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1.
Cureus ; 16(5): e60472, 2024 May.
Artículo en Inglés | MEDLINE | ID: mdl-38764710

RESUMEN

Pseudobulbar affect (PBA) is a neurological condition characterized by recurrent, inappropriate, and involuntary outbursts of emotion, primarily crying and laughter, which are dissociated from the individual's emotional experience. The precise underlying cause of PBA remains unknown; however, existing evidence suggests the involvement of dopaminergic, serotonergic, and glutamatergic neurotransmission within the corticopontine-cerebellar pathways responsible for regulating the motor expression of emotions. Additionally, PBA has been observed to co-occur with other neurocognitive and psychiatric disorders. Therefore, it is crucial to consider the possibility of a PBA diagnosis in patients with underlying neurological damage and disorders.

2.
Cureus ; 16(3): e56116, 2024 Mar.
Artículo en Inglés | MEDLINE | ID: mdl-38618343

RESUMEN

Swallowing disorders resulting from pseudobulbar palsy are characterized by deficiencies in the oral preparatory and oral stages of the swallowing process. In certain cases, obstruction can occur when the tongue base comes into contact with the palate, impeding the intraoral bolus flow into the pharyngeal cavity. In this report, we discuss a case of severe pseudobulbar palsy, in which an intraoral bolus flowed into the pharyngeal cavity with pinching the nose. A 78-year-old man with a history of recurrent cerebral infarction was evaluated. The patient had severe dysphagia and cognitive impairment due to pseudobulbar palsy. A videofluoroscopic examination of swallowing (VF) was conducted while the patient was in a reclined position. In the oral cavity, when the bolus reached the posterior tongue section, the flow was hindered by the functional obstruction caused by the tongue base pressing against the palate. Despite the clinician's instructions to swallow, the patient was unable to comply due to the severity of his cognitive impairment. To alleviate this obstruction, the clinician pinched the patient's nose. This action opened the fauces, facilitating breathing and relieving the functional obstruction. Subsequently, the bolus flowed into the pharyngeal cavity and successfully flowed into the esophagus while swallowing. This maneuver was named the "pinching nose maneuver" (PNM). The PNM, as described here, can serve as a technique to improve the movement of an intraoral bolus into the pharyngeal cavity in patients with cognitive dysfunction.

3.
Neurol Ther ; 13(3): 739-747, 2024 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-38625649

RESUMEN

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.

4.
Artículo en Chino | WPRIM (Pacífico Occidental) | ID: wpr-1030164

RESUMEN

Objective:To observe the effect of Tongguan Liqiao(opening orifices)needling plus tongue pressure resistance feedback training in treating post-stroke deglutition disorders. Methods:A prospective randomized controlled trial was conducted.A total of 120 patients with post-stroke deglutition disorders were divided into a control group and an observation group using the random number table method,with 60 cases in each group.Both groups were treated with routine treatments.The control group was treated with additional tongue pressure resistance feedback training,and the observation group was treated with additional Tongguan Liqiao needling based on the same intervention in the control group.After 4 weeks of treatment,the clinical efficacy was evaluated,and the complications that occurred during treatment were summarized.The FUJISHIMA Ichiro food intake level scale(FILS)and the swallowing quality of life questionnaire(SWAL-QOL)were scored before treatment and after 4 weeks of treatment. Results:The total effective rate of the observation group was higher than that of the control group(P<0.05).After 4 weeks of treatment,the FILS and SWAL-QOL scores in both groups were higher than those before treatment,and the scores in the observation group were higher than those in the control group(P<0.05).There was no statistical difference in the complication occurrence between the two groups(P>0.05). Conclusion:On the basis of routine treatments,Tongguan Liqiao needling plus tongue pressure resistance feedback training has a better effect in the treatment of post-stroke deglutition disorders.This method can effectively improve the swallowing function of patients,improve the quality of life,and is safe.

5.
Cureus ; 15(5): e38976, 2023 May.
Artículo en Inglés | MEDLINE | ID: mdl-37313061

RESUMEN

Pseudobulbar affect (PBA) manifests as a disconnect between emotional feelings and emotional expressions. The impact of pseudobulbar affect on social, occupational, and interpersonal functioning is substantial. It results in poor quality of social interactions and poor overall quality of life. Instances of pseudobulbar affect with no underlying neuropsychiatric disorders are rarely reported in the literature. Although alcohol use has been associated with traumatic brain injuries (TBI), alcohol as a direct cause of pseudobulbar palsy has rarely been reported. Our case presents a unique situation with no known underlying primary neurologic disorder but evidence from clinical history, physical examination, and laboratory tests indicative of severe alcohol use disorder. This case represents the rare instances where the disease etiology is unusual and reminds the health care provider to consider the role of alcohol in the pathophysiology of pseudobulbar affect. More research is needed to understand the role of alcohol in the etiology of pseudobulbar affect in the absence of any known underlying neuropsychiatric disorder.

