Lagophthalmos and Ptosis in Inclusion Body Myositis

Abstract

Sporadic inclusion body myositis is the most commonly acquired type of idiopathic inflammatory myopathy in people aged 50 and above. There is early weakness and atrophy of forearms and quadriceps and a third of patients also have mild facial weakness. Extraocular muscles are not affected and ptosis is rarely seen. The authors describe a unique case in which inclusion body myositis presented with early mid face weakness and atrophy resulting in unilateral lagophthalmus, and ptosis, which have not been documented before. This case is not only unique in its presentation but also emphasizes the importance of considering differential diagnoses and conservative measures before contemplating surgery.

Lagophthalmos as a presenting sign in dermatomyositis with muscle involvement limited to the ocular muscles

“…One case described what the investigators claim as the first report of left-sided lagophthalmos, right-sided ptosis, and complaints of epiphora and eye irritation in a patient later diagnosed with inclusion body myositis, an inflammatory myopathy. 9 Our patient clearly differs from the above finding as she does not have inclusion body myositis. Another case mentioned lagophthalmos in a patient with DM with muscle inflammation.…”

Bilateral Facial Palsy: A Rare Presenting Symptom for Inclusion Body Myositis

“…15 A single previous report documented findings of asymmetric orbicularis oculi weakness and unilateral ptosis with facial weakness and e109 muscle atrophy leading to eye symptoms before the development of the classical pattern of weakness seen in IBM. 9 Other reports have documented facial asymmetry with asymmetric midface weakness years after the presentation of the classic pattern of muscular weakness. 16 One report similarly documented the onset of facial plegia prior to the onset of typical muscular weakness; however, progression of facial weakness occurred over the course of 6 years in that case with primary initial involvement of the temporalis muscles, symmetrical midface weakness, and neck flexor weakness in the absence of ptosis or ocular abnormalities.…”

“…Given lack of muscle biopsy, the patient did not meet diagnostic criteria according to Griggs et al 2 However, per the European Neuromuscular Center IBM diagnostic criteria, this patient met the mandatory features of age of onset later than 45 years old, duration of symptoms more than 12 months, and serum creatine kinase level not more than 15 times upper limit of normal, as well as the clinical features of weakness of quadriceps more than hip flexors and weakness of finger flexors more than shoulder abductors. 9,11 In recent years, anti-cN-1A antibody testing has become an increasingly popular method to diagnose IBM via a blood test, with additional clinical associations of disease manifestations (presence of anti-cN-1A antibodies associated with greater risk of more severe dysphagia). 12 However, the sensitivity (33-76%) and specificity (87-100%) of such antibody testing vary in different studies.…”

“…4,9 Muscle fibers may appear atrophic with interfascicular fibrosis and surrounding rimmed cytoplasmic and subsarcolemmal vacuoles. 4,9 The current standard of treatment focuses on minimizing the inflammatory and atrophic features with immunomodulatory therapy. However, IBM frequently is refractory to most treatments thought to be effective in other inflammatory myopathies, including prednisone, β-interferon-1a, methotrexate, tumor necrosis factor blockers (e.g., etanercept), and monoclonal antibodies (e.g., alemtuzumab).…”

Physical therapy improves motion in a patient with inclusion body myositis - a case report

“…IBM is characterised by chronic, progressive weakness and atrophy of skeletal muscles, especially distal parts of the upper and proximal parts of lower extremities, sometimes followed by the weakness of facial muscles and dysphagia 2,8 . Since muscle weakness is the main clinical symptom, the diagnosing of IBM often lasts for a long time due to rare occurrence of the disease.…”