Weber-Dimitri Syndrome

Weber-Dimitri syndrome

Weber-Dimitri syndrome is a rare neurological disorder characterized by unilateral damage to the cranial nerves.

The syndrome is named after the British neurologist Frederick Parkes Weber (1863-1962) and the Argentine neurologist Victor Dimitri (1885-1956), who independently described the disease in the early 20th century.

In Weber-Dimitri syndrome, the third, fourth, sixth and sometimes first pairs of cranial nerves on one side of the face are affected. This leads to ptosis (drooping) of the upper eyelid, limited mobility of the eyeball and diplopia (double vision).

The causes of Weber-Dimitri syndrome are not completely clear. It is believed that its development is associated with inflammation, trauma, or compression of the brain stem in the area where the cranial nerves exit.

For diagnosis, an MRI of the brain is performed, as well as an examination to exclude tumors and vascular diseases. Treatment is mainly symptomatic with the use of drugs to improve innervation of the extraocular muscles. The prognosis is conditionally favorable, partial restoration of the functions of the oculomotor nerves is possible.

Weber-Dimitri syndrome (Weber-Dimitrovsky syndrome) is a pathological condition characterized by a combination of hemiparesis (hemiplegia), sensory disturbances and damage to the thalamus on one side. Other names for the syndrome: “thalamic neuronocular syndrome”, according to some sources, was first described in 1918 by the Austrian neurologist A. Breuer under the name “thalamic amputation” and by the French neurologist P. Waber in 1875 under the name **“complex homolateral syndrome* * (translated from Latin “cerebri”, Latin “brain”).”

P. Weber associates the development of the syndrome with the removal of tonsils in a patient with tuberculosis, several years after the operation. A. Breuer claims that he identified a discirculatory process in the thalamus as the cause of the disease. The German neurologist J. O. Wilman (1879–1930) interpreted the syndrome as a local ischemic process of the thalamus. He explained the pathogenesis of the syndrome by a violation of hemodynamics in the internal capsule, which is manifested by local paresis of fibers located medially from the midline in both halves of the thalamus, which ensures the transmission of impulses of movement and sensitivity to the brain, including the auditory and gustatory brain. With a significant blood supply to the brain by a tumor localized near the posterior cortex of the parietal lobe, or with a small blood supply to this area, optic tubercle neuritis syndrome or cortical “blanching” syndrome is clinically manifested, which explains the hypothesis of A. Bailey and M. Denisch about the independence of the pathogenesis of the syndrome in the thalamus. cortical tracts from the site of the defect to the cortical projection of this part of the body on the opposite side of the body. These tracts are not protected from the effects of pathogenic processes occurring in the brain. Over time, the patient may develop various neurological diseases that involve the above structures or regressive diseases that were previously located in the affected half of the brain. This once again indicates an acute inflammatory (polyneuritic) or demyelinating genesis of the syndrome. One of the main causes is brain infection in spinocerebellar degenerations. Consequently, the term “amyotrophic destabilization of Nash-Smithson paralysis” should be applied to the syndrome, that is, an extensive spasm of the cerebral muscles associated only with unilateral changes in the area of ​​visual, associative and subcortical motor neurons.