|Year : 2016 | Volume
| Issue : 2 | Page : 140-143
A stochastic variant of Wallenberg syndrome with ipsilateral central facial palsy
K Venugopal, DP Kushal, G Shyamala, MZ Mohammed, Shankar Naik, DP Santosh Kumar
Department of General Medicine, Vijayanagara Institute of Medical Sciences, Bellary, Karnataka, India
|Date of Web Publication||31-Aug-2016|
Department of General Medicine, Room No. 17, 17th Block, Vijayanagara Institute of Medical Sciences, Bellary - 583 104, Karnataka
Source of Support: None, Conflict of Interest: None
Lateral medullary syndrome or Wallenberg syndrome is an interesting clinical entity with varied presentations. Its clinical features include ipsilateral Hornerfs syndrome, ataxia, pain, numbness, decreased sensation over face, palate, pharynx, vocal cord palsy, dysphagia, dysarthria and contralateral decreased sensation of pain and temperature over trunk and extremities. However, variability in the presentation of this syndrome is the rule, as illustrated in this case presentation and literature review. Facial nerve involvement can occur in variant of lateral medullary syndrome with pontomedullary sulcus infarct. Its involvement in pure lateral medullary infarct is very unusual. We hereby report a case of a 55-year-old male with classical features of lateral medullary syndrome with ipsilateral central facial palsy. Magnetic resonance imaging brain showed dorsolateral medullary infarct. The facial nerve involvement can be explained by possible damage to ascending the hypothetical looping pathway of supranuclear cortico.facial fibers, which is dispersed in dorsolateral medulla.
Keywords: Central facial palsy, corticobulbar tract fibers, cortico facial tract, lateral medullary syndrome
|How to cite this article:|
Venugopal K, Kushal D P, Shyamala G, Mohammed M Z, Naik S, Santosh Kumar D P. A stochastic variant of Wallenberg syndrome with ipsilateral central facial palsy. J Mahatma Gandhi Inst Med Sci 2016;21:140-3
|How to cite this URL:|
Venugopal K, Kushal D P, Shyamala G, Mohammed M Z, Naik S, Santosh Kumar D P. A stochastic variant of Wallenberg syndrome with ipsilateral central facial palsy. J Mahatma Gandhi Inst Med Sci [serial online] 2016 [cited 2019 Dec 12];21:140-3. Available from: http://www.jmgims.co.in/text.asp?2016/21/2/140/189529
| Introduction|| |
The lateral medullary syndrome was first descried in 1808 by Gaspard Vieussux. First descriptions by Wallenberg was in 1895 (clinical) and 1901 (autopsy findings). It is most often secondary to intracranial vertebral artery (67%) or posterior inferior cerebellar artery (10%) occlusion. Spontaneous dissections of the vertebral arteries are a common cause. The syndrome has also been described with cocaine abuse, medullary neoplasms (usually metastases), abscess, and demyelinating disease.
Clinical features and probable involvement of structures are given in [Table 1].
The onset was sudden in 75% patients and gradual in 25% patients. The patterns included ipsilateral trigeminal ± contralateral limb/body pattern in 26%. Trigeminal involvement without limb/body involvement in 10%. The triad of Horner syndrome, ipsilateral ataxia, and contralateral hypalgesia clinically identifies patients with lateral medullary infarction. Along with these classical features, it presents with many rare manifestations. Ipsilateral central facial palsy (CFP) is one of the very unusual manifestations. The frequency of facial nerve involvement in lateral medullary syndrome is said to be 23.5% and 25.5%. Its occurrence is unusual. Only one case has been reported in India  to the best of our knowledge; hence we are here reporting a case of lateral medullary syndrome with unusual ipsilateral CFP.
| Case Report|| |
A 55-year-old male, smoker, nonhypertensive, nondiabetic, presented with a history of sudden onset ataxia with tendency to fall toward the left side, vomiting, dysphagia, nasal regurgitation and drooling of saliva from the mouth. He also complained of decreased sensation and pain over the left half of the face. On examination, his vitals were stable. His higher mental functions were normal except for dysarthria with nasal twang and difficulty to pronounce gutturals. He had partial ptosis and miosis of left eye, decreased pain and temperature over left half of face, absent direct corneal reflex, deviation of angle of mouth to right side [Figure 1], loss of nasolabial fold on left side, with preserved forehead wrinkles and without bell's phenomenon. Uvula was central and normal position of the soft palate during quite breathing [Figure 2]. On phonation, uvula deviated to the right, drooping of left soft palate [Figure 3], absent gag reflex. Motor system was normal. No sensory disturbances over limb and extremities. No bowel and bladder involvement. Patient was able to stand with a broad base with tendency to fall to the left side; other cerebellar tests were normal. Probable diagnosis of lateral medullary syndrome was made based on history and examination. Magnetic resonance imaging (MRI) brain showed infarction in dorsolateral medulla in posterior inferior cerebellar artery territory [Figure 4]. Laboratory and other investigations done are given in [Table 2]. Patient treated with mannitol, aspirin, and atorvastatin. Nasogastric tube has been put to avoid aspiration. Patient improved with treatment and discharged with physiotherapy advice for facial palsy.
