Benedikt Syndrome
| Autor: | Burr P; Greater Glasgow and Clyde, Mandalaneni K; St.George's University |
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| Jazyk: | angličtina |
| Zdroj: | 2022 Jan. |
| Abstrakt: | Benedikt syndrome is an extremely rare eponymously named cluster of symptoms relating to anatomically specific damage of varying etiology to the midbrain.[1] It is characterized by: 1. Ipsilateral oculomotor nerve palsy. 2. Contralateral hemiparesis. 3. Contralateral cerebellar ataxia and/or Holmes tremor and/or choreoathetosis.[1][2]. A more precise description remains elusive for Benedikt syndrome owing to historical legacy with significant variance in the description in textbooks and clinical case studies. Moiz Benedikt, an Austrian neurologist, first described this constellation of symptoms in a lecture delivered in 1889. He described three similar cases involving oculomotor palsy, contralateral hemiparesis, and contralateral tremors.[1] One of these cases was a 4-year-old with a right oculomotor palsy, contralateral hand jerking/tremor, contralateral involuntary leg movements, and contralateral upper motor neuron facial weakness. An autopsy revealed a tuberculoma a "pigeon's egg" in size amongst other smaller tuberculomas in the cerebral hemispheres and cerebellum.[1] In 1893 Charcot was the first to use the term Benedikt syndrome to describe a similar presentation of a 37-year-old male with left-sided ptosis, adduction weakness, mydriasis, right arm choreoathetosis and right leg weakness with resting tremor. 2 more cases were presented in 1900: the first with right hemiplegia, involuntary movements, and bilateral oculomotor palsy; the second case with hemiplegia on the left side, tremor of the left arm, right exotropia and right mydriasis. These first and subsequent descriptions over the next 40 years describe patients with Benedikt syndrome with slightly varying symptoms, etiology, and lesion location.[1] This historical legacy has subsequently led to patients with pupil sparing, associated "plus-minus lid" syndrome, and varied associated tremors and choreoathetosis' being included in the umbrella nomenclature of "Benedikt syndrome."[3][4][5][1] Current consensus requires the above three symptoms to be considered to be Benedikt syndrome, but as mentioned, additional symptoms do not exclude the diagnosis.[1][2][5] (Copyright © 2022, StatPearls Publishing LLC.) |
| Databáze: | MEDLINE |
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