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Child's Nervous System : ChNS :... Jun 2018Hashimoto's encephalopathy (HE) is an autoimmune condition with varied neurological and psychiatric features. HE is very unusual as a cause of pseudobulbar palsy (PSP).
INTRODUCTION
Hashimoto's encephalopathy (HE) is an autoimmune condition with varied neurological and psychiatric features. HE is very unusual as a cause of pseudobulbar palsy (PSP).
CASE PRESENTATION
A 14-year-old male was admitted with right-sided weakness, dysphagia, speech disorder, and aggressiveness. Brain magnetic resonance imaging showed increased intensity in bilateral temporal, insular cortex, amygdala, and parahippocampal area on T2-weighted and fluid-attenuated inversion recovery images. Autoimmune encephalitis was considered as the patient had subacute onset of psychiatric and motor disturbances with normal findings for cerebrospinal fluid. N-methyl-D-aspartate receptor, anti-glutamate-type α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid 1 and 2, anti-contactin-associated protein-like 2, anti-gamma-aminobutyric acid receptor, anti-Leucine-rich, and glioma-inactivated 1 antibodies were negative but the anti-thyroperoxidase (antiTPO) level was greater than 998 IU/ML (n:0-9). Steroid therapy was initiated as pulse therapy and maintained with 2-mg/kg/day dose with the diagnosis of HE. He was symptom free for 6 months. In the follow-up period, he had two recurrences which responded to steroid therapy.
CONCLUSION
The common causes of PSP are demyelinating, vascular, and motor neuron diseases and congenital malformations of the opercular or insular cortex. However, there are no cases of PSP developing after any autoimmune encephalitis. This case highlights the importance of early detection of antiTPO antibodies with the findings of PSP due to autoimmune encephalitis.
Topics: Adolescent; Encephalitis; Hashimoto Disease; Humans; Male; Pseudobulbar Palsy
PubMed: 29368307
DOI: 10.1007/s00381-018-3720-2 -
European Neurology 2009In 1949, Victor and Adams observed an alcoholic patient who developed quadriplegia and pseudobulbar palsy, and inability to chew, talk or swallow. A post-mortem...
In 1949, Victor and Adams observed an alcoholic patient who developed quadriplegia and pseudobulbar palsy, and inability to chew, talk or swallow. A post-mortem confirmed their suspicion of 'a large, symmetrical, essentially demyelinative lesion occupying the greater part of the basis pontis.' This paper follows the historical evolution of central pontine myelinolysis and the changing concepts of its metabolic aetiology. Too rapid a rate of correction of hyponatraemia is the most common, but not invariable aetiology.
Topics: History, 20th Century; Humans; Hyponatremia; Myelinolysis, Central Pontine
PubMed: 19033724
DOI: 10.1159/000175124 -
Ugeskrift For Laeger Jul 2022Sudden onset anarthria and dysphagia without lateralised neurologic symptoms should prompt an investigation for pseudobulbar palsy, either due to bilateral vascular...
Sudden onset anarthria and dysphagia without lateralised neurologic symptoms should prompt an investigation for pseudobulbar palsy, either due to bilateral vascular lesions of the corticobulbar tracts or, less frequently, Foix-Chavany-Marie Syndrome (FCMS). Here, bilateral damage to the frontal opercular cortex leads to loss of voluntary control of muscles supplied by cranial nerves V, VII, IX, X, XI, and XII. This case report presents a rare case of FCMS on the background of traumatic cerebral lesions following a bicycle incident.
