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Extraocular Muscle Responses to High Dose Intravenous Methylprednisolone in Myasthenia Gravis

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Extraocular Muscle Responses to High Dose Intravenous Methylprednisolone in Myasthenia Gravis 214 J Neurol Neurosurg Psychiatry 2000;68:214–217 J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.68.2.214 on 1 February 2000. Downloaded from SHORT REPORT Extraocular muscle responses to high dose intravenous methylprednisolone in myasthenia gravis Atsushi Komiyama, Hiroshi Arai, Masanori Kijima, Keizo Hirayama Abstract In myasthenia gravis, initial exacerbation of Three patients with generalised myasthe- symptoms after treatment with corticosteroids nia gravis and three with ocular myasthe- is a well known, frequent complication,1–3 the nia gravis received two to five courses of mechanisms of which are not fully understood. high dose intravenous methylpred- Generally, this steroid induced exacerbation nisolone because of the failure of standard has been attributed to the direct inhibitory immunomodulating therapies. Changes in eVect of corticosteroids on the impaired 4–6 myasthenic signs were assessed using a neuromuscular junction. All of our knowl- four step system for grading muscle weak- edge, however, is based on information ob- ness and fatiguability in 10 test items. tained from non-ocular skeletal muscles, and Although a brief and modest amelioration the extraocular muscles have many distinctions that separate them from limb and diaphragm was found from day 1 to day 2 after the muscles, including a specific subgroup of initial infusion in two patients with gener- neuromuscular junctions and the antigenic alised myasthenia gravis, all three experi- properties of the acetylcholine receptor enced a prolonged phase of worsening (AChR).7 To elucidate any diVerences in the followed by improvement before the next direct responses to corticosteroids between course. Conversely, for two of the patients extraocular and other skeletal muscles, we with ocular myasthenia gravis, a transient quantitatively assessed changes in myasthenic but dramatic improvement of ptosis and signs after treatment with high dose intra- ocular immobility was noted from 90 min- venous methylprednisolone. utes to 5 hours after initiating the first infusion, followed by mild or no exacerba- tion. This 3 hour improvement may be Patients and methods related not only to possible diVerences in PATIENTS http://jnnp.bmj.com/ the neuromuscular junction, but also to Three patients with generalised myasthenia corticosteroids unmasking the central ad- gravis and three with ocular myasthenia gravis Department of aptation for the peripheral ocular muscle received high dose intravenous methylpred- Neurology, Urafune weakness by increasing the acetylcholine nisolone because of the failure of standard Hospital of Yokohama release. therapies (table 1). Informed consent was City University, (J Neurol Neurosurg Psychiatry 2000;68:214–217) obtained from the patients or their parents. Yokohama, Japan The diagnosis of myasthenia gravis was based A Komiyama Keywords: myasthenia gravis; extraocular muscles; on accepted clinical findings, positive re- on October 2, 2021 by guest. Protected copyright. corticosteroids; direct facilitatory e ect Department of V sponses to edrophonium chloride, and raised Neurology, Chiba University School of Medicine, Chiba, Table 1 Responses to treatment with high dose IV methylprednisolone Japan H Arai Responses after the last M Kijima Early responses† infusion No of K Hirayama Patient No/ Age Duration of Preinfusion infusion Transient Initial (y)/sex disease MG score* courses improvement worsening 1 week 1 month Correspondence to: Dr Atsushi Komiyama, Generalised myasthenia gravis: Department of Neurology, 1/29/F 10 months 13 5 11 (1–2 days) 19 (3–8 days) 7 3 Hiratsuka Kyousai Hospital, 2/48/M 10 months 15 5 14 (1–2 days) 18 (3–5 days) 8 5 9–11 Oiwake, Hiratsuka 3/14/F 2 y 2 months 9 3 ND 12 (2–5 days) 8 5 254–0047, Japan Ocular myasthenia gravis: email Komiyama@ 4/4/F 7 months 4 2 1 (1.5–5 h) 5 (3 days) 1 0 kkr.hiratsuka.kanagawa.jp 5/11/F 6 y 4 2 ND ND 1 1 6/57/F 2 months 5 3 3 (2–5 h) ND 1 0 Received 12 March 1999 and in final form *For calculation of clinical score, grades of 10 items with four-point score are added in 7 September 1999 generalised patients and those of two items in ocular patients. Accepted 17 September †The best or worst clinical score is shown. Paretheses indicate when clinical changes occurred. 