Wernekink commissure syndrome with palatal myoclonus at onset: a case report and review of the literature

Mullaguri, Naresh; Battineni, Anusha; Chuquilin, Miguel

doi:10.1186/s13256-018-1657-4

Cited by 10 publications

(3 citation statements)

References 16 publications

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“…Other findings that have been frequently reported in WCS are ophthalmoplegia and nystagmus [3,5,10]. In their study, Zhou et al reported that in 12 patients with caudal paramedian midbrain infarction, diplopia and internuclear ophthalmoplegia (INO) were frequently seen [11].…”

Section: Discussionmentioning

confidence: 93%

Wernekink Commissure Syndrome With Bilateral Cerebellar Signs and Holmes Tremor in a Patient With a Preexisting Movement Disorder

Agarwal¹,

Biswas²,

Kumar³

2023

Cureus

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Wernekink commissure syndrome (WCS) is an extremely rare midbrain syndrome, in which there is the selective destruction of the decussation of the superior cerebellar peduncle, which commonly presents with bilateral cerebellar signs. We describe a case of WCS with Holmes tremor in a patient having an undiagnosed involuntary movement disorder since childhood following an undocumented case of meningitis. The patient presented with sudden onset gait instability with bilateral cerebellar signs (more prominent on the left side), Holmes tremor in bilateral limbs, slurred speech, and marked dysarthria. No ophthalmoplegia or palatal tremors were noted. The patient was conservatively managed along the lines of a stroke, and there was a marked improvement in cerebellar signs and Holmes tremor with time but no evolution (improvement or worsening) was observed in the involuntary movements of limbs and face that were present before the onset of WCS.

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Section: Discussionmentioning

confidence: 93%

Wernekink Commissure Syndrome With Bilateral Cerebellar Signs and Holmes Tremor in a Patient With a Preexisting Movement Disorder

Agarwal¹,

Biswas²,

Kumar³

2023

Cureus

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“…Traktus dentato-rubro-olivary yang turun menghubungkan dentate dan inti sela otak kecil dengan inti merah kontralateral melalui batang otak superior dan nukleus olivari inferior di medula. Lesi pada commissure Wernekink di cauda midbrain dapat merusak jalur dentato-rubrothalamic atau dentato-rubro-olivary, yang masing-masing menyebabkan tremor Holmes dini atau tertunda dan myoclonus palatal atau tremor onset tertunda (Mullaguri, Battineni, & Chuquilin, 2018).…”

Section: Hasil Dan Diskusiunclassified

Sindrom Komisura Wernekink; Patofisiologi dan Diagnosa: Sebuah Tinjauan Literatur

Putri

2021

maj. kesehat. indones.

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Sindroma commissure Wernekink adalah sindrom midbrain yang langka yang secara selektif mempengaruhi komisura Wernekink, ditandai dengan ataksia serebelar bilateral dan gangguan pergerakan mata, terutama oftalmoplegia internuklear. Defisit neurologis yang terlihat pada WCS diyakini disebabkan oleh gangguan pada tangkai serebelar superior, fasciculi longitudinal medial, dan traktus tegmental sentral. Pemeriksaan radiologis yang berguna yaitu pada magnetic resonance imaging (MRI) dimana didapatkan gambaran "Lesi berbentuk hati" yang dianggap sebagai gambaran khas dari infark medular medial bilateral. Wernekink commissure syndrome is a rare midbrain syndrome selectively affecting the Wernekink commissure, characterized by bilateral cerebellar ataxia and eye movement disorders, especially internuclear ophthalmoplegia. Neurologic deficits seen in WCS are believed to be caused by disruptions of the superior cerebellar peduncles, medial longitudinal fasciculi, and the central tegmental tract. Initially, a “heart-shaped lesion” on magnetic resonance imaging (MRI) was thought to be a distinctive presentation of bilateral medial medullary infarction.

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“…Wernekink commissure syndrome is a rare syndrome , due to involvement of the midbrain and it is characterized by bilateral cerebellar ataxia, internuclear ophthalmoplegia and delayed onset palatal myoclonus. [1][2][3] The structures located in the medial part of midbrain include oculomotor nuclei, descending cerebellar tracts, medial longitudinal fasciculus, and central tegmental tract, and a lesion in this region can result in the clinical features described in this syndrome. 4 Wernekink commissure syndrome was first described by Lhermitte and it was named after German anatomist Friedrich Wernekink, who described it as a horseshoe-shaped commissure.…”

Section: Introductionmentioning

confidence: 99%

Wernekink commissure syndrome: clinico-radiological criteria

Sheetal

Thomas

2020

Nep J Neurosci

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Introduction: Wernekink commissure syndrome is a rare midbrain syndrome selectively affecting the Wernekink commissure, characterized by bilateral cerebellar ataxia and eye movement disorders, especially internuclear ophthalmoplegia. This article aims at proposing clinico-radiological criteria for Wernekink commissure syndrome with review of the neural circuitary responsible, to aid in recognition and reporting. Methods: This was a prospective study conducted at Department of Neurology, at Pushpagiri institute of medical sciences and research centre, Thiruvalla, Kerala, India, over a period of 5 years among patient with pure midbrain syndromes. All patients with pure midbrain infarction were studied. Subjects presenting with clinical features of Wernekink commissure syndrome were shortlisted and were assessed by investigators independently. Neuroradiology was assessed by investigators 1 and 2, independently. The demographic profile, risk factors, clinical features, neuroimaging findings and outcomes were analysed using SPSSv21. Results: Details of 43 subjects with pure midbrain stroke were included in the study. 8 had clinical features of Wernekink commissure syndrome. The most common findings were bilateral ataxia and unilateral or bilateral internuclear ophthalmoplegia. None of our patients had palatal tremor. Unilateral caudal paramedical infarction was seen on MRI in 5 patients, whereas it was bilateral in 3 patients. Conclusions: The proposed clinico-radiological criteria consisting of all of the essential criteria (Ipsilateral internuclear ophthalmoplegia, Unilateral or bilateral ataxia and Neuroradiological evidence of Caudal midbrain involvement) with or without one among the supportive criteria (Rubral Tremor, Palatal myoclonus) can safely point at a diagnosis of Wernekink commissure syndrome.

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Wernekink commissure syndrome with palatal myoclonus at onset: a case report and review of the literature

Cited by 10 publications

References 16 publications

Wernekink Commissure Syndrome With Bilateral Cerebellar Signs and Holmes Tremor in a Patient With a Preexisting Movement Disorder

Wernekink Commissure Syndrome With Bilateral Cerebellar Signs and Holmes Tremor in a Patient With a Preexisting Movement Disorder

Sindrom Komisura Wernekink; Patofisiologi dan Diagnosa: Sebuah Tinjauan Literatur

Wernekink commissure syndrome: clinico-radiological criteria

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