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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.
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.
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.
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]
UN: Estimate of life expectancy for various ages in 2023; Locations Life expectancy for population in general Life expectancy for male Life expectancy for female Sex gap; at birth bonus 0→15 at 15 bonus 15→65 at 65 bonus 65→80 at 80 at birth at 15 at 65 at 80 at birth at 15 at 65 at 80 at birth at 15 at 65 at 80 Hong Kong: 85.51: 0.21: 70 ...
Spinal and bulbar muscular atrophy (SBMA), popularly known as Kennedy's disease, is a rare, adult-onset, X-linked recessive lower motor neuron disease caused by trinucleotide CAG repeat expansions in exon 1 of the androgen receptor (AR) gene, which results in both loss of AR function and toxic gain of function.
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.
While there is a lot of variation in how CP affects people, it has been found that "independent gross motor functional ability is a very strong determinant of life expectancy". [160] According to the Australian Bureau of Statistics , in 2014, 104 Australians died of cerebral palsy. [ 161 ]