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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.
The rate of disease progression is extremely variable with survival ranging from 14 months to 46 years. Of those who died from FOSMN, the mean duration of disease was 7.5 years. [6] Cranial nerve (bulbar) weakness is a common causes of death in those with FOSM, with aspiration pneumonia or respiratory failure commonly leading to death. [6]
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.
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.
The term pseudobulbar (pseudo-+ bulbar) came from the idea that the symptoms seemed similar to those caused by a bulbar lesion (that is, a lesion in the medulla oblongata). Terms such as forced crying, involuntary crying, pathological emotionality, and emotional incontinence have also been used, although less frequently. [4]
Generalized MG has muscle weakness with a variable combination of the bulbar, axial, or limb and respiratory muscles. [ 53 ] Patients can also be sub-grouped by the age of onset: juvenile-onset MG (onset age ≤ 18 years of age), early-onset MG (EOMG; 19–50 years of age), late-onset MG (LOMG; onset > 50 years of age), and very late-onset ...
Berger, in 1876, first reported a case of 12-year-old boy with progressive bulbar paralysis. His medical history revealed a neurological illness with difficulty in swallowing liquids and solids, nasal regurgitation to liquids, nasal twang 2 years ago. He was treated for post-diptheritic bulbar palsy and had a residual bulbar weakness.
More modern editions simply refer to a man who is paralysed. Although the term has historically been associated with paralysis generally, "is now almost always used in connection to the word cerebral—meaning the brain". [1] Specific kinds of palsy include: Bell's palsy, partial facial paralysis; Bulbar palsy, impairment of cranial nerves