D Y Patil Journal of Health Sciences

CASE REPORTS
Year
: 2021  |  Volume : 9  |  Issue : 1  |  Page : 33--35

Bilateral facial palsy in cerebral venous sinus thrombosis – An uncommon association


Ajit Prasad Mishra1, AK Mallick2, SD Nayak2, LK Sahoo1,  
1 Department of Neurology, IMS and SUM Hospital, Bhubaneswar, India
2 Department of Neurology, SCB Medical College, Cuttack, Odisha, India

Correspondence Address:
Ajit Prasad Mishra
IMS and SUM Hospital, Bhubaneswar, Odisha.
India

Abstract

Background: Cranial nerve palsy in cerebral venous sinus thrombosis (CVST) is not a very common presentation, its pathophysiology is not well understood. Case Description: We report a 27 year old young man with CVST having protein-S deficiency & hyperhomocystinemia, who during his initial days of hospitalization developed bilateral facial palsy. With conservative treatment of CVST, complete recovery of facial palsy occurred with recanalization of transverse sinus. Conclusions: Facial palsy in this patient was probably caused by transient neurapraxia in the intracranial segment of the nerve. It may be hypothesised that elevated venous transmural pressure in the nerve’s satellite vein, which belongs to the affected drainage territory of the transverse sinus, might have caused venous blood-brain barrier dysfunction in the intrinsic vascular system of the nerve, with leakage of fluids and ions into the endoneurial space and thus an increase in interstitial resistance causing conduction defects.



How to cite this article:
Mishra AP, Mallick A K, Nayak S D, Sahoo L K. Bilateral facial palsy in cerebral venous sinus thrombosis – An uncommon association.D Y Patil J Health Sci 2021;9:33-35


How to cite this URL:
Mishra AP, Mallick A K, Nayak S D, Sahoo L K. Bilateral facial palsy in cerebral venous sinus thrombosis – An uncommon association. D Y Patil J Health Sci [serial online] 2021 [cited 2022 Jan 23 ];9:33-35
Available from: http://www.dypatiljhs.com/text.asp?2021/9/1/33/331105


Full Text



 Introduction



Cerebral venous sinus thrombosis (CVST) is a comparatively uncommon disorder and predominantly affects the young population inflicting significant morbidity. However, the clinical manifestation is variable and a wide spectrum of neurological symptoms has been delineated with it.[1],[2],[3],[4],[5] Cranial nerve palsy in CVST is rare and is often thought to result from cavernous sinus occlusion, elevated intracranial pressure (ICP),[2],[4],[5],[6],[7],[8] or attributed to the extension of thrombosis into contiguous venous tributaries,[2] presumptively resulting in direct pressure impact on the nerves in proximity to the clot. Kuehnen et al. have delineated cranial nerve syndromes in thrombosis of the transverse and sigmoid sinus and suggested that local stasis within the cranial nerve veins draining into the transverse sinus may cause temporary nerve dysfunction.[9] This case report of isolated bilateral facial palsy in CVST associated with protein S deficiency and hyperhomocysteinemia refines their hypothesis of the pathologic process of facial palsy in CVST.

 Case Report



We describe a 27-year–old man whose routine workup included daily exercise and running for fitness. One morning upon waking up from sleep, he developed a headache, which was holocranial and continuous. This progressively increased in intensity over the next 2–3 days. It was associated with constant head reeling and multiple episodes of vomiting. Although initially normal, gradually he became a little drowsy. The illness was not associated with fever, rash, tinnitus, ear discharge, hearing impairment, photophobia, phonophobia, convulsion, weakness, unsteadiness, swallowing difficulty, or change in a quality speech before admission to our department. He had no history of any chronic disease, but he used to have intermittent holocranial headache for the last few months. He was treated outside symptomatically for a brief period and was evaluated with routine blood tests such as complete blood count, liver function test, urea creatinine, and magnetic resonance imaging (MRI) of the brain, before admission to our side. All reports were within the normal limits.

