Facial Palsy in Cerebral Venous Thrombosis: An Atypical Case in a Young Girl.

Cerebral venous thrombosis (CVT) is a rare and potentially life-threating cause of stroke. A number of etiologies and risk factors for CVT have been identified so far. These include head trauma, local and systemic infectious diseases, malignancies, autoimmune diseases, and oral contraceptive use. The most common clinical symptoms are headache and changes in consciousness. Cranial nerve palsy in CVT is uncommon, and there are very few reports of facial nerve palsy. This case report highlights an atypical manifestation in a CVT patient, who presented with peripheral facial palsy. The patient was successfully treated with anticoagulation.

INTRODUCTION

Cerebral venous thrombosis (CVT) is an uncommon, multifactorial disease presenting with a wide spectrum of neurological symptoms. The most common clinical symptoms are nausea, vomiting, headache, and change in consciousness.[1] Cranial nerve palsy has been rarely reported as a manifestation in CVT, and it is often thought to result from increased intracranial pressure or cavernous sinüs thrombosis.[2] Involvement of the facial nerve is very unusual, and its underlying mechanism is not fully understood. In this report, we describe a patient who presented with unilateral facial palsy in addition to the other common clinical symptoms of CVT.

CASE REPORT

A 17-year-old previously healthy girl presented to our emergency department with a 5-day history of headache, neck pain, a 1-day history of projectile vomiting, severe occipital headache, and drowsiness. She had been taking oral contraceptives for 2 months before presentation.

On admission, physical examination findings revealed peripheral facial paralysis on the right side. Neurologic examination showed a confused, lethargic female with left hemiplegia and extensor plantar response on the left side. Papilledema was observed on fundoscopic examination. Neck stiffness was present, but Kernig's and Brudzinski's signs were negative; all other findings on general examination were normal.

Laboratory investigations which include blood counts, chemistry, electrolytes, lipid profile, C-reactive protein level, erythrocyte sedimentation rate, folate, prothrombin time, activated partial thromboplastin time, fibrinogen, proteins C, protein S, homocysteine, factors V, VII, VIII, XII, and antithrombin III, lupus anticoagulants, antinuclear antibody, and anticardiolipin antibodies were all normal. Factor V Leiden and prothrombin GA20210 mutations were not detected. Transthoracic echocardiographic examination was normal.

Cerebrospinal fluid (CSF) examinations were normal except for an elevated CSF pressure (45 cm H2O). CSF and blood cultures were unremarkable.

On cranial magnetic resonance (MR) imaging and cerebral MR venography, thrombosis were seen on the straight, superior longitudinal, bilaterally transverse, and right sigmoid sinuses [Figure 1].

Figure 1

Sagittal (a) and axial (b) brain magnetic resonance imaging and magnetic resonance venography (c) showing thrombosis in the straight, superior longitudinal, bilaterally transverse, and right sigmoid sinuses (arrows)

The patient was hospitalized with the diagnoses of CVT and pseudotumor cerebri due to CVT. Treatment with subcutaneous low-molecular-wight heparin and acetazolamide were initiated. At the end of the 2nd week of treatment, the right peripheral facial paralysis and left hemiplegia had resolved, and the patient was discharged with subcutaneous low-molecular-weight heparin.

DISCUSSION

CVT is rare condition affecting 0.6–7/100,000 population and accounts for 1%–2% of cerebral infarctions. It is defined as thrombosis of the superficial or deep venous system in the brain.[3]

CVT has been associated with various etiologies including hypercoagulable states, head trauma, dehydration, oral contraceptives use, neoplastic invasion of a venous sinus, vasculitis, intracranial and systemic infections, congenital heart disease, anemia, pregnancy, and puerperium.[4] Oral contraceptive use is the only known risk factor in our patient. In addition to the absence of risk factors such as trauma, systemic or intracranial infection, dehydration, and vasculitis, the extensive laboratory evaluation for prothrombotic conditions showed normal findings. Similar to our patient, Tan et al. reported two CVT women in whom oral contraceptive use was the only identified risk factor.[5] Both patients were in the 18-year-old age group.

Clinical signs and symptoms of CVT are usually attributable to the increased intracranial pressure and cortical damage, alterations in the metabolism of underlying nervous tissues, or a combination of these processes.[6] Patients may show an acute or a subacute picture related to disease progression. The common presenting signs and symptoms of CVT are headache, changes in mental status, focal weakness, seizures, and papilledema. Cranial nerve palsy has been reported in 12% of CVT cases.[7] The most commonly involved cranial nerves (CN) are CN II, IV, and VI. Our patient presented with peripheral facial nerve palsy in addition to the classic symptoms.

There have been a few reports that have found an association between CVT and peripheral nerve palsy. Cranial nerve palsy due to CVT has been postulated to be caused by elevated intracranial pressure, extension of thrombosis to venous channels, or direct pressure from the clot itself. Our patients' findings on presentation of thrombosis of the straight, superior longitudinal, bilaterally transverse, and right sigmoid sinuses with ipsilateral peripheral facial palsy may be explained by either one or more of the above mechanisms. Similar to our case, Straub et al. previously described a 17-year-old girl with peripheral facial palsy, who had ipsilateral transverse sinus thrombosis.[2]

Although the presenting signs and symptoms of CVT are variable, a diagnosis of CVT should be considered in young patients with peripheral facial palsy. This case highlights peripheral facial palsy as a rare clinical presentation of CVT.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.