[Systemic lupus erythematosus with affection to brainstem: report of three cases]

The frequency with which the central nervous system (CNS) is affected by systemic lupus erythematosus (SLE) varies, according to different series, between 13 and 59%, whereas the brain stem is affected in 5%.

Case 1: a 33 year old male who was submitted to a Nissen funduplicature as a treatment of (hypo)incoercible hiccups. The singultus persisted and some time after a paraplegia appeared. Magnetic resonance (MRI) showed images in the medulla oblongata, and in the cervical and thoracic spine. A biopsy was also performed to examine the cervical lesions and vasculitis was diagnosed. The patient began treatment with prednisone (1 mg/kg) and two months after symptoms had begun to improve he presented an episode of bilateral optic neuritis. Until this last event, the immunological studies had been positive. Case 2: female aged 19 who had had SLE for eight months. The illness began suddenly with bilateral paralysis of the sixth cranial nerve, vertical and horizontal nystagmus, dysdiadochokinesia, truncal ataxia, 4/5 muscular strength in the upper limbs and 3/5 in the lower limbs, and left flexor plantar response, but indifferent on the right hand side. MR showed T2 hyperintensities in the pons, medulla oblongata and the junction of medulla and upper spinal cord. Case 3: female aged 31 with sudden onset of the illness, characterised by diplopy and presence of internuclear ophthalmoplegia. Brain MR showed images of T1 hypointense and T2 hyperintense in the pontobulbar region.

A brain stem disorder in patients suffering from SLE is one of the rarest manifestations of this pathological condition of the CNS and is probably caused by vasculitis