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Short Lasting Unilateral Neuralgiform Headache with Conjunctival Injection and Tearing as a Presenting Manifestation of Contralateral Cerebellopontine Angle Tumor
Correspondence Address: Source of Support: None, Conflict of Interest: None DOI: 10.4103/0028-3886.284366
Sir, Short-lasting unilateral neuralgiform headache with conjunctival injection and tearing (SUNCT) is a rare primary headache syndrome characterized by sudden brief attacks of severe unilateral headache in orbital, periorbital, or temporal regions, accompanied by ipsilateral cranial autonomic symptoms.[1] Although most cases of SUNCT are idiopathic, there are few reported cases in the literature that are secondary to intracranial lesions. These are mostly due to ipsilateral pituitary or posterior fossa lesions.[2] However, no case has been reported in the literature of SUNCT, occurring due to contralateral space occupying lesion. We report a 45-year-old female who presented with SUNCT and was subsequently found to have contralateral cerebellopontine angle tumor.
A 45-year-old female, presented with recurrent episodes of severe right-sided retroorbital pain for the past 1 month. She described the pain as sharp, lancinating, and lasting for around 30 sec to 1 minute. The pain would arise spontaneously and get relieved on its own. She denied any trigger, like touching or washing face. The pain was severe and was associated with intense redness and tearing of the right eye. She experienced around 5 to 6 episodes per day. On inquiry, she revealed hearing impairment on the left side that was present for more than 10 years. She denied any weakness, ataxia, drooping of eyelids, diplopia, or facial deviation. She had no difficulty in speech or deglutition. On examination, her mental functions were intact. All the cranial nerves were found normal on examination except the left-sided VIIIth cranial nerve. Gaze evoked nystagmus was present on the left side and mild incoordination was apparent on left-side finger-nose test. Gait was normal and the rest of the neurological examination were also within normal limits. MRI of brain revealed a large tumor in the left cerebellopontine angle [Figure 1] and [Figure 2] causing brainstem displacement.
The patient was treated with oxcarbamazepine (up to 600 mg twice daily) and lamotrigine (up to 100 mg twice daily) to which she reported decreased frequency of attacks. She was operated for the cerebellopontine angle tumor and the excision was done via retromastoid suboccipital retrosigmoid approach. The tumor was extra-axial, firm, moderately vascular with well-defined plane between tumor capsule and petrosal surface of cerebellar hemisphere. Tumor excision was done by bipolar cautery and CUSA. The facial nerve was identified and preserved intraoperatively and was displaced anteriorly. The origin of tumor was traced to internal acoustic meatus. Gross total excision was achieved and was confirmed as Schwannoma based on histopathological examinations. She has been in remission after surgical intervention.
Short-lasting unilateral neuralgiform headache with conjunctival injection and tearing (SUNCT) is a rare primary headache syndrome, that was first described in 1978.[1] The pathophysiology of these syndromes revolve around the trigeminal-autonomic reflex[3] Most cases of SUNCT are idiopathic, but there are a few cases reported in the literature that are secondary to intracranial lesions. These are either due to pituitary pathology or posterior fossa lesions.[2] There were two cases described of secondary SUNCT due to ipsilateral cerebellopontine AV malformations.[4],[5] Although data are limited, several studies have reported the presence on MRI of a vascular loop contacting or compressing the trigeminal nerve ipsilateral to the pain of Short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT)/short-lasting unilateral neuralgiform headache with cranial autonomic symptoms (SUNA). In a systematic literature review that identified 201 cases of SUNCT/SUNA evaluated by MRI, vascular compression of trigeminal nerve was evident in 34 patients.[6] Other cases of symptomatic SUNCT, secondary to a posterior fossa abnormality include the following: brainstem cavernous hemangioma, a posterior fossa lesion associated with HIV/AIDS, severe basilar impression causing pontomedullary compression in a patient with osteogenesis imperfecta, craniosynostosis resulting in shortened posterior fossa, and ischemic brainstem infarction.[3],[7],[8],[9],[10] There are case reports of trigeminal neuralgia with contralateral cerebellopontine tumor.[11],[12],[13],[14] Pathophysiology of these cases are controversial. There was displacement of brainstem in most cases causing contralateral nerve dysfunction. The trigeminal sensory root may be compressed between the brain stem and temporal bone or edge of tentorium. Distortion of the brainstem makes the sharp angulation entry of Vth nerve at Meckels' cave. Another hypothesis suggests the CPA tumors may cause rotation of the ventral surface of brainstem and the anteriorly directed trigeminal nerve may be stretched at its dural foramen. The activation of trigeminal autonomic reflex due to involvement of trigeminal nerve and facial parasympathetic outflow and irritation of the trigeminal root is responsible for the pain and other features of SUNCT syndrome.[15] The trigeminal autonomic reflex and central disinhibition play an important role in the pathophysiological mechanism of SUNCT syndrome. The modulation of posterior hypothalamus resulting in activation of trigeminal autonomic reflex is a key factor for producing SUNCT features.[16] In our patient, the SUNCT features were on the opposite site of tumor. We postulate that due to tumor, the brainstem structures have some degree of rotation to the opposite side, leading to activation of the trigeminal autonomic reflex on the other side with resultant SUNCT features. There are no reports of SUNCT occurring due to contralateral CP angle tumors. Therefore, our patient was unique in such that she presented with SUNCT and was subsequently found to have a cerebellopontine tumour on the contralateral side. She had a large left cerebellopontine angle tumor that was apparently causing displacement of the brainstem possibly leading to right trigeminal nerve dysfunction. Neurologists must be aware of this association of SUNCT with contralateral CP angle tumor. 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 Nil. Conflicts of interest There are no conflicts of interest.
[Figure 1], [Figure 2]
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