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Foix-Chavany-Marie syndrome, which has been reported since the 19th century, is a cortical type of pseudobulbar palsy. Although previously most cases were due to acute recurrent stroke, recent reports have indicated that there are various causes, including infectious, developmental, epileptic and degenerative processes and various clinical courses, which may be transient or slowly progressive. Moreover, modern imaging techniques have revealed the significance of the injury of the white matter tract, such as the frontal aslant tract, rather than classical lesions of bilateral motor cortices. Considering both the findings of the bedside examination of patients with this "old" syndrome and of "new" imaging studies performed at appropriate times is expected to contribute to our understanding of the neural underpinnings of this syndrome.

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., (Copyright © 2017 Elsevier Inc. All rights reserved.)

This activity will update pharmacists and other healthcare professionals on current treatments for pseudobulbar affect (PBA). Points of discussion will focus on the off-label therapies traditionally used to treat PBA, the FDA-approved combination drug product with PBA as an indication, and managed care aspects of treating PBA.

Pseudobulbar affect (PBA), despite its prevalence and distinctive symptoms, is widely underrecognized and undertreated. It is characterized by uncontrollable laughing or crying that can occur in an exaggerated manner or inappropriately to a given situation or stimuli. PBA is thought to center around preexisting neurological conditions, which include Parkinson disease, multiple sclerosis, amyotrophic lateral sclerosis, Alzheimer disease, traumatic brain injury, and stroke. The PBA Registry Series trial was created to measure the prevalence of PBA among patients with these underlying neurological conditions. Through greater awareness, recognition, and diagnosis, treatment for patients with PBA can be improved.

Pseudobulbar affect (PBA) is a neurologic condition that can happen after a patient has had some kind of neurological insult. In this syndrome, involuntary, uncontrollable, and inappropriate emotional outbursts unrelated or out of proportion to the situation are common symptoms. This can be very frustrating and scary. Because stroke survivors and their caregivers are overloaded with information during the transition from hospital to home, information about PBA is not usually discussed. In a survey by the National Stroke Association, 53% of stroke survivors reported that they had some of the symptoms of this disorder. Even if they discussed these symptoms with their health care providers, less than half were given a diagnosis, and less than a quarter received any kind of treatment. The purpose of this article is to give nurses more information about PBA so they can share this with patients and families/significant others and encourage them to seek help if they experience these symptoms once discharged. It is hoped that increased knowledge about this condition will lead to better diagnosis and treatment and increased quality of life for stroke survivors.

Pseudobulbar affect, thought by many to be a relatively newly described condition, is in fact a very old one, described as early as the 19th century. It refers to those who experience inappropriate affect, disconnected from internal state, or mood, generally thought to be the result of an upper motor neuron injury or illness. One possible explanation for this condition's relative obscurity is the dearth of treatment options; clinical medicine is not typically in the habit of identifying conditions that cannot be modified. Now, however, there is good evidence for the treatment of pseudobulbar affect, and even a therapy approved for use by the U.S. Food and Drug Administration (FDA). As a result, appropriate identification and subsequent management of pseudobulbar affect is more important than ever. This article purports to summarize the origins of pseudobulbar affect, most current hypotheses as to its physiopathology, clinical identification, and evidence for management.

The classic anterior (frontal) opercular syndrome (Foix-Chavany-Marie sy.) is a cortical pseudobulbar palsy mainly due to bilateral lesions of anterior brain operculum. In 2000 the authors had a 70-year old female patient with acute onset of swallowing and speaking difficulty. Neurological examination established a left facial central palsy, the palsy of the tongue and the soft palate, dysarthry, difficulty in chewing with left side hemiparesis. The CT scan showed a right side (one-sided) frontal opercular ischemic lesion. This event switched their attention especially to this group of cases and subsequently the authors collected 12 patients with these symptoms. Authors discuss the patomechanism of transient pseudobulbar palsy that occurs due to unilateral opercular lesion that the diaschisis effect might explain.

