A Unique Case of Churg–Strauss Syndrome with the Coexistence of Acute Neuropathy and Myositis
Makaleler > Tam Makale · 2023
Yazarlar: Şeyma AYKAÇ, Ayşe Nur YÜCEYAR, Taner AKALIN, Zafer Colakoglu
Neurology India, Uluslararası, Scopus, 2023, cilt 71, ss 1300-1301
Özet
Sir, A 57-year-old man was evaluated because of the complaint of a 10-day-old bilateral hand weakness with palm numbness. He had asthma in his past medical history. On admission, the neurological examination revealed hypoesthesia in both hands and weakness in the distal upper limbs. After 3 weeks, the patient developed quadriparesis with the complaint of myalgias all over his body. In nerve conduction studies, upper extremity sensorimotor action potentials were not recorded. In the leg, the sural response was absent, and fibular and posterior tibial nerve compound muscle action potentials diminished. The needle EMG revealed normal motor unit action potentials (MUPs) and denervation potentials in the distal upper and lower limbs. We found polyphasic, short-duration MUPs with denervation potentials and early interference during maximum muscular contraction in proximal muscles. Prior to EMG, serum creatine kinase levels were also elevated (1040 U/L, N = 41–71 U/L). Laboratory tests revealed leukocytosis 27 × 109/L (N = 4–11 × 109/L), eosinophilia 16 × 109/L (N = 0–0.7 × 109/L), an elevated total IgE level of 752 kU/L (N = 100 kU/L), and an elevated C-reactive protein level of 79 mg/L (N = 0–5 mg/L). Immunofluorescence for anti-cytoplasmic antibody (P-ANCA) was positive, and ELISA for anti-myeloperoxidase antibody (MPO-ANCA) was very positive at >200. The sural nerve biopsy demonstrated vasculitis with fibrin exudation and a mixed inflammatory cell infiltration containing many eosinophils in the vessel wall. For 5 days, he received a high dose of intravenous methylprednisolone (1 g/day), and then he switched to oral prednisolone (60 mg/day). Mycophenolate mofetil was added afterward. The patient was able to walk with assistance after 3 months of therapy. The present case depicts a rare episode of CSS presenting as acute neuropathy and myositis seen together. Diagnosis of CSS was fulfilled based on the presence of asthma, eosinophilia, neuropathy with myopathy, and eosinophilic vasculitis.[1] There are no reported cases of neuropathy and myositis together. Woo et al. reported a case of CSS that showed inflammatory myopathy that preceded 2 months before the onset of peripheral neuropathy.[2] In addition, only one retrospective study revealed that six of 24 CSS patients with peripheral neuropathy had a proximal weakness, perhaps owing to concurrent muscle involvement, which is clinically ignored.[3] In conclusion, acute neuropathy and myositis might occur simultaneously in the attacks of CSS and clinicians should be aware of that neurologic presentation. Declaration of patient consent The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed. Financial support and sponsorship Nil. Conflicts of interest There are no conflicts of interest.