A RARE NEUROLOGICAL PRESENTATION OF SLE Dr Jayachandra Dr Yoganand M N Dr Prithvi P Nayak Presenter: Dr Shambhavi K R
CHIEF COMPLAINTS A 30 year old lady hailing from Nepal presented to OPD with complaints of Low grade fever since 6 months Swelling and joint pain since 6 months No history of cough, weight loss, night sweats
PAST HISTORY No similar complaints in the past No history of abortions No history suggestive of TIA
TREATMENT HISTORY Patient had visited multiple hospital with these complaints in Nepal and Northern India She was treated symptomatically But her fever and joint pain persisted
ON EXAMINATION She was moderately built and poorly nourished PR- 100 bpm BP- 110/70 mmhg Temperature- 100.1 F Pallor + Swollen tender joints + which was symmertrical and predominantly involved small joints of hands,knees and wrists
Sparse hair was present
Malar rash was present Nasolabial folds were spared
No clubbing, cyanosis, icterus, lymphadenopathy or edema Cardivascular system- S1 S2 heard no murmurs Respiratory system- normal vesicular breath sounds Central nervous system- no focal neurological deficits Per abdomen- no hepatospleenomegaly
CLINICAL DIAGNOSIS Pyrexia of unknown origin?autoimmune?sle
INVESTIGATIONS: Hb - 9.7g/dl Total counts -3100cells/mm3 Platelet - 1,21,000 Peripheral smear - normocytic and normochromic anaemia Reticulocyte count-1.2 ESR- 78mm/h HsCRP-12.28
LFT- normal RFT- normal Urine routine- albumin +,no pus cells, no RBCs, no casts Chest X ray- normal HIV negative HbsAg, HCV- negative 2DECHO- normal Blood, urine and sputum cultures- negative ANA screening- positive 2+ Lipid profile- normal
ON FURTHER WORK UP RA factor- negative 24 hour urine protein- 432mg ANA profile- dsdna +, anti smith -ve C3, C4- low APLA profile- negative DCT,ICT- negative
DIAGNOSIS SYSTEMIC LUPUS ERYTHEMATOSUS
SLEDAI-2K MILD- <6 MODERATE- 6-12 SEVERE- >12
TREATMENT She was started on analgesics Tab hydroxychloroquine 200mg Inj methylprednisolone 1gm for 3days Tab prednisolone 40mg OD Tab enalapril 2.5mg OD With this patient improved, fever and joint pain subsided
ON DAY 7 Patient developed foot drop on right side As it involved only common peroneal nerve Mononeuropathy was considered
Foot drop on right side
ON DAY 10 Patient developed sudden onset of weakness in bilateral lower limb Loss of sensation below axilla Unable to sense bowel and bladder No H/O band like sensation or back pain
On examination Power- 0/5 in bilateral lower limbs Tone -Hypotonia Reflexes- absent Bilateral plantar- mute Sensory system- complete loss of all modalities of sensation below T2
DIFFERENTIALS Guillain-Barre syndrome Transverse myelitis Multiple sclerosis Inflammatory secondary to SLE Haemorrage- AV Malformation,epidural haematoma Demyelination Infarction
Transverse myelitis secondary to SLE was the clinical suspicion As it is the most common cause of paraplegia in SLE and clinical examination revealed pan cord involvement
FURTHER WORK UP Lumbar puncture : Cell count 3 Cell type lymphocytes Protein 30 Sugar - 45 NORMAL
To rule out other causes MRI brain and spine was done MRI Brain- normal MRI angio spine o Anterior spinal artery infarction at the level of C7 to T2 o Cord edema
MRI angio showing anterior spinal artery infarction with thrombus at C7 level
FINAL DIAGNOSIS ANTERIOR SPINAL ARTERY INFARCTION SECONDARY TO SLE INDUCED VASCULITIS
TREATMENT Patient was pulsed with low dose cyclophosphamide Started on antiplatelets Anti edema measures were given Steroids and HCQs continued Physiotherapy was given
WHY POSTERIOR CORD INVOLVEMENT IN ANTERIOR SPINAL ARTERY INFARCTION? Cord edema Innermost part of dorsal columns that belongs to central territory of anterior spinal artery may be involved Additional lesion in parts of posteriolateral column If posterior column signs are present in anterior spinal artery infarction, it says that the infarction is more extensive and the prognosis is poor
COURSE IN HOSPITAL Patient improved with immuno suppression Power in lower limb improved to 2/5 over 3 weeks She could sit up with support Patient was discharged But lost to follow up
DISCUSSION Systemic lupus erythematosus a systemic inflammatory disease and affects 0.1% of the global population The incidence of stroke in 5-18% in SLE patients It is very rare and accounts for only <1% of all strokes in the general population No data on incidence of spinal cord infarction in SLE patients
The pathogenesis of cerebrovascular disesase could be primary or secondary primary causes being vasculitis, specific antineuronal antibodies, lupus anticoagulant rarely libman sacks endocarditis secondary causes could be hypertension,chronic kidney disease
Association with APLA was the most common cause of cerebrovascular accidents in SLE Vasculitis causing infarcts leading to CNS manifestations in SLE and accounts for only 7%.
WHY IS THIS CASE RARE????? Anterior spinal srtery infarction is rare and represents a diagnostic challenge Posterior column involvement is rare in anterior spinal cord infarction APLA is the most common cause of thrombosis in SLE but it is negative
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