Effect of acupuncture treatment on dysphagia caused by pseudobulbar paralysis after stroke: a systematic review and meta-analysis.

BACKGROUND: The efficacy of acupuncture in the treatment of dysphagia caused by pseudobulbar paralysis after stroke is lack of evidence-based medicine. Our objective was to synthesize the efficacy of acupuncture in treating dysphagia caused by pseudobulbar paralysis after stroke. METHODS: A comprehensive literature search was performed in 9 databases [PubMed, Web of Science, Embase, Cochrane, Chinese BioMedical Literature Database (CBM), China National Knowledge Infrastructure (CNKI), WanFang Data, Chinese Science and Technology Periodicals database (VIP), and Open Grey online database] to screen eligible randomized controlled studies that evaluated the effect of acupuncture in dysphagia caused by pseudobulbar paralysis after stroke. The search time limit is from establishing the database to October 1, 2020. The random-effects model was used to calculate the significant effect size. RESULTS: A total of 7 studies comprising 637 participants were included in our meta-analysis. The results showed that compared with rehabilitation, acupuncture had a significant effect on improving dysphagia caused by pseudobulbar paralysis after stroke [the significant effective size: risk ratio (RR)sig =1.51; 95% confidence interval (CI): 1.30-1.75; I2=0%]. In the subgroup analyses, the RRsig of acupuncture + rehabilitation vs. rehabilitation was 1.56 (95% CI: 1.30-1.87; I2=0%), and the RRsig of acupuncture vs. rehabilitation was 1.38 (95% CI: 1.08-1.76; I2=0.8%). DISCUSSION: Acupuncture can be used as an effective treatment for dysphagia caused by pseudobulbar paralysis after stroke. Acupuncture combined with rehabilitation therapy has better effects.

Isolated acute pseudobulbar palsy with infarction of artery of percheron: case report and literature 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.

Thoracic Dural Arteriovenous Fistula Presenting with Isolated Pseudobulbar Palsy Mimicking Brainstem Lesion.

BACKGROUND: Spinal dural arteriovenous fistulas (DAVFs) are usually associated with neurologic dysfunction adjacent to the shunt point; however, the symptoms are uncommon far from the site of the fistula. To our knowledge, this is the first report of a patient with rapidly progressive isolated pseudobulbar palsy because of thoracic DAVF. CASE DESCRIPTION: We report a patient with thoracic DAVF presenting with remote symptoms of brainstem congestion. The patient was a 36-year-old man who presented with a sudden history of vomiting, dysphagia, and flaccid weakness in the 4 limbs. Intracranial magnetic resonance (MR) imaging at a local hospital demonstrated T2 signal hyperintensity within the medulla, and he was referred to our hospital for a suspected brainstem lesion. However, cervical MR imaging revealed a dilated and tortuous perimedullary venous plexus, and spinal angiography revealed DAVF in T5-6 with a feeding artery from the intercostal artery. After obliteration of the fistula, the progression of the disease was stopped and the symptoms improved. CONCLUSIONS: Although rare, thoracic DAVFs may present symptoms resembling brainstem infarction. Prompt surgical intervention is necessary for patients with thoracic DAVF presenting with rapidly progressive pseudobulbar palsy.

PRISM II: an open-label study to assess effectiveness of dextromethorphan/quinidine for pseudobulbar affect in patients with dementia, stroke or traumatic brain injury.

