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Neuromyelitis Optica in Patients with Myasthenia Gravis Who Underwent Thymectomy

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Neuromyelitis Optica in Patients with Myasthenia Gravis Who Underwent Thymectomy ORIGINAL CONTRIBUTION Neuromyelitis Optica in Patients With Myasthenia Gravis Who Underwent Thymectomy Ilya Kister, MD; Sandeep Gulati, MD; Cavit Boz, MD; Roberto Bergamaschi, MD; Guiseppe Piccolo, MD; Joel Oger, MD; Michael L. Swerdlow, MD Background: Myasthenia gravis (MG) and neuromy- Patients: Four patients with MG who underwent elitis optica (NMO, also known as Devic disease) are rare thymectomy. autoimmune disorders, with upper-limit prevalence es- timates in the general population of 15 per 100 000 and Interventions: None. 5 per 100 000, respectively. To our knowledge, an asso- ciation between these diseases has not been previously Results: The prevalence of MG within the published co- reported. hort of patients with NMO is more than 150 times higher than that in the general population. Objectives: To describe 4 patients with MG who de- Conclusion: Dysregulation of B-cell autoimmunity in my- veloped NMO after thymectomy and to analyze possible asthenia, possibly exacerbated by loss of control over au- causes of apparent increased prevalence of NMO among toreactive cells as a result of thymectomy, may predis- patients with MG. pose patients to the development of NMO. Design: Case series. Arch Neurol. 2006;63:851-856 EUROMYELITIS OPTICA REPORT OF CASES (NMO) is characterized by 1 or more attacks of CASE 1 optic neuritis (ON) and myelitis. It can be dif- An African American woman with mild Nferentiated from multiple sclerosis (MS) asthma, distant history of smoking and with the aid of magnetic resonance imag- cocaine snorting, and family history of ing (MRI),1-3 cerebrospinal fluid anal- MS in her mother developed symptoms ysis4-8 and NMO-IgG antibody.9 The of ocular myasthenia at age 38 years. The lesions in NMO differ from those of MS diagnosis was confirmed by neostigmine with respect to patterns of immunoglob- test and an elevated anti–acetylcholine ulin and complement deposition and receptor (anti-AChR) antibodies titer. 10,11 Author Affiliations: populations of inflammatory cells. That same year, her thymus was excised, Departments of Neurology, Patients with NMO often have coexisting and histologic examination revealed Albert Einstein College of autoimmune disorders, such as systemic hyperplasia. The symptoms resolved and Medicine, Bronx, NY lupus erythematosus, Sjo¨gren syndrome, pyridostigmine bromide therapy was (Drs Kister and Swerdlow), and and pernicious anemia.12,13 Ours is the tapered. Long Island Jewish Medical first case series, to our knowledge, of A year after the surgery, she experi- Center, New Hyde Park, NY coexisting MG and NMO. All 4 of our enced acute-onset visual loss in her right (Dr Gulati); Multiple Sclerosis patients were diagnosed as having MG, eye, followed by visual loss in the left eye Clinic, University of British underwent thymectomy, and subse- 10 days later. Her clinical picture and vi- Columbia, Vancouver (Drs Boz and Oger); and Multiple quently developed NMO. We discuss the sual evoked potentials were typical of ON. Sclerosis Center, Neurological evidence for B-cell dysregulation in Magnetic resonance imaging of the brain Institute “C. Mondino,” Pavia, NMO and for the possible role of thy- disclosed a questionable increased T2 sig- Italy (Drs Bergamaschi and mectomy in potentiating the emergence nal in the right optic nerve and a few non- Piccolo). of autoimmune disease. enhancing scattered subcortical foci of in- (REPRINTED) ARCH NEUROL / VOL 63, JUNE 2006 WWW.ARCHNEUROL.COM 851 ©2006 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 09/29/2021 bar ON. Brain MRI was normal, and increased T2 signal was seen in the left optic nerve. Visual acuity did not improve with time. At age 42 years, she was admitted to the hospital with swelling and pain in the forearms precipitated by vigor- ous clapping. Her urine was dark red, and her creatine kinase level was 25 000 U/L. Rhabdomyolysis resolved with aggressive hydration. At age 43 years, she developed leg paresthesias and midthoracic back pain. She had profound sensory dys- function in all modalities below T8 level, symmetric hy- perreflexia of the legs, and bilateral extensor plantar re- sponses. Magnetic resonance imaging demonstrated abnormal cord signal from the lower cervical spine to T9 and enhancement in T2 through T4. An MRI of the brain was normal, as were rheumatologic and infectious sero- logic test results, except for a mildly elevated anti- nuclear antibody titer (1:80). She was started on aza- thioprine therapy. Later that year, she was readmitted for progressive stiff- ness and spasms of all extremities. The clinical picture suggested stiff-person syndrome. Her serum anti– glutamic acid decarboxylase antibody titer was 2 U/mL (reference