Pathological laughing, one subgroup of psuedobulbar affect, is known as laughter inappropriate to the patient's external circumstances and unrelated to the patient's internal emotional state. The authors present the case of a 76-year-old woman with no significant medical history who experienced pathological laughing after subarachnoid hemorrhage (SAH) due to rupture of an aneurysm, which was successfully treated with craniotomy for aneurysm clipping. In the acute stage after the operation she suffered from severe vasospasm and resulting middle cerebral artery territory infarction and conscious disturbance. As she regained consciousness she was afflicted by pathological laughing 6 months after the onset of SAH. Her involuntary laughter was inappropriate to the situation and was incongruent with the emotional state, and she could not control by herself. Finally the diagnosis of pathological laughing was made and treatment with sertraline, a selective serotonin reuptake inhibitor (SSRI), effectively cured the symptoms. Her pathological laughing was estimated to be consequence of infarction in the right prefrontal cortex and/or corona radiata, resulting from vasospasm. To the authors' knowledge, this is the first report of pathological laughing after aneurysmal SAH. The authors offer insight into the pathophysiology of this rare phenomenon. Effectiveness of sertraline would widen the treatment modality against pathological laughing.

Background: Thalamotomy was formerly used to treat different tremor syndromes. Nowadays, deep brain stimulation has become an established technique to treat -different movement disorders. The combination of these two stereotactic interventions is rare., Clinical Presentation: We present a patient in which a right-sided tremor -syndrome with an underlying pathology of combined essential tremor and Parkinsonian tremor was successfully treated initially with a left-sided thalamotomy and subsequently with -bilateral deep brain stimulation in the subthalamic nucleus., Results: Deep brain stimulation in the subthalamic nucleus resulted in hemidystonia, pathological laughing and crying, dysarthria and dysphagia, all due to dislocation of the stimulation electrodes contacting the internal capsule. After discontinuation of the high-frequency stimulation these side-effects disappeared, but were then reactivated by an LCD television in stand-by mode., Conclusion: In this report we discuss the pathophysiology of pseudobulbar symptoms and pathological laughing and crying in context of thalamotomy and dislocated DBS electrodes. Furthermore, we report on the occurrence that magnetic fields in the household have an impact on deep brain stimulation, even if they are in stand-by mode.

We retrospectively analyzed the clinical features of two cases of neurodegenerative disease, whose initial symptoms were motor speech disorder and dementia, brought to autopsy. We compared the distributions of pathological findings with the clinical features. The main symptom of speech disorder was dysarthria, involving low pitch, slow rate, hypernasality and hoarseness. Other than these findings, effortful speech, sound prolongation and initial difficulty were observed. Moreover, repetition of multisyllables was severely impaired compared to monosyllables. Repetition and comprehension of words and sentences were not impaired. Neither atrophy nor fasciculation of the tongue was observed. Both cases showed rapid progression to mutism within a few years. Neuropathologically, frontal lobe degeneration including the precentral gyrus was observed. The bilateral pyramidal tracts also showed severe degeneration. However, the nucleus of the hypoglossal nerve showed only mild degeneration. These findings suggest upper motor neuron dominant motor neuron disease with dementia. We believe the results indicate a subgroup of motor neuron disease with dementia whose initial symptoms involve pseudobulbar palsy and dementia, and which shows rapid progression to mutism., (Copyright © 2013 S. Karger AG, Basel.)

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.

Symptomatic treatment of amyotrophic lateral sclerosis (ALS) is relevant in preventing complications and improving quality of life as long as curative therapies are still out of sight. About one third of ALS patients show disabling problems associated with dysarthria, dysphagia, sialorrhea, and a pseudobulbar affective disorder already in the early stages of ALS. A multidisciplinary approach is the cornerstone of symptomatic treatment of bulbar and pseudobulbar ALS features. Except for riluzole randomized controlled trials are lacking. Here, we review the current views with regard to epidemiology, pathophysiology, diagnosis, and practical aspects of treating bulbar and pseudobulbar symptoms.

Slowly progressive dysarthria over many years may be the only sign of primary lateral sclerosis (PLS). Clinically it presents as pseudobulbar palsy which can be differentiated from amyotrophic lateral sclerosis (ALS) by the longer disease duration (> or =4 years), central pathological magnetic-evoked potentials to the tongue and lack of denervation in EMG. In contrast, hereditary spastic paraplegia (HSP) is characterized by a primary spasticity of the lower limbs, mostly later onset, the fact that other family members are affected and in isolated cases by positive genetic testing for mutations.