|Figure 3: On phonation, deviation of uvula to right, drooping of left soft palate|
Click here to view
|Figure 4: Magnetic resonance imaging brain showing left dorsolateral medullary infarct|
Click here to view
| Discussion|| |
The facial nucleus in humans is located dorsolaterally in the caudal pons. The corticobulbar tract (CBT) fibers that connect the motor cortex with the facial nucleus provide strongly unilateral innervation to the contralateral lower facial muscles and bilateral innervation to the upper facial muscles. Classic symptom localization has postulated that lesions rostral to the upper mid-pons result in contralateral facial paresis of central type, whereas ipsilateral facial paresis of peripheral type peripheral facial palsy (PFP) ensues from lesions involving the inferolateral part of the pons. The presence of facial palsy is very unusual or atypical in otherwise typical case of lateral medullary syndrome. Ipsilateral PFP may be due to involvement of the facial nucleus when lateral medullary syndrome extends rostrally. Ipsilateral CFP may be explained in pure lateral medulla infarct by damage to hypothetical looping supranuclear CBT fibers which have been hypothesized to descend down in contralateral ventromedial medulla, decussate at the level of upper medulla and ascend dorsolaterally to reach facial nucleus [Figure 5].,
|Figure 5: Schematic diagram of brainstem showing pathway of facial fibers and site of lesion with types of facial involvement. (A) Produces contralateral central facial palsy (CFP) (B) nuclear lesion; ipsilateral peripheral facial palsy, (C) hypothetical loop before decussation; contralateral CFP (D) hypothetical loop in dorsolateral medulla; ipsilateral CFP – (1) Corticospinal tract, (2) corticobulbar tract (cortico facial fibers), (3) bilateral innervation to upper face, (4) hypothetical looping of supranuclear corticobulbar tract fibers|
Click here to view
Currier  has hypothesized that facial CBT fibers leave the pyramidal tract at the pontomedullary junction and descend caudally to at least the middle medullary levels before most of them cross to the opposite facial nucleus. Interruption of these fibers by infarction at a predecussation level has been postulated to result in a contralateral CFP. Cavazos et al.
Have described some fibers of the facial CBT as descending ipsilaterally, making a loop as caudally as the upper medulla before decussating and ascending to the contralateral facial nucleus. Kim et al. have also reported that, in patients with lateral medullary infarction,
CFP was seen more often with high than middle to low medullary lesions.
Since MRI of our patient localizes the infarct in dorsolateral medulla, CFP in our patient could be due to interruption of this ascending limb of hypothetical pathway.
Clinical recognition of patients with lateral medullary infarction is of particular importance due to its association with vertebral artery dissection in 15–26% of cases  and the favorable prognosis associated with optimal management.
| Conclusions|| |
Various studies conducted outside India have shown varied frequency of facial nerve involvement in lateral medullary syndrome. Very few cases of lateral medullary syndrome with facial palsy have been reported in India. Whether it is because of underreporting, under recognition or it is a rare occurrence is unclear.
This case report emphasizes the fact that the possibility of involvement of facial nerve in lateral medullary syndrome is not an impossible occurrence and thus one should not exclude the diagnosis of lateral medullary syndrome if the facial nerve lesion is present, for the clear fact that the medulla is the most important part of the brain that houses many vital centers, which are crucial for the survival of human body. Hence, if diagnosed earlier and promptly treated, mortality and morbidity can be reduced.
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.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
| References|| |
Kim H, Chung CS, Lee KH, Robbins J. Aspiration subsequent to a pure medullary infarction: Lesion sites, clinical variables, and outcome. Arch Neurol 2000;57:478-83.
Wallenberg A. Acute bulbar palsy. Posterior inferior cerebellar artery embolism. Arch Psychiatry 1895;27:504-40.
Kim JS. Pure lateral medullary infarction: Clinical-radiological correlation of 130 acute, consecutive patients. Brain 2003;126:1864-72.
Mody CK, Miller BL, McIntyre HB, Cobb SK, Goldberg MA. Neurologic complications of cocaine abuse. Neurology 1988;38:1189-93.
Smith DB, Demasters BK. Demyelinating disease presenting as Wallenberg's syndrome. Report of a patient. Stroke 1981;12:877-8.
Sacco RL, Freddo L, Bello JA, Odel JG, Onesti ST, Mohr JP. Wallenberg's lateral medullary syndrome. Clinical-magnetic resonance imaging correlations. Arch Neurol 1993;50:609-14.
Kim JS, Lee JH, Choi CG. Patterns of lateral medullary infarction: Vascular lesion-magnetic resonance imaging correlation of 34 cases. Stroke 1998;29:645-52.
Norrving B, Cronqvist S. Lateral medullary infarction: Prognosis in an unselected series. Neurology 1991;41:244-8.
Srinivasan M, Bindu B, Gobinathan S, Balasubramanian S, Nityanandan A, Shanbhogue KR. An unusual presentation of lateral medullary syndrome with ipsilateral UMN facial palsy- An anatomical postulate. Ann Indian Acad Neurol 2005;8:37-40.
Patten J, editor. The brain stem. In: Neurological Differential Diagnosis. 2nd
ed. London: Springer; 1996. p. 162-77.
Caplan LR. Caplan's Stroke: A Clinical Approach. 3rd
ed. Saunders, released date 24 june 2000. p. 207-8.
Terao S, Takatsu S, Izumi M, Takagi J, Mitsuma T, Takahashi A, et al.
Central facial weakness due to medial medullary infarction: The course of facial corticobulbar fibres. J Neurol Neurosurg Psychiatry 1997;63:391-3.
Urban PP, Wicht S, Vucorevic G, Fitzek S, Marx J, Thömke F, et al.
The course of corticofacial projections in the human brainstem. Brain 2001;124:1866-76.
Currier RD. The medial medullary syndrome. J Univ Mich Med Cent 1976;42:96-104.
Cavazos JE, Bulsara K, Caress J, Osumi A, Glass JP. Pure motor hemiplegia including the face induced by an infarct of the medullary pyramid. Clin Neurol Neurosurg 1996;98:21-3.
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.
Mokri B, Houser OW, Sandok BA, Piepgras DG. Spontaneous dissections of the vertebral arteries. Neurology 1988;38:880-5.
[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]
[Table 1], [Table 2]