Case Report

Cerebral vein thrombosis and Guillain-Barre syndrome: a challenging association

Abstract

A 53-year-old male patient came to our observation for an unusual association between cerebral vein thrombosis (CVT) and Guillain-Barre syndrome (GBS). Headache and focal neurologic deficit of the facial and hypoglossal Cranial nerves were the main clinical findings on presentation at the emergency department. The presence of CVT, as suggested by initial CT scan, was confirmed by subsequent magnetic resonance imaging (MRI) and low-molecular-weight hepar- in treatment was begun.

In the following 24 hours, acute respiratory failure, rapidly progressive paralysis of the limbs, and areflexia occurred. Cerebral fluid analysis and electromyography were diagnostic for GBS, whereas serologic examination demon- strated previous Epstein-Barr virus infection. Intravenous immunoglobulin administration was then started. The patient was dismissed after 30 days, and after a follow-up of 60 days, he is alive without residual neurologic deficit.

Local and Systemic Infections are recognized risk factors for developing both CVT and GBS, but an etiologic linkage between the 2 conditions has not yet been demonstrated. However, the simultaneous association between 2 rare diseases suggests a possible common pathway consisting in an infective process that triggers GBS and subsequently involves the meningeal spaces and the cerebral sinus to cause CVT. This hypothesis may support the indication of a more accurate investigation including the routine use of MRI venography for an early detection of CVT in patients presenting GBS.

Cerebral vein thrombosis (CVT) and Guillain-Barre syndrome (GBS) are rare diseases (0.3-0.4 and 1-3 per 100 000 respectively), with variable clinical presentation [1-3]. Both are associated with low mortality but significant morbidity [3-5]. The widespread use of sensitive Imaging techniques increases the number of CVT discovered, even in mildly symptomatic patients [6,7].

Both diseases present with wide spectrum of symptoms ranging from a mild focal neurologic defect to severe neurologic impairment with coma.

Headache, vomiting, and Visual disturbances are the most common symptoms associated with CVT, although hemi- paresis, ataxia, seizures, cranial nerve palsy, and aphasia also occur [3,5]. Heparin is generally agreed to be the mainstay of treatment of CVT. Recently, local thrombolysis was also considered a therapeutic option in selected patients [8-11].

We report an unusual association between CVT and GBS and their challenging clinical management because of their simultaneous and overlapping manifestation. A 53-year-old male patient presented to the emergency department with headache, dizziness, difficulty swallowing, vomiting, and unilateral facial and hypoglossal nerve palsy lasting for less than 1 hour. At presentation, his Glasgow Coma Scale was 15, NIHSS was less than 5, and Vital parameters and blood tests were within normal range. He had a history of hypertension, polycythemia, myalgic complaints, and recur- rent urinary tract infections. A computed tomographic scan ruled out Hemorrhagic lesions but suggested a right transverse cerebral sinus thrombosis. The diagnosis was confirmed by magnetic resonance imaging (MRI), and weight-adjusted low-molecular-weight heparin treatment was begun. The clinical course was complicated by sudden respiratory failure, worsening of the Glasgow Coma Scale, and gastric bleeding. The patient required mechanical ventilation and was referred to an intensive care unit. After 24 hours, rapidly progressive paralysis of the limbs and areflexia occurred. The computed tomography and MRI results remained unchanged. Neurologic examination, cere- bral fluid analysis, and electromyography allowed to the diagnosis of GBS. The patient was treated with intravenous immunoglobulin (Ig). The serology was negative for circulating autoantibodies but positive for previous Epstein-Barr virus infection. The patient was dismissed after 30 days with mild neurologic deficit. After a follow-up of 60 days, he is alive without residual neurologic deficit. Magnetic resonance imaging showed the recanalization of the thrombus.

Isolated reports exist of the occurrence of CVT in the course of GBS, as a complication only of Ig adminis- tration or of deep venous thrombosis of the limbs [12- 15]. Local and systemic infections are risk factors for developing both GBS and CVT, the former triggered by an immune-mediated process after bacterial/viral infection and subsequent acute inflammatory demyelinating

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polyneuropathy [1,2]. Other recognized predisposing conditions are thrombophilia, trauma, malignancies, collagen-vascular, and hematologic disorders for CVT [3,5] and invasive surgical and Vascular procedures or administration of vaccines for GBS [1,2].

The simultaneous occurrence of these 2 Rare conditions in our patient gives rise to some considerations.

First, in about 30% of patients, CVT may follow a subacute course with no or mild aspecific symptoms [3,5]; therefore, CVT could have preceded the GBS in our patient. Second, in a challenging clinical setting because of the somewhat overlapping symptoms of the 2 diseases and the possible misdiagnosis of an acute ischemic stroke at first presentation, the diagnostic key was the accurate imaging studies. Finally, there is no evidence that CVT may cause GBS or vice versa; however, the exceptional association between 2 rare diseases could be more than coincidental and suggests a possible common infective process that triggers GBS and progresses through the meningeal spaces to the cerebral veins and sinus to cause CVT. A poorly sympto- matic GBS may, therefore, precede CVT and may represent a predisposing condition to CVT.

If this hypothesis were confirmed, a more accurate investigation including routine use of MRI venography may become mandatory in patients with GBS.

Alfonso Lagi MD Simona Spini MD, PhD Sergio Gallori MD

Emergency and Accident Department Ospedale Santa Maria Nuova Azienda Sanitaria Firenze

Florence, Italy E-mail address: [email protected]

doi:10.1016/j.ajem.2008.10.029

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