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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.
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.
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.
Infantile progressive bulbar palsy is a rare type of progressive bulbar palsy that occurs in children. The disease exists in both rapid and slow onsets, and involves inflammation of the gray matter of the bulb. [ 1 ]
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
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]
Spinal and bulbar muscular atrophy (SBMA) Kennedy's disease (KD) 313200: NR3C4: Xq12: X-linked recessive: Affects primarily bulbar muscles as well as sensory nerves mainly in adult men, progressive X-linked spinal muscular atrophy type 2 (SMAX2) Arthrogryposis multiplex congenita – X-linked type 1 (AMCX1) 301830: UBA1: Xp11.23: X-linked recessive
Focal neurologic signs, also known as focal neurological deficits or focal CNS signs, are impairments of nerve, spinal cord, or brain function that affects a specific region of the body, e.g. weakness in the left arm, the right leg, paresis, or plegia. [citation needed]