Wernekink commissure syndrome
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Wernekink commissure syndrome (sometimes spelled Wernekinck) is a rare brainstem syndrome caused by lesions (usually infarcts) involving the decussation of the superior cerebellar peduncles (Wernekink commissure) at the caudal midbrain.
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The syndrome is characterized by bilateral cerebellar dysfunction 1-3. This can manifest as gait and truncal ataxia, dysmetria on finger-nose-finger and heel-knee-shin tests, and dysdiadochokinesis. In addition, marked dysarthria is a consistent finding 1-3. Variable eye movement disorders and Holmes tremor are also described 6.
Midbrain lesions may involve the nearby medial longitudinal fasciculus, resulting in internuclear ophthalmoplegia 2. In some patients, involvement of the dentatorubral fibers, part of the triangle of Guillain and Mollaret, can manifest as delayed onset palatal tremor/myoclonus 1,2.
The Wernekink commissure is supplied by inferior paramedian mesencephalic arteries, which are a subset of interpeduncular fossa perforating branches that may arise from the tip of the basilar artery, superior cerebellar artery, and/or P1 segment of the posterior cerebral artery 2. In a minority of cases, bilateral inferior paramedian mesencephalic arteries can be derived from a common trunk 2.
An acute infarct of the Wernekink commissure appears as a hyperintensity on DWI in the caudal paramedian midbrain, ventral to the cerebral aqueduct 1,2. The lesion on DWI or FLAIR may be round, oval, heart, or V-shaped 2. Most cases are unilateral but some infarcts cross midline 2.
Because the lesion disrupts the triangle of Guillain and Mollaret, follow-up MRI may show hypertrophic olivary degeneration 1,2.
History and etymology
The disorder was first reported in 1958 by Lhermitte 4.
The commissure is named after Friedrich Christian Gregor Wernekinck (1798-1839), a German minerologist and anatomist 5. His pupil, Franz Joseph Julius Wilbrand (1811-1894), attributed the discovery of the commissure to Wernekinck and named it the horseshoe-shaped commissure of Wernekinck (commissura hipposideroformis Wernekincki).
The normal fibers of the Wernekink commissure appear slightly hyperintense on DWI compared to surrounding brainstem in some people. Thus, caution is warranted so as to not over-call infarction when the area is actually normal.
- 1. Liu H, Qiao L, He Z. Wernekink commissure syndrome: a rare midbrain syndrome. (2012) Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology. 33 (6): 1419-21. doi:10.1007/s10072-012-0966-4 - Pubmed
- 2. Zhou C, He Y, Chao Z et-al. Wernekink Commissure Syndrome Secondary to Bilateral Caudal Paramedian Midbrain Infarction Presenting with a Unique "Heart or V" Appearance Sign: Case Report and Review of the Literature. (2017) Frontiers in neurology. 8: 376. doi:10.3389/fneur.2017.00376 - Pubmed
- 3. Kim TW, Yoo S, Koo J. Wernekink commissure syndrome secondary to ischemic stroke: severe dysarthria is one of the main characteristics of this syndrome. (2014) Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology. 35 (9): 1475-7. doi:10.1007/s10072-014-1789-2 - Pubmed
- 4. LHERMITTE F. [The cerebellar syndrome: anatomo-clinical study in the adult]. (1958) Revue neurologique. 98 (6): 435-77. Pubmed
- 5. Voogd J, van Baarsen K. The horseshoe-shaped commissure of Wernekinck or the decussation of the brachium conjunctivum methodological changes in the 1840s. (2014) Cerebellum (London, England). 13 (1): 113-20. doi:10.1007/s12311-013-0520-9 - Pubmed
- 6. Mullaguri N, Battineni A, Chuquilin M. Wernekink commissure syndrome with palatal myoclonus at onset: a case report and review of the literature. (2018) Journal of Medical Case Reports. 12 (1): 1. doi:10.1186/s13256-018-1657-4