6.
Cureus ; 14(10): e30164, 2022 Oct.
Artículo en Inglés | MEDLINE | ID: mdl-36397902

RESUMEN

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.

7.
J Neurol Sci ; 440: 120325, 2022 09 15.
Artículo en Inglés | MEDLINE | ID: mdl-35779417

RESUMEN

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.


Asunto(s)
Esclerosis Amiotrófica Lateral , Laringoscopios , Trastornos Respiratorios , Anciano , Esclerosis Amiotrófica Lateral/complicaciones , Esclerosis Amiotrófica Lateral/diagnóstico , Deglución/fisiología , Humanos , Masculino , Examen Neurológico , Músculos Faríngeos
8.
Cureus ; 14(6): e26013, 2022 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-35855258

RESUMEN

Foix-Chavany-Marie syndrome (FCMS) is characterized by bilateral facio-glosso-pharyngo-masticatory paralysis of voluntary muscles due to bilateral infarction in the anterior opercular region of the brain. Here, we report a case of a 52-year-old female who presented with FCMS due to an acute left anterior opercular stroke in the setting of a chronic asymptomatic right opercular infarct and asymptomatic bilateral cerebellar infarcts. She also had a concurrent acute-on-chronic episode of congestive heart failure exacerbation. She made a significant recovery by the time of hospital discharge.

9.
Rev Colomb Psiquiatr (Engl Ed) ; 51(2): 163-166, 2022.
Artículo en Inglés, Español | MEDLINE | ID: mdl-35803687

RESUMEN

OBJECTIVE: Inhalant users may develop toluene leukoencephalopathy, a devastating neuropsychiatric disorder. We present a case of toluene-induced damage to the corticospinal and the corticonuclear tracts, which presented with involuntary emotional expression disorder. METHODS: Case study of a 20-year-old man with a 3-year history of frequent solvent abuse was admitted to the Neuropsychiatry Unit of the National Institute of Neurology and Neurosurgery because "he could not speak or walk" but would keep "laughing and crying without reason". RESULTS: Neuropsychiatric examination revealed pathological laughter and crying, facial and speech apraxia, a bilateral pyramidal syndrome, and lack of control of urinary sphincter. Magnetic resonance imaging revealed a highly selective bilateral damage to the pyramidal system and the somatosensory pathway. SPECT imaging showed left fronto-parietal hypoperfusion. CONCLUSIONS: This document provides support for the understanding of involuntary emotional expression disorders as a differential diagnosis in the clinical practice of psychiatrists, as well as the functional anatomy of these conditions.


Asunto(s)
Risa , Leucoencefalopatías , Adulto , Llanto/psicología , Humanos , Risa/psicología , Leucoencefalopatías/inducido químicamente , Leucoencefalopatías/diagnóstico por imagen , Imagen por Resonancia Magnética , Masculino , Tolueno , Adulto Joven
10.
Rev. colomb. psiquiatr ; 51(2): 163-166, abr.-jun. 2022. graf
Artículo en Inglés | LILACS-Express | LILACS | ID: biblio-1394987

RESUMEN

ABSTRACT Objective: Inhalant users may develop toluene leukoencephalopathy, a devastating neuropsychiatric disorder. We present a case of toluene-induced damage to the corticospinal and the corticonuclear tracts, which presented with involuntary emotional expression dis-order. Methods: Case study of a 20-year-old man with a 3-year history of frequent solvent abuse was admitted to the Neuropsychiatry Unit of the National Institute of Neurology and Neurosurgery because "he could not speak or walk" but would keep "laughing and crying without reason". Results: Neuropsychiatric examination revealed pathological laughter and crying, facial and speech apraxia, a bilateral pyramidal syndrome, and lack of control of urinary sphincter. Magnetic resonance imaging revealed a highly selective bilateral damage to the pyramidal system and the somatosensory pathway. SPECT imaging showed left fronto-parietal hypoperfusion. Conclusions: This document provides support for the understanding of involuntary emotional expression disorders as a differential diagnosis in the clinical practice of psychiatrists, as well as the functional anatomy of these conditions.


