Nothnagel syndrome
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Citation:
Sharma R, Gaillard F, Thurston M, Nothnagel syndrome. Reference article, Radiopaedia.org (Accessed on 24 Mar 2025) https://doi.org/10.53347/rID-54279
rID:
54279
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Disclosures:
At the time the article was created Rohit Sharma had no recorded disclosures.
View Rohit Sharma's current disclosures
Last revised:
Disclosures:
At the time the article was last revised Frank Gaillard had no recorded disclosures.
View Frank Gaillard's current disclosures
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6 times, by
3 contributors -
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Synonyms:
- Nothnagel's syndrome
Nothnagel syndrome is a rare midbrain syndrome that involves the tectum of the midbrain (quadrigeminal plate) and superior cerebellar peduncles 1-4,6.
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Clinical presentation
Classically, the syndrome involves the oculomotor nerve fascicles and superior cerebellar peduncle, leading to ipsilateral CN III palsy and limb ataxia respectively 1-4.
Pathology
Although it can be caused by stroke, especially haemorrhages rather than infarctions, it is more commonly seen due to neoplasm extending from the tectal plate into the superior cerebellar peduncles 1-4,6.
Radiographic features
Lesions are in the tectum of the midbrain with imaging characteristics depending on the underlying cause.
History and etymology
The syndrome was first described by Hermann Nothnagel (1841-1905), a German-Austrian physician, in 1879 5.
References
- 1. Liu GT, Crenner CW, Logigian EL, Charness ME, Samuels MA. Midbrain syndromes of Benedikt, Claude, and Nothnagel: setting the record straight. Neurology. 42 (9): 1820-2. Pubmed
- 2. Ropper AH, Samuels MA, Klein JP. Adams and Victor's principles of neurology 10th ed. New York: McGraw-Hill Medical Pub. Division; 2014.
- 3. Patten J. Neurological differential diagnosis 2nd ed. Springer Science & Business Media; 1996.
- 4. Moncayo J. Midbrain infarcts and hemorrhages. Frontiers of neurology and neuroscience. 30: 158-61. doi:10.1159/000333630 - Pubmed
- 5. Nothnagel H. Corpora quadrigemina. In: Topische Diagnostik der Gehirnkrankheiten. A Hirschwald, 1879:204-220.
- 6. Sara Sciacca, Jeremy Lynch, Indran Davagnanam, Robert Barker. Midbrain, Pons, and Medulla: Anatomy and Syndromes. (2019) RadioGraphics. 39 (4): 1110-1125. doi:10.1148/rg.2019180126 - Pubmed
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