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|LETTER TO EDITOR
|Year : 2017 | Volume
| Issue : 3 | Page : 638-640
A rare presentation of trigeminal neuralgia in lateral medullary syndrome
Aju A John, Masoom M Abbas, Mahendra Javali, Rohan Mahale, Anish Mehta, R Srinivasa
Department of Neurology, M.S. Ramaiah Institute of Neurosciences, M.S. Ramaiah Medical College, Bengaluru, Karnataka, India
|Date of Web Publication||9-May-2017|
Aju A John
Department of Neurology, M.S. Ramaiah Medical College, New BEL Road, Bengaluru, Karnataka
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
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
|How to cite this URL:|
John AA, Abbas MM, Javali M, Mahale R, Mehta A, Srinivasa R. A rare presentation of trigeminal neuralgia in lateral medullary syndrome. Neurol India [serial online] 2017 [cited 2020 May 25];65:638-40. Available from: http://www.neurologyindia.com/text.asp?2017/65/3/638/205900
According to the International Classification of Headache Disorders – 3 (ICHD-3), painful trigeminal neuropathy (PTN) is commonly caused by vascular compression on the trigeminal nerve; however, it may also be caused by a structural lesion or a plaque associated with multiple sclerosis. Ipsilateral facial pain resulting from spinal tract involvement is often seen in lateral medullary syndrome (LMS). Facial pain can be intermittent, with brief attacks, which is very similar to PTN. However, a painful trigeminal neuropathy is distinctly rare as per our literature review.
A 73-year-old lady presented with a history of acute-onset giddiness, vomiting, clumsiness in the left hand and leg, gait unsteadiness, and numbness on the right half of her body. She was a diabetic and hypertensive for the last 10 years and was on regular treatment for the conditions. On neurological examination, she was conscious, oriented, and her higher mental functions were normal. Cranial nerve examination showed a left-sided horizontal gaze evoked nystagmus, left-sided Horner's syndrome, and cerebellar dysarthria; there was no bulbar involvement. Her motor system examination was normal. Sensory examination revealed impaired sensation to touch and pain on the left side of the face and right half of the body, impaired joint position and vibration sense in the left upper and lower limbs, and cerebellar signs on the left side. The clinical findings were suggestive of a left LMS. Magnetic resonance imaging (MRI) of the brain showed an acute infarct in the left cerebellum and left lateral medulla [Figure 1]. MR angiogram of the brain and neck vessels showed an absent left vertebral artery, a diseased basilar artery, but with no evidence of dissection [Figure 2]. There was no evidence of a vascular contact at the root entry zone of the trigeminal nerve. Carotid and vertebral artery Doppler showed the absent flow in the left vertebral artery, which was otherwise normal. On day 4 of the hospitalization, she developed brief paroxysms of pain on the left lower half of the face, which were sharp electric shock-like, lasting for 2–3 seconds. The pain was precipitated by touch, movement of jaw and chewing. She even found it difficult to sleep at night as a blow of air by the fan precipitated the paroxysms. Trigger points, as reported by the patient, were on the left cheek in front of the ear, and there were no autonomic symptoms. Initially, there were only few episodes of the pain per day; however, the frequency of the episodes increased by the fifth day, with at least 3 attacks occurring per hour, with a pain intensity of 8 out of 10 on the visual analogue pain intensity scale. She was pain-free between the paroxysms. The paroxysmal episodes fulfilled the diagnostic criteria for PTN, attributable to other causes according to ICHD-3. Repeat imaging with MRI brain did not reveal any new infarction.
|Figure 1: Diffusion-weighted images (a and c) and apparent diffusion coefficient (b and d) images showing diffusion restriction and ADC reversal in the left cerebellum and lateral medulla|
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|Figure 2: MR angiogram of the brain showing absent left vertebral artery and diseased basilar artery|
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The patient was treated with carbamazepine that was titrated to 600 mg per day in divided doses over the next one week. She showed a significant decrease in the frequency and intensity of pain with a pain scale score of 2 out of 10, and its frequency was reduced to 1–2 episodes per day at the time of discharge. She was symptom-free following 2 weeks of administration of medication and at a 6-month follow up, there were no further episodes of the pain.
The first documented case of TN in brainstem infarction was published by Balestrino and Leandri in 1997, who reported a 58-year old man with pain in the second division of trigeminal nerve with an ischemic lesion at the trigeminal root entry zone. Ordas et al., also reported a case of symptomatic trigeminal neuralgia in a 41-year old man with a recent onset infarction in the right lateral medulla because of occlusion of the right vertebral artery secondary to dissection. He also described the other cases reported by Golby et al., and Warren et al.
Facial pain may be a feature in some cases of LMS. The episodes of facial pain may be sharp, single stabs, or jolts of pain felt around the eye, which are very similar to that seen in TN; however, pain with the typical characteristics of TN has rarely been reported. Classical TN is usually attributed to neurovascular compression by a blood vessel, with demyelinating plaques associated with multiple sclerosis, space occupying lesions in the posterior fossa, or herpes zoster forming the differential diagnoses.
The pathogenesis of TN in the presence of brainstem infarction may be the demyelization in the central trigeminal pathways that causes abnormal electrical impulses to develop similar to that observed in multiple sclerosis. An alternative hypothesis postulates that irritation of the trigeminal structures due to the presence of a scar could cause excessive reactivity in an epileptic manner.
Treatment guidelines for PTN are still unclear and most studies have been reported in patients with multiple sclerosis. Our patient responded to carbamazepine and was symptom-free at 2 weeks with no further episodes observed on a 6-month follow up. Although there are no guidelines in an acute setting of stroke, carbamazepine may be an effective treatment even for poststroke TN.
I thank Dr. Abhinandan KS, Dr. Kiran Buddaraju, Dr. Loveline Singh and Dr. Abhishek for their contribution and help in the preparation of this manuscript.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2]