Nothnagel syndrome

Last revised by Frank Gaillard on 7 Sep 2019

Nothnagel syndrome is a rare midbrain syndrome that involves the tectum of the midbrain (quadrigeminal plate) and superior cerebellar peduncles 1-4,6.

Classically, the syndrome involves the oculomotor nerve fascicles and superior cerebellar peduncle, leading to ipsilateral CN III palsy and limb ataxia respectively 1-4.

Although it can be caused by stroke, especially hemorrhages rather than infarctions, it is more commonly seen due to neoplasm extending from the tectal plate into the superior cerebellar peduncles 1-4,6.

Lesions are in the tectum of the midbrain with imaging characteristics depending on the underlying cause.

The syndrome was first described by Hermann Nothnagel (1841-1905), a German-Austrian physician, in 1879 5.

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