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.

[Foix-Chavany-Marie Syndrome: A Clinical Overview].

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.

Identification of pseudobulbar affect symptoms in the nursing home setting: Development and assessment of a screening tool.

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.

Pseudobulbar Affect: What Nurses, Stroke Survivors, and Caregivers Need to Know.

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.

Introduction to pseudobulbar affect: setting the stage for recognition and familiarity with this challenging disorder.

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.

Pharmacotherapeutic management of pseudobulbar affect.

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.

Recognizing and treating pseudobulbar affect.

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.

[TRANSIENT PSEUDOBULBAR SYNDROME IN UNILATERAL FRONTAL OPERCULAR INFARCTS]. / ÁTMENETI PSEUDOBULBARIS TÜNETCSOPORT EGYOLDALI FRONTÁLIS OPERCULARIS INFARKTUSOKNÁL.

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.

Effective use of sertraline for pathological laughing after severe vasospasm due to aneurysmal subarachnoid hemorrhage: case report.

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.

Behind the screen: pseudobulbar symptoms after deep brain stimulation.

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.

Pseudobulbar dysarthria in the initial stage of motor neuron disease with dementia: a clinicopathological report of two autopsied cases.

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.

Pseudobulbar affect: an under-recognized and under-treated neurological disorder.

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.

[Slowly progressive dysarthria in primary lateral sclerosis]. / Langsam progressive Dysarthrophonie bei primärer Lateralsklerose.

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.

[Amyotrophic lateral sclerosis: management of bulbar symptoms]. / Amyotrophe Lateralsklerose: Symptomatische Therapie bulbärer Symptome.

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.

Congenital bilateral perisylvian syndrome: a case study.

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: a case report].

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.