Acute myeloneuropathy: An uncommon presentation of Sjögren's syndrome
A 40-year-old female presented to our hospital with complaints of diffuse low intensity pain in all four limb since 20 days. She also noticed tingling sensation in all four limbs, which started distally and progressed proximally within 24 h. Simultaneously she noticed weakness in all four limbs, which started proximally and progressed to distal muscles over 24-30 h in all 4 limbs. She also complained of dysuria, urinary retention and incomplete emptying of the bladder. She had a history of gritty sensation in both eyes and dryness of mouth for last 8 months, for which she had to repeatedly take sips of water while having her food. Clinical examination revealed normal higher mental functions and cranial nerves. Hypotonia was noted in all four limbs. Power was Medical Research Council (MRC) grade 3/5 proximally and MRC grade 2/5 distally in both upper and lower limbs. Both superficial reflexes and deep tendon jerks were absent. Sensory examination was normal. No neck rigidity, local spinal deformity or nerve thickening was noted.\nInvestigations showed normal complete hemogram blood sugars (fasting and post-prandial), serum Vitamin B12 levels, thyroid function tests, renal function tests, enzyme-linked immunosorbent assay for human immunodeficiency virus, hepatitis B surface antigen and anti- hepatitis C virus tests. Both erythrocyte sedimentation rate and C-reactive protein were raised 24 mm at the end of 1 h and 19 mg/L respectively. Liver function tests were normal except serum albumin was low (1.9 g/dl) with reversal of globulin albumin ratio. Serum protein electrophoresis was normal. Magnetic resonance imaging (MRI) spine revealed diffuse T2 hyperintensity throughout the cord []. The nerve conduction studies revealed sensorimotor axonal polyneuropathy with absent F and H reflex []. Cerebrospinal fluid (CSF) analysis revealed mild pleocytosis with total cells of 20, predominantly lymphocytes (95%), CSF protein was raised (50 mg/dl) and CSF sugar was normal. CSF analysis for tuberculosis-polymerase chain reaction and virology (Epstein-barr virus, JE, cytomegalovirus and herpes simplex virus) was negative. Anti-nuclear antibodies were raised in the titre of 1:100. Anti-SS-A was positive, but anti –Sm was negative. Rheumatiod factor and anti-cyclic citrullinated peptide were negative. Histopathology of sural nerve biopsy showed mild perineural fibrosis, focal foamy changes in endoneurium with mild lymphocytic infiltration []. Wade Fite stain for Mycobacterium leprae was negative. Schirmer's test was positive in both eyes (2 mm/5 min in the right eye and 3 mm/5 min in the left eye). Patient didn’t give consent for salivary gland biopsy. She was treated with IV methylprednisolone 1 g/d for 5 days followed by oral prednisolone 50 mg/day. She received anti-cholinergic medications for urinary incontinence. The clinical improvement was seen in the patient with power improvement to MRC grade 4+ in proximal as well as distal muscle groups in all 4 limbs. Follow-up MRI study demonstrated normalization of altered signals on the spinal cord []. However, urinary incontinence persisted and subsequent uroflowmetry showed hypocontractile bladder with low compliance, which partly improved with anticholinergic drugs.