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Neurology and Therapy Jun 2024Pseudobulbar palsy is a common symptom in patients with amyotrophic lateral sclerosis (ALS), but it is often underdiagnosed or misdiagnosed as other diseases. The Center...
INTRODUCTION
Pseudobulbar palsy is a common symptom in patients with amyotrophic lateral sclerosis (ALS), but it is often underdiagnosed or misdiagnosed as other diseases. The Center for Neurologic Study Lability Scale (CNS-LS) is a self-report scale consisting of seven questions designed for evaluating pseudobulbar affect (PBA). The current study aimed to validate a Chinese version of the CNS-LS.
METHODS
The Chinese version of the CNS-LS was obtained through a standardized forward-backward translation and cultural adaptation. A total of 105 patients with ALS were recruited from the ALS database of Peking University Third Hospital in Beijing, China, to complete the CNS-LS. The reliability of the Chinese version was determined by the test-retest method, and receiver operating characteristic (ROC) analysis was performed for criterion validity.
RESULTS
Of 105 patients with ALS, 37 had symptoms of PBA and were diagnosed with that condition by neurologists. Forty-two patients completed the CNS-LS twice, and there was no statistically significant difference between the scores (Z = -0.896, p = 0.37). The Spearman correlation coefficient between the test and retest scores was 0.940 (p < 0.0005), and the Cronbach alpha coefficient was high (α = 0.905, n = 105). Scores of 12 or higher on the CNS-LS identified PBA with sensitivity of 0.919 and specificity of 0.882. The area under the ROC curve was 0.924.
CONCLUSION
The Chinese version of the CNS-LS demonstrated good sensitivity and specificity in the group of patients with ALS enrolled in this study. The CNS-LS should be a useful instrument for clinical and research purposes for patients in this language group.
PubMed: 38625649
DOI: 10.1007/s40120-024-00605-w -
Cureus Oct 2022Dysphagia in pseudobulbar palsy is characterized by impairment of the oral stage of swallowing. The flow of the bolus from the oral cavity into the pharynx at the fauces...
Dysphagia in pseudobulbar palsy is characterized by impairment of the oral stage of swallowing. The flow of the bolus from the oral cavity into the pharynx at the fauces may be blocked in some patients, which prevents the bolus flow by contact of the tongue with the palate. Herein, we demonstrated a case with pseudobulbar palsy who could deliver bolus from the oral cavity to the pharynx by vocalizing "ee." An 81-year-old man presented with a recurrent cerebral infarction due to cardiogenic embolism. He presented with pseudobulbar palsy and had severe dysphagia due to bilateral cerebral hemisphere lesions. On day 84, a videofluoroscopic examination of swallowing was performed in a 30° reclining posture. When the bolus reached the posterior part of the tongue in the oral cavity, the clinician asked the patient to say "ee." The base of the tongue moved forward and downward, and the anterior to the middle part of the tongue was elevated in the mouth. As a result, the fauces opened, the functional blockage was released, and the bolus flowed into the pharyngeal cavity. Shortly after the swallowing reflex, the bolus passed through the pharynx. We have named this swallowing maneuver the "ee" maneuver. The "ee" maneuver can be one of the swallowing methods to improve bolus transport from the oral cavity to the pharynx in patients with dysphagia and cognitive impairment due to pseudobulbar palsy.
PubMed: 36397902
DOI: 10.7759/cureus.30164 -
Cureus Jul 2021Posterior fossa tumors constitute the most common brain tumor in pediatrics with 25% development postresection. Cerebellar mutism can manifest as neurobehavioral...
Posterior fossa tumors constitute the most common brain tumor in pediatrics with 25% development postresection. Cerebellar mutism can manifest as neurobehavioral abnormalities that can occur within days to months after surgery but usually peak on the third postoperative day. It can be caused by discontinuation of dento-thalamo-cortical pathway in the vermian lesion, due to edema, tumors, and hypoperfusion. We report a seven-year-old patient with posterior fossa lesion (pilocytic astrocytoma in histopathology) and learning difficulties with symptoms of urinary retention, pseudobulbar palsy, and motor incoordination that were treated successfully with zolpidem 2.5 mg with regain of function by the third month.
PubMed: 34447647
DOI: 10.7759/cureus.16616 -
Korean Journal of Neurotrauma Apr 2022Central pontine myelinolysis (CPM) is a disorder wherein variable symptoms are associated with pontine dysfunction. It has been known to occur inconstantly, particularly...