1999 ND=not detected. Extraocular muscle responses to methylprednisolone in myasthenia gravis 215 J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.68.2.214 on 1 February 2000. Downloaded from Table 2 Listing of clinical score for patients with myasthenia gravis Weakness grade None Mild Moderate Severe 01 2 3 Test items: (A) Muscles of limbs and trunk: Test items (1) Arms outstretched (90°, sitting) (s) >240 90–240 10–90 <10 (2) Leg outstretched (45°, supine) (s) >100 30–100 0–30 0 (3) Head lifted (45°, supine) (s) >120 30–120 0–30 0 (4) Grip strength (dynamometer) <15 15–30 30–75 >75 (Decrement after 10 maximal closure) (%) (5) Vital capacity (l) 3.5 (men) 2.5–3.5 1.5–2.5 <1.5 2.5 (women) 1.2–2.5 1.2–1.8 <1.2 (B) Oropharyngeal muscles: (6) Facial muscles Normal Mild weakness on lid closure, Incomplete lid closure No mimic expressions snarl (7) Chewing Normal Faitgue on chewing solid foods Only soft foods Gastric tube (8) Swallowing Normal Fatigue on normal foods Incomplete palatal closure nasal Gastric tube speech (C) Extraocular muscles: (9) Palpebral muscles Normal Mild ptosis Apparent ptosis with fatigability Complete ptosis (10) Rectus/oblique muscles Normal Diplopia without detectable Apparent limitation Fixed eyes limitation concentrations of anti-AChR antibody.8 Diag- Results nosis of ocular myasthenia gravis in seronegat- Before the infusion, no obvious fluctuations in ive patients 4 and 5 also was supported by their clinical severity were detected. From day 1 to positive responses to a cold test9 and an day 2 after the initial infusion, two of the three “enhanced ptosis” manoeuvre.10 Thymoma patients with generalised myasthenia gravis was present in patients 1, 2, and 6. All patients (patients 1 and 2) experienced a slight with generalised myasthenia gravis had experi- improvement, which was reflected in a minimal enced a myasthenic crisis over the previous reduction in the myasthenia gravis scores of several months, and had undergone extended palpebral muscles and vital capacity (table 1). thymectomy and plasmapheresis. Despite re- However, from day 3 a marked weakness ceiving oral prednisolone (30 to 100 mg/ requiring artificial ventilation developed (table alternate days) and azathioprine (75 to 100 1, figure A), whereas only mild ptosis recurred. mg/day), their persistent, moderate clinical Despite initial worsening, however, titres of symptoms continued. However, their ocular anti-AChR antibody decreased (figure A). As signs were minimal to mild. The three patients the second course began, the exacerbated with ocular myasthenia gravis had been placed symptoms tended to improve; patient 1 was on oral prednisolone (30 to 60 mg/alternate weaned from ventilator support. Thereafter, a days) with or without pyridostigmine but failed similar but less severe worsening occurred as to recover from severe ptosis and ocular immo- the treatment course advanced. Patient 2 bility. In all but patient 1, pyridostigmine had recovered from supported ventilation after been discontinued due to crisis (patients 2 and completion of his third course. Patient 3 did 3) or lack of benefit (patient 6). not experience any transient improvement; her symptoms worsened 3 or 4 days after each http://jnnp.bmj.com/ TREATMENT PROTOCOL treatment, but the degree of worsening became Methylprednisolone sodium succinate (30 mg/ less severe. Their symptoms began to alleviate kg/day (maximal dose at 1 g/day)) was infused 5 or 6 days after the last infusion and this intravenously in 500 ml normal saline solution improvement continued for 5 to 22 months over 3 hours for 3 consecutive days, followed by a 4 day infusion free period. This course was after treatment. Along with the clinical allevia- repeated three to five times in patients with tion, titres of anti-AChR antibody decreased in patients 1 and 2. generalised myasthenia gravis and two to three on October 2, 2021 by guest. Protected copyright. times in patients with ocular myasthenia gravis. By contrast, two of the three patients with To avoid any possible symptomatic alterations, ocular myasthenia gravis (patients 4 and 6) previous medications were continued in all exhibited immediate but short lived ameliora- patients. tion of ptosis and ophthalmoparesis that began 90 minutes to 2 hours after initiation of the first CLINICAL MUSCLE TESTING (MYASTHENIA GRAVIS infusion (table 1, figure B). This steroid SCORE) induced alleviation of ocular signs lasted about Muscle weakness and fatigability were graded 3 hours, followed by a gradual return to the on a four point scale modified from the report original condition (patient 6) or mild worsen- by Besinger et al (table 2).11 For calculation, the ing at day 3 (patient 4). Ptosis and ocular scores of all 10 items were totalled in patients immobility responded similarly to intravenous with generalised myasthenia gravis, and two methylprednisolone. After treatment with items in patients with ocular myasthenia gravis. methylprednisolone, their ocular symptoms All the patients were assessed by the one gradually improved; however, they did not observer (AK). All muscle tests were carried experience similar immediate improvement out around 800 am, before morning medi- with subsequent infusions. Patient 5 gradually cation, except for changes in ocular
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