Topics: Deglutition Disorders; Dysarthria; Facial Paralysis; Humans; Syndrome
PubMed: 35959814
DOI: No ID Found -
Journal of Neural Transmission (Vienna,... Apr 2023Progressive supranuclear palsy (PSP) is a neurodegenerative disorder characterized by early postural instability and falls, oculomotor dysfunction (vertical supranuclear... (Review)
Review
Progressive supranuclear palsy (PSP) is a neurodegenerative disorder characterized by early postural instability and falls, oculomotor dysfunction (vertical supranuclear gaze palsy), parkinsonism with poor response to levodopa, pseudobulbar palsy, and cognitive impairment. This four-repeat tauopathy is morphologically featured by accumulation of tau protein in neurons and glia causing neuronal loss and gliosis in the extrapyramidal system associated with cortical atrophy and white matter lesions. Cognitive impairment being frequent in PSP and more severe than in multiple system atrophy and Parkinson disease, is dominated by executive dysfunction, with milder difficulties in memory, and visuo-spatial and naming dysfunctions. Showing longitudinal decline, it has been related to a variety of pathogenic mechanisms associated with the underlying neurodegenerative process, such as involvement of cholinergic and muscarinergic dysfunctions, and striking tau pathology in frontal and temporal cortical regions associated with reduced synaptic density. Altered striatofrontal, fronto-cerebellar, parahippocampal, and multiple subcortical structures, as well as widespread white matter lesions causing extensive connectivity disruptions in cortico-subcortical and cortico-brainstem connections, support the concept that PSP is a brain network disruption disorder. The pathophysiology and pathogenesis of cognitive impairment in PSP, as in other degenerative movement disorders, are complex and deserve further elucidation as a basis for adequate treatment to improve the quality of life of patients with this fatal disease.
Topics: Humans; Supranuclear Palsy, Progressive; Quality of Life; Parkinsonian Disorders; Parkinson Disease; Neurodegenerative Diseases; Cognitive Dysfunction
PubMed: 36862189
DOI: 10.1007/s00702-023-02613-w -
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 -
CNS Spectrums May 2005This monograph summarizes the proceedings of a roundtable meeting convened to discuss pseudobulbar affect (PBA). Two didactic lectures were presented followed by a... (Review)
Review
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.
Topics: Diagnosis, Differential; Humans; Mass Screening; Mood Disorders; Pseudobulbar Palsy
PubMed: 15962457
DOI: 10.1017/s1092852900026602 -
Journal of the Neurological Sciences Aug 2007A variety of neurological conditions and disease states are accompanied by pseudobulbar affect (PBA), an emotional disorder characterized by uncontrollable outbursts of... (Review)
Review
A variety of neurological conditions and disease states are accompanied by pseudobulbar affect (PBA), an emotional disorder characterized by uncontrollable outbursts of laughing and crying. The causes of PBA are unclear but may involve lesions in neural circuits regulating the motor output of emotional expression. Several agents used in treating other psychiatric disorders have been applied in the treatment of PBA with some success but data are limited and these agents are associated with unpleasant side effects due to nonspecific activity in diffuse neural networks. Dextromethorphan (DM), a widely used cough suppressant, acts at receptors in the brainstem and cerebellum, brain regions implicated in the regulation of emotional output. The combination of DM and quinidine (Q), an enzyme inhibitor that blocks DM metabolism, has recently been tested in phase III clinical trials in patients with multiple sclerosis and amyotrophic lateral sclerosis and was both safe and effective in palliating PBA symptoms. In addition, clinical studies pertaining to the safety and efficacy of DM/Q in a variety of neurological disease states are ongoing.
Topics: Animals; Brain; Dextromethorphan; Enzyme Inhibitors; Excitatory Amino Acid Antagonists; Humans; Multiple Sclerosis; Pseudobulbar Palsy; Quinidine
PubMed: 17433820
DOI: 10.1016/j.jns.2006.06.030 -
Amyotrophic Lateral Sclerosis and Other... Sep 2004
Topics: Amyotrophic Lateral Sclerosis; Electromyography; Endpoint Determination; Humans; Muscle Weakness; Neuropsychological Tests; Pseudobulbar Palsy; Regression Analysis; Time Factors
PubMed: 15512885
DOI: 10.1080/17434470410020058 -
Developmental Medicine and Child... Mar 2002
Review
Topics: Adolescent; Bulbar Palsy, Progressive; Child; Child, Preschool; Cognition Disorders; Cranial Nerve Diseases; Diagnosis, Differential; Dysarthria; Female; Humans; Magnetic Resonance Imaging; Male; Paresis; Pseudobulbar Palsy; Seizures; Syndrome; Terminology as Topic
PubMed: 12005322
DOI: 10.1017/s0012162201001931