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Neurology and Clinical Neuroscience May 2017Early clinical diagnosis of progressive supranuclear palsy (PSP) remains challenging.
BACKGROUND AND AIM
Early clinical diagnosis of progressive supranuclear palsy (PSP) remains challenging.
AIM
We attempted to identify any sign or symptom to diagnose PSP earlier.
METHODS
A total of 401 patients, 40 with PSP and 361 with other neurodegenerative disorders, were included. We followed these patients for at least 1 year since 2009. We reviewed the signs and symptoms of patients with PSP in a standardized manner, and observed four manifestations: "vertical supranuclear gaze abnormality," "movement disorders," "pseudobulbar palsy" and "dementia of frontal type." Features, such as symmetric parkinsonism, freezing of gait, postural instability, dysarthria and/or dysphagia, or dementia of frontal type, were considered core clinical features.
RESULTS
In patients with PSP, "movement disorders" was the most common manifestation, whereas "vertical supranuclear gaze abnormality" was uncommon during the early disease course. A total of 16 patients fulfilled the National Institute for Neurological Disorders and Stroke and Society for PSP criteria for possible PSP at their first clinic visit. Of the remaining 24 patients, 15 presented with one or more core clinical features before fulfilling the criteria for possible PSP; nine patients had a clinical diagnosis of PSP but never fulfilled the criteria. A total of 49 of the 361 patients with other neurodegenerative disorders had core clinical features. A comparison showed that freezing of gait differentiated the groups the best over the disease course.
CONCLUSION
Freezing of gait is an early feature that might improve the clinical diagnosis of PSP, whereas vertical supranuclear gaze abnormality is not.
PubMed: 28702192
DOI: 10.1111/ncn3.12122 -
Cureus May 2024Pseudobulbar affect (PBA) is a neurological condition characterized by recurrent, inappropriate, and involuntary outbursts of emotion, primarily crying and laughter,...
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.
PubMed: 38764710
DOI: 10.7759/cureus.60472 -
Experimental and Therapeutic Medicine Jul 2022Rare neurodegenerative disorders may be considered in the differential diagnosis of Parkinsonism in patients with schizophrenia who show worsening signs of Parkinsonism...
Rare neurodegenerative disorders may be considered in the differential diagnosis of Parkinsonism in patients with schizophrenia who show worsening signs of Parkinsonism under treatment with antipsychotics. To the best of our knowledge, the present study is the first report describing probable progressive supranuclear palsy (PSP) in a patient with chronic schizophrenia. A 64-year-old man presented with hallucinations, delusions and asociality. He had received treatment with both typical and atypical antipsychotics for ~13 years. He began experiencing short-term memory impairment and bradykinesia two years before presentation, and then showed increased dysphagia, upper-limb muscle rigidity, extrapyramidal symptoms, vision loss and photophobia. Psychological manifestations included chronic depression, irritability and, occasionally, euphoria. His gait worsened, leading to repeated falls. Antipsychotics were discontinued, and the patient was almost completely dependent on a wheelchair in daily life. In a neurology consultation, he was diagnosed with probable progressive supranuclear palsy-Richardson's syndrome presenting as vertical supranuclear gaze palsy and prominent postural instability with falls. Brain magnetic resonance imaging (MRI) revealed atrophy of the mesencephalic tegmentum, and I-ioflupane single-photon emission computed tomography (SPECT) revealed reduced bilateral striatal reuptake. Overall, PSP should be considered in patients with schizophrenia with worsening Parkinsonism, especially when it is accompanied by supranuclear ophthalmoplegia, pseudobulbar palsy, dysarthria and dystonic stiffness of the neck and upper body. In the present case, the combination of brain MRI and I-ioflupane SPECT helped to discriminate PSP from other Parkinsonian syndromes, including drug-induced Parkinsonism, in the differential diagnosis.