BACKGROUND: Phase 3 trials supporting dextromethorphan/quinidine (DM/Q) use as a treatment for pseudobulbar affect (PBA) were conducted in patients with amyotrophic lateral sclerosis (ALS) or multiple sclerosis (MS). The PRISM II study provides additional DM/Q experience with PBA secondary to dementia, stroke, or traumatic brain injury (TBI). METHODS: Participants in this open-label, multicenter, 90-day trial received DM/Q 20/10 mg twice daily. The primary outcome was the Center for Neurologic Study-Lability Scale (CNS-LS), assessing change in PBA episode frequency and severity. The CNS-LS final visit score was compared to baseline (primary analysis) and to the response in a previously conducted placebo-controlled trial with DM/Q in patients with ALS or MS. Secondary outcomes included change in PBA episode count and Clinical Global Impression of Change with respect to PBA as rated by a clinician (CGI-C) and by the patient or caregiver (PGI-C). RESULTS: The study enrolled 367 participants with PBA secondary to dementia, stroke, or TBI. Mean (standard deviation [SD]) CNS-LS score improved significantly from 20.4 (4.4) at baseline to 12.8 (5.0) at Day 90/Final Visit (change, -7.7 [6.1]; P < .001, 95 % CI: -8.4, -7.0). This magnitude of improvement was consistent with DM/Q improvement in the earlier phase-3, placebo-controlled trial (mean [95 % CI] change from baseline, -8.2 [-9.4, -7.0]) and numerically exceeds the improvement seen with placebo in that study (-5.7 [-6.8, -4.7]). Reduction in PBA episode count was 72.3 % at Day 90/Final Visit compared with baseline (P < .001). Scores on CGI-C and PGI-C showed that 76.6 and 72.4 % of participants, respectively, were "much" or "very much" improved with respect to PBA. The most frequently occurring adverse events (AEs) were diarrhea (5.4 %), headache (4.1 %), urinary tract infection (2.7 %), and dizziness (2.5 %); 9.8 % had AEs that led to discontinuation. Serious AEs were reported in 6.3 %; however, none were considered treatment related. CONCLUSIONS: DM/Q was shown to be an effective and well-tolerated treatment for PBA secondary to dementia, stroke, or TBI. The magnitude of PBA improvement was similar to that reported in patients with PBA secondary to ALS or MS, and the adverse event profile was consistent with the known safety profile of DM/Q. TRIAL REGISTRATION: Clinicaltrials.gov, NCT01799941, registered on 25 February 2013.

Clinical Features and Related Factors of Poststroke Pathological Laughing and Crying: A Case-Control Study.

OBJECTIVES: The purpose of this study was to analyze clinical features and related factors of poststroke pathological laughing and crying (PSPLC) and to differentiate PSPLC patients with and without pseudobulbar signs. METHODS: We performed a case-control study in which 56 patients with PSPLC were matched to 56 control stroke patients by age and gender. The pathological laughing and crying scale was used to identify patients with PSPLC. Characteristics of PSPLC outbursts, presence of pseudobulbar signs and autonomic symptoms, lesion locations, and different clinical data were analyzed. Mild cognitive impairment (MCI) was evaluated by the Montreal Cognitive Assessment. Poststroke anger proneness (PSAP) was evaluated by comparison of the patients' premorbid states. RESULTS: Significantly more patients in the PSPLC group showed MCI, PSAP, and pseudobulbar signs than those in the control group. Most patients with PSPLC showed bilateral multiple lesions and the pons (especially the bilateral paramedian basal and basal-tegmental areas) stood out as the most important lesion location. Logistic regression analysis showed that pontine lesion, MCI, and PSAP were independently related to PSPLC; however, the presence of pseudobulbar signs was not related. PSPLC patients with pseudobulbar signs showed more recurrent strokes in the previous 2 years, more severe neurological deficits, as well as higher severity of PSPLC. In addition, more patients in the group with pseudobulbar signs showed concomitant autonomic symptoms. CONCLUSIONS: PSPLC, MCI, and PSAP could be manifestations of a more general disorder, in which pontine lesion plays an important role. PSPLC patients with pseudobulbar signs and those without show different features.

Síndrome de Foix-Chavany-Marie como forma de presentación de esclerosis múltiple / Foix-Chavany-Marie syndrome presenting as multiple sclerosis

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Consecutive Lacunar Strokes Mimicking Brainstem Symptoms in a Patient With Pseudobulbar Palsy.

