|Year : 2015 | Volume
| Issue : 4 | Page : 156-159
Internuclear Ophthalmoplegia in Pontomesencephalic Stroke as the First Manifestation of Takayasu Arteritis
Chetana Sen, Krishnarpan Chatterjee
Department of Medicine, ESI Medical College, Joka, Kolkata, West Bengal, India
|Date of Web Publication||13-Apr-2016|
Department of Medicine, ESI Medical College, Joka, Kolkata, West Bengal
Source of Support: None, Conflict of Interest: None
Takayasu arteritis (TA) is a chronic inflammatory disease affecting the aorta and its main branches. Patients generally present with symptoms such as fever, myalgia, limb claudication, carotid vessel tenderness, hypertension, and asymmetric pulses. Neurological manifestations are present in a small percentage of patients, with stroke occurring in about 10% of cases. Stroke, occurring as the very first presentation of the disease, is however unusual. We present an atypical case of unilateral internuclear ophthalmoplegia (INO) due to acute pontomesencephalic stroke in the absence of cerebral angiographic abnormality, in a 36-year-old woman as the initial presenting feature of TA. The patient had angiographic evidence of bilateral subclavian artery stenosis but no abnormality in the cerebral vessels, thus pointing toward a possible embolic origin from inflamed vessels. Though other unusual neurological manifestations have been reported, INO as the first manifestation has not been found in medical literature so far.
Keywords: Arteritis, internuclear ophthalmoplegia, stroke, Takayasu
|How to cite this article:|
Sen C, Chatterjee K. Internuclear Ophthalmoplegia in Pontomesencephalic Stroke as the First Manifestation of Takayasu Arteritis. Indian J Vasc Endovasc Surg 2015;2:156-9
|How to cite this URL:|
Sen C, Chatterjee K. Internuclear Ophthalmoplegia in Pontomesencephalic Stroke as the First Manifestation of Takayasu Arteritis. Indian J Vasc Endovasc Surg [serial online] 2015 [cited 2019 Dec 8];2:156-9. Available from: http://www.indjvascsurg.org/text.asp?2015/2/4/156/180124
| Introduction|| |
Takayasu arteritis (TA), also known as pulseless disease, is a chronic inflammatory arteritis affecting large vessels, most markedly, the aorta and its main branches. Vessel inflammation leads to wall thickening, fibrosis, stenosis, thrombi, and aneurysm formation. Strokes may occur in 10% of the patients. 
We present an unusual case of unilateral internuclear ophthalmoplegia (INO) due to pontomesencephalic stroke in the absence of cerebral angiographic abnormality, in a young female as the initial presentation of TA. Though other unusual neurological manifestations have been reported, INO as the first manifestation has not been found in medical literature so far.
| Case Report|| |
A 36-year-old homemaker from West Bengal presented with a history of an acute onset diplopia, more on looking to the left with a slurring of speech. There was no history of headache or loss of consciousness or seizures, with no history of facial deviation or limb weakness. She was not on any medications and was not a known case of hypertension or diabetes.
Physical examination revealed a middle aged woman with normal sensorium, with a right-sided INO with upbeat nystagmus in all directions of gaze. Optic fundi and pupil responses were normal bilaterally. She had a dysarthria with no evidence of bulbar palsy. There was no other cranial nerve palsy. No motor or sensory deficit or cerebellar signs were present, autonomic function was normal with no bowel or bladder abnormality. She had feeble upper limb pulses although the other peripheral pulses including the carotids and the lower limb pulses were felt normally. Blood pressure was 120/80 in right and 136/90 in left upper limb and 150/90 in both lower limbs. Cardiovascular examination was within normal limits with no bruit over major arteries. No other systemic abnormality was found.
On retrospective history, she gave no history of fever, malaise, limb claudication, or amaurosis. The patient had no history of tuberculosis in the past.
Investigations revealed an acute right-sided pontomesencephalic hypodensity on plain computed tomography brain [Figure 1]. Magnetic resonance imaging brain showed multiple hypointensities on T1-weighted images in bilateral paraventricular regions, right-sided mid brain, and pons with hyperintensities on T2-weighted images in the same areas. Blood parameters revealed a normal hemogram with an erythrocyte sedimentation rate of 29 mm, with normal renal and hepatic profile and normal lipid profile. Her prothrombin time and activated partial thromboplastin time were normal. Antinuclear antibody was negative. Her echocardiography was unremarkable. A digital subtraction angiography of aorta and cerebral arteries revealed a total cut-off of left subclavian artery just distal to the origin of the vertebral artery for a distance of 19 mm, with extensive collaterals with beaded appearance [Figure 2]. The right subclavian artery showed multiple areas of narrowing and stenosis, extending up to the axillary artery with multiple collaterals with beaded appearance [Figure 3]. The common carotids, internal carotids, vertebral arteries and basilar arteries, and their continuation into the cerebral circulation showed no thrombus or stenosis [Figure 4] and [Figure 5]. The arch of the aorta and descending thoracic aorta was normal. Ultrasonography color Doppler of the renal arteries was unremarkable. Mantoux test was negative.
