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The Journal of Neuropsychiatry and... 2005Pseudobulbar affect (PBA) is an affective disinhibition syndrome associated with various neuropathologies, which is characterized by involuntary and inappropriate... (Review)
Review
Pseudobulbar affect (PBA) is an affective disinhibition syndrome associated with various neuropathologies, which is characterized by involuntary and inappropriate outbursts of laughter and/or crying. The PBA syndrome can be socially and occupationally disabling, and it is largely unrecognized in clinical settings. Validated instruments to distinguish PBA from other disorders of affective regulation exist and could be used to improve recognition of the disorder. There is no pharmacological therapy with a Food and Drug Administration indication for PBA, although antidepressants and dopaminergic agents have been reported to show varying levels of treatment success. Recent evidence suggests that treatment with a fixed combination of dextromethorphan and the cytochrome P450 2D6 enzyme inhibitor, quinidine, can improve PBA. This review describes the clinical and neuropathological features of PBA, and presents an overview of current and future treatment approaches.
Topics: Analgesics, Non-Narcotic; Cytochrome P-450 CYP2D6 Inhibitors; Dextromethorphan; Drug Therapy, Combination; Humans; Mood Disorders; Pseudobulbar Palsy; Quinidine
PubMed: 16387982
DOI: 10.1176/jnp.17.4.447 -
Journal of Neurology Feb 2016The aim of this study is to investigate the frequency and the clinical correlations of pseudobulbar affect (PBA) in a population-based incident cohort of ALS patients....
The aim of this study is to investigate the frequency and the clinical correlations of pseudobulbar affect (PBA) in a population-based incident cohort of ALS patients. Incident ALS cases, diagnosed in 2011 and 2012, according to El Escorial criteria were enrolled from a prospective population-based registry in Apulia, Southern Italy. Neurological status was assessed using a standard neurological examination and the revised ALS Functional Rating Scale (ALSFRSr). The Center for Neurologic Study-Lability Scale (CNS-LS), a self-administered questionnaire, was used to evaluate the presence and severity of PBA. Total scores range from 7 to 35. A score ≥13 was used to identify the presence of PBA. One-hundred thirty-two sporadic incident ALS cases were enrolled. Median disease duration was 20 months (range 2-143), median onset-diagnosis interval (ODI) 12 months (range 2-131), median ALSFRSr at baseline 36/48 (range 2-47) and median ALSFRSr bulbar sub-score 10/12 (range 0-12). Neurological examination revealed presence of PBA in 34/132 patients (26%). Pathological CNS-LS score was found in 45/132 patients (34%). Median total CNS-LS score was 9/35 (range 7-29). The subgroup with pathological CNS-LS was characterized by a short disease duration from symptom onset, ODI, time to diffusion to a second region, time to generalization and ALSFRSr bulbar sub-score, bulbar onset, "definite" diagnostic category, bulbar upper motor-neuron involvement and presence of PBA at neurological examination. In population-based setting, one-third of ALS patients present PBA at diagnosis. The presence of PBA is associated with bulbar UMN involvement and markers of a more severe phenotype.
Topics: Adult; Aged; Aged, 80 and over; Amyotrophic Lateral Sclerosis; Cohort Studies; Female; Humans; Incidence; Italy; Male; Middle Aged; Prospective Studies; Pseudobulbar Palsy; Registries; Surveys and Questionnaires
PubMed: 26588919
DOI: 10.1007/s00415-015-7981-3 -
Geriatric Nursing (New York, N.Y.) 2018Pseudobulbar Affect (PBA) is a neurologic condition characterized by involuntary outbursts of crying and/or laughing disproportionate to patient mood or social context....
Pseudobulbar Affect (PBA) is a neurologic condition characterized by involuntary outbursts of crying and/or laughing disproportionate to patient mood or social context. Although an estimated 9% of nursing home residents have symptoms suggestive of PBA, they are not routinely screened. Our goal was to develop an electronic screening tool based upon characteristics common to nursing home residents with PBA identified through medical record data. Nursing home residents with PBA treated with dextromethorphan hydrobromide/quinidine sulfate (n = 140) were compared to age-, gender-, and dementia-diagnosis-matched controls without PBA or treatment (n = 140). Comparative categories included diagnoses, medication use and symptom documentation. Using a multivariable regression and best decision rule analysis, we found PBA in nursing home residents was associated with chart documentation of uncontrollable crying, presence of a neurologic disorder (e.g., Parkinson's disease), or by the documented presence of at least 2 of the following: stroke, severe cognitive impairment, and schizophrenia. Based on these risk factors, an electronic screening tool was created.
