Isolated Acute Dysphagia as a Probable Rare Presentation of Guillain-Barré Syndrome with Complete Recovery: A Case Report.

Dysphagia is prevalent among the elderly and can lead to serious complications, often manifesting as a clinical symptom of various neurological or muscular pathologies, including Guillain-Barré Syndrome (GBS). GBS is an acute immune-mediated polyradiculoneuropathy, and dysphagia may arise during its course due to cranial nerve involvement. In rare GBS variants, dysphagia may present as the initial or sole clinical manifestation, posing diagnostic challenges. In this study, we present the case of an elderly female patient with dysphagia, eventually diagnosed with an atypical variant of GBS. Initially, the patient required nasogastric tube feeding; however, complete recovery was achieved through immunotherapy. This case underscores the importance of clinicians conducting thorough evaluations of factors influencing the swallowing mechanism and remaining vigilant about identifying uncommon causative factors. Such approaches enable the implementation of effective disease-modifying therapies, potentially leading to the resolution of dysphagic symptoms.

A case of variant of GBS with positive serum ganglioside GD3 IgG antibody.

BACKGROUND: Acute bulbar palsy-plus (ABPp) syndrome is an unusual variant of Guillain-Barré syndrome (GBS). Anti-GT1a and anti-GQ1b antibodies have been reported in patients with ABPp, but without reports related to GD3 antibodies. METHODS: Clinical data of a patient diagnosed as ABPp syndrome were reviewed clinically. And we summarized the GBS patients with ABP and facial paralysis reported in the literature. RESULTS: We reported a 13-year-old girl presented with asymmetric bifacial weakness, bulbar palsy and transient limb numbness, and had positive serum IgG anti-GD3 antibody. Through reviewing the GBS patients with ABP and facial paralysis reported previously, we found that facial palsy could be unilateral or bilateral. The bilateral facial palsy could present successively or simultaneously, and could be symmetrical or asymmetrical. Other common symptoms included ophthalmoplegia, sensory abnormality and ataxia. IgG anti-GT1a and IgG anti-GQ1b antibodies were the most frequent. Most of the patients had full recovery within two weeks to one year of follow-up. CONCLUSIONS: We reported a patient with asymmetric bifacial palsy and bulbar palsy, which seemed to fit the diagnosis of ABPp syndrome. This was the first report of ABPp variant of GBS with positive serum ganglioside GD3 IgG antibody.

Brown-Vialetto-Van Laere syndrome.

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.

Myasthenia Gravis Presenting as Bulbar Palsy.

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.

Symptoms of Myasthenia Gravis Obscured by Old Age and Unilateral Presentation.

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.

Anti-GT1a and anti-GQ1b immunoglobulin G antibody positivity with overlapping Miller Fisher/Guillain-Barré syndromes and prominent headache: a case report.

Guillain-Barré syndrome (GBS) and Miller Fisher syndrome (MFS) are acute immune-mediated peripheral neuropathies. In addition to their classic presentations, a variety of other signs and symptoms have been reported; however, headache appears to be relatively uncommon. We describe a 53-year-old woman who presented with acute bulbar palsy as the first symptom of overlapping MFS/GBS accompanied by severe headache. The first important clinical impairment of the patient was acute bulbar palsy along with prominent headache, without limb weakness. Although her initial diagnosis was acute bulbar palsy plus, she subsequently developed lower limb diffuse weakness, and her final clinical diagnosis was overlapping MFS/GBS. Anti-ganglioside antibodies were positive for anti-GQ1b and anti-GT1a immunoglobulin G. The patient received intravenous immunoglobulin on day 2 of admission. Early identification of these overlapping syndromes is important for the management of patients, to avoid respiratory failure or severe weakness with axonal degeneration. We therefore remind clinicians of the importance of further examination in patients with headache and acute bulbar palsy of unknown origin.

A rare case of rebleeding brainstem cavernoma in a 5-month-old-girl.

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.

Gender differences in clinical features at the initial examination of late-onset amyotrophic lateral sclerosis.

BACKGROUND: 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. METHODS: 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. RESULTS: 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. CONCLUSION: 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.

Bulbar Onset Amyotrophic Lateral Sclerosis in a COVID-19 Patient: A Case Report.

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.

Good functional outcome following bilateral external capsule hemorrhage mimicking bilateral basal ganglia hemorrhage in a coma patient.

Simultaneous spontaneous bilateral external capsule hemorrhage is a rare clinical entity with extremely poor outcome. However, knowledge on the effective management of this fatal disease is limited. Herein,we described a case of a 42-year-old man with acute coma and quadriplegia as well as respiratory failure related to the disease. The patient underwent minimally invasive surgery plus local thrombolysis. Consequently, he recovered with satisfactory neurological function recovery on the 180th day of follow-up.

Anti-GQ1b Antibody Syndrome Presented as Locked-In Syndrome: A Rare Case Report.

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.

Guillain-barré syndrome without limb weakness: A rare variant with acute bulbar palsy.

