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Year : 2020  |  Volume : 68  |  Issue : 4  |  Page : 934-936

A Rare Variant of the Lateral Medullary Syndrome with Craniovertebral Junction and Vascular Anomalies

Department of Medicine, Jawaharlal Institute of Postgraduate Medical Education and Research, Puducherry, India

Date of Web Publication26-Aug-2020

Correspondence Address:
Dr. Srinath Ramaswamy
Department of Medicine, Jawaharlal Institute of Postgraduate Medical Education and Research, Puducherry
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0028-3886.293463

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How to cite this article:
Ramaswamy S, Surendran D, Bammigatti C, Palamalai SR. A Rare Variant of the Lateral Medullary Syndrome with Craniovertebral Junction and Vascular Anomalies. Neurol India 2020;68:934-6

How to cite this URL:
Ramaswamy S, Surendran D, Bammigatti C, Palamalai SR. A Rare Variant of the Lateral Medullary Syndrome with Craniovertebral Junction and Vascular Anomalies. Neurol India [serial online] 2020 [cited 2023 Feb 4];68:934-6. Available from: https://www.neurologyindia.com/text.asp?2020/68/4/934/293463

Dear Sir,

Lateral medullary syndrome (LMS) is an interesting neurological condition with excellent clinical teaching points. It commonly presents with loss of sensation on the ipsilateral face and contralateral body. Various other patterns of sensory involvement can occur.[1] Facial weakness is not uncommon but infranuclear palsy is notably rare.[2] We hereby present a case of LMS with the previously unreported combination of contralateral face and body sensory loss with ipsilateral infranuclear facial weakness. Interestingly, our patient with LMS also possessed craniovertebral (CV) junction anomalies. We noted that CV junction anomalies are reported to predispose to stroke and abnormalities of the posterior vasculature are commonly observed in these patients.[3],[4]

A 21-year-old male construction worker presented with acute onset, two-day history of numbness of the right face and body, deviation of his mouth to the right, slurring of speech, difficulty in swallowing, and nasal regurgitation of liquids. He had no weakness, imbalance, vision and hearing issues, vertigo, tinnitus, or bowel and bladder complaints. He denied no fever, trauma, fall, dog bite, or recent vaccinations. Antenatal, birth, family, and past histories were noncontributory.

Examination revealed a short neck (height-neck ratio of 15) with notable scoliosis but with a normal range of movements and posterior hairline. Neurological exam was positive for loss of touch and pain sensations on the right face, deviation of mouth to right, left-sided loss of forehead wrinkling and lagophthalmos, deviation of uvula to the right, impaired gag reflex, left-sided Horner syndrome, and loss of pain, temperature, and crude touch on the right side of the body. The lesion was localized based on the above cranial nerve and sensory involvement to the left medulla. Initial causes considered were stroke due to embolus and vertebral artery dissection, and demyelinating disorders, given young age and acute onset.

A non-contrast CT brain was reported normal. An MRI Brain was pursued, which revealed an acute infarct of the left upper medulla at the junction with the lower pons [Figure 1].
Figure 1: (a) Sagittal T1 hypodensity at left upper medulla. (b) Coronal T2 MRI hyperintensity at the same location with kinking of the brainstem, cervical spine scoliosis, and occipital assimilation of the atlas (arrow). (c) Axial FLAIR left medullary hyperintensity

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MR angiography was performed which revealed a myriad of vascular abnormalities. Hypoplastic A1 segment of the right anterior cerebral artery (ACA) was noted with flow across the anterior communicating artery (AcommA). Bilateral vertebral arteries (VA) were observed to be low calibre and hypoplastic from their origin. Bilateral fetal posterior cerebral arteries (PCA) with thinned P1 segments and prominent posterior communicating artery (PcommA) were seen. BA, posterior inferior cerebellar (PICA) and superior cerebellar artery (SCA) were all hypoplastic and poorly visualized [Figure 2]. Most interestingly, prominent carotid-to-vertebrobasilar anastomoses were identified in the form of a right hyperplastic anterior choroidal artery (AchA) and a left persistent trigeminal artery (PTA) connecting the internal carotid arteries (ICA), respectively to the basilar arteries (BA).
Figure 2: (a) Hypoplastic right A1 segment of ACA (arrow). (b) Bilaterally, VA not visualised from the origin. (c) Bilateral fetal PCA (asterisks) with poor BA, VA, PICA visualization. Carotid-vertebral anastomosis via - (d and e) Right Hyperplastic AchA (arrow) and (f) Left PTA (arrow)

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Interestingly, further evaluation of craniovertebral (CV) junction revealed basilar invagination and occipital assimilation of the atlas as defined by Chamberlain line measurement [Figure 3].[5]
Figure 3: Chamberlain line: Odontoid process of atlas seen to extend well above the line

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A final diagnosis of young stroke (atypical LMS) with CV junction anomaly and anomalous posterior circulation with carotid-vertebral anastomoses was made. The patient was started on aspirin and atorvastatin. Nasogastric tube feeding was required due to prominent dysphagia. Complete blood counts, lipid profile, urinalysis, renal and liver function tests, EKG, and echocardiogram were normal. Bubble contrast echo did not reveal intracardiac defects. The patient was observed in the hospital and showed significant improvement with minimal residual facial palsy, sensory deficit, and dysphagia. He was discharged on aspirin and statin and is currently deficit free on follow up except for mild sensory impairment of the right body and limbs, pending further neurosurgical evaluation.

