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Brain & NeuroRehabilitation Jul 2021Bilateral cerebral peduncular infarction (BCPI) is a very rare disorder among stroke patients. The main clinical manifestations in the previously reported BCPI case...
Bilateral cerebral peduncular infarction (BCPI) is a very rare disorder among stroke patients. The main clinical manifestations in the previously reported BCPI case reports was associated with locked-in syndrome or persistent vegetative state. Here, we present a 51-year-old woman who had pseudobulbar palsy and quadriplegia. Magnetic resonance imaging showed an acute infarction in the middle areas of the cerebral peduncle with a unique "Mickey Mouse ears" sign. Diffusion tensor imaging and tractography showed relatively preserved corticospinal tracts, but the corticobulbar tracts were not detected. Magnetic resonance angiography showed posterior cerebral artery and vertebrobasilar artery occlusion. Cerebral perfusion insufficiency due to stenosis or occlusion of the vertebrobasilar artery and its branches may lead to BCPI. The prognosis and clinical manifestations of BCPI are related to the extent of the infarction in the involved cerebral peduncle and whether other territories are involved. Isolated BCPI may present a severe pseudobulbar palsy with relatively preserved limb function depending on the involvement pattern.
PubMed: 36743434
DOI: 10.12786/bn.2021.14.e16 -
Cureus Sep 2022Rapid correction of hyponatremia is the most frequent predisposing factor for the development of central pontine myelinolysis (CPM). Alcoholism, cirrhosis, malnutrition,...
Rapid correction of hyponatremia is the most frequent predisposing factor for the development of central pontine myelinolysis (CPM). Alcoholism, cirrhosis, malnutrition, and severe burns are associated conditions that often present in combination with a rapid rise in serum sodium concentration. However, its association with hyperglycemia has not been as well established. There have been recent reports of acute to subacute presentation of CPM with hyperglycemia. We report an unusual case of a 48-year-old Caucasian male who presented with pseudobulbar palsy, ataxia, and quadriplegia with worsening pontine hyperintensities and was diagnosed with CPM in the setting of persistent hyperglycemia with normal serum sodium.
PubMed: 36312619
DOI: 10.7759/cureus.29470 -
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 -
Journal of Traditional Chinese Medicine... Feb 2018To evaluate the therapeutic effects of nape acupuncture combined with rehabilitative swallowing training for dysphagia caused by pseudobulbar palsy, and to compare it...
OBJECTIVE
To evaluate the therapeutic effects of nape acupuncture combined with rehabilitative swallowing training for dysphagia caused by pseudobulbar palsy, and to compare it with rehabilitative swallowing training alone, and to observe the improvement in quality of life after the therapy.
METHODS
One hundred patients were randomly divided into two groups: the rehabilitative swallowing training group (control group, n=50) and the nape acupuncture combined with rehabilitative swallowing training group (experimental group, n=50). Each group had 8 weeks' therapy, 5 times a week. Patients in the control group received rehabilitative swallowing training, while those in the experimental group received nape acupuncture therapy based on swallowing rehabilitation. The outcomes were assessed by the repetitive saliva-swallowing test (RSST), water swallow test (WST), standardized swallowing assessment (SSA), and a swallow quality-of-life questionnaire (SWAL-QOL). Correlations of onset age, onset frequency and lesion location with the efficacy of the acupuncture treatment were also observed.
RESULTS
The scores for RSST, WST, and SSA in both groups were lower than before the therapy (P<0.001), although the changes were more marked in the experimental group than in the control group (RSST and WST, P<0.005; SSA, P<0.001). Both groups recorded changes in SWAL-QOL index after the therapy (P<0.001); and the experimental group scored higher than the control group (P<0.001). The efficacy of acupuncture was not correlated with location (P>0.05), but was related to onset age (P<0.05) and onset frequency (P<0.01).
CONCLUSION
Nape acupuncture combined with rehabilitative swallowing training has an effect on dysphagia caused by pseudobulbar palsy and improves quality of life.
PubMed: 32185960
DOI: No ID Found -
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 -
Annals of Rehabilitation Medicine Aug 2016Bilateral paramedian thalamic infarction is a rare subtype of stroke caused by occlusion of the artery of Percheron, an uncommon variant originating from one of the...
