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Nothnagel Syndrome

  • Cerebellar Classics
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Abstract 

The internist Hermann Nothnagel (1841–1905) took a special interest in the cerebellum. In an early experimental study on rabbits conducted in 1876, he demonstrated the involvement of the vermis in the pathophysiology of motor ataxia. Between 1879 and 1889, he reported four cases of tectal tumors that clinically manifested with bilateral ophthalmoplegia and unilateral gait ataxia, culminating in the Cerebellar Classic highlighted here. Nothnagel attributed this clinical syndrome to lesions of the colliculi (“quadrigeminal bodies”) and compression of the nuclei of the third cranial nerves, but also left open the possibility of the involvement of neighboring structures, such as the cerebellar vermis. Today, the ataxic component of Nothnagel syndrome is explained by a dorsal midbrain abnormality of either neoplastic or vascular origin, involving the superior cerebellar peduncles, besides the oculomotor nerves.

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Fig. 1

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Authors and Affiliations

  1. Sector of Experimental Cognitive Psychology, Department of Psychology, Faculty of Philosophy, Aristotelian University, 54124, Thessaloniki, Greece

    Lazaros C. Triarhou

  2. Unité des Ataxies Cérébelleuses, CHU-Charleroi, Charleroi, Belgium

    Mario Manto

  3. Service des Neurosciences, University of Mons, Mons, Belgium

    Mario Manto

Authors
  1. Lazaros C. Triarhou
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  2. Mario Manto
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Concept, writing, and approval of the final version: LCT and MM.

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Correspondence to Lazaros C. Triarhou.

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Triarhou, L.C., Manto, M. Nothnagel Syndrome. Cerebellum 22, 487–505 (2023). https://doi.org/10.1007/s12311-022-01437-w

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