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Progressive bulbar palsy (PBP) is a medical condition. It belongs to a group of disorders known as motor neuron diseases. [1] PBP is a disease that attacks the nerves supplying the bulbar muscles. These disorders are characterized by the degeneration of motor neurons in the cerebral cortex, spinal cord, brain stem, and pyramidal tracts.
Symptoms of motor neuron diseases can be first seen at birth or can come on slowly later in life. Most of these diseases worsen over time; while some, such as ALS, shorten one's life expectancy, others do not. [2] Currently, there are no approved treatments for the majority of motor neuron disorders, and care is mostly symptomatic. [2]
In contrast, pseudobulbar palsy is a clinical syndrome similar to bulbar palsy but in which the damage is located in upper motor neurons of the corticobulbar tracts in the mid-pons (i.e., in the cranial nerves IX-XII), that is the nerve cells coming down from the cerebral cortex innervating the motor nuclei in the medulla.
Fazio–Londe disease (FLD), also called progressive bulbar palsy of childhood, [1] [2] is a very rare inherited motor neuron disease of children and young adults and is characterized by progressive paralysis of muscles innervated by cranial nerves.
The child suffers a progressive degradation of the respiratory system until respiratory failure. There is no consensus on the life expectancy in DSMA1 despite a number of studies being conducted. A small number of patients survive past two years of age but they lack signs of diaphragmatic paralysis or their breathing is dependent on a ...
Life expectancy is reduced, even as most people with SMA 2 live well into adulthood even without treatment. 253550: SMA 3 (Juvenile) Kugelberg–Welander disease >12 months The juvenile form, diagnosed in around 30% of patients, manifests after 12 months of age, or after the children have already learned to make at least a few independent steps.
Brown-Vialetto-Van-Laere syndrome (BVVL), is a rare, progressive, inherited neurodegenerative disorder that most often manifests in infancy or early childhood.Since 2010, mutations in the SLC52A1, SLC52A2, or SLC52A3 genes, which encode riboflavin transporters, have been identified as the cause of BVVL.
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: [3] Progressive supranuclear palsy; Amyotrophic lateral sclerosis