|Year : 2021 | Volume
| Issue : 2 | Page : 349-351
Facial palsy as an atypical presenting sign of cortical venous thrombosis
Kowsalya Akkayasamy, Anju Ajitha Jayaprakash, Jayasri Narayanamoorthy, Mahesh Kumar
Department of Neuro-Ophthalmology, Aravind Eye Hospital, Madurai, Tamil Nadu, India
|Date of Submission||18-Jun-2020|
|Date of Acceptance||23-Dec-2020|
|Date of Web Publication||01-Apr-2021|
Dr. Anju Ajitha Jayaprakash
Aravind Eye Hospital, Anna Nagar, Madurai, Tamil Nadu
Source of Support: None, Conflict of Interest: None
A 39-year-old woman presented with inability to close left eye for 1 day and headache for 1 week. Neurologist evaluation revealed left lower motor neuron facial palsy and was treated for migraine with facial palsy. On developing nausea and vomiting, fundus examination showed papilledema and magnetic resonance imaging (MRI) with magnetic resonance venogram (MRV) revealed cortical venous thrombosis. Blood investigations revealed microcytic hypochromic anemia. She recovered completely by 6 months after anticoagulant therapy, hematinics, and anti-edema medications. This rare presentation of facial palsy in CVT highlights the need for early fundus examination and imaging with MRV to prevent morbidity and mortality.
Keywords: Anticoagulation, cerebral venous sinus thrombosis, cranial nerve diseases, facial nerve palsy, magnetic resonance venography, papilledema, sinus thrombosis
|How to cite this article:|
Akkayasamy K, Jayaprakash AA, Narayanamoorthy J, Kumar M. Facial palsy as an atypical presenting sign of cortical venous thrombosis. Indian J Ophthalmol Case Rep 2021;1:349-51
|How to cite this URL:|
Akkayasamy K, Jayaprakash AA, Narayanamoorthy J, Kumar M. Facial palsy as an atypical presenting sign of cortical venous thrombosis. Indian J Ophthalmol Case Rep [serial online] 2021 [cited 2021 Apr 11];1:349-51. Available from: https://www.ijoreports.in/text.asp?2021/1/2/349/312345
Cortical venous sinus thrombosis (CVT) is the thrombosis of the superficial or deep venous system in the brain and presents with headache with nausea, vomiting, and altered consciousness. Although involvement of cranial nerves has been described in CVT in up to 12% of cases, initial presentation with facial palsy is extremely rare and little is known about its patho-physiology. To the best of our knowledge, only five previous reports exist of facial nerve palsy associated with CVT.,,, In this report, we describe one atypical case of CVT presenting with facial palsy.
| Case Report|| |
A 39-year-old woman presented to our Neuro-ophthalmology department with complaints of headache for 1 week, associated with nausea and five episodes of vomiting. She also gave history of inability to close the left eye and deviation of angle of mouth to the right for 2 days. She had consulted a neurologist earlier and was diagnosed and treated for migraine with left lower motor neuron (LMN) facial palsy.
General physical examination was within normal limits. Central nervous system examination revealed left LMN facial palsy [Figure 1]. Blood pressure was normal. Visual acuity in both eyes was 20/20. Anterior segment examination of right eye was normal, left eye showed lagophthalmos with poor Bell's phenomenon. Fundus examination revealed bilateral disc edema with hemorrhages.
|Figure 1: Clinical photo at presentation showing left facial palsy with deviation mouth to right (a) and left lagophthalmos (b)|
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Color vision was normal and central field's examination showed enlargement of blind spot bilaterally. Clinical diagnosis of papilledema with left LMN facial nerve palsy was made.
Magnetic resonance imaging (MRI) brain plain and contrast with whole spine screening and magnetic resonance venogram (MRV) revealed filling defect in superior, inferior, straight sinus, right transverse and sigmoid sinus and frontoparietal cortical veins suggestive of CVT [Figure 2].
|Figure 2: Magnetic resonance image of brain with MRV showing filling defects in superior, inferior, straight sinus, right transverse and sigmoid sinus and frontoparietal cortical veins suggestive of cortical venous thrombosis (red arrows) and optic nerve thickening (yellow arrow)|
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Serological investigations which showed leukocytosis (total count 21300), anemia (hemoglobin 7.8 g%), and low hematocrit (26%) and peripheral smear examination revealed microcytic hypochromic anemia. Renal parameters were normal.