PubMed: 35761809
DOI: 10.3892/etm.2022.11411 -
African Health Sciences Mar 2021Pseudobulbar palsy (PBP) is characterized by supranuclear lesions in the corticobulbar pathway. Neoplasia, inflammatory, demyelinating, and stroke are possible... (Review)
Review
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.
Topics: Aged; Arteries; Brain; Cerebral Infarction; Computed Tomography Angiography; Diffusion Magnetic Resonance Imaging; Dysarthria; Female; Humans; Infarction; Magnetic Resonance Imaging; Neuroimaging; Pseudobulbar Palsy; Thalamus; Tomography, X-Ray Computed
PubMed: 34394294
DOI: 10.4314/ahs.v21i1.22 -
Evidence-based Complementary and... 2023The objective is to evaluate the clinical efficacy of cross electro-nape-acupuncture (CENA) in the treatment of pseudobulbar palsy in patients with tracheotomy...
A Multicentre Randomized Controlled Clinical Study of Cross Electro-Nape-Acupuncture (CENA) for the Treatment of Pseudobulbar Palsy in Patients with Tracheotomy Intubation for Severe Cerebral Haemorrhage.
OBJECTIVE
The objective is to evaluate the clinical efficacy of cross electro-nape-acupuncture (CENA) in the treatment of pseudobulbar palsy in patients with tracheotomy intubation for severe cerebral haemorrhage and to provide an innovative acupuncture method for the treatment of such patients.
METHODS
A total of 126 patients from six trial centres who met the inclusion criteria were randomly divided into three groups according to the random number table method in the ratio of 1 : 1 : 1, with 42 patients in each group, and the three groups were divided into CENA group, electro-acupuncture group, and acupuncture group. Each group's acupuncture treatment lasted for 30 minutes, and the needles were removed at the end of the treatment. Acupuncture was performed once a week on Sunday only and twice a day from Monday to Saturday, a total of 4 weeks of treatment. The SWT, FDA, ChSWAL-QOL, and TCRGS scores of the three groups of patients before and after treatment were compared to evaluate the effect of CENA on remodelling the function of swallowing reflex and cough reflex and promoting the recovery of dysarthria and swallowing quality of life in pseudobulbar palsy in patients with tracheotomy intubation for severe cerebral haemorrhage.
RESULTS
After treatment, the WST and TCRGS grade scores decreased and the FDA and ChSWAL-QOL scores increased significantly in all three groups compared with the pretreatment scores and were statistically significant. There was a significant difference between the three groups for these four indicators after treatment; the comparison between groups showed significant differences in the CENA group compared to the electro-acupuncture and acupuncture groups. The efficiency of the CENA group was significantly better than that of the electro-acupuncture and acupuncture groups.
CONCLUSION
Compared with the acupuncture and electro-acupuncture groups, the CENA could better promote the remodelling of swallowing function and cough reflex function, promote the recovery of dysarthria, and better improve the quality of life of patients with pseudobulbar palsy from tracheotomy intubation in severe cerebral haemorrhage.
PubMed: 37152374
DOI: 10.1155/2023/9304934 -
The Lancet. Neurology Dec 2018Understanding how prevalence, incidence, and mortality of motor neuron diseases change over time and by location is crucial for understanding the causes of these...
BACKGROUND
Understanding how prevalence, incidence, and mortality of motor neuron diseases change over time and by location is crucial for understanding the causes of these disorders and for health-care planning. Our aim was to produce estimates of incidence, prevalence, and disability-adjusted life-years (DALYs) for motor neuron diseases for 195 countries and territories from 1990 to 2016 as part of the Global Burden of Diseases, Injuries, and Risk Factors Study (GBD) 2016.