Topics: Aged; Aged, 80 and over; Cross-Sectional Studies; Dextromethorphan; Drug Combinations; Emotions; Female; Humans; Male; Middle Aged; Nursing Homes; Pseudobulbar Palsy; Quinidine; Surveys and Questionnaires
PubMed: 28807457
DOI: 10.1016/j.gerinurse.2017.06.002 -
Neurologic Clinics Aug 1984A distinct clinicopathologic entity, PSP is differentiated from Parkinson's disease by the presence of supranuclear ophthalmoparesis. Downward gaze palsy that can be... (Review)
Review
A distinct clinicopathologic entity, PSP is differentiated from Parkinson's disease by the presence of supranuclear ophthalmoparesis. Downward gaze palsy that can be overcome by oculocephalic maneuver is the most characteristic clinical finding. The other distinguishing clinical features of PSP include axial distribution of rigidity and broad-based gait with early postural instability. Furthermore, pseudobulbar palsy is an early feature, while profound dementia usually occurs late in the course of the disease. Because of the variable clinical presentation and occasional lag in the onset of ophthalmoparesis and other distinguishing signs, the diagnosis of PSP is often delayed for many years. However, the constellation of axial rigidity, pseudobulbar signs, and parkinsonism without tremor when combined with ophthalmoparesis should suggest the correct diagnosis. Pathologic examination of the PSP brain reveals neuronal cell loss, gliosis, granuolvacuolar degeneration, and unique neurofibrillary tangles in the pontomesencephalic tegmentum, tectum, basal ganglia, vestibular nuclei, periaqueductal gray matter, and dentate nuclei. The etiology of this neurodegenerative disorder is unknown and the neurodiagnostic studies usually are not helpful in proving the diagnosis. The treatment of PSP is unsatisfactory, but the anti-parkinson drugs, particularly dopamine agonists, may be useful in the early stages of the disease.
Topics: Aged; Brain; Brain Diseases; Diagnosis, Differential; Female; Humans; Male; Middle Aged; Ophthalmoplegia; Paralysis; Parkinson Disease; Syndrome
PubMed: 6398402
DOI: No ID Found -
Issues in Mental Health Nursing 2015
Topics: Affective Symptoms; Dextromethorphan; Drug Combinations; Excitatory Amino Acid Antagonists; Humans; Pseudobulbar Palsy; Quinidine
PubMed: 26735510
DOI: 10.3109/01612840.2015.1091869 -
Annals of Internal Medicine Jan 1997Myelinolysis is a neurologic disorder that can occur after rapid correction of hyponatremia. Initially named "central pontine myelinolysis," this disease is now known to... (Review)
Review
Myelinolysis is a neurologic disorder that can occur after rapid correction of hyponatremia. Initially named "central pontine myelinolysis," this disease is now known to also affect extrapontine brain areas. Manifestations of myelinolysis usually evolve several days after correction of hyponatremia. Typical features are disorders of upper motor neurons, spastic quadriparesis and pseudobulbar palsy, and mental disorders ranging from mild confusion to coma. Death may occur. The motor and localizing signs of myelinolysis differ from the generalized encephalopathy that is caused by untreated hyponatremia. Experiments have duplicated the clinical and pathologic features of myelinolysis by rapidly reversing hyponatremia in animals. Myelinolysis is more likely to occur after the treatment of chronic rather than acute hyponatremia and is more likely to occur with a rapid rate of correction. The exact pathogenesis of myelinolysis has not been determined. Optimal management of hyponatremic patients involves weighing the risk for illness and death from untreated hyponatremia against the risk for myelinolysis due to correction of hyponatremia. Experiments in animals and clinical experience suggest that correction of chronic hyponatremia should be kept at a rate less than 10 mmol/L in any 24-hour period.