Central pontine myelinolysis (CPM) is a disorder wherein variable symptoms are associated with pontine dysfunction. It has been known to occur inconstantly, particularly when serum sodium in patients with prolonged hyponatremia is rapidly corrected. Further, it is known that patients with liver diseases, malnutrition, malignancy, adrenal insufficiency, and metabolic derangements are more vulnerable to this disorder. However, there is limited literature about the occurrence of CPM in patients with traumatic brain injury, especially in those with normal serum sodium levels. A 36-year-old man having no medical history was bought to our hospital due to an open skull fracture and underwent surgery. During the hospitalization period, he showed a sudden pseudobulbar palsy and rigidity. Imaging study of the brain was characteristic for CPM. He had no fluctuation of serum sodium levels during the hospitalization period. We speculate that the brain trauma itself might cause a CPM, and its pathophysiology may not be related to rapid serum sodium correction.
PubMed: 35557639
DOI: 10.13004/kjnt.2022.18.e3 -
BMJ Case Reports Nov 2020Carotid artery dissection is one of the most common causes of ischaemic stroke in young and middle-aged population. We report a case of bilateral carotid artery...
Carotid artery dissection is one of the most common causes of ischaemic stroke in young and middle-aged population. We report a case of bilateral carotid artery dissection presenting with opercular syndrome or Foix-Chavany-Marie syndrome. This 46-year-old obese and hypertensive man with a history of fall from bike 1 week prior, presented with sudden onset of anarthria, dysphagia and deviation of angle of mouth. His speech and dysphagia gradually improved over 10 days to normal, but he developed pseudobulbar affect and difficulty in calculations 4 weeks later. MRI showed acute infarcts in bilateral operculum. CT angiography showed dissection in bilateral cervical ICAs. He was managed conservatively with oral anticoagulation, given for 6 months. Probability of dissection must be considered in patients with a history of trauma or falls developing focal neurological deficits. To our knowledge, this is the first case report of bilateral ICA dissection presenting with Foix-Chavany-Marie syndrome.
Topics: Aortic Dissection; Brain; Carotid Arteries; Carotid Artery Diseases; Computed Tomography Angiography; Deglutition Disorders; Dysarthria; Facial Paralysis; Humans; Ischemic Stroke; Magnetic Resonance Imaging; Male; Middle Aged; Obesity
PubMed: 33257395
DOI: 10.1136/bcr-2020-239080 -
Proceedings (Baylor University. Medical... Feb 2021Foix-Chavany-Marie syndrome (FCMS) is a cortical-subcortical pseudobulbar palsy characterized by automatic voluntary dissociation of...
Foix-Chavany-Marie syndrome (FCMS) is a cortical-subcortical pseudobulbar palsy characterized by automatic voluntary dissociation of facio-masticatory-pharyngo-glosso-laryngeal movements. FCMS is typically caused by vascular insults on the bilateral anterior opercular or adjacent subcortical areas. Acute onset of FCMS secondary to a unilateral lesion is extremely rare. Herein we present a case of FCMS caused by acute unilateral anterior opercular infarction with preexisting bilateral leukoaraiosis. Our case shows that an acute unilateral anterior opercular lesion can decompensate preexisting corticobulbar-subcortical lesions and cause the typical features of FCMS.
PubMed: 33953472
DOI: 10.1080/08998280.2021.1878976 -
Prion Dec 2021Swallowing function in long-term survivors of Creutzfeldt-Jakob disease (CJD) has not been elucidated. Herein, we report a patient with MM2-cortical-type sporadic CJD...
Swallowing function in long-term survivors of Creutzfeldt-Jakob disease (CJD) has not been elucidated. Herein, we report a patient with MM2-cortical-type sporadic CJD (MM2C-type sCJD) with long-term preservation of pharyngeal swallowing function using videofluoroscopic (VF) examination of swallowing. A 55-year-old woman was admitted to hospital because of dyscalculia and memory disturbance 3 years after the onset of these symptoms. Neurological examination revealed dementia, extrapyramidal signs, and delusion. Diffusion-weighted MRI revealed bilateral hyperintensity in the basal ganglia and frontal, temporal, and parietal cortices. No mutation with the methionine homozygote at codon 129 was found on PRNP gene analysis. VF was performed 68 months after the onset. Although bolus transport from the oral cavity to the pharynx worsened, the pharyngeal swallowing function was preserved even 68 months after onset. Serial MRI examinations revealed no apparent atrophy of the brainstem. Single photon emission computed tomography revealed that the regional cerebral blood flow in the brainstem was preserved. These findings suggest that pseudobulbar palsy is the pathophysiology underlying dysphagia in long-term survivors of MM2C-type sCJD, probably owing to preserved brainstem function even in a state of akinetic mutism.
