We report a case of a 61-year-old man, smoker, with arterial hypertension, who was admitted to our hospital with a 4-month history of constitutional symptoms (16% body weight loss and anorexia) and numbness of the plantar surface of the feet, and since the day before, with diplopia, ptosis and sudden changes in oculomotricity in the left eye (Fig. 2). No family history of rheumatic or neurologic diseases was reported.
The patient's vital signs were normal, except for blood pressure which was high. On physical examination, he looked underweight. Neurological evaluation revealed weakness of ankle dorsiflexion (grade 4/5 according to the Medical Research Council scale), with normal plantar flexion, "glove and stocking" dysesthesias distal to the knees, reduced pallesthesia distal to the iliac crest, complete ptosis and exotropia of the left eye and anisocoria greater in bright light. Slit lamp and fundus examination of both eyes was normal, with a normal sized optic disc. Visual acuity was also normal.
Beside elevated inflammatory markers (erythrocyte sedimentation rate - ESR 51 mm/h, C-reactive protein - CRP 20.2 mg/l), laboratory workup was normal. Autoimmune workup, including antinuclear antibodies (ANA), rheumatoid factor (RF), anti-cyclic citrullinated peptide (ACPA) antibodies, antineutrophil cytoplasmic antibodies (ANCA), antibodies against extractable nuclear antigen (ENA), antineuronal antibodies and cryoglobulins, was negative and complement levels were normal. Angiotensin converting enzyme (ACE) and ceruloplasmin levels were normal.
Negative hepatitis B surface antigen (HbsAg), positive hepatitis B surface antibody (anti-HBs) and positive hepatitis B core antibody (anti-HBc) were found, consistent with a past infection. Hepatitis B surface antigen (HbsAg) was negative. Serological testing for Epstein-Barr virus, cytomegalovirus, herpes simplex viruses types 1 and 2, parvovirus b12 and human immunodeficiency virus was negative.
Electromyography (EMG) of the limbs showed a severe, acute and symmetric distal axonal sensorimotor polyneuropathy. To look for underlying malignancy, computed tomography (CT) of chest, abdomen and pelvis and endoscopic evaluation were performed and no evidence of malignancy was found. Chest CT scan revealed a cluster of small nodules in the superior lobe, associated with tree-in-bud lesions and no lymphadenopathy was found. Sputum examination did not reveal acid-fast bacilli and cultures were negative. Bronchoalveolar lavage mycobacterial culture and polymerase chain reaction for detection of Mycobacterium tuberculosis were negative.
The first brain magnetic resonance imaging (MRI) was unremarkable. A second study was performed and the MRI angiography revealed a small aneurysm of the left posterior communicating artery (Fig. 3), without compression of the oculomotor nerve pathway (Fig. 4 C). Additionally, heavily T2-weighted sequences showed thickening and hyperintensity of the left oculomotor nerve (Fig. 4 A, B), with avid enhancement after gadolinium administration, more conspicuous on fat-saturated T1-weighted images (Fig. 4 D, E).
Polyarteritis nodosa with cranial and peripheral neuropathies was diagnosed. Intravenous methylprednisolone pulse (1 g daily for 3 days), followed by 0.5 mg/kg daily of oral prednisolone and azathioprine (AZT) (2 mg/kg daily), was started. At 3 months' follow-up, a huge improvement was observed with resolution of the diplopia and oculomotricity changes, and improvement of the numbness (Fig. 2 B). Also, inflammatory markers returned to normal and, EMG showed improvement of the polyneuropathy. Prednisolone was slowly tapered and 2 years later the patient remains in remission on AZT.