ÓRgÃO OfICIAL dA SOCIEdAdE PORTUgUESA dE REUMATOLOgIA 286 CASO CLÍNICO 1. Institute of Neurology; Catholic University of Sacred Heart, Rome-Italy sural nerve biopsy revealed uncommon pathological findings. cAse report A 64-year-old woman was admitted to our department to investigate a three months history of distal numbness involving hands and feet, associated with lower limbs weakness more pronounced in left leg. Anamnesis re- vealed only a two years history of asthma, although spe- cific allergic factors were not identified and serum IgE level was within the normal range. Neurological exa- mination revealed a strength weakness in tibialis ante- rior (TA) and extensor hallucis longus (EHL) graded 2 on the MRC scale in right muscles and 1 in the left ones, paraesthesias and hypoesthesias with stocking-and-glo- ve distribution and the absence of tendon reflexes. Extensive laboratory studies, including complete blood count, erythrocyte sedimentation rate, concen- trations of electrolytes, C-reactive protein, fasting glu- cose, glycosylated hemoglobin, FT3, FT4, TSH, anti- thyroid antibodies, serum vitamin B12 and folate, he- patic enzymes, creatinine, urinalysis, immunofixation electrophoresis, serum IgE level, antinuclear antibodies (ANA), anti-extractible nuclear antigens (ENA), anti- -DNA antibodies, antineutrophil cytoplasmic antibo- dies (ANCA), circulating C3 and C4, anti-Hu antibo- dies, serologic tests for HBV, HCV and HIV and scree- ning for infections, malignancies, malabsorption and systemic autoimmune disorders, revealed only an in- crease of eosinophils count (1.29 x 10 9 /l, normal value: 0.0-0.7) and percentage (23.5%, normal value: 0-5). Nerve conduction studies were consistent with an axonal multiple mononeuropathy (Table I). Electro- myographic examination, performed in TA, EHL, rec- tus femoris and first digiti interosseous bilaterally, sho- wed in both TA and EHL a neurogenic pattern, more se- Uncommon pathological findings in sural nerve biopsy from a patient with Churg-Strauss related multiple mononeuropathy Marco Luigetti 1 , Alessandra Del Grande 1 , Angela Romano 1 , Mario Sabatelli 1 AbstrAct We describe a patient with severe multiple mononeu- ropathy associated with hypereosinophilia, asthma and pulmonary non cavitating micronodules. Sural nerve biopsy revealed marked perineural thickening and mi- crofasciculation with inflammatory infiltrates in the pe- rinerium and in the epinerium. The patient markedly improved with steroid therapy. Our final diagnosis was Churg-Strauss related multiple mononeuropathy. Thus, we report a case of Churg-Strauss related multiple mo- noneuropathy with uncommon pathological findings on sural nerve and we underline the importance of cli- nical evaluation for this diagnosis. Keywords: Nerve biopsy; Multiple mononeuropathy; Churg-Strauss Syndrome; Perineural microfascicula- tion; Inflammatory infiltrates. IntroductIon Eosinophilic granulomatosis with polyangiitis (EGPA) is essentially a pathological entity characterized by a necrotizing vasculitis with eosinophilic infiltration and extravascular granuloma formation in tissues obtained by biopsy or necropsy 1,2 . Clinically, this disorder is usually referred to as Churg-Strauss syndrome (CSS) a rare and potentially fatal vasculitis characterized by a prodromal phase with allergic rhinitis, chronic sinusi- tis, bronchial asthma, pulmonary infiltrations and blood and tissue eosinophilia, followed by a vasculitic phase with a generalized multisystemic disease process that often involves peripheral nerves 2 . Herein we describe a patient, affected by a Churg- -Strauss related multiple mononeuropathy, in which ACTA REUMATOL PORT. 2013;38:286-289