603 results on '"Bulbar palsy"'
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2. A Nerve Block Therapy for Bulbar Palsy Pharyngeal Dysphagia
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Babujinaya Cela, Director
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- 2024
3. Stellate Ganglion Block in Bulbar Palsy
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Copka Sonpashan, Research Director
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- 2024
4. The Effectiveness of Stellate Ganglion Block in Managing Dysphagia in Patients With Medullary Infarction
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Muhammad, Research Director
- Published
- 2024
5. Efficacy of Stellate Ganglion Block in Dysphagic Patients With Bulbar Palsy After Ischemic Stroke
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Zeng Changhao, Research Director
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- 2024
6. Brown-Vialetto-Van Laere syndrome.
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IMANNEZHAD, Shima, GHAYOOR KARIMIANI, Ehsan, SEZAVAR, Majid, KHADEMI, Gholam Reza, NASERI, Maryam, and ASHRAFZADEH, Farah
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BROWN-Vialetto-Van Laere syndrome ,NEUROLOGIC manifestations of general diseases ,VITAMIN B2 ,TREATMENT effectiveness ,HEARING disorders ,GENETIC mutation ,DIETARY supplements ,GENETIC testing ,SYMPTOMS - Abstract
Brown-Vialetto-Van Laere syndrome (BVVLS) is a rare neurodegenerative disorder of childhood. According to the previous reports, it has various primary signs and symptoms. Because of the simple treatment with riboflavin supplementation, it is important to have suspicious to this disease and begin treatment even before genetic test confirm. We report a five-year-old girl with BVVLS that manifest with hearing problems, first. There was obvious improvement in her disease clinical signs with riboflavin supplementation treatment. [ABSTRACT FROM AUTHOR]
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- 2024
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7. Motoric Automatisms of the Maxillofacial Region: a Lecture
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Konstantin B. Petrov, Natalya A. Ivonina, and Tatiana V. Mitichkina
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kinesitherapy ,bulbar palsy ,pseudobulbar palsy ,facial nerve ,synkinesis ,brachiofacial region ,Medicine (General) ,R5-920 ,Sports medicine ,RC1200-1245 - Abstract
INTRODUCTION. Effective kinesitherapy of peripheral and central paralysis of the mimic, masticatory, lingual and pharyngeal muscles requires in-depth study of the features of the cooperative work of these muscles in the norm and in pathology. AIM. To acquaint rehabilitation specialists with motor automatisms of the brachiofacial area and show the principles of their practical use. MATERIAL AND METHODS. We studied more than 70 literature sources on involuntary activity of the maxillofacial region and adjacent regions muscles as well as we summarized our own experience in rehabilitation of patients with peripheral facial neuropathies and with bulbar and pseudobulbar syndromes RESULTS AND DISCUSSION. The study showed that the face, neck, and arm are involved in various mesencephalic-bulbar automatisms, both normal (ontogenetic and psychosomatic synkinesia, postural-ocular and postural-mandibular reflexes) and pathological (trunk eye-facial synkinesia, oral automatic reflexes), much more closely than other body parts. All of them, with the exception of facial hyperkinesias, may serve as a basis for optimizing kinesitherapy methods for movement disorders of the brachiofacial region. CONCLUSION. The face, neck, and hand are closely involved in a variety of stem and subcortical automatisms. The better the physical therapist is familiar with the features of the existing or potentially available to the patient reflex activity in the part of the body being rehabilitated, the more effective the rehabilitation treatment will be. The face, neck and arm are closely involved in a variety of stem and subcortical automorphisms. The better a doctor or an instructor in exercise therapy is acquainted with the reflex activity present or potentially available in the part of the body to be rehabilitated, the more effective the rehabilitation treatment will be.
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- 2022
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8. A rare case of rebleeding brainstem cavernoma in a 5-month-old-girl.
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Ndandja, Dimitri T. K., Musa, Gerald, Nosov, Ilya, Chmutin, Gennady E., Livshitz, Matvey I., Annanepesov, Nazar S., and Mwela, Bupe Mumba
- Abstract
Background: Brainstem cavernomas (BSCs) are relatively rare intracranial vascular lesions that, if left untreated, can be devastating to the patient. The lesions are associated with a myriad of symptoms, depending on their size and location. However, medullary lesions present acutely with cardiorespiratory dysfunction. We present the case of a 5-month-old child with a BSC. Case Description: A 5-month-old child presented for the 2nd time with sudden respiratory distress and excessive salivation. On the first presentation, brain magnetic resonance imaging (MRI) showed a 13 × 12 × 14 mm cavernoma at the pontomedullary junction. She was managed conservatively but presented 3 months later with tetraparesis, bulbar palsy, and severe respiratory distress. A repeat MRI showed enlargement of the cavernoma to 27 × 28 × 26 mm with hemorrhage in different stages. After hemodynamic stabilization, complete cavernoma resection was performed through the telovelar approach with neuromonitoring. Postoperatively, the child recovered motor function, but the bulbar syndrome persisted with hypersalivation. She was discharged on day 55 with a tracheostomy. Conclusion: BSCs are rare lesions that are associated with severe neurological deficits due to the compactness of important cranial nerve nuclei and other tracts in the brainstem. Early surgical excision and hematoma evacuation for superficially presenting lesions can be lifesaving. However, the risk of postoperative neurological deficits is still a major concern in these patients. [ABSTRACT FROM AUTHOR]
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- 2023
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9. Disease duration of progression is helpful in identifying isolated bulbar palsy of amyotrophic lateral sclerosis
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Huagang Zhang, Lu Chen, Jinzhou Tian, and Dongsheng Fan
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Bulbar palsy ,Amyotrophic lateral sclerosis ,Survival ,Motor neuron ,Prognosis ,Neurology. Diseases of the nervous system ,RC346-429 - Abstract
Abstract Background Compared with typical bulbar onset amyotrophic lateral sclerosis (ALS), isolated bulbar palsy (IBP), an often under-understood variant of ALS, is characterized by symptoms confined to bulbar region for extended periods and relative preservation of limb and ventilation function. To find a cutoff value of disease duration that can distinguish IBP from typical bulbar onset ALS well, the association of survival with disease progression in bulbar onset ALS patients was analyzed. Methods Clinical data of bulbar onset ALS patients were collected from January 2009 to December 2013. The duration from bulbar onset to first significant limb involvement was analyzed by a cutoff point analysis with maximally selected log-rank statistics and dichotomized to categorize patient outcomes. The patients were divided into two groups, the IBP and typical bulbar onset ALS groups, according to the cutoff value. Clinical features were compared. Results 115 bulbar onset ALS patients were recruited, and the duration from bulbar onset to first significant limb involvement was associated with survival (P
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- 2021
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10. Effectiveness of Intravenous Cyclophosphamide in a Patient With Anti-amphiphysin Autoimmunity Presenting With Bulbar Palsy and Cerebellar Ataxia: A Case Report.
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Nomoto J, Takatsu H, Yoshida K, Matsuzawa H, and Omoto S
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Anti-amphiphysin antibody is a rare paraneoplastic autoantibody. A case of a 74-year-old man with anti-amphiphysin antibody and multiple symptoms, including bulbar palsy along with cerebellar ataxia, who responded to treatment with intravenous cyclophosphamide is reported. The patient presented with progressive unsteady gait and difficulty in swallowing food and water for three months. On admission, he had severe ataxia, downbeat and horizontal nystagmus, dysarthria, dysphagia, loss of tendon reflexes, and dysuria. Anti-amphiphysin antibodies were detected in the serum, resulting in the diagnosis of non-stiff anti-amphiphysin syndrome. No significant abnormalities were observed in imaging studies of the brain and the whole body. The patient was treated with high-dose intravenous immunoglobulin and steroids, yielding only slight improvement. After two courses of intravenous cyclophosphamide pulse therapy, his neurological symptoms, notably dysphagia and cerebellar ataxia, improved. Follow-up computed tomography and fluorodeoxyglucose-positron emission tomography/computed tomography showed enlarged mediastinal lymph nodes and hypermetabolic uptake of F-18 fluorodeoxyglucose six months after the onset of the neurological symptoms. Histological examination of a lymph node showed metastatic small cell lung cancer. This case highlights the efficacy of cyclophosphamide as second-line immunotherapy for anti-amphiphysin syndrome., Competing Interests: Human subjects: Consent was obtained or waived by all participants in this study. Conflicts of interest: In compliance with the ICMJE uniform disclosure form, all authors declare the following: Payment/services info: All authors have declared that no financial support was received from any organization for the submitted work. Financial relationships: All authors have declared that they have no financial relationships at present or within the previous three years with any organizations that might have an interest in the submitted work. Other relationships: All authors have declared that there are no other relationships or activities that could appear to have influenced the submitted work., (Copyright © 2024, Nomoto et al.)
