|LETTER TO EDITOR
|Ahead of print publication
Neuropsychiatric lupus: A perpetual quandary
Vaibhav Deorari, Sujay Halkur Shankar, Neha Chopra, Prabhat Kumar
Department of Medicine, All India Institute of Medical Sciences, New Delhi, India
Assistant Professor, Department of Medicine, All India Institute of Medical Sciences, New Delhi - 110 029
Source of Support: None, Conflict of Interest: None
Neuropsychiatric manifestations in systemic lupus erythematosus (NPSLE) are frequently encountered with a prevalence of almost 35%–90%. Herein, we present a case of male lupus who had IV cranial nerve palsy and peripheral neuropathy simultaneously.
An 18-year-old male was diagnosed to be having NPSLE 2 years back, when he had presented with complaints of acute-onset bilateral foot drop and polyarthritis. He was treated with steroids and mycophenolate. He responded well to the treatment, but he discontinued his medications after 1 year. This time, he presented to us with complaints of intermittent fever for 3 weeks along with bilateral ankle joint pain and weakness in the lower limbs for 2 weeks. On neurological examination, he had symmetrically decreased power in bilateral lower limb and was associated with sensory loss over dorsum of bilateral feet. During hospital stay, he started complaining of monocular diplopia and ocular movement examination was suggestive of right superior oblique palsy. Musculoskeletal system examination showed mild synovitis with tenderness in both ankle joints.
Blood investigation showed normal hemogram with raised erythrocyte sedimentation rate levels. Renal and liver function tests and urine examination were normal. Antinuclear antibody was positive and anti-dsDNA was also positive with a titer of 382 IU/ml (normal: 0–100). Serum protein electrophoresis, thyroid function test, antiphospholipid antibody, Vitamin B12, and complement levels were normal. Cerebrospinal fluid analysis showed 10 mononuclear cells with protein of 170 mg/dl and sugar of 58 mg/dl. Nerve conduction study showed axonal pattern of sensorimotor polyneuropathy involving all limbs. Nerve biopsy was planned, but consent for the same was not given. Magnetic resonance imaging brain showed diffusion restriction in the left basal ganglia and left midbrain posterior to substantia nigra and T2 flair hyperintensity in the same region, which was suggestive of vasculitic infarct [Figure 1]. A final diagnosis of NPSLE (cranial mononeuropathy with peripheral neuropathy) was made after ruling out other differential such as toxin and monoclonal gammopathy-associated neuropathies. He was given 3 days of methylprednisolone pulse therapy, followed by 1 mg/kg of oral prednisolone. He also received monthly intravenous cyclophosphamide (500 mg/m2) for 6 months. He showed considerable improvement in his symptoms, fever, and polyarthritis resolved immediately. After 1 month, his cranial nerve palsy improved and peripheral neuropathy resolved in 3 months, except residual weakness of bilateral extensor hallucis longus muscle.
|Figure 1: Magnetic resonance imaging brain showing T2 flair hyperintensity in the left midbrain|
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The American College of Rheumatology identifies 19 neuropsychiatric conditions in SLE, of which 12 are of central nervous system (CNS) and 7 of peripheral nervous system. The commonly observed manifestations of NPSLE are headache (28.3%), mood disorders (20.7%), cognitive dysfunction (19.7%), peripheral neuropathy (10%–15%), seizures (9.9%), and cerebrovascular accidents (8%). CNS vasculitis is rare and is seen in only 1% of SLE patients.
Our case is unique because neurological involvement in male lupus is less frequently seen. Further, to have both cranial mononeuropathy and peripheral neuropathy simultaneously in NPSLE is even rarer. Ocular movement disorders in SLE are a frequent finding and commonly involved nerves are the sixth and third cranial nerves. Isolated involvement of trochlear nerve resulting in superior oblique palsy due to midbrain vasculitis has never been reported in SLE. The suggested mechanism for CNS vasculitis in SLE is interaction of intrathecal immune complex and inflammatory cytokines with vascular endothelium. Similarly, peripheral neuropathy in SLE results from immunological reaction to nerve tissue. To conclude, NPSLE is a conundrum and can have wide array of manifestations. It is prudent for the clinicians to look for subtle neurological signs and treat them aggressively.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
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Conflicts of interest
There are no conflicts of interest.
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