BACKGROUND: The pseudobulbar palsy affects both corticobulbar tracts leading to symptoms of the caudal brain nerves. These bulbar symptoms often may be misinterpreted and lead to a false localization of the ischemic lesion. Here we report on a patient with an acute small lacunar ischemic lesion who presented clinically with severe affection because of an identical old contralateral lesion. CASE REPORT: The patient presented with sudden onset of dysphagia, anarthria, and glossplegia. The clinical examination was suspicious of a brainstem lesion, however, stroke magnetic resonance imaging revealed a small ischemic lacuna within the right internal capsule. However, because of an old ischemic lesion within the left internal capsule, both corticobulbar tracts were involved and this pseudobulbar palsy was mimicking bulbar brainstem symptoms. DISCUSSION: Patients who suffer from a pseudobulbar palsy usually have­compared with the rather small lesions within the central nervous system­clinically dramatic presentations and due to bulbar symptoms severe medical secondary complications and social-economic consequences to deal with.

Crying and suicidal, but not depressed. Pseudobulbar affect in multiple sclerosis successfully treated with valproic acid: Case report and literature review.

OBJECTIVE: Pseudobulbar affect/emotional incontinence is a potentially disabling condition characterized by expressions of affect or emotions out of context from the normal emotional basis for those expressions. This condition can result in diagnostic confusion and unrelieved suffering when clinicians interpret the emotional expressions at face value. In addition, the nomenclature, etiology, and treatment for this condition remain unclear in the medical literature. METHOD: We report the case of a 60-year-old woman with multiple sclerosis who was referred to an inpatient psychiatry unit with complaints of worsening depression along with hopelessness, characterized by unrelenting crying. Our investigation showed that her symptoms were caused by pseudobulbar affect/emotional incontinence stemming from multiple sclerosis. RESULTS: The patient's history of multiple sclerosis and the fact that she identified herself as depressed only because of her incessant crying suggested that her symptoms might be due to the multiple sclerosis rather than to a depressive disorder. Magnetic resonance imaging demonstrated a new plaque consistent with multiple sclerosis lateral to her corpus callosum. Her symptoms resolved completely within three days on valproic acid but returned after she was cross-tapered to dextromethorphan plus quinidine, which is the FDA-approved treatment for this condition. SIGNIFICANCE OF RESULTS: This case provides important additional information to the current literature on pseudobulbar affect/emotional incontinence. The existing literature suggests a selective serotonin reuptake inhibitor (SSRI) and dextromethorphan/quinidine (Nuedexta) as first-line treatments; however, our patient was taking an SSRI at the time of presentation without appreciable benefit, and her symptoms responded to valproic acid but not to the dextromethorphan/quinidine. In addition, the case and the literature review suggest that the current nomenclature for this constellation of symptoms can be misleading.

Bilateral perisylvian ulegyria: an under-recognized, surgically remediable epileptic syndrome.

