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Table of Contents    
LETTER TO EDITOR
Year : 2016  |  Volume : 64  |  Issue : 7  |  Page : 113-114

A probable cavernoma in the medulla oblongata presenting as Opalski syndrome: A rare entity


Department of Neurology, MS Ramaiah Medical College and Hospital, Bengaluru, Karnataka, India

Date of Web Publication3-Mar-2016

Correspondence Address:
Rohan R Mahale
Department of Neurology, MS Ramaiah Medical College and Hospital, Bengaluru, Karnataka
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0028-3886.178051

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How to cite this article:
Mahale RR, Mehta A, Miryala A, Buddaraju K, Javali M, Srinivasa R. A probable cavernoma in the medulla oblongata presenting as Opalski syndrome: A rare entity. Neurol India 2016;64, Suppl S1:113-4

How to cite this URL:
Mahale RR, Mehta A, Miryala A, Buddaraju K, Javali M, Srinivasa R. A probable cavernoma in the medulla oblongata presenting as Opalski syndrome: A rare entity. Neurol India [serial online] 2016 [cited 2019 Aug 24];64, Suppl S1:113-4. Available from: http://www.neurologyindia.com/text.asp?2016/64/7/113/178051


Sir,

The classical presentation of lateral medullary syndrome (LMS) consists of crossed sensory deficits, specifically loss of pain and temperature sensation involving the trunk and extremities contralateral to the lesion along with ipsilateral facial numbness. Other features include vertigo, nystagmus, hoarseness, dysphagia, ipsilateral cerebellar signs, and Horner's syndrome. The occurrence of hemiplegia in a patient presenting with LMS suggests either an Opalski syndrome or a  Babinski-Nageotte syndrome More Details. In Babinski-Nageotte syndrome, there is contralateral hemiparesis because pyramidal tract is affected before decussation. [1] In Opalski syndrome, hemiplegia is ipsilateral due to the extension of the lesion caudally to involve the corticospinal fibers distal to the pyramidal decussation. [2] Opalski syndrome has been reported in association with a lateral medullary infarct with caudal extension due to vertebral artery occlusion. [3] The occurrence of Opalski syndrome in association with a medullary bleed has not been reported. Hereby, we report a male patient who presented with symptoms suggestive of right LMS with right hemiplegia, thus having Opalski syndrome. Brain magnetic resonance imaging (MRI) showed a well-circumscribed right dorsal medullary lesion in the right dorsolateral medulla with caudal extension suggestive of a probable cavernoma.

A 54-year old man presented to us with a history of giddiness, which was of rotatory type lasting for half an hour. It was associated with 2 episodes of projectile vomiting. One hour later, he noticed sensory symptoms in the form of shock-like sensations in the left half of the body sparing the face that lasted for 2 h. Four hours later, he developed weakness of the right upper limb and lower limb. He had difficulty in swallowing liquids with choking and nasal regurgitation. There was no facial weakness, headache, visual, or auditory symptoms. He was hypertensive and was on medications. There were no other comorbidities. His pulse rate was 92/min and blood pressure was 170/90 mmHg in supine position. He was conscious and oriented. Speech had a nasal quality. Right-sided Horner's syndrome with miosis was present. The fundus examination was normal. There was fine, horizontal, jerky, gaze-evoked nystagmus on looking towards the right side. There was no facial weakness, hearing abnormality, or tongue weakness. There was right palatal palsy. Motor examination showed hypotonia with weakness (Medical Research Council 1/5) in the right upper and lower limbs. The planter response was extensor on the right and flexor on the left side. There was hypoesthesia over the left half of the body to pain and temperature sensation. The brain MRI showed a well-circumscribed right dorsal medullary lesion which was hyperintense on fluid-attenuated inversion recovery and blooming on T2 gradient echo suggesting the existence of a probable cavernoma with bleed [Figure 1], [Figure 2] and [Figure 3]. The magnetic resonance angiography was normal. He was treated with dexamethasone and anti-hypertensive medication. His weakness improved, but sensory abnormalities remained.
Figure 1: Brain magnetic resonance imaging fluid-attenuated inversion recovery (a) and (b) axial view showing a well-circumscribed hyperintense lesion with an isointense nucleus in the dorsal medulla oblongata with caudal extension (white arrows)

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Figure 2: Brain magnetic resonance imaging susceptibility-weighted imaging (a) and (b) axial view showing susceptibility hemosiderin artifacts (white arrows)

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Figure 3: Brain magnetic resonance imaging T2* gradient echo (a) and (b) axial view showing blooming in the dorsal medulla oblongata suggestive of bleed (white arrows)

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Our patient had acute onset vertigo, right-sided Horner's syndrome, right palatal palsy and left hemibody hypoesthesia to pain and temperature suggestive of right LMS. He also had right-sided limb weakness with extensor plantar response suggesting the presence of corticospinal dysfunction after decussation. Ipsilateral hemiplegia along with LMS indicated the presence of Opalski syndrome.

