Alessandra Cicia
Focal myositis in a patient with anti-AChR positive Myasthenia Gravis
Autori
- ALESSANDRA CICIA (UNIVERSITÀ CATTOLICA DEL SACRO CUORE – FONDAZIONE POLICLINICO AGOSTINO GEMELLI – NEUROLOGIA)
- RAFFAELE IORIO (UNIVERSITÀ CATTOLICA DEL SACRO CUORE- FONDAZIONE POLICLINICO AGOSTINO GEMELLI – NEUROLOGIA)
- VINCENZO CARLOMAGNO (UNIVERSITÀ CATTOLICA DEL SACRO CUORE – FONDAZIONE POLICLINICO AGOSTINO GEMELLI – NEUROLOGIA)
- SILVIA FALSO (UNIVERSITÀ CATTOLICA DEL SACRO CUORE- FONDAZIONE POLICLINICO AGOSTINO GEMELLI – NEUROLOGIA)
- SOFIA MARINI (UNIVERSITÀ CATTOLICA DEL SACRO CUORE- FONDAZIONE POLICLINICO AGOSTINO GEMELLI – NEUROLOGIA)
- MASSIMILIANO MIRABELLA (UNIVERSITÀ CATTOLICA DEL SACRO CUORE- FONDAZIONE POLICLINICO AGOSTINO GEMELLI – NEUROLOGIA)
- MATTEO LUCCHINI (UNIVERSITÀ CATTOLICA DEL SACRO CUORE- FONDAZIONE POLICLINICO AGOSTINO GEMELLI – NEUROLOGIA)
Presentatore
ALESSANDRA CICIA
Modalità
Poster Session
Abstract
“A 58-year-old woman was diagnosed with thymoma in 2018. In May 2023, she presented the subacute appearance of severe and fluctuating dysphagia followed after few weeks by a distal left upper limb hyposthenia in the extensor muscles. An electromyography of the upper limbs revealed a myopathic pattern while chest CT scan demonstrated a slight enlargement of the thymoma. CPK serum level was 415 mcg/L and anti-Acetylcholine receptor antibodies were detected (firstly through ELISA and then with RIA and CBA).
The patient underwent thymectomy and one cycle of intravenous immunoglobulins, with partial recovery. Afterwards, pyridostigmine and low doses of corticosteroids (prednisone up to 25 mg/die) were introduced, with improvement of dysphagia but only mild recovery of the distal upper limb weakness.
Six months later, she referred to our outpatient’s clinic, where neurological examination documented muscular atrophy and hyposthenia of the left wrist extensor, finger extensors, and first dorsal interosseus. Myositis- specific and associated antibodies resulted negative with normal CPK levels. She continued treatment with prednisone and pyridostigmine, without further clinical exacerbation.
Myasthenia Gravis and inflammatory myopathies are both rare acquired disorders caused by autoimmune response against neuromuscular junction and muscle tissue, respectively. Their association has been reported in less than 100 patients and represents a diagnostic challenge. Clinical phenotypes may vary, but cases of isolated focal distal muscle involvement in upper limbs have been recently described. Increasing clinical awareness on these conditions is needed to better optimize treatment choice and goals and to avoid potentially poorer clinical outcomes.”