PURPOSE: Interest in the association of epilepsy and pseudobulbar palsy was rekindled since the identification through magnetic resonance imaging (MRI) of bilateral perisylvian polymicrogyria (PMG). Seizures are often intractable, but resective epilepsy surgery has not been recommended. However, a similar clinical picture can be encountered in patients with bilateral perisylvian destructive lesions, which fit the description of ulegyria (ULG). We report a series of patients with epilepsy and pseudobulbar palsy due to bilateral perisylvian ULG (BP-ULG), show that hippocampal sclerosis (HS) is often associated and highlight the fact that in this entity, unlike in malformative bilateral perisylvian PMG, seizures may be surgically treated. METHODS: The motor, cognitive, epileptologic, and imaging features of 12 patients with perisylvian ULG followed at three institutions are described. For patients with refractory seizures, we detail extracranial and intracranial electrographic recordings, surgical strategies, histopathologic analyses of the resected tissue, and outcome of surgical treatment. Descriptive statistics were used for quantitative and categorical variables. Student's t-test was used to compare means, and a p < 0.05 was considered significant. KEY FINDINGS: Pseudobulbar palsy and mental retardation were present in all patients with symmetrical BP-ULG. Five had refractory seizures. There was no relationship between the severity of the pseudobulbar palsy or of the mental retardation and the degree of seizure control with medication. The five patients in whom seizures were refractory to medication had significantly earlier age of onset and longer duration of epilepsy (p < 0.05). Dual pathology with associated unilateral HS was present in four. One patient with dual pathology had a temporolimbic electroclinical picture and had an anterior temporal lobectomy (ATL) based upon noninvasive evaluation. The other four had ictal semiology suggesting involvement of both temporolimbic and perisylvian cortex. Intracranial electroencephalography (EEG) showed concomitant seizure onset in the anterior temporal region and in the ipsilateral ULG in three of the four with dual pathology and in the ulegyric cortex in the one without HS. Resection guided by a combination of semiology, MRI, and extra and intracranial EEG led to complete seizure control in two and almost complete seizure control (Engel class II) in two other patients. The only surgical failure was an isolated ATL in a patient with dual pathology, and concomitant seizure onset in both lesions according to semiology and intracranial EEG. SIGNIFICANCE: Our findings suggest that BP-ULG mimics the clinical features of bilateral perisylvian PMG. In patients with refractory seizures, recognition of this entity should lead to consideration of resective surgery despite the bilateral ULG.

Dental management of the tropical disease human African trypanosomiasis: an unusual case of pseudobulbar palsy.

Human African trypanosomiasis (sleeping sickness) is a parasitic tropical disease endemic to sub-Saharan Africa. Due to migration and holiday travel patterns cases are increasing in the United Kingdom. The neurological sequelae have dental management implications both directly from the consequent physical disability and indirectly from the oral side-effects of the medications used to manage symptoms. Changes in disease demographics require the dental profession to increase its awareness of migration medicine and the appropriate dental management of such diseases.

Mielinolisis central pontina en el contexto de un trasplante hepático reducido / No disponible

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Pseudobulbar syndrome in two patients with human immunodeficiency virus infection.

Different forms of motor neuron disease occurring in association with HIV infection have been described. We present two patients with pseudobulbar syndrome and HIV infection, with no clinical or electromyographic signs of lower motor neuron loss. In patient 1, on follow-up, focal seizures led to additional investigations that identified unsuspected HIV infection and progressive multifocal leucoencephalopathy (PML). In patient 2, all investigations excluded an active HIV infection or central nervous system involvement, and the disease progression made primary lateral sclerosis (PLS) with pseudobulbar onset the most likely diagnosis. ALS-like syndrome can occur in association with HIV infection; however, the causal relationship remains uncertain. Patient 1 shows that PML is a possible cause for pseudobulbar syndrome, and our second patient demonstrates that ALS may also occur by chance in patients with HIV infection.

[Spasmodic laughter syncope. An unusual complication of pseudobulbar palsy]. / Syncope du rire spasmodique. Une complication inhabituelle du syndrome pseudobulbaire.

INTRODUCTION: Spasmodic laughter is a classical sign of pseudobulbar palsy, but it has never been reported, to our knowledge, to provoke syncope. CASE REPORT: A 63-year-old hypertensive and diabetic man with peripheral neuropathy and lacunar pseudobulbar palsy presented with three episodes of spasmodic laughter which had induced syncope. No new episode was observed after the beginning of low dose bisoprolol. DISCUSSION: Sustained or spasmodic laughter is accompanied by repetitive bursts of forced expiration, corresponding to short repetitive Valsalva maneuvers. Laughter-induced syncope is considered as one of the many Valsalva-type/vagally mediated syncopal attacks leading to rapid fall in blood pressure without compensatory tachycardia. The presence of autonomic diabetic neuropathy may also contribute to these attacks.

Brain responses to verbal stimuli among multiple sclerosis patients with pseudobulbar affect.

