Cerebellar Syndromes in Children: A Nationally Ascertained Case Series From Australia 2013-2018.

Postinfectious acute cerebellar syndromes show a wide spectrum of acute severity and can occur with acute febrile illness or vaccine receipt. Varicella has historically been the most common cause, associated with up to 25% of cases in large cohorts. This study aimed to describe the spectrum of syndromes in a setting with high varicella vaccine coverage. Data were collected on children initially identified as "suspected encephalitis" subsequently designated "not-encephalitis" at participating children's hospitals in the Paediatric Active Enhanced Disease Surveillance (PAEDS) network, Australia, as part of the Acute Childhood Encephalitis study. A comprehensive descriptive analysis was undertaken on prospectively identified, national series of children with postinfectious acute cerebellar syndromes from 2013 to 2018. Cases were classified using a previously validated severity score, and the outcome was assessed at 12 months using the Liverpool Outcome Scale score. A total of 20 cases (65% were vaccinated for varicella) were included, of which 70% were subcategorized as acute cerebellar ataxia (ACA), 20% acute cerebellitis (AC), and 10% acute fulminant cerebellitis (AFC). An acute febrile illness was noted in 55% and none were related to varicella or were temporally related to varicella vaccination or other childhood vaccines. A subset (total of 7 children) followed up at 12 months all showed reduced Liverpool Outcome Scale scores. The study provides an overall description of this uncommon spectrum of neurologic syndromes and shows the infrequency of varicella zoster virus as a cause in a vaccinated population.

Pharyngolaryngeal semiology and prognostic factors in multiple system atrophy

IntroductionMultiple system atrophy (MSA) is a rare degenerative neurological disorder in adults. It induces parkinsonian and/or cerebellar syndrome associated with dysautonomia. Pharyngolaryngeal symptoms are common. Our aim is to describe the Pharyngolaryngeal semiology on one hand, and to ascertain whether the presence of these symptoms represents a prognostic factor for MSA on the other.MethodsThus, we carried out a retrospective, single-centre study, on a cohort receiving care at the centre of reference for MSA. The patients were referred for otorhinolaryngology assessment. The data was collected over the year 2020 with the help of computer software from the university hospital centre (UHC). Firstly, we described the Pharyngolaryngeal semiology specific to MSA by questioning patients, and by the results of nasofibroscopic examinations and swallowing tests. We then used multivariate analysis of variance to describe the prognostic factors of MSA progression (in UMSARS I and II points per month of progression) and survival (number of years between the first symptoms and death).ResultsThis study included a hundred and one patients and made it possible to define a Pharyngolaryngeal semiology profile of MSA, which is: a reduction in laryngeal mobility (primarily vocal cord abduction defects), abnormal movements (particularly at rest or when initiating a movement) and a defect in the protection mechanisms of the upper airways. The swallowing difficulties are moderate and the main mechanisms are delayed pharyngeal swallow and/or an oro-pharyngeal transport defect. In the multivariate analyses, the contributing factors are laryngeal anomalies, modification of solid food to fluid food and nutritional complication.ConclusionENT specialists should pay close attention to problems in the Pharyngolaryngeal dynamic and then consider a neurological cause. They can also itemize the clinical factors that could have a negative effect on the prognosis of the patient with MSA. Indeed, early detection makes it possible to provide care for respiratory and nutritional complications.

Efficacy and Safety of Amantadine in Cerebellar Mutism Syndrome - a Pediatric Case Series (P16-9.007)

Efficacy and Safety of Amantadine in Cerebellar Mutism Syndrome - a Pediatric Case Series (P16-9.007)