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Journal of Neurology, Neurosurgery, and... May 2019To determine the evolution and profile of cognitive and behavioural deficits in amyotrophic lateral sclerosis (ALS) and behavioural variant frontotemporal dementia...
OBJECTIVE
To determine the evolution and profile of cognitive and behavioural deficits in amyotrophic lateral sclerosis (ALS) and behavioural variant frontotemporal dementia (bvFTD) to disentangle the development of FTD in ALS and vice versa.
METHODS
In a prospective design, cognitive and behavioural profiles of 762 patients with motor predominant ALS (flail arm/leg syndrome, primary lateral sclerosis, pseudobulbar palsy, ALS) and behavioural predominant FTD (bvFTD, ALS-FTD) were determined and caregivers of patients with ALS were asked on the evolution of behavioural symptoms. Data were compared with 49 healthy controls. Cognition was measured with the Edinburgh Cognitive and Behavioral ALS Screen.
RESULTS
Evolution and features of cognitive profile of patients with motor predominant ALS were distinctly different from patients with behavioural FTD with regard to number and degree of affected cognitive domains. Also, in ALS mostly minus symptoms evolved after physical symptom onset whereas in ALS-FTD plus and minus symptoms were reported with an onset before physical degradation.
CONCLUSION
Evolution of cognitive and behavioural profile in patients with motor predominant ALS is distinctly different from those psychocognitive findings in patients with behavioural variant dementia. This may support the hypothesis that (possibly genetic) triggers decide in the preclinical phase on either motor or psychocognitive phenotypes.
Topics: Adult; Aged; Amyotrophic Lateral Sclerosis; Behavior; Case-Control Studies; Cognition; Female; Frontotemporal Dementia; Humans; Male; Middle Aged; Prospective Studies
PubMed: 30257969
DOI: 10.1136/jnnp-2018-318800 -
The Journal of Craniofacial Surgery Jan 2017This study was designed to investigate the effect of palatal lift prosthesis (PLP) on the speech of individuals with different types of dysarthria.
PURPOSE
This study was designed to investigate the effect of palatal lift prosthesis (PLP) on the speech of individuals with different types of dysarthria.
PARTICIPANTS
Thirty (19 males and 11 females) native speakers of Jordanian Arabic with dysarthria participated in the study. The age of the participants ranged from 8 to 67 years with an average of 34.1 years. Traumatic brain injury was the most common etiology of dysarthria among 12 participants, stroke among 11, multiple sclerosis among 3, and pseudobulbar palsy among 2; 1 participant had Parkinson disease, and another participant had amyotrophic lateral sclerosis.
METHODS
Five acoustic and aerodynamic measures were evaluated to determine the speech outcomes including nasalance scores, sequential motion rate, speech rate, vital capacity, and sound pressure level. The acoustic measures were obtained from the participants in PLP-out and PLP-in conditions.
RESULTS
Results showed statistically significant decrease in the nasalance scores of the syllable repetition, vowel prolongation, and sentence repetition tasks in the PLP-in condition below the 28% cutoff score. Furthermore, results revealed statistically significant increase in sequential motion rate, speech rate, vital capacity, and sound pressure level (P = 0.000).
CONCLUSION
The use of PLP is an effective treatment option of dysarthric speech. Besides nasalance scores, the sequential motion rate, speech rate, vital capacity, and sound pressure level are considered reliable speech measures that may be used to evaluate the effect of PLP on dysarthria.
Topics: Adolescent; Adult; Aged; Child; Dysarthria; Female; Follow-Up Studies; Humans; Male; Middle Aged; Palate; Prosthesis Implantation; Sound Spectrography; Speech; Speech Acoustics; Speech Intelligibility; Speech Production Measurement; Treatment Outcome; Vital Capacity; Young Adult
PubMed: 27831974
DOI: 10.1097/SCS.0000000000003167 -
European Journal of Neurology Jan 2020
Topics: Brain; Dysarthria; Humans; Leukoencephalopathy, Progressive Multifocal; Male; Sarcoidosis; Speech
PubMed: 31448461
DOI: 10.1111/ene.14067 -
Acta Clinica Croatica Jun 2015The aim of the study was to determine the prevalence of pseudobulbar affect (PBA) in patients with multiple sclerosis (MS) and to analyze the link between PBA and...