To our awareness, there are no previously reported cases of LMS presenting with ipsilateral infranuclear palsy and contralateral face-body-limb sensory loss. This provides excellent neuroanatomical teaching points [Figure 4]. The contralateral pain and temperature loss on the face occur in lateral medullary infarcts that extend medially to involve the ventral trigeminothalamic tract (VTT) relaying sensations from the contralateral face.[6] Interestingly, painful trigeminal neuralgia is also noted to occur in LMS due to involvement of the central trigeminal pathways.[7] The ipsilateral loss of facial sensation in classical LMS is not observed here due to possible sparing of the spinal trigeminal tract dorsolateral to the VTT. Involvement of the adjacent ascending spinothalamic tract explains contralateral body-limb sensory loss observed here. The lack of cerebellar involvement, vertigo, and tinnitus in our patient is possibly explained by atypical sparing of further dorsolaterally situated structures namely cerebellar peduncle, vestibular nuclei, and spinocerebellar tracts. Central facial palsy in LMS is reported to occur due to involvement of corticobulbar fibers that loop in the medulla before reaching the pontine facial nucleus.[2] Infranuclear palsy is limited to a few case reports and is explained by the involvement of exiting facial nerve fascicles at caudal pons in infarcts extending to the pontomedullary junction.[8] In our patient, these findings are strongly corroborated by the location of the infarct on MRI.
Figure 4: Illustration of upper-medullary cross-section depicting probable infarct location and the tracts involved and spared

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Although posterior circulation stroke is seen in young patients with CV junction anomalies, stroke as the presenting feature of CV anomaly is rarely observed.[3],[9] In our patient, a workup for stroke showed basilar invagination, occipital assimilation of the atlas, and a mixture of vascular anomalies which probably occurred at the fetal stages due to unidentified causes. Angiographic studies reveal a high prevalence of vertebral artery insufficiency in patients with CV junction anomalies.[4] Our patient showed [Figure 2] bilateral VA hypoplasia with poor BA, PICA, and SCA flows. A fetal left PTA of Saltzmann type 2 variety was seen supplying the BA, characterised by hypoplastic P1 segment and P2 supply from PcommA (fetal PCA). The most common type 1 variant is associated with PCommA hypoplasia, which is not observed in our patient. The population incidence of PTA varies between 0.1

0.7%, and is associated with a higher risk for aneurysm, hemorrhage, embolism, and trigeminal neuralgia.[10] These complications were not observed. We hypothesize that thrombosis and local distal embolization due to compressive forces on the thinned-out VA by the bony anomalies at the CV junction could possibly be the mechanism of stroke in our patient.[11] The severity of CV anomaly and scoliosis pointed towards a high possibility of shear-stress on the posterior vasculature. It is, therefore, reasonable to stress the importance of close evaluation of the CV junction and vasculature in posterior circulation strokes.[4] Neurosurgical correction of the CV anomaly is hindered by the vulnerable vasculature in these cases. Elective surgery is planned in our patient after further angiographic assessment.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

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Conflicts of interest

There are no conflicts of interest.

 » References Top

Kim JS, Lee JH, Lee MC. Patterns of sensory dysfunction in lateral medullary infarction. Neurology 1997;49:1557-63.  Back to cited text no. 1
Kim JS, Lee JH, Suh DC, Lee MC. Spectrum of lateral medullary syndrome. Correlation between clinical findings and magnetic resonance imaging in 33 subjects. Stroke 1994;25:1405-10.  Back to cited text no. 2
Satishchandra P, Ramdas GV, Gaikwad SB. Cranio-vertebral junction anomalies with posterior circulation stroke. Neurocon 2001;23-32 Abstract book.  Back to cited text no. 3
Sivaraju L, Mani S, Prabhu K, Daniel RT, Chacko AG. Three-dimensional computed tomography angiographic study of the vertebral artery in patients with congenital craniovertebral junction anomalies. Eur Spine J 2017;26:1028-38.  Back to cited text no. 4
Smoker WR. Craniovertebral junction: Normal anatomy, craniometry, and congenital anomalies. Radiographics 1994;14:255-77.  Back to cited text no. 5
Zhang SQ, Liu MY, Wan B, Zheng H-M. Contralateral body half hypalgesia in a patient with lateral medullary infarction: Atypical Wallenberg syndrome. Eur Neurol 2008;59:211-5.  Back to cited text no. 6
John AA, Abbas MM, Javali M, Mahale R, Mehta A, Srinivasa R. A rare presentation of trigeminal neuralgia in lateral medullary syndrome. Neurol India 2017;65:638-40.  Back to cited text no. 7
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Park JH, Yoo HU, Shin HW. Peripheral type facial palsy in a patient with dorsolateral medullary infarction with infranuclear involvement of the caudal pons. J Stroke Cerebrovasc Dis 2008;17:263-5.  Back to cited text no. 8
Verma R, Sahu R, Ojha BK, Junewar V. Thalamic syndrome as the heralding manifestation of atlantoaxial dislocation. BMJ Case Rep 2013;bcr2012007712.  Back to cited text no. 9
Alcalá-Cerra G, Tubbs RS, Niño-Hernández LM. Anatomical features and clinical relevance of a persistent trigeminal artery. Surg Neurol Int 2012;3:111.  Back to cited text no. 10
Song SH, Roh HG, Kim HY, Choi JW, Moon W-J, Choe WJ, et al. Recurrent posterior circulation Infarction caused by anomalous occipital bony process in a young patient. BMC Neurol 2014;14:252.  Back to cited text no. 11


  [Figure 1], [Figure 2], [Figure 3], [Figure 4]

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