Bulbar Palsy Research Articles

Clinical Profile and Predictors of Mechanical Ventilation in Guillain-Barre Syndrome in North Indian Children.

To describe the clinical-laboratory profile of pediatric Guillain-Barre syndrome and delineate features associated with need of mechanical ventilation. 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. 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. 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.

Atlantoaxial dislocation with congenital \u201csandwich fusion\u201d in the craniovertebral junction: a retrospective case series of 70 patients

BackgroundIn the setting of congenital C1 occipitalization and C2–3 fusion, significant strain is placed on the atlantoaxial joint. Vertebral fusion both above and below the atlantoaxial joint (i.e., a “sandwich”) creates substantial instability. We retrospectively report on a case series of “sandwich fusion” atlantoaxial dislocation (AAD), describing the associated clinical characteristics and detailing surgical treatment. To the best of our knowledge, the present study is the largest investigation to date of this congenital subgroup of AAD.MethodsSeventy consecutive patients with sandwich fusion AAD, from one senior surgeon, were retrospectively reviewed. The clinical features and the surgical treatment results were assessed using descriptive statistics. No funding sources or potential conflict of interest-associated biases exist.ResultsThe mean patient age was 42.2 years (range: 5–77 years); 36 patients were male, and 34 were female. Fifty-eight patients (82.9%) had myelopathy, with Japanese Orthopaedic Association (JOA) scores ranging 4–16 (mean: 12.9). Cranial neuropathy was involved in 10 cases (14.3%). The most common presentation age group was 31 to 40 years (24 cases, 34.3%). Radiological findings revealed brainstem and/or cervical-medullar compression (58 cases, 82.9%), syringomyelia (16 cases, 22.9%), Chiari malformation (12 cases, 17.1%), cervical spinal stenosis (10 cases, 14.3%), high scapula deformity (1 case, 1.4%), os odontoideum (1 case, 1.4%), and dysplasia of the atlas (1 case, 1.4%). Computed tomography angiography was performed in 27 cases, and vertebral artery (VA) anomalies were identified in 14 cases (51.9%). All 70 patients underwent surgical treatment, without spinal cord or VA injury. Four patients (5.7%) suffered complications, including 1 wound infection, 1 screw loosening, and 2 cases of bulbar paralysis. In the 58 patients with myelopathy, the mean JOA score increased from 12.9 to 14.5. The average follow-up time was 50.5 months (range: 24–120 months). All 70 cases achieved solid atlantoaxial fusion at the final follow-up.ConclusionsSandwich fusion AAD, a unique subgroup of AAD, has distinctive clinical features and associated malformations such as cervical-medullar compression, syringomyelia, and VA anomalies. Surgical treatment of AAD was associated with myelopathy improvement and minimal complication occurrence.

A case of amyotrophic lateral sclerosis with frontotemporal dementia manifested as naming and sentence comprehension disorder

AbstractBackgroundApproximately half of the patients with motor neuron disease (MND) will develop frontotemporal dysfunction. Most of case reports showed a strong association between amyotrophic lateral sclerosis (ALS) and behavioral variant frontotemporal dementia (bvFTD) and mostly presented as FTD at the onset, while the relationship between ALS and primary progressive aphasia (PPA) has not been fully understood.MethodA patient suspected to be ALS‐PPA admitted to Beijing Tiantan Hospital, Capital Medical University in January 2020. We want to describe this case to summarize its clinical characteristics.ResultThe patient is 70 years old,female, illiteracy and with severe hearing impairment. She developed bulbar paralysis manifestations like hoarseness, dysarthria and dysphagia without obvious mental, character and behavior changes in the course of the disease.Both upper limbs tendon reflex was hyperactive and lower limbs tendon reflex was decreased. Sucking reflex existed. Neuropsychological test suggested dementia, impaired naming and sentence comprehension. Electromyography suggested neurogenic changes around the upper and lower extremities. Magnetic resonance imaging showed symmetrical anomaly signal of bilateral basal ganglia, where manifested as low metabolism on PETCT. Amyloid β (1‐42) was elevated, the total protein Tau was reduced and the phosphorylated tau protein was normal in cerebrospinal fluid.ConclusionALS‐PPA is a relatively rare disease. Sometimes maybe genetic and pathological examinations are required for diagnosis.

Cerebrotendinous xanthomatosis with peripheral neuropathy: a clinical and neurophysiological study in Chinese population.

