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Those with a bulbar onset have a worse prognosis than limb-onset ALS; a population-based study found that bulbar-onset ALS patients had a median survival of 2.0 years and a 10-year survival rate of 3%, while limb-onset ALS patients had a median survival of 2.6 years and a 10-year survival rate of 13%. [52]
Prognosis for PBP patients is poor. Progressive bulbar palsy symptoms can include progressive difficulty with talking and swallowing. [4] Patients can also exhibit reduced gag reflexes, weak palatal movements, fasciculations, and weak movement of the facial muscles and tongue.
In ALS the main type of onset is bulbar followed by limb-onset ALS which describes the region of motor neurons first affected. [3] Individuals may also present with respiratory-onset ALS, [7] but this occurs very rarely. Since there are three different types of ALS, ALSFRS-R scores are often grouped in categories depending on type of onset. [7]
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Paul Berry, 71, has devoted his professional life to helping others find peace, and is now finding his own after a diagnosis of Bulbar Onset ALS.
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.
Differential diagnosis can be challenging due to the number of overlapping symptoms, shared between several motor neuron diseases. [14] Frequently, the diagnosis is based on clinical findings (i.e. LMN vs. UMN signs and symptoms, patterns of weakness), family history of MND, and a variation of tests, many of which are used to rule out disease ...
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.
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