RESUMEN Objetivo: Los usuarios de inhalantes pueden contraer leucoencefalopatía por tolueno, un trastorno neuropsiquiátrico devastador. Se presenta un caso de daño inducido por tolueno en el tracto corticoespinal y corticonuclear, que se manifestó con un trastorno involuntario de la expresión emocional. Métodos: Un varón de 20 años con antecedente de 3 años de abuso de solventes ingresó en la Unidad de Neuropsiquiatría del Instituto Nacional de Neurología y Neurocirugía porque «no podía hablar ni caminar¼ y presentaba episodios súbitos de risa y llanto sin razón aparente. Resultados: La valoración neuropsiquiátrica reveló risa y llanto patológicos, apraxia facial y fonatoria, síndrome piramidal bilateral y ausencia de control del esfínter urinario. La resonancia magnética cerebral mostró un daño bilateral muy selectivo del sistema piramidal y la vía somatosensorial. La imagen de tomografía computarizada por emisión monofotónica mostró hipoperfusión frontoparietal izquierda. Conclusiones: Este documento proporciona apoyo para la comprensión de los trastornos de la expresión emocional involuntaria como diagnóstico diferencial en la práctica clínica de los psiquiatras, así como de la anatomía funcional de estas condiciones.

11.
Zhongguo Zhen Jiu ; 42(4): 465-70, 2022 Apr 12.
Artículo en Chino | MEDLINE | ID: mdl-35403412

RESUMEN

OBJECTIVE: To analyze the acupoint selection rules of acupuncture for pseudobulbar palsy dysphagia using data mining technology. METHODS: The literature of acupuncture for pseudobulbar palsy dysphagia published from January 1, 1990 to May 1, 2021 was retrieved from CNKI, SinoMed, Wanfang, VIP, and PubMed databases. Acupuncture prescription database was established. The frequency of acupoint selection was analyzed by Microsoft Excel 2016; Apriori algorithm was used to analyze the association rules and draw the high-frequency acupoint co-occurrence network diagram; SPSS21.0 was used to perform clustering analysis. RESULTS: A total of 87 literature was included, involving 89 acupuncture prescriptions and 71 acupoints. Fengchi (GB 20) was the most frequently-used acupoint; the commonly-selected meridians were gallbladder meridian, conception vessel, governor vessel and stomach meridian; the acupoints located at the neck were the most frequently-used acupoints; the crossing points were commonly selected among the special acupoints. The most commonly-used acupoint combination was Jinjin (EX-HN 12) plus Yuye (EX-HN 13). CONCLUSION: The modern acupuncture for pseudobulbar palsy dysphagia usually selects local acupoints, especially the neck acupoints such as Fengchi (GB 20) and Lianquan (CV 23). The acupoints in the front and back are concurrently selected with needles towards the disease location.


Asunto(s)
Terapia por Acupuntura , Trastornos de Deglución , Meridianos , Parálisis Seudobulbar , Puntos de Acupuntura , Trastornos de Deglución/terapia , Humanos
12.
Artículo en Chino | WPRIM (Pacífico Occidental) | ID: wpr-927409

RESUMEN

OBJECTIVE@#To analyze the acupoint selection rules of acupuncture for pseudobulbar palsy dysphagia using data mining technology.@*METHODS@#The literature of acupuncture for pseudobulbar palsy dysphagia published from January 1, 1990 to May 1, 2021 was retrieved from CNKI, SinoMed, Wanfang, VIP, and PubMed databases. Acupuncture prescription database was established. The frequency of acupoint selection was analyzed by Microsoft Excel 2016; Apriori algorithm was used to analyze the association rules and draw the high-frequency acupoint co-occurrence network diagram; SPSS21.0 was used to perform clustering analysis.@*RESULTS@#A total of 87 literature was included, involving 89 acupuncture prescriptions and 71 acupoints. Fengchi (GB 20) was the most frequently-used acupoint; the commonly-selected meridians were gallbladder meridian, conception vessel, governor vessel and stomach meridian; the acupoints located at the neck were the most frequently-used acupoints; the crossing points were commonly selected among the special acupoints. The most commonly-used acupoint combination was Jinjin (EX-HN 12) plus Yuye (EX-HN 13).@*CONCLUSION@#The modern acupuncture for pseudobulbar palsy dysphagia usually selects local acupoints, especially the neck acupoints such as Fengchi (GB 20) and Lianquan (CV 23). The acupoints in the front and back are concurrently selected with needles towards the disease location.