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- 2024
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11. SLC52A2 [p.P141T] and SLC52A3 [p.N21S] causing Brown-Vialetto-Van Laere Syndrome in an Indian patient: First genetically proven case with mutations in two riboflavin transporters
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Udhayabanu, Tamilarasan, Subramanian, Veedamali S, Teafatiller, Trevor, Gowda, Vykuntaraju K, Raghavan, Varun S, Varalakshmi, Perumal, Said, Hamid M, and Ashokkumar, Balasubramaniem
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Biological Sciences ,Biomedical and Clinical Sciences ,Genetics ,2.1 Biological and endogenous factors ,Aetiology ,Adolescent ,Bulbar Palsy ,Progressive ,Cells ,Cultured ,Hearing Loss ,Sensorineural ,Humans ,India ,Membrane Transport Proteins ,Mutation ,Receptors ,G-Protein-Coupled ,Riboflavin ,hRFVF-2 ,hRFVT-3 ,BWLS ,Motor neuronopathy ,BVVLS ,hRFVT-2 ,Clinical Sciences ,General Clinical Medicine ,Biochemistry and cell biology ,Clinical sciences - Abstract
BackgroundBrown-Vialetto-Van Laere Syndrome (BVVLS), a rare neurological disorder characterized by bulbar palsies and sensorineural deafness, is mainly associated with defective riboflavin transporters encoded by the SLC52A2 and SLC52A3 genes.MethodsHere we present a 16-year-old BVVLS patient belonging to a five generation consanguineous family from Indian ethnicity with two homozygous missense mutations viz., c.421C>A [p.P141T] in SLC52A2 and c.62A>G [p.N21S] in SLC52A3.ResultsFunctional characterization based on 3H-riboflavin uptake assay and live-cell confocal imaging revealed that the effect of mutation c.421C>A [p.P141T] identified in SLC52A2 had a slight reduction in riboflavin uptake; on the other hand, the c.62A>G [p.N21S] identified in SLC52A3 showed a drastic reduction in riboflavin uptake, which appeared to be due to impaired trafficking and membrane targeting of the hRFVT-3 protein.ConclusionsThis is the first report presenting mutations in both riboflavin transporters hRFVT-2 and hRFVT-3 in the same BVVLS patient. Also, c.62A>G [p.N21S] in SLC52A3 appears to contribute more to the disease phenotype in this patient than c.421C>A [p.P141T] in SLC52A2.
- Published
- 2016
12. Amyloid cranial polyneuropathy: A rare neurological presentation of immunoglobulin light‐chain amyloidosis
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Abhinav Agarwal, Benny Paul Wilson, Prasad Kuruvilla Mathews, Surekha Viggeswarpu, and Gopinath Kango Gopal
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amyloidosis ,bulbar palsy ,cranial polyneuropathy ,plasma cell dyscrasia ,Geriatrics ,RC952-954.6 - Abstract
Abstract Amyloidosis, a disease with extracellular tissue deposition of fibrils, results in clinical manifestations based on deposition of these fibrils in multiple organ systems. Usual manifestations include nephrotic‐range proteinuria, cardiac failure, hepatosplenomegaly, and skin manifestations. Common neurological manifestations include peripheral and autonomic neuropathies. Cranial neuropathy has been seldom reported and is an unusual clinical feature of amyloidosis. Here, we report an older man who presented with cranial nerve palsies along with other clinical features, including heart failure, proteinuria, weight loss, anorexia and distal symmetric polyneuropathy and was diagnosed with immunoglobulin light‐chain (AL) amyloidosis.
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- 2021
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13. West Nile Meningoencephalitis Presenting as Isolated Bulbar Palsy With Hypercapnic Respiratory Failure
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Tso, Geoffrey, Kaldas, Kirsten, Springer, Joseph, Barot, Nikhil, and Kamangar, Nader
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Prevention ,Rare Diseases ,West Nile Virus ,Perinatal Period - Conditions Originating in Perinatal Period ,Pediatric ,Infectious Diseases ,Vaccine Related ,Emerging Infectious Diseases ,Vector-Borne Diseases ,Good Health and Well Being ,Bulbar Palsy ,Progressive ,Humans ,Hypercapnia ,Male ,Middle Aged ,Respiratory Insufficiency ,United States ,West Nile Fever ,West Nile virus ,bulbar palsy ,neuromuscular weakness ,respiratory failure ,Clinical Sciences ,Nursing ,Emergency & Critical Care Medicine - Abstract
BackgroundSince the outbreak of West Nile virus (WNV) in the United States in 1999, the WNV neuroinvasive disease has been increasingly reported with a wide spectrum of neuromuscular manifestations.CaseWe submit a case of a 46-year-old male with a history of alcohol abuse, diabetes, hypertension, and hepatitis C who presented with fever, nausea, shortness of breath, and dysphagia. The patient rapidly developed hypercapnic respiratory failure and was found to have WNV meningoencephalitis without obvious neuromuscular weakness. His hospital course was significant for repeated failures of extubation secondary to persistent bulbar weakness eventually requiring tracheotomy.ConclusionThis is a unique case of WNV meningoencephalitis with bulbar palsy without other neuromuscular manifestations resulting in recurrent hypercapnic respiratory failure.
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- 2016
14. Disease duration of progression is helpful in identifying isolated bulbar palsy of amyotrophic lateral sclerosis.
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Zhang, Huagang, Chen, Lu, Tian, Jinzhou, and Fan, Dongsheng
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AMYOTROPHIC lateral sclerosis , *PROGRESSIVE supranuclear palsy , *DISEASE duration , *DISEASE progression , *SURVIVAL rate , *LOG-rank test - Abstract
Background: Compared with typical bulbar onset amyotrophic lateral sclerosis (ALS), isolated bulbar palsy (IBP), an often under-understood variant of ALS, is characterized by symptoms confined to bulbar region for extended periods and relative preservation of limb and ventilation function. To find a cutoff value of disease duration that can distinguish IBP from typical bulbar onset ALS well, the association of survival with disease progression in bulbar onset ALS patients was analyzed.Methods: Clinical data of bulbar onset ALS patients were collected from January 2009 to December 2013. The duration from bulbar onset to first significant limb involvement was analyzed by a cutoff point analysis with maximally selected log-rank statistics and dichotomized to categorize patient outcomes. The patients were divided into two groups, the IBP and typical bulbar onset ALS groups, according to the cutoff value. Clinical features were compared.Results: 115 bulbar onset ALS patients were recruited, and the duration from bulbar onset to first significant limb involvement was associated with survival (P < 0.001). The cutoff duration was 20 months. 19 patients were identified as IBP and 96 patients as typical bulbar onset ALS using 20 months as the cutoff duration. Female was more common, limb weakness was less frequent and pure upper motor neuron (UMN) bulbar signs were more frequent in the IBP group than in the typical bulbar onset ALS group (P = 0.047; P = 0.004; P = 0.031). The median survival time of the IBP group was significantly longer than that of the typical bulbar onset ALS group (64 months and 26 months, respectively; P < 0.001).Conclusions: A cutoff duration of 20 months from bulbar onset to first significant limb involvement may be used to specifically distinguish IBP from typical bulbar onset ALS. IBP was characterized by female predominance, relative preservation of limb function, more pure UMN bulbar signs and a relatively benign prognosis. [ABSTRACT FROM AUTHOR]- Published
- 2021
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15. Clinical Profile and Predictors of Mechanical Ventilation in Guillain-Barre Syndrome in North Indian Children.