threshold, Ͻ1.0 U/mL). A course of intrave- nous immunoglobulin and methylprednisolone acetate Figure 1. Axial fluid-attenuated inversion recovery magnetic resonance imaging sequence of the brain of patient 1 shows a few nonenhancing yielded a substantial improvement of her spasticity. scattered subcortical foci of increased T2 signal. At age 44 years, she had a milder relapse of myelitis. An MRI showed cord edema and enhancement from T3 through T5 and abnormal T2 signal at C5-C6. She again creased T2 signal (Figure 1). These were unchanged responded well to a short course of intravenous immu- after several years of follow-up. noglobulin and methylprednisolone. During the next 5 years the patient experienced 7 re- lapses of ON. Four years after the initial attack, she was CASE 3 given a brief trial of interferon beta-1a (Avonex) but soon developed progressive left-sided weakness. She was ad- mitted to the hospital with a partial Brown-Se´quard syn- A 17-year-old white girl complained of fluctuating diffi- drome and T7 sensory level. An eccentric lesion extend- culties with speech and chewing. She exhibited nasal ing from C2 to T1 was seen on MRI. She was diagnosed voice and facial, tongue, and neck weakness that wors- as having NMO and began azathioprine therapy. Dur- ened with exercise. Decrementing response on repetitive ing the next 2 years she experienced 2 relapses of ON stimulation test, elevated anti-AChR antibodies, and and 2 relapses of myelitis. At last follow-up, she had only positive response to neostigmine confirmed the diagno- shade perception and ambulated with assistance. Re- sis of MG. Good control of symptoms was achieved with sults of an extensive rheumatologic and infectious workup pyrostigmine. Her thymus was resected and revealed were unremarkable. Table 1 and Table 2 summarize hyperplasia. the essential clinical, radiological, and laboratory data, At age 19 years, she sustained acute severe loss of vi- including NMO-IgG antibody status and cerebrospinal sion in both eyes. Visual evoked response was absent on fluid analysis for all of our patients. Figures 2, 3, 4, the right and of low amplitude and delayed P100 la- and 5 show representative MRI views of spinal cord dur- tency on the left. She was diagnosed as having bilateral ing myelitis attack for each of our 4 patients. ON. During the subsequent 5 months, she had 3 re- lapses of ON leading to blindness in the right eye and CASE 2 severely impaired vision in the left. At age 33 years, she developed leg weakness and sen- A healthy 36-year-old African American woman pre- sory loss below the thorax. A large swollen lesion ex- sented with ocular symptoms of MG and was found to tending from C8 to T3 was seen on MRI. During 10 years have an elevated anti-AChR antibodies titer, decrement- of follow-up, she experienced 9 relapses of myelitis and ing responses on repetitive stimulation, and positive 1 of ON. At last follow-up, she had a sensory T2 level response to neostigmine. She was treated with pyri- and required a cane for walking because of moderate para- dostigmine therapy and thymectomy, with excellent paresis. During the relapses, a cervical-dorsal lesion was response. Thymus hyperplasia was seen on histologic always detected on MRI, variable in size from 3 to 5 spi- examination. nal segments. On 2 occasions the lesion was enhanced At age 41 years, she acutely lost vision in the right with gadolinium. Brain MRI continued to show normal eye. The examination results were consistent with bul- findings. (REPRINTED) ARCH NEUROL / VOL 63, JUNE 2006 WWW.ARCHNEUROL.COM 852 ©2006 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 09/29/2021 Table 1. Clinical and Radiological Findings in 4 Patients With MG and NMO Age at Event, y Patient No./ Onset Onset First Attack No. of ON No. of Myelitis MRI Lesion at First Other Medical Sex/ Race of MG Thymectomy of NMO of NMO Relapses Relapses Myelitis Attack Problems 1/F/AA 38 38 39 ON 7 3 C6-T1 HTN, asthma 2/F/AA 36 37 42 ON 1 2 C7-T9 Asthma, SPS 3/F/white 17 17 19 ON 6 9 C8-T3 None 4/F/Asian 27 28 38 ON/M 2 2 C2, C4-6 None Abbreviations: AA, African American; HTN, hypertension; M, myelitis; MRI, magnetic resonance imaging; NMO, neuromyelitis optica; ON, optic neuritis; SPS, stiff-person syndrome. Table 2. Laboratory Findings in 4 Patients With MG and NMO CSF Analysis Antibodies Patient No./ Sex/ Race WBC Count, Cells/µL Oligoclonal Bands NMO-IgG Anti-AChR Other 1/F/AA 10 None ϩϩ − 2/F/AA 2 None ϩϩAnti-GAD; ANA, 1:80 3/F/white 55 2 − ϩ ANA, 1:1024 4/F/Asian NA NA NA ϩ − Abbreviations: AA, African American; anti-AChR, anti–acetocholine receptor; ANA, antinuclear antibodies; anti-GAD, anti–glutamic acid decarboxylase antibody; CSF, cerebrospinal fluid; NA, not available; NMO-IgG, neuromyelitis optica–IgG; WBC, white blood cell; ϩ, present; −, absent. CASE 4 servation that autoimmune disorders are vastly overrep- resented within this subset of patients with NMO.
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