The aim of the study was to determine the prevalence of pseudobulbar affect (PBA) in patients with multiple sclerosis (MS) and to analyze the link between PBA and patient age, sex, clinical course of MS, disease duration and degree of disability. The study was conducted on 79 MS patients that underwent inpatient rehabilitation at the Lipik Special Hospital for Medical Rehabilitation in the period from August 15, 2014 to February 15, 2015. PBA is a term used for an emotional disinhibition syndrome characterized by sudden and involuntary episodes of crying or laughing which are not in proportion to the stimulus applied or occur without stimulus. The condition can be present in patients with various neurological disorders, such as amyotrophic lateral sclerosis, Alzheimer's disease, Parkinson's disease, patients having recovered from stroke, or following traumatic brain injury. The estimated prevalence in patients with MS ranges from 10% to 46.2%. As a measuring instrument in the study, we used the Center for Neurologic Study-Lability Scale (CNS-LS), where a sum 17 denoted positive finding. The total number of respondents was 79, of which 33 (41.8%) met the CNS-LS criteria for the diagnosis of PBA. There was no statistically significant correlation between PBA, age and degree of disability, although PBA was more common in women and in patients with a secondary progressive form of the disease. We found that 42.4% of respondents with positive CNS-LS criteria for PBA did not inform their neurologist on the presence of sudden mood changes. The high frequency of PBA and the fact that a significant proportion of patients did not inform the neurologist on their affective disturbances call for an active approach to diagnosis and treatment.
Topics: Adult; Aged; Antipsychotic Agents; Croatia; Disability Evaluation; Female; Follow-Up Studies; Humans; Male; Middle Aged; Multiple Sclerosis; Prevalence; Prognosis; Pseudobulbar Palsy; Retrospective Studies; Young Adult
PubMed: 26415311
DOI: No ID Found -
Child's Nervous System : ChNS :... Jun 2018Hashimoto's encephalopathy (HE) is an autoimmune condition with varied neurological and psychiatric features. HE is very unusual as a cause of pseudobulbar palsy (PSP).
INTRODUCTION
Hashimoto's encephalopathy (HE) is an autoimmune condition with varied neurological and psychiatric features. HE is very unusual as a cause of pseudobulbar palsy (PSP).
CASE PRESENTATION
A 14-year-old male was admitted with right-sided weakness, dysphagia, speech disorder, and aggressiveness. Brain magnetic resonance imaging showed increased intensity in bilateral temporal, insular cortex, amygdala, and parahippocampal area on T2-weighted and fluid-attenuated inversion recovery images. Autoimmune encephalitis was considered as the patient had subacute onset of psychiatric and motor disturbances with normal findings for cerebrospinal fluid. N-methyl-D-aspartate receptor, anti-glutamate-type α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid 1 and 2, anti-contactin-associated protein-like 2, anti-gamma-aminobutyric acid receptor, anti-Leucine-rich, and glioma-inactivated 1 antibodies were negative but the anti-thyroperoxidase (antiTPO) level was greater than 998 IU/ML (n:0-9). Steroid therapy was initiated as pulse therapy and maintained with 2-mg/kg/day dose with the diagnosis of HE. He was symptom free for 6 months. In the follow-up period, he had two recurrences which responded to steroid therapy.
CONCLUSION
The common causes of PSP are demyelinating, vascular, and motor neuron diseases and congenital malformations of the opercular or insular cortex. However, there are no cases of PSP developing after any autoimmune encephalitis. This case highlights the importance of early detection of antiTPO antibodies with the findings of PSP due to autoimmune encephalitis.
Topics: Adolescent; Encephalitis; Hashimoto Disease; Humans; Male; Pseudobulbar Palsy
PubMed: 29368307
DOI: 10.1007/s00381-018-3720-2 -
Frontiers in Neurology 2019To investigate the anatomical characteristics, clinical manifestations, and imaging features of bilateral cerebral peduncular infarction. A retrospective analysis was...
To investigate the anatomical characteristics, clinical manifestations, and imaging features of bilateral cerebral peduncular infarction. A retrospective analysis was performed on 11 patients diagnosed with bilateral cerebral peduncular infarction in the Affiliated Hospital of Xuzhou Medical University from December 2014 to December 2018. Their clinical and imaging features were analyzed and summarized in combination with the relevant national and international literature. Among all the patients, there were eight cases with a history of hypertension, four cases with a history of diabetes mellitus, and four cases with a history of smoking. Conscious disturbance was observed in nine cases, quadriplegia in seven cases, pseudobulbar paralysis in three cases, and ataxia in one case. Brain magnetic resonance (MR) scans of bilateral cerebral peduncles showed patchy abnormal shadows with a hypointense signal on T1-weighted imaging (T1WI) and apparent diffusion coefficient (ADC) and hyperintense signal on T2-weighted imaging (T2WI), fluid-attenuated inversion recovery (FLAIR), and diffusion-weighted imaging (DWI). Computed tomography angiography (CTA) scans of head and neck showed severe stenosis or occlusion of vertebral artery, basilar artery, or posterior cerebral artery. All the patients received standardized treatment for cerebral infarction. Six patients died while five were left disabled. Bilateral cerebral peduncle infarction may be related to cerebral perfusion insufficiency caused by the stenosis or occlusion of vertebrobasilar artery and its branches. The main clinical manifestations are locked-in syndrome and persistent vegetative state. The specific imaging feature of "Mickey Mouse ear"-like infarction is associated with a poor prognosis.