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Tiwari, Ishita, Alam, Areesha, kanta, Chandra, Koonwar, Sciddhartha, Garg, Ravindra Kumar, Pandey, Shweta, Jain, Amita, and Kumar, Rashmi
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GUILLAIN-Barre syndrome , *ARTIFICIAL respiration , *MOTOR neuron diseases , *MEDICAL research , *POLYNEUROPATHIES , *HOSPITAL care of children - Abstract
Objective: To describe the clinical-laboratory profile of pediatric Guillain-Barre syndrome and delineate features associated with need of mechanical ventilation. Methods: In a prospective observational study at tertiary care hospital, clinical-laboratory assessment and nerve conduction studies were documented in consecutive children hospitalized with Guillain-Barre syndrome according to Brighton criteria. Clinical-laboratory features were compared between ventilated and nonventilated patients using univariate and multivariate analysis. Results: Forty-six children (27 boys) with a mean age of 69.1±35.2 months were enrolled. History of preceding infection was present in 47.8%, bulbar palsy in 43.5%, feeble voice in 41.3%, sensory involvement in 13%, and autonomic involvement in 39.5%. Tetraparesis was noted in 87% of cases. Hughes disability scale >3 was noted in 44 children at admission and 39 (84.7%) at discharge. The most common electrophysiological type was acute motor axonal neuropathy (46.5%) followed by acute motor sensory axonal neuropathy (39.5%), acute inflammatory demyelinating polyneuropathy (7%), and inexcitable nerves (7%). Nine (19.7%) children were ventilated, 3 (6.5%) died or were lost, and 43 were discharged. Factors associated with need of mechanical ventilation on univariate analysis were older age, hypertension, bulbar palsy, feeble voice, lower Medical Research Council (MRC) sum, raised total leucocyte count, and history of preceding infection. Logistic regression revealed older age, history of predisposing illness, lower MRC sum at presentation, and bulbar palsy as independent predictors of mechanical ventilation. Conclusions: The most common electrophysiological subtype in northern Indian children is acute motor axonal neuropathy. Older age, preceding infection, low MRC sum, and bulbar palsy are predictors of mechanical ventilation in pediatric Guillain-Barre syndrome. [ABSTRACT FROM AUTHOR]
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- 2021
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16. TIME-FREQUENCY ANALYSIS OF SPEECH SIGNALS IN THE DIAGNOSIS OF BULBAR PALSY
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M. M. Mezhennaya, Yu. N. Rushkevich, and A. A. Boriskevich
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speech signal ,time-frequency analysis ,main tone frequency ,bulbar syndrome ,bulbar palsy ,Electronics ,TK7800-8360 - Abstract
The method of qualitative and quantitative differential diagnosis of bulbar palsy has been offered on the basis of digital processing of speech signals. The software with the graphic user interface has been developed by authors for implementation of this method which allows to increase the accuracy and speed of diagnosis.
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- 2019
17. Amyloid cranial polyneuropathy: A rare neurological presentation of immunoglobulin light‐chain amyloidosis.
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Agarwal, Abhinav, Paul Wilson, Benny, Kuruvilla Mathews, Prasad, Viggeswarpu, Surekha, and Kango Gopal, Gopinath
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POLYNEUROPATHIES ,CARDIAC amyloidosis ,AMYLOIDOSIS ,AMYLOID ,CUTANEOUS manifestations of general diseases ,HEART failure - Abstract
Amyloidosis, a disease with extracellular tissue deposition of fibrils, results in clinical manifestations based on deposition of these fibrils in multiple organ systems. Usual manifestations include nephrotic‐range proteinuria, cardiac failure, hepatosplenomegaly, and skin manifestations. Common neurological manifestations include peripheral and autonomic neuropathies. Cranial neuropathy has been seldom reported and is an unusual clinical feature of amyloidosis. Here, we report an older man who presented with cranial nerve palsies along with other clinical features, including heart failure, proteinuria, weight loss, anorexia and distal symmetric polyneuropathy and was diagnosed with immunoglobulin light‐chain (AL) amyloidosis. [ABSTRACT FROM AUTHOR]
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- 2021
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18. A CADASIL Case Presenting with Progressive Bulbar Palsy Caused by Acute Simultaneous Multiple Subcortical Infarcts
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Elif GÖKÇAL, Mehmet KOLUKISA, Nihat MUSTAFAYEV, and Talip ASİL
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CADASIL ,simultaneous infarcts ,progression ,bulbar palsy ,Medicine (General) ,R5-920 - Abstract
Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is an adult-onset inherited small vessel disease of the brain caused by NOTCH3 mutations. Clinical characteristics of CADASIL include recurrent infarctions, migraine with aura, mood disturbances and cognitive impairment. We report a 35 year-old migraine-free patient with unusual presentation with acute simultaneous multiple subcortical infarctions causing progressive bulbar palsy as the initial manifestation. Hepresented with slurred speech progressing to anarthria and bilateral palsy of lower motor cranial nerves within 24 hour. Initial acute subcortical infarcts enlarged and new infarcts developed in bilateral hemispheres. There was diffuse leukoencephalopathy involving temporal horns and the patient had positive familiy history of migraine and young stroke. CADASIL was diagnosed genetically when NOTCH3 mutationwas shown. The patient improved after treatment with liquid hydration and antiplatelet agent. We experienced with the patient that CADASIL may present with unusual progressive manifestations and bilateral simultaneous infarctions. Irrespective of clinical presentation, patients with leukoencephalopathy and positive familiy history should be tested for NOTCH3 mutation.
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- 2019
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19. Differences in nerve excitability properties between isolated bulbar palsy and bulbar-dominant amyotrophic lateral sclerosis.
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Pyun, So Young, Kim, Yerim, Oh, Seong-Il, and Bae, Jong Seok
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AMYOTROPHIC lateral sclerosis ,PARALYSIS ,NERVES - Abstract
Objectives: Isolated bulbar palsy (IBP) is a rare variant that can show a benign course, while progressive bulbar palsy (PBP) has been regarded as a bulbar-dominant type of classical amyotrophic lateral sclerosis (cALS). This study aimed to identify differences in the excitability properties between them. Methods: We consecutively collected data on 22 ALS patients: 13 with cALS, 5 with PBP, and 4 with IBP. An automated nerve excitability test (NET) was applied to measure the strength–duration time constant, threshold electrotonus (TE), current–threshold relationship, and recovery cycle. The axonal excitability properties were compared between the ALS groups and 25 controls. Results: Compared to controls, the cALS group showed a greater change in the depolarizing phase of TE of 90–100 ms after depolarizing current [TEd
(90–100) ] (53.3±1.3 [mean±SEM] for cALS and 49.0±0.7 for control, P<0.01) and lower S2 accommodation (19.6±0.8 and 22.6±0.7, respectively; P=0.01). There was a nonsignificant tendency for a high TEd(90–100) pattern to be less prominent in the IBP group than in the PBP group (51.5±4.22 and 48.8±1.5, respectively). In addition, all of the parameters of nerve excitability other than S2 accommodation in the PBP and IBP groups did not differ significantly from those in the controls. Conclusions: The excitability properties of IBP and PBP differ from those of cALS. The pattern of NET in PBP was more similar to that in cALS than that in IBP. These findings suggest that IBP is a different entity from bulbar-dominant ALS and PBP. [ABSTRACT FROM AUTHOR]- Published
- 2020
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20. Spirometry using facemask versus conventional tube in patients with neuromuscular disorders
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Taha Taha Abdelgawad, Ahmed Mohammed Abumossalam, Dina Abouelkheir Abdalla, and Mohamed Elsayed Mahmoud Elsayed
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Neuromuscular disorders ,Bulbar palsy ,Facemask ,Tube spirometry ,PImax ,PEmax ,Diseases of the respiratory system ,RC705-779 - Abstract
Background: Respiratory derangement is a major health hazard in patients with neuromuscular disorders (NMD). This study aimed to compare spirometry results using facemask with that of conventional tube in patients with NMD and accordingly possible use of facemask instead of conventional tube in this group of patients. Methods: Thirty-six patients with NMD and fifty normal volunteers were recruited within 9 months. Assessment of patients included history and physical examination specially presence of respiratory symptoms, duration and stage of neuromuscular illness, bulbar symptoms, sensory, motor and cranial nerve assessment. Then all participants were subjected to spirometry and respiratory pressures assessment using both conventional tube and facemask. Results: Spirometry and respiratory pressure assessment using facemask versus tube measured in all participants. For patients with NMD; spirometric measures PImax and PEmax were higher in tube than facemask. FEV1, FEV1%, FEV1/FVC %, and PImax showed statistically significant difference. For patients with bulbar palsy; SVC was higher in facemask without statistically significant difference. For other spirometric measures, PImax and PEmax; using tube was higher than facemask with only FEV1, FEV1% showed statistically significant difference (p = 0.023 and 0.015 respectively). For patients without bulbar palsy; all spirometric measures, PImax and PEmax were higher when using tube with only FEV1/FVC % showed statistically significant difference (p = 0.004). Also for control group; data were higher in tube with only FEV1 showed statistically significant difference (p = 0.031). For patients with NMD; fractional functional defect (FFD) was very high for PImax and PEmax. Conclusion: The use of facemask during assessment of spirometric and respiratory pressures measures in patients with NMD and more specifically in patients with bulbar palsy is promising and can be considered a good alternative to conventional tube assessment for functional screening rationale.
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- 2017
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21. Pharyngeal–Cervical-Brachial Variant: A Rare Form of Guillain–Barre Syndrome in a Paediatric Patient.