A final diagnosis of CVT with left LMN facial palsy and papilledema was made and patient was referred to a neurologist. Cerebrospinal fluid (CSF) analysis was within normal limits and CSF opening pressure was 30 mm Hg. Treatment was initiated with parenteral followed by oral anticoagulants, hematinics, and anti-edema measures which included intravenous injection Heparin 5000 IU Q6H, Acenocoumarol (Acitrom, Abbott Pharmaceuticals) 3 mg OD dose, Amitriptyline (Tryptomer, Wockhardt Ltd) 12.5 mg OD dose, Clonezepam 0.5 mg + Escitalopram oxalate 5 mg (Nexito Plus, Sun Pharmaceutical Industries Ltd) OD dose, Folic acid 1.5 mg, Ferrous fumarate 152 mg (Livogen, Procter and Gamble) BD dose Esomeprazole 40 mg Domperidone 30 mg (Sompraz D, Sun Pharmaceutical Industries Ltd) 40 mg BD dose, Lorazepam (Ativan, Wyeth Pharmaceuticals, Pfizer Ltd) 2 mg OD and Acetazolamide (Diamox, Pfizer Ltd) 250 mg BD. Lagophthalmos was managed by lid taping at night, lubricants and physiotherapy was started for the facial nerve palsy.
On the first month of follow-up, the patient was symptomatically better, headache decreased but papilledema still persisted, patient was referred back to neurologist to increase the dose of Acetazolamide.
By sixth month of follow-up, headache and facial weakness had subsided and fundus examination revealed reduction in disc edema and resolution of hemorrhages suggestive of resolving papilledema [Figure 3].
|Figure 3: Fundus images showing bilateral disc edema at presentation and resolution by 6 months|
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| Discussion|| |
CVT is defined as thrombosis of the superficial or deep venous system in the brain. It is a rare condition affecting 0.6–7/100,000 population and 1%–2% of cerebral infarctions. Etiologies include hypercoagulable states, head trauma, dehydration, oral contraceptives (OCP) use, neoplastic invasion of a venous sinus, vasculitis, systemic infections, congenital heart disease, anemia, pregnancy, and puerperium. The usual symptoms and signs include headache, nausea, vomiting, changes in mental status, focal weakness, seizures, and papilledema. Cranial nerve palsy has been reported in 12% of CVT cases with cranial nerves II, IV, and VI being the most commonly involved. Our literature search revealed five cases of CVT with facial palsy. [Table 1] Kuehnen et al. reported five cases of CVT with cranial nerve palsies of which two were associated with facial nerve palsy. They postulated that local stasis in the cranial nerve veins draining into the transverse sinus can cause temporary facial nerve dysfunction. Straub et al. in their case report of a 17-year-old woman with familial protein S deficiency on oral contraceptives has postulated the possible cause as transient neuropraxia in the intracranial segment of the nerve caused by elevated venous transmural pressure in the nerve's satellite vein leading to venous blood–brain barrier dysfunction in the intrinsic vascular system of the nerve. The transmural thrombosis of the distal transverse sinus, which involves the drainage site of the superior petrosal sinus and thereby affects the drainage territory of the lateral pontine vein (via the superior petrosal vein), could have raised the intraluminal pressure in the nerve's satellite vein. Impaired venous drainage of the nerve root's satellite vein is transmitted to the intrinsic vascular system of the nerve. Electromagnetic stimulation studies ruled out a coincidental facial nerve palsy and showed the conduction block was proximal to the facial canal. Kartal et al. had also a reported a case of a 17-year-old girl on OCP for 2 months, who presented with right facial palsy and left hemiplegia due to CVT. In our patient, the possible etiology of the CVT could be due to anemia similar to the case reported by Kulkarni et al. The involvement of the transverse sinus seems to be a common risk factor for facial nerve involvement in CVT which was also present in our patient. The other cases had a particular causative factor for CVT-OCP intake/Protein S deficiency, etc., whereas in ours it was due to anemia. The initial misdiagnosis as migraine with facial palsy leading to delayed treatment probably explains the delay in recovery in facial palsy compared to the other patients previously reported.
|Table 1: Review of literature of five cases of cortical venous thrombosis with facial palsy|
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| Conclusion|| |
Cortical vein thrombosis is a potentially life-threatening condition. Association with facial nerve palsy is extremely rare and a high degree of suspicion is needed especially when associated with headache and papilledema. MRI brain with MRV is essential and prompt treatment with thrombolytics is crucial in preventing morbidity and mortality.
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.
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[Figure 1], [Figure 2], [Figure 3]