Fatal Morvan Syndrome Associated With Myasthenia Gravis  

Oleh:

Nagappa, Madhu ; Mahadevan, Anita ; Sinha, Sanjib ; Bindu, Parayil S. ; Mathuranath, Pavagada S. ; Bineesh, Cheminikara ; Bharath, Rose D. ; Taly, Arun B.

Jenis: Article from Journal - ilmiah internasional
Dalam koleksi: The Neurologist vol. 22 no. 01 (Jan. 2017), page 29-33.
Topik: CASPR2 Antibodies; Dysautonomia; Morvan Syndrome; Myasthenia Gravis; Peripheral Nerve Hyperexcitability; Thymoma
Fulltext: N06 v22 n1 p29 kelik2017.pdf (208.78KB)

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Perpustakaan FK Nomor Panggil: N06.K Non-tandon: 1 (dapat dipinjam: 0) Tandon: tidak ada

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Isi artikel

Introduction: Morvan syndrome is a rare and complex autoimmune disorder affecting multiple sites of neuraxis. Case Report: We present fulminant Morvan syndrome, developing on a background of chronic myasthenia gravis. A 54-year-old gentleman presented with fluctuating ophthalmoplegia and proximal muscles weakness of 7 years duration that remitted with pyridostigmine and prednisolone. He developed insomnia of 2 months duration, worsening of myasthenic symptoms and respiratory distress, dysautonomia, encephalopathy, and peripheral nerve hyperexcitability. Antibodies against contactin-associated protein (CASPR) 2 were detected in serum. Computed tomography of thorax showed a thymic mass. He received intravenous methyl prednisolone and plasmapheresis. Antibodies against CASPR and thymic lesion reduced with immunotherapy. However, he developed persistent hypotension and expired after 11 weeks of hospital stay. Conclusions: Clinical clues for diagnosis of Morvan syndrome and therapeutic changes faced by the treating team are highlighted in this report. Increased awareness and prompt testing for CASPR2 antibody is warranted so that early immunotherapy can be initiated.

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