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Kohona Jayasinghearachchi, Tilani Maheshika and Peiris, Sakunthala
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CHILD patients , *GUILLAIN-Barre syndrome , *PHYSICIANS , *DIAGNOSIS , *ATAXIA - Abstract
This case report describes a successfully managed rare variant of Guillain-Barre´ Syndrome (GBS), the pharyngeal-cervical-brachial (PCB) variant, in a 5-year-old child which is characterized by the involvement of oropharyngeal, neck and upper limb muscles, without disturbed consciousness or ataxia. Since early treatment and supportive care yield successful outcome, it is important for the treating physician to be familiar with other diseases that mimic the disease and variants for prompt and accurate diagnosis. [ABSTRACT FROM AUTHOR]
- Published
- 2021
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22. Clinical Features of Isolated Bulbar Palsy of Amyotrophic Lateral Sclerosis in Chinese Population
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Hua-Gang Zhang, Lu Chen, Lu Tang, Nan Zhang, and Dong-Sheng Fan
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Amyotrophic Lateral Sclerosis ,Bulbar Palsy ,Prognosis ,Vital Capacity ,Medicine - Abstract
Background: Progressive bulbar palsy (PBP) is a classic phenotype of bulbar onset amyotrophic lateral sclerosis (ALS) with more rapid progression and worse prognosis. However, as an often under-understood variant of ALS, isolated bulbar palsy (IBP) appears to progress more slowly and has a relatively benign prognosis. This study aimed to investigate the natural course and clinical features of IBP in Chinese population and to compare them with those of PBP. Methods: The clinical data of patients with bulbar onset ALS were collected from January 2009 to December 2013. Revised ALS Functional Rating Scale (ALSFRS-R), forced vital capacity (FVC), and follow-up evaluation were performed, and the differences in basic clinical features, ALSFRS-R, FVC, and primary outcome measures between IBP and PBP were analyzed. The independent t-test, Chi-square test, Mann-Whitney U-test, and Kaplan-Meier analysis were used. Results: Totally 154 patients with bulbar onset ALS were categorized into two groups, 33 with IBP and 121 with PBP. In the IBP group, the male to female ratio was 0.7 to 1.0, and the mean onset age was 58.5 years. The mean duration from the onset was 16.0 months, and the mean ALSFRS-R score was 43.4 at patients' first visit to our hospital. In 14 IBP patients performing FVC examination, the mean FVC value was 90.5% and there were only two cases with abnormal FVC. In 26 IBP patients completing follow-up, 15 (58%) suffered death or tracheotomy and the mean survival time was 40.5 months. Significant differences were noted in sex ratio, onset age, ALSFRS-R score, upper motor neuron limb signs, pure lower motor neuron (LMN) bulbar signs, FVC, and survival time between IBP and PBP. Conclusions: IBP was evidently different from PBP, which was characterized with the predominance of female, pure LMN bulbar signs, an older onset age, a relative preservation of respiratory function, and a better prognosis.
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- 2017
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23. Anti-GQ1b Antibody Syndrome Presented as Locked-In Syndrome: A Rare Case Report.
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D'Angelo KM, Williams J, and Wu L
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Anti-GQ1b antibodies are considered a hallmark of Miller-Fisher syndrome (MFS), a rare variant of Guillain-Barré syndrome (GBS). The typical clinical presentation of MFS includes ophthalmoplegia, ataxia, and areflexia. Here, we present an unusual case of a 65-year-old man with acute-onset quadriplegia and bulbar weakness resembling locked-in syndrome. Imaging studies did not show structural lesions as a cause for his clinical symptoms. Nerve conduction studies showed severe axonal sensory-motor polyneuropathy. Serum studies were all negative except for a positive anti-GQ1b antibody. He was treated with plasmapheresis as MFS, with a quick improvement in muscle strength. Our case report provided further information on the clinical variation of anti-GQ1b syndrome. Physicians should pay more attention to unusual presentations of anti-GQ1b syndrome because, when it is recognized early with prompt treatment, patients are expected to have a good recovery., Competing Interests: The authors have declared that no competing interests exist., (Copyright © 2023, D'Angelo et al.)
- Published
- 2023
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24. Flavin adenine dinucleotide synthase deficiency due to FLAD1 mutation presenting as multiple acyl-CoA dehydrogenation deficiency-like disease: A case report.
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Yamada, Kenji, Ito, Michinori, Kobayashi, Hironori, Hasegawa, Yuki, Fukuda, Seiji, Yamaguchi, Seiji, and Taketani, Takeshi
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FLAVIN adenine dinucleotide , *ASPIRATION pneumonia , *ACYL coenzyme A , *REPORTING of diseases , *RESPIRATORY aspiration , *LACTIC acidosis - Abstract
Multiple acyl-CoA dehydrogenase deficiency (MADD), also known as glutaric acidemia type II, is classically caused by a congenital defect in electron transfer flavoprotein (ETF) or ETF dehydrogenase (ETFDH). Flavin adenine dinucleotide synthase (FADS) deficiency caused by mutations in FLAD1 was recently reported as a novel riboflavin metabolism disorder resembling MADD. Here, we describe a Japanese boy with FADS deficiency due to a novel mutation (p.R249*) in FLAD1. In the asymptomatic male infant born at full term, newborn screening showed positive results with elevated C5 and C14:1 acylcarnitine levels and an increased C14:1/C2 ratio. Biochemical studies were unremarkable except for lactic acidosis (pH 7.197, lactate 61 mg/dL). A diagnosis of MADD was suspected because of mild abnormalities of the acylcarnitine profile and apparent abnormalities of urinary organic acids, although mutations in the ETFA , ETFB , ETFDH , and riboflavin transporter genes (SLC52A1 , SLC52A2 , and SLC52A3) were not detected. Administration of riboflavin and L-carnitine was initiated at one month of age based on the diagnosis of "biochemical MADD" despite a lack of symptoms. Nevertheless, the acylcarnitine profile was not normalized. Symptoms resembling bulbar palsy, such as vocal cord paralysis and dyspnea with stridor, were present from 3 months of age. At 4 months of age, he became bedridden because of hypoxic-ischemic encephalopathy due to fulminant respiratory failure with aspiration pneumonia. At 2 years and 5 months of age, a homozygous c.745C > T (p.R249*) mutation in the FLAD1 gene was identified, confirming the diagnosis of FADS deficiency. His severe clinical course may be caused by this nonsense mutation associated with poor responsiveness to riboflavin. Persistent lactic acidosis and neuropathy, such as bulbar palsy, may be important for diagnosing FADS deficiency. Although the biochemical findings in FADS deficiency are similar to those in MADD, their clinical symptoms and severity may not be identical. [ABSTRACT FROM AUTHOR]
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- 2019
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25. Cough peak flow decline rate predicts survival in patients with amyotrophic lateral sclerosis.
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Matsuda, Chiharu, Shimizu, Toshio, Nakayama, Yuki, and Haraguchi, Michiko
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AMYOTROPHIC lateral sclerosis treatment , *AMYOTROPHIC lateral sclerosis , *ARTIFICIAL respiration , *COMPARATIVE studies , *COUGH , *RESEARCH methodology , *MEDICAL cooperation , *NONPARAMETRIC statistics , *RESEARCH , *RESPIRATORY measurements , *SURVIVAL analysis (Biometry) , *TRACHEOTOMY , *EVALUATION research , *BODY mass index , *RETROSPECTIVE studies , *SEVERITY of illness index , *DISEASE complications - Abstract
Introduction: In this study we investigated the prognostic significance of cough peak flow (CPF) decline rate in patients with amyotrophic lateral sclerosis (ALS).Methods: Thirty-four patients with ALS participated in our investigation. We examined CPF, forced vital capacity (FVC), body mass index, and the revised ALS Functional Rating Scale (ALSFRS-R), and followed patients to death, tracheostomy, or non-invasive ventilator dependency. We analyzed the relationship between the rate of decline of each parameter and survival prognosis.Results: The CPF decline rate significantly correlated with the decline rates of the ALSFRS-R bulbar score (P < 0.0001) and FVC (P < 0.0001). Patients with a CPF decline rate ≥25% had shorter overall survival (P < 0.0001). Cox model multivariate analyses showed that the CPF decline rate was an independent prognostic factor for survival (P = 0.021).Discussion: The CPF decline rate reflects the progression of bulbar and respiratory dysfunction and predicts survival. Muscle Nerve 59:168-173, 2019. [ABSTRACT FROM AUTHOR]- Published
- 2019
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26. Gender differences in clinical features at the initial examination of late-onset amyotrophic lateral sclerosis.