Topics: Creutzfeldt-Jakob Syndrome; Deglutition; Diffusion Magnetic Resonance Imaging; Female; Humans; Middle Aged; Pharynx; Tomography, Emission-Computed, Single-Photon
PubMed: 34078217
DOI: 10.1080/19336896.2021.1930851 -
PloS One 2019Congenital Zika Syndrome (CZS) is a unique pattern of congenital abnormalities found in fetuses and neonates infected with the Zika virus (ZIKV). Here, we clinically...
Congenital Zika Syndrome (CZS) is a unique pattern of congenital abnormalities found in fetuses and neonates infected with the Zika virus (ZIKV). Here, we clinically identify and characterize infants with CZS between 2015 and 2018 in Mato Grosso do Sul, Brazil-a border area with Paraguay and Bolivia. This cross-sectional study, based on primary and secondary data, tracks the cases registered in the Brazilian Public Health Reporting System through the following stages: (1) preliminary data analysis, (2) identification of the congenital syndrome cases, (3) etiologic classification of the cases, (4) active search, and (5) clinical assessment. Of the 72 investigated cases, 16 were probable cases of CZS. Of these, it was only possible to clinically assess 11 infants. Considering the 16 probable cases of CZS, nine were classified as confirmed cases, and five as potential cases of the syndrome. Regarding clinical features, brain palsy was identified in all analyzed infants. Moreover, microcephaly and pseudobulbar syndrome were found in eight infants, and hydrocephalus was found in three individuals. In addition to these conditions, seven children were malnourished. Our study may provide significant insights for other researches that aim to elucidate CZS and its clinical and populational consequences.
PubMed: 31584972
DOI: 10.1371/journal.pone.0223408 -
Case Reports in Neurology 2019Foix-Chavany-Marie syndrome (FCMS) is a rare type of pseudobulbar palsy characterized by automatic-voluntary dissociation of movements of the face, tongue, pharynx, and...
Foix-Chavany-Marie syndrome (FCMS) is a rare type of pseudobulbar palsy characterized by automatic-voluntary dissociation of movements of the face, tongue, pharynx, and masticatory muscles. Most cases are due to bilateral ischemic lesions of the anterior operculum, but the syndrome has also been described after unilateral opercular damage, either isolated or associated with contralateral cortico-nuclear tract involvement. We report a patient with FCMS due to right anterior opercular lesion with contralateral infarction of the corona radiata. The patient presented with paralysis of the face and tongue with automatic and voluntary dissociation. To our knowledge, FCMS with this peculiar lesion topography has rarely been reported. We discuss the underlying mechanism with reference to MRI and diffusion tensor imaging.
PubMed: 31824287
DOI: 10.1159/000503856 -
Tremor and Other Hyperkinetic Movements... Jun 2020Delayed parkinsonism and dystonia are recognized phenomena in osmotic demyelinating syndrome (ODS). Dopamine receptor agonists and levodopa have been reported to benefit...
BACKGROUND
Delayed parkinsonism and dystonia are recognized phenomena in osmotic demyelinating syndrome (ODS). Dopamine receptor agonists and levodopa have been reported to benefit select patients.
CASE REPORT
We report a patient with ODS with severe pseudobulbar deficits, parkinsonism and dystonia, poorly responsive to levodopa, who experienced a remarkable improvement with pramipexole.
DISCUSSION
A marked response to pramipexole with lack of response to levodopa suggests a pre-synaptic source for his deficits coupled with injuries to non-nigral compensatory structures.
HIGHLIGHTS
This case highlights a dramatic response of osmotic demyelination-induced parkinsonism/dystonia to pramipexole. A lack of response to levodopa suggests deficits in the pre-synaptic nigral as well as non-nigral compensatory structures.
Topics: Adult; Antiparkinson Agents; Deamino Arginine Vasopressin; Demyelinating Diseases; Dystonia; Epistaxis; Hemostatics; Humans; Hyponatremia; Levodopa; Locked-In Syndrome; Male; Myelinolysis, Central Pontine; Osmotic Pressure; Parkinsonian Disorders; Postoperative Hemorrhage; Pramipexole; Pseudobulbar Palsy; Rhinoplasty; Tetrahydronaphthalenes; Thiophenes; Treatment Failure; Treatment Outcome; von Willebrand Disease, Type 1
PubMed: 32775023
DOI: 10.5334/tohm.66