Magnetic resonance imaging-negative myeloneuropathy and bilateral facial paresis unfurling systemic lupus erythematosus

Abstract

Systemic lupus erythematosus is a chronic autoimmune connective tissue disorder that can affect all the neuroaxes in the central and peripheral nervous systems. Myelopathy in systemic lupus erythematosus is one of the least common neuropsychiatric syndromes accounting for 1%–2% of cases. Myelopathy has long been diagnosed based on clinical findings, laboratory tests, and gold-standard gadolinium-enhanced magnetic resonance imaging (MRI). MRI-negative myelopathy is a recently described subset of myelopathies. Here, we report the case of a young woman from rural West Bengal, India, who presented with overlapping features of white-matter and gray-matter myelopathy associated with peripheral neuropathy and bilateral asymmetric lower motor neuron-type facial paresis. The historical analysis yielded clues toward an etiological diagnosis of systemic lupus erythematosus, further substantiated by seropositivity of lupus-specific autoantibodies. Her neurological disabilities responded poorly to oral administration of hydroxychloroquine, bolus intravenous administration of methylprednisolone, and high-dose cyclophosphamide therapy but eventually responded remarkably well to cyclical rituximab therapy. This case adds to the tally of cases of MRI-negative lupus myelopathy. MRI-negative myelopathy in systemic lupus erythematosus can be easily missed if not meticulous attention is paid during clinical history taking and examinations.

Persistent “MRI-negative” lupus myelitis-disease presentation, immunological profile and outcome

“…UMN-related bladder symptoms following a cord injury share great similarity with symptoms of urinary tract infection, and sensory symptoms are often vague and non-specific ( 14 , 24 , 25 ). The diagnostic dilemma of myelitis becomes compounded in the absence of correlating MRI findings ( 8 , 26 ). Thus, diagnosis of MRI-negative lupus myelitis is often difficult, and only meticulous history taking and clinical examination with a low threshold of suspicion can help identify this entity.…”

Approach to non-compressive myeloneuropathy through a rendezvous of 11 cases from an Indian backdrop

“… 5 Co-presence of sensory symptoms involving the upper and lower extremities and other myelopathic symptoms will forewarn the clinician regarding concomitant myeloneuropathy. 6 Like the sensory symptoms, weakness ascribable to peripheral neuropathy also commences from the distal extremities and accompanies atrophy. Whereas other common sensory symptoms like “plaster-cast” sensation, dysesthesias (allodynia, hyperalgesia, or profound loss of sensations), “Lhermitte sign”, girdle-like sensation, and root-pain, when present, are suggestive of myelopathy.…”