BackgroundCerebrotendinous xanthomatosis (CTX) is an inborn disorder of bile acid metabolism caused by deficiency of sterol 27-hydroxylase (CYP27A1) gene. CTX-related peripheral neuropathy has rarely been discussed in Chinese population. Here, we reported 6 CTX cases and performed a literature review focused on CTX with neuropathy to summarize its clinical and neurophysiological features.MethodsAll clinical data of 6 CTX cases were collected, and 21 reported Chinese CTX patients (including this study) were reviewed and summarized.ResultsClinical manifestations of 6 CTX cases showed great heterogeneity. Cognitive decline, spastic paraplegia, cerebellar ataxia and advanced bulbar palsy were common neurological disorders, often accompanied by non-neurological signs like xanthomas, cataract, diarrhea and pes cavus. Dentate nuclei hyperintensity with or without hyposignal is a valuable MRI hallmark. Pooling our patients and literature review together, peripheral neuropathy was predominant sensorimotor demyelinating type in Chinese population, with an evident length dependent pattern and increased vulnerability in motor nerves. Demyelinating and axonal degeneration tend to exist in severe neuropathy. Three novel mutations including c.1055C>A; c.432T>G; c.472T>G were identified in CYP27A1 and predicted to be pathogenic. Oral CDCA therapy could ameliorate some of the existing symptoms and provide clinical stability, but it could not cease disease progression completely.ConclusionsOur study broadens the phenotype and mutation spectrum of CTX. Patients with cognitive decline, spastic tetraparesis, cerebellar ataxia and bulbar palsy, should be highly suspicious of CTX even no xanthomas disclosed. Peripheral neuropathy was predominant sensorimotor demyelinating type in Chinese population, with mixed axonal and demyelinating type in severe cases. Three novel likely pathogenic mutations including c.1055C>A; c.432T>G; c.472T>G were identified in CYP27A1.

小脳・脳幹梗塞後に重度球麻痺，中枢性低換気を呈した患者に対する包括的リハビリテーションの訓練経過 ～摂食嚥下リハビリテーションを中心に～

小脳・脳幹梗塞後に重度球麻痺，中枢性低換気を呈した患者に対する包括的リハビリテーションの訓練経過 ～摂食嚥下リハビリテーションを中心に～

Neurodegenerative Diseases and the Auditory-Vestibular System

Neurodegenerative Diseases and the Auditory-Vestibular System

An Erratic Exanthem

Varicella zoster (Chickenpox) usually occurs in childhood due to infection with varicella zoster virus. It manifests as an acute febrile exanthem which is usually self-limiting. Varicella zoster virus infection, in most cases, provides lifelong immunity to an individual after primary infection. In adults, primary varicella is uncommon, and most cases occur as a result of reactivation of the dormant virus. Subsequently, in adults, varicella infection can behave erratically to present with various unusual systemic and neurological complications, which are again uncommonly seen after primary varicella. The most common neurological complications seen are herpes zoster, postherpetic neuralgia, cerebellitis, meningoencephalitis and rarely stroke, myelitis, and Guillain-Barre syndrome. We hereby present the case of a young male who developed Guillain-Barre syndrome with facial diplegia and bulbar palsy after primary varicella infection.

Changes in serum complements and their regulators in generalized myasthenia gravis.

To investigate changes in serum complements and their regulators in the pathogenesis of myasthenia gravis (MG). Forty-four patients with acetylcholine receptor antibody-positive MG, as well as 20 patients with non-inflammatory neurological disorders were enrolled. Serum complements (C3, C4 and soluble C5b-9) and complement regulators (vitronectin, clusterin and properdin) were extensively analysed by enzyme-linked immunosorbent assay and their associations with clinical profiles of MG were examined. Serum C3, C4 and clusterin levels were not significantly different between patients with MG and controls. The patients with MG had higher soluble C5b-9 (P=0.09) and vitronectin (P=0.001) levels than the controls; moreover, vitronectin levels decreased after treatment (P=0.09). Serum properdin (P=0.03) levels were lower in the patients with MG than in the controls, and negatively correlated with the MG Activities of Daily Living score (rs=-0.26, P=0.09) and with the presence of bulbar palsy (P=0.04). Our results show that activation of complements and an altered complement network could contribute to the inflammatory pathogenesis of MG.

Cranial nerve involvement in typical and atypical chronic inflammatory demyelinating polyneuropathies.