In 1949, Opalski described the findings of two patients with lateral medullary infarcts who had mild hemiparesis with hyperreflexia and Babinski's sign, along with features of LMS. Thus, patients with LMS who have ipsilateral hemiparesis, hyperreflexia, and Babinski's sign are stated to have Opalski syndrome. [2] Following are the postulated mechanisms: First, the involvement of corticospinal fibers caudal to pyramidal decussation due to the extension of the ischemia from the lateral medulla to the upper cervical cord may explain ipsilateral hemiparesis. Second, it may occur due to the involvement of medullary penetrating arteries arising from anterior spinal artery or distal vertebral artery that supply the pyramidal fibers distal to the decussation. [4] Third, the involvement of pyramidal fibers may be due to regional perfusion failure in the border-zone area between the anterior and posterior spinal arteries due to vertebral artery occlusion or stenosis. [3]

Opalski syndrome has been described in association with infarction in the lateral medulla with caudal extension due to vertebral artery atherothrombosis or dissection. Dembo and Tanahashi reported a patient with Opalski syndrome due to compression of the lateral surface of the medulla oblongata by vertebral artery. [5] The association of medullary cavernoma with bleed and Opalski syndrome has not been reported so far.

Brainstem cavernomas typically present with headache, vertigo, and cranial nerve deficits. They account for 9-35% of all cavernomas. [6] Histopathologically, cavernomas are composed of multiple thin-walled vascular channels devoid of smooth muscle, surrounded by fibrous tissue, with hemosiderin deposits around the walls and small low-flow feeding arteries and draining veins. There is no brain tissue intervening between these vascular channels. [7] Medullary cavernous angiomas usually present with headache, diplopia, truncal ataxia, paresis of facial nerve, weakness of arms and/or legs, dysphagia, numbness of the face or body, dizziness, nausea and vomiting, and hiccups. [8] Cavernomas appear as well-circumscribed hyperintense lesions with an isointense core on axial T2-weighted MRI and with hemosiderin susceptibility artifacts on susceptibility-weighted imaging.

Opalski syndrome is rare and has not been reported earlier as a presenting manifestation in a medullary cavernoma. The patient in this study had a right dorsal medullary cavernoma and clinical manifestations suggestive of Opalski syndrome.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

 
  References Top

1.
Babinski J, Nageotte J. Hemiasynergine, lateropulsion et myosis bulbaires avec hemianesthesia et hemiplegia croisees. Rev Neurol (Paris) 1902;10:358-65.  Back to cited text no. 1
    
2.
Opalski A. A new sub-bulbar syndrome: Partial syndrome of the posterior vertebro-spinal artery. Paris Med 1946;214-20.  Back to cited text no. 2
    
3.
Dhamoon SK, Iqbal J, Collins GH. Ipsilateral hemiplegia and the Wallenberg syndrome. Arch Neurol 1984;41:179-80.  Back to cited text no. 3
[PUBMED]    
4.
Liu CY, Chang FC, Hu HH, Hsu LC. Ipsilateral crural monoparesis in lateral medullary infarction due to vertebral artery dissection. Eur J Neurol 2006;13:e8-9.  Back to cited text no. 4
    
5.
Dembo T, Tanahashi N. Opalski syndrome caused by vertebral artery compression of the lateral surface of the medulla oblongata. Intern Med 2013;52:1115-20.  Back to cited text no. 5
    
6.
Moriarity JL, Clatterbuck RE, Rigamonti D. The natural history of cavernous malformations. Neurosurg Clin N Am 1999;10:411-7.  Back to cited text no. 6
    
7.
Jellinger K. Vascular malformations of the central nervous system: A morphological overview. Neurosurg Rev 1986;9:177-216.  Back to cited text no. 7
[PUBMED]    
8.
Kashiwagi S, van Loveren HR, Tew JM Jr, Wiot JG, Weil SM, Lukin RA. Diagnosis and treatment of vascular brain-stem malformations. J Neurosurg 1990;72:27-34.  Back to cited text no. 8
    


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