We report the case of a 14-year-old boy with bilateral congenital perisylvian syndrome as evidenced by polymicrogyria in the left and right frontotemporal regions, pseudobulbar paresis, seizures, and spastic diplegia. This is the first description of comprehensive neuropsychological assessment in a child with this syndrome. The results reflect significant impairments in auditory processing and independent learning and retention, but relatively preserved cognitive performance on interactive tasks involving frequent feedback and redirection. The findings are interpreted in the context of the neuropathology of this syndrome, with an emphasis on pragmatic implications for rehabilitation.

Congenital bilateral perisylvian syndrome (CBPS) is rare in literature, especially in China. In this article, we report the clinical and treatment of a patient with CBPS and discuss its mechanism, clinical features and therapy. This patient was a 28-year-old man. His main clinical features were pseudobulbar palsy, cognitive deficits and intractable epilepsy. MRI shows bilateral thickening of the cortex around the sylvian fissures which were deeper than normal and polymicrogyria. The electroencephalogram demonstrated slow spike in right temporal lobe and left frontal lobe. Rhythmal 4 Hz theta waves exist in left frontal and parietal lobe. As the epilepsy was poorly controlled by antiepileptic, section of the corpus callosum was carried out. After callosotomy, there was pronounced seizure reduction and intelligence development improvement. CBPS is characterized by pseudobulbar palsy, cognitive deficits, and bilateral perisylvian abnormalities in imaging studies. If intractable epilepsy is combined, callosotomy may be effective.

A new agent containing a combination of dextromethorphan (DM) and quinidine (Q) is currently under development for the treatment of pseudobulbar affect (PBA). PBA is a disorder of emotional regulation, characterized by uncontrollable outbursts of laughing and/or crying that are disproportionate to the emotions being experienced. The pathophysiology of PBA is currently unknown, although the disorder is thought to occur exclusively in the setting of neurological disease. The most influential theory on PBA posits that emotional outbursts are being generated autonomously in the brain stem due to loss of regulatory control by the frontal lobe. Although rarely life-threatening, PBA can have significant impact on patient quality of life, and thus merits treatment. There are currently no approved treatments for PBA. Several agents have been found to be effective in small placebo-controlled trials and case series, with the most commonly used agents being tricyclic antidepressants and selective serotonin reuptake inhibitors. Both these treatments are inexpensive and relatively low-risk, although the quality and quantity of data available on their efficacy are not optimal. DM has several pharmacological mechanisms of action relevant to the brain. It is an N-methyl-D-aspartate (NMDA) receptor antagonist, which prompted investigators to study its potential for slowing progression in amyotrophic lateral sclerosis (ALS), where glutamate toxicity is thought to be a factor. The combination agent DM/Q was developed to slow the metabolism of DM by P450 2D6 enzymes in the liver. DM/Q was not effective in slowing ALS progression, but patients noted that it helped to control their emotional outbursts, suggesting it might be useful as a treatment for PBA. DM is also a sigma-1 receptor agonist. These receptors are widely distributed in the brain, but probably most heavily in the limbic system, suggesting that DM may exert its emotion-controlling effects via these receptors. The endogenous ligands for sigma-1 receptors are not altogether known, although they appear to include gonadal steroids. DM/Q was recently shown to be effective in reducing the severity of PBA in two large studies of ALS and multiple sclerosis, which are probably the most common neurological settings. These are the largest treatment studies of PBA ever done. The agent was safe and relatively well tolerated. Further studies are being conducted to see if efficacy can be maintained with lower doses of quinidine. If DM/Q is approved by the U.S. Food and Drug Administration for treatment of PBA, it would be the first agent approved for this purpose. Currently, the antidepressants are probably the most attractive pharmacologic options for treatment of PBA. The choice of whether to use DM/Q in this setting will likely depend on individual patient factors as well as cost., (Copyright 2008 Prous Science, S.A.U. or its licensors. All rights reserved.)

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.

This monograph summarizes the proceedings of a roundtable meeting convened to discuss pseudobulbar affect (PBA). Two didactic lectures were presented followed by a moderated discussion among 11 participants. Post-meeting manuscript development synthesized didactic- and discussion-based content ad incorporated additional material from the neuroscience literature. A conceptual framework with which to distinguish between disorders of mood and affect is presented first, and disorders of affect regulation are then reviewed briefly. A detailed description of the most common of these disorders, PBA, is the focus of the remainder of the monograph. The prevalence, putative neuranatomic and neurochemical bases of PBA are reviewed, and current and emerging methods of evaluation and treatment of persons with PBA are discussed. The material presented in this monograph will help clinicians better recognize, diagnose, and treat PBA, and will form a foundation for understanding and interpreting future studies of this condition.