Cranial nerve palsy is occasionally present in patients with chronic inflammatory demyelinating polyneuropathy (CIDP), but its prevalence, characteristics and relations with the CIDP subtypes have rarely been investigated. The aim of this study was to systematically assess cranial nerve involvement in typical and atypical CIDP. Clinical data were reviewed in 132 consecutive patients with CIDP, including typical CIDP (n=89), multifocal acquired demyelinating sensory and motor neuropathy (MADSAM) (n=31), distal acquired demyelinating symmetric (DADS) (n=9) and others (n=3). The frequency of cranial nerve palsy was 11% in typical CIDP, 48% in MADSAM and 11% in DADS. Facial and bulbar palsy was most frequently present (9%), followed by ocular motor nerve palsy (5%). Bilateral involvement was seen in all typical CIDP and DADS patients, whereas 80% of MADSAM patients had unilateral palsy. The presence of cranial nerve involvement was associated with more severe limb muscle weakness in typical CIDP, but not in MADSAM. Cranial nerve palsy fully recovered in 90% of typical CIDP and in 67% of MADSAM patients. Amongst the CIDP subtypes, cranial palsy is frequent and unilateral in MADSAM, and less frequent and bilateral in typical CIDP and DADS. In typical CIDP, facial and bulbar palsy reflects more severe and extensive inflammation.

Dropped head syndrome in patient with progressive bulbar palsy

The dropped head syndrome (DHS) is a rare clinical syndrome with a broad differential diagnosis. The basic manifestation is inappropriate kyphosis of the cervico-thoracic link, usually caused by weakness of the extensor muscles of the nape. DHS is a source of significant discomfort and reduces quality of life (visual impairment, dysphagia, secondary pain, etc.).

A Case of Old-onset Myasthenia Gravis with Bulbar Palsy Alone

A Case of Old-onset Myasthenia Gravis with Bulbar Palsy Alone

Fazio-Londe Syndrome and Patient-centered Nursing Care: A Case Report

Introduction: Fazio-Londe disease is a genetic rare disorder manifesting by acute respiratory distress. This article is a case report of Fazio-Londe syndrome, its comparison with other case reports in the literature, and its patient-centered nursing care. Case presentation: In this study, we reported a case with Fazio-Londe syndrome with respiratory distress, bulbar palsy, muscle weakness, and other symptoms. Management and outcome: The patient was treated with oral riboflavin (100 mg/day), ribavirin (200 mg/day), and intramuscular Vit-B12 (1000 mg/day). She was managed using a patient-centered nursing care model. Discussion: Health care providers should be aware of the new and constant symptoms of this rare disease. A patient-centered nursing care model is suggested to manage the disease.

103 Atypical Progressive Bulbar Palsy presenting with Dropped Head

Typical amyotrophic lateral sclerosis (ALS) presents on neurological examination with specific signs of upper and lower motor neuron degeneration (Brooks et al, 1995), which can account for 85% of patients with ALS (Turner and Talbot, 2013). There are different types of clinical presentations, including progressive bulbar palsy (PBP), Limb-onset ALS, progressive muscular atrophy (PMA) and upper motor neuron (UMN) predominant ALS. PBP has mainly brainstem signs. There are a few case reports of dropped head syndrome in ALS, mainly in patients with the limb involvement variant. Case report. A 56 year old right-handed male, presented to the clinic with four months of dysphagia to liquids and solids, neck pain and progressive neck weakness causing constant drop head. No dysarthria or other neurological symptoms, no dyspnea. Neurological examination: Cranial Nerve (CN) CN XII: Nasal voice, bilateral atrophy of the tongue with tremor and fasciculations. Motor: Diffuse atrophy and decreased tone of the sternocleidomastoid and trapezii bilaterally, strength: 2/5 in neck flexors and extensors. Sensory: Hypoesthesia of the tongue. The rest of his neurological examination was normal. Labs: Routine blood work, thyroid function tests, collagen vascular work-up, and protein electrophoresis were normal. Creatine Phosphokinase (CPK) and Acetylcholine Receptor Antibodies (AChR Ab) were negative. Brain and Spinal Cord MRI: Showed mild brainstem, cerebellar and cervical spinal atrophy. Patients with ALS initially present with symptoms localized to the limbs or bulbar muscles. A very small percentage 1-2% of ALS patients had neck muscle weakness with head drop (Jokelainen et al, 1977; Gourie-Devi et al, 2003). However, in all the previously reported cases, the patients had limb involvement at the time of presentation which was absent in this case, and the head drop occurred after the onset of symptoms (Lange et al, 1986; Katz et al; 1996). Dropped head syndrome can be seen in inflammatory myopathies, myasthenia gravis, facioscapulohumeral muscular dystrophy, spinal muscular atrophy, nemaline myopathy and carnitine deficiency (Umapathy et al, 2003) but ALS should also be considered in patients with atypical presentations.