|Figure 1: Noncontrast computed tomography brain showing right-sided hypodensity in paramedian midbrain and pons|
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|Figure 2: Digital subtraction angiography showing left-sided subclavian artery with stenosis just distal to vertebral artery origin, foe a distance of 19 mm, with collaterals showing beaded appearance|
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|Figure 3: Digital subtraction angiography showing right subclavian artery showing stenosis with stenosis of axillary artery with collaterals showing beaded appearance|
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|Figure 4: Digital subtraction angiography of cerebral vessels showing normal right vertebral artery, basilar, and their branches|
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|Figure 5: Digital subtraction angiography of cerebral vessels showing normal left vertebral artery, basilar, and their branches|
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According to the revised Ishikawa diagnostic criteria and the angiographic classification of TA as stated in the Takayasu Conference 1994, the diagnosis of TA Type I was made. ,
The patient was subsequently started on high-dose oral steroid, prednisolone 40 mg, to be tapered slowly. Her upbeat nystagmus disappeared soon after and was clinically stable, when she was discharged from the hospital and advised for regular follow-ups.
| Discussion|| |
TA is an idiopathic chronic inflammatory disease affecting large vessels, predominantly, the aorta and its branches. A triphasic pattern of progression of the disease has been described.  Phase I or the inflammatory period is characterized by nonspecific symptoms of fever, myalgia, and weight loss. Phase II is manifested as blood vessel pain and tenderness due to vessel inflammation. Phase III or the final phase is the burnt-out or fibrotic stage, in which bruits and ischemia may occur in multiple organs. Neurological symptoms in TA may develop in Phases II and III, depending on the degree of involvement of the branches of the aortic arch.  Transient ischemic attacks or stroke may occur in TA due to either steno-occlusion or embolus from the inflamed region of the vessel. 
TA is a rare disease, but most commonly seen in Japan, South East Asia, India, and Mexico. Neurological manifestations are seen in a small percentage of patients of TA, with stroke occurring in about 10% of cases.  Neurological manifestation as the very first and sole manifestation, as in our case has however been reported rarely. ,,, Isolated INO with supranuclear dysarthria as a presenting feature of TA has not been reported so far. Our patient had a right-sided INO with an upbeat nystagmus on presentation. The upbeat nystagmus disappeared after a few days once the acute phase had subsided. She also has supranuclear dysarthria, with no evidence of any other cranial nerve palsy. The lesion was localized to the right-sided pontomesencephalic region. The clue to her diagnosis was in the asymmetry of her peripheral pulses. Among Indian patients, about 15-50% of the patients present with nonspecific symptoms such as fever, malaise, myalgia, weight loss, and arthralgia.  Thirty percentage of the patients have anemia and 10-30% have various cardiac symptoms.  Our patient had none of these common manifestations. Other unusual neurological findings reported in literature include Horner's syndrome, posterior reversible encephalopathy syndrome, recurrent epileptic seizures, spinal cord compression, and optic neuritis simulating multiple sclerosis. ,,,,
In a study done by Kumral et al., transcranial Doppler ultrasound over middle cerebral artery was done to detect microembolic signals, present in one-fourth of patients with infarction, suggesting an embolic origin.  Conventional angiography and ultrasound can only comment on luminal morphology but gives no information on the inflammatory status of the vessel wall. Thus, the finding of a patent vessel does not exclude the presence of early inflammation and active disease.  Our patient's cerebral angiography showed no narrowing or stenosis of intracranial vessels, with narrowing of subclavian vessels, thus pointing toward a possibility of an embolic stroke. 
Finally, it is to be noted that in our patient, the diagnosis of TA was made, following the vital clue lying in the simple clinical finding of decreased pulses in the upper limbs, with asymmetric blood pressure, which was detected only after she presented to us with diplopia and dysarthria, thus reiterating the utmost importance of the simple clinical examination of peripheral pulses in a young patient with acute neurological event. Although neurological features do occur in patients with TA, the rarity of the disease and its unusual initial presentation with acute neurological syndrome can cause further delay in diagnosis. Therefore, the possibility of TA, even in the absence of the classical features, should also be kept in mind while evaluating a stroke in young.
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[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]