Pseudobulbar palsy

Specialty| Neurology

Pseudobulbar palsy is a medical condition characterized by the inability to control facial movements (such as chewing and speaking) and caused by a variety of neurological disorders. Patients experience difficulty chewing and swallowing, have increased reflexes and spasticity in tongue and the bulbar region, and demonstrate slurred speech (which is often the initial presentation of the disorder), sometimes also demonstrating uncontrolled emotional outbursts.[1]

The condition is usually caused by the bilateral damage to corticobulbar pathways, which are upper motor neuron pathways that course from the cerebral cortex to nuclei of cranial nerves in the brain stem.

## Contents

* 1 Signs and symptoms
* 2 Causes
* 3 Pathophysiology
* 4 Diagnosis
* 5 Treatment
* 6 See also
* 7 References
* 8 External links

## Signs and symptoms

Signs and symptoms of pseudobulbar palsy include:

* Slow and indistinct speech
* Dysphagia (difficulty in swallowing)
* Small, stiff and spastic tongue
* Brisk jaw jerk
* Dysarthria
* Labile affect[2]
* Gag reflex may be normal, exaggerated or absent
* Examination may reveal upper motor neuron lesion of the limbs

## Causes

Pseudobulbar palsy is the result of damage of motor fibers traveling from the cerebral cortex to the lower brain stem. This damage might arise in the course of a variety of neurological conditions that involve demyelination and bilateral corticobulbar lesions. Examples include:[citation needed]

* Vascular causes: bilateral hemisphere infarction, CADASIL syndrome, artery of percheron infarct[3]
* Progressive supranuclear palsy
* Amyotrophic lateral sclerosis
* Parkinson's disease and related multiple system atrophy
* Various motor neuron diseases, especially those involving demyelination
* Multiple sclerosis and other inflammatory disorders
* High brain stem tumors
* Metabolic causes: osmotic demyelination syndrome[4]
* Neurological involvement in Behçet's disease
* Brain trauma

## Pathophysiology

The proposed mechanism of pseudobulbar palsy points to the disinhibition of the motor neurons controlling laughter and crying, proposing that a reciprocal pathway exists between the cerebellum and the brain stem that adjusts laughter and crying responses, making them appropriate to context.[5] The pseudobulbar crying could also be induced by stimulation in the region of the subthalamic nucleus of the brain.[6]

## Diagnosis

Diagnosis of pseudobulbar palsy is based on observation of the symptoms of the condition. Tests examining jaw jerk and gag reflex can also be performed. It has been suggested that the majority of patients with pathological laughter and crying have pseudobulbar palsy due to bilateral corticobulbar lesions and often a bipyramidal involvement of arms and legs.[7] To further confirm the condition, MRI can be performed to define the areas of brain abnormality.[citation needed]

## Treatment

Since pseudobulbar palsy is a syndrome associated with other diseases, treating the underlying disease may eventually reduce the symptoms of pseudobulbar palsy.[citation needed]

Possible pharmacological interventions for pseudobulbar affect include the tricyclic antidepressants, serotonin reuptake inhibitors, and a novel approach utilizing dextromethorphan and quinidine sulfate. Nuedexta is an FDA approved medication for pseudobulbar affect. Dextromethorphan, an N-methyl-D-aspartate receptor antagonist, inhibits glutamatergic transmission in the regions of the brainstem and cerebellum, which are hypothesized to be involved in pseudobulbar symptoms, and acts as a sigma ligand, binding to the sigma-1 receptors that mediate the emotional motor expression.[5]

## See also

* Corticobulbar tract
* Bulbar palsy, a similar syndrome caused by the damage of lower motor neurons.
* Motor neuron disease

## References

1. ↑ "Bulbar and Pseudobulbar Palsy. What is Bulbar Palsy?" (in en-GB). 21 October 2021. http://patient.info/doctor/bulbar-and-pseudobulbar-palsy.
2. ↑ "Pseudobulbar palsy caused by a large petroclival meningioma: report of two cases". Skull Base 12 (2): 67–71. May 2002. doi:10.1055/s-2002-31568-1. PMID 17167648.
3. ↑ "Isolated acute pseudobulbar palsy with infarction of artery of percheron: case report and literature review". African Health Sciences 21 (1): 166–171. March 2021. doi:10.4314/ahs.v21i1.22. PMID 34394294.
4. ↑ "Subcortical white matter lesions in osmotic demyelination syndrome". AJNR. American Journal of Neuroradiology 16 (7): 1495–1497. August 1995. PMID 7484639. ]
5. ↑ 5.0 5.1 "Pseudobulbar palsy and affect in a case of progressive multifocal leukoencephalopathy". The Journal of Neuropsychiatry and Clinical Neurosciences 20 (1): 110–111. 2008. doi:10.1176/jnp.2008.20.1.110. PMID 18305298.
6. ↑ "Pseudobulbar crying induced by stimulation in the region of the subthalamic nucleus". Journal of Neurology, Neurosurgery, and Psychiatry 75 (6): 921–923. June 2004. doi:10.1136/jnnp.2003.016485. PMID 15146017.
7. ↑ "Is the syndrome of pathological laughing and crying a manifestation of pseudobulbar palsy?". Journal of Neurology, Neurosurgery, and Psychiatry 52 (4): 523–525. April 1989. doi:10.1136/jnnp.52.4.523. PMID 2738597.

