Cerebellar ataxia as a first manifestation of systemic lupus erythematosus
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LETTER TO THE EDITOR
Cerebellar ataxia as a first manifestation of systemic lupus erythematosus Mansur A. Kutlubaev1 · Rimma F. Idrisova2 · Elvira N. Zakirova3 · Todd A. Hardy4,5 Received: 25 March 2020 / Accepted: 12 May 2020 © Belgian Neurological Society 2020
Dear Sir, Adult-onset cerebellar ataxias can be a diagnostic challenge. Cerebellar ataxia can occur rarely in systemic lupus erythematosus (SLE) but it is very rare for it to occur as the presenting symptom [1]. The following case emphasizes the importance of an early diagnosis of this severe but potentially treatable cause of cerebellar ataxia. A 52-year-old woman presented with a 2-year history of fatigue and unsteadiness. There was no relevant past medical or family history. Neurological exam revealed direction changing nystagmus, moderate scanning speech dysarthria, severe truncal instability, and bilateral cerebellar incoordination, dysmetria, and intention tremor. She had brisk deep tendon reflexes in both the upper and lower limbs with upgoing plantar responses without muscle weakness or sensory findings. She could take only a few steps with bilateral support and started using a wheelchair a month later. Routine blood tests, TSH, ACE, HIV, HBV and syphilis were normal/negative. Chest X-ray was normal. Cerebral * Mansur A. Kutlubaev [email protected] Rimma F. Idrisova [email protected] Elvira N. Zakirova [email protected] Todd A. Hardy [email protected] 1
Department of Neurology, Bashkir State Medical University, Ufa, Russia
2
Republican Cardiology Center, Ufa, Russia
3
Department of Neurology, G.G. Kuvatov Republican Clinical Hospital, Ufa, Russia
4
Brain and Mind Centre, University of Sydney, Camperdown, NSW, Australia
5
Neuroimmunology Clinic, Concord Repatriation General Hospital, Sydney, NSW, Australia
MRI revealed mild, diffuse cerebellar atrophy with no evidence of focal pathology or specific findings such as hot cross bun sign (Fig. 1). CSF examination showed a mononuclear cell count 15 × 10 9/L (≤ 5), protein 0.8 g/L (0.15–0.45), glucose 3.0 mmol/L, oligoclonal bands were positive. She was treated for presumed autoimmune cerebellitis with methylprednisolone 1000 mg for 5 days followed by prednisolone 1 mg/kg daily tapering over 2 weeks. She was also diagnosed with chronic, minimally active hepatitis C and managed conservatively. A whole-body 18F-FDG PET–CT revealed no metabolically active lesions. Screening for GAD, antiphospholipid and onconeuronal autoantibodies (Hu, Yo-1, CV-2, PNMA2, Ri, amphiphysin), ANCA, Borrelia, varicella zoster, and HSV was negative. She developed arthralgia, fever, weight loss, alopecia and mild lupus dermatitis 3 months after neurologic presentation. Blood tests showed leucopenia (3.6 × 109/L), thrombocytopenia (91 × 109/L), elevated ESR (40 mm/h), positive ANA 1:640 granular type (
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