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Yamashita, Satoshi, Tawara, Nozomu, Hara, Kentaro, and Ueda, Mitsuharu
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- *
AMYOTROPHIC lateral sclerosis , *VITAL capacity (Respiration) , *MUSCLE mass , *BODY mass index , *OLDER patients - Abstract
Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease that mainly affects motor neurons in the brain and spinal cord. With the advent of aging societies, the proportion of elderly patients with ALS is expected to increase. We retrospectively compared the clinical characteristics at the initial examination of patients with onset of ALS at age 74 years or younger (early onset) and those aged 75 years or older at onset (late-onset) at a single regional ALS diagnostic center in Japan. The phenotype of late-onset ALS differed between males and females, with late-onset females having more bulbar-onset ALS and significantly lower body mass index, late-onset males having more frequent bulbar and respiratory symptoms at the initial examination, and significantly lower forced vital capacity at the initial examination in both groups compared to early onset patients. For late-onset patients, maintenance of skeletal muscle mass by early intervention for bulbar and respiratory symptoms may be useful for prolonging survival; however, a prospective analysis is warranted. • In a super-aging society, it is important to establish how to manage elderly amyotrophic lateral sclerosis (ALS) patients. • Late-onset females had more bulbar-onset ALS and significantly lower body mass index. • Late-onset males had more frequent bulbar and respiratory symptoms at the initial examination. • Both groups had a significantly lower forced vital capacity at the initial examination than those with early onset. • Maintenance of skeletal muscle mass by early intervention for bulbar and respiratory symptoms may be useful for prolonging survival. [ABSTRACT FROM AUTHOR]
- Published
- 2023
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27. BVVLS2 overlooked for 3 years in a pediatric patient caused by novel compound heterozygous mutations in SLC52A2 gene
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Chunbao Rao, Jianwei Li, Qi Peng, Ziqiang Liu, and Xiaomei Lu
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Male ,Proband ,medicine.medical_specialty ,Anemia ,Hearing Loss, Sensorineural ,Bulbar Palsy, Progressive ,Clinical Biochemistry ,Pure red cell aplasia ,Neurological disorder ,Compound heterozygosity ,Biochemistry ,Gastroenterology ,Receptors, G-Protein-Coupled ,Internal medicine ,medicine ,Humans ,Bulbar palsy ,business.industry ,Biochemistry (medical) ,Membrane Transport Proteins ,General Medicine ,Normocytic anemia ,medicine.disease ,Child, Preschool ,Mutation ,Sensorineural hearing loss ,medicine.symptom ,business - Abstract
Background Brown-Vialetto-Van Laere syndrome-2 (BVVLS2) is a rare autosomal recessive neurological disorder caused by mutations in the SLC52A2 gene, which is characterized by early childhood onset of sensorineural hearing loss, bulbar palsy, peripheral neuropathy, and respiratory insufficiency. We aimed to investigate the genetic cause of a 4-year-old boy who suffered from BVVLS2 whose initial presentation was severe normocytic anemia and had been overlooked for three years in a local hospital. He was misdiagnosed with pure red cell aplasia (PRCA) and treated with hormones and chemotherapy drugs, but there was no obvious effect. Methods The targeted capture of 927 genes associated with neuromuscular disorders and next-generation sequencing were performed. Sanger sequencing was employed to verify the variant mutations. Results The proband was found to be heterozygous for c.350T > C (p.L117P) in exon 3 and c.1135_1137delTGG (p.W379del) in exon 5 of SLC52A2 gene. His anemia and neurological symptoms improved significantly after treatment with low dose oral riboflavin. Conclusions This study expands the mutational spectrum of SLC52A2 and phenotypic spectrum of BVVLS2, which provides a foundation for further investigations elucidating the SLC52A2 related mechanisms of BVVLS2. A low-dosage of riboflavin supplementation was used to obtain good curative effect, which provides further future references for the clinical treatments of BVVLS.
- Published
- 2021
28. An Unusual Cause of Bulbar Palsy in the Emergency Department
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M. Vivekanandan, R. Gunaseelan, G. Ezhilkugan, N. Balamurugan, and U. Amaravathi
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Male ,Pediatrics ,medicine.medical_specialty ,Bulbar Palsy, Progressive ,Hypokalemia ,Neurological examination ,Liver disease ,medicine ,Humans ,Aged ,Bulbar palsy ,medicine.diagnostic_test ,business.industry ,nutritional and metabolic diseases ,Emergency department ,medicine.disease ,Magnetic Resonance Imaging ,Dysphagia ,Myelinolysis, Central Pontine ,Emergency Medicine ,medicine.symptom ,Differential diagnosis ,Emergency Service, Hospital ,Hyponatremia ,business - Abstract
Background Osmotic demyelination syndrome commonly occurs after rapid correction of hyponatremia. But it has also been reported after graded correction of hyponatremia in the presence of other risk factors like chronic alcoholism, malnutrition, liver disease, and hypokalemia. Case Report We report a case of a 67-year-old man who presented with dysphagia and nasal regurgitation and had features suggestive of bulbar palsy on neurological examination. He had spontaneous rapid correction of hyponatremia from a serum sodium level of 122 mEq/L to 132 mEq/L after discharge from our hospital. Neuroimaging was suggestive of extrapontine myelinolysis involving the basal ganglia. Why Should an Emergency Physician Be Aware of This? An emergency physician should be aware of this because osmotic demyelination syndrome should also be considered in the differential diagnosis of patients presenting with bulbar palsy to the emergency department.
- Published
- 2021
29. Myasthenia Gravis With Thymoma, Manifesting as AChR-Ab-Positive, Distinct Bulbar Palsy Accompanied by Dysgeusia: A Case Series and Review of Literature
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Kai Zhu, Jiaxin Chen, Jingjing Li, Haiyan Wang, Xin Huang, Yan Li, and Huiyu Feng
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myasthenia gravis ,dysgeusia ,antibody positive ,thymoma ,bulbar palsy ,Neurology. Diseases of the nervous system ,RC346-429 - Abstract
In this review, we summarized three cases of myasthenia gravis (MG) with taste disorder and describe their clinical features in detail. Three MG patients presented with significant bulbar palsy symptoms, high AChR-Ab titers, and negative MuSK-Ab, were diagnosed with thymoma. Furthermore, we observed that dysgeusia could manifest earlier than the occurrence of typical MG symptoms, even predict a MG relapse or a myasthenic crisis in the course of MG. We believe that dysgeusia is a non-motor symptom of MG, which especially exists in MG patients with thymoma and serious bulbar palsy. Therefore, being alert to this symptom may facilitate the early diagnosis of MG and judge the progress of the disease.
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- 2018
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30. Myasthenia Gravis Presenting as Bulbar Palsy.
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Gosain D and Das T
- Abstract
Myasthenia gravis is a rare autoimmune condition that affects postsynaptic cholinergic receptors, resulting in symptoms of muscular fatigue. Clinical signs could be subtle and variable, often leading to many differentials. This leads to inappropriate tests being performed and a delay in diagnosis. Although ocular signs are more common, it may rarely present as bulbar palsy. Our patient, in her 30s, was referred to the emergency department after six months of symptom onset when she was discovered to be at a high risk of silent aspiration. Her presentation was predominantly bulbar palsy, but after appropriate tests, she was eventually diagnosed with generalized myasthenia gravis with a concurrent thymoma. Her treatment included pyridostigmine, corticosteroid, and immunoglobulins, while a thymectomy was scheduled as a planned procedure. Prompt diagnosis and timely management can reduce morbidity and mortality in such cases., Competing Interests: The authors have declared that no competing interests exist., (Copyright © 2023, Gosain et al.)
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- 2023
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31. Symptoms of Myasthenia Gravis Obscured by Old Age and Unilateral Presentation.
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Gracey C and Balladares R
- Abstract
Myasthenia gravis (MG) is a neuromuscular junction disorder involving autoantibodies affecting the postsynaptic muscle membrane. We report an 81-year-old man who presented to the emergency department with three days of left facial droop, who later developed worsening bilateral ptosis, cervical weakness, dysphagia, and dysarthria following an assessment for Bell's palsy. Ultimately, he was diagnosed with MG. This patient's presentation was atypical and challenging. Specifically, the patient had droopy eyelids from a redundancy of skin and an anatomical neck droop, non-specific findings in older adults, which obscured the development of bilateral ptosis and cervical weakness, a classic sign of bulbar disease. The patient also presented with unilateral facial weakness, a rare finding in MG and concerning stroke in the elderly population. Our aim is to discuss the challenges of identifying MG in older populations and to discuss pharmacological challenges in assessing elderly patients with suspected bulbar palsies., Competing Interests: The authors have declared that no competing interests exist., (Copyright © 2023, Gracey et al.)
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- 2023
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32. 吞咽综合训练对脑损伤后真性球麻痹和假性球麻痹的疗效观察.
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田闪, 胡瑞萍, 张备, 刘罡, 陈颖, 谢鸿宇, 李丹丹, 朱玉连, and 吴毅
- Abstract
Objective To analyze the difference of clinical efficacy of bulbar palsy and pseudobulbar palsy in swallowing rehabilitation and briefly clarified the reasons for the different sensitivity of two types of patients to swallowing rehabilitation.Methods Seventy cases of cerebral palsy from the Department of Rehabilitation Medicine,Huashan Hospital,Fudan University during Jan.,2015 and Oct.,2016 were recruited in this study,including 47 cases in pseudobulbar palsy group and 23 cases in bulbar palsy group.There was no statistical difference in age and course of disease in the two groups.All the patients were given comprehensive swallowing rehabilitation with the same mode and time of intervention for 4 weeks.Watian drinking water test,standardized swallowing assessment (SSA) and video fluoroscopic swallowing study (VFSS) were adopted to evaluated curative effect difference of bulbar palsy and pseudobulbar palsy before and after treatment.Single factor ANOVA test and the rank sum test was used for measurement data.Results After 4 weeks of rehabilitation treatment,the improvement degree of dysphagia induced by pseudobulbar palsy was better than the bulbar paralysis through the comprehensive swallowing training.Conclusions The efficacy of swallowing rehabilitation therapy for pseudobulbar palsy was better than that of bulbar palsy. [ABSTRACT FROM AUTHOR]
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- 2018
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33. Immunotherapy-responsive childhood neurodegeneration with systemic and central nervous system inflammation.