## External links

Classification|

* ICD-9-CM: 335.23
* MeSH: D020828
* DiseasesDB: 10826

|

* v
* t
* e

Diseases of the nervous system, primarily CNS (G04–G47, 323–349)

Inflammation|

| Brain|

* Encephalitis
* Viral encephalitis
* Herpesviral encephalitis
* Limbic encephalitis
* Encephalitis lethargica
* Cavernous sinus thrombosis
* Brain abscess
* Amoebic

|

Spinal cord|

* Myelitis: Poliomyelitis
* Demyelinating disease
* Transverse myelitis
* Tropical spastic paraparesis
* Epidural abscess

Both/either|

* Encephalomyelitis
* Acute disseminated
* Myalgic
* Meningoencephalitis

Brain/
encephalopathy|

| Degenerative|

| Extrapyramidal and
movement disorders|

* Basal ganglia disease
* Parkinsonism
* PD
* Postencephalitic
* NMS
* PKAN
* Tauopathy
* PSP
* Striatonigral degeneration
* Hemiballismus
* HD
* OA

* Dyskinesia
* Dystonia
* Status dystonicus
* Spasmodic torticollis
* Meige's
* Blepharospasm
* Athetosis
* Chorea
* Choreoathetosis
* Myoclonus
* Myoclonic epilepsy
* Akathisia

* Tremor
* Essential tremor
* Intention tremor
* Restless legs
* Stiff-person

|

Dementia|

* Tauopathy
* Alzheimer's
* Early-onset
* Primary progressive aphasia
* Frontotemporal dementia/Frontotemporal lobar degeneration
* Pick's
* Dementia with Lewy bodies
* Posterior cortical atrophy

* Vascular dementia

Mitochondrial disease|

* Leigh syndrome

Demyelinating|

* autoimmune
* Multiple sclerosis
* Neuromyelitis optica
* Schilder's disease
* hereditary
* Adrenoleukodystrophy
* Alexander
* Canavan
* Krabbe
* ML
* PMD
* VWM
* MFC
* CAMFAK syndrome
* Central pontine myelinolysis
* Marchiafava–Bignami disease
* Alpers' disease

Episodic/
paroxysmal|

| Seizure/epilepsy|

* Focal
* Generalised
* Status epilepticus
* Myoclonic epilepsy

|

Headache|

* Migraine
* Familial hemiplegic
* Cluster
* Tension

Cerebrovascular|

* TIA
* Amaurosis fugax
* Transient global amnesia
* Acute aphasia

* Stroke
* MCA
* ACA
* PCA
* Foville's
* Millard–Gubler
* Lateral medullary
* Medial medullary
* Weber's
* Lacunar stroke

Sleep disorders|

* Insomnia
* Hypersomnia
* Sleep apnea
* Obstructive
* Congenital central hypoventilation syndrome
* Narcolepsy
* Cataplexy
* Kleine–Levin
* Circadian rhythm sleep disorder
* Advanced sleep phase disorder
* Delayed sleep phase disorder
* Non-24-hour sleep–wake disorder
* Jet lag

CSF|

* Intracranial hypertension
* Hydrocephalus/NPH
* Choroid plexus papilloma
* Idiopathic intracranial hypertension
* Cerebral edema
* Intracranial hypotension

Other|

* Brain herniation
* Reye's
* Hepatic encephalopathy
* Toxic encephalopathy
* Hashimoto's encephalopathy

Spinal cord/
myelopathy|

* Syringomyelia
* Syringobulbia
* Morvan's syndrome
* Vascular myelopathy
* Foix–Alajouanine syndrome
* Spinal cord compression

Both/either|

| Degenerative|

| SA|

* Friedreich's ataxia
* Ataxia–telangiectasia

|

MND|

* UMN only:
* Primary lateral sclerosis
* Pseudobulbar palsy
* Hereditary spastic paraplegia

* LMN only:
* Distal hereditary motor neuronopathies
* Spinal muscular atrophies
* SMA
* SMAX1
* SMAX2
* DSMA1
* Congenital DSMA
* SMA-PCH
* SMA-LED
* SMA-PME
* Progressive muscular atrophy
* Progressive bulbar palsy
* Fazio–Londe
* Infantile progressive bulbar palsy

* both:
* Amyotrophic lateral sclerosis

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