Pseudo Bulbar Palsy a Rare NEURO-COVID Presentation

Background: The Global pandemic of COVID-19 with more than half million people passed away from their families tragically, wide variety of signs and symptoms resulted from this Novel SARS Cov2 virus. Although the respiratory and Gastro-intestinal symptoms are dominant, however, neurological symptoms whether specific or nonspecific have been reported. Till now the extents of knowledge regarding the neurological sequel of COVID-19 infection are finite. Pseudo Bulbar Palsy is a neurological disease characterised by dysarthria, dysphagia, facial and tongue weakness in addition to emotional liability resulted from an upper motor neuron lesion in the corticoid bulbar tracts. Peripheral neuropathy, Demyelisation and vascularise are underlying aetiologies. This will result in deglutition, and speech disturbance. Case presentation: 60 years old male from Najaf, went to the emergency department with three days history of drowsiness and anorexia after COVID-19 related fever and myalgias. He had significant risk factors. He had 2 weeks fever following a contact with COVID-19 patient. Examination revealed drowsiness, dehydration, the patient was not aware for time and place, eye refluxes was symmetrical. The face was normal with exaggerated jaw jerk CT of the chest showed evidence of COVID-19 infection confirmed by positive PCR. Brain CT showed only brain atrophy without signs of localised ischemia. Farther blood investigations confirm activity of infection. During this the patient developed tonic clonic convulsion required anticonvulsant therapy; he remained semiconscious, with difficulty to swallow and dysarthria, Irritability, drowsiness and disorientation. He received anticoagulant, Steroids, mannitol. The condition improved and the patient discharged in better condition with two weeks anticoagulation. Discussion: this is a story for a rare complication belong to COVID-19 infection, treated as a stroke, as mention in literatures, many recorded cases of Neurocovid that required treatment accordingly. Conclusion: neurological symptoms of COVID-19are extreme and might be nonspecific but some time life threatening, these might overlap severe infection. The improvement in the neurological sequel overlap the COVID-19 improvement both clinical aspect and investigation.

Epidemiology and Outcomes of Early Morning Neuroparalytic Syndrome Following Snake Bite-A Retrospective Study.

Snake envenomation has been poorly studied in developing countries. 'Early morning neuroparalytic syndrome' (EMNS), the classical clinical constellation caused by krait bites, refers to nighttime, indoor bites where nonspecific symptoms progress to neuroparalysis. Literature regarding EMNS in children is scarce. This study was planned to describe the clinical profile, intensive care needs and predictors of outcome in children with EMNS. It is a retrospective study of children below 12 years admitted with a clinical diagnosis of snake envenomation to the pediatric intensive care unit (PICU) of a tertiary care hospital in North India. Patient records were reviewed from the electronic patient database manager. Comparison was made between the EMNS group and the non-EMNS group and between survivors and nonsurvivors within the EMNS group. Of the 111 children with snake envenomation, 76 had neuroparalysis (68%) and 51 had EMNS. In the EMNS cohort, 37 (72.5%) belonged to rural areas, 46 (90.2%) had indoor bites and 39 (76.5%) were witnessed. Patients with EMNS were more likely to have absent fang marks, hypoxemia at admission, bulbar palsy and need for PICU admission. Mortality rate was 13.7% in EMNS; predictors included younger age, presence of ptosis, cardiac arrest at admission and nonavailability of PICU bed (univariable analysis) but none of them independently predicted mortality. Younger age, presence of ptosis, cardiac arrest at admission and nonavailability of intensive care beds increase the risk of mortality in children with EMNS. Timely recognition and respiratory support may reduce mortality in these children.