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Sa, Mario, Hacohen, Yael, Alderson, Lucy, Chong, W.K. Kling, Anderson, Glenn, Jacques, Thomas S., Neubauer, David, Szczepanik, Elzbieta, Lim, Ming, and Kaliakatsos, Marios
- Abstract
Abstract Subacute neuroregression in association with raised neopterin and overexpression of interferon stimulated genes (ISGs) could indicate a type 1 interferonopathy. Here we describe a novel immunotherapy-responsive, clinico-immunological and imaging phenotype with evidence of innate immune activation. Three children (patient 1: 22-month-old boy; patient 2: 5-year-old girl; patient 3: 4-year-old girl) presented with asymmetric bilateral mixed dystonia and spasticity, regression in language (expressive more than receptive) and bulbar symptoms with no evidence of seizures. Symptoms evolved over several weeks to months. Brain MRI changes mimicked cerebral atrophy, initially asymmetric. CSF revealed raised neopterins. Blood RNA assay showed abnormal overexpression of ISGs and transient raised alanine aminotransferase (ALT). Importantly, all three children were treated with intravenous methylprednisolone and immunoglobulin with significant and sustained improvement in their motor and language function, and normalisation of imaging. Immune-mediated encephalitis can masquerade as subacute neuroregression. [ABSTRACT FROM AUTHOR]
- Published
- 2018
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34. Myasthenia Gravis With thymoma, Manifesting as aChR-ab-positive, Distinct Bulbar palsy accompanied by Dysgeusia: a Case series and Review of Literature.
- Author
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Zhu, Kai, Chen, Jiaxin, Li, Jingjing, Wang, Haiyan, Huang, Xin, Li, Yan, and Feng, Huiyu
- Subjects
MYASTHENIA gravis ,TASTE disorders ,NEUROMUSCULAR diseases ,DIAGNOSIS - Abstract
In this review, we summarized three cases of myasthenia gravis (MG) with taste disorder and describe their clinical features in detail. Three MG patients presented with significant bulbar palsy symptoms, high AChR-Ab titers, and negative MuSK-Ab, were diagnosed with thymoma. Furthermore, we observed that dysgeusia could manifest earlier than the occurrence of typical MG symptoms, even predict a MG relapse or a myasthenic crisis in the course of MG. We believe that dysgeusia is a non-motor symptom of MG, which especially exists in MG patients with thymoma and serious bulbar palsy. Therefore, being alert to this symptom may facilitate the early diagnosis of MG and judge the progress of the disease. [ABSTRACT FROM AUTHOR]
- Published
- 2018
- Full Text
- View/download PDF
35. Neurological Manifestations of COVID-19 Associated Multi-system Inflammatory Syndrome in Children: A Systematic Review and Meta-analysis
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Sandesh Panthi, Gaurav Nepal, Gentle Sunder Shrestha, Rajeev Ojha, Bharat Khatri, Ishan Adhikari, Bikram Prasad Gajurel, Jessica Holly Rehrig, and Aditi Agrawal
- Subjects
Pediatrics ,medicine.medical_specialty ,Pneumonia, Viral ,Encephalopathy ,Anosmia ,Splenium ,children ,medicine ,Humans ,mis-c ,Child ,kawasaki disease ,Bulbar palsy ,Cerebellar ataxia ,business.industry ,neurology ,Meningism ,General Medicine ,medicine.disease ,Systemic Inflammatory Response Syndrome ,covid-19 ,Meta-analysis ,Nervous System Diseases ,Public aspects of medicine ,RA1-1270 ,medicine.symptom ,Headaches ,business - Abstract
Background: Children comprise only 1–5% of COVID-19 cases. Recent studies have shown that COVID-19 associated multisystem inflammatory syndrome in children (MIS-C) can present with neurological signs and symptoms. In this systematic review and meta-analysis, we have reviewed neurological involvement in these patients. Methods: A comprehensive electronic literature search was done on PubMed, Google Scholar, Embase, Cochrane database, and SCOPUS for the published English language articles from December 1, 2019, to February 28, 2021. A meta-analysis of the proportion was expressed as a pooled proportion with a 95% confidence interval (CI). Representative forest plots showing individual studies and the combined effect size were generated to provide an overview of the results.Results: This systematic review and meta-analysis analyzed 15 published MIS-C studies with a total of 785 patients. Neurological manifestations in patients with MIS-C was found in 27.1%. We found that 27% developed headaches, 17.1% developed meningism/meningitis and 7.6 % developed encephalopathy. Other uncommon neurological manifestations of MIS-C includes anosmia, seizures, cerebellar ataxia, global proximal muscle weakness and bulbar palsy. In MIS-C patients with neurological feature, neuroimaging showed signal changes in the splenium of the corpus callosum. Electroencephalography showed slow wave pattern and nerve conduction studies and electromyography showed mild myopathic and neuropathic changes. Conclusions: Our study revealed that neurological manifestations are not uncommon in patients with MIS-C. Further large prospective studies are needed to better explore the disease spectrum and to unravel the underlying pathophysiology.Keywords: Children; COVID-19; kawasaki disease; MIS-C, neurology
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- 2021
36. Adult-onset Leigh′s disease: A rare entity
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Shaik Afshan Jabeen, G Sandeep, Kandadai Rukmini Mridula, Angamuttu Kanikannan Meena, Rupam Borgohain, and Challa Sundaram
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Adult onset ,brain stem hyperintensities ,bulbar palsy ,Leigh′s disease ,serum lactate ,Neurology. Diseases of the nervous system ,RC346-429 - Abstract
Leigh syndrome (LS) is a heterogeneous familial or sporadic neurodegenerative disorder. It is typically seen in infancy or childhood, although rare cases of adult onset have been described. The authors describe a 37-year-old woman who presented with protracted gastrointestinal symptoms followed by acute brain stem syndrome with severe metabolic acidosis and who subsequently showed dramatic clinical and neuroradiological improvement.
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- 2016
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37. J
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Larner, A.J.
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- 2006
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38. Amyloid cranial polyneuropathy: A rare neurological presentation of immunoglobulin light‐chain amyloidosis
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Benny Paul Wilson, Abhinav Agarwal, Gopinath Kango Gopal, Prasad Mathews, and Surekha Viggeswarpu
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amyloidosis ,Aging ,Pathology ,medicine.medical_specialty ,Amyloid ,business.industry ,Amyloidosis ,Plasma cell dyscrasia ,Hepatosplenomegaly ,RC952-954.6 ,cranial polyneuropathy ,Case Report ,Case Reports ,medicine.disease ,plasma cell dyscrasia ,Immunoglobulin Light-chain Amyloidosis ,Geriatrics ,Heart failure ,medicine ,bulbar palsy ,Geriatrics and Gerontology ,medicine.symptom ,business ,Polyneuropathy ,Bulbar palsy - Abstract
Amyloidosis, a disease with extracellular tissue deposition of fibrils, results in clinical manifestations based on deposition of these fibrils in multiple organ systems. Usual manifestations include nephrotic‐range proteinuria, cardiac failure, hepatosplenomegaly, and skin manifestations. Common neurological manifestations include peripheral and autonomic neuropathies. Cranial neuropathy has been seldom reported and is an unusual clinical feature of amyloidosis. Here, we report an older man who presented with cranial nerve palsies along with other clinical features, including heart failure, proteinuria, weight loss, anorexia and distal symmetric polyneuropathy and was diagnosed with immunoglobulin light‐chain (AL) amyloidosis.