PURPOSE: To characterize the brain activity and associated cortical structures involved in pseudobulbar affect (PBA), a condition characterized by uncontrollable episodes of emotional lability in patients with multiple sclerosis (MS). METHODS: Behavioral responses and event related potentials (ERP) in response to subjectively significant and neutral verbal stimuli were recorded from 33 subjects in 3 groups: 1) MS patients with PBA (MS+PBA); 2) MS patients without PBA (MS); 3) Healthy control subjects (HC). Statistical non-parametric mapping comparisons of ERP source current density distributions between groups were conducted separately for subjectively significant and for neutral stimuli. RESULTS: Behavioral responses showed more impulsive performance in patients with PBA. As expected, almost all ERP waveform comparisons between the MS groups and controls were significant. Source analysis indicated significantly distinct activation in MS+PBA in the vicinity of the somatosensory and motor areas in response to neutral stimuli, and at pre-motor and supplementary motor areas in response to subjectively significant stimuli. Both subjectively significant and neutral stimuli evoked higher current density in MS+PBA compared to both other groups. CONCLUSIONS: PBA of MS patients involves cortical structures related to sensory-motor and emotional processing, in addition to overactive involvement of motor cortical areas in response to neutral stimuli. SIGNIFICANCE: These results may suggest that a 'disinhibition' of a "gate control"-type mechanism for emotional expression may lead to the lower emotional expression threshold of pseudobulbar affect.

[The auditory late potentials (P300, MMN) in central speech disorders in children]. / Pózne potencjaly wywolane (P300, MMN) w osrodkowych zaburzeniach mowy u dzieci.

The aim of this paper was introduction of preliminary study of late auditory evoked potentials P300 and MMN insome central communication process disorders. The investigations were performed in the group of three children with development dysphasia and pseudobulbar dyzarthria. The registration of endogenic late cognitive potentials was performed using the alternate application of stimulus of pure tones 1 and 2 kHz, and logatoms (da)-(ga). In all patients the normal hearing threshold and the unilateral disturbances in registration of P300 and mismatch negativity (MMN) were found.

Transplante hepático en una paciente con daño neurológico ¿un recurso terapéutico excepcional?: algunas reflexiones bioéticas / Liver transplantation in a patient with neurological damage: a one-off treatment?: some bioethical considerations

Se presenta el dilema ético-clínico planteado por el caso de una paciente de 8 años 6 meses de edad, portadora de enfermedad hepática con daño neurológico irreversible y severo en la cual los padres solicitan la posibilidad de realizar un transplante hepático. Se trata de la única hija de padres sanos, preocupados y responsables del manejo de su hija, con un riesgo de recurrencia de esta enfermedad de 25 porciento para otro hijo. Se discuten las posibilidades de hacer todo lo terapéuticamente posible o si se debe tomar la decisión de limitar los tratamientos. Con el objetivo de mostrar un proceso de deliberación bioética y de permitir al lector confrontar sus apreciaciones personales, se presenta el caso clínico, seguido de dos comentarios independientes

[Bilateral hand dystonia secondary to a bilateral opercular syndrome or Foix-Chavany-Marie syndrome]. / Mano distónica bilateral secundaria a síndrome biopercular o síndrome de Foix-Chavany-Marie.

INTRODUCTION: The main clinical feature of the opercular syndrome (Foix Chavany Marie) is the automatic voluntary dissociation of the facio glosso pharyngeal movements (that is, the alteration of voluntary motility with preservation of authomatic movements). Less frequently, it is presented with movement disorders as dystonia. CASE REPORT: We report a male patient aged 40 years who developed a biopercular syndrome of vascular etiology (confirmed by neuroimaging), in the context or a clinical picture of global hipoxemia, for which the most outstanding clinical manifestation was the presence of dystonic posturing. CONCLUSION: Although dystonia is usually related with damage or dysfunction of the basal ganglia or thalamus, in some case it can be caused by lesions in other locations, such as in some patients with biopercular syndrome as in the present case