Our clinical examination revealed normal neurological status except little drowsiness (Glasgow Coma Scale OF 14/15) which improved the next day and the presence of bilateral papilledema. But on the 2nd day of admission to our side, after waking up in the morning, the patient complained of difficulty in brushing teeth and spitting. He also complained of difficulty in eye closure and decreased taste sensation. Clinical examination revealed bilateral lower motor neuron type of facial palsy without any other new signs and symptoms.

On evaluation magnetic resonance venography (MRV) of the brain revealed diffuse sinus thrombosis involving the superior sagittal sinus, torcula, bilateral transverse sinus, and right sigmoid sinus with developed collaterals.

We evaluated for the common cause of bilateral facial palsy with lumbar puncture revealing a high cerebrospinal fluid (CSF) opening pressure of 360 mm of water with other CSF parameters within the normal range. CSF cartridge-based nucleic acid amplification techniques were negative for tuberculosis. Erythrocyte sedimentation rate and chest X-ray were normal. The serum angiotensin-converting enzyme level was within the normal range. Nerve conduction study was normal except slowing of conduction in bilateral facial nerves. He was found to have hyperhomocysteinemia with a low protein S level. The rest of the vasculitic profile was normal and high-resolution computed tomography of the thorax and contrast-enhanced CT of the abdomen did not reveal any evidence of malignancy. Serum electrophoresis did not reveal any gamma-globulin spike (M-band).

We started low-molecular-weight heparin for treatment of CVST, with bridging therapy of acenocoumarol subsequently.

Under anticoagulant and analgesic treatment with the maintenance of hydration, the patient gradually recovered in the next 7–10 days, and subsequent MR venography showed partial recanalization of the left transverse with complete canalization of SSS, right transverse sinus, and most of the other sinuses. The patient was discharged after 10 days of hospitalization after complete recovery from facial palsy within 10 days and normalization of nerve conduction studies. He was advised long-term anticoagulation and prophylaxis for hyperhomocysteinemia [Figure 1] and [Figure 2].{Figure 1} {Figure 2}

 Discussion



In our patient, the chronology of events documented by repeat MRI showed occlusion and later recanalization of the bilateral transverse sinus concomitant with onset and recovery from facial palsy. This led us to believe that the facial palsy was a direct consequence of the transverse sinus occlusion. Although the involvement of most cranial nerves has been described in CVST,[2],[3],[5],[9] bilateral facial palsy in CVST is incredibly rare, and little is known about its pathophysiology. The cranial nerve syndromes identified in the earlier literature have been thought to be caused by pressure palsy, either directly by the thrombotic clot lying in proximity to the nerves[5] or by raised ICP.[7] Another explanation for cranial nerve palsy in CVST was given by Kuehnen et al.,[9] who reported five cases of cranial nerve palsy as the only feature of isolated transverse sinus thrombosis. The authors hypothesized that the palsy was due to neurapraxia from reversible compromised oxygen or glucose consumption caused by impaired drainage of the cranial nerve veins into the transverse sinus. Clinically, the rapid recovery of the nerve function supported the hypothesis of their study.[9]

In this patient, transmural thrombosis of the distal transverse sinus is evident in MRV, as it involves the drainage site of the superior petrosal sinus and hence the drainage territory of the lateral pontine vein, it could have raised the intraluminal pressure in the nerve’s satellite vein. Impaired venous drainage of the nerve root’s satellite vein was probably transmitted to the intrinsic vascular system of the nerve. A rise in venous intraluminal pressure in the extrinsic system can increase the pressure in these intraneural venules and lead to venous blood–brain barrier dysfunction, with leakage of fluids and ions into the endoneurial space; thus, an impairment of the salutatory current flow, with reversible slowing of nerve conduction, could account for the neurapraxia in our patient. A similar hypothesis was also proposed by Straub et al.[10],[11],[12]

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.

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