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- 2021
39. Fırtına Sonrası Sessizlik; Bickerstaff Beyin Sapı Ensefalitli Bir Olgu
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Emine Tekin, Haydar Ali Taşdemir, Hamit Özyürek, and Turgay Çokyaman
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Pediatrics ,medicine.medical_specialty ,Ataxia ,medicine.diagnostic_test ,business.industry ,Lumbar puncture ,Bickerstaff brainstem encephalitis ,Unconsciousness ,Electroencephalography ,medicine.disease ,medicine ,Paralysis ,General Earth and Planetary Sciences ,medicine.symptom ,business ,Encephalitis ,Bulbar palsy - Abstract
A pediatric case with Bickerstaff’s brainstem encephalitis (BBE), which is a very rare monophasic post-infectious condition characterized by central nervous system involvement, unconsciousness, ophthalmoplegia and ataxia, is presented. Twelve years old patient was brought with difficulty in eye movements and ataxia. On the second day, she became agitated and lethargic, and then bulbar palsy and whole body paralysis developed. Upper motor neuron involvement was evident. Routine biochemical parameters and serologic tests, cranial magnetic resonance imaging, lumbar puncture, autoimmune, paraneoplastic, and electrophysiological studies were evaluated. All were normal except for the encephalopathic first electroencephalography (EEG) and the EEG repeated on the 25th day was reported to be normal. Anti-ganglioside antibody, anti-GQ1b was found positive. Intravenous immunoglobulin (IVIG) started on the fourth day. A very rapid improvement was seen in the first week of IVIG treatment. She was able to walk in the second week. She was completely normal in 3 months. Although seven years passed, our patient has not had any relapse or neurological deficit. We presented a case diagnosed as BBE treated successfully with single dose intravenous Immunoglobulin. We wanted to emphasize that BBE has a good prognosis even though it is an acutely developing severe condition.
- Published
- 2021
40. Polyneuritis cranialis with generalized hyperreflexia as a presenting manifestation of thyrotoxicosis
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Gaurav M Kasundra, Amita Narendra Bhargava, Bharat Bhushan, Khichar Shubhakaran, and Isha Sood
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Bulbar palsy ,dysphagia ,neuropathy ,polyneuritis cranialis ,thyrotoxicosis ,Neurology. Diseases of the nervous system ,RC346-429 - Abstract
A 22-year-old male student with no past medical illness, presented with acute onset dysarthria, binocular diplopia, and dysphagia over 10 hours. On examination, he had tachycardia, hypertension, generalized hyper-reflexia, and bilateral pupil sparing oculomotor, troclear, abducens, trigeminal, facial, glossopharyngeal, and vagus nerve palsy. Rest examination was unremarkable. Facial nerve conduction study (NCS) showed decreased amplitude bilaterally and neurogenic pattern on electromyography. Limb NCS, repetitive nerve stimulation, neostigmine test, brain magnetic resonance imaging, cerebrospinal fluid, and biochemical tests were normal. Only positive tests were low thyroid-stimulating hormone (TSH) (
- Published
- 2015
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41. Clinical Profile and Predictors of Mechanical Ventilation in Guillain-Barre Syndrome in North Indian Children
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Sciddhartha Koonwar, Ishita Tiwari, Ravindra Kumar Garg, Rashmi Kumar, Shweta Pandey, Chandra Kanta, Areesha Alam, and Amita Jain
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Male ,Axonal neuropathy ,medicine.medical_treatment ,Bulbar Palsy, Progressive ,Neural Conduction ,India ,Guillain-Barre Syndrome ,Cohort Studies ,03 medical and health sciences ,0302 clinical medicine ,medicine ,Humans ,Prospective Studies ,030212 general & internal medicine ,Child ,Bulbar palsy ,Mechanical ventilation ,Guillain-Barre syndrome ,business.industry ,medicine.disease ,Respiration, Artificial ,Hospitalization ,Acute Inflammatory Demyelinating Polyneuropathy ,Child, Preschool ,Anesthesia ,Pediatrics, Perinatology and Child Health ,Muscle strength ,Female ,Neurology (clinical) ,medicine.symptom ,business ,030217 neurology & neurosurgery - Abstract
Objective: To describe the clinical-laboratory profile of pediatric Guillain-Barre syndrome and delineate features associated with need of mechanical ventilation. Methods: In a prospective observational study at tertiary care hospital, clinical-laboratory assessment and nerve conduction studies were documented in consecutive children hospitalized with Guillain-Barre syndrome according to Brighton criteria. Clinical-laboratory features were compared between ventilated and nonventilated patients using univariate and multivariate analysis. Results: Forty-six children (27 boys) with a mean age of 69.1±35.2 months were enrolled. History of preceding infection was present in 47.8%, bulbar palsy in 43.5%, feeble voice in 41.3%, sensory involvement in 13%, and autonomic involvement in 39.5%. Tetraparesis was noted in 87% of cases. Hughes disability scale >3 was noted in 44 children at admission and 39 (84.7%) at discharge. The most common electrophysiological type was acute motor axonal neuropathy (46.5%) followed by acute motor sensory axonal neuropathy (39.5%), acute inflammatory demyelinating polyneuropathy (7%), and inexcitable nerves (7%). Nine (19.7%) children were ventilated, 3 (6.5%) died or were lost, and 43 were discharged. Factors associated with need of mechanical ventilation on univariate analysis were older age, hypertension, bulbar palsy, feeble voice, lower Medical Research Council (MRC) sum, raised total leucocyte count, and history of preceding infection. Logistic regression revealed older age, history of predisposing illness, lower MRC sum at presentation, and bulbar palsy as independent predictors of mechanical ventilation. Conclusions: The most common electrophysiological subtype in northern Indian children is acute motor axonal neuropathy. Older age, preceding infection, low MRC sum, and bulbar palsy are predictors of mechanical ventilation in pediatric Guillain-Barre syndrome.
- Published
- 2020
42. B
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Larner, A.J.
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- 2001
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43. ИСПОЛЬЗОВАНИЕ СИНКИНЕЗИЙ ПРИ РЕАБИЛИТАЦИИ ДВИГАТЕЛЬНЫХ РАССТРОЙСТВ ЧЕЛЮСТНО-ЛИЦЕВОЙ ОБЛАСТИ
- Subjects
neuropathy of the facial nerve ,pseudobulbar palsy ,нейропатия лицевого нерва ,synergy ,реабилитация ,синкинезия ,кинезитерапия ,псевдобульбарный паралич ,rehabilitation ,бульбарный паралич ,synkinesis ,kinesitherapy ,bulbar palsy ,синергия - Abstract
Эффективная кинезитерапия периферических и центральных параличей мимической, жевательной, язычной и гортанно-глоточной мускулатуры требует углубленного изучения особенностей содружественной работы этих мышц в норме и при патологии. Цель – познакомить реабилитологов с двигательными автоматизмами брахиофациальной области и показать принципы их практического использования. Материал. Изучено около семидесяти литературных источников по непроизвольной активности мышц челюстно-лицевой области и смежных с ней регионов. Кроме того, был обобщён собственный опыт реабилитации больных с периферическими нейропатиями лицевого нерва, а также с бульбарным и псевдобульбарным синдромом. Результаты. Проведенное исследование показало, что лицо, шея и рука значительно теснее, чем другие части тела, вовлечены в разнообразные мезенцефально-бульбарные автоматизмы, встречающиеся как в норме (онтогенетические и психосоматические синкинезии, постурально-глазодвигательные и постурально-нижнечелюстные рефлексы), так и при патологии (стволовые глазнично-лицевые синкинезии, рефлексы орального автоматизма). Заключение. Многие содружественные движения могут быть использованы при разработке техник кинезитерапии двигательных нарушений, в том числе и в челюстно-лицевой области. Чем лучше врач или инструктор по лечебной физкультуре знакомы с особенностями имеющейся или потенциально доступной больному рефлекторной активности в реабилитируемой части тела, тем эффективнее будет проходить восстановительное лечение., Effective kinesitherapy of peripheral and central paralysis of mimic, masticatory, lingual and laryngeal-pharyngeal muscles require an in-depth study of the features of the friendly work of these muscles in normal and pathological conditions. Objective – to acquaint rehabilitation specialists with the motor automatisms of the brachiofacial area and show the principles of their practical use. Material. More than seventy literature sources on involuntary activity of the muscles of the maxillofacial region and adjacent regions were studied, in addition, their own experience in the rehabilitation of patients with peripheral neuropathies of the facial nerve, as well as with bulbar and pseudobulbar syndrome, was summarized. Results. The study showed that the face, neck and hand are much more closely involved than other parts of the body in a variety of mesencephalic-bulbar automatisms that occur both in the norm (ontogenetic and psychosomatic synkinesis, postural-oculomotor and postural-mandibular reflexes) and in pathology (stem orbito-facial synkinesis, reflexes of oral automatism). Conclusion. Many friendly movements can be used in the development of kinesitherapy techniques for movement disorders, including those in the maxillofacial area. The better the physical therapist is familiar with the features of the existing or potentially available to the patient reflex activity in the part of the body being rehabilitated, the more effective the rehabilitation treatment will be.