Bilateral paramedian thalamic infarction is a rare subtype of stroke caused by occlusion of the artery of Percheron, an uncommon variant originating from one of the posterior cerebral arteries. This type of stroke has several major clinical presentations: altered mental status, behavioral amnestic impairment, aphasia or dysarthria, ocular movement disorders, motor deficits, cerebellar signs, and others. Few cases of bilateral paramedian thalamic infarction-related pseudobulbar palsy characterized by dysarthria, dysphagia, and facial and tongue weakness have been reported. We report here a rare case of acute severe pseudobulbar palsy as a manifestation of bilateral paramedian thalamic infarction.
PubMed: 27606284
DOI: 10.5535/arm.2016.40.4.751 -
Case Reports in Neurological Medicine 2017Isolated bilateral cerebral peduncular infarctions (BCPI) presenting as acute pseudobulbar palsy are rarely reported and, to the best of our knowledge, most of the...
Isolated bilateral cerebral peduncular infarctions (BCPI) presenting as acute pseudobulbar palsy are rarely reported and, to the best of our knowledge, most of the previous reports of BCPI were related to locked-in syndrome and disturbance of consciousness. Herein, we described a case of a 55-year-old man who presented with acute pseudobulbar palsy and mild tetraparesis, but preserved eye movements, with no consciousness disturbance. DWI revealed an acute infarction involving the central portion of the cerebral peduncle with a characteristic "traditional Chinese eight character" sign. The relationship between the infarcted range in the cerebral peduncle and the clinical manifestation was discussed in our report.
PubMed: 29158929
DOI: 10.1155/2017/9845917 -
Internal Medicine (Tokyo, Japan) Feb 2019Foix-Chavany-Marie syndrome (FCMS) is a rare cortical type of pseudobulbar palsy characterized by the loss of voluntary control of the facial, pharyngeal, lingual, and...
Foix-Chavany-Marie syndrome (FCMS) is a rare cortical type of pseudobulbar palsy characterized by the loss of voluntary control of the facial, pharyngeal, lingual, and masticatory muscles with preserved reflexive and autonomic functions. FCMS is generally associated with cerebrovascular diseases affecting the bilateral opercular regions. We herein report the clinical features of an 84-year-old right-handed Japanese man with FCMS due to a unilateral brain abscess. The patient's symptoms were resolved after treating the brain abscess. The present clinical results suggest that a unilateral brain abscess in the temporal operculum with a persistent old lesion in the contralateral insular cortex can induce FCMS.
Topics: Aged, 80 and over; Brain Abscess; Deglutition Disorders; Dysarthria; Facial Paralysis; Functional Laterality; Humans; Male; Treatment Outcome
PubMed: 30210128
DOI: 10.2169/internalmedicine.1500-18 -
Tissue Engineering. Part C, Methods Mar 2015Motor neurons loss plays a pivotal role in the pathoetiology of various debilitating diseases such as, but not limited to, amyotrophic lateral sclerosis, primary lateral...
Motor neurons loss plays a pivotal role in the pathoetiology of various debilitating diseases such as, but not limited to, amyotrophic lateral sclerosis, primary lateral sclerosis, progressive muscular atrophy, progressive bulbar palsy, pseudobulbar palsy, and spinal muscular atrophy. However, advancement in motor neuron replacement therapy has been significantly constrained by the difficulties in large-scale production at a cost-effective manner. Current methods to derive motor neuron heavily rely on biochemical stimulation, chemical biological screening, and complex physical cues. These existing methods are seriously challenged by extensive time requirements and poor yields. An innovative approach that overcomes prior hurdles and enhances the rate of successful motor neuron transplantation in patients is of critical demand. Iron, a trace element, is indispensable for the normal development and function of the central nervous system. Whether ferric ions promote neuronal differentiation and subsequently promote motor neuron lineage has never been considered. Here, we demonstrate that elevated iron concentration can drastically accelerate the differentiation of human embryonic stem cells (hESCs) toward motor neuron lineage potentially via a transferrin mediated pathway. HB9 expression in 500 nM iron-treated hESCs is approximately twofold higher than the control. Moreover, iron treatment generated more matured and functional motor neuron-like cells that are ∼1.5 times more sensitive to depolarization when compared to the control. Our methodology renders an expedited approach to harvest motor neuron-like cells for disease, traumatic injury regeneration, and drug screening.
Topics: Animals; Cell Differentiation; Cell Line; Cell Lineage; Embryonic Stem Cells; Fluorescent Antibody Technique; Humans; Iron; Mice; Motor Neurons; Neural Stem Cells; Reactive Oxygen Species; Transferrin
PubMed: 25036750
DOI: 10.1089/ten.TEC.2013.0725 -
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