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Pseudobulbar affect (PBA), or emotional incontinence, is a type of neurological disorder characterized by uncontrollable episodes of crying or laughing. PBA occurs secondary to a neurologic disorder or brain injury .
[1] [2] They include amyotrophic lateral sclerosis (ALS), [3] [4] progressive bulbar palsy (PBP), pseudobulbar palsy, progressive muscular atrophy (PMA), primary lateral sclerosis (PLS), spinal muscular atrophy (SMA) and monomelic amyotrophy (MMA), as well as some rarer variants resembling ALS. Motor neuron diseases affect both children and ...
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 ...
ALS is the most common motor neuron disease in adults and the third most common neurodegenerative disease [61] after Alzheimer's disease and Parkinson's disease. [121] Worldwide the number of people who develop ALS yearly is estimated to be 1.9 people per 100,000 per year, while the number of people who have ALS at any given time is estimated ...
The cause of PBP is unknown. One form of PBP is found to occur within patients that have a CuZn-superoxide dismutase (SOD1) mutation. [7] Progressive bulbar palsy patients that have this mutation are classified with FALS patients, Familial ALS (FALS) accounts for about 5%-10% of all ALS cases and is caused by genetic factors.
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.
Public awareness of the disease gained prominence upon the diagnosis of baseball player Lou Gehrig, whose name would become an alternative title for the disease. Astrophysicist Stephen Hawking, whose ALS was diagnosed in 1963, had the disease for 55 years, the longest recorded time one had the disease. He died at the age of 76 in 2018.
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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