A 76-year-old male was brought to the emergency room with an acute onset of breathlessness and difficulty swallowing. Examination revealed bilateral ptosis, bilateral vocal cord abductor palsy with diaphragmatic paralysis. He did not have any limb weakness. A diagnosis of acute bulbar palsy was made. Cerebrospinal fluid showed albumino-cytological dissociation. Magnetic resonance imaging of the brain (MRI) was normal, and a nerve conduction study (NCS) showed Acute Motor and Sensory Axonal Neuropathy (AMSAN). Guillain-Barré syndrome with acute bulbar palsy was considered. Here, we report a case of suspected Acute Bulbar Palsy plus (ABPp) syndrome. ABPp may be considered as a variant of GBS between the Miller-fisher and Pharyngeal-cervical-brachial variant and does not have any definite limb weakness. This patient also had ABPp with diaphragmatic palsy. However, whether this syndrome is an isolated variant of GBS or a continuum between the Miller-fisher syndrome (MFS) and Pharyngo-cervical brachial (PCB) variants remains to be elucidated. This case is relevant to primary care physicians as the disability with GBS remains high and may render a large burden to carers. The initial symptom of acute dysphagia must lead on the primary care physician to keep this disease in mind to prevent an unwarranted delay in diagnosis.

A Severe Pharyngeal-Sensory-Ataxic Variant of Guillain-Barré Syndrome With Transient Cardiac Dysfunction and a Positive Anti-sulfatide IgM.

Guillain-Barré syndrome (GBS) is a heterogeneous group of acute immune-mediated polyradiculoneuropathy that typically presents with classic axonal or demyelinating sensory-motor type. However, there are variants of GBS with atypical presentation. We report a rare case of severe pharyngeal-sensory-ataxic variant of GBS associated with poor cardiac systolic function, elevated troponin, and positive anti-sulfatide IgM. The sensory symptom atypically started in the hands in an ascending pattern, which progressed to involve the trunk and face and, later, all limbs. It was associated with severe dysphagia, ataxia, and generalized areflexia but with preserved muscle strength in all extremities. Recognizing the atypical pattern of presentation and the ability to perform an accurate clinical localization are the utmost important initial steps in making the diagnosis. The patient showed complete recovery after immunoglobulin therapy.

Fiberoptic laryngoscopic neurological examination of amyotrophic lateral sclerosis patients with bulbar symptoms.

OBJECTIVE: Bulbar symptoms in amyotrophic lateral sclerosis (ALS) are variable, reflecting bulbar and pseudobulbar palsy. The current study sought to characterize the pharyngeal findings in ALS using a fiberoptic laryngoscope and compare them with the findings of general neurological examination. METHODS: We enrolled ALS patients with bulbar symptoms who were admitted between 2014 and 2020. All participants were evaluated on salivary status, velopharyngeal movement during speech and swallowing, pharyngeal constriction, and vocal cord movement using fiberoptic laryngoscopy. The laryngoscopic findings were compared with general neurological examination results. RESULTS: A total of 50 patients (31 men; median age: 69 years) were enrolled. Salivary residue in the hypopharynx was the most common abnormal finding on laryngoscopy (40 patients; 80%). Twenty-three patients (46%) exhibited velopharyngeal insufficiency, 18 of which exhibited good velopharyngeal closure in swallowing and poor velopharyngeal closure in speech. Thus, these patients presented speech-swallow dissociation (SSD) in velopharyngeal closure. Five patients (10%) exhibited unilateral weakness of the pharyngeal constrictor muscles in phonation. Compared with general neurological examinations, emotional incontinence was more frequent in patients who presented with SSD in velopharyngeal movement, compared with those who did not. None of the five patients with unilateral weakness of pharyngeal constriction showed curtain movement on examination via the mouth. CONCLUSION: The specific findings of laryngoscopy in ALS patients, such as SSD in velopharyngeal closure and laterality in pharyngeal constriction, could not be evaluated by general neurological examination via the mouth.

Palliative Care Challenges of Patients With Progressive Bulbar Palsy: A Retrospective Case Series of 14 Patients.

Progressive bulbar palsy (PBP) is a form of motoneuron disease and is widely classified as a subtype of amyotrophic lateral sclerosis (ALS) with a shorter time of survival and female predominance. In this retrospective case series of 14 patients with PBP, we focus on challenges in palliative care for this patient cohort, including symptom control, gastrostomy, non-invasive ventilation, and end-of-life phase. We show that rapid physical decline at the end of life is associated with bronchopulmonary infection and excessive oral secretion leading to a high level of symptom burden. Early and regular advance care planning discussions with a focus on oral secretion management with patients and caregivers are crucial.

Disease duration of progression is helpful in identifying isolated bulbar palsy of amyotrophic lateral sclerosis.

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.

An Unusual Cause of Bulbar Palsy in the Emergency Department.

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.

Amyloid cranial polyneuropathy: A rare neurological presentation of immunoglobulin light-chain amyloidosis.

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.

Bulbar Palsy as the Initial Manifestation of Multiple Myeloma: A Case Report.

Multiple Myeloma (MM) is a neoplastic disorder derived from the malignant proliferation of monoclonal plasma cells. It is characterized by the overproduction of immunoglobulins (Ig). We report a rare case in which bulbar palsy was the initial manifestation of IgG-MM. A 66-year-old woman initially presented with progressive dysphagia and dysarthria for half a year. Physical examination demonstrated a deviation of the uvula, difficulty in protruding tongue, and bilateral tongue atrophy. Laboratory assessments revealed anemia and prominent monoclonal elevation of IgG levels both in serum and cerebrospinal fluid (CSF). The diagnosis of IgG-MM was confirmed by the identification of plasmacytosis in bone marrow aspiration and biopsy and elevation of γ-M protein in serum protein electrophoresis (SPEP). Therefore, the patient began to receive the chemotherapy with PAD (bortezomib-doxorubicin-dexamethasone) regimen. Her condition had been under control. MM as a hematological malignancy can affect cranial nerves and present as chronic progressive bulbar palsy.

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

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.