METHODS
The motor neuron diseases included in this study were amyotrophic lateral sclerosis, spinal muscular atrophy, hereditary spastic paraplegia, primary lateral sclerosis, progressive muscular atrophy, and pseudobulbar palsy. Incidence, prevalence, and DALYs were estimated using a Bayesian meta-regression model. We analysed 14 165 site-years of vital registration cause of death data using the GBD 2016 cause of death ensemble model. The 84 risk factors quantified in GBD 2016 were tested for an association with incidence or death from motor neuron diseases. We also explored the relationship between Socio-demographic Index (SDI; a compound measure of income per capita, education, and fertility) and age-standardised DALYs of motor neuron diseases.
FINDINGS
In 2016, globally, 330 918 (95% uncertainty interval [UI] 299 522-367 254) individuals had a motor neuron disease. Motor neuron diseases have caused 926 090 (881 566-961 758) DALYs and 34 325 (33 051-35 364) deaths in 2016. The worldwide all-age prevalence was 4·5 (4·1-5·0) per 100 000 people, with an increase in age-standardised prevalence of 4·5% (3·4-5·7) over the study period. The all-age incidence was 0·78 (95% UI 0·71-0·86) per 100 000 person-years. No risk factor analysed in GBD showed an association with motor neuron disease incidence. The largest age-standardised prevalence was in high SDI regions: high-income North America (16·8, 95% UI 15·8-16·9), Australasia (14·7, 13·5-16·1), and western Europe (12·9, 11·7-14·1). However, the prevalence and incidence were lower than expected based on SDI in high-income Asia Pacific.
INTERPRETATION
Motor neuron diseases have low prevalence and incidence, but cause severe disability with a high fatality rate. Incidence of motor neuron diseases has geographical heterogeneity, which is not explained by any risk factors quantified in GBD, suggesting other unmeasured risk factors might have a role. Between 1990 and 2016, the burden of motor neuron diseases has increased substantially. The estimates presented here, as well as future estimates based on data from a greater number of countries, will be important in the planning of services for people with motor neuron diseases worldwide.
FUNDING
Bill & Melinda Gates Foundation.
Topics: Age Distribution; Aged; Aged, 80 and over; Bayes Theorem; Cause of Death; Disabled Persons; Female; Global Burden of Disease; Humans; Incidence; Life Expectancy; Male; Middle Aged; Motor Neuron Disease; Prevalence
PubMed: 30409709
DOI: 10.1016/S1474-4422(18)30404-6 -
The Tohoku Journal of Experimental... Mar 2023Pseudobulbar palsy and bulbar palsy cause dysphagia in patients with amyotrophic lateral sclerosis (ALS). Dysphagia in patients with ALS not only increases the risk of...
Pseudobulbar palsy and bulbar palsy cause dysphagia in patients with amyotrophic lateral sclerosis (ALS). Dysphagia in patients with ALS not only increases the risk of aspiration and pneumonia but also leads to malnutrition and weight loss, which are poor prognostic factors. Gastrostomy is the preferred route of feeding and nutritional support in patients with dysphagia. However, there are no established standards to determine the ideal timing of gastrostomy for patients with ALS. Therefore, we used the videofluoroscopic dysphagia scale (VDS), which objectively quantifies swallowing function, in videofluoroscopic swallowing study (VFSS) to investigate whether this scale at diagnosis can be a useful predictor for the timing of gastrostomy. We retrospectively evaluated 22 patients with ALS who were diagnosed at our hospital. We assessed the VDS scores in all patients within 3 months of diagnosis. A decline in the ALS functional rating scale revised (ALSFRS-R) scores was used as an indicator of disease progression. As a result, we found that the VDS score of the pharyngeal phase and the total VDS score were significantly correlated with the ΔALSFRS-R scores. These scores were also associated with the existing indicators for the timing of gastrostomy, i.e., decreased body weight and percent-predicted forced vital capacity. We demonstrated the noninferiority of the VDS scores relative to the existing indicators. In addition, the VDS score of the pharyngeal phase was significantly correlated with the time from diagnosis to gastrostomy. The VDS score could estimate the timing of gastrostomy in patients with ALS with dysphagia at diagnosis.