PubMed: 31708855
DOI: 10.3389/fneur.2019.01107 -
European Journal of Case Reports in... 2021Foix-Chavany-Marie syndrome (FCMS) is a type of pseudobulbar palsy that affects facio-pharyngo-glosso-masticatory muscles.
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.
PubMed: 33987126
DOI: 10.12890/2021_002462 -
Dysphagia Mar 2024Patients with pseudobulbar palsy often present with velopharyngeal incompetence. Velopharyngeal incompetence is usually observed during expiratory activities such as...
Patients with pseudobulbar palsy often present with velopharyngeal incompetence. Velopharyngeal incompetence is usually observed during expiratory activities such as speech and/or blowing during laryngoscopy. These patients typically exhibit good velopharyngeal closure during swallowing, which is dissociated from expiratory activities. We named this phenomenon "speech-swallow dissociation" (SSD). SSD on endoscopic findings can help in diagnosing the underlying disease causing dysphagia. This endoscopic finding is qualitative, and the quantitative characteristics of SSD are still unclear. Accordingly, the current study aimed to quantitatively evaluate SSD in patients with pseudobulbar palsy. We evaluated velopharyngeal pressure during swallowing and expiratory activity in 10 healthy subjects and 10 patients with pseudobulbar palsy using high-resolution manometry, and compared the results between the two groups. No significant differences in maximal velopharyngeal contraction pressure (V-Pmax) were observed during dry swallowing between the pseudobulbar palsy group and healthy subjects (190.5 mmHg vs. 173.6 mmHg; P = 0.583). V-Pmax during speech was significantly decreased in the pseudobulbar palsy group (85.4 mmHg vs. 34.5 mmHg; P < 0.001). The degree of dissociation of speech to swallowing in V-Pmax, when compared across groups, exhibited a larger difference in the pseudobulbar palsy group, at 52% versus 80% (P = 0.001). Velopharyngeal pressure during blowing was similar to that during speech. Velopharyngeal closure in patients with pseudobulbar palsy exhibited weaker pressure during speech and blowing compared with swallowing, quantitatively confirming the presence of SSD. Pseudobulbar palsy often presents with SSD, and this finding may be helpful in differentiating the etiology of dysphagia.
PubMed: 38492048
DOI: 10.1007/s00455-024-10687-1 -
The Journal of the Association of... Feb 2017Opercular syndrome, also known as Foix-Chavany-Marie syndrome, is characterised by paralysis of the facial, masticatory, pharyngeal, laryngeal, brachial and tongue...
Opercular syndrome, also known as Foix-Chavany-Marie syndrome, is characterised by paralysis of the facial, masticatory, pharyngeal, laryngeal, brachial and tongue muscles. It is a cortical form of pseudobulbar palsyo which is commonly caused by a vascular aetiology. The clinical presentation is anarthria, weakness of voluntary muscles involving face, tongue, pharynx, larynx, and masticatory muscles. However, autonomic reflexes and emotional functions of these structures are preserved. We report a case of a 61 year old man who had difficulty in chewing, swallowing and vocalising since one and a half month, which on imaging was found to be opercular syndrome.
Topics: Deglutition Disorders; Dysarthria; Facial Paralysis; Humans; Male; Mastication; Middle Aged; Sialorrhea; Voice Disorders
PubMed: 28457042
DOI: No ID Found -
BMJ Case Reports Oct 2021We present a case of a 73-year-old man who developed sudden onset dysarthria, dysphagia and bilateral facial weakness with automato-voluntary dissociation, which...
We present a case of a 73-year-old man who developed sudden onset dysarthria, dysphagia and bilateral facial weakness with automato-voluntary dissociation, which deteriorated rapidly to anarthria and aphonia within a few days. MRI scan of the head showed acute infarct in right internal capsule and an old infarct in the left corona radiata while the rest of the investigations were normal. Based on these findings, diagnosis was thought to be subopercular syndrome. He recovered significantly in a few weeks' time.
Topics: Aged; Dysarthria; Humans; Internal Capsule; Magnetic Resonance Imaging; Male; Pseudobulbar Palsy; Syndrome
PubMed: 34711624
DOI: 10.1136/bcr-2021-245613