Demyelination may be a pathogenic mechanism of post-malarial neurological sequelae. It can cause pseudobulbar palsy, which has not been documented earlier. In the present communication we report two cases of pseudobulbar palsy after cerebral malaria with evidence of demyelination.

A case of left atrial (LA) myxoma presenting as pseudobulbar palsy, due to multiple cerebral infarcts, without any cardiac manifestations, is presented. LA myxoma is rare cause of embolization to CNS causing ischemic infarcts. Due to multiple CNS infarcts patient can present with varied clinical picture and pseudobulbar palsy is not a very common presentation. It was a real diagnostic dilemma before LA myxoma was diagnosed on echocardiography.

Anarthria and bilateral central faciolinguopharyngeomasticatory paralysis with automatic voluntary dissociation are the clinical hallmarks of Foix-Chavany-Marie syndrome (FCMS), the cortico-subcortial type of suprabulbar palsy. We present a 64-year-old man who was admitted due to acute onset of speechlessness for days. Neurological examinations demonstrated typical features of FCMS. Cranial MRI showed a right opercular lesion and several lacunar infarcts in bilateral hemispheres. Hexamethylpropylenamine oxime (HMPAO) brain SPECT revealed hypoperfusion in bilateral opercular regions and notably on the right side. In review of previous reports, the most common etiology of FCMS is stroke in the region of either operculum. Our case may be a good example of FCMS after multiple cerebrovascular insults over bilateral opercular cortico-subcortical areas as demonstrated in MRI and SPECT studies.

Purpose: The main features of congenital bilateral perisylvian syndrome (CBPS) are pseudobulbar palsy, cognitive deficits, epilepsy, and perisylvian abnormalities on imaging studies, however, the clinical spectrum of this syndrome is much wider than previously believed and may vary from minor speech difficulties to severely disabled patients. The objective of this study was to present the different imaging and clinical findings of 17 patients with CBPS, their genetic background, and the occurrence of prenatal injury during their pregnancies., Methods: We evaluated 17 consecutive patients with CBPS and divided them into two groups according to the imaging findings: (a) diffuse polymicrogyria around the sylvian fissure and (b) posterior polymicrogyria at the posterior parietal regions. They were systematically interviewed regarding history of prenatal events during their pregnancies, family history of speech difficulties, epilepsy, or other neurologic abnormality., Results: There were seven women, ages ranging from 3 to 41 years (mean, 11.5; median, 7 years). Seven patients had bilateral posterior parietal polymicrogyria (BPPP), and 10 had diffuse bilateral perisylvian polymicrogyria. All seven patients with BPPP had only minor speech difficulties, none had epilepsy, and all but one had a family history of epilepsy or cortical dysgenesis. In contrast, 10 patients with diffuse bilateral perisylvian polymicrogyria had pseudobulbar palsy, four had epilepsy, eight had a history of a major prenatal event, and only four had a family history of epilepsy or developmental delay., Conclusions: These findings suggest that diffuse bilateral perisylvian polymicrogyria appears to be more related to injuries caused by environmental factors, whereas BPPP has a stronger genetic predisposition. In addition, BPPP appears to have a wider clinical spectrum than previously believed, and may represent a milder extreme within the spectrum of CBPS.

We report a case of cerebral deep venous thrombosis that manifested clinically by a pseudobulbar syndrome with major trismus, abnormal movements and static cerebellar syndrome. To our knowledge, only three other cases of deep cerebral venous thrombosis associated with cerebellar or pseudobulbar syndrome have been published since 1985. The relatively good prognosis in our patient could be explained by the partially intact internal cerebral veins as well as use of early anticoagulant therapy. There was a spontaneous hyperdensity of the falx cerebri and the tentorium cerebelli on the brain CT scan, an aspect highly contributive to diagnosis. This hyperdensity of the falx cerebri was found in 19 out of 22 cases of deep venous thrombosis detailed in the literature.

