Introduction
Antineutrophil cytoplasmic antibody associated vasculitis (ANCA-associated vasculitis; AAV) is a group of rare autoimmune diseases that show typical characteristic inflammatory cell infiltration and blood vessel wall necrosis. This vascular damage can occur in many parts of the body, resulting in serious kidney failure, skin lesions and respiratory distress. In some cases, AVV affects the brain. Here, we report a rare patient who presented with AAV and concomitant mononeuritis multiplex.
Case Report
A 63-year-old man was admitted for fever of unknown origin for two months. He also experienced weight loss and motor weakness of his left upper and lower extremities. During the effort to find the cause of the fever, the patient was diagnosed with AVV in a blood test and biopsy. While the patient was treated with steroid therapy, sudden dysarthria and motor weakness of the left upper and lower extremities occurred. Magnetic resonance imaging (MRI) revealed acute cerebral infarction on the right corona radiata, putamen and external capsule with hemorrhagic transformation. After 2 weeks later, sudden weakness of the right upper extremity was noted. Imaging study revealed intracerebral hemorrhage in the left basal ganglia. The patient did not have any stroke risk factors such as old age (>65), hypertension, diabetes, cardiovascular problems, dyslipidemia, smoke and drink except sex (male).
On the 14 days of onset, muscle strength of both upper and lower extremities were good (G) grade on manual muscle test except his hand grip was poor (P) grade on the right side and the left ankle dorsiflexion, great toe extension, ankle plantarflexion were zero (Z) grade on manual muscle test with focal decreased deep tendon reflex. To diagnose the cause of his motor weakness of distal part of extremities an electromyography was done and bilateral median, left ulnar, common peroneal nerve and tibial nerves lesions were found. These findings were compatible with mononeuritis multiplex. His motor strength showed recovery on his ankle plantarflexion from Z to poor (P) grade on discharge, but ankle dorsiflexion was still Z grade. To overcome imbalance caused by his left foot drop, the ankle-foot-orthosis was prescribed for gait training.
Conclusion
AVV rarely involves the central nervous system (CNS). Vasculitis associated with CNS lesion should be suspected if patients have neurological manifestations consistent with CNS involvement, particularly if they have evidence of disease activity elsewhere, and if imaging study shows ischemic or haemorrhagic lesions on brain. In addition, our patient showed asymmetric progression of revealed concomitant peripheral nervous system (PNS) lesion showing focal lower motor neuron signs including foot drop which is characteristically seen in vasculitic polyneuropathy. Therefore, it is important to conduct detail physical examination not to overlook complex CNS and PNS lesions associated with this autoimmune diseases. |
