Vulpian-Bernhardt muscular atrophy is a progressive neural amyotrophy of the upper limbs, described by the French physiologist and neurologist Vulpian in 1886 and the German neurologist Bernhardt in 1891.
Main signs of the disease:
-
The proximal muscles of the upper extremities are affected, and less commonly, the muscles of the legs.
-
Muscle atrophy begins in the distal parts of the upper limbs and gradually spreads proximally.
-
The strength of the muscles of the hands and feet is maintained.
-
There are no sensory disturbances.
-
Reflexes from the limbs are alive.
-
The course is slowly progressive.
-
The cause of the disease is unknown; primary damage to the anterior horns of the spinal cord is assumed.
Vulpian-Bernhardt muscular atrophy differs from Werdnig-Hoffmann spinal amyotrophy in that it affects predominantly the upper extremities and has a slower course. Treatment is symptomatic.
Vulpian-Bernhard was founded by the French neurologist Alexandre Vulpian and the German neuropathologist Maximilian Bernhard in the 19th century. This brain disorder belongs to a group of acquired speech disorders that include epilepsy, dysarthria and aphasia. The so-called Vulpi-Behrend syndrome develops slowly and gradually with gradual deterioration