Asunto(s)
Humanos , Puntos de Acupuntura , Terapia por Acupuntura , Trastornos de Deglución/terapia , Meridianos , Parálisis Seudobulbar
13.
Afr Health Sci ; 21(1): 166-171, 2021 Mar.
Artículo en Inglés | MEDLINE | ID: mdl-34394294

RESUMEN

INTRODUCTION: Pseudobulbar palsy (PBP) is characterized by supranuclear lesions in the corticobulbar pathway. Neoplasia, inflammatory, demyelinating, and stroke are possible etiologies of this disorder. CASE REPORT: We report an elderly female who presented with dysarthria. She was dysarthric with a hypernasal voice, no apraxia or aphasia was observed. Tongue movements were slow with limited amplitude. Her soft palate dropped bilaterally; gag reflex was present. Also, she reported swallowing difficulty and choking with her saliva. Bilateral vertical and horizontal gaze were intact to either voluntary or oculocephalic movements. A cranial CT scan was suggestive of artery of Percheron (AOP) infarction. Brain magnetic resonance imaging showed hypersignal on diffusion-weighted and T2-weighted images and hyposignal on apparent diffusion coefficient in both thalami. CT angiography scan revealed an AOP originating from the left posterior cerebral artery. The swallowing study with a videofluoroscopic demonstrated oral and pharyngeal phases with severe dysfunction. CONCLUSION: To the authors' knowledge, there are two cases of individuals with artery of Percheron infarction who developed PBP associated with other clinical syndromes. Still, isolated PBP following infarction of Percheron's artery was not reported. We hypothesized that the PBP may have occurred because of the existence of vascular territory variations in the perforating arteries that arise from the AOP.


Asunto(s)
Encéfalo/diagnóstico por imagen , Infarto Cerebral/diagnóstico por imagen , Imagen de Difusión por Resonancia Magnética/métodos , Disartria/etiología , Infarto/diagnóstico por imagen , Parálisis Seudobulbar/complicaciones , Tálamo/diagnóstico por imagen , Anciano , Arterias/patología , Infarto Cerebral/complicaciones , Angiografía por Tomografía Computarizada , Femenino , Humanos , Infarto/complicaciones , Imagen por Resonancia Magnética , Neuroimagen/efectos adversos , Tálamo/irrigación sanguínea , Tálamo/fisiopatología , Tomografía Computarizada por Rayos X
15.
Prion ; 15(1): 82-86, 2021 12.
Artículo en Inglés | MEDLINE | ID: mdl-34078217

RESUMEN

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.


Asunto(s)
Síndrome de Creutzfeldt-Jakob , Síndrome de Creutzfeldt-Jakob/diagnóstico por imagen , Síndrome de Creutzfeldt-Jakob/genética , Deglución , Imagen de Difusión por Resonancia Magnética , Femenino , Humanos , Persona de Mediana Edad , Faringe/diagnóstico por imagen , Tomografía Computarizada de Emisión de Fotón Único
16.
Eur J Case Rep Intern Med ; 8(4): 002462, 2021.
Artículo en Inglés | MEDLINE | ID: mdl-33987126

RESUMEN

INTRODUCTION: Foix-Chavany-Marie syndrome (FCMS) is a type of pseudobulbar palsy that affects facio-pharyngo-glosso-masticatory muscles. MATERIALS AND METHODS: A 62-year-old man was admitted to the emergency department after 9 hours of acute dysarthria and dysphagia. MRI showed restricted diffusion in the right operculum on diffusion-weighted imaging (DWI). No thrombolytic therapy was given. The patient had a history of mechanical aortic valve replacement under anticoagulation with a vitamin K antagonist. Work-up demonstrated suboptimal levels of INR. Due to severe dysphagia during hospitalization, a percutaneous endoscopic gastrostomy (PEG) was performed. RESULTS: The patient was discharged 5 days later, with a modified Rankin scale (mRs) score of 3, and secondary stroke prevention. He had achieved an excellent functional outcome (mRs 1) at 6-month follow-up. CONCLUSION: Our patient had a satisfactory recovery due to prompt diagnosis, secondary stroke prevention, and compliance with treatment. LEARNING POINTS: In the presence of acute dysarthria and dysphagia, Foix-Chavany-Marie syndrome (FCMS) should be considered.FCMS may occur in the presence of unilateral opercular stroke.Swallowing and speech therapy play an essential role in rehabilitation after the acute setting.