Topics: Aged; Animals; Dogs; Female; Humans; Hyponatremia; Myelinolysis, Central Pontine; Saline Solution, Hypertonic; Sodium
PubMed: 8992924
DOI: 10.7326/0003-4819-126-1-199701010-00008 -
CNS Spectrums Dec 2016The symptoms of emotional dysregulation associated with the syndrome known as pseudobulbar affect (PBA) can be effectively treated by the sigma, glutamate, and...
The symptoms of emotional dysregulation associated with the syndrome known as pseudobulbar affect (PBA) can be effectively treated by the sigma, glutamate, and serotonergic agent dextromethorphan combined with quinidine. If the same brain circuits affected in PBA are also compromised in related disorders of emotional expression, dextromethorphan-quinidine and other novel sigma-glutamate-serotonin agents could prove to be novel psychopharmacologic treatments for these conditions as well.
Topics: Dextromethorphan; Drug Combinations; Emotions; Excitatory Amino Acid Antagonists; Humans; Models, Theoretical; Pseudobulbar Palsy; Psychopharmacology; Quinidine; Receptors, Glutamate; Receptors, Serotonin; Receptors, sigma
PubMed: 27855728
DOI: 10.1017/S1092852916000742 -
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 -
Advances in Therapy Jul 2011Pseudobulbar affect (PBA) is a neurologic syndrome of emotional affect disinhibition, characterized by uncontrollable, exaggerated, and often inappropriate emotional...
INTRODUCTION
Pseudobulbar affect (PBA) is a neurologic syndrome of emotional affect disinhibition, characterized by uncontrollable, exaggerated, and often inappropriate emotional outbursts, which may cause severe distress, embarrassment, and social dysfunction. However, the US prevalence of PBA remains unknown.
METHODS
An online survey was conducted primarily to estimate the US prevalence of PBA in patients with the six most commonly associated conditions: Alzheimer's disease, amyotrophic lateral sclerosis, multiple sclerosis, Parkinson's disease, stroke, and traumatic brain injury. Invitations to participate were randomly sent online to adults (aged ≥ 18 years) registered in the Harris Poll Online Panel who were patients or belonged to a household with a patient diagnosed with one of the six conditions (identified through previous screening by Harris Interactive). Participants were screened for PBA using the Pathological Laughing and Crying Scale (PLACS) and the Center for Neurologic Study-Lability Scale (CNS-LS). PBA estimates were made using a cut-off score of ≥ 13 on the PLACS and two different cut-off thresholds on the CNS-LS, a lower one of ≥ 13 and a more rigorous one of ≥ 21. Existing US prevalence data for the six underlying conditions were used to estimate US prevalence of PBA.
RESULTS
Of 38,000 individuals invited to participate, 8876 responded (23%) and 2318 (26%) completed the questionnaire. Mean prevalence of PBA across all six conditions was 10.1%, 9.4%, and 37.5% with the PLACS ≥ 13, CNS-LS ≥ 21, and CNS-LS ≥ 13 thresholds, respectively. Using disease population estimates from government agencies and professional organizations, the estimated US population with PBA ranged from 1.8 to 7.1 million. Among patients who discussed their laughing and/or crying episodes with a physician, 41% were diagnosed, and about half received a medication for their episodes.
CONCLUSIONS
The overall prevalence of PBA was estimated to be about 10% across these commonly associated underlying neurological conditions and appears to be under-recognized.
Topics: Adult; Alzheimer Disease; Amyotrophic Lateral Sclerosis; Brain Injuries; Female; Humans; Male; Middle Aged; Multiple Sclerosis; Nervous System Diseases; Parkinson Disease; Pseudobulbar Palsy; Stroke; United States; Young Adult
PubMed: 21660634
DOI: 10.1007/s12325-011-0031-3 -
Journal of Traditional Chinese Medicine... Mar 2004
Clinical Trial Randomized Controlled Trial
Topics: Acupuncture Therapy; Adult; Aged; Cerebral Infarction; Female; Humans; Intracranial Hemorrhages; Male; Middle Aged; Pseudobulbar Palsy; Scalp; Tongue
PubMed: 15119166
DOI: No ID Found