Topics: Humans; Deglutition Disorders; Amyotrophic Lateral Sclerosis; Gastrostomy; Retrospective Studies; Deglutition
PubMed: 36696983
DOI: 10.1620/tjem.2023.J005 -
Case Reports in Critical Care 2024Traumatic brain injury (TBI) can have profound acute and chronic effects, leading to permanent disabilities and diminished quality of life. Pseudobulbar palsy and its...
Traumatic brain injury (TBI) can have profound acute and chronic effects, leading to permanent disabilities and diminished quality of life. Pseudobulbar palsy and its infrequent subtype, Foix-Chavany-Marie Syndrome (FCMS), represent rare complications of TBI, manifesting as deficits in craniofacial motor function and automatic-voluntary dissociation. We present a case of a 58-year-old male who developed FCMS following severe TBI from a cycling accident. Initial imaging revealed extensive brain injury with subsequent development of FCMS characterised by bilateral cranial nerve dysfunction, notably facio-pharyngo-glosso-masticatory diplegia with preserved automatic motor function. This case contributes to the limited literature on traumatic FCMS, highlighting its distinct clinical features and potential for favourable outcomes compared to nontraumatic cases. Early recognition and comprehensive management, including supportive therapy and addressing underlying conditions, are paramount for optimising patient outcomes.
PubMed: 38766550
DOI: 10.1155/2024/6652867 -
Emotional Incontinence: A Case Report of Pseudobulbar Affect in the Setting of Alcohol Use Disorder.Cureus May 2023Pseudobulbar affect (PBA) manifests as a disconnect between emotional feelings and emotional expressions. The impact of pseudobulbar affect on social, occupational, and...
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.
PubMed: 37313061
DOI: 10.7759/cureus.38976 -
BMC Neurology Mar 2019We aimed to produce a detailed neuropathological analysis of pyramidal motor system pathology and provide its clinical pathological correlation in cases with definite...
BACKGROUND
We aimed to produce a detailed neuropathological analysis of pyramidal motor system pathology and provide its clinical pathological correlation in cases with definite progressive supranuclear palsy (PSP).
METHODS
Pyramidal motor system pathologies were analyzed in 18 cases with neuropathologically confirmed PSP. Based on a retrospective clinical analysis, cases were subtyped according to Movement Disorder Society criteria for clinical diagnosis of PSP as probable, possible or suggestive of PSP with Richardson's syndrome (n = 10), PSP with predominant corticobasal syndrome (n = 3), PSP with predominant parkinsonism (n = 3), PSP with predominant speech/language disorder (n = 1), and PSP with progressive gait freezing (n = 1). Clinical manifestations of motor neuron involvement (pseudobulbar or bulbar signs and spasticity) were retrospectively assessed semiquantitatively. Neuropathologically, hyperphosphorylated tau-related pyramidal motor system neuronal, neuritic, and glial pathology using anti-tau AT8 clone immunohistochemistry, was also evaluated.
RESULTS
Clinical manifestations of pyramidal motor system involvement were found in patients with different PSP subtypes. A statistically significant higher load of tau pathology was found in the pyramidal system in PSP-Richardson's syndrome compared to other PSP subtypes (p = 0.016); however, there was no significant correlation between pyramidal system tau pathology and related motor clinical symptoms.
CONCLUSIONS
Tau pathology in the spinal cord and pyramidal motor system structures is very common in progressive supranuclear palsy and may neuropathologically supplement the distinction between classic Richardson's syndrome from other progressive supranuclear palsy subtypes.
Topics: Aged; Aged, 80 and over; Cerebral Cortex; Female; Humans; Immunohistochemistry; Male; Middle Aged; Movement Disorders; Parkinsonian Disorders; Pyramidal Tracts; Retrospective Studies; Supranuclear Palsy, Progressive; tau Proteins
PubMed: 30894142
DOI: 10.1186/s12883-019-1270-1