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- 2022
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44. Obstructive sleep apnea syndrome: age aspects
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N. L. Кunelskaya, M. V. Tardov, A. Y. Ivoylov, I. I. Arkhangelskaya, Z. O. Zaoyeva, and V. V. Kondrashina
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синдром обструктивных апноэ сна ,депри-вация сна ,бульбарный синдром ,ожирение ,obstructive sleep apnea ,sleep deprivation ,bulbar palsy ,obesity ,Medicine - Abstract
Medical histories and results of examinations of 165 patients with obstructive sleep apnea syndrome were analyzed. The role of sleep deprivation, innervation of the pharynx and rapid weight gain in different age groups in the genesis of the syndrome was demonstrated. The details of 165 case histories of the patients with obstructive sleep apnea syndrome and results of their cardiorespiratory examinations are analyzed. Role of chronic sleep deprivation, pharyngeal innervation disturbances and fast body mass growth in syndrome genesis in different age groups is discussed.
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- 2014
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45. Insertion of intra-oral electrodes for cranial nerve monitoring using a Crowe-Davis retractor.
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Trentman, Terrence, Thunberg, Christopher, Gorlin, Andrew, Koht, Antoun, Zimmerman, Richard, Bendok, Bernard, Trentman, Terrence L, and Zimmerman, Richard S
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Acoustic neuroma resection is an example of a neurosurgical procedure where the brainstem and multiple cranial nerves are at risk for injury. Electrode placement for monitoring of the glossopharyngeal and hypoglossal nerves during acoustic neuroma resection can be challenging. The purpose of this report is to illustrate the use of a device for intra-oral electrode placement for intraoperative monitoring of the glossopharyngeal and hypoglossal nerves. A 60-year-old male presented for acoustic neuroma resection. Under general anesthesia, a Crowe-Davis retractor was used to open the mouth, providing access to the posterior pharynx. For glossopharyngeal monitoring, two bent subdermal needle electrodes were inserted just lateral to the uvula. Two additional electrodes were inserted on the lateral tongue to monitor the hypoglossal nerve. Cranial nerves monitoring was conducted utilizing both free running and triggered electromyography of the trigeminal and facial nerves in addition to the lower cranial nerves. The tumor was resected successfully. Monitoring of the cranial nerves (including the glossopharyngeal and hypoglossal nerves) revealed no concerning responses. The Crowe-Davis retractor and the technique described allowed insertion of electrodes for neural monitoring, contributing to neural preservation. [ABSTRACT FROM AUTHOR]
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- 2017
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46. Brief introduction to Professor Gao Wei-bin's needling technique of 'Ten Acupuncture Skills'.
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Liu, Shuang-ling and Gao, Wei-bin
- Abstract
Copyright of Journal of Acupuncture & Tuina Science is the property of Springer Nature and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)
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- 2017
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47. Acoustic reflex patterns in amyotrophic lateral sclerosis.
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Canale, Andrea, Albera, Roberto, Lacilla, Michelangelo, Canosa, Antonio, Albera, Andrea, Sacco, Francesca, Chiò, Adriano, and Calvo, Andrea
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ACOUSTIC reflex , *AMYOTROPHIC lateral sclerosis , *AMPLITUDE modulation , *WILCOXON signed-rank test , *MOTOR neuron diseases - Abstract
The aim of the study is to investigate acoustic reflex testing in amyotrophic lateral sclerosis patients. Amplitude, latency, and rise time of stapedial reflex were recorded for 500 and 1000 Hz contralateral stimulus. Statistical analysis was performed by the Wilcoxon test and the level of significance was set at 5 %. Fifty-one amyotrophic lateral sclerosis patients and ten sex- and age-matched control subjects were studied. Patients were further divided in two groups: amyotrophic lateral sclerosis-bulbar (38 cases, with bulbar signs at evaluation) and amyotrophic lateral sclerosis-spinal (13 cases, without bulbar signs at evaluation). Stapedial reflex was present in all patients. There was a statistically significant difference in the mean amplitude, latency, and rise time between the amyotrophic lateral sclerosis patients as compared with the controls. Amplitude was lower in both the amyotrophic lateral sclerosis-bulbar and the amyotrophic lateral sclerosis-spinal patients than in the controls ( p < 0.05) and rise time was longer in both patient groups compared with the controls ( p < 0.05). These results confirm the presence of abnormal acoustic reflex patterns in amyotrophic lateral sclerosis cases with bulbar signs and, moreover, suggesting a possible subclinical involvement of the stapedial motor neuron even in amyotrophic lateral sclerosis-spinal patients. Amplitude and rise time seem to be good sensitive parameters for investigating subclinical bulbar involvement. [ABSTRACT FROM AUTHOR]
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- 2017
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48. Transient Isolated Lower Bulbar Palsy With Elevated Serum Anti-GM1 and Anti-GD1b Antibodies During Aripiprazole Treatment.
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Han, Tae Hwan, Kim, Do Yeon, Park, Dong Woo, and Moon, Jin-Hwa
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- *
BLOOD serum analysis , *MOTOR neuron diseases , *ARIPIPRAZOLE , *MAGNETIC resonance imaging of the brain , *DIAGNOSIS , *THERAPEUTICS - Abstract
Background: Transient bulbar palsy without involvement of the facial or extraocular muscles is a rare presentation. It is considered a form of cranial polyneuropathy, a variant of Guillain-Barré syndrome that is related to the autoimmune mechanisms induced by preceding infections or vaccinations. However, drug-induced cranial polyneuropathy has not previously been reported. We describe a boy with isolated bulbar palsy and positive serum antiganglioside antibodies during aripiprazole treatment.Patient Description: This 12-year-old boy was admitted with a seven-day history of dysarthria, tongue discomfort, and tinnitus. Three weeks before symptom onset, aripiprazole was added to the patient's medications for attention-deficit hyperactivity disorder. On examination, he showed curtaining of the pharyngeal wall, tongue fasciculation and deviation, and a weak gag reflex. Cranial magnetic resonance imaging suggested lower cranial nerve involvement. Serum anti-GM1 IgG and anti-GD1b IgG antibodies were positive. After stopping aripiprazole, his bulbar symptoms improved. However, on readministration of aripiprazole seven weeks later, dysarthria recurred and again resolved after stopping the drug.Conclusion: We describe the first patient with anti-GM1 IgG and anti-GD1b IgG antibodies-associated transient cranial polyneuropathy presenting as isolated bulbar palsy. These findings could be an adverse effect of aripiprazole treatment. [ABSTRACT FROM AUTHOR]- Published
- 2017
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49. Bulbar Onset Amyotrophic Lateral Sclerosis in a COVID-19 Patient: A Case Report.
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Abu-Abaa M, Mousa A, Chadalawada S, and Abdulsahib A
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Amyotrophic lateral sclerosis (ALS) is a devastating neurodegenerative disorder with a largely unknown etiology. In this case, we are presenting an 84-year-old male patient who was admitted for acute hypoxemic respiratory failure secondary to coronavirus disease 2019 (COVID-19) infection. He was neurologically intact. His infection improved and oxygen requirement was gradually weaned off allowing for discharge. However, he was admitted again a month later with progressive dysphagia and aspiration that were confirmed on videofluoroscopic study. He was also found to have mild dysarthria, bulbar muscle weakness, bilateral lower motor neuron facial nerve palsy, diffuse hyporeflexia on four extremities with intact sensory function. Diagnosis of ALS was suspected after extensive workup was pursued and ruled out nutritional, structural, autoimmune, infectious and inflammatory disorders. This case is only the third reported case in medical literature to suggest COVID-19 infection as a triggering/accelerating factor of ALS progression., Competing Interests: The authors have declared that no competing interests exist., (Copyright © 2023, Abu-Abaa et al.)
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- 2023
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50. A CADASIL Case Presenting with Progressive Bulbar Palsy Caused by Acute Simultaneous Multiple Subcortical Infarcts.
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GÖKÇAL, Elif, KOLUKISA, Mehmet, MUSTAFAYEV, Nihat, and ASİL, Talip
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- *
PARALYSIS , *MIGRAINE aura , *CRANIAL nerves , *THERAPEUTICS , *BRAIN diseases , *PLATELET aggregation inhibitors - Abstract
Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is an adult-onset inherited small vessel disease of the brain caused by NOTCH3 mutations. Clinical characteristics of CADASIL include recurrent infarctions, migraine with aura, mood disturbances and cognitive impairment. We report a 35 year-old migraine-free patient with unusual presentation with acute simultaneous multiple subcortical infarctions causing progressive bulbar palsy as the initial manifestation. Hepresented with slurred speech progressing to anarthria and bilateral palsy of lower motor cranial nerves within 24 hour. Initial acute subcortical infarcts enlarged and new infarcts developed in bilateral hemispheres. There was diffuse leukoencephalopathy involving temporal horns and the patient had positive familiy history of migraine and young stroke. CADASIL was diagnosed genetically when NOTCH3 mutationwas shown. The patient improved after treatment with liquid hydration and antiplatelet agent. We experienced with the patient that CADASIL may present with unusual progressive manifestations and bilateral simultaneous infarctions. Irrespective of clinical presentation, patients with leukoencephalopathy and positive familiy history should be tested for NOTCH3 mutation. [ABSTRACT FROM AUTHOR]
- Published
- 2019
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