Introduction: Acute pseudobulbar palsy produced by bilateral cerebral infarctions is a rare syndrome, which includes among its symptoms mutism, severe dysphagia and diverse sensory-motor signs., Clinical Cases: We report two middle-aged patients who suddenly developed a severe dysarthria and dysphagia, which impeded their ability to speak and to feed themselves, with spasmodic laughing and crying and slight motor deficit ('pure' pseudobulbar palsy). The acute lesions, using conventional and diffusion-weighted magnetic resonance imaging, corresponded partially to the anterior choroidal artery (case 1: acute lesion in the left periventricular white matter and a subacute one in the right semioval centrum; case 2: acute lesions in the right frontal subcortical white matter and in the periventricular white matter adjacent to the left lateral ventricle). The favorable evolution of these patients in contrast to previously described patients with acute pseudobulbar palsy could indicate that the motor deficit is a prognostic factor for this syndrome., Conclusions: Diffusion-Weighted magnetic resonance imaging permits differentiation with high precision of the acute lesions in patients who present old ones. Sometimes multiple acute lacunar infarctions (MALI) are found to be responsible of the syndrome. Hypertension and diabetes are the risk factors for the small vessel disease underlying these MALI.

Dysarthria is a leading disability in ALS patients with motor neurone degeneration in the bulbar region. Although different approaches have been tried in the past, currently, no test is available to detect and follow the progression of dysarthria. We studied 53 patients with definite (n=27) or probable (n=26) ALS (the bulbar onset group n=15, the limb onset group n=38, mean age 53. 66/29-76 years/) according to El Escorial criteria. Each patient was seen by a neurologist every 10-12 weeks and clinical performance was assessed using the Norris scale. To evaluate dysarthria we developed a computer-based acoustic method. All patients had computer-analysed speech sound tests done three times. The most significantly affected vowels were selected for further studies. A method based on the Euclidian principle was used and the results were compared with 30 age, sex-matched, healthy control subjects. Our results demonstrated the existence of a specific dysarthria profile in ALS patients with most significantly affected vowels: 'B', 'O', 'I', 'W', 'T' in the bulbar group, and: 'B', 'I', 'T', 'W', 'O' in the limb group. This study suggests that it is possible to detect and monitor the progression of the disease based on the acoustic analysis of only several sounds. Abnormalities detected in the dysarthria profile may appear prior to any clinical symptoms of the disease.

Bulbar and pseudobulbar symptoms are diagnostic criteria of amyotrophic lateral sclerosis (ALS). One of the earliest symptoms of bulbar involvement is voice deterioration. Until now voice assessment in ALS patients has been done mainly by perceptual analysis. The objective parameters, including acoustic measures, one aerodynamic measure and the maximal phonation time, have been measured only in a few small series of patients. The first purpose of this prospective study was to determine which vocal parameters discriminate ALS patients with bulbar involvement from control patients. The second was to identify sensitive parameters for early detection of voice deterioration due to bulbar involvement in pre-symptomatic ALS patients. The voices of 63 female ALS patients, including 40 with bulbar symptoms (sALS patients) and 23 without bulbar symptoms (aALS patients), were studied using an objective voice analysis system that allows simultaneous analysis of acoustic and aerodynamic parameters. Measurements were compared with those obtained in 40 normal female subjects (control patients). Five of eight acoustic parameters were significantly different among the three groups: jitter, coefficient of variation for frequency, shimmer, number of harmonics, and maximum phonatory frequency range. Three aerodynamic parameters, phonatory airflow, cycle-to-cycle variation for phonatory airflow, and coefficient of variation for phonatory airflow were significantly different between sALS patients and control patients. No aerodynamic parameter allowed discrimination between aALS patients and control patients. This study shows that acoustic parameters are more sensitive than aerodynamic parameters for early detection of bulbar involvement. Nevertheless, the measurements used can predict bulbar involvement in 73% of those in the sALS group, but only in 52% of those in the aALS group.

Four patients with pseudobulbar palsy, mental retardation and various degrees of speech disturbance associated with perinatal difficulties are described as having an acquired type of opercular syndrome. There were two patients with fetal bradycardia and three with subarachnoid haemorrhage and neonatal convulsion. Magnetic resonance imaging revealed cortical atrophy in the bilateral opercula with some signal abnormalities in the underlying white matter in common. Single photon emission computed tomography (SPECT) also confirmed the presence of hypoperfusion in the regions. Although the opercular syndrome is a clinical entity with a multitude of underlying pathologies, perinatal difficulties could be an important cause of the acquired type.