Peripheral neuropathyMonday, June 09, 2008, 17:30 - 19:00
Multiple cranial neuropathy revealing a final stage of Wegener´s granulomatosisL. Romero, S. Dimas, S. Vargas, R. Vega, I. Horta (Mexico City, MX)
Wegener granulomatosis (WG) is a necrotizing vasculitis that primarily affects small-caliber vessels of the arterial system. Microvasculitis produces necrotizing granuloma in lungs, focal necrotizing vasculitis and glomerulonephritis. Left untreated, the disease is rapidly fatal.
Purpose: Report a rare case of multiple cranial neuropathy revealing a final stage of Wegener´s Granulomatosis.
Case history: A 41-year-old male with symptoms that resemble an upper respiratory tract infection associated with frontal headache. The clinical examination presented a protruded mass in the left nasal mucosa. The lesion was excised and reported as unspecified inflammatory process. Lesion in the nasal mucosa recurred 4 times in a 5 years period, three years later he presented a nodular cutaneous lesion in malar region. Two years later he developed a recurrent sinusitis. He was admitted at the hospital in a first time with fever and vertical diplopia. The diagnosis of Tolosa Hunt syndrome was established and treated with oral prednisone with totally remission. When the oral dose of prednisone was diminished, bilateral ophthalmoplegia showed up again, and develop V, VII, IX, X, XI, and XII cranial nerve affection. No other neurological manifestation was detected. Lung tomography showed a cavitary apical lesion. Axial T1-weighted postcontrast cranial magnetic resonance imaging revealed meningeal enhancing. Lumbar puncture was normal. One month later he develops bilateral optic atrophy, hypocusia, distal symmetrical sensorimotor polyneuropathy and cerebral hemorrhage. The antineutrophil cytoplasmic antibodies directed against protinase 3 were positives. Skin biopsy showed vasculitis granulomas and necrosis. Two days later the patient died of a massive lung hemorrhage.
Comment: Peripheral neuropathy is a frequently neurological feature in Wegener´s disease because involve mainly large diameter nerves and this correlate with the typical mononeuritis multiplex presentation. Nevertheless multiple cranial neuropathy as the first and principal neurological affection with rapid progression to the death is less frequent. This is a possible remarkable clinic point for an appropriate and timely treatment of wegener´s granulomatosis with other fatal potential vasculitic organ manifestation.