Pseudobulbar palsy

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Clinic

  • It is a medical condition characterized by the inability to control facial movements (such as chewing and speaking) and slurred speech
  • It is caused by a variety of neurological disorders, usually 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 brainstem.
  • Patients experience difficulty chewing and swallowing, have increased reflexes and spasticity in tongue and the bulbar region, and uncontrolled emotional outbursts.
  • It Describes bilateral supranuclear (UMN) lesions of lower cranial nerves producing weakness of the tongue and pharyngeal muscles.
  • It resembles, superficially, a bulbar palsy: hence pseudobulbar
  • Slow speech+ Dysphagia + Dysarthria + Spastic tongue + Pseudobulbar affect


Signs / Symptoms

  • Slow and indistinct speech
  • Dysphagia
  • Small, stiff and spastic tongue
  • Brisk jaw jerk
  • Dysarthria
  • Labile affect
  • Gag reflex may be normal, exaggerated or absent
  • UMN 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 including:

  • Vascular causes: bilateral hemisphere infarction, CADASIL syndrome, artery of percheron infarct
  • Progressive supranuclear palsy
  • ALS
  • Parkinson's disease and related MSA
  • Various motor neuron diseases, especially those involving demyelination
  • MS and other inflammatory disorders
  • High brain stem tumors
  • Metabolic causes: osmotic demyelination syndrome
  • 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. T
  • Pseudobulbar crying could also be induced by stimulation in the region of the subthalamic nucleus of the brain.

Entities

  • Dysphagia
  • Dysarthria / Speech difficult
  • Emotional lability / Pseudobulbar affect
  • Hyper reflexia
  • Spasm
Face, Palsy
Dysathria
Dysphagia
Hyper Reflexia
Emotional lability


Miasms

GSS [1]

  1. Zhao MM, Feng LS, Hou S, Shen PP, Cui L, Feng JC. Gerstmann-Sträussler-Scheinker disease: A case report. World J Clin Cases. 2019 Feb 6;7(3):389-395. doi: 10.12998/wjcc.v7.i3.389. PMID: 30746381; PMCID: PMC6369391.