17.
Proc (Bayl Univ Med Cent) ; 34(3): 389-390, 2021 Feb 02.
Artículo en Inglés | MEDLINE | ID: mdl-33953472

RESUMEN

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.

18.
Brain Neurorehabil ; 14(2): e16, 2021 Jul.
Artículo en Inglés | MEDLINE | ID: mdl-36743434

RESUMEN

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.

19.
Prion ; 14(1): 180-184, 2020 12.
Artículo en Inglés | MEDLINE | ID: mdl-32627665

RESUMEN

Swallowing function in long-term survivors with Creutzfeldt-Jakob disease (CJD) remains unknown. Herein, we demonstrated serial evaluation of swallowing function in a case with V180I genetic CJD (gCJD) using videofluoroscopic examination of swallowing (VF). A 69-year-old woman was admitted to our hospital because of bradykinesia and memory disturbances 4 months after the onset of symptoms. Neurological examination revealed dementia, bradykinesia and frontal signs. Diffusion-weighted MRI revealed bilateral cortical hyperintensity in the frontal, temporal, and parietal cortices, and PRNP gene analysis indicated a V180I mutation. Her dysphagia gradually progressed, and she received percutaneous gastrostomy 42 months after the onset. VF was performed at 27, 31, 39, and 79 months after the onset. Although bolus transport from oral cavity to pharynx gradually worsened and initiation of the pharyngeal swallow was gradually delayed, the pharyngeal swallowing function was preserved even at 72 months after onset. MRI revealed no apparent atrophy of brainstem, and single photon emission computed tomography showed preserved regional cerebral blood flow in the brainstem. These findings suggest that the pathophysiology of dysphagia in a long-term survivor of V180I gCJD is that of pseudobulbar palsy, likely owing to preserved brainstem function even in the akinetic mutism state.


Asunto(s)
Síndrome de Creutzfeldt-Jakob/genética , Síndrome de Creutzfeldt-Jakob/fisiopatología , Deglución/fisiología , Mutación/genética , Proteínas Priónicas/genética , Sobrevivientes , Anciano , Femenino , Fluoroscopía , Humanos , Grabación en Video
20.
Zhongguo Zhen Jiu ; 40(4): 347-51, 2020 Apr 12.
Artículo en Chino | MEDLINE | ID: mdl-32275359

RESUMEN

OBJECTIVE: To observe the clinical effect of fire needling on dysphagia due to pseudobulbar paralysis after stroke and to compare the difference in clinical effect between fire needling and swallowing function rehabilitation training. METHODS: A total of 76 patients with dysphagia due to pseudobulbar paralysis after stroke were randomly divided into an observation group and a control group, 38 cases in each group (1 case dropped out in the control group). The both groups were based on conventional western medication treatment. Fire needle pricking was exerted at Lianquan (CV 23), Fengchi (GB 20), Wangu (GB 12), Shuigou (GV 26), Neiguan (PC 6) and Zusanli (ST 36) without needle retaining every other day in the observation group. The control group was treated with the swallowing function rehabilitation training. In both groups, treatment for 2 weeks was as one course and 2 courses of treatment with 2-day interval were required. After treatment, swallowing scores of Fujishima Ichiro and swallow quality of life questionnaire (SWAL-QOL) scores were observed in both groups, and the clinical effect was compared. Follow-up of swallowing scores of Fujishima Ichiro in 4 weeks after treatment was completed to evaluate the clinical effect. RESULTS: The clinical effective rates after treatment and follow-up were 92.1% (35/38) and 94.7% (36/38) in the observation group, higher than 75.7% (28/37) and 83.8% (31/37) in the control group (P<0.05). After treatment, the swallowing scores of Fujishima Ichiro and SWAL-QOL scores were increased in the two groups (P<0.05), and those in the observation group were higher than the control group (P<0.05). The swallowing scores of Fujishima Ichiro were increased during follow-up in the two groups (P<0.05). CONCLUSION: Fire needling has a better effect than conventional rehabilitation training in the treatment of dysphagia due to pseudobulbar paralysis after stroke, which can obviously improve the swallowing function and quality of life in patients with dysphagia.


Asunto(s)
Terapia por Acupuntura/métodos , Trastornos de Deglución/terapia , Parálisis Seudobulbar/terapia , Accidente Cerebrovascular/complicaciones , Puntos de Acupuntura , Trastornos de Deglución/etiología , Humanos , Parálisis Seudobulbar/etiología , Calidad de Vida , Resultado del Tratamiento
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