Miller Fisher syndrome with bilateral vocal cord paralysis: a case report

BackgroundMiller Fisher syndrome is a variant of acute inflammatory demyelinating polyneuropathy classically characterized by ataxia, ophthalmoplegia, and areflexia. Miller Fisher syndrome can present with uncommon symptoms such as bulbar, facial, and somatic muscle palsies and micturition disturbance.Case presentationWe describe the case of a 76-year-old white man with new-onset ataxia, stridor, areflexia, and upper and lower extremity weakness who required intubation at presentation. An initial work-up including imaging studies and serum tests was inconclusive. Eventually, neurophysiological testing and cerebrospinal fluid analysis suggested a diagnosis of Miller Fisher syndrome. Our patient responded to treatment with intravenous immunoglobulin and supportive therapy.ConclusionThe occurrence of acute or subacute descending paralysis with involvement of bulbar muscles and respiratory failure can often divert clinicians to a diagnosis of neuromuscular junction disorders (such as botulism or myasthenia gravis), vascular causes like stroke, or electrolyte and metabolic abnormalities. Early identification of Miller Fisher syndrome with appropriate testing is essential to prompt treatment and prevention of further, potentially fatal, deterioration.

Acupuncture at pharyngeal acupoints for true bulbar paralysis after cerebral infarction: a randomized controlled trial

To compare the clinical effect of acupuncture at the affected side and both sides of the pharyngeal acupoints for true bulbar paralysis after cerebral infarction. A total of 66 patients with true bulbar paralysis after cerebral infarction were randomly divided into an unilateral group and a bilateral group, 33 cases in each group, 1 case dropped off in each group at last. On the basis of conventional drugs and electro-nape-acupuncture treatment, the patients in the unilateral group were treated with acupuncture at the affected side's Tunyan, Fayin and Zhifanliu (Extra), while patients in the bilateral group were treated with acupuncture at the both sides' Tunyan, Fayin and Zhifanliu. The treatment was performed once a day, 6 days a week, and the clinical effect was assessed after 21-day therapy. The swallowing and vocalization of the two groups were evaluated by dysphagia grading scale, Kubota water swallowing test and GRBAS grading scale before and after treatment. After 21 days of treatment, the scores of the dysphagia grading scale in the two groups were both improved (P<0.01), and the unilateral group was superior to the bilateral group (P<0.01). The GRBAS grading scale and Kubota water swallowing test grading after treatment were improved in both groups (P<0.01), and the unilateral group was superior to the bilateral group (P<0.05, P<0.01). Acupuncture at the pharyngeal acupoints of the affected side achieves much better therapeutic effect on true bulbar paralysis as compared with the acupoints of the bilateral sides.

A case report of adult-onset Alexander disease clinically presenting as Parkinson\u2019s disease: is the comorbidity associated with genetic susceptibility?

BackgroundAlexander disease is a rare neurological disease characterized by progressive spastic quadriparesis and bulbar palsy. Moreover, certain patients with adult-onset Alexander disease were often misdiagnosed as other neurodegenerative disorders.Case presentationHerein, we report an adult a 58-year-old woman presented with typical parkinsonism with good levodopa-responsiveness. The patient’s dopamine transporter scanning showed significant striatal depletion, while her brain magnetic resonance imaging revealed bilateral tadpole shape of medulla oblongata and bilateral high signal intensity at both cerebellar dentate nuclei in T2-weighted images, suggesting the possibility of a genetic disorder beyond Parkinson’s disease. The patient’s genetic test resulted in known pathogenic glial fibrillary acidic protein variant, indicating Alexander disease.ConclusionThis unique case highlights genetically diagnosed Alexander disease may present with clinical Parkinson’s disease.

Differences in nerve excitability properties between isolated bulbar palsy and bulbar-dominant amyotrophic lateral sclerosis

ABSTRACTObjectives: 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.

The role of pyridostigmine in recovery from motor paralysis in a snakebite patient with an allergic reaction to anti-snake venom

A 30-year-old woman presented as an emergency with a history of snakebite 5 h previously with signs of bulbar palsy, ptosis, respiratory distress and weakness of all four limbs. Mechanical ventilation, anti-snake venom (ASV) and supportive management were immediately instituted. With the third dose of ASV, an early anaphylactic reaction ensued. Subsequent management with corticosteroids and antihistamines over the next few days allowed consciousness to return but muscle power did not improve beyond 2/5. A trial of intravenous neostigmine with glycopyrrolate, however, improved motor power in all four limbs to 3/5. Oral pyridostigmine at 60 mg every 8 h allowed subsequent full motor recovery in all four extremities. We suggest consideration of pyridostigmine to promote motor recovery after an allergic reaction to ASV.